Cleft Ultimate Treatment

CLEFT ULTIMATE TREATMENT Oro-facial and Cranio-maxillo-facial Deformities

SECOND EDITION

Kurt Bütow and Roger Zwahlen

Copyright © 2016 Kurt Bütow and Roger Zwahlen 2nd edition 2016 All rights reserved. No part of this book may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopying, recording or any information storage or retrieval system without permission from the copyright holder.

The Author has made every effort to trace and acknowledge sources/resources/individuals. In the event that any images/information have been incorrectly attributed or credited, the Author will be pleased to rectify these omissions at the earliest opportunity.

ISBN 978-0-620-67204-7 eISBN 978-0-620-67208-5 Published by Author using Reach Publishers’ services, P O Box 1384, Wandsbeck, South Africa, 3631 Edited by Rendale Snow for Reach Publishers Cover designed by Reach Publishers Printed and bound by Mega Digital Printers Website: www.reachpublishers.co.za E-mail: reach@reachpublishers.co.za

ULTIMATE RESULT

ULTIMATE RELATED TO AESTHETIC APPEARANCE • Soft tissue - scar and balanced lip + nose appearance • Mid-facial bony structure support • Aesthetic dental appearance(s)

ULTIMATE RELATED TO FUNCTIONAL ABILITY • Speech competency • Occlusal function

ULTIMATE RELATED TO WHAT THE SOCIETY SHALL ACCEPT AS NORMAL

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Professor Kurt Bütow BSc, BChD, MChD(MFOSurg), DrMedDent, PhD, DSc(Odont), FCMFOS, FIBCSOMS Professor for Cleft Lip and Palate and Cranio-maxillofacial Surgery University of Pretoria Professor and Head Emeritus in Department of Maxillo-Facial and Oral Surgery, University of Pretoria, South Africa Professor Kurt Bütow has received the following degrees: BSc[Chemistry and Botany-Biochemistry], BChD, MChD[in Maxillo‑facial and Oral Surgery], DrMedDent, PhD, DSc[Odont], FCMFOS (Colleges of Medicine of South Africa fellowship[pr]), FIBCSOMS[cr] and Fellowships (LFIBA and FABI) by invitation. He is a Maxillofacial and Oral Surgeon who holds three doctoral degrees. In August 1983 he was appointed Professor/Chief Specialist in Maxillofacial and Oral Surgery and in October 1984 as Head of the Department at the University of Pretoria and Honorary Consultant of the I Military-Hospital and retired in April 2013 as Head of the Department. He has been a fractional Research Professor and presently a Honorary Professor at the College of Health Sciences, University of KwaZulu-Natal and part-time temporary Adjunct-Professor of the University of North Carolina, USA. Visiting Professor and Guest Surgeon in Jerusalem, Israel and Honour Professor in Hong Kong. He is the Past-President of the College of Maxillofacial and Oral Surgery (CMFOS) of the Colleges of Medicine of South Africa (CMSA) and Director of the Senate of the International Board for Certification of Specialist in Oral-maxillofacial Surgery (IBCSOMS). The Facial Cleft Deformity Clinic, the largest in Africa with more than 4300 patients, was established by him in 1983.

He was a International Program Director for the IAOMS-Fellowship program in Cleft Lip and Palate and Cranio-maxillofacial Surgery for 3 years. He has published more than 360 scientific papers and abstracts (more than 130 on cleft treatment), including six books (3 on cleft treatment) and presented more than 390 extra-curricular lectures, nationally and internationally in 18 countries. Various awards and honours have been conferred on him, examples: Top Achiever of the University of Pretoria (7X), Commemorative Research Medal University of Pretoria’s Centenary Celebration for honouring Leading Minds, International Cleft Palate Foundation-Lifetime Achievement Award, Distinguished International Fellow (IAOMS ), and has been cited in the Transaction (J of CMSA), South African Dental Journal (2X), Who’s Who in Medicine - Health Care, Who’s Who of Intellectuals and Who’s Who in the World.

KURT-W. BÜTOW – Abridged auto-biographical sketch. Reach Publishers. 2014: ISBN 978-0-62-TBC; eISBN 978-0-620-TBC. reach@webstorm.co.za

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Professor Roger Zwahlen MD, DMD, PD, FMH, FEBOMFS Professor for Oral and Maxillofacial Surgery, University of Hong Kong, China; Honorary Professor Department of Oral and Maxillofacial Surgery, Shenzhen Hospital, Peking University, Shenzhen, China; Visiting Professor, Department of Head and Neck Surgery, Sun Yat-sen Cancer Centre, University of Guangzhou, China; Guest Professor, School of Stomatology, Wuhan University, China; Private Docent, Division of Cranio-maxillofacial and Oral Surgery, University of Zurich, Switzerland Professor Roger A. Zwahlen has had his training in General Surgery at two regional hospitals in Switzerland, his Cranio-maxillofacial and Oral Surgery training at the University of Zurich and his Advanced Cleft Surgery Training at the University of Pretoria. He received the following degrees: M.D. (Bern), D.M.D. (Zurich). He obtained both the Swiss specialist physician title (FMH) and the European Fellowship (FEBOMFS) in Oral and Maxillofacial Surgery. He was awarded with the highest academic degree in German speaking Universities, the Private Docent (PD) in the Division of Cranio-maxillofacial and Oral Surgery at the University of Zurich. He is an appointed Examiner of the International Board of Certification of Specialists in Oral and Maxillofacial Surgery [IBCSOMS]. In March 2011 he was appointed Full Professor with Tenure in Oral and Maxillofacial Surgery at the Faculty of Dentistry of the University of Hong Kong, where he further serves as the Undergraduate Programme Director of Oral and Maxillofacial Surgery and in various senior management committees. Professor Zwahlen is associated with five national and international Professional Bodies, has published more than 140 scientific papers/abstracts, including four chapters in books, and presented various lectures and courses, nationally and internationally in countries such as South Africa, Malaysia, Singapore, Vietnam, Thailand, People’s Republic of China, Germany, France, Italy, Spain and Switzerland. Various awards and honours have been conferred on him, as the Award for Excellent Clinical and Scientific Activity and Assistance in the Formation of Dentists from the Clinic for Dental, Oral and Maxillofacial Diseases and Cranio-maxillofacial and Oral Surgery, International Who’s Who in Medicine and Health Care, Who’s Who of Intellectuals and Who’s Who in the World. Due to his excellent clinical work in Hong Kong, he was awarded with monetary donations by Sir Gordon Wu. He is interested in soccer, ice-hockey and rugby and enjoys travelling to Italy.

He speaks German, English, Italian and French.

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PREAMBLE Book 1: TREATMENT OF FACIAL CLEFT DEFORMITIES AN ILLUSTRATED GUIDE It is stated on page iii: DEDICATED To the Child born with a facial cleft deformity and to the Team Members, Academicians and Practitioners treating these children

Book 2: FACIAL CLEFT DEFORMITIESThe Comprehensive Treatment It is stated on page 5: DEDICATED To the Postgraduate Student, in the sub-specialisation/fellowship program, who is dedicated to obtaining and applying, through his/her special ability, the knowledge of and insight into treatment of these neonates, babies, children, adolescents and adults born with a facial cleft deformity

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Very important notice Medicine and Dentistry is an ever-changing science. As new research and clinical experience broadens the clinician’s knowledge, changes in treatment are constantly required. Every effort was undertaken to ensure that the treatment protocols and procedures presented here are as accurate as possible. In this book our own publications are prioritized and each of these publications includes an essential reference list for those who wish to read more on a particular aspect.

Worldwide, thousands of important publications have been written on cleft treatment. We try to push the boundaries to achieve the best possible clinical outcome with our research and know-how, but also humbly acknowledge that very important aspects in this book are based on knowledge and efforts of former scientists and clinicians. As this scientific field deals with the face and its features, it is often impossible to keep the eyes unrecognizable. Every chapter is about a different topic. However, as multiple problems may occur in the same patient, similar or the same photographs may appear in different chapters.

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Contents 1.

Introduction And Philosophy What Is Cleft Surgery?

History Of The Clinic Charity Or Missionary Surgery Ultimate Treatment

15 15 16 19 20

2.

Different Types Of Cleft Deformities Anatomical Classification Of Clefts (Tessier) Diagnostic Key

21 21 22

3.

Treatment Protocols From Neonate To Adult Cleft Protocols (4 Basic + 1 Specific Protocol)

35 37

4.

Functional Jaw-Orthognathic Plate Or Primary Orthopaedic Treatment

47

Primary Cleft Treatment

57

5.

59 60 63 86 90 92 93

Unilateral Cleft Lip A. Diamond Or Straight-Line Lip-Repair B. Anatomical Lip-Repair C. Millard Lip-Plasty D. Tennison Lip-Repair; Similar: Randall and Malek Unilateral Lip-Repair - The Ultimate - Clinical Pearls Unilateral Lip-Repair - The Ultimate Clinical Pearls, Continued

9

6.

Bilateral Cleft Lip A. Modified Broadbent Lip-Repair B. Noordhoff-Trott Lip-Repair Bilateral Lip-Repair - The Ultimate Clinical Pearls Bilateral Lip-Repair - The Ultimate Clinical Pearls, Continued

95 96 109 112 113

7.

Unilateral Cleft Nose Reconstruction / Primary Rhinoplasty Unilateral Primary Rhinoplasty – The Ultimate Clinical Pearls

115 121

8.

Bilateral Cleft Nose / Columella Lengthening Columella Lengthening (Primary Rhinoplasty) - The Ultimate Clinical Pearls

123 131

9.

Anterior Nasal Floor Anterior Nasal Floor - The Ultimate Clinical Pearls

133 142

10.

Hard Palate Cleft 143 A. Basic Surgical Repair 145 Releasing Incision (Von Langenbeck Type) 149 B. Advanced Surgical Repair 153 Hart Palate Repair - The Ultimate Clinical Pearls 158

11.

Soft Palate Cleft A. Basic Surgical Repair 1. Intravelar Veloplasty (Type ii) 2. Widmaier Veloplasty With Muscle Repair And Tensor Sling Procedure 3. Furlow Veloplasty With Tensor Procedure (Procedure Only In Narrow Clefts) and Parker-Furlow Plasty 4. Intravelar Veloplasty (Type iii) Intravelar Veloplasty (IVV)

10

159 160 161 171 173 181 182

5. Primary Cleft Repair for The Older Child And Adult B. Advanced Surgical Repair C. Tensor Sling Procedure Soft Palate Repair - The Ultimate Clinical Pearls Soft Palate Repair - The Ultimate Clinical Pearls, Continued

188 192 200 204 205

Primary And/Or Secondary Cleft Treatment

207

12.

Velopharyngeal Incompetency A. Fluorography B. Velopharyngeal Flap C. Dynamic Sphincter Plasty D. Palatal Distraction Osteogenesis Velopharyngeal Incompetency And Its Repair - The Ultimate Clinical Pearls

209 210 214 224 227 231

13.

Oro-Nasal Fistula A. Local Flaps B. Distant Flaps Oro-Nasal Fistulae And Their Repair- The Ultimate Clinical Pearls

233 234 242 248

Secondary Cleft Treatment 14.

249

Osteoplasty/Osteofusion 251 A. Unilateral Dento-Alveolar Cleft 252 B. Bilateral Dento-Alveolar Cleft 256 C. Bone Harvesting 258 Osteoplasty/Osteofusion – The Ultimate Clinical Pearls 260

11

Osteoplasty And Alar Base Augmentation - The Ultimate Clinical Pearls, Continued

261

15.

Orthognathic Treatment (Applicable For Facial Cleft Deformities) A. Cephalometry B. Maxillary Osteotomies C. Mandibular Osteotomies D. Osteotomies Followed By Osteodistraction Orthognathic Repair For Cleft Deformities - The Ultimate Clinical Pearls Orthognathic Repair For Cleft Deformities - The Ultimate Clinical Pearls, Continued Orthognathic Repair For Cleft Deformities - The Ultimate Clinical Pearls, Continued (2)

263 264 268 271 274 290 291 292

16.

Cleft Nose Reconstruction (Rhinoplasty) A. Unilateral Cleft Nose B. Advanced Unilateral Cleft Rhinoplasty C. Bilateral Cleft Nose Repair Of Cleft Nose Deformities- The Ultimate Clinical Pearls Repair Of Cleft Nose Deformities- The Ultimate Clinical Pearls, Continued

293 294 299 307 310 311

17.

Cleft Lip Revision Secondary Cleft Lip Deformities And Their Revision - The Ultimate Clinical Pearls

313 323

18.

Dental Implant In Cleft Alveolar Ridge Dental Implants In Cleft Patients - The Ultimate Clinical Pearls

325 333

12

Exceptional Clefts Primary And Secondary Treatment

335

19.

Pierre Robin Sequence Treatment Pierre Robin Sequence A. Siebold-Robin Sequence B. Fairbairn-Robin Triad Pierre Robin Sequence - The Ultimate Clinical Pearls Pierre Robin Sequence - The Ultimate Clinical Pearls, Continued

337 338 340 345 364 365

20.

Median Cleft Reconstruction With Agenesis Of Premaxilla-Prolabium Columella Surgical Procedures Median Clefts With Premaxilla-Prolabium-Columella Agenesis - The Ultimate Clinical Pearls

367 370 374

21.

Lateral Or Transverse Cleft A. Diagnosis And Classification B. Surgical Repair Lateral Facial Cleft - The Ultimate Clinical Pearls

375 376 382 393

22.

P63 Gene Mutation – Three Syndromes, Complications And Surgery A. Three Syndromes Linked To Gene P63 B. Peri-Operative Complications Aec – Rhs - Eec Cleft Associated With P63-Gene - The Ultimate Clinical Pearls

395 396 404 405

23.

Oblique Facial Cleft Repair Oblique Facial Cleft - The Ultimate Clinical Pearls

407 418

13

Tertiary Treatment “The Cleft Syndrome”

419

24.

421 422 426 437

Severe Cleft Disfigurement Or “Cleft Syndrome” A. Protocol For A “Cleft Syndrome” B. Multiple Interventions Severe Cleft Disfigurement - The Ultimate Clinical Pearls

Data And Tables

439

25.

Syndromes Associated With Cleft Deformities

441

26.

Cleft Data Collection And Research

443

27.

Treatment Tables

447

Epilogue 449

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-1Introduction And Philosophy What is CLEFT SURGERY?

Cleft surgery comprises – A) The execution of almost all types of cranio-maxillo-facial and oral surgical interventions in perfection, i.e.  Surgery:

- -

Soft tissue (always 3-dimensions) Hard tissue (in 2- and 3-dimensions)

and B) A comprehensive background knowledge of:

 Function:  Aesthetics:

- -

Speech, occlusion, nasal air flow, middle ear function and facial muscles Including different racial features

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Cleft - Ultimate Treatment

HISTORY of the CLINIC The first author was one of the first three Directors for the International Cleft Lip and Palate and Cranio-maxillofacial Surgery Training Fellowship Program designated by the International Association of Oral and Maxillofacial Surgeons (IAOMS). In the year 2000, the International Cleft Lip & Palate Foundation (ICPF) rated the Pretoria Clinic as “A Centre of Excellence”

Description of the Deformity The term “facial cleft deformity” comprises cleft lip and palate deformities, as well as clefts involving other facial structures not associated with a cleft lip or palate.

Referral and Case Numbers The University of Pretoria in conjunction with The Wilgers Hospital in Pretoria, represent the largest Facial Cleft clinic in Africa. It serves patients from all over South Africa, as well as from Namibia and Botswana as a reference centre.

Furthermore, it receives some patients from Angola, Zimbabwe, Zambia, Congo, Mauritius, Mozambique, Nigeria, Egypt, the Middle East, Europe, Canada, Uganda, USA and China. For more than 30 years, more than 4000 patients suffering from cleft lip and palate, cranio-maxillo-facial cleft deformities and speech problems have been treated and fully documented. As such, more than 8000 primary surgical and more than 5000 secondary interventions were performed.

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Introduction And Philosophy

Multidisciplinary Clinic versus Charity Surgery The Multidisciplinary Clinic Optimal treatment for children with a facial cleft deformity can only be achieved in a multidisciplinary setting. In such a setting various specialities and divisions constantly evaluate, examine, diagnose and treat patients according to treatment protocols.

Related to their congenital growth defects, patients with facial cleft deformities should receive the most appropriate treatment at the best point in time, in the neonatal period, toddler stage and childhood or during adolescence. It is expected that the patients together with their parents/care takers are reviewed by all the involved specialties and disciplines of the multidisciplinary clinic during their birthday week on a yearly or two-yearly basis.

The ideal system for the optimal treatment of facial cleft deformities involves: - - - -

A multidisciplinary setting Maintaining enthusiasm of recruited team key-players Community Nurses - most important health professionals of the clinic Surgeons, who: - Are able to treat every surgical aspect from child to adulthood, being familiar with primary, secondary and tertiary reconstructive procedures of facial soft tissue, facial hard tissue, intra-oral soft and intra-oral hard tissue defects. VERY IMPORTANT: Cleft surgeons needs to be comprehensively trained in all aspects of cleft surgery and care, being competent in every primary, secondary and tertiary cleft treatment.

BĂœTOW K‑W. Treatment of cleft lip and palate. Part I: Registration, documentation, case history and plan of therapy. J Dent Ass S Afr 1984: 39; 255.

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Cleft - Ultimate Treatment

- -

- Know and can advise their auxiliaries, in treating children by means of a jaw-orthognathic suction and drinking plate (= jaw-orthopaedic device) - Have an excellent feedback system with all involved disciplines - Know basic paediatric treatment regimens Paediatric anaesthetists Paediatric-trained nurses in a paediatric ward

Optimal treatment for children with facial cleft deformities can only be achieved in a multidisciplinary setting, where various specialties and divisions are constantly evaluating the patient, from birth to adulthood.

Financial matters represent the major disadvantage in a multidisciplinary clinic. Parents of cleft patients might need a medical insurance/aid cover for the continuous treatment of their infant or child. Fully government funded or nongovernmental health care clinics, might represent an option.

A typical multidisciplinary team consists of the following members: - - - - - -

Surgeon(s) (cleft-cranio-maxillofacial surgeon) Orthodontist Speech and language pathologist Audiologist Paediatrician Community nurse(s)

- - - - -

Clinical psychologist Medical geneticist Paedodontist Oral hygienist Prosthodontist

BĂœTOW K‑W The multidisciplinary approach and the therapy protocol. In: Treatment of Facial Cleft Deformities. Ishiyaku EuroAmerica, Inc. Publishers, St Louis, Tokyo 1995: pp. 1-2.

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Introduction And Philosophy

Charity or Missionary Surgery Especially in developing and underdeveloped countries, the majority of children with facial cleft deformities has no access to any multidisciplinary clinic and particularly to any kind of advance treatment. The only hope for these toddlers, children or adolescents, lies in nationally or internationally sponsored, charity or missionary surgical organisations. Most travel to certain places providing basic surgery for patients with such deformities. The cleft lip is the most obvious facial deformity. It constitutes an important part of the aesthetic appearance, being of utmost importance for the patient’s personality. Few cases undergo surgery for the comprehensive cleft treatment including palate repair, which is very important for speech development and feeding. An example of exception is the Mercyships’ protocol. These children, living mostly in underdeveloped or partially developed countries, represent the vast majority of facial cleft deformities worldwide.

It has been estimated that around 90% of charity or missionary surgeries consist of cleft lip repair and 10% of cleft palate repair. A multidisciplinary clinic records a totally different picture, with 62% of patients presenting a cleft lip, 79% a cleft lip and/or cleft palate (see page 181). Continuous follow-up and treatment interventions for secondary reconstructions, speech pathology, orthodontic and prosthodontic treatment do not exist, or exist only in a very limited aspect for these unfortunate patients. These children shall therefore never experience a multidisciplinary clinic approach with comprehensive treatment.

It is still of utmost importance that the poorest of the poor, which represent the majority, receive some type of basic treatment, even a cleft lip repair only, in order to be accepted in their community.

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Cleft - Ultimate Treatment

Ultimate Treatment Whereas the ultimate treatment for any type of facial cleft deformity can only be offered in multidisciplinary cleft / cranio-maxillo-facial clinics, solitary cleft lip deformities (6%) might be treated successfully in a non-multidisciplinary environment. During long-term follow-up, however, unpredictable abnormal tooth eruption overlying the cleft side and secondary deformities may occur. Secondary reconstruction due to cleft lip scarring, as well as cleft rhinoplasty at around 14 years of age might become necessary. This clearly indicates the necessity that even patients suffering from minimal variations of cleft deformities need to be followed up in multidisciplinary clinics during their growth period.

In general the ultimate result depends on: - - - - -

20

Surgical protocols for facial cleft deformities, the sequence of surgical interventions, the avoidance of revision surgery in the growing face, and a continuous follow-up for about 16 years; The alignment of dento-alveolar arches and the nasal ala moulding, where possible, and the columella stretchmoulding in bilateral cleft lip; Choosing those surgical procedures that cause the least long-term growth disturbances of facial hard and soft tissues and avoid occlusal disturbances and midfacial growth deficiency; Choosing those surgical procedures that provide the best aesthetic appearance; Choosing those surgical procedures that provide optimal results for soft palate function in terms of feeding and speech production, and reduce middle ear effusion/infection for better hearing.

-2Different Types Of Cleft Deformities ANATOMICAL CLASSIFICATION OF CLEFTS (Tessier)

 FACIAL

 LATERO-FACIAL

(transverse)

 CRANIO-FACIAL

(central and oblique)

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Cleft - Ultimate Treatment

Diagnostic Key R right side L

left side

Upper triangle

Nasal ala

Next triangle

Nasal floor

1st trapezius square

Lip

2nd trapezius square

Alveolus

Two squares

Hard palate

Lower triangle

Soft palate

Hatched

Partial

Filled

Complete

Example (shaded part indicates the cleft) : left

left

- CLAP - upper triangle deformed - nasal ala (“subcutaneous”)

BÜTOW K‑W. Visual symbolic and numeric classification for cleft lip and palate cases. J Dent Ass S Afr, 1985: 40; 331‑336.

22

Different Types Of Cleft Deformities

Cleft lip (CL) Types of cleft lip: 1. Micro-microform 2. Mini-microform 3. Microform 4. Minor-form 5. Total / partial-incomplete (subcutaneous) 6. Total / complete 7. Median facial dysgenesis type

YUZURIHA S, OH AK, MULLIKEN JB. Asymmetrical bilateral cleft lip: Complete or incomplete and contralateral lesser defect (minor-form, microform, or mini-microform) Plast Reconstr Surg , 2008: 122; 1494-1504.

23

Cleft - Ultimate Treatment

Cleft lip–alveolus (CLA)

Atrophic prolabium-premaxilla (median facial dysgenesis type)

24

Different Types Of Cleft Deformities

Lip and/or alveolar bands Lip-to-lip (Simonartz’) band Lip-to-alveolar band Alveolar-to-alveolar band

ENGELBRECHT H, BÜTOW K-W. Alveolar band in patients with cleft lip and cleft alveolar deformities. J Craniofac Surg 2013: 24; e153.

NAIDOO S, BÜTOW K-W. Oblique lip-alveolar banding in patients with cleft lip and palate. Br J Oral Maxillofac Surg 2015: 53; 390-2.

25

Cleft - Ultimate Treatment

Cleft lip, alveolus and palate (CLAP)

Atrophic premaxilla (Median facial dysgenesis)

26

Different Types Of Cleft Deformities

Hard palate (hP) cleft

BÜTOW K‑W, JACOBS FJ, ZWAHLEN RA. Variations of the isolated cleft of the hard palate. S Afr Dental Journal 2008: 63; 164 – 168.

Engelbrecht H, Bütow K-W, Botha SJP Exceptional isolated cleft of the hard palate. Br J Oral Maxillofacial Surg 2012: http://dx.doi. org/10/1016/j.bjoms.2012.10.012.

27

Cleft - Ultimate Treatment

Hard and soft palate (hPsP) cleft Hard + soft palate cleft with synechiae (“oral bands”)

NAIDOO S, BÜTOW K‑W. Nonsyndromic palate synechia with floor of mouth. Ann Maxillofac Surg 2015: 5; 100-103.

28

Different Types Of Cleft Deformities

Soft palate (sP) cleft

29

Cleft - Ultimate Treatment

Combination of clefts (Combi)

Combi cleft cases may present with a cleft lip and a cleft soft palate (CL+sP), without necessarily involving the alveolar bone and the hard palate as a CLAP case.

30

Different Types Of Cleft Deformities

Median cleft (CL,CLA,CLAP) (a)

(a)

(b) (a)

DeMyer group II (a) + DeMyer group I (b) (holoprosencephaly)

31

Cleft - Ultimate Treatment

Median cleft (lower lip, tongue)

Cleft tongue, with tumour (of tongue and palate

32

)

Different Types Of Cleft Deformities

Lateral cleft (transverse or Tessier 7)

33

Cleft - Ultimate Treatment

Oblique facial clefts

Unilateral Tessier 2-3 + 4(soft tissue)

Unilateral Tessier 3

34

Unilateral Tessier 5 (left) + 7.4 (right)

-3Treatment Protocols From Neonate To Adult (A) general protocol (b) surgical protocol (c) nursing protocol (d) pharmacology Before any treatment starts, a long-term treatment plan has to be established, a so-called treatment / therapy protocol.

BÜTOW K-W. “Kaak-, Gesig- en Mondchirurgie vir die algemene praktisyn.” Cape Town: Jute & Co 1988: 24: 402-403.

BÜTOW K-W. The multidisciplinary approach and the treatment protocol. Treatment of Facial Cleft Deformities. St Louis: Ishiyaku EuroAmerica, Inc. 1995: 3-4.

35

Cleft - Ultimate Treatment

Infants need a fixed long-term protocol

36

Adults and adolescents need a short-term personalized protocol

Treatment Protocols From Neonate To Adult

Cleft protocols (4 basic + 1 specific protocol) A. General treatment protocol

(Regular follow-ups, pre-surgical examinations, non-surgical interventions, timing with other specialists)

B. Nursing protocol

(Hospitalisation, feeding – naso-gastric/bottle/cups, microbiology swabs)

C. Surgical protocol

(Timing of surgery, type of surgical procedure for particular facial cleft, determine patient’s age of intervention )

D. Paediatric pharmacological protocol

(Medication and care)

[E. Protocol for Pierre Robin Sequence (Chapter 19)] MYBURGH HP, BÜTOW K-W. The peri-operative intra-oral microbiological profile of babies with cleft facial deformities – a South African database. Int J Oral Maxillofac Surg 2009: 38; 928-932.

Du Plessis D, Bütow K-W, Van den Berg HJS, Hoogendijk CF Airway and feeding problems in Fairbairn-Robin triad (Pierre Robin): nursing and surgical approach. Curationis 2013: 36 – doi:10.4102/curationis.v36iI.84.

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Cleft - Ultimate Treatment

Cleft patients’ treatment starts with: - - - - - - -

Paediatric examination Community nurse consultation, especially for airway and feeding issues * Speech therapy consultation for the parents Surgical consultation and introduction of the custom made surgical protocol Psychological support for parents * Functional jaw-orthognathic treatment or primary jaw-orthodontics directly after birth Genetic counselling for parents and investigation of family history

*as deemed necessary

A. General Treatment Protocol 1.

Regular follow-ups - Plate treatment for jaw-orthognathic or primary orthopaedic reasons – 4 weekly - Postoperative follow-up at week 2 and 8 - Annual follow-up during the patient’s birthday week (exception: for CL and sP, every 2 years, during the patient’s birthday week)

2. Other non-surgical specialists and involved disciplines - Regular specialists, such as an orthodontist, a community nurse, a paediatrician and a speech pathologist should attend every clinic - If needed, attendance of other specialists, such as a paedontist, a prosthodontist and an audiologist - Others based on referral, such as an anaesthetist and a paediatric neurologist 38

Treatment Protocols From Neonate To Adult

3.

Pre-surgical examination - Photographs - Dental casts (if applicable) - Blood tests (if applicable)

4. Non-surgical intervention - Psychological support for parents with babies, for children and for adolescents

B. Nursing Protocol 1. Regular follow-up duties of community nurses - Clinic management: arrange clinic times and patients’ records, maintain photo documentation, liaise with auxiliary disciplines related to social and family problems - Check patients’ attendance during normal and outside clinic hours, such as new-born consultation, appointments for plate manufacturing - Help and support with feeding problems - Organise pre-operative visits of patients at their homes - Advise on pre-operative hospital admission and on inward period

39

Cleft - Ultimate Treatment

2.

Other duties of community nurses - Call other hospitals to consult parents with new-born cleft babies - Arrange surgical bookings for cleft babies - Gather statistical data on new arrivals and on surgical - interventions of cleft patients - Can be reached by phone for parents’ nursing questions - Are involved in research and publications

3. Paediatric nursing in the surgical ward - Conduct a paediatric nursing protocol about medication, nasogastric feeding, oral cavity cleaning, prevention of vomiting, and assistance in suture removal

C. Surgical Protocol CLEFT SURGERY - the MIRROR-IMAGE PROTOCOL

Why a “mirror-image” protocol to completely close primary cleft lip and palate ?

1. To achieve a watertight closure of the entire cleft: Perfect closure of the lip, the anterior nasal floor, the area between the dento-alveolar arches, from the anterior nasal floor to the hard palate and from the hard palate to the soft palate, can only be achieved if the surgical closure starts from the soft palate towards the lip. Primary closure of cleft lip proceeding the hard palate cleft closure will always lead to an anterior or a buccal oro-nasal fistula.

40

Treatment Protocols From Neonate To Adult

2. The closure of the soft palate should be considered between 5 to 7 months, as the phonetic age of babies begins around this time.

3. The closure of the hard palate should be done after the soft palate closure. This way babies do not suffer from compromised breathing during the recovery period. During the reconstructed soft palate healing period, the hard palate is still open toward the nasal cavity i.e. breathing is not limited. This is of utmost importance particularly in combined hard and soft palate clefts and especially in patients suffering from a Fairbairn-Robin triad, a subdivision of a Pierre Robin sequence.

“Mirror-image” surgical protocol OR “reverse” surgical protocol OR “Principle: from inside to outside” Implemented in 1986 BÜTOW K-W. “Kaak-, Gesig- en Mondchirurgie vir die algemene praktisyn.” Cape Town: Jute & Co. 1988: 24; 402-403.

BÜTOW K-W. The multidisciplinary approach and the treatment protocol. Treatment of Facial Cleft Deformities. St Louis: Ishiyaku EuroAmerica, Inc. 1995: 3-4.

41

Cleft - Ultimate Treatment

The surgical protocol (i)

The surgical protocol (ii)

Age in months (mths) or years; *as deemed necessary

Age in months or years; *as deemed necessary

5 or 7 months - sP = Soft palate 5 mths (CLAP) or 7 mths (hPsP, sP) - CL = Cleft lip at 5 mths - CLA = Cleft lip + anterior nasal floor at 5 mths - CLAP = Cleft lip, anterior nasal floor + hard palate at 7 mths - sP and hPsP = Soft palate and hard palate (partial + narrow) at 7 mths (During surgery, hP is closed before the lip and the anterior nasal floor = mirror-image protocol )

4 years - CLAP, hPsP, sP = * Velopharyngeal flap

12 months - bilateral CLA, CLAP = Columella lengthening

14 – 18 years - CL, CLA, CLAP = * Revision cleft lip and/or cleft nose (The ideal age is around 14 years, however, it depends on eventual orthognathic surgical reconstruction of the midface or mandible)

18 months - hPsP = Hard palate cleft 2 – 4 years - * CLAP, hPsP, sP = * Speech therapy 4 years - * CLAP, hPsP, sP = * Fluorography examination 42

9 – 13 years - CLA, CLAP = Secondary osteoplasty (Ideal age: 9 years, but this also depends on the completeness of the orthodontic pre-surgical treatment) 14 – 18 years - Any cleft type = * Orthognathic surgery

BÜTOW K-W. Treatment of cleft lip and palate. Part III: Surgical techniques and discussion, different phases from birth to adulthood. J Dent Ass S Afr 1984: 39; 395.

BÜTOW K-W. The multidisciplinary approach and the treatment protocol.

Treatment of Facial Cleft Deformities. St Louis: Ishiyaku EuroAmerica, Inc. 1995: 3-4.

Treatment Protocols From Neonate To Adult

Reasons for a surgical protocol 1. To achieve optimal closure and to avoid oro-nasal fistulae (principle → closure from inside to outside, the mirror-image protocol) 2. To utilise the best predictable surgical techniques for a complete closure:

sP:

an intravelar veloplasty with

a tensor stitch to activate the Eustachian tube

a Sommerlad’s levator and palato-glosso-pharyngeal muscle repositioning and reconstruction, and an Ivanov’s uvula reconstruction

an inferior or caudally-based vomer flap or

hP:

a superior or cranially-based vomer flap with a PdLAlLA* resorbable sheet

CA:

an anterior nasal floor flap in the cranial part of the cleft

CL:

an anatomical lip-plasty [unilateral type] and

a Broadbent’s modification or Noordhoff-Trott [bilateral] type technique

3. To achieve optimal speech development and occlusal function 4. To avoid midfacial growth disturbances (= dysgnathia) by choosing adequate primary surgical techniques 5. To avoid revision surgeries and excessive orthodontic treatment * Poly-lactic acid sheet (a sheet to cover the vertical triangular defect for maintaining the blood clot; as such to achieve an ideal flat palatal healing)

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Cleft - Ultimate Treatment

- - - -

44

This surgical protocol has not been implemented to prescribe particular types of surgical procedures.

Different types of procedures may be used, however, there are preferences, based on proper and international research results and clinical observations.

This surgical protocol helps to achieve both a complete closure using minimal invasive surgery and to provide minimum long-term growth disturbances.

This surgical protocol is one of the best ways to ensure ideal life-long results for patients with cleft deformities.

Treatment Protocols From Neonate To Adult

D. Paediatric pharmacological protocol (pharmacology + care) The post-operative medical protocol

Children Intravenous fluid: Paediatric Maintenance Solution – 4ml/kg/hour until 1st feeding, with nasogastric feeding reduced by 1/3.

Antibiotics: Augmentin IV (preferred) [or Erythromycin IV], as long as IV is open, 125mg q6h (or 50mg/kg/24h IV); with nasogastric application – 125mg q8h.

Oedema control: Solu-Medrol 20mg IV q6h X 3 or 4 (between 4 – 9kg, 30mg > 9kg) Analgesics (always 2X): Valoron drops - 1 dr q4h max or Perfalgan IV 15mg/kg q6h max; Stopayne 2.5ml (4 – 8 kg) or 3ml (> 8kg) q4h max or Ponstan 2.5ml (4 – 8 kg) or 3ml (> 8kg) q4h max, alternate; intraoperative suppository of Empaped 125mg can be administered.

Oral rinse: Corsodyl 200ml diluted 50:50 H2O – application with spray bottle.

Lip ointment: Chloromycetin 3.5gr qid – corner of the mouth (lip reconstruction - only on lip red mucosa).

Lip dressing: Mepiform, cut to size of lip height (application 40 to 50 X, if possible for 24h), in between Dermastine + Vitamin A 150ml intensive massage 4X day starting 3 weeks post-operative up to 9 months.

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Cleft - Ultimate Treatment

Soft palatal surgery: Every 2nd day a microbiological swab is taken and sensitivity testing is performed to evaluate for most appropriate antibiotic regimen.

OTHER TREATMENTS Constipation: Glycerine suppository or/and plum juice, if needed.

Nappy rash - persistent: Daktarin skin cream.

Surgeons need to be familiar with basic paediatric conditions and treatments, however, a paediatrician should be consulted in more serious conditions, such as pneumonitis (= lung infection), viral gastro-intestinal infection, as well as for administration of non-paediatric anti-microbial medication.

Myburgh HP, Bßtow K-W. The peri-operative intra-oral microbiological profile of babies with cleft facial deformities – a South African database. Int J Oral Maxillofac Surg 2009: 38; 928-932.

46

-4Functional Jaw-Orthognathic Plate Or Primary Orthopaedic Treatment

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Cleft - Ultimate Treatment

Functional jaw-orthognathic suction and drinking (FJO) plate activates the rotation of the smaller dentoalveolar segment.

Result after 5 months of treatment [Functional jaworthognathic movement]

McNEIL CK. Orthodontic procedures in treatment of congenital cleft palate. Dent Res 1950: 70: 126.

HOTZ M, GNOINSKI W. Comprehensive care of cleft lip and palate children at Zurich University: A preliminary report. Am J Orthod 1976: 70: 48.

BĂœTOW K-W. Treatment of cleft lip and palate. Part II: The jaw-orthognathic suction and drinking plate. J Dent Ass S Afr 1984: 39: 331.

CUMBER E, BĂœTOW K-W, VAN DER MERWE CA. Presurgical jaw-orthognathic expansion in babies with complete unilateral cleft lip and palate. J Dent Res 1987: 66: 950.

48

Functional Jaw-Orthognathic Plate Or Primary Orthopaedic Treatment

Functional jaw-orthognathic plate

Suction and drinking plate (SDP) [Obturator with dorsal extension] SDP with external facial stabiliser for a hPsP cleft

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Cleft - Ultimate Treatment

Bilateral CLAP

50

Functional Jaw-Orthognathic Plate Or Primary Orthopaedic Treatment

Bilateral CLAP pre-surgical treatment with FJO-plate and head-gear (alternatively used: lip bandage)

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Cleft - Ultimate Treatment

Redressing of premaxilla-prolabium treatment

Protruded premaxilla-prolabium

Bilateral CLAP -Premaxilla positioning, head-gear

Before CLAP surgery, after conservative pre-maxilla-prolabium treatment

Bilateral CLAP - Premaxilla positioning by use of lip bandage and FJO for dento-alveolar moulding: at 1 day; at 2ž months; at 5 months.

HOTZ M, PERKO M, GNOINSKI W. Early orthopaedic stabilization of the praemaxilla in complete bilateral cleft lip and palate in combination with the Czelesnik lip repair. Scand J Plast Reconstr Surg 1987: 21; 45.

GEORGIADE NG, MANSON R, RIEFKOHL R, GEORGIADE G, BARWICK W. Preoperative positioning of the protruding premaxilla in the bilateral cleft lip patient. Plast Reconstr Surg 1989: 83; 32.

52

Functional Jaw-Orthognathic Plate Or Primary Orthopaedic Treatment

Suction and drinking plate with additional extension (for hPsP-cleft in Pierre Robin sequence [FRT]) ZSCHIESCHE S. “ Profilveränderung bei Patienten mit Pierre-Robin-Syndrom.” Fortschr Kieferorthop 1985: 46; 72.

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Cleft - Ultimate Treatment

To avoid glossoptosis: a special SPD with additional extension facial retention (hPsP-cleft in Pierre Robin sequence [FRT])

54

and external

Functional Jaw-Orthognathic Plate Or Primary Orthopaedic Treatment

Nasal-alveolar moulding (NAM)

Treatment by orthodontist: Dr Emad Ghabrial Result on cleft nasal-alar architecture after 5 months treatment (pre-op + 6 days post-op)

Nasal-alar moulding (NAM) after soft palate repair

NAM after the soft palate repair and before the hard palate, anterior nasal floor reconstruction and lip-repair MONASTERIO L, FORD A, GUTIÉRREZ C, TASTETS ME, GRACÍA RN. Comparative study of nasoalveolar molding methods: Nasal elevator plus DynaCleft™ versus NAM-Grayson in patients with complete unilateral cleft lip and palate. Cleft Palate-Craniofacial J 2013:50; 548-554.

55

PRIMARY CLEFT TREATMENT

57

-5Unilateral Cleft Lip

A. DIAMOND or/and STRAIGHT-LINE LIP-PLASTY

(minor or partial cleft lip deformity)

B. ANATOMICAL LIP-REPAIR

C. MILLARD-II LIP-REPAIR

D. TENNISON or RANDALL or MALEK LIP-REPAIR

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Cleft - Ultimate Treatment

A. DIAMOND or STRAIGHT-LINE LIP-REPAIR DIAMOND PLASTY in microform CL should be done only around the age of 14 (i)

Muscle reconstruction underneath the skin for proper alignment 60

Unilateral Cleft Lip

DIAMOND REPAIR and NASAL ALAR ROTATION

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Cleft - Ultimate Treatment

DIAMOND combined with STRAIGHT-LINE REPAIR

The incision is more extended superiorly to encompass the length of the defect.

62

Unilateral Cleft Lip

B. ANATOMICAL LIP-REPAIR Design for a complete cleft lip

Design for a partial cleft lip BÜTOW K-W. Primary unilateral cleft lip repair without primary rhinoplasty. J Cranio-Maxillofac Surg 1990: 18; 14.

BÜTOW K-W. Primary surgical repair of the lip and nose. Treatment of Facial Cleft Deformities. St Louis: Ishiyaku EuroAmerica, Inc. 1995: 36-39.

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Cleft - Ultimate Treatment

Schematic design for muscle reconstruction to enhance the philtrum edge and the philtrum dimple.

Maximum aesthetics can only be achieved in also enhancing the philtrum dimple and the philtrum edge.

64

Unilateral Cleft Lip

Muscle reconstruction to enhance the philtrum edge and the philtrum dimple (left-side cleft and right-side cleft).

65

Cleft - Ultimate Treatment

Anatomical lip-repair (partial CL)

Partial CL repair

66

Unilateral Cleft Lip

Anatomical lip-repair (partial CL)

2 weeks postoperative

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Cleft - Ultimate Treatment

Anatomical lip-repair (partial CL) 6 months after operation

68

Unilateral Cleft Lip

Anatomical lip-repair (complete CL) (i)

Tattoo most important points of incision lines with a fine needle and methylen blue

NOTE! Avoid any tension due to drapes and intubation tube 1st step: carefully analyze and draw the planned incision lines using a thin feather Use a caliper to measure distances Carefully analyze the situation in relation to nose and symmetry of the face Any tissue excess has to be balanced to the opposite as a triangle

Incise while compressing the superior labial artery and vein between thumb and index finger 69

Cleft - Ultimate Treatment

Anatomical lip-repair (complete CL (ii)

Preparation of the flap to reconstruct the anterior nasal floor (This flap is derived as part of the excess lip vermilion rotated superiorly to the nasal orifice) 70

Loosen the tissue around the alar base to facilitate the ala position Note! Otherwise too much tension remains at the ala on the cleft side

Unilateral Cleft Lip

Anatomical lip-repair (complete CL) (iii) M. orbicularis oris Superior labial artery (arrow)

Coagulation of the superior labial artery

Dissect orbicularis oris muscles with knife, while compressing the superior labial artery

Check orbicularis oris muscle of the non-cleft side 71

Cleft - Ultimate Treatment

Anatomical lip-repair (complete CL) (iv) Lengthening of the upper lip Triangle Nasal floor flap M. Orbicularis oris

ďƒ Double layer: nasal + oral (vomerine) mucosa

Provisional adaptation of the lip Suture fixation with two mosquitos to hold the lip exactly in the midline for further exact suturing Do not exert too much pull!!

72

Suturing of the nasal floor flap

Unilateral Cleft Lip

Anatomical lip-repair (complete CL ) (v) Create oral lip layer with a sulcus and tight closure with the anterior nasal floor Suture with knots extraoral at the wet red lip and therafter intraorally Suture of the M. orbicularis oris Special reverse mattress suture to accentuate the philtrum edge beyond the incision (scar) The assistant surgeon carefully approximates the skin for muscle suturing

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Cleft - Ultimate Treatment

Anatomical lip-repair (complete CL) (vi) Suturing of the skin The triangle is the last part. It has to be trimmed to a perfect size.

LIP COVERAGE 1. Friar balsam (TBCo) (it cleans and creates a sticky layer on the skin) 2. Mepiform strips (trimmed to size) OR Chloramphenicol ointment AND 3. Steristrips

FEEDING Naso-gastric tube 74

Unilateral Cleft Lip

Anatomical lip-repair (complete CL)

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Cleft - Ultimate Treatment

Muscle reconstruction to enhance the philtrum edge and the philtrum dimple (6 days later).

76

Unilateral Cleft Lip

Anatomical lip-repair (complete CL)

77

Cleft - Ultimate Treatment

Anatomical lip-repair; clinical result 3 months post-operatively

78

Unilateral Cleft Lip

Anatomical lip-repair; clinical result 1 year post-operatively

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Cleft - Ultimate Treatment

Anatomical lip-repair; clinical result 3 years post-operatively

80

Unilateral Cleft Lip

Anatomical lip-repair; clinical result 5 years post-operatively

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Cleft - Ultimate Treatment

Anatomical lip-repair; clinical result 8 and 14 years post-operatively

Prior to secondary nasal repair

BĂœTOW K-W. Anatomical lipplasty: Eight-year follow-up and applied surgical modifications. J Dent Res 1995: 74 [3]; 1023 (125).

82

Unilateral Cleft Lip

Anatomical lip-repair; clinical result 16 years post-operatively

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Cleft - Ultimate Treatment

Anatomical lip-repair – ULTIMATE RESULT (i)

84

Unilateral Cleft Lip

Anatomical lip-repair – ULTIMATE RESULT (ii) THE ULTIMATE

Winner of the South African Photogenic Competition 2010

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Cleft - Ultimate Treatment

C. MILLARD-II LIP-PLASTY The incision on the greater lip segment rotates towards the columella and encroaches on the natural area of the philtrum ridge. The nasal sill on the cleft side does not have a natural rotation.

MILLARD DR. A radical rotation in single hare lip. Am J Surg 1958: 95: 318.

MILLARD DR. Refinements in rotation-advancement cleft lip technique. Plast Reconstr Surg 1964: 33: 26.

ASENCIO OE. A variation of the rotation-advancement operation for repair of the wide unilateral cleft lips. Plast Reconstr Surg 1974: 53: 167.

86

Unilateral Cleft Lip

Millard-II Lip-Plasty

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Millard-II Lip-Plasty; 2 years post-operatively

88

Unilateral Cleft Lip

Millard-II Lip-Plasty; 16 years post-operatively

Prior to any secondary repair

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Cleft - Ultimate Treatment

D. TENNISON LIP-REPAIR; similar: RANDALL and MALEK Triangle is positioned inferiorly in the lip (incision is made into the natural dimple area), it appears that as such more pressure is exerted on the maxilla. Therefore an increased midfacial growth disturbance can be expected in a complete unilateral cleft lip, alveolus and palate deformity.

TENNISON CW. The repair of the unilateral cleft lip by stencil method. Plast Reconstr Surg 1952: 9: 115.

RANDALL P. A triangular flap operation for the primary repair of unilateral clefts of the lip.Plast Reconstr Surg 1959: 23: 331.

MALEK R, GROSSMAN JAI. Cleft repair by systematic z-plasty. Clin Plast Surg 1984: 11: 739.

STEINHĂ„USER EW, BĂœTOW K-W. The influence op lip closure technique, Millard or Tennison, on the dysgnathial incidence. 5th Int Congr Cleft Palate Craniofacial Anomalies 1985: 4: 85.

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Unilateral Cleft Lip

Tennison / Randall / Malek lip-repair

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Cleft - Ultimate Treatment

Unilateral lip-repair - The ULTIMATE Clinical pearls To achieve the best clinical outcome, various aspects have to be followed. Ignoring one or more of these aspects will lead to suboptimal aesthetic and functional results.

The principles for the ultimate result in unilateral cleft lip repair are: 1. To recognize that a part of the lip congenitally is missing (measure the length on the non-cleft side from the natural philtrum edge, at the lip red-skin junction, and the junction of the lip commissure and compare this length at the cleft side) 2. The vertical length of the upper lip at the cleft side is always shorter. Therefore lengthening the lip on the cleft side by designing a triangle(s) is mandatory to achieve an ideal balance. In case this is ignored, a shorter lip at the cleft side will be immediately visible.

3. The upper lip horizontal length at the cleft side is always shorter, especially in wide clefts. Unfortunately, very little can be done to lengthen this part to achieve aesthetic balance. It is of utmost importance to achieve appropriate vertical (superior-inferior) lip dimensions at cleft side. Never compromise the vertical aspect in favour of the horizontal dimension.

4. The scar should be positioned as a mirror image of the philtrum edge to the non-cleft side, with the exception of the superior triangle.

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Unilateral Cleft Lip

Unilateral lip-repair - The ULTIMATE Clinical pearls, continued 5. The muscles of the upper lip should be connected, without disturbing the central philtrum dimple while carefully dissecting the underlying muscles. Inaccurate dissection will result in a flat upper lip. Furthermore, lip muscles should be connected in such a way that the overlying cutaneous scar mimics the opposite natural philtrum.

6. Do not position a straight line incision at or into the nasal floor, as the upcoming scar might present as a shiny area, mimicking a permanent runny nose.

7. The white roll, the prominent ridge where the lip mucosa meets the skin, should not be transected with a straight lip incision line, as this aesthetic landmark can immediately be recognised as a scar.

8. The same as above applies to the red lip dry – wet junction. This aesthetic landmark shall also immediately be recognised as a lip scar.

9. The alar sill should be medially rotated to create a similar aesthetic appearance compared to the non-cleft side.

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-6Bilateral Cleft Lip

A. MODIFIED BROADBENT LIP-REPAIR

B. NOORDHOFF-TROTT LIP-REPAIR

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Cleft - Ultimate Treatment

A. MODIFIED BROADBENT LIP-REPAIR

The “philtrum edges” are determined by the width measurement of the prolabium (see 5-6-5) and should not be wider than the columella root. n = nasal floor flaps.

The prolabium red lip is laterally de-epithelised and folded to create more central tissue bulk.

BROADBENT TR, WOOLF RM. Bilateral cleft lip repairs. Plast Reconstr Surg 1972: 50: 36.

BÜTOW K-W. Primary surgical repair of the lip and nose. Treatment of Facial Cleft Deformities. St Louis: Ishiyaku EuroAmerica 1995: 41 – 44.

96

Bilateral Cleft Lip

Schematic design of muscle repair enhancing philtrum edges and its dimple.

The “philtrum dimple� is caused by an incomplete approximation of the orbicularis oris muscle, therefore, the lateral muscle stumps should not be attached to each other.

Furthermore the central part is sutured, involving the subdermal prolabium skin, to create the dimple effect, therefore avoiding a bubblelike prolabium. The approach for the suture starts at the labial frenum and proceeds towards the subdermal region.

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Cleft - Ultimate Treatment

Partial Bilateral Cleft Modified Broadbent lip-repair

The healing process of philtrum edges and dimple over a period of 4 years (no additional surgery)

Lateral stumps of the muscle not attached

98

Bilateral Cleft Lip

Modified Broadbent lip-repair (partial cleft) THE ULTIMATE

Result after 21 years (no additional surgery)

99

Cleft - Ultimate Treatment

Complete Bilateral Cleft Bilateral cleft lip-repair- procedure (i) -

-

-

Evaluate the general site Intubation tube in middle position, avoid pressure on the cheecks with the draping towels Carefully analyze the surgical site Lip symmetry and soft tissue volume Develop the operation plan and sequence!!

Evaluate in detail:

Measure carefully and draw planned incision lines!

100

- Lip volume - Cleft width - Lip red volume - Lip height - Lip width - Nose width - Columella length

Bilateral Cleft Lip

Bilateral cleft lip-repair - procedure (ii) - -

Tattoo important landmarks

-

-

Incise at premaxilla Additionally deepithelialize the laterally created cutaneous + red lip mucosa for central bulk of red lip Dissect anterior central part of the prolabium De-epithelialize a part to create bulky tissue from the prolabium red lip.

- Remove excessive tissue [BUT remember tissue is needed for the anterior nasal floor!]

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Cleft - Ultimate Treatment

Bilateral cleft lip-repair - procedure (iii) - -

-

-

Incise and dissect orbicularis oris muscle, coagulate (similar to unilateral lip repair)

Suture the nasal floor flap to create an anterior nose floor [ďƒ Double layer: nasal mucosa (= anterior nasal floor flap) + oral mucosa (= vomer flap) overlap]

102

- Alar stitches Inrotation of nasal alar cartilages to create a patent nasal passage (first detach the nasal mucosa from the lateral alar cartilage!)

Loosen the soft tissue at the alar base (sill) appropriately Important: visualize carefully and dissect from extra- and intraoral!

Bilateral Cleft Lip

Bilateral cleft lip-repair - procedure (iv)

- Create prolabium red lip part: Rotate and sublimate the prolabium using deepithelialized tissue ďƒ Creates red lip tissue volume -

Nasal base (1) First suture to align superior muscle parts, involving the anterior nasal spine- septum area

-

Repair of the intraoral sulcus

Premaxilla

Lateral lip segments -

Nasal base (2) First suture to align these muscle parts superiorly and laterally

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Cleft - Ultimate Treatment

Bilateral cleft lip-repair- procedure (v) Final result Friar balsam for the skin Mepiform-strips OR Chloramphenicol ointment plus Steristrips for post-operative care Feeding by naso-gastric tube

104

Bilateral Cleft Lip

Modified Broadbent lip-plasty, 3 months post-operatively

105

Cleft - Ultimate Treatment

Modified Broadbent lip-plasty 3 years post-operatively

106

Bilateral Cleft Lip

Modified Broadbent (CLAP) 4 years post-operatively (i) THE ULTIMATE

107

Cleft - Ultimate Treatment

Modified Broadbent (CLAP) 24 years post-operatively (ii) THE ULTIMATE

108

Bilateral Cleft Lip

B. NOORDHOFF-TROTT LIP-REPAIR With a prominent white role - -

extending nearly on the lateral lip segment over the entire distance towards the nasal floor and with an non-existing prolabium white role,

The Noordhoff-Trott technique should be considered for aesthetical reasons

NOORDHOFF MS. Bilateral cleft lip reconstruction. Plast Reconstr Surg 1986: 78: 45-54.

TROTT JA, MOHAN N. A preliminary report on one stage open tip rhinoplasty at the time of lip repair in bilateral cleft lip and palate: the Alor Setar experience. Br J Plast Surg 1993; 46; 215-222.

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Cleft - Ultimate Treatment

NOORDHOFF-TROTT lip-repair

Extended flap

110

Bilateral Cleft Lip

NOORDHOFF-TROTT lip-repair, after birth, 5 months and 1 year post-operatively

111

Cleft - Ultimate Treatment

Bilateral lip-repair - The ULTIMATE Clinical pearls To achieve the ultimate result various aspects have to be considered. Ignoring one or more of these aspects will lead to suboptimal aesthetic and functional results in bilateral cleft lip cases.

1. To achieve best aesthetic results, incision lines on both sides, i.e. the future scar, should mimic philtrum edges. Just connecting the lateral lip stumps directly to the prolabium is suboptimal. Too much preservation of the prolabium will result in a philtrum that is too broad, with a poor aesthetic result.

2. The length of the prolabium is mostly shorter than in unilateral cleft lip cases. No specific lengthening is needed, as long as the entire cutaneous part (prolabium) is released from the premaxilla. If minimum lengthening is needed, incisions at the lateral segments might be performed Âą 0.5 mm longer than at the prolabium.

3. The incisions at the cleft, i.e. the future scar, should be positioned as a “bilateral mirror image� to represent the philtrum edges.

4. The upper lip muscles should be aligned, but not attached to each other. Create the philtrum dimple in the prolabium central part by not approximating the lateral muscle stumps. Ignoring this principle will result in either a bulging prolabium, or in a flat upper lip, resulting in a long-term poor aesthetic outcome.

112

Bilateral Cleft Lip

Bilateral lip-repair - The ULTIMATE Clinical pearls, continued 5. The incisions, i.e. future scars, in the nasal floor should not be planned as straight lines, as these might appear as shiny areas, mimicking a permanent running nose. A triangle from the internal cutaneous nasal sill (lateral aspect) needs to be sutured towards the columella-prolabium junction.

6. The white roll, a prominent lip ridge where lip mucosa meets prolabium skin, needs to be evaluated before the operative technique is chosen. If a prolabium white roll is present, a modified Broadbent lip-repair can be chosen. If there is no prolabium white roll, but a white roll at each lateral lip segments that extend towards the nasal cavity, a Noordhoff-Trott lip-repair may be the first choice.

7. The red lip dry – wet junction in a modified Broadbent lip-repair should be incised in a curvilinear way, not straight. A straight incision will be recognized immediately as a scar.

8. Accentuation of cupid’s bow can be achieved with a Noordhoff-Trott lip-repair. However, a prominent scar formation might develop superiorly, adjacent to the white roll at the designed cupid’s bow.

9. Various procedures for bilateral CLAPs exist. Depending on the surgeon’s choice and skills, best possible results can be achieved, if the basic principles are followed.

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-7Unilateral Cleft Nose Reconstruction / Primary Rhinoplasty

A. CLEFT NOSE reconstruction with resorbable PdLAlLA* sheet or plate (In patients with CL + CLA at 5 months; in patients with CLAP at 7 months)

Poly-d + l-Lactic Acid (PdLAlLA), also abbreviated as PdLLA sheet

BĂœTOW K-W. Primary unilateral lip surgery improving nasal deformity asymmetry without early nasal surgery. 6th Intern. Congress of Cleft Palate and related Craniofacial Anomalies 1989: p.7, Jerusalem, Israel.

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Cleft - Ultimate Treatment

Approach during primary rhinoplasty for the support of the nasal ala (orange arrow to indicate the insertion of the PdLAlLA resorbable plate/sheet)The nasal floor needs to be repaired before primary rhinoplasty occurs (orange circle) 116

Unilateral Cleft Nose Reconstruction / Primary Rhinoplasty

Nasal ala reconstruction (evaluation of the deformity)

117

Cleft - Ultimate Treatment

Nasal ala reconstruction using a PdLAlLA sheet

118

Unilateral Cleft Nose Reconstruction / Primary Rhinoplasty

Nasal ala reconstruction using a PdLAlLA plate

119

Cleft - Ultimate Treatment

PdLAlLA sheet for nasal ala and dento-alveolar reconstruction

120

Unilateral Cleft Nose Reconstruction / Primary Rhinoplasty

Unilateral primary rhinoplasty – The ULTIMATE Clinical pearls To achieve the ultimate result various aspects have to be considered. Ignoring one or more of these aspects will lead to suboptimal aesthetic and functional results in unilateral primary rhinoplasty.

1. Extensive dissection of the nasal dome, superior alar and septal cartilages, may lead to nasal growth disturbance resulting in adults with infant-like noses.

2. Release the obliquely positioned septum cartilage from the hard palate and anterior nasal spine for around 5 to 8 mm, to achieve auto-correction during growth and a more straight anterior nasal septum.

3. Intrauterine, cleft side alar cartilages are mostly concavely moulded. Do not carve this alar cartilage to weaken its concave shape. Release the mucosa from the cartilage. Insert as a support additional resorbable material between the nasal mucosa and deformed cartilage. (Note: in adolescents/adults it should be placed between skin and alar cartilage).

4. 0.3mm thick PdLAlLA sheet, moulded according to the ala shape of the non-cleft side, may help to support the cleft sided concave alar cartilage deformity.

5. The resorbable material can be underpinned onto the dento-alveolar arch to support the nasal structure at the nasal alar sill region.

6. To avoid nasal growth disturbance, secondary rhino-septoplasty should generally be performed after the age of 14 years, even though the nose will become more asymmetric during growth.

121

-8Bilateral Cleft Nose / Columella Lengthening

CLEFT NOSE (bilateral) CLA and CLAP at 12 months (CL - rarely indicated)

123

Cleft - Ultimate Treatment

Columella lengthening using a modified Cronin technique in bCLAP

CRONIN TD. Lengthening columella by use of skin from nasal floor and alae. Plast Reconstr Surg 1958; 21; 417-426.

BĂœTOW K-W. Nasal dome projection with columella lengthening in bilateral cleft. Hands-On 1996: 8: 57.

124

Bilateral Cleft Nose / Columella Lengthening

Incisions: Inverse V-shape in columella Burrow`s triangles

BĂœTOW K-W. Primary surgical repair of the lip and nose. Treatment of Facial Cleft Deformities. St Louis: Ishiyaku EuroAmerica 1995: pp. 46, 47.

BĂœTOW K-W. Columella lengthening in complete bilateral cleft - a modification. Transcr. Annual Congr. Plast Reconstr Surg. Cleft Lip and Palate. 1995: Ses.II; 2.

125

Cleft - Ultimate Treatment

126

Bilateral Cleft Nose / Columella Lengthening

Procedure of columella lengthening (i) Extraoral incision ďƒ transfixation to prepare the anterior septum border

Position for 1st dome suture !!!

Dissection of the nasal septum and the medial crura of the alar cartilages.

Note: dissect the tissue between medial crura slightly cranial.

Retract tightly for adequate dissection Dissect mucosa from the lateral crus of the alar cartilage ďƒ thereafter a suture is set to form the nostril.

Nostril shape is achieved by suturing the lateral crus of the alar cartilage and the nasal mucosa Extranasal stitch through the same cutaneous hole ďƒ invisible from outside, as knot lies intranasally

127

Cleft - Ultimate Treatment

128

Bilateral Cleft Nose / Columella Lengthening

129

Cleft - Ultimate Treatment

Columella lengthening

(at age 12 months; 3 years post-operatively)

130

Bilateral Cleft Nose / Columella Lengthening

Columella lengthening (primary rhinoplasty) - The ULTIMATE Clinical pearls To achieve the ultimate result various aspects have to be considered. Ignoring one or more of these aspects will lead to suboptimal aesthetic and functional results in columella lengthening (a minimal anterior primary bilateral cleft rhinoplasty).

1. Keep nasal sill and columella dissection minimal. Avoid extensive dissection of nasal dome and intra-crural soft tissue, as both are potential nasal growth centres. Do NOT destroy them as they help to provide normal nasal growth.

2. Adequately mobilise the skin of the columella as well as the anterior nasal floor including the nasal sill, this helps to avoid a “pig nose� appearance. To achieve appropriate nasal dome projection, the superior and median part of the crura are sutured.

3. Use former bilateral cleft lip scar, medial to the nasal sill, to place Burrow`s triangles, to avoid new scar formation and eliminate cutaneous tissue bulging at the lateral upper lip area. To avoid an additional scar, do not place a Burrow`s triangle lateral to the nasal sill!

4. A Burrow`s triangle may be used as an approach to the medio-central philtrum dimple to enhance a natural appearance of the central lip dimple with its philtrum edges (= the lip scars).

5. The superior aesthetic aspect of the philtrum edges can be enhanced, using the cranial part of the Burrow`s triangles to rotate these edges medially towards the naso-labial area.

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-9Anterior Nasal Floor

Unilateral and bilateral clefts (CLA and CLAP at lip-repair)

BĂœTOW K-W. Primary surgical repair of the anterior nasal floor. 7th Int Congr Cleft Palate and related Cranio-facial Anomalies 1993:224 [474] Broadbeach, Australia. Abstract.

BĂœTOW K-W. Primary anterior nasal floor reconstruction in CLP: its success. J Dent Res 1994: 73 [4], 998 (102).

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“Mirror-image” protocol

= reverse protocol

= “from inside to outside”

The anterior nasal floor is repaired during the lip-repair, however, before lip closure

134

Anterior Nasal Floor

Hard palate closure in a CLAP case is done prior to the anterior nasal floor repair, however, during the same operation The drawing below shows the position of the caudally-based vomer flap

Caudally-based vomer flap When using the cranial-based vomer flap (Lannelongue-Pichler), the anterior nasal floor flap has to be approached from the oral cavity to overlap raw surface closure

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Cleft - Ultimate Treatment

Typical appearance in a referred, already elsewhere operated or “inherited� patient, where the anterior nasal floor was NOT repaired (= bilateral anterior oro-nasal fistula)

136

Anterior Nasal Floor

Site from where tissue can be utilized to repair the anterior nasal floor

Anterior nasal floor repair in an unilateral CLAP.

When positioning the anterior nasal flap, the epithelial layer is turned towards the nasal cavity (for caudally-based vomer flap)

BÜTOW K-W. Caudally-based single-layer septum-Vomer flap for cleft palate closure. J Cranio-Maxillofac Surg 1987: 15: 10.

BÜTOW K-W. Primary anterior nasal floor reconstruction in facial cleft deformities. HandsOn 1994: 6: 33.

BÜTOW K-W. Primary surgical repair of the lip and nose. Treatment of Facial Cleft Deformities.

St Louis: Ishiyaku EuroAmerica 1995: 36-39.

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Anterior nasal floor flap derives from lip tissue excess.

Whereas it forms the nasal sheet, the vomer flap constitutes the oral sheet.

138

Anterior Nasal Floor

Bilateral CLAP (n = anterior nasal floor flap)

BROADBENT TR, WOOLF RM. Bilateral cleft lip repairs. Plast Reconstr Surg 1972: 50: 36.

BÜTOW K-W. Primary surgical repair of the lip and nose. Treatment of Facial Cleft Deformities. St Louis: Ishiyaku EuroAmerica 1995: 41 – 44.

139

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140

Anterior Nasal Floor

Anterior nasal floor can be used to cover a resorbable PdLAlLA plate

141

Cleft - Ultimate Treatment

Anterior nasal floor - The ULTIMATE Clinical pearls To achieve the ultimate result various aspects have to be considered. Ignoring one or more of these aspects will lead to suboptimal aesthetic and functional results in anterior nasal floor repair.

1. Not to repair the anterior nasal floor will lead to buccal and inter-alveolar oro-nasal fistulae. Delayed primary closure of such fistulae is much more challenging than a secondary oro-nasal fistulae repair of the hard and/or soft palate.

2. The repair of the anterior nasal floor avoids dislodgement of food and/or fluid from the oral into the nasal cavity and most other negative otorhinolaryngeal sequelae.

3. The anterior nasal floor lap creates a permanent ideal nasal layer to accommodate future bone transplants during secondary or tertiary osteoplasty.

4. Secondary closure of buccal and inter-alveolar oro-nasal fistula should only take place during the secondary osteoplasty at around nine years of age. Any attempt to create tissue for repair before this age will probable lead to midfacial growth disturbance (= dysgnathic growth).

5. The anterior nasal floor flap can also serve as a layer to cover a resorbable PdLAlLA plate, a means to facilitate alveolar arch growth and bone induction in a narrow alveolar cleft.

6. In bilateral CLAP cases the anterior nasal floor flap is larger in size in a modified Broadbent compared to the one for a Noordhoff-Trott lip-repair.

142

- 10 Hard Palate Cleft

A. BASIC SURGICAL REPAIR

B. ADVANCED SURGICAL REPAIR

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CLEFT HARD PALATE narrowed during the ALVEOLAR ARCHES alignment after a 5-month period of jaw-orthognathic movement

CUMBER E, BĂœTOW K-W, VAN DER MERWE CA. Presurgical jaw-orthognathic expansion in babies with complete unilateral cleft lip and palate. J Dent Res 1987: 66: 950.

144

Hard Palate Cleft

A. BASIC SURGICAL REPAIR Caudally-based vomer-flap Basically minimal growth disturbance 4 other types of Vomer flaps: - Lannelongue-Pichler, - Campbell, - Veau-Borel, - Stenström-Thilander

BÜTOW K-W. Caudally‑based septum‑vomer flap for primary cleft palate closure. 9th International Conference on Oral and Maxillo‑facial Surgery. 1986: 220; Vancouver, Canada.

BÜTOW K-W. Caudally-based single-layer septum-vomer flap for cleft palate closure. J Cranio-Maxillofac Surg 1987: 15; 10.

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Soft palate repaired 2 months before

 Tight closure between hP and sP  Minimum dissection = minimum growth disturbances  Minimum blood loss

146

Hard Palate Cleft

Cranially-based Vomer-flap (Lannelongue-Pichler vomer flap)  Very limited palatal dissection (= minimal growth disturbance)  Minimum blood loss  Often no tight closure between hP and sP, especially in wide cleft, due to a triangular connection (with soft palate closure proceeding)

BÜTOW K-W. “Dysgnathie nach Gaumenspaltenverschluss mit Vomerlappen” [German]. Doctorate publication, University of Erlangen‑Nuremburg [Germany] 1983.

BÜTOW K-W. A relationship measurement method for the analysis of complete lip and palate cases. Cleft Palate J 1984: 21; 317‑322.

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Cranially-based Vomer-flap (Lannelongue-Pichler vomer flap)

BÜTOW K‑W, STEINHÄUSER EW. Follow‑up of palatal closure by means of a one‑layer based vomer‑flap. Int J Oral Surg, 1984: 13; 396‑400.

148

Hard Palate Cleft

Releasing incision (Von Langenbeck type) Indication/requirements  Only for hard and soft palate cleft (hPsP) cases (NOT in presence of a alveolar cleft, as a CLAP)  In cases where a vomer-flap procedure is not feasible  In cases where a double layer closure is possible

Consequences  Wider palatal dissection leads to more growth disturbance  Increased blood loss  A tighter closure in the hP

VON LANGENBECK BM. Operationen der angeborenen totalen Spaltung des harten Gaumens nach einer neuen Methode. Dtsch Kliniken 1861: 8: 231.

DANNEMANN C, BÜTOW K-W. Introduction of two mattress sutures: a displaced and a crossover type, for hard palate closure. Br J Oral & Maxillofac Surg, 2009: 47; 564-565.

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Hard palate repair ďƒ Unilateral Von Langenbeck in an 18-month-old COMBI-cleft case Soft palate cleft repaired 11 months ago Hard palate cleft

150

Hard Palate Cleft

Hard palate reconstruction → Bilateral Von Langenbeck type, in an 18-month-old hPsP-cleft case (soft palate cleft closed at 7 months of age)

NAIDOO S, ROODE GJ, BÜTOW K-W. (2014) Palatal pain due to exostosis of posterior palatal spine in a cleft patient. J Cleft Lip Palate Craniofac An 1:124-126.

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Primary hard palate repair (Von Langenbeck) and soft palate (intravelar veloplasty) in an adult patient

152

Hard Palate Cleft

B. ADVANCED SURGICAL REPAIR

Resorbable PdLAlLA-sheet Engelbrecht H, Bütow K-W, Htun, SY, Heijnsbroek, S (2013) Novel use of a resorbable sheet in combination with a vomerine flap in cleft palate reconstruction. 21st Int Conf Oral Maxillofac Surg 176: T4.OR012.

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Use of a soluble PdLAlLA-sheet → The palatal cleft should be less than 6mm in width

154

in a narrow hP cleft case (i)

Hard Palate Cleft

Use of a soluble PdLAlLA-sheet in a narrow hP cleft case (ii) → The sheet is laid in warm water where it becomes malleable. Then it can be custom-made and inserted. On the left: sheet overlies vomer flap.

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Resorbable PdLAlLA-sheet (i)

156

Hard Palate Cleft

Resorbable PdLAlLA-sheet (ii)

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Hart palate repair - The ULTIMATE Clinical pearls To achieve the ultimate result various aspects have to be considered. Ignoring one or more of these aspects will lead to suboptimal aesthetic and functional results in hard palate (hP) repair.

1. Stripping muco-periosteal layer from the hard palate in uni- or bi-lateral CLAPs leads to midfacial growth disturbance or dysgnathia. This has to be avoided by all means.

2. In hPsP clefts, the dento-alveolar arch is a single unit. Therefore the hard palate can be repaired by partially stripping the mucoperiosteal layer from the palate. The flap width should not be less than 1.0X, and not exceed 1.5X of the hP cleft width, respectively. Furthermore, if teeth are present, the palatal gingival tooth margins should not be infringed.

3. In a V-shaped hPsP the cleft can be repaired during the same surgery. In a U-shaped hPsP the repair should be done in two surgical sessions to avoid possible formation of oro-nasal fistula. The hP is usually repaired 11 to 12 months after the sP. 4. In CLAP cases the cranially- or superiorly-pedicled vomer flap can be covered by a PdLAlLA-sheet to achieve palatal healing ideally without a possible central groove. Nevertheless an oro-nasal fistula might occur at the junction of the hP and sP. 5. In hPsP cleft cases, a hard palate cleft of 6 mm width or less, can be repaired with a single nasal mucosal layer with a PdLAlLA sheet.

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- 11 Soft Palate Cleft

A. BASIC SURGICAL REPAIR

B. ADVANCED SURGICAL REPAIR

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A. BASIC SURGICAL REPAIR SOFT PALATE REPAIR 1. INTRAVELAR VELOPLASTY (Type II)

2. WIDMAIER V-Y

3. FURLOW and TPE

4. INTRAVELAR VELOPLASTY (Type III)

5. Primary cleft repair for the older children and adult

160

Soft Palate Cleft

1. INTRAVELAR VELOPLASTY (Type II)  

At 5 months of age in complete CLAP cases At 7 months of age in sP or hPsP cases

KRIENS OB. An anatomical approach to veloplasty. Plast Reconstr Surg 1969: 43: 27.

BÜTOW K-W, JACOBS FJ. Intravelar veloplasty: Surgical modification according to anatomical defect. Int J Oral Maxillofac Surg 1991: 20: 296.

BÜTOW K-W, LOUW B, HUGO SR, GRIMBEECK RJ. Tensor veli palatini muscle tension sling for Eustachian tube function in cleft palate. J Cranio-Maxillofac Surg 1991: 19: 71.

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Soft palate cleftMuscle repair in intravelar veloplasty (type II)

162

Soft Palate Cleft

Intravelar veloplasty - Type II in a soft palate cleft (i)

Soft palate cleft - Muscle repair in intravelar veloplasty (type II) 163

Cleft - Ultimate Treatment

Intravelar veloplasty - Type II in a soft palate cleft (ii)

164

Soft Palate Cleft

Intravelar veloplasty - Type II in a soft palate cleft (i) Adenoid tissue Incision line – at oronasal mucosal junction

Repair – at 5 months of age in CLAP and at 7 months in hPsP and sP cases Watch carefully!

- Symmetrical appearance of hard and soft palate, - Distance of pharyngeal wall from the soft palate, - Cleft width → generally the wider the cleft, the shorter the soft palate

Local infiltration: - 1: 200 000 Adrenalin in palatal area - Inject pure saline into the uvula 165

Cleft - Ultimate Treatment

Intravelar veloplasty - Type II in a soft palate cleft (ii)

Incision → dissect the three layers - First nasal mucosa - Oral mucosa from the muscle layer beneath - Release muscles at the posterior nasal spine and from the posterior hard palate edge

166

Soft Palate Cleft

Intravelar veloplasty - Type II in a soft palate cleft (iii)

- Nasal facing layer is sutured, starting from the uvula.

- Place inverted sutures with knots toward the nasopharynx.

Palatoglossal and palatopharngeal muscle sutures (these should be separate, otherwise one figure 8 suture for the bundle of these two mucles) 167

Cleft - Ultimate Treatment

Intravelar veloplasty - Type II in a soft palate cleft (iv)

Suture levator palatini muscles with vertical mattress.

NOTE! Do not knot the stitch at this stage, just position the sutures!

Lateral palatal incision to release oral mucosa layer tension (arrow) M. Tensor veli palatini

Trick! Including the nasal mucosal layer in the first stitch prevents dead space and hematoma formation! (arrow) 168

Hamulus area

Soft Palate Cleft

Intravelar veloplasty - Type II in a soft palate cleft (v) (1)

(2)

(3)

1.

2.

3.

Suture of oral mucosa – starting at uvula Suture of levator muscle avoiding any tension Spongostan and running suture lateral to incision

Soft palate repair in a CLAP case 169

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Soft palate repair Intravelar velo-plasty; 3 years after operation

170

Soft Palate Cleft

2. WIDMAIER VELOPLASTY with muscle repair and tensor sling procedure At 5 months in complete CLAP cases

 

At 7 months in sP and hPsP cases

WIDMAIER W. Ein neues Verfahren zum Verschluss der Gaumenspalten. Chir 1959: 30: 274.

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FURLOW PLASTY (PROCEDURE IN NARROW CLEFTS) AND PARKER-FURLOW (WITH TENSOR STITCH PROCEDURE)

Tensor sling procedure

172

Soft Palate Cleft

3. FURLOW VELOPLASTY with Tensor procedure (Procedure only in narrow clefts) and Parker-Furlow plasty  

At 5 months in complete CLAP cases At 7 months in sP and hPsP cases

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Furlow z-plasty for the soft palate cleft

FURLOW LT. Cleft palate repair by double opposing Z-plasty. Plast Reconstr Surg 1986: 78: 724.

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Soft Palate Cleft

Furlow plasty

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Parker-Furlow veloplasty using buccal fat pad for lengthening of the soft palate

Bilateral buccal fat pad for lengthening TVEDT D, PARKER G. A modification of the Furlow palatoplasty utilizing bilateral buccal fat pad grafts. 95th Ann Meeting Am Ass Oral Maxillofac Surg 2013: P 14: e-47 – e-48.

176

Soft Palate Cleft

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Parker-Furlow advanced veloplasty

178

Soft Palate Cleft

Single uvula reconstruction for posterior palate lengthening Tensor Stitch for Eustachian tube ventilation Bilateral pedicled Buccal Fat Pad

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180

-

Bilateral buccal fat pad for central lengthening

-

Tensor stitch for Eustachian tube patency

-

Palatoglossal + palato-pharyngeal muscle suture

-

Single uvula reconstruction for posterior lengthening

Soft Palate Cleft

4. INTRAVELAR VELOPLASTY (type III) as SOMMERLAD’S LEVATOR + PALATOPHARYNGEUS, - GLOSSUS MUSCLE REPAIR, TENSOR SLING PROCEDUREwith IVANOV’S UVULA RECONSTRUCTION

 

At 5 months in complete CLAP cases At 7 months in sP and hPsP cases

BÜTOW K-W, JACOBS FJ. Intravelar veloplasty: Surgical modification according to anatomical defect. Int J Oral Maxillofac Surg 1991: 20: 296.

SOMMERLAD BC. A technique for cleft palate repair. Plast Reconstr Surg 2003: 112: 1542.

IVANOV A, AGUEEVA L. Veloplasty using single uvular and double-opposing flaps. J Cranio-Maxillofac Surg 2008: 36:S22.

BÜTOW K-W, LOUW B, HUGO SR, GRIMBEECK RJ. Tensor veli palatini muscle tension sling for Eustachian tube function in cleft palate. J Cranio-Maxillofac Surg 1991: 19: 71.

BÜTOW K-W, ENGELBRECHT H, NAIDOO S. Asymmetrical soft palate cleft construction. Int J Oral Maxillofac Surg 2014; 43: 696-701.

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INTRAVELAR VELOPLASTY (IVV) Type I

Described originally by Kriens, 1969 [Braithwaite & Maurice, 1968] BRAITHWAITE F, MAURICE DG. The importance of the levator palatine muscle in cleft plate closure. Br J Plast Surg 1968; 21: 60-62.

KRIENS OB. An anatomical approach to veloplasty. Plast Reconstr Surg 1969; 43: 29-41.

Type II (= Type I) Bütow, K-W & Jacobs, FJ

According to the anatomical defect: 7 surgical modifications in preventing oro-nasal fistulae, as well as the non-fracture of the hamulus with adjacent lateral releasing incisions and insertion of the tensor stitch.

BÜTOW K-W, JACOBS FJ. Intravelar veloplasty: surgical modification according to anatomical defect. Int J Oral Surg. 1991; 20;296-300.

Type III (= Type II modification) Additional muscle alignments and specific uvula reconstruction, according to Sommerlad BC; Ivanov A and Agueeva L SOMMERLAD BC. A technique for cleft palate repair. Plast Reconstr Surg 2003; 112: 1542-1548.

IVANOV A, AGUEEVA L. Veloplasty using single uvular and double opposing flaps. J Cranio-Maxillofac Surg. 2008; 36: s22 [O.088].

BÜTOW K-W, ENGELBRECHT H, NAIDOO S. Asymmetrical soft palate cleft construction. Int J Oral Maxillofac Surg 2014: http://dy.doi.org/10.1016/j.ijom.2013.05.018.

182

Soft Palate Cleft

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Intravelar veloplasty and Sommerlad’s levator + palatopharyngeus, -glossus repair and Ivanov’s uvula repair and tensor sling procedure –Type III 184

Soft Palate Cleft

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Feeding tube for 7 days (NO oral feeding)

Arms are splinted, to prevent flexion in elbows !!! → This protects the intravenous line and nasogastral feeding tube

186

Soft Palate Cleft

Infection control Infection = the biggest threat during the healing process ▪ Visible infection = NO operation ▪ Invisible = apparently healthy → microorganisms in the nasal cavity, oro-, naso- and laryngopharynx and/or lungs.

(Feco-oral cross infection = severe form, with often partial breakdown) Prevention = inoperative, before surgical cleaning, take a pus swap. Thereafter every 2nd day until hospital discharge.

Change the antimicrobial drug according to the pus swap result.

MYBURGH HP, BŰTOW K-W. The peri-operative intra-oral microbiological profile of babies with cleft facial deformities – a South Africa database.

Int J Oral Maxillofac Surg 2009: 38; 928-932.

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5. Primary Cleft Repair for The Older Child And Adult

(The majority is unable to attend follow-up controls in a multidisciplinary clinic)

188

Primary Cleft Repairfor The Older Child And Adult

Initial primary repair on an adult (hPsP cleft case)

Von Langenbeck procedure with muscle repair 189

Cleft - Ultimate Treatment

Primary hard palate repair (Ernst-Dorrance) in an adult patient (must not be used in infants → as it causes midfacial growth disturbance)

190

Primary Cleft Repairfor The Older Child And Adult

Primary hard palate repair (Ernst-Dorrance) in an adult patient

191

Cleft - Ultimate Treatment

B. ADVANCED SURGICAL REPAIR The repair of the asymmetrical soft palate cleft with tissue deficiency on one side represents a big challenge.

BĂœTOW K-W, NAIDOO S, JACOBS FJ. Advanced intravelar veloplasty for asymmetrical soft palate cleft reconstruction. International Conference on Oral and Maxillofacial Surgery, Santiago, Chile 2011: abstract on congress CD.

192

Primary Cleft Repairfor The Older Child And Adult

Severe asymmetrical cleft soft palate

The defect

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IVV-type III on a symmetrical soft palate cleft (1)

(2)

(4)

194

(3)

(5)

Primary Cleft Repairfor The Older Child And Adult

Levator -, palatoglossal and palatopharyngeal muscles alignment

195

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Tensor sling

Mattress sutures

Releasing incision

Commencement & Final

196

Primary Cleft Repairfor The Older Child And Adult

Asymmetrical soft palate cleft reconstruction

Levator-, palatoglossal and palatopharyngeal muscles’ alignment 197

Cleft - Ultimate Treatment

198

Primary Cleft Repairfor The Older Child And Adult

Post-operative: asymmetry in a case with partial submucosal cleft (in some cases, minor obliqueness of the uvula remains as shown by the arrow) 199

Cleft - Ultimate Treatment

C. TENSOR SLING PROCEDURE A “tensor sling” is positioned in sP cleft to activate the tensor veli palatini muscles being responsible for the Eustachian tube patency

BÜTOW K-W, LOUW B, HUGO SR, GRIMBEECK RJ. Tensor veli palatini muscle tension sling for Eustachian tube function in cleft palate. J Cranio-Maxillofac Surg 1991: 19: 71.

REDELINGHUYS IF, BÜTOW K-W. Improved patency of the Eustachian tube in cleft palate patients after veloplasty by means of a tensor sling. Triangular maxillofacial and oral surgeons congress (S Afr, Brit, Netherl) Cape Town 1996: 83 [83].

200

Primary Cleft Repairfor The Older Child And Adult

Tensor sling activates tensor veli palatini muscles

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M. tensor veli palatini tendon activation

Always medially to the hamulus

To activate the Eustachian tube

Out again

Tighten!! and re-tighten by pulling the stitch tightly towards an inferior direction!

Knot

202

Primary Cleft Repairfor The Older Child And Adult

(1) Tensor sling and closure of soft palate (2) intraoperative and (3) 3 weeks after operation

(2)

(1)

(3)

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Soft palate repair - The ULTIMATE Clinical pearls To achieve the ultimate result various aspects have to be considered. Ignoring one or more of these aspects will lead to suboptimal aesthetic and functional results in soft palate (sP) repair.

1. The sP repair is the most delicate part of cleft repair. Healing could be compromised by various complications, of which post-operative infection is the most common.

2. The broader and/or the shorter the non-operated soft palate, generally the shorter the repaired soft palate. Even though surgical procedures lengthen soft palate, post-operatively it might still be too short. The IVV iii-technique is recommended for the repair of the narrow (< 5mm) cleft in the long soft palate, whereas the PF-adv.-technique should be used in cases of a narrow cleft for a short soft palate.

3. The closure of nasal and oral mucosa has to be tension-free. Any tension might lead to the breakdown of wound healing. Type III-intravelar veloplasty enhances a tension-free closure of the nasal mucosa.

4. Baseline microbial evaluation has to precede each surgical intervention. Though patients might appear clinically non-infectious, pre-operative microbial culture obtained during general anaesthesia, might reveal pathogenic microorganisms.

5. Microbial cultures must be repeated post-operatively on day 2, 4 and 6, as the spectrum of micro-organisms can change, due to emerging opportunistic micro-organisms resulting from intravenous antibiotic treatment and antiseptic mouth rinse.

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Primary Cleft Repairfor The Older Child And Adult

Soft palate repair - The ULTIMATE Clinical pearls, continued 6. As soon as microbial results are available, the antimicrobial regimen has to be adapted to the most appropriate paediatric antibiotic drug and tolerable dose.

7. When strings, small blisters and/or cloudy-whitish saliva are observed at the surgical wound during the daily postoperative clinical control, the patient may suffer from a gram-negative bacterial infection. Antibiotics against gramnegative micro-organisms might be indicated before the final result of the antibacterial culture is available.

8. To keep the oral cavity free from any fluid and solid food and to avoid food becoming trapped within the surgical wound and within the releasing incisions, patient feeding via nasogastric tube should be maintained for 6 to 7 days post-operatively.

9. Gastric reflux is detrimental for the healing process of the soft palate. Persistent pre-operative reflux has to be treated medically or surgically before the soft palate repair.

10. Vomiting needs to be avoided by all means, therefore each intake should be minus 10% compared to the pre-operative intake at home.

11. In case of vomiting, immediate extensive oral rinsing is indicated, especially at the site of the releasing incisions, as resorbable filling and haemostatic package material in these sites might act like a sponge, accumulating gastric fluid and/or milk products, which are both detrimental for healing.

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PRIMARY and/or SECONDARY CLEFT TREATMENT

207

- 12 Velopharyngeal Incompetency

A. FLUOROGRAPHY B. VELOPHARYNGEAL FLAP C. DYNAMIC SPHINCTERPLASTY D. ANTERO-POSERIOR PALATAL DISTRACTION

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A. FLUOROGRAPHY Fluorography (the visualization of soft palate and pharyngeal movement during speech) can be examined by video- or cinematography or standard radiographs in a young patient or by naso-endoscopic examination in the adolescent or adult patient.

210

Velopharyngeal Incompetency

RADIOGRAPHIC FLUOROGRAPHY to evaluate velopharyngeal competency (±4 years of age)

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FLUOROGRAPHY

1. soft palate - arrow 212

2. adenoid glands - arrow

3. velopharyngeal incompetency â&#x20AC;&#x201C; arrows

Velopharyngeal Incompetency

Velopharyngeal incompetence

HISTORY: Treatment with speech bulb (often including an obturator part for the oro-nasal fistula)

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B. VELOPHARYNGEAL FLAP The superior pedicled velopharyngeal flap is the 1st choice to repair velopharyngeal incompetency Competency is derived by two aspects: bridging the soft palate – naso-oro-pharynx gap and reducing the volume of the naso-oro-pharynx

ROSENTHAL W. Die Velopharyngoplastiek. Langenbecks Arch Klin Chir 1960: 295: 912.

SANVENERO-ROSSELLI G: Cleft palate plastic surgery using a pharyngeal flap. Langenbecks Arch Klin Chir Ver Dtsch Z Chir 1960: 295: 895-900.

BÜTOW K‑W . Speech-language therapy and velopharyngeal incompetence. In: Treatment of Facial Cleft Deformities. Ishiyaku EuroAmerica, Inc. Publishers, St Louis, Tokyo 1995: pp.

56-58.

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Velopharyngeal Incompetency

Velopharyngeal flap (NB automatically the naso-oro-pharynx volume is reduced in lifting the flap) (if indicated; at Âą 4 years )

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Pharynx Soft palate

Evaluation of: - Adenoid quality)

glands

(soft

tissue

- Palatal width - Note: course of aberrant internal carotid artery (in velo-cardiofacial or Shprintzen syndrome) (Check ridge and pulsating area!)

Carefully watch and measure distance from the soft palate to the pharyngeal wall utilize a Freer elevator with artery forceps to determine the length of the flap

216

Velopharyngeal Incompetency

Complete preparation of the flap (dissected area of naso-oropharynael mucosa) Prevertebral fascia

Next step: Closure of the pharyngeal wall; first in the middle then from caudal to cranial!

Pharyngeal flap

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Preparation of the â&#x20AC;&#x153;flap bed â&#x20AC;? at the soft palate Carefully dissect parallel to the directions of the muscle fibres and finally through the naso-pharyngeal muscosa

218

Velopharyngeal Incompetency

Start suturing at the palate

After penetrating the “flap bed ” through the opening in the soft palate, the suture for the flap is placed from the raw through to the mucosal surface of the flap.

PDS stitch (first): Most important suture of entire operation!

Similar to a horizontal mattress suture

Back into the “flap bed ”

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Place an additional suture at both corners of the flap



Do not tie them yet!!

Flap has to be positioned through the incision into the “flap bed” of the soft palate

The operation can be done without major blood loss – immediate feeding post-operatively, initially with clear fluid

Flap

The darker tissue seen between the forceps is the velopharyngeal flap (presents always slightly darker) (arrow)

VAN DER MERWE AEA, BÜTOW K-W. Post-operative speech evaluation after velopharyngeal flap reconstruction in cleft palate. J Dent Res, 1995: 74 [3]; 1024 (129).

220

Velopharyngeal Incompetency

Superior pedicled velopharyngeal flap (i)

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Superior pedicled velopharyngeal flap (ii)

222

Velopharyngeal Incompetency

Revision of an inferior-pedicled velopharyngeal flap (â&#x20AC;&#x153;inheritedâ&#x20AC;? patient ) NB: An inferior-pedicled flap position should never be the choice in this type of reconstruction

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C. DYNAMIC SPHINCTER PLASTY The dynamic sphincter plasty is mostly a 2nd choice for the repair in velopharyngeal incompetency cases (generally bilateral flaps)

ORTICOCHEA M. The dynamic muscle sphincter of the pharynx. In Bardach J, Morris HL: Multidisciplinary management of cleft lip and palate. Philadelphia, WB Saunders & Co 1990: 378.

BÜTOW K‑W . Speech-language therapy and velopharyngeal incompetence. In: Treatment of Facial Cleft Deformities. Ishiyaku EuroAmerica, Inc. Publishers, St Louis, Tokyo 1995: pp. 58+59.

224

Velopharyngeal Incompetency

Dynamic sphincter plasty

225

Cleft - Ultimate Treatment

Dynamic sphincter plasty (incorporated to a superior pedicled velopharyngeal flap)

226

Velopharyngeal Incompetency

D. PALATAL DISTRACTION OSTEOGENESIS Distraction osteogenesis is an additional choice to repair velopharyngeal incompetency cases Only for patients above 9 years of age [â&#x2026;&#x201D; completion of skeletal facial growth] and in a partial [up to 70%] or complete bony hP

BĂ&#x153;TOW K-W. Palatal distraction an option for velopharyngeal incompetence? J Cranio-Maxillofac Surg 2002: 30: Suppl 1: 159.

227

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Distraction osteogenesis

228

Velopharyngeal Incompetency

The distractor and the osteotomy lines (The greater palatine vascular bundle should not be involved. The osteotomy incisions are adjacent and medially to the greater palatine foramen)

229

Cleft - Ultimate Treatment

Distraction osteogenesis

Radiographic control after distractionosteogenesisfor velo-pharyngeal incompetence

230

Velopharyngeal Incompetency

Velopharyngeal incompetency and its repair - The ULTIMATE Clinical pearls To achieve the ultimate result various aspects have to be considered. Ignoring one or more of these aspects will lead to suboptimal functional results in velopharyngeal incompetency repair.

1. The velopharyngeal repair is indicated in children at 4 to 4½ years of age, if intensive speech therapy does not lead to sufficient improvement and anatomical velopharyngeal sphincter competence.

2. The wider the initial defect of the sP cleft and the shorter the repaired soft palate, the higher the incidence of velopharyngeal incompetence .

3. The shorter the repaired soft palate and the less adenoid gland tissue, the higher the incidence of velopharyngeal incompetence. Therefore, adenoid gland tissue should never be removed or trimmed. Tonsillectomy, however, can be performed in cases where adequate muscular repair was done during the primary soft palate repair.

4. A velopharyngeal flap should never ever be inferiorly pedicled, as it will limit the normal superior lifting of the soft palate.

5. Dynamic sphincter plasty should not be used as the 1st choice, however, as an adjunct surgical procedure to achieve sufficient closure, with incomplete closure after a velopharyngeal flap procedure or for a very fibrotic soft palate.

Bilateral flaps may be created from the tonsillar pillars. If this tissue is not available, then from the oropharyngeal wall.

6. Palatal distraction osteogenesis in antero-posterior direction should only be performed after 2/3 facial skeleton growth has been completed, at around 9 years of age. Furthermore it should only be used in non-hP cleft cases or in cases which have up to 30% hP cleft deformity.

231

- 13 Oro-Nasal Fistula

A. LOCAL FLAPS

B. DISTANCE FLAPS

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A. LOCAL FLAPS Various local flap designs are available to repair oro-nasal fistulae. The choice depends on the type, the position, the depth and the scars surrounding the fistula.

BĂ&#x153;TOW Kâ&#x20AC;&#x2018;W Oronasal fistula. In: Treatment of Facial Cleft Deformities. Ishiyaku EuroAmerica, Inc. Publishers, St Louis, Tokyo 1995: pp. 61 -68.

234

Oro-Nasal Fistula

ORO-NASAL FISTULAE repair Âą 9 - 13 years (Unilateral technique of von Langenbeck)

VON LANGENBECK BM. Operationen der angeborenen totalen Spaltung des harten Gaumens nach einer neuen Methode. Dtsch Kliniken 1861: 8: 231.

235

Cleft - Ultimate Treatment

(Bilateral von Langenbeck technique)

Placement of Spongostan into the lateral releasing incision

236

Oro-Nasal Fistula

Special mattress suturing for oro-nasal fistula during unilateral or bilateral von Langenbeck flap technique A) Displaced mattress suture B) Crossover mattress suture DANNEMANN C, BĂ&#x153;TOW K-W. Introduction of two mattress sutures: a displaced and a crossover type, for hard palate closure. Br J Oral & Maxillofac Surg, 2009: 47; 564-565.

237

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Oro-nasal fistula 238

closed by single-layered buccal fat pad

Oro-Nasal Fistula

Buccal fat pad-flap in a triple layer closure

BĂ&#x153;TOW K-W. Tongue flap, buccal fat pad, attachment for recurring oronasal fistula. J Dent Ass S Afr, 1987: 42; 210â&#x20AC;&#x2018;204.

239

Cleft - Ultimate Treatment

Advanced repair of a narrow hard palate cleft using a custom prepared resorbable sheet (PdLAlLA)

240

Oro-Nasal Fistula

Custom made resorbable sheet

Before placement

After placement and sutured 241

Cleft - Ultimate Treatment

B. DISTANT FLAPS Various distant flaps are available to repair oro-nasal fistulae. The choice depends on the size, the position of the fistulae, as well as on the patient’s consent for the area of harvest.

BÜTOW K‑W The compromised adolescent / adult. In: Treatment of Facial Cleft Deformities. Ishiyaku EuroAmerica, Inc. Publishers, St Louis, Tokyo 1995: pp. 115-119.

242

Oro-Nasal Fistula

Tongue flap (posteriorly pedicled)

243

Cleft - Ultimate Treatment

244

Oro-Nasal Fistula

Major oro-nasal fistula (â&#x20AC;&#x153;inheritedâ&#x20AC;? patient) Dissection of a tripartite tongue flap

245

Cleft - Ultimate Treatment

Additional rotational tongue flap (“inherited” patient) Derived from a previous tongue- flap

BÜTOW K-W, DUVENAGE JG Pedicled “flap” derived from a tongue flap. Int J Oral and Maxillofac Surg 1986: 15; 581‑584

246

Oro-Nasal Fistula

Major oro-nasal fistula in a resected premaxilla case Tongue flap “sandwich” + dento-alveolar ridge bone graft with dental implants

BÜTOW K-W, BRIEDENHANN SJ & KOEPP WG Comprehensive secondary cleft deformity reconstruction: Case report. Hands-On, 1996: 8(1); 61-65.

247

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Oro-nasal fistulae and their repair - The ULTIMATE Clinical pearls To achieve the ultimate result various aspects have to be considered. Ignoring one or more of these aspects will lead to suboptimal aesthetic and functional results in oro-nasal fistulae repair.

1. An “oro-nasal fistula” as an incomplete closure in a hP cleft after primary sP cleft surgery, should be called “unrepaired partial hard palate cleft” (hPsP). True oro-nasal fistulae are residues, mainly of unsuccessful hard and/or less soft palate cleft repair. The repair of true oro-nasal fistulae is performed by secondary surgical intervention.

2. As most procedures entail stripping off the palatal mucoperiosteal layer, such secondary repair should only be performed after 2/3 of the facial growth has been completed, after ±9 years of age. Surgery prior to this age leads mostly to midfacial growth disturbance. Therefore, in rare cases, prosthodontic obturator therapy may be indicated up to the age of 9 years.

2. Local flaps are the preferred choice for repair. Only after unsuccessful palatal flap closure, local-pedicled flaps, as buccal fat pad, tongue, temporal muscle, and naso-labial flap, or distant and microvascular flaps should be considered.

Distant flap repair is generally reserved for more compromised cleft patients, the so-called non-ultimate repair cases (see chapter 25: ‘cleft syndrome appearances’) and those cases with underlying syndromes, mostly involving the embryological ectodermal component.

4. A large number of different local and distant flap procedures are available. The tongue flaps, anterior-, posterior-, lateral-pedicled or as a rolling flap, are very versatile. Additional rotational flaps can be performed from a tongue flap due to its exceptional blood supply.

5. Very seldom one has to revert to a regional pedicled or a distant microvascular flap to repair oro-nasal fistulae.

248

SECONDARY CLEFT TREATMENT

249

- 14 Osteoplasty/Osteofusion

A. UNILATERAL DENTO-ALVEOLAR CLEFT B. BILATERAL DENTO-ALVEOLAR CLEFT C. BONE HARVESTING D. TIMING OF SECONDARY AND TERTIARY OSTEOPLASTY

251

Cleft - Ultimate Treatment

A. UNILATERAL DENTO-ALVEOLAR CLEFT There are two basic local buccal flap designs available to accommodate the grafted bone in the repaired dento-alveolar cleft. If an anterior nasal floor is nonexistent, an additional flap repair for this area is needed.

DRACHTER R. “Die Gaumenspalte und deren operative Behandlung.” Dtsch Z Chir 1914;131: 1-89.

STELLMACH RK. Bone grafting of the alveolus followed by orthopedic alignment of the alveolar arch in infants with complete cleft lip and cleft palate. Oral Surg 1963; 16: 897.

BÜTOW K‑W Perioplasty and osteoplasty. In: Treatment of Facial Cleft Deformities. Ishiyaku EuroAmerica, Inc. Publishers, St Louis, Tokyo 1995: pp. 82-86.

252

Osteoplasty/Osteofusion

SECONDARY OSTEOPLASTY Âą9 - 13 years Utilising a transpositional flap with attached gingiva ideally at Âą 9 years, however, pre-surgical orthodontic treatment is generally indicated first

253

Cleft - Ultimate Treatment

Secondary Osteoplasty + augmentation of the right alar base area using an autologous bone graft

Autologous bone graft with cortical and cancellous portions en bloc. Hard cortical bone positioned buccally, cancellous bone toward palatal side.

254

Osteoplasty/Osteofusion

Charity case : Osteoplasty in an once-off procedure with palatal reconstruction, anterior maxillary segmental osteotomy, anterior nasal floor reconstruction and lip-plasty

255

Cleft - Ultimate Treatment

B. BILATERAL DENTO-ALVEOLAR CLEFT Bilateral transpositional gingival flaps created out of tissue posteriorly to the cleft are used to close the cleft area.

BÜTOW K‑W Perioplasty and osteoplasty. In: Treatment of Facial Cleft Deformities. Ishiyaku EuroAmerica, Inc. Publishers, St Louis, Tokyo 1995: pp. 82-86.

256

Osteoplasty/Osteofusion

Bilateral osteoplasty

Final closure

Right side

Right side

Left side

Right side

257

Cleft - Ultimate Treatment

C. BONE HARVESTING Bone may be harvested from the iliac crest (either involving the superior border or not) or from the tibia, calvaria, chin or rib. Bone may also be harvested during orthognathic procedure.

BĂ&#x153;TOW Kâ&#x20AC;&#x2018;W Perioplasty and osteoplasty. In: Treatment of Facial Cleft Deformities. Ishiyaku EuroAmerica, Inc. Publishers, St Louis, Tokyo 1995: P. 85.

258

Osteoplasty/Osteofusion

Bone harvesting for the osteoplasty from the iliac crest

The en bloc part harvested for the size of alveolar defect

259

Cleft - Ultimate Treatment

Osteoplasty and alar base augmentation - The ULTIMATE Clinical pearls To achieve the ultimate result various aspects have to be considered. Ignoring one or more of these aspects will lead to suboptimal aesthetic and functional results in osteoplasty and alar base augmentation.

1. If anterior nasal floor repair was performed during infancy for primary cleft repair of the hard palate and lip, tight boundaries at the nasal floor might be found with tissue layer between the nasal and oral cavity. Sometimes it is necessary to reduce part of this nasal floor tissue to provide additional space for the bone graft.

2. In existing buccal and/or anterior palatal oro-nasal fistulae, an anterior nasal floor has to be created to achieve a tight superior layer to accommodate the bone graft. Even a small opening to the nasal cavity leads to bone graft infection and necrosis. (Note: bone graft exposure to the maxillary sinus, as in LeFort I osteotomies does not lead to bone necrosis).

3. Basically, alveolar bone grafts do not resorb in their cranio-caudal (supero-inferior) dimension, if the cortical bone part covers the total buccal surface of the alveolar cleft. Using cancellous bone only for augmentation leads to vertical and often to horizontal bone resorption.

4. Alveolar bone remodelling can occur from the palatal side due to soft tissue pressure. If at a later stage dental implantation is considered in the augmented former alveolar cleft, it may be necessary to remove soft tissue at the palatal side and transplant additional bone to this site prior to the dental implantation.

260

Osteoplasty/Osteofusion

Osteoplasty and alar base augmentation - The ULTIMATE Clinical pearls, continued 5. Alveolar cleft bone margins may be taper-, not box-shaped in the antero-posterior dimension. An adequate amount of cancellous bone therefore has to be positioned at the dento-alveolar arch at palatal side to increase its anteroposterior thickness.

6. Gingival transposition flaps should extend distally to at least the 1st molar, to allow for an adequate range of rotation.

To facilitate rotation, the flap base can be dissected slightly narrower. At the base the periosteal layer has to be incised along its full length. The flap should be entirely mobile, being extendable towards the occlusal plane. The dentoalveolar cleft defect must be covered and tension free. This rotational flap also transports attached gingiva to cover the dento-alveolar cleft area, providing optimal long-term gingival and periodontal health.

7. Preferably the flap should not include dental papillae. After bone grafting, flap closure should be without tension due to papillae position.

8. Bone can be harvested from various sites, such as the iliac crest, tibia, calvaria, mandibular symphyseal area, retromolar area and ribs. An en bloc bone graft includes cortical and cancellous bone. Additionally cancellous bone pieces can be harvested from the anterio-superior iliac spine.

9. Paranasal bone augmentation: Additional bone may be harvested and positioned on one each side of the piriform aperture to augment mild midfacial deficiency or mild maxillary retrognathism in a Class I occlusion.

261

- 15 Orthognathic Treatment (Applicable For Facial Cleft Deformities) (A) Cephalometry (B) MAXILLARY osteotomies (C) MANDIBULAR osteotomies (D) OSTEOTOMIES in osteo-distraction cases

263

Cleft - Ultimate Treatment

a. cephalometry There are various cephalometric, soft and hard tissue analyses systems available, both 2- and 3-dimensional.

The selected analysis should include the evaluation of facial soft tissue which is of paramount importance, as well as the analysis of facial bones and teeth to determine the extent of orthognathic deformity in cleft patients.

BÜTOW K-W. A lateral photometric analysis for aesthetic- orthognathial surgery. J Max‑fac Surg, 1984: 12; 201‑207.

BÜTOW K-W. Extension of cephalo‑photometric analysis. J Cranio‑Max‑Fac Surg 1987: 15; 74‑78.

264

Orthognathic treatment (applicable for facial cleft deformities)

Profilo-cephalometric analysis - a combination of soft tissue, skeletal and dental tissue analysis

BÜTOW K-W, MÜLLER, GW, DE MÛELENAERE JJGG. Profilocephalometric analysis: a combination of the cephalo-photometric and the architectural‑structural craniofacial analyses.

Int J Adult Orthod Orthogn Surg 1989: 4; 87‑104.

265

Cleft - Ultimate Treatment

PROFILO-CEPHALOMETRIC EVALUATION AND DIAGNOSIS: Retro-longi-zygoma, retro-longi-nasal structure, maxillary retrognathia, apertognathia (anterior open bite), mandibular retro-longigenion (a mild Treacher-Collins syndrome)

266

Orthognathic treatment (applicable for facial cleft deformities)

PA-cephalometry (for 3-D-cephalometry)

Basilar cephalometry (for 3-D-cephalometry)

Essential for cleft deformity cases with asymmetric facial appearance, in particular for the Goldenhar, oto-mandibular dysostosis (or hemifacial microsomia) and others.

BÜTOW K-W, VAN DER WALT PJ. PA ‑ and basal cephalometry: A three dimensional analysis of facial deformities. J Dent Res, 1981: 60; 1269.

BÜTOW K-W, VAN DER WALT PJ. The “Stellenbosch” triangle analysis of postero‑anterior and basilar cephalograms. J Dent Ass S Afr 1981: 36; 461‑467.

267

Cleft - Ultimate Treatment

B. Maxillary OSTEOTOMIES Cleft maxillae exhibit a more compromised blood supply due to the abnormal anatomy and scar tissue formation resulting from former primary cleft repair procedures.

The majority of midfacial osteotomies may also be carried out in cleft maxillae cases as in non-cleft maxillae cases, however with caution.

268

Orthognathic treatment (applicable for facial cleft deformities)

Typical Maxillary and midfacial deformity in a cleft patient Maxillary retrognathia (hypoplasia)

Naso-maxillo-acro-dysostosis (Binder syndrome) (Tessier 0) with or without standard cleft lip (Tessier 2-3) or hPsP

BÜTOW K-W, JACOBSOHN PV, DE WITT TW. Naso‑maxillo‑acro‑dysostosis. S Afr Med J, 1989: 75, 5‑11.

ZELTSER R, BÜTOW K-W. Cleft lip and/or palate associated with naso-maxillo-acro-dysostosis. J Dent Res, 1992: 71; 1108 [57].

269

Cleft - Ultimate Treatment

Diagnosis: maxillary brevi-retrognathia LeFort I osteotomy (to position maxilla antero-inferiorly)

Diagnosis: midfacial hypoplasia (with or without palatal cleft) Pyramidal LeFort II osteotomy and nasal reconstruction using a midfacial degloving approach

BÜTOW K‑W, JACOBSOHN PV, DUVENAGE JG, NEL F. SWART R, DE MÛELENAERE JJGG. Treatment of the facial skeleton in naso-maxillo-acro- dysostosis.

J Cranio‑Max‑Fac Surg, 1988:16; 266‑272.

270

Orthognathic treatment (applicable for facial cleft deformities)

c. Mandibular OSTEOTOMIES Mandibular osteotomies in cleft deformities are usually indicated to correct the occlusion and often, in terms of anterior mandibular subapical osteotomies, to position the lower lip.

Anterior occlusal adjustments show positive aesthetic effects on the fullness of the lower lip, due to its in-rotation.

Furthermore, these segmental osteotomies can achieve rotational tilting or correction of a less severe apertognathia (anterior open bite).

271

Cleft - Ultimate Treatment

Unilateral CLAP deformity (i): normal midface, class III occlusion, prominent lower lip (â&#x20AC;&#x153;roll-out appearanceâ&#x20AC;?)

Osteotomy: Anterior mandibular subapical osteotomy to correct occlusion and position lower lip posteriorly 272

Orthognathic treatment (applicable for facial cleft deformities)

Mandibular subapical anterior osteotomy (ii) (correction of an anterior cross-bite) (immediate pre- and post-operatively)

For lower lip repositioning and occlusal correction. NEW LIP POSITION 273

Cleft - Ultimate Treatment

D. OSTEOTOMIES followed by osteodistraction Any cleft case (CLAP , hPsP and sP) may clinically present with a short soft palate and a borderline or complete velopharyngeal incompetence.

In such cases, conventional Le Fort I, II or III osteotomies with more than Âą5 millimetres movement might additionally compromise the speech in terms of velopharyngeal competence.

It should be considered to treat midfacial deformities urging this dimension of movement with distraction osteogenesis, a treatment possibility that allows gradual changes and adaptation of soft and hard tissue over time.

BĂ&#x153;TOW K-W, NAIDOO S. Surgical challenges during internal and external distraction in maxillary cleft deformities. Transactions of 4th Intern Congr Craniofac Osteobiol & Distrac 2014: 222-3,.

274

Orthognathic treatment (applicable for facial cleft deformities)

(1) Palatal distraction (transverse dimension)

Maxillary transverse expansion

275

Cleft - Ultimate Treatment

(2) Midfacial distraction with face mask Midfacial growth disturbance in young patients Distraction (face mask type) osteogenesis after LeFort I releasing-osteotomy (NO treatment of the mandible)

276

Orthognathic treatment (applicable for facial cleft deformities)

LeFort I releasing osteotomy, treated with external distractor (face mask system)

277

Cleft - Ultimate Treatment

(3) Midfacial distraction with internal distractor LeFort I osteotomy and insertion of an internal distractor Pre-operative, before insertion of distractor Post-operative, after distractor removal Unilateral cleft lip, alveolus and palate (CLAP)

278

Orthognathic treatment (applicable for facial cleft deformities)

Unilateral cleft lip, alveolar and palate (CLAP) LeFort I osteotomy; jaw advancement using internal distractors

279

Cleft - Ultimate Treatment

Bilateral CLAP case (i) LeFort I osteotomy; upper jaw advancement using internal distractors

280

Orthognathic treatment (applicable for facial cleft deformities)

Bilateral CLAP (ii)

An anterior movement and superior rotation of the upper jaw was achieved with osteodistraction after Le Fort I osteotomy (removal of a posterior bone strip) Intraoral one-dimensional distractors on both sides Pre- and post-operative radiographs and anterior-superior rotation (angulated arrow) of the upper jaw.

281

Cleft - Ultimate Treatment

Bilateral CLAP case LeFort I osteotomy + mandibular osteotomies; maxillary advancement using internal distractors Intraoral maxillary distraction completed + anterior subapical mandibular segmental osteotomy performed

282

Orthognathic treatment (applicable for facial cleft deformities)

(4) Midfacial distraction with external distractor device in two-dimensions Le Fort I osteotomy with subsequent osteo-distraction using an external device in a unilateral CLAP case (i)

The external haloframe pins of the distractor are embedded in the external cortex of the cranial vault 283

Cleft - Ultimate Treatment

Le Fort I osteotomy in a unilateral CLAP case (ii) Cranio-maxillary distraction - pre- and post-operative

284

Orthognathic treatment (applicable for facial cleft deformities)

Le Fort I osteotomy with subsequent osteo-distraction using an external device + bilateral sagittal split osteotomy to correct an anterior open bite in a unilateral CLAP patient 285

Cleft - Ultimate Treatment

Le Fort I osteotomy with Cranio-Maxillary Distraction in a bilateral CLAP case (Pre- and post-operative appearances)

286

Orthognathic treatment (applicable for facial cleft deformities)

(5) Midfacial distraction with external distractor in three-dimensions Le Fort I osteotomy + posterior ostectomy (strip of bone removed for contraction, a postero-superior rotation) in a unilateral cleft lip, alveolus and palate case

3-D-distraction â&#x20AC;&#x201C; screw adjustment to tilt upper jaw

3-D cranio-maxillary distraction (= 2 vector-distraction and rotation) 287

Cleft - Ultimate Treatment

(6) Midfacial distraction with external distractor + palatal distraction Soft palate cleft case with velopharyngeal incompetency and naso-maxillo-facial-acro-dysostosis (Binder syndrome) Pyramidal Le Fort II osteotomy + palatal osteotomy

288

Orthognathic treatment (applicable for facial cleft deformities)

(7) Total midfacial distraction with external distractor Unilateral CLAP case with sleep apnoea syndrome Le Fort III advancement

ZWAHLEN RA, BĂ&#x153;TOW K-W. Maxillary distraction resulting in facial advancement at LeFort III level in cleft lip and palate patients: A report on two patients. Oral Surg, Oral Med, Oral Path, Oral Rad 2004: 98: 541.

289

Cleft - Ultimate Treatment

Orthognathic repair for cleft deformities - The ULTIMATE Clinical pearls To achieve the ultimate result various aspects have to be considered. Ignoring one or more of these aspects will lead to suboptimal aesthetic and functional results.

1. About 20% to 30% of facial cleft deformity patients need orthognathic surgical intervention.

2. Accurate planning of orthognathic deformity in cleft lip and palate cases is essential. Besides the occlusal and skeletal aspects, soft tissue planning for the facial appearance is very important.

3. Sometimes optimal aesthetics as well as optimal functions in patients with facial cleft deformity cannot be achieved equally, even with perfect orthognathic surgery. Optimal aesthetics might sometimes lead to compromised function.

4. Conventional surgical approaches for osteotomies may be used in CL and CLA cases. In CLAP, hPsP and sP cases, velopharyngeal competence always needs to be assessed. In these cases with full velopharyngeal competence, but NOT for cases with a borderline competence, conventional orthognathic surgery is feasible. Maxillary anterior positioning in CLAP, hPsP and sP of more than 5 to 6 mm should be done by distraction osteogenesis.

290

Orthognathic treatment (applicable for facial cleft deformities)

Orthognathic repair for cleft deformities - The ULTIMATE Clinical pearls, continued 5. Distraction osteogenesis in children between 5 - 12 years of age may initially result in perfect facial harmony. However, the midface (in CLAP, hPsP, sP cases) and the mandible (in lateral facial cleft and FRT and SRS cases) quite often do not grow sufficiently until the age of 14 years. Therefore, in order to achieve the best possible aesthetic and functional result, a second distraction osteogenesis or conventional orthognathic surgery might be indicated in patients of 14 years of age or older.

6. Hypoplastic maxillae can be corrected by internal or external distraction. Internal distraction provides less accurate maxillary movement, eventually with more unexpected superior, inferior or lateral rotational directions. Such unexpected movements might occur either due to accidental plate bending during the distraction procedure or due to impingement between the distractor rod and mandibular coronoid process.

7. Cranio-maxillary or external distraction provides better control of the maxillary distraction vector. Further maxillary vertical tilting can be controlled simultaneously to anterior distraction using a 3-D cranio-maxillary distractor. This facilitates the treatment of maxillary anterior open bite (apertognathia). An ideal occlusion can be achieved by callus manipulation with orthodontic elastics after the removal of the distraction device.

291

Cleft - Ultimate Treatment

Orthognathic repair for cleft deformities - The ULTIMATE Clinical pearls, continued (2) 8. Osteotomies on Le Fort II (pyramidal) and III level are feasible. In uni- and bi-lateral CLAP and hPsP cases, however, they might jeopardize the midfacial blood supply. Therefore the indication of those types of osteotomies has to be considered carefully. Three months pre-operatively intra-oral soft tissue incisions at the site of future osteotomy lines can be performed to pre-condition perfusion patterns.

9. It is recommended to overextend the anterior position of the maxilla by 20% to compensate for eventual relapse.

Additional mandibulo-maxillary elastics should be worn at least for 9 â&#x20AC;&#x201C; 15 months after distractor removal. The paperthin distracted bone needs to be stabilised to undergo osteogenesis in order to keep the maxilla in its new position.

This stabilisation and final positioning of the jaws is described as callus manipulation when using strong mandibulomaxillary inter-occlusal elastics for final occlusal adjustment.

292

- 16 Cleft Nose Reconstruction (Rhinoplasty)

(A) UNILATERAL CLEFT NOSE (B) ADVANCED UNILATERAL RHINOPLASTY (C) BILATERAL CLEFT NOSE

293

Cleft - Ultimate Treatment

A. UNILATERAL CLEFT NOSE The unilateral cleft nose repair represents the most difficult rhinoplasty due to asymmetric cleft deformity.

To prevent growth centres from being damaged, maximum correction should be avoided during the primary lip-repair.

Yet, growth will enhance the asymmetry due to the skew nasal septum and the shorter alar wing of the nose on the cleft side.

Open rhinoplasty is mainly performed through a Carolina incision.

294

Cleft Nose Reconstruction (Rhinoplasty)

Unilateral cleft nose Intranasal (closed) rhinoplasty

295

Cleft - Ultimate Treatment

Unilateral cleft nose open rhinoplasty

296

Cleft Nose Reconstruction (Rhinoplasty)

Unilateral cleft nose open rhinoplasty (direct pre-operative + post-operative)

297

Cleft - Ultimate Treatment

Unilateral cleft nose. Open rhinoplasty with a Carolina incision

298

Cleft Nose Reconstruction (Rhinoplasty)

B. ADVANCED UNILATERAL CLEFT RHINOPLASTY To improve outcomes of difficult unilateral cleft rhinoplasty, additional resorbable material, plates or sheets, may be used. Inserted in the nose, they both support the nasal structure and the additionally augmented cartilage at the nasal dome, the lobular segment, the intermediate, lateral, and often the medial crura in the columella.

299

Cleft - Ultimate Treatment

The most difficult cases are unilateral CLAP with a median facial dysgenesis Unilateral lack of tissue and tissue deformity on the cleft side Often a 2nd rhinoplasty needs to be performed to achieve more balance between the one and other side of the nasal stucture

300

Cleft Nose Reconstruction (Rhinoplasty)

Unilateral cleft nose (i) Open rhinoplasty with a Carolina incision, bending and positioning of the PdLAlLA plate

301

Cleft - Ultimate Treatment

Unilateral cleft nose (ii) 1st part of reconstruction = septoplasty; 2nd part of reconstruction = with PdLAlLA plate in situ

302

Cleft Nose Reconstruction (Rhinoplasty)

Unilateral CLAP as a median facial dysgenesis (i) Reconstruction with PdLAlLA plate

303

Cleft - Ultimate Treatment

Unilateral CLAP with a median facial dysgenesis (ii) Positioning of PdLAlLA plate

304

Cleft Nose Reconstruction (Rhinoplasty)

Unilateral CLAP with a median facial dysgenesis (iii) Peri-operative views

305

Cleft - Ultimate Treatment

Unilateral CLAP as a median facial dysgenesis (iv) (12 months after rhinoplasty) THE ULTIMATE

306

Cleft Nose Reconstruction (Rhinoplasty)

C. BILATERAL CLEFT NOSE Bilateral secondary cleft nose repair is less challenging due to its symmetrical deformity.

Quite often additional cartilage has to be used in the columella as a strut and in the nasal dome to provide nasal tip support.

307

Cleft - Ultimate Treatment

Bilateral cleft nose (CLAP) â&#x20AC;&#x201C; Columella, dome + bridge repair

308

Cleft Nose Reconstruction (Rhinoplasty)

Bilateral cleft nose Columella, dome + nasal sill repair

309

Cleft - Ultimate Treatment

Repair of cleft nose deformities - The ULTIMATE Clinical pearls To achieve the ultimate result various aspects have to be considered. Ignoring one or more of these aspects will lead to suboptimal aesthetic and functional results.

1. Accurate aesthetic planning with drawings of various aspects of repair such as soft tissue, cartilage and bone of CL, CLA, and CLAP abnormalities is essential, to accurately execute surgery and predict post-operative outcome.

2. Often the optimal aesthetic effect cannot be achieved at one go, especially in unilateral cleft nose deformities. In secondary surgeries, quite often additional cartilage and/or resorbable plates/sheets have to be used to ultimately mould nasal structures in order to achieve the best result.

3. In unilateral cleft nose deformities the alar margin of the cleft side (â&#x20AC;&#x153;the hanging rimâ&#x20AC;?) often requires specific soft tissue repair, without involving the superior part of the lateral alar cartilage. This part of the alar usually shows a deformed facet and the cartilage should be preserved to avoid alar collapse.

4. An open-sky nasal approach is indicated in most cases. A slight columella lengthening which projects the nasal tip can be achieved using a Carolina incision. The standard transcolumellar v-shape incision is only indicated in a preoperative perfect superior nasal tip projection.

5. On the cleft side, quite often, an intra-nasal mucosal-cartilage band pulls the lower lateral cartilage at the nasal facet region inferiorly and this band has to be released with a z-plasty or an ellipse with cartilage removal.

310

Cleft Nose Reconstruction (Rhinoplasty)

Repair of cleft nose deformities - The ULTIMATE Clinical pearls, continued 6. All unilateral cleft deformities present with deviated or buckled cartilaginous nasal septa, which often involves the vomer-bone, too. A septoplasty, often combined with a turbinate plasty, is mostly indicated, due to the asymmetrical nasal blockage of the airway. This should be performed with the rhinoplasty during the same anaesthesia. However, the septoplasty should be completed before eventual indicated nasal osteotomies.

7. The septum cartilage is the preferred cartilage for transplantation to the superior lateral crura and/or dome and/or columella and/or nasal sill. For more extensive augmentation, rib grafts can be used for nasal bridge repair, with or without additional columella support.

8. The cleft nose repair in patients with bilateral cleft deformity is usually less difficult, as the nasal structure and its deformities are generally symmetrical. In bilateral cleft nose deformities, the columella length and the domeâ&#x20AC;&#x2122;s appearance, are the major areas of concern. Addressing these issues provides optimal aesthetic results regarding the nasal tip, as well as the supra-tip break, especially by narrowing the intermediate crura in the dome.

9. For long-term post-operative support of the transplanted cartilage, additional resorbable plates or sheets (e.g.

PdLAlLA) can be used. This eventually reduces secondary surgical interventions for the nasal framework (skin, cartilage and bone).

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- 17 Cleft Lip Revision

UNILATERAL AND BILATERAL CLEFT LIP REVISION

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TISSUE IMBALANCE AND SHORTAGE (i) Imbalance and shortage has to be considered in cases of revision

Lack of tissue: Non-cleft versus cleft-side

314

-

in horizontal and vertical dimension

-

in lip red thickness

Cleft Lip Revision

TISSUE IMBALANCE AND SHORTAGE (ii) Imbalance and shortage has to be considered in cases of revision

Very atrophic pro-labium â&#x20AC;&#x201C; different vertical length of lip

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Place scar into the philtrum edge, to mimic a more natural appearance of a philtrum edge and to create a dimple effect.

316

Cleft Lip Revision

Scar location mimics the philtrum edge

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Webster plasty To camouflage the straight line scar

318

Cleft Lip Revision

Cleft lip revision: preoperative view and postoperative result

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Total lip revision

Mimicking philtrum edges + dimple effect

320

Cleft Lip Revision

Sabattini-AbbĂŠ-flap (i)

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Sabattini-AbbĂŠ-flap (ii)

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Cleft Lip Revision

Secondary cleft lip deformities and their revision - The ULTIMATE Clinical pearls To achieve the ultimate result various aspects have to be considered. Ignoring one or more of these aspects will lead to suboptimal aesthetic and functional results.

1. As in secondary rhinoplasty, accurate aesthetic planning, drawings of lip deformity and the planned revision in CL, CLA, and CLAP are essential to predict post-operative outcome of skin and underlying muscles.

2. There are more than a hundred different possibilities and variations of surgical techniques to achieve optimal aesthetic results. As such, only a few examples could be shown in this chapter. To recognize and execute minor aesthetic deformities, surgeons often apply their understanding of art. Therefore, minor deviations from preparation and clinical planning may still be applied with the patient under general anaesthesia, before surgery starts.

3. The most important aspects for consideration: (a) scar presentation and eventual improvement; (b) does the scar mimic the philtrum edge and can the scar be repositioned to that location; (c) can the philtrum edge in unilateral cleft lip cases be raised to simulate the opposite natural one; (d) can an aesthetic philtrum dimple be created between the â&#x20AC;&#x153;philtrum edgesâ&#x20AC;?; (e) can the nasal sill and lip-nasal floor in unilateral cleft cases be raised equally and supported especially if compared to the opposite side? The latter may be adjusted by adding cartilage or by superiorly rotating scar tissue mobilised during muscle repair.

4. Achieve tension-free wound closure using non-soluble suture material that is as thin as possible. In muscles and the red-lip area, the use of resorbable suture material should be limited as it might create a tissue reaction which enhances hyperplastic scar formation.

323

- 18 Dental Implant In Cleft Alveolar Ridge

Advantages and disadvantages of dental implants in cleft cases

325

Cleft - Ultimate Treatment

Implant in non-cleft area

326

Implant in cleft area

Dental Implant In Cleft Alveolar Ridge

IMPLANTS After oro-nasal fistula repair with a tongue flap and secondary osteoplasty to create an anterior dento-alveolar ridge in a patient with a history of a total pre-maxillary resection in infancy (i)

327

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IMPLANTS After oro-nasal fistula repair with secondary osteoplasty and tongue flap (ii)

328

Inserted tongue flap Non-denuded tongue flap

Dental Implant In Cleft Alveolar Ridge

IMPLANTS (17 years post-operatively) After oro-nasal fistula repair, tongue flap and secondary osteoplasty (iii)

BĂ&#x153;TOW K-W, BRIEDENHANN SJ, KOEPP WG. Comprehensive secondary cleft deformity reconstruction: Case report. Hands-On, 1996: 8(1): 61-65

329

Cleft - Ultimate Treatment

Dental implants: In an â&#x20AC;&#x153;inheritedâ&#x20AC;? patient (i)

Before anterior pre-maxillary osteotomy, tertiary osteoplasty + placement of dental implants

330

Dental Implant In Cleft Alveolar Ridge

Dental implants: Same â&#x20AC;&#x153;inheritedâ&#x20AC;? patient (ii)

331

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Dental implants: Same “inherited” patient (iii) 10 years after implant insertion

Kind of “peri-implantitis” starting from the nasal floor. Loss of grafted bone in the former dento-alveolar cleft.

332

Dental Implant In Cleft Alveolar Ridge

Dental implants in cleft patients - The ULTIMATE Clinical pearls To achieve the ultimate result various aspects have to be considered. Ignoring one or more of these aspects will lead to suboptimal aesthetic and functional results.

1. Usually good results can be achieved in CLA cases, as the palatal bone is physiologically formed.

2. In CLAP cases both excellent long-term results and early dental implant loss may occur. The latter can be attributed to palatal mucosa encroachment into the original bone graft within the former dento-alveolar cleft. In most cases the buccal cortical bone surface and its radiographic height indicate successful osteofusion, even though the palatal cancellous side of this composite bone graft may underlie resorption. This resorption can be prevented by additional augmentation with cortical bone directed towards the palate after removal of palatal soft tissue.

3. A rather non-understood and unpublished type of â&#x20AC;&#x153;peri-implantitisâ&#x20AC;? has been observed. This type of chronic bone breakdown starts in the nasal floor and has been diagnosed in a previously bone grafted alveolar cleft site after dental implantation.

4. The success rate of dental implantation in bone grafted cleft dento-alveolar ridges are rather unpredictable compared to those in non-cleft dento-alveolar ridges. Patients and treating prosthodontists need to be aware of this variation in outcomes.

5. A well-structured and aesthetically acceptable tooth borne bridge may often be an excellent alternative to more complex implant borne prosthetic solutions.

333

EXCEPTIONAL CLEFTS PRIMARY and SECONDARY TREATMENT

335

- 19 Pierre Robin Sequence Treatment A. Siebold-Robin Sequence B. Fairbairn-Robin Triad Important publications on Pierre Robin sequence: 1. Robin, Pierre - 1923 and 1934 First publications: 2. Von Siebold, E - 1835 3. Fairbairn, P - 1846

VON SIEBOLD E. Siebold‘s Journal Göttingen 1835: Vol XV; 18. Quoted in - Grimm G, Pfefferkorn A, Taatz H. Die kliniese Bedeutung des Pierre-Robin-Syndroms und seine Behandlung.

Dtsch Zahn-Mund-Kieferheilk 1964: 43; 385-416.

FAIRBAIRN P. Suffocation in an infant from retraction of the base of the tongue. Monthly J Med Sc 1846: 6; 280-281.

ROBIN P. La glossoptose. Son diagnostic, ses consequences, son traitement. Bull de L’Academie Nat Med (J Med Paris) 1923 : 89; 37-41.

ROBIN P. Glossoptosis due to atresia and hypotrophy of the mandible. Am J Dis Child 1934: 48; 541-547.

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Cleft - Ultimate Treatment

Pierre Robin sequence Features: 1. Micrognathia 2. Microglossia 3. Glossoptosis 4. Without or with cleft palate Features:

Features:

1+2+3

1+2+3+4

Without cleft palate

With cleft palate

= subdivision

= subdivision

Siebold-Robin sequence (SRS)

Fairbairn-Robin triad (FRT)

HOOGENDIJK CF, BĂ&#x153;TOW K-W. Fairbairn-Robin appearance: a controversial syndrome, 171 cases. J Cranio-Maxillofac Surg 2008: 36 (suppl); 15(O.059).

338

Pierre Robin Sequence Treatment

SRS and FRT No visible differences in lateral profiles

339

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A. SIEBOLD-ROBIN SEQUENCE Treatment protocol for the Pierre Robin sequenceâ&#x20AC;&#x2122;s subdivision â&#x20AC;&#x201C; Siebold-Robin sequence Two main problems (SRS) 1. Airway problem 2. Feeding problem Is anaesthetic intubation feasible to perform tracheostomy or distraction?

After birth (in sequence) Breathing problem + treatment 1. Intubation and ventilation (if necessary) 2. Non-cleft suction plate with distal extension (to maintain the tongue - pharyngeal space) 3. Glossopexia 4. Long-term nasopharyngeal intubation 5. Tracheotomy 6. Mandibular distraction (afterwards - closure of tracheostomy) Feeding problem + treatment 1. Nasogastric tube 2. Gastrostomy

340

Pierre Robin Sequence Treatment

SIEBOLD-ROBIN SEQUENCE

NO cleft palate ! Breathing problem

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SIEBOLD-ROBIN SEQUENCE

Persistent apnoea Placement of 2 internal paediatric distractors to distract the mandible

342

Pierre Robin Sequence Treatment

SIEBOLD-ROBIN SEQUENCE (severe)/Bird face (i) Breathing problems, Trismus, Distractor treatment to elongate mandible

Before osteodistraction, bilateral rib transplantation was performed

to increase the mandibular ramus volume

343

Cleft - Ultimate Treatment

Bi-dimensional mandibular distraction (in a SRS/Bird face case)

Breathing problems: Bi-dimensional distraction

344

Pierre Robin Sequence Treatment

B. FAIRBAIRN-ROBIN TRIAD Treatment protocol for the Pierre Robin sequence’s subdivision – Fairbairn-Robin Triad Three main problems (FRT) - Airway problem - Feeding problem - Oro-/nasopharyngeal intubation

345

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FAIRBAIRN-ROBIN TRIAD

Wide cleft !

Airway + feeding problem

Folding of tongue into a more narrow cleft

BÜTOW K-W, HOOGENDIJK CF, ZWAHLEN RA. Pierre Robin sequence: appearances and 25 years of experience with an innovative treatment protocol.

J Ped Surg 2009: 44; 2112–1118.

346

Pierre Robin Sequence Treatment

FAIRBAIRN-ROBIN TRIAD (hPsP appearances)

347

Cleft - Ultimate Treatment

Protocol (FRT) Protocol after birth (in sequence) Airway problem + treatment 1. Intubation and ventilation, if necessary, directly or after ± 3weeks due to exhaustion 2. Cleft suction and feeding plate with dorsal extension to separate the tongue and the cleft and maintain the glosso-pharyngeal space (see pp. 47 + 48) 3. Glossopexia 4. Long-term nasopharyngeal intubation 5. Tracheotomy 6. Mandibular distraction (afterwards - closure of tracheotomy) Feeding problem + treatment 1. Nasogastric tube 2. Suction and drinking plate 3. Gastrostomy Du Plessis SM, Van den Berg HJS, Bütow KW, Hoogendijk CF. Airway and feeding problems in infants with Fairbairn-Robin triad deformities.

Curationis 2013: 36 – doi:10.4102/curationis.v36iI.84.

348

Pierre Robin Sequence Treatment

FAIRBAIRN-ROBIN TRIAD Airway problem = Glossopexia

349

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FAIRBAIRN-ROBIN TRIAD sP cleft only

(10.2% in FRT) with severe mandibular micrognathia

Persistent airway problem

Mandibular distraction (afterwards - closure of tracheotomy)

350

Pierre Robin Sequence Treatment

FAIRBAIRN-ROBIN TRIAD Persistent airway problem (arrow = occlusion) Tracheostomy (2.4% in FRT) and mandibular telescope distraction (afterwards - closure of tracheotomy)

351

Cleft - Ultimate Treatment

FAIRBAIRN-ROBIN TRIAD Feeding problem Initially: feeding tube

4 years later 352

Pierre Robin Sequence Treatment

FAIRBAIRN-ROBIN TRIAD Suction and drinking plate with dorsal extension

ZSCHIESCHE S. Profilveränderung bei Patienten mit Pierre-Robin-Syndrom. Fortschr Kieferorthop 1985: 46; 72.

353

Cleft - Ultimate Treatment

FAIRBAIRN-ROBIN TRIAD Persistent feeding problem: Gastrostomy

354

Pierre Robin Sequence Treatment

FAIRBAIRN-ROBIN TRIAD

355

Cleft - Ultimate Treatment

Protocol (FRT) Protocol for Anaesthesia 1. Paediatric straight blade laryngoscope 2. Special point-bend paediatric laryngoscope 3. External paediatric viewer for intubation 4. Endoscopic device with intubation tube aligned for vocal cord visualisation 5. Not possible to intubate, wait for 6 - 9 months and retry

Protocol for Surgical intervention 1. sP cleft repair at 7 months (using: IVP-type III â&#x20AC;&#x201C; probably the best chance to close a very wide sP cleft) - Wait 11 months, leave the hP cleft open. It will function as an additional airway passage.

- The hP cleft growth in this period contributes to a more narrow cleft; hP cleft easier to be repaired.

2. hP cleft repair at 18 months (usually using a von Langenbeck plasty)

356

Pierre Robin Sequence Treatment

11 months after sP repair before hP repair

357

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20 months, sP repair

358

+ hP repair

Pierre Robin Sequence Treatment

FAIRBAIRN-ROBIN TRIAD Post-natal development theories related to this sequence (ii) Is “Catch-up” growth a valid theory, occurring post-natally in FRT babies, whose mothers had been diagnosed with oligo- or poly-hydramnios?

MARKOVIC M. Growth changes in cases of mandibular micrognathia. Trans Eur Orthod Soc 1972: 48; 133-145.

FIGUEROA A, GLUPKER T, FITZ M, BeGOLE E. Mandible, tongue, and airway in Pierre Robin sequence: A longitudinal cephalometric study. Cleft Palate-Craniofac J 1991: 28; 425-434.

359

Cleft - Ultimate Treatment

FAIRBAIRN-ROBIN TRIAD

NO CATCH-UP GROWTH 360

CATCH-UP GROWTH

Pierre Robin Sequence Treatment

FAIRBAIRN-ROBIN TRIAD hP and sP repair and velopharyngeal flap / catch-up growth

361

Cleft - Ultimate Treatment

FAIRBAIRN-ROBIN TRIAD 23 years post-repair of hP and sP; No other surgery performed; Catch-up growth

362

Pierre Robin Sequence Treatment

FAIRBAIRN-ROBIN TRIAD

22 years after sP and hP cleft repair No other surgery performed No catch-up growth !

Daughter, born with a narrow hP and sP cleft Not a FRT

363

Cleft - Ultimate Treatment

Pierre Robin sequence - The ULTIMATE Clinical pearls To achieve the best clinical outcome various aspects have to be followed. Ignoring one or more of these aspects may lead to suboptimal aesthetic and functional results.

1. The Pierre Robin sequence (PRS) is subdivided into TWO different categories: 1) the Siebold-Robin sequence (SRS) without a cleft palate, and 2) the Fairbairn-Robin triad (FRT) with a cleft palate. Both subcategories have a wider spectrum of diversity than assumed.

2. With 266 patients (FRT + SRS) this PRS-database appears to be the largest documented in the world.

3. Syndrome: Whereas SRS (7.9% - 21 of 266, FRT 92.1% - 245 of 266) may be associated with one syndrome in 9.5% (2 of 21), in FRT 20.8% (51 of 245) were associated with 18 other syndromes (mostly Stickler â&#x20AC;&#x201C; 9.0% and Demarquay-van der Woude â&#x20AC;&#x201C; 2.4%) .

4. Catch-up-growth: Verifiable only in 34.7% (51 of 147 reviewed cases) of FRT cases.

5. Midfacial growth: Whereas normal midfacial growth occurred in 73.2%, midfacial hypoplasia developed in 3.8% (6 of 157 reviewed cases) of FRT cases.

6. Oligohydramnios: 65.3% of mothers with FRT children could reveal a pregnancy history. In 20.0% (32 of 160 reviewed case) of FRT patients it was verified.

7. In facial cleft deformities, FRT revealed an incidence rate of 5.9% (245 of an overall of 4158 cleft cases). The reason for the development of micrognathia in utero, a previous cornerstone of the oligohydramnios and mandibular catch-up growth theory, could not be verified.

364

Pierre Robin Sequence Treatment

Pierre Robin sequence - The ULTIMATE Clinical pearls, continued 8. Management of PRS is multifaceted and determined by: (i) compromised airway; (ii) feeding difficulties; (iii) sequence of palatal reconstruction (FRT) (sP: 7 months; hP: 18 months), and (iv) orthognathic deformities.

9. Distraction osteogenesis for mandibular micro- or severe retrognathia can be performed, if needed, at 1 year of age using an internal paediatric distractor device, FRT 0.8% (2 of 245) and SRS 33.3% (7 of 21). It is a difficult surgical procedure. External distractor devices can be used earlier, causing subsequent permanent facial cutaneous scarring.

Tracheostomy tubes can only be removed after completion of the distraction osteogenesis.

10. Orthognathic surgery for hypoplastic/retrognathic mandibles and/or chins (retro- or retro-brevigenion) is performed at 14+ years of age.

11. In PRS the indication for tracheostomy is low (3.4%; 9 of 266) (FRT 2.4%; 6 of 245), if appropriate measures like (i) a suction and feeding plate with extension into the soft palate cleft, or alternatively (ii) glossopexia, or (iii) longterm nasopharyngeal intubation are implemented. Tracheostomy is often indicated and/or required in cases where a comorbidity like a laryngomalacia or tracheomalacia exists.

12. Mortality is relatively low: 3.4%. Death may occur one or two days after birth due to the inability of endotracheal intubation. However, most casualties happen after hospital discharge due to fluid and food aspiration or due a syndromic PRS. Therefore long-term hospitalisation has to be considered in SRS and FRT cases.

365

- 20 Median Cleft Reconstruction With Agenesis Of Premaxilla-Prolabium Columella

BÜTOW K-W. The reconstruction of the congenital agenetic columella in midline clefts. J. Cranio-Maxillofac Surg 2007: 35; 287 – 292.

HTUN SY, BÜTOW K-W. Epidemiology of holoprosencephaly and single-stage lip and columella reconstruction. 95th Ann Meeting Am Ass Oral Maxillofac Surg 2013: P 12, e-46 – e-47.

367

Cleft - Ultimate Treatment

MEDIAN CLEFT, PREMAXILLA-PROLABIUM-COLUMELLA AGENESIS (variations)

368

Median Cleft Reconstruction With Agenesis Of Premaxilla-Prolabium Columella

MEDIAN CLEFT, PREMAXILLA-PROLABIUM-COLUMELLA AGENESIS

369

Cleft - Ultimate Treatment

SURGICAL PROCEDURES One step repair (= one general anaesthetic procedure only): Lip and columella are repaired in one go Two steps repair (= two general anaesthetic procedures): Lip reconstruction (1st procedure) and Cronin columella reconstruction (2nd procedure)

Columella repair - Intranasal skin flap is derived from the intermediate crus of the lower lateral nasal cartilage - Closure of the median cleft lip by means of a straight line closure, red-lipskin triangle and upper lip triangle Connection in the form of a triangular wedge between the cleft lip and created columella (Incisional area

370

)

Median Cleft Reconstruction With Agenesis Of Premaxilla-Prolabium Columella

Type of incision

371

Cleft - Ultimate Treatment

Wide cleft with agenesis of the prolabium and the columella

372

Median Cleft Reconstruction With Agenesis Of Premaxilla-Prolabium Columella

Midfacial growth in the short-term and long-term (1 year and 4 years)

Short-term

Long-term (4 year)

Long-term (1 year) 373

Cleft - Ultimate Treatment

Median clefts with premaxillaprolabium-columella agenesis - The ULTIMATE Clinical pearls To achieve the best clinical outcome various aspects have to be followed. Ignoring one or more of these aspects may lead to suboptimal aesthetic and functional results.

1. Median facial clefts are rare with a prevalence of 2.3% among all clefts. In 65.3% they are associated with a holoprosencephaly and in 9.5% with another syndrome.

2. Patients born with a holoprosencephaly, with median cleft and premaxilla-prolabium-columella agenesis, are operated under the following criteria: (i) being in good health for more than one year and if suffering from epileptic seizures, being well-controlled; (ii) if parents are well-informed informed about the long-term prognosis, but still desire surgery, and (iii) if the baby is to be baptized in public or needs to have a normal facial appearance for similar occasions.

3. One anaesthetic procedure only is of major benefit to patients with a holoprosencephaly as they are prone to develop complications during and/or after the anaesthesia.

4. A modified Cronin columella repair is suitable for patients who previously had cleft lip surgery only.

5. To reconstruct the columella, an intra-nasal skin flap, without strut, is used, and is rotated and connected during the simultaneous median lip closure.

374

- 21 Lateral Or Transverse Cleft A. DIAGNOSIS AND CLASSIFICATION B. SURGICAL Repair 1. A lateral facial cleft is not a unitary cleft type 2. Appearance and classification

TESSIER P. Anatomical classification of facial, craniofacial and latero-facial clefts. J Maxillofac Surg 1976: 4: 69.

EPPLEY BL, VAN AALST JA, ROBEY A, HAVLIK RJ, SADOVE AM. The spectrum of orofacial clefting. Plast Reconstr Surg 2005: 115: 101e.

BÃ&#x153;TOW K-W, BOTHA A. A classification and construction of congenital lateral facial clefts. J. Cranio-Maxillofac Surg 2010: 38; 477-484.

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A. DIAGNOSIS AND CLASSIFICATION Descriptions for the same type of cleft

- Transverse facial cleft

- Lateral cleft lip

- Tessier 7 cleft

- Macrostomia

VERHEYDEN CN. Anatomical considerations in the repair of macrostomia. Ann Plast Surg 1988: 20: 374.

376

Lateral Or Transverse Cleft

377

Cleft - Ultimate Treatment

Lateral cleft lip - right-side

378

Lateral Or Transverse Cleft

Lateral cleft lip - bilateral

379

Cleft - Ultimate Treatment

Tessier 7 cleft with 4 subdivisions

Muscles involved in the 4 subdivisions (Tessier No. 7 cleft) - separating clefts or partially aplastic (subdivided in T7.1 â&#x20AC;&#x201C; T7.4)

380

Lateral Or Transverse Cleft

Four different transverse clefts: T7.1 â&#x20AC;&#x201C; T7.4 (T = Tessier)

381

Cleft - Ultimate Treatment

B. Surgical repair Lateral facial cleft or transverse cleft repair

Repair for each type of the four subdivisions

Short cleft - Âą 1cm Single Z-plasty

Long cleft Double or triple Z-plasty

NB - Always try to create a smile by rotating the repair superiorly

382

Lateral Or Transverse Cleft

Goldenhar with a lateral facial cleft (T7.1); distraction osteogenesis apparatus (see arrow) before closure of the lateral facial cleft (i)

383

Cleft - Ultimate Treatment

Goldenhar with a lateral facial cleft (T7.1); distraction osteogenesis (see arrow) before closure of the lateral facial cleft (ii)

384

Lateral Or Transverse Cleft

Single Z-plasty - short cleft

385

Cleft - Ultimate Treatment

Single Z-plasty - short cleft Active modiolus

386

Lateral Or Transverse Cleft

Double Z-plasty - long cleft (i) Surgical repair - Mucosal straight line closure - Rotational commissure closure

387

Cleft - Ultimate Treatment

Double Z-plasty - long cleft (ii) Surgical repair - Mucosal straight line closure - Rotational commissure closure Modiolus muscle connection Cutaneous double Z-plasty

388

Lateral Or Transverse Cleft

Double Z - long T 7.2 cleft

389

Cleft - Ultimate Treatment

Double Z - long T7.2 cleft bilateral

390

Lateral Or Transverse Cleft

Double Z-plasty - long T7.3 cleft (‘fish mouth appearance’)

391

Cleft - Ultimate Treatment

Long T7.3 cleft (long-term result)

392

Lateral Or Transverse Cleft

Lateral facial cleft - The ULTIMATE Clinical pearls To achieve the ultimate result various aspects have to be considered. Ignoring one or more of these aspects will lead to suboptimal aesthetic and functional results.

1. Lateral or transverse facial clefts are rare. Their prevalence is 0.8% among all clefts. In 55.9% they might be associated with syndromes, such as Goldenhar, oto-mandibular dysostosis or Treacher-Collins syndrome.

2. Lateral facial clefts may present unilaterally on the left or right side or even bilaterally. Four different types can be identified: i) superior lateral (T7.1), ii) middle lateral (T7.2), iii) inferior lateral, also known as ‘fish mouth type’ (T7.3), and iv) aplastic type (T7.4) with partial agenesis of skin, mucosa and muscles.

3. Macrostomia represents a lateral facial variation in that the total cleft area presents with normal dry and wet lip red mucosa lining and manifests in any of the four above-mentioned variations (T7.1 – T7.2). A lateral cleft without normal lip red mucosa is not considered to be a macrostomia.

4. Repair involves: 1) straight line mucosal closure; 2) rotational commissural closure, preventing both future scar formation in the new commissure and cheilitis; 3) modiolus muscle repair and 4) straight line cutaneous closure in T7.1, or otherwise a rotated cutaneous Z-plasty. Straight line closure in T7.2 – T7.4 types may lead to fish mouth appearance.

393

- 22 P63 Gene Mutation – Three Syndromes, Complications And Surgery A. Three SYNDROMES LINKED TO GENE p63 1. Ankyloblepharon ectodermal dysplasia clefting syndrome (AEC)

2. Rapp Hodgkin syndrome (RHS)

3. Ectrodactyly ectodermal dysplasia clefting syndrome (EEC)

B. PERI-OPERATIVE COMPLICATIONS

VAN STRATEN C, BÜTOW K-W. p63 gene: In ectrodactyly-ectodermal dysplasia cleft, ankyloblepharon-ectodermal dysplasia, Rapp-Hodgkin syndrome.

Ann Maxillofac Surg (2013) 3: 58-61.

395

Cleft - Ultimate Treatment

A. THREE SYNDROMES LINKED TO GENE p63 Physiology - - -

p63-gene Transcription factor Key regulator

Mutations - p63 - -

- -

396

Variable clinical expressions Etiology of disruptions in embryonic development is not clear yet Inherited from parents New mutations in 50%

P63 Gene Mutation â&#x20AC;&#x201C; Three Syndromes, Complications And Surgery

Syndromes associated with p63

397

Cleft - Ultimate Treatment

Ankyloblepharon-Ectodermal dysplasia clefting syndrome (AEC) - Hay-Wells syndrome (1st described by Hay and Wells 1976) - Ankyloblepharon, classical feature (Small band of vascularized connective tissue spanning the eyelids

)

HAY RJ, WELLS RS. The syndrome of ankyloblepharon, ectodermal defects and cleft lip and palate: an autosomal dominant condition. Br J Dermatol 1976; 94: 277â&#x20AC;&#x201C;89.

398

P63 Gene Mutation â&#x20AC;&#x201C; Three Syndromes, Complications And Surgery

AEC

-

- -

Sparse hair, + high frontal hairline, sparse eyebrows and eyelashes Photophobia Narrow pinched nose

- - -

Low set ears Hypoplastic maxilla Dystrophic finger nails + toe nails

399

Cleft - Ultimate Treatment

Rapp-Hodgkin Syndrome (RHS)

- - - - - -

Photophobia Dry and red-blond hair Nasal orifice stenosis Sparse hair Thin mucosa Perioral ulcer

- -

Hypoplastic mandible Dystrophic nails

RAPP RS, HODGKIN WE. Anhidrotic ectodermal dysplasia: autosomal dominant inheritance with palate and lip anomalies. J Med Genet 1968; 5: 269â&#x20AC;&#x201C;272.

400

P63 Gene Mutation â&#x20AC;&#x201C; Three Syndromes, Complications And Surgery

RHS

401

Cleft - Ultimate Treatment

Ectrodactyly-ectodermal dysplasia clefting syndrome (EEC) - - - - - -

Alveolar ridge abnormalities (cleft mandible) Thin skin Syndactyly Nail abnormalities Blue sclera Cupped ears

RUDIGER RA, HAASE W, PASSARGE E. Association of ectrodactyly, ectodermal dysplasia, and cleft lip-palate. Am J Dis Child 1970; 120:160â&#x20AC;&#x201C;163.

402

P63 Gene Mutation â&#x20AC;&#x201C; Three Syndromes, Complications And Surgery

EEC

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Cleft - Ultimate Treatment

b. PERI-OPERATIVE COMPLICATIONS AEC â&#x20AC;&#x201C; RHS - EEC Peri-operative complications due to ectodermal component -

Oro-nasal fistula development

-

Nasal stenosis

-

Prolabium-premaxilla fusion

-

Midfacial growth disturbance

404

P63 Gene Mutation â&#x20AC;&#x201C; Three Syndromes, Complications And Surgery

Cleft associated with p63-gene - The ULTIMATE Clinical pearls To achieve the ultimate result various aspects have to be considered. Ignoring one or more of these aspects will lead to suboptimal aesthetic and functional results.

1. Clefts associated with the p63-gene are part of rare syndromes with a 0.3% prevalence. The three most common syndromes diagnosed with the p63-gene show the following distribution ratio: AEC 23.1% : RHS 7.7% : EEC 69.2%.

2. The ectodermal component associated with the three syndromes, seems to have a negative effect on the postoperative wound healing of nasal, vestibular and palatal mucosa, as well as on the development of skeletal midfacial region.

3. As partial hypo- and adontia often do occur in these syndromes, best possible dental rehabilitation becomes a major challenge for the orthodontist and prosthodontist.

4. In patients with a p63-gene abnormality, the treatment and follow-up should take place in a multidisciplinary setting to ensure the most favourable treatment outcome. Nevertheless, both primary cleft and future secondary surgical repairs of upcoming defects in these patients remain challenges for surgeons and allied specialties.

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- 23 Oblique Facial Cleft Repair The oblique facial clefts are classified into 14 different types and may present combined or not

TESSIER P. Anatomical classification of facial, craniofacial and latero-facial clefts. J Maxillofac Surg 1976: 4: 69.

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Oblique cleft variations

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Oblique Facial Cleft Repair

Oblique facial cleft (T2 nose)

BĂ&#x153;TOW K-W, ENGELBRECHT H. Tessier 2 nasal cleft: design and reconstruction with a resorbable plate. Br J Oral Maxillofac Surg 2014: 52; 93-94.

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Oblique facial cleft (T3) repair in an adolescent

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Oblique Facial Cleft Repair

Oblique facial cleft (T4) (i)

Individual design

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Oblique facial cleft (T4) (ii)

Approximation of the canthal ligament

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Oblique Facial Cleft Repair

Oblique facial cleft (T5); addressing the median canthal ligament

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Oblique facial cleft - bilateral (T4); Individual design (i)

BÜTOW K‑W, DE WITT TW. Bilateral oblique facial cleft ‑ Tissue expansion with primary reconstruction. J Dent Ass S Afr 1990: 45; 507 ‑ 511.

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Oblique Facial Cleft Repair

Oblique facial cleft - bilateral (T4); Individual design (ii)

Oblique facial cleft reconstruction, with pyramidal LeFort II osteotomy

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Central facial duplication (Tessier 1+2 duplication): remodelling maxilla and mandible and reconstructing the cleft in an infant (i)

Lip adhesion procedure initially 416

Oblique Facial Cleft Repair

Central facial duplication (Tessier 1+2 duplication): remodelling maxilla and mandible and reconstructing the cleft in an infant (ii)

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Oblique facial cleft - The ULTIMATE Clinical pearls To achieve the best possible clinical outcomes in oblique facial clefts various aspects have to be considered. Ignoring one or more of these aspects may lead to suboptimal aesthetic and functional results.

1. Oblique facial clefts are rare with a prevalence of 1.4%. The median and lateral facial clefts are excluded from these statistics. They are classified under standard clefts although they are Tessier 0 and Tessier 7 clefts.

2. Each oblique facial cleft has a different appearance with a different tissue deficiency. As such, their reconstruction always entails different designs to achieve optimal surgical outcomes.

3. Depending what tissue is most lacking, structures like eyes, eyelids, tear ducts, nasal cutaneous tissue, nasal bones, zygoma and maxilla, need to be considered during surgical planning, to achieve the best possible outcome. Quite often tissue expanders and lip adhesion are needed before major facial reconstruction can take place.

4. The reconstruction of the medial canthal ligament position with wires attached to bilateral miniplates and/or screws, represents one of the most essential parts in eyelid positioning and the surrounding soft tissue(s).

5. The ultimate result depends very much on the type of lacking tissue, the surgical design and the repositioning of the available soft tissue. It is impossible to make a long-term prediction for the sagging and limited growth of the repositioned soft tissue.

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TERTIARY TREATMENT “THE CLEFT SYNDROME”

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- 24 Severe Cleft Disfigurement Or “Cleft Syndrome” These are compromised patients, mostly operated at a different centre(s), also known as “inherited patients”. These patients were likely part of a charity surgical programme or born with a medial facial dysgenesis or born with another severe facial deformity.

A. PROTOCOL for cleft syndrome B. MULTIPLE INTERVENTIONS

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A. PROTOCOL FOR A “CLEFT SYNDROME” There is no established treatment protocol for patients with severe facial cleft disfigurement.

However, principles in treatment sequence should be applied.

The main points when performing corrective surgery are:  

Skeletal deformity - needs to be corrected before addressing the soft tissue; this includes cleft nasal deformities 



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Speech impairment – always to be addressed as of paramount importance

Dental deformity (especially the position of the anterior teeth) - should be corrected before the final cleft lip correction

Aesthetic soft tissue deformity(ies) - cleft lip, cleft nose or other aspects of the facial soft tissue region

Severe Cleft Disfigurement Or “Cleft Syndrome”

“CLEFT SYNDROME” Main problem Presenting with severe midfacial growth disturbances (dysgnathia), sometimes born with a median facial dysgenesis

Plus Non-functional occlusion and often aesthetically unacceptable dental/occlusal appearances

Other associated problems 

“Below standard” soft tissue repair or ugly scar or insufficient tissue due to wrong design  Severe nasal deformity  Speech impairment

Usually the protocol “from inside to outside” or from bone towards soft tissue reconstruction must be followed Orthodontic treatment, secondary osteoplasty, orthognathic surgery, soft tissue , nose and lip correction, oro-nasal fistula closure, velopharyngeal plasty, dental implants, prosthodontic rehabilitation

BÜTOW K-W. Treatment of cleft lip and palate. Part IV: Cleft lip and palate cases presenting at the clinic at a late age. J Dent Ass S Afr, 1984: 39; 473.

BÜTOW K-W. A single stage surgical treatment combining a Le Fort I osteotomy, an oro‑nasal fistula closure and a secondary osteoplasty. 5th Intern. Congress on Cleft Palate and related Cranio‑facial Anomalies. 1985: 81; p. 77, Monte Carlo, Monaco.

BÜTOW K-W. A three‑in‑one surgical approach for unilateral cleft lip and palate. Brit J Oral and Maxillofac Surg, 1986: 24; 190‑197.

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â&#x20AC;&#x153;Cleft syndromeâ&#x20AC;? appearance - examples of patients with unilateral and bilateral clefts

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Severe Cleft Disfigurement Or “Cleft Syndrome”

“Cleft syndrome” appearance – an example of a bilateral cleft with severe deformities of lip, premaxilla, occlusion, hypoplastic midfacial growth and presenting with an oro-nasal fistula

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B. MULTIPLE INTERVENTIONS The evaluation consists of

1. An â&#x20AC;&#x153;outside to insideâ&#x20AC;? approach, step by step.

2. Compilation of a detailed diagnosis.

3. Definition of treatment sequence and combinations.

4 Information of the patient who shall decide if this is what she/he wants or if she/he prefers an alternative treatment, as those patients are mostly adolescent or adult

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Severe Cleft Disfigurement Or “Cleft Syndrome”

Compromised patient (5 years) –with a fractured protruding premaxilla-vomer junction (i) Reconstruction in infancy – NO premaxilla repositioning before primary bilateral lip repair; columella lengthening “below standard”

ZWAHLEN RA, JAYARATNE YSN, HTUN SY, BÜTOW K‑W. Fracture of the vomero-premaxillary junction in a repaired bilateral cleft lip and palate patient. Craniomaxillofacial Trauma Reconstr 2014: 10-1055-s-0034-1378180_130283cr.xml

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Compromised patient (5 years) â&#x20AC;&#x201C;with a fractured protruding premaxilla-vomer junction (ii) Immediately after trauma surgery involving the premaxilla-vomer junction. The osteotomised premaxilla was fused to the lateral dentoalveolar ridges by transmucosal/-periosteal sutures (Second picture). Third picture shows patient one year after trauma and a columella lengthening

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Severe Cleft Disfigurement Or “Cleft Syndrome”

Compromised patient (bilat CLAP - 30 years of age) – presenting with an abnormal lip-prolabium and blocked nasal patency Surgery: 1. Septoplasty and remodelling of the inferior turbinate; 2. Sabattini-Abbé-flap reconstruction with dimple effect

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Midfacial growth disturbance occurring between 4 and 13 years of age (most probably due to total stripping of the hard palate mucoperiosteum during primary repair â&#x20AC;&#x201C; an Ernst-Dorrance procedure?)

Unilateral CLAP: 4 years = no growth disturbance; 13 years = severe growth disturbance; 15 years = facial correction (Treatment: LeFort I and anterior subapical mandibular osteotomies)

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Severe Cleft Disfigurement Or “Cleft Syndrome”

Bilateral CLAP Severe midfacial growth deformity and mandibular hyperplasia (occlusion – one molar contact only)

Therapy – LeFort I and bilateral sagittal split osteotomy

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Bilateral â&#x20AC;&#x201C; CLAP (i)

Midfacial osteotomy with external craniomaxillary distraction before, followed by cleft nasal and cleft lip

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Severe Cleft Disfigurement Or “Cleft Syndrome”

Bilateral – CLAP (ii)

Bilateral cleft lip reconstruction; radiographic control after completed reconstructive surgery

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A â&#x20AC;&#x153;below standardâ&#x20AC;? treatment protocol in infant years Treatment - LeFort I distraction + anterior subapical mandibular osteotomy

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Severe Cleft Disfigurement Or “Cleft Syndrome”

Severe facial growth deformity of midface and anterior growth of mandible (occlusion – two molar contact) Treatment - Bilateral tertiary osteoplasty with stabilization of premaxilla; LeFort I cranio-maxillary distraction ; bilateral sagittal split osteotomy; genioplasty

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Unilateral CLAP: a midfacial hypoplasia â&#x20AC;&#x201C; as a severe zygomatico-maxillary retrognathia LeFort III osteotomy and craniomaxillary distraction

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Severe Cleft Disfigurement Or “Cleft Syndrome”

Severe cleft disfigurement - The ULTIMATE Clinical pearls To achieve the best possible clinical outcomes in severe cleft disfigurement or cleft syndromes, various aspects have to be considered. Ignoring one or more of these aspects may lead to suboptimal aesthetic and functional results.

1. The term “cleft syndrome” should not be confused with “facial cleft deformity with a syndrome”. The former term describes facial deformities mostly due to inadequate or insufficient cleft repair in infancy. The latter describes facial cleft deformities with additional genetic abnormalities, which may or may not be part of a known syndrome.

2. Severe cleft disfigurement may be described as “cleft syndrome”, in that such patients present facial disfigurement after the cleft repair. Usually the disfigurement becomes more visual during adolescence. Most patients were exposed to “below standard” cleft surgery and/or treatment protocols, causing impaired midfacial growth disturbance OR to no protocols at all OR were born with a median facial cleft dysgenesis OR with another additional facial deformity.

Mainly they are “inherited” patients, treated somewhere else, then seeking further reconstruction.

3. For these adolescents and/or adults, no definite treatment protocols are available. There are basic principles to achieve the optimal functional and aesthetic result, avoiding any surgical and other overtreatment. If possible, various different surgical procedures should be performed during the same general anaesthesia.

4. Patients with severe cleft disfigurement usually need to undergo multiple surgical interventions for eventual best possible outcome.

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DATA and TABLES

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- 25 Syndromes Associated With Cleft Deformities Syndromes in the overall cleft clinic population as well as cases with genetic abnormalities lacking the proof of concomitant syndromes

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Syndromes associated with cleft deformities (the 15 most associated) Fairbairn-Robin triad (Pierre Robin sequence)

5.89%

Demarquay-van der Woude syndrome (gene 1Q32 IRF6)

1.71%

Holoprosencephaly

1.56%

Naso-maxillo-acro dysostosis (Binder’s syndrome)

0.87%

Goldenhar syndrome / oto-mandibular dysostosis

0.82%

Treacher Collins’s syndrome

0.58%

Stickler’s syndrome

0.55%

Trisomy 13 (Patau’s syndrome)

0.46%

Oro-facial-digital syndrome

0.34%

Trisomy 21 (Down’s syndrome)

0.34%

p63 gene syndromes (AEC, RHS, EEC)

0.31%

22q11.2 deletion syndromes (DiGeorge, Sphrintzen, and other)

0.31%

Popliteal pterygium syndrome (gene 1Q32 IRF6)

0.29%

Amniotic band syndrome

0.24%

Möbius’ syndrome

0.22%

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- 26 Cleft Data Collection And Research Data collection is one of the most important features in a multidisciplinary clinic. The data available allows analyses and comparisons of numerous aspects related to cleft types, racial and gender differences, family trees, syndromes, eventual events during pregnancy, treatment quality control, and more.

Data should be collected in a prospective way, using electronic data storage software. (Below a small part extracted)

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Detailed cleft classification of 32 years

CL = cleft lip; CLA = cleft lip + alveolus; CLAP = cleft lip, alveolus + palate; hP = cleft hard palate; hPsP = cleft hard + soft palate; sP = cleft soft palate; COMBI = CLAP with interruption in CA and/or hP; Oblique = any other facial clefts (excluded median [Tessier 0] and lateral [Tessier 7] clefts)

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Female to male ratio

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Racial diversity Birth ratio - Blacks 1:2340, Whites 1:725 (3.2 x more Whites)

W ♂(56.9%) - W ♀ (43.1%) B ♂ (45.2%) - B ♀ (54.8%) More black females with clefts VAN WYK PJ, BÜTOW K‑W, VAN DER MERWE CA, KLEYNHANS EE. Die insidensie en kliniese voorkoms van gesplete gesigsdeformiteite in Transvaal.” J Dent Ass S Afr 1987: 42; 403‑406.

BÜTOW K‑W, VAN WYK PJ, ZWAHLEN RA. Differences in the clinical appearances of white versus black patients with facial cleft deformities: a retrospective study. S Afr Dental Journal, 2007: 62: 298 – 304.

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- 27 Treatment Tables Each cleft type needs a number of interventions at specific dates Summary Based on the surgical protocol (Chapter 3, page 30-36)

CL (uni- and bi-lateral) (Isolated cleft lip)

CLA (uni- and bi-lateral) (Cleft lip and cleft alveolus)

05 months:

05 months:

CL – repair

CL – and anterior nasal floor repair

14 years: CL – revision (seldom) 12 months:

9 years:

(bilateral lip) – columella lengthening secondary rhinoplasty (sometimes) alveolar secondary osteoplasty

14 years:

lip revision (seldom) secondary rhinoplasty (probable)

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CLAP (u and b)

hPsP

(Cleft lip, alveolus and palate)

(Cleft hard and cleft soft palate)

05 months:

soft palate repair

07 months:

soft palate repair

07 months:

lip â&#x20AC;&#x201C; repair + anterior nasal floor repair + hard palate repair

18 months:

hard palate repair

04 years:

velopharyngeal flap (sometimes)

14 years:

mandibular orthognathic surgery (seldom)

12 months:

(bilateral lip) - columella lengthening

04 years:

velopharyngeal flap (sometimes)

09 years:

alveolar secondary osteoplasty oro-nasal fistula closure (sometimes)

sP (Cleft soft palate)

14 years:

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maxillary orthognathic surgery (sometimes) lip revision (sometimes) secondary rhinoplasty (mostly)

07 months:

soft palate repair

04 years:

velopharyngeal flap (sometimes)

Epilogue

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We would like to thank all our national and international surgical fellows, including the IAOMS surgical fellows, as well as the numerous international specialists and national registrars / residents / trainees as visitors and our 35 national registrars for their continuous stimulation and exchange in knowledge, as well as for their photographs taken during their training.

We would further like to thank the numerous team members (specialists and non-specialists; past and present) of the multidisciplinary facial cleft deformity clinic for their selfless contribution to the treatment of these patients and their knowledge shared with us, but also especially for the training and teaching of other team members regarding their particular fields. The various fields connected to the total treatment concept for Facial Cleft Deformities may therefore be better understood holistically and as such, the ultimate treatment for the individual patientâ&#x20AC;&#x2122;s deformity can be optimized.

Painting: â&#x20AC;&#x153;The circle of training in cleft surgery â&#x20AC;?

(Kuwibu 2013)

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The Ultimate Comprehensive Cleft Treatment Cleft surgery is not only a science, or a technique or art; it encompasses the subtle integration of all these to achieve the ultimate result. However, in various exceptional facial cleft deformities, extraordinary surgery elicits much more the heart of creative art.

Kurt BĂźtow and Roger Zwahlen 452