Unilateral and Bilateral Cleft Lip
18.
Unilateral Cleft Lip §
Failure of fusion of the medial nasal process with the maxillary process
Full Thickness Defect: Bone, Mucosa, Muscle, Skin
Abnormal Anatomy 1. Poorly defined philtral ridge on cleft side 2. Vertical shortness 3. Thinning of vermillion 4. White roll blurred 5. Hypoplastic musculature 6. Abnormal muscle insertion Orbicularis oris § Cleft side: Abnormal insertion into alar base § Noncleft side: Abnormal insertion into nasal spine Osseous Deformities § Projection and outward rotation of premaxilla with retropositioning of the lateral maxillary segment
Nasal Deformity 1. 2. 3. 4. 5.
Caudal septum deviated to noncleft side Posterior septum convex on cleft side, impinging airway Columella shortened, and base deviated to noncleft side Nasal tip deviated to noncleft side, and dome depressed on cleft side LLC attenuated and caudally displaced § Medial crura separated at dome from noncleft side § Lateral crura flattened and spans cleft in obtuse angle 6. Loss of overlap ULC and LLC 7. Inferior displacement and hooding of alar rim 8. Alar base outwardly rotated, flared, posteriorly displaced, and farter from midline than noncleft side 9. Hypoplastic maxilla and posterior displaced piriform margin on cleft side 10. Absent alar-facial groove on cleft side 11. Widened nostril floor on cleft side 12. Vestibular lining deficient on cleft side
Bilateral Cleft Lip § Failure of fusion of the medial nasal processes with the maxillary processes Bilateral Cleft Lip Anatomy § § § § § §
Bilateral CL deformity Orbicularis inserts into alar base b/l Paucity of tissue of the central lip element o NO muscle Inadequate or diminutive columella Limited blood supply to the prolabium Osseous Deformities o Premaxilla: Outwardly rotated and projecting o Lateral maxillary element: retropositioned and collapsed medially
Epidemiology § § § §
Overall incidence around 1 in 700 live births; varies significantly by racial group 1 in 1,000 births in whites, 1 in 500 births in Asians and Native Americans, and approximately 1 in 2,400 to 2,500 births in people of African descent . Cleft Lip & Palate – 46%; M:F 2:1 Isolated Cleft Palate – 33%; M:F 2:1 § Isolated Cleft Lip – 21% § `6:3:1 - Unilateral Left CL : Unilateral Right CL : Bilateral CL
Predisposing Factors Prenatal Nutrition (Folic Acid) • • • • •
Smoking (2x) Alcohol Social Class Parental age > 30 Altitude
• • • • • •
Anticonvulsants (10x) Retinoic acids Infections (rubella, toxo) Oligohydramnios GH deficiency Pituitary insufficiency
Genetic Models § §
Multifactorial Model: genetic susceptibility governed by many genes and their interaction with environmental variables CL (with or without CP) is an embryologically, anatomically, and genetically distinct entity from isolated CP.
Malformations Associated with Cleft Lip and Cleft Palate § Incidence in CL/CP (14%) § Incidence in isolated CP (42%) Syndromes § 3% associated with syndrome § More common with CP than CL/CP § > 154 syndromes § Most commonly recognized syndrome *Stickler syndrome (17.5%) Van der Woudeʼs Syndrome § Autosomal dominant (risk 50%) § Lower lip pits, CL+/- P, absent 2nd molars § 70-80% penetrance
Classification Unilateral vs Bilateral Incomplete vs Complete Cleft Lip +/- Cleft Palate
Microform (forme fruste) Cleft Lip § § § § §
Mild Incomplete Cleft Vertical scar or furrow extending from vermillion to nasal floor Notch in vermillion border Varying degrees of vertical lip shortness Alar deformity
Unilateral Incomplete Cleft Lip
Unilateral Complete Cleft Lip
1. 2. 3. 4.
Lips Alveolus Hard palate Soft palate
Unilateral Complete Cleft Lip and Cleft Palate
Bilateral Incomplete Cleft Lip
Bilateral Complete Cleft Lip and Cleft Palate
The Smile Index for Grading Severity of Primary Unilateral Cleft Lip/Nose Deformities Developed at the Guwahati Comprehensive Cleft Care Center The Smile Index has been developed apply a subjective grading scale to individual subjects that evaluates the overall appearance of the deformity. The Smile Index separates patients into five Grades according to the severity of their primary deformity. Grade I includes the least severe deformities and is characterized by minimal nasal deformity, an indented free mucosal margin, and notched vermillion-cutaneous junction extending upward less than one fourth of labial height. 1 This corresponds to the microform or mini-microform designation as described by Onizuka and Mullikan.1 Grade II also describes lesser forms of an incomplete labial cleft. Features include a vermillion notch that extends upward more than one fourth of lip height, a muscular depression above the cutaneous cleft into the sill, an obvious nasal deformity (short hemicolumella, dislocated genu, and displaced alar base). This corresponds to the minor designation as described by Mullikan.1 Grade III describes more severe forms of incomplete labial cleft, separated from complete cleft lips by the presence of a band of tissue on the nostril floor, often referred to as Simonart’s band. 1 Features include complete clefting of vermillion and muscle, with worsened nasal deformity (short hemicolumella, dislocated genu, and displaced alar base, and progressive deviation of the columella base to the noncleft side). Grade IV describes a complete cleft lip with corresponding increasingly severe nasal deformity. There is complete absence of the nasal floor, marked deviation of the columella toward the noncleft side and tip deviation toward the cleft side. The hemicolumella is very short. The cleft side alar base was more posterior than the noncleft side alar base. Progressive dislocation of the genu results in a worsened slump of the lover lateral cartilage, though some degree of residual anatomic alar curvature is still present. Grade V deformities are the most severe. There is wide separation between the medial and lateral elements, and a severe nasal deformity. Large cleft width combined with nasal floor absence often results in a wide passage to the nasopharynx that easily accomidates the tongue or endotracheal tube during surgery. There is an extreme columellar angle and marked septal deviation. The nasal tip is greatly deviated toward the cleft side and the hemicolumella is almost entirely deficient. The cleft side alar base is severely displaced laterally from midline, and markedly more posterior and inferior than the noncleft side alar base. The ala is completely splayed across the cleft, with complete distortion of normal alar curvature.
Treatment of Cleft Lip Multi-Disciplinary Team Nurse • • • •
Geneticist Audiologist Speech Pathologist Psychologist
• • • • •
Plastic Surgeon Pediatric Dentist Orthodontist Otolaryngologist Anesthesiologist
Feeding § § § § § §
Cleft lip and cleft palate patients more likely to be underweight than age matched control (30% vs. 13% in USA study); 56% in Assam below 3rd percentile Best to breast feed Squeezable bottles Haberman nipple Obturators Early repair
Timing: Cleft Lip Surgery § Controversial: No agreement in lit on ideal timing of CL repair § Rule of Ten (Minimize anesthesia risks): 10 weeks age; 10 lbs; 10g of Hgb Cleft Lip Management § Results are surgeon dependent § Differences in algorithms do exist § Different management algorithms have yielded excellent results
Surgical Treatment: Goals § § § § § § § §
Lengthen cleft lip height to vertical height of non-cleft side Balance Cupidʼs Bow Reorient and re-establish orbicularis oris Establish continuity of white roll Establish continuity of red line Re-establish philtrum column Recreate philtral dimple/tubercle pout Primary repair of cleft nose deformity
Suggested Reading: Hopper R, Cutting C, Grayson B. (2007) Cleft Lip and Cleft Palate. In: Thorne C, et al: Grabb and Smithʼs Plastic Surgery, 6th ed (pp. 201-225). Philadelphia: Lippincott Williams & Wilkins. Randall P, Jackson O. (2009) A Short History of Cleft Lip and Cleft Palate. In: Losse J, Kirschner R: Comprehensive Cleft Care (pp. 187-210). McGraw-Hill Companies. Huang M, Lee S, Lee S. (2009) Anatomy of Cleft Lip and Palate Short History of Cleft Lip and Cleft Palate. In: Losse J, Kirschner R: Comprehensive Cleft Care (pp. 223240). McGraw-Hill Companies.