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.

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.

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.