Case Reports Upper Eyelid Coloboma Repair Using Accessory Preauricular Cartilage in a Patient With Goldenhar Syndrome: Technique Revisited Jeremy C. Sinkin, M.D.*, Sojung Yi, B.A.*, Benjamin C. Wood, M.D.*, Susie Kwon, B.S.*, Lauren Z. Gavaris, M.D.†, Paul T. Gavaris, M.D.†, Gary F. Rogers, M.D., J.D., M.B.A., M.P.H.*, Tina M. Sauerhammer, M.D.* ABSTRACT: We present an unusual case of upper eyelid coloboma repair in a patient with Goldenhar syndrome. We describe the use of a modified Cutler–Beard flap with concurrent inlay graft using cartilage from a preauricular appendage. This technique provides the benefits of autologous tissue, while minimizing donor site morbidity and reducing the risk of upper eyelid retraction.

INTRODUCTION Congenital palpebral coloboma is a rare defect affecting the upper or lower eyelid margin. The exact pathoetiology of eyelid coloboma remains unclear, but can occur in isolation or with abnormal corneopalpebral adhesions.1 Coloboma may also present as a feature of hemifacial microsomia (HFM), the most common congenital disorder of the face after cleft lip and palate.2 Hemifacial microsomia, also known as oculoauriculo-vertebral sequence, is known to result from defects in the development of the first and second branchial arches.3 Clinical features can include orbital and periorbital defects, mandibular hypoplasia, microtia, accessory preauricular tags and pits, middle ear defect with hearing impairment, or facial nerve impairment.4,5 Goldenhar syndrome is a name given to patients who express a constellation of anomalies that occur along the hemifacial microsomia spectrum. Although strict diagnostic criteria are not universally agreed on, the diagnosis of Goldenhar syndrome is typically made in patients with microtia, facial asymmetry, epibulbar dermoids, and cervical spine anomalies.6 The necessity of surgical intervention varies greatly depending on the severity of the phenotypic presentation. However, upper eyelid coloboma may require early intervention due to the serious sequelae that can result from delayed surgical repair, including blindness from prolonged corneal exposure and ulceration. In this report, we describe a case of upper eyelid coloboma repair utilizing an eyelid-sharing procedure and an accessory preauricular cartilage graft for structural support. We present this case for the purpose of emphasizing an uncommon technique that truly exemplifies the classic plastic surgical principles of spare part surgery, and replacing “like tissue with like.”

*Division of Plastic Surgery, Children’s National Medical Center, Washington, D.C.; and †Eye Associates of Washington, D.C., P.C., Washington, D.C., U.S.A. Accepted for publication October 20, 2014. The authors have no financial or conflict of interest to disclose. Address correspondence and reprint requests to Gary F. Rogers, M.D., J.D., M.B.A., M.P.H., Chief, Plastic and Reconstructive Surgery, Children’s National Medical Center, 111 Michigan Avenue, NW Washington, DC 20010. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000360

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CASE REPORT Our patient was diagnosed with Goldenhar syndrome shortly after birth. Examination revealed bilateral upper eyelid colobomata, with an approximate 60% right upper eyelid defect and a 40% left upper eyelid defect. In addition, the patient had small bilateral corneal limbus dermoids, mild bilateral macrostomia, left mild conchal-type microtia, and bilateral preauricular appendages (Fig. 1). The parents were instructed on the regular application of eye ointment (Lacrilube, Allergan, Inc., Irvine, CA) to each eye to prevent keratitis and corneal ulceration. At 10 weeks of age, the patient underwent the first stage of right eyelid reconstruction and left preauricular appendage excision. Informed written surgical and photographic consent was obtained in compliance with the Health Insurance Portability and Accountability Act. Surgical Technique. Right upper eyelid repair began with delineation of the upper eyelid crease. The margins of the coloboma were sharply excised, and a sliding myocutaneous flap pedicled on the levator was fashioned from the lateral eyelid remnant to close the central eyelid defect. Following eyelid eversion, a lateral incision was made through the posterior lamella and carried cephalad toward the fornix. The incision was then brought medially at the superior tarsal border to separate Mueller’s muscle from the overlying levator. The flap was completed by incising through pretarsal skin and orbicularis. The anterior lamellar structures were dissected cephalad to free them from the underlying levator. The flap was then advanced nasally and secured to the small nasal remnant of healthy eyelid. Conjunctiva, tarsus, and skin were closed in layers. This maneuver resulted in a full thickness defect of the lateral eyelid. A Cutler–Beard flap was then designed. A full thickness incision was made several millimeters below the lower eyelid tarsal border. The flap was passed deep to the lower eyelid margin and pulled superiorly to fill the upper eyelid defect, and the conjunctiva was closed. Lenticular skin incisions were then made at the base of the preauricular appendages, and the cartilage was dissected free of surrounding soft tissue down to its base. Each appendage was excised, with the largest measuring 1 cm in length (Fig. 2). To add support and reestablish lateral canthal integrity, the excised preauricular cartilage was placed as a graft and secured to the tarsus medially and to the canthal tendon laterally. The orbicularis and overlying skin from the lower eyelid flap were then closed. The patient returned 4 weeks postoperatively (Fig. 3), for division and inset of the Cutler–Beard flap, and closure of the contralateral upper eyelid coloboma with a semicircular flap. Figure 4 depicts the patient at 3 months postoperatively with well-healed incisions and an aesthetically acceptable eyelid appearance.

DISCUSSION The primary goal of eyelid reconstruction is to create a functional eyelid that protects the ocular surface and preserves adequate motility for eyelid movement and unimpeded vision.7 Smaller defects up to one-third the length of the eyelid can be closed primarily or with use of a semicircular flap. The Cutler–Beard procedure has been a widely accepted technique for the reconstruction of large upper eyelid defects since its original description in 1955.8 This 2-stage operation involves initially advancing a lower eyelid flap, composed of full thickness eyelid inferior to the tarsus, under the bridge of lower

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FIG. 3.  Healed sliding myocutaneous flap and Cutler–Beard flap at 4 weeks postoperatively, prior to division and inset.

FIG. 1.  Preoperative photograph of the patient demonstrating clinical features consistent with Goldenhar syndrome, including bilateral upper eyelid colobomata, bilateral preauricular appendages, bilateral macrostomia, and left ear microtia. A, anterior view, B, lateral view.

FIG. 2.  Excised left preauricular appendages and cartilaginous stalk.

eyelid margin, which contains the tarsus. The flap is delayed for 3 to 4 weeks prior to being divided and inset to complete the reconstruction. To minimize eyelid retraction and entropion development, some authors advocate the addition of a posterior lamellar support, or neotarsus, in the form of a graft between the conjunctiva and orbicularis oculi.9 The use of various materials, such donor sclera,10 autologous ear cartilage,9 and

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tarsoconjunctival graft,11 has been proposed to provide posterior lamellar support. Entropion of the reconstructed upper eyelid due to posterior lamellar contraction was first described and treated by Wesley and McCord.12 Their modification of the Cutler–Beard flap involved placement of a banked scleral spacer graft in between conjunctiva and orbicularis oculi during the first stage of surgery. The authors reported no resorption or shrinkage of the graft in their follow-up period ranging from 8 months to 9 years, but they speculated that graft resorption may occur if it is not covered by conjunctiva. Carroll,9 indeed, then observed entropion development from scleral graft shrinkage, occurring in 3 of 10 patients who had received banked sclera per the Wesley–McCord modification, and recommended autologous auricular cartilage graft as an alternative. Different graft materials and variations in the timing of their placement with regard to flap advancement or division have been described. Hsuan and Selva11 reported a series of 4 patients in whom contralateral upper eyelid tarsoconjunctival grafts were used to reconstruct the posterior lamella at the first stage of a modified Cutler–Beard advancement flap using lower eyelid-skin only. The authors argue that a skin-only flap allows earlier division than a standard myocutaneous flap. Yoon and McCulley7 report that secondary graft placement may avoid the challenge of dividing the eyelid precisely at the border of the tarsal graft, thus simplifying the procedure. This modification also allows for coverage of the eyelid margin with conjunctiva. However, tarsoconjunctival grafts are limited by the relatively small amounts of graft material that can be harvested without risking increased morbidity to the donor eyelid. Autologous auricular cartilage is a practical option for a tarsal graft.13,14 Conchal cartilage is relatively abundant and it is not as invasive to harvest as other donor sites. The natural curve of the conchal cartilage makes it a good match for the convexity of the eyelid. However, auricular cartilage harvest is not without inherent risk, including donor site hematoma, infection, and deformity.

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Ophthal Plast Reconstr Surg, Vol. XX, No. XX, 2014

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Strong argument can be made for omitting the sliding myocutaneous flap we performed prior to the eyelid sharing procedure. Performing a Cutler–Beard flap centered opposite the upper eyelid defect could conceivably reduce operative time and surgical complexity; however, it would most certainly obstruct the visual axis, risking occlusion amblyopia. Because a staged surgical repair of the patient’s bilateral upper eyelid colobomata was planned, we wished to minimize potential detrimental effects on our patient’s visual development. By creating a lateral defect and then completing a Cutler–Beard flap, we in essence repaired the central upper eyelid coloboma while creating a temporary lateral tarsorrhaphy.

CONCLUSIONS This case presents an unusual technique in the surgical management of bilateral upper eyelid colobomata in a patient with Goldenhar syndrome. Autologous cartilage inlay graft from preauricular appendages proved to be an effective adjunct to the modified Cutler–Beard flap coloboma repair. We believe that in this scenario, a durable reconstruction can be performed with minimal morbidity.

REFERENCES

FIG. 4.  Three months postoperative photograph demonstrating healing following bilateral upper eyelid coloboma repair.

To our knowledge, there has been only 1 other report of using accessory preauricular appendages for coloboma repair. Spano et al. describe the use of accessory preauricular cartilage and skin as a composite graft. Their patient was diagnosed with a Tessier no. 0–1 cleft and upper eyelid coloboma with ipsilateral large lower eyelid subconjunctival choristoma. Coloboma repair was performed with conjunctival flap pedicled off of the lower eyelid choristoma, and the appendage was used as a composite graft for anterior lamella reconstruction. Division and inset was completed 20 days later.15 As evidenced in their report, a particularly large preauricular appendage may provide suitable cartilaginous and cutaneous donor tissue. One may also consider carefully dissecting the skin of the appendage away from the cartilage, for use as a full thickness skin graft. In our case, it was unnecessary to use an alternate donor site because the cartilage component of the accessory preauricular appendage provided excellent structural graft for the eyelid reconstruction. The cartilage from the excised preauricular appendage had the appropriate rigidity and structure, and obviated the need to harvest additional graft. Utilizing cartilage from the excised preauricular appendage therefore retains all the benefits of an autologous graft, while also being readily available and conveying no additional donor site morbidity.

1. Nouby G. Congenital upper eyelid coloboma and cryptophthalmos. Ophthal Plast Reconstr Surg 2002;18:373–7. 2. Grabb WC. The first and second branchial arch syndrome. Plast Reconstr Surg 1965;36:485–508. 3. Stark RB, Saunders DE. The first branchial syndrome. The oralmandibular-auricular syndrome. Plast Reconstr Surg Transplant Bull 1962;29:229–39. 4. Converse JM, Coccaro PJ, Becker M, et al. On hemifacial microsomia. The first and second branchial arch syndrome. Plast Reconstr Surg 1973;51:268–79. 5. Tasse C, Böhringer S, Fischer S, et al. Oculo-auriculo-vertebral spectrum (OAVS): clinical evaluation and severity scoring of 53 patients and proposal for a new classification. Eur J Med Genet 2005;48:397–411. 6. Birgfeld CB, Heike C. Craniofacial microsomia. Semin Plast Surg 2012;26:91–104. 7. Yoon MK, McCulley TJ. Secondary tarsoconjunctival graft: a modification to the Cutler-Beard procedure. Ophthal Plast Reconstr Surg 2013;29:227–30. 8. Cutler NL, Beard C. A method for partial and total upper lid reconstruction. Am J Ophthalmol 1955;39:1–7. 9. Carroll RP. Entropion following the Cutler-Beard procedure. Ophthalmology 1983;90:1052–5. 10. Kadoi C, Hayasaka S, Kato T, et al. The cutler-beard bridge flap technique with use of donor sclera for upper eyelid reconstruction. Ophthalmologica 2000;214:140–2. 11. Hsuan J, Selva D. Early division of a modified Cutler-Beard flap with a free tarsal graft. Eye (Lond) 2004;18:714–7. 12. Wesley RE, McCord CD, Jr. Transplantation of eyebank sclera in the Cutler–Beard method of upper eyelid reconstruction. Ophthalmology 1980;87:1022–8. 13. Moon JW, Choung HK, Khwarg SI. Correction of lower lid retraction combined with entropion using an ear cartilage graft in the anophthalmic socket. Korean J Ophthalmol 2005;19:161–7. 14. Hashikawa K, Tahara S, Nakahara M, et al. Total lower lid support with auricular cartilage graft. Plast Reconstr Surg 2005;115:880–4. 15. Spano A, Piozzi E, Cavallini M, et al. Surgical approach in a rare case of coloboma-choristoma. Br J Plast Surg 2005;58:732–5.

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Upper Eyelid Coloboma Repair Using Accessory Preauricular Cartilage in a Patient With Goldenhar Syndrome: Technique Revisited.

We present an unusual case of upper eyelid coloboma repair in a patient with Goldenhar syndrome. We describe the use of a modified Cutler-Beard flap w...
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