Scleral Perforation During Inferior Rectus Muscle Recession in Congenital Fibrosis of the Extraocular Muscles

PURPOSE: We report a case of a scleral perforation during inferior rectus recession in congenital fibrosis of extraocular muscles and the management of this perforation with a scleral patch graft. CASE SUMMARY: A 20-month-old female with bilateral ptosis, absence of elevation and a chin-up position was diagnosed with congenital fibrosis of extraocular muscles. Because severe esotropia in the downward gaze was observed, we first performed esotropia surgery. After 1 year, she underwent a bilateral ptosis correction. We decided to perform bilateral inferior rectus recession due to an abnormal head posture and the absence of elevation. Because the inferior rectus muscles were extremely tight and adhered to the sclera, hooking and isolating these muscles during surgery was difficult. After muscle suture placement, a portion of the sclera that contacted the left inferior rectus was chipped off as this muscle was disinserted with blunt Westcott scissors. A scleral perforation was observed, thus, we placed a scleral patch graft using the donor sclera and finished the bilateral inferior rectus recession. No abnormal findings for the vitreous or retina were detected. At 8 months after surgery, the patient exhibited exotropia of 12 prism diopters in her primary gaze. Her abnormal head posture nearly disappeared. CONCLUSIONS: Careful isolation and disinsertion of the muscle from the globe is necessary in the treatment of patients who are expected to exhibit severe adhesions between the muscle and sclera, such as patients with congenital fibrosis of extraocular muscles.

A Case Report of the Congenital Fibrosis of Extraocular Muscles

PURPOSE: We report the clinical findings and surgical results of congenital fibrosis of extraocular muscles in a 5-year-old boy. METHODS: For a 5-year-old boy diagnosed with congenital fibrosis of extraocular muscles, we performed myectomy with disinsertion of both lateral rectus muscles and inferior rectus muscles and the conjunctival recession. We checked the position and motility of the eyeball before and after the operation, computerized tomography(CT) preoperatively, and the muscle biopsy postoperatively. RESULTS: In a 5-year-old boy complaining of blepharoptosis and ophthalmoplegia of both eyes from birth, and gaze limitation in all direction except abduction, atrophy of the extraocular muscles was found in CT. After myectomy with disinsertion of both lateral rectus muscles and inferior rectus muscles and conjunctival recession, the eyeball position improved but lower eyelid retraction occurred. The biopsy of the inferior rectus muscle confirmed the diagnosis of fibrosis of extraocular muscles. CONCLUSIONS: Congenital fibrosis of extraocular muscles shows ophthalmoplegia and blepharoptosis from birth. With clinical findings, extraocular muscle atrophy in CT is a useful finding in diagnosis. In treatment, extraocular muscle recession or disinsertion can be done but the treatment of postoperative lower lid retraction is required. Treatment of combined blepharoptosis can also be required.