Accepted Manuscript The Tessier number 3 cleft: a report of 10 cases and review of literature Karam A. Allam , MD Alan A. Lim , MD Ahmed Elsherbiny , MD Henry K. Kawamoto , MD, DDS PII:

S1748-6815(14)00182-X

DOI:

10.1016/j.bjps.2014.04.020

Reference:

PRAS 4165

To appear in:

Journal of Plastic, Reconstructive & Aesthetic Surgery

Received Date: 21 September 2013 Revised Date:

19 February 2014

Accepted Date: 22 April 2014

Please cite this article as: Allam KA, Lim AA, Elsherbiny A, Kawamoto HK, The Tessier number 3 cleft: a report of 10 cases and review of literature, British Journal of Plastic Surgery (2014), doi: 10.1016/ j.bjps.2014.04.020. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

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The Tessier number 3 cleft: a report of 10 cases and review of literature Karam A. Allam*, MD; Alan A. Lim**, MD; Ahmed Elsherbiny*, MD; and Henry K. Kawamoto**, MD, DDS Sohag Cleft-Craniofacial Unit

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*

Plastic Surgery Department Sohag University

Division of Plastic and Reconstructive Surgery

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**

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Sohag, Egypt, 82524

Department of Surgery

University of California Los Angeles Medical Center 200 Medical Plaza, Suite 465

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Los Angeles, California 90024

Corresponding author:

Dr. Karam Allam

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Lecturer and Consultant of Plastic Surgery

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Sohag Cleft-Craniofacial Unit Sohag University 82524 Sohag, Egypt [email protected]

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Summary The Tessier number 3 cleft is one of the most intricate and destructive of all facial clefts, presenting surgeons with a difficult task for reconstruction. We present a series of 10 patients

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with this rare cleft all treated by a single surgeon over 30 years. All patients with Tessier number 3 clefts treated between 1978 and 2008 by the senior surgeon were reviewed. Demographic data and all associated clinical findings including cranial and extracranial anomalies were recorded.

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Methods used to reconstruct each patient were also noted. Seven males and three females were identified and age at initial treatment ranged from 12 months to 12 years. Mean follow-up was

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6.3 years. Multiple craniofacial anomalies were appreciated including other rare facial clefts, hypertelorbitism, lacrimal obstruction, anopthalmia, choanal atresia, and hemifacial microsomia. Amniotic banding was the most prominent extracranial finding noted in these patients. Tessier number 3 clefts can be associated with multiple other craniofacial anomalies making

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reconstruction challenging. Soft tissue and bony reconstruction must be considered separately, and a variety of tools may be employed to accomplish each goal. As the presentation can be

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highly variable, an individualized treatment plan must be made to meet each patient’s specific

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Keywords: Tessier cleft; number 3 cleft.

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Introduction Historically, the first case of an oblique facial cleft was recorded in Latin by von Kulmus in 1732.1 In 1823, Laroche made the distinction between what he called cheek clefts and

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ordinary clefts of the lip.2, 3 Subsequently, Walter Dick of Glasgow described the first case of an oblique facial cleft in the English medical literature. Pelvet later separated oblique facial clefts involving the nose from other cheek clefts.1 And in 1887, Morian provided a classification for

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oblique facial clefts, dividing them into naso-ocluar clefts which extended from the nostril to the lower eyelid, and oro-ocluar clefts which extended from the eye to the lip.1, 2 This classification

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was later adopted in 1962 by the Nomenclature Committee of the American Association for Cleft Palate Rehabilitation.

Paul Tessier provided one of the most elaborate and comprehensive numerical classification systems for rare facial clefts in 1976.4 In his scheme, Tessier assigned numbers to

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each craniofacial cleft on the basis of its position relative to the sagittal midline and the orbit. Based on this classification, the number 3 cleft extends from the philtrum of the lip to the medial canthus of the eye, with foreshortening of this distance.5 The bony cleft occurs at the lateral

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incisor/canine area of the alveolus, extending through the frontal process of the maxilla to the lacrimal groove of the medial orbit.6 Soft tissue defects, including colobomas of the nasal ala

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and lower eyelid, and an inferiorly displaced medial canthus and globe, are characteristic.6 Concurrent absence or dysfunction of the nasolacrimal system is predictably high.7 The etiology of rare facial clefts remains unknown.

Lack of fusion, insufficient

mesodermal penetration, or failure of the naso-optic groove to invaginate and form the tubular nasolacrimal system, however, have all been suggested.8,9

The exact incidence of Tessier

number 3 clefts is unknown, but cases are typically sporadic with no syndromic association or

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sex predilection.7 Complete, incomplete, unilateral, and bilateral forms have all been described in the literature. 1,9 Furthermore, Tessier clefts number 7,9, 10 or 11 have been associated with number 3 clefts as the cranial extension.1,9 Frequently, patients with these clefts have also been

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found to have epibulbar cysts. Given these findings, the number 3 cleft is perhaps the most intricate and destructive of all the facial clefts.4

As Tessier number 3 clefts rank near the top in challenge and degree of difficulty

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presented to the reconstructive surgeon, treatment is often individualized to the patient. This article aims to describe different clinical features and associated malformations in 10 patients

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with the rare number 3 craniofacial cleft, as well as the obstacles faced in functional and aesthetic restoration. These patients span 30 years of experience by the senior author (HKK) and highlight the evolution in surgical treatment.

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Patients and methods

We reviewed all Tessier number 3 craniofacial cleft cases seen and treated by the senior author (HKK) during the last 30 years (1978-2008). The assessment included retrospective

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evaluation of demographic data (age of initial treatment and sex distribution), prenatal history (infection, exposure to radiation, chemicals, or medications), and family history of craniofacial

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anomalies. All clinical findings including cranial and extracranial anomalies were recorded. Different surgical procedures employed to correct deformities were recorded. The surgical treatment varied according to each case, time of presentation, severity of the clefting condition, and associated cranial and extracranial anomalies. Preoperative and postoperative photographs were taken of patients for diagnostic purposes and for evaluation of different surgical treatments.

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Results The study included 10 patients, seven males and three females; their age at initial treatment ranged from 6 weeks to 20 years. Mean follow-up was 6.3 years, ranging from 12

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months to 12 years. Three patients were previously operated on by other surgeons before initial evaluation. In all patients, antenatal history was negative for serious infections, exposure to radiation, chemicals, or illicit drugs.

All patients had no family history of craniofacial

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malformations. One patient was noted to have associated craniosynostosis which was released during early childhood.

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On physical examination, five cases were affected on the right side of the face, three on the left, and two bilaterally. The cleft was complete in six patients and incomplete in four patients. Of the four incomplete clefts, one patient had medial canthus dystopia, one was noted to have a coloboma of the nasal ala, and two were found have both alar and medial lid

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colobomas with foreshortened naso-ocluar distances. The lacrimal system was obstructed in four patients. Associated cranial and extracranial anomalies are summarized in Table 1. Other rare facial clefts were present in six patients. Of the two patients with bilateral

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involvement, one was noted to also have bilateral Tessier number 10 clefts as the cranial extension. Among unilateral cases, cranial extension of the number 3 cleft was present in three

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patients, assuming the form of number 10, 11, or 13 clefts. A contralateral Tessier number 4 cleft was present in one case with a number 10 northbound extension. Number 7 facial clefts with other features of hemifacial microsomia were found in three patients. Interestingly the number 7 clefts were noted to be ipsilateral to the number 3 cleft in two patients and contralateral to the number 3 cleft in one. An associated midline 0 cleft with a bifid nose was found in one

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patient. Bilateral cleft lip and palate was noted in five patients, two of which had bilateral number 3 clefts while the other three patients had only unilateral involvement (Table 1). Hypertelorbitism was present in three patients. Unilateral anophthalmia was present in

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one case who also had right ear grade III microtia, a right transverse number 7 facial cleft, an absent right mandibular ramus, hydrocephalus, a low set left ear with preauricular tags, and a large VSD. This patient only underwent placement of a ventriculoperitoneal shunt and died

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before any further craniofacial surgery was performed (Figure 1).

Bilateral microphthalmos was present in one patient who also had left choanal atresia, left

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mandibular hypoplasia, left mala hypoplasia, left orbital dystopia, a protruding left ear, and plagiocephalic skull with synostosis release. Left torticollis with kyphosis and scoliosis were also noted. Finally, this patient was found to have blindness in the right eye with limited vision in the left.

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With respect to limb malformations, three patients had amniotic band syndrome. Autoamputation of multiple digits with constriction rings were noted in both upper and lower extremities.

In one patient, this was limited to both hands.

In another patient, all four

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extremities were found to be involved by amniotic banding. Treatment was individualized to each patient depending largely on the senior author’s

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own experience and recommendations in some of the early cases made by Paul Tessier. Definitive cleft lip repair was performed at three months of age and cleft palate repair was typically done at the age of 12 months. In one patient, cleft lip was very wide requiring lip adhesion which was performed along

with soft palate closure at the age of three months. Formal lip repair was then postponed until the age of six months.

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With regard to surgical correction of the Tessier number 3 cleft, a z-plasty repair was performed in mild cases while a cheek rotation flap was necessary in more severe cases. Lower lid deficiency was repaired by a transposition flap from the upper lid. A proboscis-like structure

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was found in the medial aspect of the cleft in one patient, and this was used to bridge over the cleft and augment the lower lid skin (Figure 3). Shortening of the vertical distance remained a problem after repair and required repeated z-plasty of the scar in two patients. In cases of

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reduced naso-ocular distance and alar base elevation without an obvious skin cleft (incomplete form), downward repositioning and elongation of the ala was done by complete degloving of the

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nose and mucosal lining on that side through an oral vestibular incision. Dissection up to the orbital rim with protection of the infra-orbital nerve was performed along with mobilization of the mucosa from the pyriform aperature to fix the ala in the new position. This resulted in excellent repositioning (Figure 4). A composite ear cartilage graft was used to augment the

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deficient ala in one patient. A V-Y advancement flap from the dorsal nasal skin was used in another case to allow inferior rotation of the ala to a more caudal position (Figure 5). A transposition flap from the upper lid was the first line treatment for deficient skin of the

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lower lids; some patients, however, required a repeat of the same procedure. These flaps, together with medial canthopexy (and sometimes lateral canthopexy) were the main surgical

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procedures used to provide skin for the lower eyelid, helping the lid function in covering and protecting the globe. Medial canthopexy was the only necessary procedure performed for a patient with a microform Tessier number 3 cleft (Figures 6). Transplantation of the tarsal plate from the upper lid to the lower lid medial defect was needed in one case with a wide coloboma and markedly deficient tarsus of the lower lid. In a different patient, a temporalis fascia graft with postauricular skin graft was employed to close a medial lid defect. Lid skin augmentation

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with postauricular full-thickness skin grafting was needed in two patients, while full-thickness palatal graft to augment deficient lower lid conjunctiva was needed in another two. Lid laxity was persistent in one case despite repeated medial and lateral canthopexies so a tunneled

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temporalis fascia flap was sutured medially to the medial canthal attachment area. Despite all these procedures, lid skin deficiency was persistent, and combined with a slight proptosis secondary to a shallow orbit, the risk of exposure keratitis remained.

Therefore, retro-

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positioning of the globe itself was necessary through out-fracturing of the medial orbital wall and medial part of the orbital floor.

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Maxillary and orbital floor bone grafting was performed in four patients with complete clefts and two patients with incomplete clefts. Five of these six patients had split calvarial bone grafts to close bony defects in the maxilla, orbit, and forehead, and to augment the nasal dorsum and malar eminence. In one of these patients, costal rib graft was needed, as the cranial bone

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graft was not sufficient to close all gaps. In the sixth patient, a costochondral bone graft (CCBG) was used to close the orbital floor defect and augment the maxilla. CCBG was also used to augment the nose and correct a bifid nose deformity in one patient. Another patient presented

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with Proplast used for closure of the forehead and cheek bony defects. The results in this patient were unsatisfactory so we had to go back to remove the Proplast and use split calvarial bone

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grafts instead. The choice of the donor site was dependant on the patient’s age and bone availability, with calvarial bone being the first option over CCBG. Facial bipartition was performed in two patients to correct orbital hypertelorbitism.

These were performed at ages 6 and 8 years. Fronto-orbital advancement was needed in another patient with marked supra-orbital and forehead recession.

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Treatment of Tessier number 7 clefts (macrostomia) consisted of commissuroplasty with z-plasty of the skin and underlying orbicularis oris muscle (Figure 6). Costal cartilage graft was enough to correct a bifid nose deformity in the patient with an associated Tessier 0 cleft (Figure

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4). For the incomplete Tessier 4 cleft, the main problem was skin deficiency of the lower lid causing sclera show. Postauricular full-thickness skin grafting was used to augment the deficient lid skin, while full-thickness palatal-grafting was used to augment the conjunctiva.

Split

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calvarial bone grafting was used to augment the malar areas on both sides to provide the needed support for the lower eyelid.

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Resection of the obstructed, aberrant lacrimal duct and/or dacryocystorhinostomy and insertion of silicone tear drainage tubes were done in four cases. Rhinoplasty and lip revisionary surgeries were needed in some patients for better balance of the face. Treatment of associated bony and soft tissue elements of hemifacial microsomia, choanal atresia, protruding ears,

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hydrocephalus, and hand anomalies were all done according to well-described treatment protocols. Table 2 summarizes the surgical procedures done in the series.

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Reoperation was the commonest reported complication due to facial cleft secondary

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deformities e.g. short scar, lid deficiency ...etc. Other complications specific for each type of procedures reported were one palatal fistula after cleft palate repair and a case of check cellulites responded to medical treatment.

Discussion Despite being rare, the occurance of Tessier number 3 clefts is greater than that reported in the literature. This is especially true if incomplete forms such as colobomas of the alar base 8

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are included.9 Boo-Chai identified only 12 cases in an extensive review and Tessier described 16 patients with oblique facial clefts (numbers 3, 4, and 5) without mentioning specific details of each patient.9-11 In 1973, Dey presented three additional patients with the rare Tessier number 3

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cleft.12 Since that time, only a few more cases have been recorded. Until recently, where two series with higher numbers were reported. In 2010, da Silva et al in one of the biggest series of Tessier number 3 reported 21 cases in 40-years. A year after, Versnel et al. reported 22 oblique

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facial clefts.13 In this study, we describe a relatively big series of patients with Tessier number 3 facial clefts all operated on a by a single surgeon over 30 years. To our knowledge, this report is

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one of the largest series in the literature that discusses the embryology, pathology, and treatment of this rare cleft.

According the Kawamoto, the number 3 facial cleft has an equal sex distribution.9 In our study, however, we found a male to female ration of 2:1. This is in contrast with da Silva et al.

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who reported a female to male ratio of 4:31. Tessier’s nomenclature allows for several different clefts to coexist. We noted six patients who had other rare facial clefts, three of which had number 0, 4, and 7 clefts associated with the number 3 cleft. In addition, another three patients

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had cranial extensions in the form number 10, 11, and 13 clefts. Cleft number 9 also had been reported to be associated with the number 3 cleft.1 Importantly, the cranial extension was not

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always the number 11 cleft, as previously described.6 This suggests a common association and possible correlation between all of these clefts. In a study performed by Fan and colleagues on 198 hemifacial microsomia patients,

ipsilateral cleft lips were found in 10%.14 In contrast, they noted only two cases (1%) of rare facial clefts associated with hemifacial microsomia in their study (Tessier 0 and Tessier 4 clefts). Nonetheless, a causative link between facial clefts and hemifacial microsomia was suggested,

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given an association between the side of the cleft and the side of the face with hemifacial microsomia.14 In our report, a Tessier number 7 cleft and other features of hemifacial microsomia were found in three cases. This was ipsilateral to the number 3 cleft in two patients Disturbance in neural crest cell

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and contralateral to the number 3 cleft in the third case.

development and migration may be the common pathway linking hemifacial microsomia with rare facial clefts.14

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Amniotic bands in the limbs were noted in three patients in our series. This is in accordance with da Silva et al. who found 6 cases with amniotic bands in the limbs from the 21

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reported cases.1 Amniotics bands could play an etiologic role in the development of facial clefts. Choanal atresia was found in one patient in our series ipsilateral to the number 3 cleft and associated with other craniofacial anomalies found with hemifacial microsomia. Choanal atresia in association with rare facial clefts has already been reported in the literature.12, 15-17 Bonafos

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and colleagues even insisted on the importance of looking for evidence of choanal atresia in any patient presenting with a Tessier 1/13 or 2/12 cleft.17 Dey et al. reported on a case of bilateral choanal atresia associated with a right number 4 and left number 3 facial cleft.12 Migration

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anomalies of the neural crest cells was thought to be a common explanation for these associated craniofacial anomalies.17, 18

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Among all Tessier facial clefts, the number 3 cleft is one of the most intricate and

destructive of all clefts, and it can often be the most difficult to repair.4 The treatment of this cleft remains controversial, and little is published because of its rarity.

Frequently, many

surgeries are necessary for correction of the defects, and in some cases, the outcomes are less than ideal.19 In this report, treatment protocols were individualized to each case. The mildest expression of the number 3 cleft is represented by a coloboma of the ala, canthus inversus, and/or

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medial canthal dystopia. Canthus inversus in our study was fixed with a medial canthopexy, yielding excellent results. With a coloboma of the nasal ala, the vertical distance between the alar base and medial canthus is usually shortened. When mild, this problem can be fixed with

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degloving of the nose and nasal mucosa alone. Care should be taken not to move the ala down at the expense of the lower eyelid. If inferior-rotation of the ala will alter lower lid function, priority should be given to eye protection. With more severe cases, composite graft from the ear

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is usually a good option. Finally, a V-Y advancement flap from dorsal nasal skin was used to elongate the shortened hemi-nose in one patient who had previous scarring. We therefore

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recommend that each case should be approached individually according to its tissue needs. And when the underlying nasolacrimal system is disturbed, excision of the malformed, obstructed complex is a must to allow for creation of a new functioning system. The next step in the ladder of severity is the lid coloboma with deficient lid skin and risk

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for exposure keratopathy. The eyes can be dangerously exposed because of downward displacement of the lower eyelids often in concert with a variable degree of proptosis.12 Early provision of soft tissue protection is of paramount importance. Trials of lower lid medial

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coloboma repair with upward and medial rotation of the patient’s own lid tissue may be initially successful, but relapse often occurs particularly in those with wide tissue deficiency.12 Medial

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canthopexy and local tissue transfer to the lower lid from the upper lid or dorsal nasal skin are the main corrective surgeries for this problem. The deficient lid element should be addressed properly with full-thickness skin grafts (postauricular), palatal mucosal grafts, fascial strips (fascia lata or temporalis fascia), or even tarsal transplant. If lid deficiency is intractable and risk of exposure keratopathy is persistent, retropositioning of the globe through outfracturing of the medial orbital wall (with or without orbital floor) may be the only solution.

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Rotation advancement of the cheek has been used for a long time to close medial face defects, as it can provide maximal tissue with the least amount of scarring.20, 21 The problem of postoperative scarring and naso-orbital shortening can often be addressed by secondary

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revisionary z-plasties.

Understanding the skeletal deformity is basic to any reconstructive surgery of facial clefts.9 Bony clefts may vary from just a groove in the maxilla to the worst form of the cleft in

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which the orbit, maxillary sinus, nasal cavity, and mouth are confluent.9 In our series, bone grafts were used to reconstruct the orbital floor, superior and inferior orbital rims, anterior

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maxillary wall, and also to augment the nose and malar regions. Split calvarial bone grafts were the first option, and costochondral grafts were used in cases of insufficient calvaral donor sites. We prefer using autogenous bone for reconstruction when available to avoid the complications inherent with any synthetic material. Although fat injection was not used in any of these patients,

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we also think it can be employed to obtain a better facial balance if there is any residual asymmetry following bony correction.

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Conclusion

Considering the rarity of the Tessier number 3 cleft, our report represents one of the

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largest series in the literature describing a single surgeon’s experience in treating this complex facial cleft. This study also elaborates on the degrees of cleft severity, the possible associated cranial and extracranial anomalies, and different treatment options available. As these clefts can be variable in presentation, each treatment approach must be individualized to the patient and their needs.

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Conflicts of interest None. Sources of funding

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None.

References

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14. 15.

16. 17.

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da Silva Freitas R, Alonso N, Busato L, et al. Oral-Nasal-Ocular Cleft: The Greatest Challenge Among the Rare Clefts. J Craniofac Surg 2010;21(2):390-5. Gorlin RJ, Cohen MM, Levin LS. Syndromes of the Head and Neck. 3rd ed. Vol. 1. New York: Oxford University Press, 1990. Kawamoto H. Rare craniofacial clefts. Vol. 4. Philadelphia, PA: WB Saunders, 1990. Tessier P. Anatomical classification facial, cranio-facial and latero-facial clefts. J Maxillofac Surg 1976; 4(2):69-92. Gunter GS. Nasomaxillary Cleft. Plast Reconstr Surg 1963;32:637-645. Thorne CH, Beasley R, Aston S, et al. Grabb & Smith's Plastic Surgery. 6th ed. Philadelpha, PA: Lippincott Williams & Wilkins, 1997. Eppley BL, van Aalst JA, Robey A, et al. The spectrum of orofacial clefting. Plast Reconstr Surg 2005;115(7):101e-114e. Mann I. Development of the human eye. London, England: British Medical Association, 1964. Kawamoto HK, Jr. The kaleidoscopic world of rare craniofacial clefts: order out of chaos (Tessier classification). Clin Plast Surg 1976;3(4):529-572. Boo-Chai K. The oblique facial cleft. A report of 2 cases and a review of 41 cases. Br J Plast Surg 1970;23(4):352-359. Boo-Chai K. The oblique facial cleft: a 20-year follow-up. Br J Plast Surg 1990;43(3):355-358. Dey DL. Oblique facial clefts. Plast Reconstr Surg 1973;52(3):258-263. Versnel SL, van den Elzen ME, Wolvius EB, et al. Long-term results after 40 years experience with treatment of rare facial clefts: Part 1-oblique and paramedian clefts. J Plast Reconstr Aesthet Surg 2011;64(10):1334-43. Fan WS, Mulliken JB, Padwa BL. An association between hemifacial microsomia and facial clefting. J Oral Maxillofac Surg 2005;63(3):330-334. Garabedian EN, Ducroz V, Leperchey F, et al. Malformations of the nasal fossa and paramedian facial clefts. New perspectives. Ann Otolaryngol Chir Cervicofac 1996;113(7-8):373-78. Garabedian EN, Ducroz V, Roger G, et al. Nasal fossa malformations and paramedian facial cleft: new perspectives. J Craniofac Genet Dev Biol 1999;19(1):12-19. Bonafos G, Capon-Degardin N, Fayoux P, Pellerin P. Choanal atresia and rare craniofacial clefts: report of three cases with a review of the literature. Cleft Palate Craniofac J 2004;41(1):78-83.

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1.

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20.

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Hengerer AS, Strome M. Choanal atresia: a new embryologic theory and its influence on surgical management. Laryngoscope 1982;92(8 Pt 1):913-921. Giglio A, Ruschel FF, Barcellos C, et al. Rotation and advancement flap of the cheek in the treatment of rare craniofacial clefts. J Craniofac Surg 2008;19(5):1411-5. van der Meulen JC. Oblique facial clefts: pathology, etiology, and reconstruction. Plast Reconstr Surg 1985;76(2):212-24. Timosca G, Vicol C, Popescu E, et al. Plastic surgery for losses of facial substance by cheek rotation. Stomatologie 1990;37(1):61-6.

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18.

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Table 1 Summary of the case series Case Sex Other cranial Craniofacial clefts no anomalies

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- Hypertelorbitism.

- Bilateral cleft lip and palate.

- Protruding right ear.

- Bilateral #3 and #10 clefts.

-Bilateral lacrimal sac obstruction and dilatation.

- Amniotic band syndrome involving all 4 extremities.

- Right ear microtia grade III.

- Cardiac problems (Large VSD, congestive heart failure).

- Bilateral cleft lip and palate. M

- Right #3 and #7 clefts

-obstructed nasolacrimal system.

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3

- Amniotic band syndrome involving both hands.

- Right # 3 cleft.

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2

F

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1

Extracranial findings

(Figure 1).

- Right anophthalmia. -Absent right mandibular ramus. - Hydrocephalus.

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F

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- Left ear: low set with preauricular tags.

- Right #3 #11 Cleft.

- Hypertelorbitism.

- Amniotic band syndrome.

- Left choanal atresia

- Left torticollis.

- Bilateral microphthalmos

- Hypnosis and scoliosis

- Left #4 #10 Cleft.

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- Left #3 and #7 Cleft

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5

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- Bilateral cleft lip and palate.

(Figure 2).

- Hemifacial microsomia, left mandibular hypoplasia, left malar hypoplasia, left orbital dystopia. - Left protruding ear.

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- Plagiocephalic skull with history of release of synostosis.

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- Right microform #3/ #11 cleft.

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- Blind right eye, limited vision left. - Hemifacial microsomia.

- Left #7 cleft.

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- Right #3 / #13 cleft. - Midline #0 cleft

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- Cystic dilatation of lacrimal sac.

(Figure 4).

- Epibulbar dermoid right eye.

- Bilateral # 3 cleft.

- Hypertelorbitism.

- Bilateral cleft lip and palate.

- Bilateral proboscislike structure in medial central area.

(Figure 3). M

- Left #3 cleft.

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- Left #3 Cleft.

- Cystic formation of lacrimal sac.

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7

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(Figure 6).

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- Bilateral cleft lip and palate.

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(Figure 5).

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Table 2 Summary of the surgical procedures done: Procedure

- Cleft lip:

Cleft lip repair (3 months).

- Cleft palate:

Cleft palate repair (12 months).

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- Facial clefts:

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Deformity

Transposition flap to elevate medial canthus and medial

1- Primary procedure:

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portion of lower eyelid usually at the same time as the cleft lip repair if the ocular globe was safely protected.

2- Older child with

Check rotation.

Scar z-plasty and base elevation.

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- Residual shortening:

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unacceptable facial scars:

- Reduced naso-ocular distance:

a. Degloving of the nasal skin and mucosa (especially in mild cases). b. V-Y advancement flap from dorsal nasal skin. c. Composite ear cartilage graft for deficient ala.

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- Lower eye lid

a. Transposition from upper lid (first choice).

reconstruction:

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b. Adjuvant canthopexies (medial and sometimes lateral), medial canthopexy is main treatment of for minor forms.

d. FTSG and palatal grafts.

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c. Tarsal graft for tarsal defects.

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e. temporalis fascia in challenging cases and persistent laxity, STSG can be used with it.

Outfracturing of medial and inferomedial walls.

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defects:

Bone grafts (split calvarial or rib).

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- Maxillary and orbital for

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- Shallow orbit:

- Nose:

Nose augmentation with bone graft whenever needed.

- Hypertelorbitism:

Facial bipartion in cases (6 and 8 years).

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FIGURE LEGENDS Figure 1. (Case no. 3 in table 1): Right number 3 and 7 facial clefts, right anophthalmia, right hemifacial microsomia with ear remnant on the middle of the right cheek

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Figure 2. (Case no. 5 in table 1): (A) Photograph showing left hemifacial microsomia, orbital dystopia, hypoplastic zygoma, bilateral microphthalmos, and torticollis associated with number 3 and 7 facial clefts. (B) Postoperative photograph after torticollis release, costal

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cartilage graft to left orbital rim and malar eminence, LeFort I osteotomy, left brow lift

left ala and nasal tip plasty.

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and lateral canthopexy. (C) Follow-up photograph after composite graft from right ear to

Figure 3. (Case no. 8 in table 1): (A) Bilateral severe number 3 facial cleft with proboscis-like structure at medial canthal region, complete cleft lip and palate. (B) Postoperative view after lip adhesion, lower lid augmentation, and premaxillary set-back. (C) Photograph

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showing patient post-lip repair and facial cleft repair.

Figure 4. (Case no. 7 in table 1): (A) Right number 3/13 facial cleft and midline facial cleft with bifid nose. (B) Photograph showing medial coloboma of lower eyelid and upward

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displacement of the ala. (C) Ten year postoperative follow-up after medial canthopexy, inferior rotation of the ala, and costal graft for the bifid nose.

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Figure 5. (Case no. 10 in table 1): (Left) Photograph showing initial presentation with left medial canthal dystopia, wide glabellar scar, deficient left ala and short lip deformity. (Right) Postoperative view showing correction of medial canthal position, repositioning

of the left ala, and lip revision.

Figure 6. (Case no. 6 in table 1): (Left) Frontal view showing right facial cleft number 3/11 with left hemifacial microsomia.

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Oblique view demonstrating left facial cleft

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number 7. (Right) Six year post-operative photograph after commissuroplasty, medial canthopexy, right eyebrow repositioning, LeFort I osteotomy and bilateral sagittal split

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osteotomy of the mandible.

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