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Year : 2016  |  Volume : 3  |  Issue : 2  |  Page : 100-102

A rare combination of Tessier 2 cleft with Goldenhar syndrome

Department of Burns and Plastic Surgery, Lok Nayak Hospital and Associated, Maulana Azad Medical College, New Delhi

Date of Web Publication2-Aug-2016

Correspondence Address:
Vybhav Deraje
Department of Burns and Plastic Surgery, Lok Nayak Hospital and Associated, Maulana Azad Medical College, JLN Marg, New Delhi - 110 002

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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2348-2125.187525

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The incidence of rare craniofacial clefts ranges from 1.43 to 4.85/100,000 live births. Most craniofacial centers report an incidence of 9.4-34 cases of rare clefts for every 1000 clefts. Among these Tessier clefts, the number 2 cleft is the rarest. We report one such case of complete number 2 nasal cleft in a 3-year-old boy, with features of Goldenhar syndrome, such as preauricular tags, epibulbar dermoid, and coloboma of middle one-third of the upper eyelid. This combination is extremely rare and has only been reported once before in literature. Reconstruction of the nasal cleft was carried out using a modification of Denonvilliers Z-plasty along with a composite graft from the right helix and rotation of dorsal nasal skin. Rare facial cleft combinations reinforce the embryological basis that they are likely to result from the disruption in migration of neural crest cells from rhombomeres to branchial arches by genetic manipulation by teratogens, rather than other simplistic theories.

Keywords: Facial clefts, Goldenhar syndrome, nasal clefts, syndromic clefts, Tessier 2 cleft

How to cite this article:
Deraje V, Ahuja RB. A rare combination of Tessier 2 cleft with Goldenhar syndrome. J Cleft Lip Palate Craniofac Anomal 2016;3:100-2

How to cite this URL:
Deraje V, Ahuja RB. A rare combination of Tessier 2 cleft with Goldenhar syndrome. J Cleft Lip Palate Craniofac Anomal [serial online] 2016 [cited 2022 Jul 6];3:100-2. Available from: https://www.jclpca.org/text.asp?2016/3/2/100/187525

  Introduction Top

Craniofacial clefts, classified by Paul Tessier in his landmark paper in 1976, [1] are extremely rare congenital anomalies with incidence ranging from 1.43 to 4.85/100,000 live births. [2] Their overall incidence per 1000 clefts ranges from 9.4 to 34. [3] The number 2 cleft is extremely rare among these rare craniofacial clefts, and Tessier could find only three of these in his series of 336 cases. [1] Similarly, in 1987, Ortiz-Monasterio et al. [4] reported 23 cases of number 2 cleft among 345 rare clefts seen by them in 176 patients. Initially, Tessier doubted whether this cleft existed as a distinct entity or as a transitional form between clefts number 1 and 3. Therefore, this cleft was represented by a dotted line in his original drawings. [5] However, the cleft does have unique soft- and hard-tissue characteristics. The most distinguishing soft-tissue characteristic of the number 2 cleft is the deformity on the middle of the alar rim. Association of Tessier number 2 cleft with any other cleft is not reported before in literature except once by Tanna et al. in which nasal cleft was represented by just an indentation. [6] We report one case of complete Tessier number 2 nasal cleft in association with features of Goldenhar syndrome such as preauricular tags, epibulbar dermoids, and coloboma of middle one-third of upper eyelid.

  Case report Top

A 3-year-old male patient presented with a nasal defect since birth. On examination, he had a Tessier number 2 nasal cleft characterized by a wide defect in the middle of the right alar rim [Figure 1]a. The lateral crus remnant was displaced supero-laterally, and the dome was displaced medially. He also had a right-sided preauricular tag, an epibulbar dermoid in the lower lateral quadrant of the right eye, and a coloboma of middle one-third of the right upper eyelid which fits the description of Goldenhar syndrome [Figure 1]a. The patient had no skeletal clefting or other vertebral or cardiac anomalies clinically. No further investigations were carried out. Since the patient's parent's primary concern was the nasal defect, it was decided that the nasal defect would be addressed first. An improvised procedure was planned for reconstruction in which a Denonvilliers flap was in turned as nasal lining to fill the defect following rotation of the lateral alar remnant [Figure 1]b. The complete overlying skin defect was covered with a dorsal nasal rotation flap [Figure 1]c. The medial alar deficiency measuring 6 mm × 9 mm was reconstructed with a composite graft from the right ear [Figure 1]d. Thus, the right ala was reconstructed with a modified Denonvilliers Z-plasty and a composite graft combination. Postoperatively, the composite graft was cooled for 3 days with an indigenous ice pack made of surgical glove filled with crushed ice. The final result at 4 months shows a reasonable cosmetic outcome [Figure 1]e and f. The patient is awaiting treatment for other features of the deformity.
Figure 1: (a) A 3-year-old boy with Tessier 2 nasal cleft and features of Goldenhar syndrome (epibulbar dermoid, preauricular skin tag, and coloboma of upper eyelid) on the right side. (b) Markings for rotation of alar remnant and a modified Denonvilliers flap to be used to reconstruct the nasal lining following alar rotation. (c) Green arrow shows the movement of alar rotation. Black arrow shows the Denonvilliers flap used for lining after alar rotation. Red arrow shows the dorsal rotation flap. (d) Final closure of Z-defect with rotation of dorsal nasal skin. The defect in the soft triangle was reconstructed with a composite wedge graft of 0.8 mm × 0.9 mm from body of the right helix of the ear. (e) Close up basal view of reconstructed nose at 4 months follow-up showing some areas of hypopigmentation of the composite graft and quite symmetrical reconstruction. (f) An oblique view of the face at 4 months follow-up showing symmetry and patchy areas of hypopigmentation

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  Discussion Top

Tessier number 2 nasal cleft is extremely rare. It usually starts within the Cupid's bow; the ala is hypoplastic, and there is clefting of the middle of the alar rim in some cases. The cleft does not normally affect the palpebral fissure but may cause a distortion of medial eyebrow. Skeletally, the cleft starts between the lateral incisor and canine and extends into the pyriform aperture. There may be an associated cleft palate. When the cleft continues as number 12 cleft, it may cause hypertelorism. Goldenhar syndrome is a combination of cleft numbers 7 and 8 and is associated with epibulbar dermoids/dermolipomas and vertebral anomalies. It is also known as "oculo-auriculo-facial-vertebral syndrome" and has a wide range of manifestations.

Craniofacial cleft associations are known to occur, but some combinations are extremely rare. As mentioned above, a combination of number 7 and 8 clefts is responsible for Goldenhar syndrome, and the association of number 6, 7, and 8 clefts is  Treacher Collins syndrome More Details or Franceschetti syndrome. However, the coexistence of number 2 cleft and Goldenhar syndrome is so rare that it has been reported only once before in literature. [6] In that case report, the nasal cleft was just interpreted by an indentation as there were no other features of Tessier 2 cleft; however, the patient had a paramandibular cleft. [6] There are case reports of some other independent combinations of Tessier clefts 2 or 7. In 2004, Sigler et al. [7] reported a case of unilateral partial Tessier 7 cleft accompanied by an incomplete number 2 and 3 clefts, along with a single median lip pit. Although this case also had a combination of cleft 2 and 7, there were no features of Goldenhar syndrome. In 2006, Bodin et al. [8] reported a case in which there was a combination of Tessier cleft number 3, 7, and 11, which was the first such case to be published.

These varied cleft combinations make one assumption that there exists no rule for such combinations of craniofacial clefts, but they are too infrequently seen to be tagged as a syndrome. These rare combinations once again put the embryological basis of facial clefting into perspective, reinforcing that these clefts may result from disruption in migration of neural crest cells from rhombomeres to the branchial arches because of genetic manipulation by teratogens [9] rather than simplistic theories such as failure in fusion of facial processes or failure in mesodermal migration.

The reconstruction of the number 2 nasal clefts can be accomplished in many ways. Local full thickness alar rotation may suffice if the deficiency is mild as was reported by Jinka et al. [10] or Novakovic et al., [11] who additionally used a Denonvilliers flap as a Z-plasty. Bütow and Engelbrecht [12] presented a novel method of using a resorbable sheet with local rotation flaps and Z-plasty. Rashid et al. described a rotation transposition flap to correct defects of the alar rim. [13] We employed an improvised method, combining alar rotation, Denonvilliers flap, and composite graft. As the deformity was quite severe, a simple alar rotation with Denonvilliers flap as a Z-plasty would have left a large nasal lining defect which would have healed by contraction and epithelialization. This could have compromised the result so it was decided to use the Denonvilliers flap to reconstruct the nasal lining following full thickness rotation of the alar rim. The ensuing skin defect was resurfaced with a large rotation flap from dorsal nasal skin. The scar from the rotation flap is very forgiving because it coincides with the junctions of the subunits of the nose. Already at 4 months follow-up [Figure 1]f, the scar is showing signs of fading. Medial deficiency of alar rim was compensated by a composite graft from the ear.

  Conclusion Top

We have reported a rare case of combination of Tessier cleft 2 with Goldenhar syndrome and presented another method for reconstruction of the nasal cleft.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Tessier P, editor. Anatomical classification of facial, craniofacial, and laterofacial clefts. In: Symposium on Plastic Surgery in the Orbital Region. St. Louis: CV Mosby; 1976. p. 189-98.  Back to cited text no. 1
Kawamoto HK Jr. The kaleidoscopic world of rare craniofacial clefts: Order out of chaos (Tessier classification). Clin Plast Surg 1976;3:529-72.  Back to cited text no. 2
Weinzweig J, editor. Craniofacial clefts. In: Plastic Surgery Secrets Plus. 2 nd ed. Philadelphia: Mosby Elsevier; 2010. p. 244-52.  Back to cited text no. 3
Ortiz Monasterio F, Fuente del Campo A, Dimopulos A. Nasal clefts. Ann Plast Surg 1987;18:377-97.  Back to cited text no. 4
Tessier P. Facial clefts. In: Mustarde JC, editor. Plastic Surgery in Infancy and Childhood. 2 nd ed. Edinburgh: Churchill Livingstone; 1979. p. 103.  Back to cited text no. 5
Tanna N, Wan DC, Perry AD, Kawamoto HK, Bradley JP. Paramedian mandibular cleft: Revisiting the Tessier classification. J Craniofac Surg 2012;23:e38-40.  Back to cited text no. 6
Sigler MO, Stein J, Zuker R. A rare craniofacial cleft: Numbers 7, 2, and 3 clefts accompanied by a single median lip pit. Cleft Palate Craniofac J 2004;41:327-31.  Back to cited text no. 7
Bodin F, Salazard B, Bardot J, Magalon G. Craniofacial cleft: A case of Tessier no 3, 7 and 11 cleft. J Plast Reconstr Aesthet Surg 2006;59:1388-90.  Back to cited text no. 8
Sadler TW, editor. Head and neck. In: Langman′s Medical Embryology. 9 th ed. Philadelphia: Lippincott Williams and Wilkins; 2003. p. 363-401.  Back to cited text no. 9
Jinka JR, Chekuri H, Annavarapu G. Isolated cleft of the ala nasi: A report of seven cases. Indian J Plast Surg 2012;45:512-5.  Back to cited text no. 10
[PUBMED]  Medknow Journal  
Novakovic M, Baralic I, Stepic N, Rajovic M, Stojiljkovic V. Denonvilliers′ advancement flap in congenital alar rim defects correction. Vojnosanit Pregl 2009;66:403-6.  Back to cited text no. 11
Bütow KW, Engelbrecht H. Tessier 2 nasal cleft: Reconstruction with a resorbable sheet. Br J Oral Maxillofac Surg 2014;52:93-4.  Back to cited text no. 12
Rashid M, Islam MZ, Tamimy MS, Haq EU, Aman S, Aslam A. Rotation-transposition correction of nasal deformity in Tessier number 1 and 2 clefts. Cleft Palate Craniofac J 2009;46:674-80.  Back to cited text no. 13


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