CLINICAL ARTICLE A Surgical Conundrum: Tessier Number 4 Cleft ATILLA CORUH, M.D. GALIP K. GUNAY, M.D. Objective: Among the rarest of the craniofacial clefts is the Tessier no. 4 cleft; and hence little has been published about its management and treatment. Complete forms of the cleft yield poor surgical results because of the shortened oculoalar and oculo-oral distance and inadequate soft and bony tissue. Multiple sequential corrective operations are required. A primary early concern is the protection of the eye, particularly in severe forms in which corneal exposure occurs. This article presents two cases of Tessier no. 4 clefts, one unilateral and the other bilateral, and discusses the problems encountered during their surgical and postoperative managements. KEY WORDS: atypical cleft, cleft palate, craniofacial cleft, Tessier no. 4 cleft

The incidence of rare facial clefts is between 1.43 and 4.85 per 100,000 births and 9.5 and 34 per 1000 clefts (Kawamoto, 1976). The Tessier no. 4 cleft is one of the rarest craniofacial clefts, with fewer than 50 cases having being reported in the literature (Resnick and Kawamoto, 1990). The Tessier no. 4 cleft is characterized by a cleft lip, decreased oculo-oral and oculoalar distance, orbital dystopia, eyelid colobomas, inferiorly displaced medial canthus, and cleft palate. This cleft may be found in two forms: complete and incomplete forme fruste–type clefting. The forme fruste– type cleft is characterized by minimal soft and bony tissue deformities. In the complete form, the orbital and oral cavities are confluent with the maxillary antrum. The soft tissue cleft is located lateral to the cupid’s bow and the philtrum, midway between the philtral ridge and the corner of the mouth. It continues on to the cheek, lateral to the nasal ala, ascending to the medial portion of the lower eyelid. The lacrimal sac and canalis nasolacrimalis are intact because the cleft passes lateral to these structures; however, the inferior canaliculus generally lies in the path of the cleft and is usually defective. The cleftside medial canthal ligament is almost normal. The alveolar bony cleft is located between the lateral incisor and the canine teeth sparing the piriform aperture. The cleft then continues on to the anterior surface of the maxilla medial to the infraorbital foramen to terminate in the medial portion of the inferior orbital rim and floor. The eye is usually present and functional, although microphthalmos and anophthalmos may

also occur (Kawamoto, 1990; Stretch and Poole, 1990; Kawamoto and Patel, 1998). Choanal atresiae may be associated with craniofacial anomalies including various degrees of nasal fossa malformation and may be part of paramedian facial clefts (Kawamoto, 1990; Garabedian et al., 1996, 1999). CASE REPORTS Case 1 A 15-day-old boy presented with a bilateral Tessier no. 4 cleft. The left- and right-side clefts were a complete form of Tessier no. 4 cleft. On the right side, the cleft began lateral to the cupid’s bow, sparing the piriform aperture, passing lateral to the alar groove and medial to the infraorbital foramen, extending to the infraorbital region as a wide cleft (Figs. 1 and 2). The orbital and oral cavities were confluent with the maxillary antrum. Only the temporal one fourth of the lower eyelid was present and the inferior punctum lacrimale could not be detected. The bony and soft tissue clefts were alike in their topographical anatomic landmarks. The left-side soft tissue cleft was a notch, reducing the oculo-oral distance. The bony alveolar cleft arose between the lateral incisor and the canine tooth, passing through the alveolus, coursing lateral to the piriform aperture, medial to the infraorbital foramen, extending to the zygomaticomaxillary buttress to the medial orbital rim and the floor all the way, as a fissure. The medial palpebral ligaments were intact and displaced inferiorly. The eyes were normal. There was a coloboma of the left upper eyelid situated nasally. There was also complete cleft palate and bilaterally situated choanal atresia; the left side was made of compact bone and the right side was membranous in character (Fig. 3). Ophthalmologic examination revealed serious bilateral corneal exposure and ulceration. Urgent correction of the facial cleft and the lower eyelids were planned using the technique

Dr. Coruh is an Assistant Professor, and Dr. Gunay is a Professor, Department of Plastic and Reconstructive Surgery, Medical Faculty, Erciyes University, Kayseri, Turkey. Submitted September 2003; Accepted December 2003. Address correspondence to: Dr. Atilla Coruh, Alpaslan Mahallesi, Esref Bitlis Bulvari, Imaj Sitesi, 12/A, Kat: 13, Daire: 26, Kayseri, Turkey. E-mail atilla. [email protected]. 102

Coruh and Gunay, TESSIER NO. 4 CLEFT

FIGURE 1 Case 1. Frontal photograph at 15 days old. Orbital, maxillary and oral cavities are confluent on the right-side complete cleft. In addition, bilateral severe corneal exposure is observed.

described by Resnick and Kawamoto (1990) when the patient was 15 days old. No bony reconstruction was carried out, including the right infraorbital floor. The coloboma of the left upper eyelid, localized nasally, was closed primarily. The right nonfunctional purulence-filled lacrimal sac was also excised. At the age of 12 months, the infant underwent repair of the palatal cleft by V-Y push-back palatoplasty to separate the antral and oral cavities. Because of inadequate reconstruction of the right lower eyelid and orbital floor (Fig. 4), at the age of 3 years, a Mustarde´ cheek flap (Mustarde´, 1980) with conchal cartilage in place of tarsal tissue and oral mucosal graft for palpebral conjunctiva was used for lower eyelid reconstruction. The orbital floor and infraorbital rim were restored with bone grafts that separated antral and orbital cavities. Bilateral transnasal canthopexies were also performed at the same operative session (Fig. 5). At the age of 6 years, otorhinolaryngology surgeons repaired the bilateral choanal atresia. The bone grafts used for the right-side orbital floor and infraorbital rim were partially resorbed when the patient was 6 years old (Fig. 6).

FIGURE 2 Case 1. Coronal computed tomography scan of patient. The right bony defect of the orbital floor, infraorbital rim, and cleft palate is seen. Inferior displacement of the globe is also evident.

103

FIGURE 3 Case 1. Choanal atresiae, membranous on the right, bony on the left on axial computed tomography.

The orbital floor and rim were reconstructed again with bone grafts, and at the same operative session, temporal scar revision was also performed when the patient was 8 years old (Fig. 7). Case 2 A 7-year-old girl presented with a right-sided Tessier no. 4 cleft. Cleft site orbita was anophthalmic and orbital cavity was hypoplastic (Fig. 8). The soft tissue cleft was only an embryonic scar, which passed between the philtral column and the oral commissure and continued lateral to the nasal ala onto the cheek and ascended to the medial portion of the lower eyelid. The cleft was medial to the inferior lacrimal punctum and the medial canthal ligament was unaffected because the path of the fault lay temporal to it. The ipsilateral nasolacrimal canal and sac were intact, although the lower canaliculus was defective. The bony cleft was a groove lying between the lateral incisor and the canine teeth and was coursing lateral to the piriform aperture through the anterior wall of the maxillary

FIGURE 4 Case 1. Frontal photograph at 3 years old. Dystopia of the globe to the maxillary antrum, epicanthal fold, insufficient lower lid, corneal exposure on the right cleft side.

104

Cleft Palate–Craniofacial Journal, January 2005, Vol. 42 No. 1

FIGURE 5 Case 1. Two years after first bone grafting. Frontal photograph at 5 years of age. The right-side orbital floor reconstruction was carried out with bone grafts, and lower eyelid reconstruction with Mustarde´ cheek flap and transnasal canthopexies with medial canthal ligament remnants were performed.

FIGURE 7 Case 1. Early postoperative view at 8 years of age. The orbital floor and the rim were reconstructed with bone grafts for the second time, and the scar at the temporal region was revised. The right upper lid is edematous.

FIGURE 6 Case 1. Three-dimensional computed tomography at the age of 6 years. Bone grafts used for right-side orbital floor and infraorbital rim reconstruction are partially resorbed.

Coruh and Gunay, TESSIER NO. 4 CLEFT

FIGURE 8 Case 2. Periorbital, three-dimensional computed tomography of patient. The hypoplasia of the right anophthalmic orbital cavity is clearly seen.

antrum and medial to the inferior orbital foramen, ending at the medial orbital rim and floor (Fig. 9). Soft tissue closure was carried out with a superiorly based nasolabial flap to fit it along the lower eyelid to increase the distance between the oculoalar region. The redundant skin between the philtral column and the cleft was excised and the nasal complex was rotated in a counterclockwise direction according to a rotation flap principle. The right upper lip was repaired with advancement cheek flap (Longaker et al., 1997). No bony reconstruction was performed for the fissure-like groove located on the anterior surface of the maxilla and the alveolar ridge. In the future, with the aid of sequential orbital confirmers, the hypoplastic orbital cavity will be expanded and an ocular prosthesis will be placed after suitable orbital cavity expansion. If required, multiple surgical revisions may be performed in the future (Fig. 10). DISCUSSION Tessier classifications brought about simplicity in the understanding and classification of rare facial clefts and also pioneered their successful surgical treatment (Tessier, 1976). The surgical treatment of this congenital anomaly includes: (1) medial canthopexy and lower eyelid reconstruction to protect the eye, which may be an emergency procedure; (2) bone grafting for orbital bony continuity and maxillary bony deformities; (3) reconstruction of the soft tissue defects of the cheek (if local flaps do not suffice, tissue expansion may be a versatile technique for reconstruction); (4) cleft lip repair; (5) serial orbital conformers for orbital expansion of anophthalmic or microphthalmic orbits; and (6) if required, multiple surgical revisions may be performed for bone and soft tissue deformities as correction of medial and lateral canthal ligament malposition, treatment of lower eyelid defects, and augmentation of anterior maxillary region and the zygoma (Resnick and Kawamato, 1990). When bony and soft tissue deformity is severe, the methods

105

FIGURE 9 Case 2. Frontal photograph at 7 years of age with a rightsided Tessier no. 4 forme fruste cleft.

used to reconstruct this deformity may be inadequate. Reconstruction of the lower eyelid especially constitutes a difficult problem and is often an urgent procedure when exposure of the cornea occurs. Using well-known corrective procedures for Tessier no. 4 cleft may not suffice, as in the outcome of the first operation of case 1. The reconstructed lower eyelid on the right side was inadequate and protected the eye poorly (Fig. 4). When the soft tissue defect of the infraorbital region and especially the lower eyelid is extensive, use of a Mustarde´ cheek flap may be an alternative for reconstruction of the lower eyelid. Tissue expansion seems to be an appropriate method in which there is a shortage of soft tissues. Tissue expansion also adds ideal similar skin color, texture, appearance, and aids in a perfect outcome of scar revisions (Toth et al., 1990; Menard et al., 1999). In case 1, orbital dystopia was minimal, which may be explained by early bone grafting. The necessity of secondary bone grafting to the orbit indicates the difficult problems of bony correction in this anatomical region. The patient had intelligible speech with quite good occlusion of the upper and lower jaw but still requires orthodontic treatment of anterior

FIGURE 10 Case 2. Frontal photograph 1 year after the surgery.

106

Cleft Palate–Craniofacial Journal, January 2005, Vol. 42 No. 1

open bite deformity and surgical treatment of the alveolar cleft. The patient also received psychological help when he attended primary school. In case 2, a superiorly based nasolabial flap below the lower eyelid was used to increase the distance between the oculooral and oculoalar regions. During the 1-year follow-up period, it was clearly seen that this technique brought about acceptable aesthetic units and less color discrepancy between the flap, and the neighboring tissues placed scars in less conspicuous areas on the face and gave sufficient support to the lower eyelid. If the soft tissue deficiency is severe, conventional techniques using flaps or Z-plasties, which are designed to replace the missing tissues, are far from being ideal. Mustarde´ cheek flaps for lower eyelid reconstruction may be an alternative for extensive facial clefts. REFERENCES Garabedian EN, Ducroz V, Leperchey F, Roger G, Denoyelle F. Malformations of the nasal fossa and paramedian facial clefts. New perspectives. Ann Otolaryngol Chir Cervicofac. 1996;113:373–378.

Garabedian EN, Ducroz V, Roger G, Denoyelle F, Catala M. Nasal fossa malformations and para median facial cleft: new perspect. J Craniofac Genet Dev Biol. 1999;19:12–19. Kawamoto HK Jr. Rare craniofacial clefts. In: McCarthy JG, ed. Plastic Surgery. Philadelphia: Saunders; 1990:2922–2973. Kawamoto HK Jr. The kaleidoscopic world of rare craniofacial clefts: order out of chaos (Tessier classification). Clin Plast Surg. 1976;3:529–572. Kawamato HK, Patel PK. Atypical facial clefts. In: Micheal LB, ed. Pediatric Plastic Surgery. Stamford, CT: Appleton and Lange; 1998:175–225. Longaker MT, Lipshutz GS, Kawamoto HK Jr. Reconstruction of Tessier no. 4 clefts revisited. Plast Reconstr Surg. 1997;99:1501–1507. Menard RM, Moore MH, David DJ. Tissue expansion in the reconstruction of Tessier craniofacial clefts: a series of 17 patients. Plast Reconstr Surg. 1999; 103:779–786. Mustarde´ JC. Repair and Reconstruction in the Orbital Region. 2nd ed. Edinburgh: Churchill Livingstone; 1980:316–325. Resnick JI, Kawamoto HK Jr. Rare craniofacial clefts: Tessier no. 4 clefts. Plast Reconstr Surg. 1990;85:843–849. Stretch JR, Poole MD. Nasolacrimal abnormalities in oblique facial clefts. Br J Plast Surg. 1990;43:463–467. Tessier P. Anatomical classification of facial, craniofacial and laterofacial clefts. J Maxillofac Surg. 1976;4:69–92. Toth BA, Glafkides MC, Wandel A. The role of tissue expansion in the treatment of atypical facial clefting. Plast Reconstr Surg. 1990;86:119–122.