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Mullerectomy for Upper Eyelid Retraction in Graves’ Orbitopathy Jonathan Liang, MD1; Laura T. Hetzler, MD2; Travis T. Tollefson, MD1; Brian S. Orisek, MD1 1Department of Otolaryngology, University of California Davis (Sacramento, CA) 2Department of Otolaryngology, Louisiana State University (New Orleans, LA) ABSTRACT INTRODUCTION Objective: To review the presentation of eyelid abnormalities in Graves’ orbitopathy (GO). To review the surgical options for addressing eyelid retraction, with a detailed focus on the mullerectomy procedure. Graves’ orbitopathy (GO) is the most common orbital inflammatory disorder in adults. It affects 10-15% of patients with thyroid disease. The incidence is 0.4% in the United States, with a female to male ratio of 4:1. Study Design: Case report and review of the literature. Methods: We describe a case of a 36 year old woman with Graves’ disease who presents with exophthalmos and upper eyelid retraction. She was bothered by the appearance of her “bulging eyes” and complained of dryness and irritation. She had no keratitis, diplopia, or evidence of optic neuropathy. Results: Patient underwent mullerectomy, or excision of Muller’s muscle. Hypertrophy of the muscle was noted. Mullerectomy was performed via a posterior conjunctival approach. It involved delicate separation of Muller’s muscle from the underlying conjunctiva and the overlying levator aponeurosis. Post-operative analysis showed improvement of upper eyelid position and patient comfort. Conclusions: Muller’s muscle is a sympathetically innervated muscle that inserts upon the upper border of the superior tarsal plate and provides 2 mm of lift. Eyelid retraction is the most common eyelid abnormality in GO. Upper eyelid surgery involves lengthening or weakening of Muller’s muscle and/or the levator aponeurosis. The mullerectomy procedure has received little attention in the otolaryngology literature. Mullerectomy is a safe and effective procedure that has been shown to improve upper eyelid position, lagophthalmos, exposure keratopathy, and patient comfort. The failure rate is low and is most often due to undercorrection. Otolaryngologists should consider mullerectomy as an option for addressing upper eyelid retraction in GO. Orbital manifestations include: •Soft tissue features: chemosis, conjunctival hyperemia, periorbital edema, orbital fat prolapse •Myopathy: diplopia, extraocular muscle limitation, exophthalmos, increased intraocular pressure •Eyelid abnormalities: eyelid retraction, lagophthalmos, scleral show, lid lag, lateral lid or temporal flare •Orbital apex compression: optic neuropathy Werner’s Classification: •Class 0: No signs or symptoms •Class 1: Only signs (retraction & stare) •Class 2: Soft tissue involvement •Class 3: Proptosis (3 mm or more) •Class 4: Extraocular muscle involvement •Class 5: Corneal involvement •Class 6: Sight loss (optic nerve involvement) Surgical management for GO follows a staged approach: (1) orbital decompression, (2) strabismus surgery, and (3) eyelid surgery. Eyelid surgery options include lateral tarsorrhaphy, lengthening or weakening of Muller’s muscle and/or the levator aponeurosis, lower lid elevation, and blepharoplasty. Mullerectomy was first described by Putterman and Urist in 1975. Muller’s muscle (Figure 1) is a sympathetically innervated smooth muscle. It arises from the levator palpebrae superioris muscle approximately 15 mm above the superior tarsal plate. It inserts onto the superior tarsal plate, and provides 2 mm of lift. RESULTS Mullerectomy was performed via a posterior conjunctival approach. Operative steps include: •Evert the upper eyelid to expose the superior palpebral conjunctiva (Figure 2). •Incise the conjunctiva at the superior tarsal border. •Dissect the conjunctiva from Muller’s muscle by raising a conjunctival flap (Figure 3). •Incise Muller’s muscle above the superior tarsal border. •Dissect Muller’s muscle from the levator aponeurosis (Figure 4). •Excise Muller’s muscle. •Close the conjunctival incision with absorbing sutures. Figure 2. The upper eyelid is everted. The conjunctiva is incised just above the upper border of the superior tarsal plate. Figure 3. A conjunctival flap is raised via careful blunt dissection. Muller’s muscle is identified and incised at the upper border of the superior tarsal plate. Figure 4. Muller’s muscle is carefully dissected from the levator aponeurosis. Subtotal mullerectomy is performed leaving a short 2-3 mm stump. Pre-operative (Figure 5) and post-operative (Figures 6 & 7) photos of the patient. Note the improvement of the upper eyelid position. Figure 1. Sagittal cross section of the upper eyelid. CASE PRESENTATION We describe a case of a 36 year old woman with Graves’ disease who presented to a tertiary care medical center. Her chief complaints are dry eyes and irritation. She was previously treated with I131, and is currently euthyroid (TSH 0.62 ng/dl, free T4 1.09 ng/dl) on thyroid replacement medication. She is bothered by the appearance of her “bulging eyes” and complains of dryness and irritation. She denies any history of keratitis, diplopia, loss of vision, or orbital pain. CONTACT Jonathan Liang, MD Department of Otolaryngology University of California Davis Med Center 2521 Stockton Blvd, Suite 7200 Sacramento, CA 95817 [email protected] Poster Design & Printing by Genigraphics® - 800.790.4001 Physical examination showed (Figure 5): •Pupils equally round and reactive to light •Extraocular muscles intact, no entrapment •MRD1 of 5 mm on L, and 6 mm on R •No inferior scleral show, R superior scleral show •No diplopia •Complete eye closure •Minimal injection, no chemosis •No evidence of keratitis Figure 5. Pre-Operative. DISCUSSION Eyelid retraction is the most common eyelid abnormality in patients with GO. This is due to sympathetic overaction of Muller’s muscle, increased sensitivity to circulating catecholamines, and fibrosis and functional shortening of the lid retractor muscles. In some patients, the upper eyelid can peak more laterally (temporal flare sign). This phenomenon has been attributed to levator aponeurosis dominance in the lateral horn and histological evidence of far lateral extension of Muller’s muscle. Surgery for upper eyelid retraction has focused on lengthening or weakening of Muller’s muscle and/or the levator aponeurosis. Techniques include marginal myotomies, recession with and without use of a spacer, and resection. Up to 2 mm of upper eyelid retraction can be ameliorated with Muller’s muscle excision. Lateral levator aponeurosis tenotomy can help decrease temporal flare. Simon et al. presented the largest series of patients who underwent transconjunctival mullerectomy. His study included 78 patients and 108 eyelids, with a mean follow-up of 16.7 months. He showed a mean MRD1 decrease of 2.6 mm and a mean lagophthalmos decrease of 0.6 mm. There was improvement in upper eyelid position, lagophthalmos, exposure keratopathy, and patient comfort. Failure was noted in 8.4% and mostly due to under-correction, which can be addressed with a second surgery. Less common complications include overcorrection, scar formation, eyelid crease asymmetry, contour abnormalities, eyelid perforation, and injury to the lacrimal apparatus. CONCLUSIONS The mullerectomy procedure has received little attention in the otolaryngology literature. Mullerectomy can be performed via a posterior conjunctival approach. It involves delicate separation of Muller’s muscle from the underlying conjunctiva and overlying levator aponeurosis. Mullerectomy is a safe and effective procedure that has been shown to improve upper eyelid position, lagophthalmos, exposure keratopathy, and patient comfort. The failure rate is low and is most often due to under-correction. Otolaryngologists should consider mullerectomy as an option for addressing upper eyelid retraction in GO. REFERENCES 1. Kakizaki H, Malhotra R, Selva D. Upper Eyelid Anatomy: An Update. Ann Plast Surg 63: 336-343, 2009. 2. Khooshabeh R, Baldwin HC. Isolated Muller’s muscle resection for the correction of blepharoptosis. Eye 22: 3. Lee HBH, Rodgers IR, Woog JJ. Evaluation and Management of Graves’ Orbitopathy. Otolaryngol Clin N Am 39: 923-942, 2006. Figure 6. Post-operative day #1. 4. Meyer DR. Surgical management of Graves orbitopathy. Curr Opin Ophthalmology 10: 343-351, 1999. 5. Putterman AM, Fett DR. Muller’s Muscle in the Treatment of Upper Eyelid Retraction: A 12-Year Study. Ophthalmic Surgery 17: 361-367, 1986. 6. Rose Jr JG, Burkat CN, Boxrud CA. Diagnosis and Management of Thyroid Orbitopathy. Otolaryngol Clin N Am 38: 1043-1074, 2005. Figure 7. Post-operative day #8. 7. Simon GJB, et al. Transconjunctival Muller Muscle Recession with Levator Disinsertion for Correction of Eyelid Retraction Associated with Thyroid-Related Orbitopathy. Am J Ophthalmology 140: 94e1-94e6, 2005.