Transverse Clefts &
29.
Rare Craniofacial Clefts
Transverse Facial Clefts (Macrostomia, Tessier 7) § § § § § §
Incidence ranges from 1:100 to 1:300 of all facial clefts. Unilateral > Bilateral Results from failure of the mandibular and maxillary processes to fuse during the fourth and fifth week of embryogenesis. May be an isolated phenomenon, but it is usually associated with other disorders such as hemifacial microsomia and / or ear tags. Presentation can vary from minimal displacement of the oral commissure to a complete cleft of the face. Usually does not extend beyond the anterior border of the masseter muscle.
Principles of Surgical Reconstruction: § § § § § § §
Repair as early as possible to assist with speech and eating (6mo.) Accurate positioning of the oral commissure Reconstruction of the orbicularis muscle is the key to functional and aesthetic construction of the commissure and is performed with an overlapping myoplasty. Skin closure with minimal scar Avoidance of late migration of oral commissure due to scar contracture Z-plasty to break up the scar and recruit cheek skin. It is generally agreed that the repair should be performed as early as possible, to prevent feeding and speech problems, as well as for aesthetic reasons.
Preoperative Marking: §
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Positioning of the new commissure based on measurements from normal side cupidʼs bow to the contralateral normal commissure, as well as careful inspection of the anatomic changes of the muscle and vermilion at the abnormal commissure. Normal commissure also falls along a straight line dropped from the medial limbus of the iris. In cases of bilateral macrostomia, the position of the new commissure is placed at the level of the medial limbus symmetric distances from midline. Utilizing above techniques the commissure points (c points) are marked on the upper and lower lateral lip elements at skin vermillion junction. A small medially based triangular vermillion flap (v flap) is raised from the upper (or lower) lip and transposed across the new commissure to prevent commissure migration.
Buccal mucosa turnover flaps (b flaps) are designed on upper and lower lips to be pedicled on the internal mucosa for intraoral reconstruction of cleft.
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A Z-plasty was placed in the skin closure to break up the scar and to prevent linear contraction. The central limb of the planned Z-plasty lies along the line of the cleft. The resultant scar after transposition of the Z-plasty is best if it has central limb of the resultant scar perpendicular to the line of the cleft.
Surgical Repair: § § § § §
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Infiltration lidocaine / eminephrine Incisions made with 15 blade. V flap elevated off of musculature and based medially at upper lip c point. B flaps raised off of intraoral musculature and pedicled on intraoral mucosa. Aberrant orbicularis oris muscle dissected free from surrounding tissues laterally from cleft margin. Orbicularis muscle is repaired in an overlapping fashion with upper portion overlapping anterior to lower portion to recreate the sphincter mechanism. Temporary cutaneous suture at c points can facilitate accurate reconstruction. Muscle sutures performed with prolene horizontal mattress sutures.
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B flaps sewn together to reconstruct intraoral buccal mucosa. V flap transposed and inset into lower lip.
Z-plasty along the margin of the cleft is been emphasized as a useful technique to not only break up a scar and to avoid linear contracture, but to recruit tissue in a transverse direction. When designing a Z-plasty to incorporate into the closure of macrostomia, the medial limb of the Z-plasty should always be planned to eventually lie along RSTLs. This has been shown to result in the most aesthetically pleasing scar. It follows naturally that the lateral limb of the Z-plasty should likewise be aligned parallel to RSTLs to have equal triangular flaps in the Z-plasty to facilitate easy closure
Skin closure
Rare Craniofacial Clefts § §
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A facial cleft is an opening or gap in the face, or a malformation of a part of the face. Facial clefts is a collective term for all sorts of clefts and affect all structures like bone, soft tissue, skin etc. Facial clefts are extremely rare congenital anomalies (2-5 per 10,000). There are many variations of a type of clefting and classifications are needed to describe and classify all types of clefting. Facial clefts hardly ever occur isolated; most of the time there is an overlap of adjacent facial clefts. The cause of facial clefts is still unclear with few different theories about the origin of the clefts. Itʼs possible that a cleft is caused by multiple factors such as disordered migration of neural crest cells, failure of the fusion process, failure of inwards growth of mesoderm, genetics, and amniotic bands.
Tessier Classification § § § §
Based on the anatomical position of the cleft Different types of Tessier clefts are numbered 0 to 14. 15 different types of clefts can be put into 4 groups, based on their position: midline clefts, paramedian clefts, orbital clefts and lateral clefts. The Tessier classification describes the clefts at soft tissue level as well as at bone level, because it appears that the soft tissue clefts can have a slightly different location on the face than the bony clefts.
Midline clefts § § § §
Tessier number 0 and number 14. The clefts comes vertically through the midline of the face. Tessier number 0 comes through the maxilla and the nose, while Tessier number 14 comes between the nose and the frontal bone. May present with bifed frontal bone and central encephalocele extending caudally with bifed nose and central cleft of the upper lip. They are accompanied by hypertelorism and may extend along the cranial base with herniation of the cranial contents (pharyngeal encephalocele).
Paramedian clefts § § § §
Tessier number 1, 2, 12 and 13 are the paramedian clefts. These clefts are quite similar to the midline clefts, but they are further away from the midline of the face. Tessier number 1 and 2 both come through the maxilla and the nose, in which Tessier number 2 is further from the midline (lateral) than number 1. Tessier number 12 is in extent of number 2, positioned between nose and frontal bone, while Tessier number 13 is in extent of number 1, also running between nose and forehead. Both 12 and 13 run between the midline and the orbit.
Orbital clefts § § § §
Tessier number 3, 4, 5, 9, 10 and 11 are orbital clefts. These clefts all have the involvement of the orbit. Tessier number 3, 4, and 5 are positioned through the maxilla and the orbital floor. Tessier number 9, 10 and 11 are positioned between the upper side of the orbit and the forehead or between the upper side of the orbit and the temple of the head. Like the other clefts, Tessier number 11 is in extent to number 3, number 10 is in extent to number 4 and number 9 is in extent to number 5.
Lateral clefts § § §
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The lateral clefts are the clefts which are positioned horizontally on the face. These are Tessier number 6, 7 and 8. Tessier number 6 runs from the orbit to the cheek bone. Tessier number 7 is positioned on the line between the corner of the mouth and the ear. A possible lateral cleft comes from the corner of the mouth towards the ear, which gives the impression that the mouth is bigger (macrostomia). Tessier number 8 runs from the outer corner of the eye towards the ear. The combination of a Tessier number 6-7-8 is seen in the Treacher Collins Syndrome. Tessier number 7 is more related to hemifacial microsomia and number 8 is more related to Goldenhar syndrome.
Treatment §
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There isnʼt one kind of treatment for facial clefts, because of the large amount of variation in these clefts. Which kind of surgery is used depends on the type of clefting and which structures are involved. There is much discussion about the timing of reconstruction of bone and soft tissue. The problem with early reconstruction is the recurrence of the deformity due to the intrinsic restricted growth. This requires additional operations at a later age to make sure all parts of the face are in proportion. A disadvantage of early bone reconstruction is the chance to damage the tooth germs, which are located in the maxilla, just under the orbit. The soft tissue reconstruction can be done at an early age, but only if the used skin flap can be used again during a second operation. The timing of the operation depends on the urgency of the underlying condition. If the operation is necessary to function properly, it should be done at early age. The best esthetic result is achieved when the incisions are positioned in areas which attract the least attention (they cover up the scars). If, however, the function of a part of the face isnʼt damaged, the operation depends on psychological factors and the facial area of reconstruction. The treatment plan of a facial cleft is planned right after diagnosis. This plan includes every operation needed in the first 18 years of the patients life to reconstruct the face fully. In this plan, a difference is made between problems that need to be solved to improve the health of the patient (coloboma) and problems that need to be solved for a better cosmetic result (hypertelorism). The treatment of the facial clefts can be divided in different areas of the face: the cranial anomalies, the orbital and eye anomalies, the nose anomalies, the midface anomalies and the mouth anomalies.
Treatment of the Cranial Anomalies Encephalocele §
The treatment of encephalocele is based on surgery to repair the bony gap and provide adequate protection of the underlying brain. The question remains if the external brain tissue should be put back into the skull or if it is possible to cut off that piece of brain tissue, because its claimed that the external piece of brain tissue often isnʼt functional, with the exception of a basal encephalocele, in which the pituitary glad can be found in the mouth.
Treatment of Orbital / Eye Anomalies Coloboma §
The coloboma which occurs often in facial clefts is a cleft in the lower or upper eyelid. This should be closed as soon as possible, to prevent drought of the eye and a consecutive loss of vision.
Vertical Orbital Dystopia §
Vertical orbital dystopia can occur in facial clefts when the orbital floor and/or the maxilla is involved in the cleft. Vertical orbital dystopia means that the eyes do not lay on the same horizontal line in the face (one eye is positioned lower than the other). The treatment is based on the reconstruction of this orbital floor, by either closing the boney cleft or reconstructing the orbital floor using a bone graft.
Hypertelorism §
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There are many types of operations which can be performed to treat a hypertelorism. 2 options are: box osteotomy and facial bipartition (also referred to as a median fasciotomy). The goal of the box osteotomy is to bring the orbits closer together by removing a part of the bone between the orbits, to detach both orbits from the surrounding bone structures and move both orbits more to the center of the face. The goal of the facial bipartition is not only to bring the orbits closer together, but also to create more space in the maxilla. This can be done by splitting the maxilla and the frontal bone, remove a triangular shaped piece of bone from the forehead and nasal bones and pulling the two pieces of forehead together. Not only will the hypertelorism be solved by pulling the frontal bone closer together, but because of this pulling, the space between the both parts of the maxilla will become wider.
Treatment of Nose Anomalies § § §
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The nose anomalies found in facial clefts vary. The main goal of the treatment is to reconstruct the nose to get a functional and esthetic acceptable result. A few possible treatment options are to reconstruct the nose with a forehead flap or reconstruct the nasal dorsum with a bone graft, for example a rib graft. The nasal reconstruction with a forehead flap is based on the repositioning of a skin flap from the forehead to the nose. A possible downside of this reconstruction is that once you performed it at a younger age, you canʼt lengthen the flap at a later stage. A second operation is often needed if the operation is done on early age, because the nose has a restricted growth in the cleft area. Repair of the ala often requires the inset of cartilage graft, commonly taken from the ear.
Treatment of Midface Anomalies §
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The treatment of soft tissue parts of midface anomalies is often a reconstruction from a skin flap of the cheek. This skin flap can be used for other operations in the further, as it can be raised again and transposed again. In the treatment of midface anomalies there are generally more operations needed. Bone tissue reconstruction of the midface often occurs later than the soft tissue reconstruction. When the cleft involves the maxilla, it is likely that the impaired growth will result in a smaller maxillary bone in all 3 dimensions (height, projection, width).
Treatment of Mouth Anomalies §
There are several options for treatment of mouth anomalies like Tessier cleft number 2-3-7. These clefts are also seen in various syndromes like Treacher Collins syndrome and hemifacial microsomia, which makes the treatment much more complicated. In this case, treatment of mouth anomalies is a part of the treatment of the syndrome.
Suggested Reading: Monasterio FO, Tayler J. (2009) Rare Craniofacial Clefts. In: Losse J, Kirschner R: Comprehensive Cleft Care (pp. 431-452). McGraw-Hill Companies. Taub P, Vecchione L, Losee J. (2009) Transverse Facial Clefts of Macrostomia. In: Losse J, Kirschner R: Comprehensive Cleft Care (pp. 453-462). McGraw-Hill Companies. Fadeyibi I, et al. The surgical repair of macrostomia. Cleft Palate Craniofac J. 2009 Nov;46(6):642-7. Chen P, et al. Repair of Tessier no. 3 and no. 4 craniofacial clefts with facial unit and muscle repositioning by midface rotation advancement without Z-plasties. Plast Reconstr Surg. 2012 Jun;129(6):1337-44.