March 1998

Hydrocephalus in Maroteaux-Lamy Syndrome

Author Affiliations

Copyright 1998 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.1998

Arch Ophthalmol. 1998;116(3):400. doi:

This is a follow-up to the "Photo Essay" published in the March 1997 issue of the ARCHIVES regarding corneal transplantation in patients with Maroteaux-Lamy syndrome.1 We describe the development of hydrocephalus in our patient and the deleterious effects it had on her vision.

When originally described, a 7-year-old girl had undergone corneal transplantation in the left eye because of corneal haze related to the Maroteaux-Lamy syndrome. Nine months later, corneal transplantation was performed on the right eye; the patient's visual acuity improved from counting fingers at 30 cm to 20/400. The patient was doing well until 8 months after her second transplantation, when her visual acuity remained at 20/400 OD but decreased from counting fingers to hand movements OS. Both corneal grafts were clear. The optic nerve in the left eye was pale. Computed tomography was recommended at this time. Three weeks later, the patient returned, complaining of right-sided headaches and drowsiness. She had been treated for headaches by her primary care physician with nonsteroidal anti-inflammatory agents. Her visual acuity had decreased further to light perception in the right eye and to no light perception in the left eye. Bilateral papilledema was noted. The results of computed tomography showed enlargement of the third and lateral ventricles compared with a study done 1 year earlier. The optic nerves were unchanged from the previous computed tomography findings and showed enlarged optic nerve sheath complexes and optic atrophy. Based on the clinical signs, symptoms, and computed tomography findings of increased intracranial pressure and hydrocephalus, a ventricular peritoneal shunt was placed emergently the same day. One week postoperatively, her visual acuity in the right eye improved to counting fingers at 150 cm. Her visual acuity in the left eye remained at no light perception. The patient's headaches and papilledema resolved. Five months after the shunt was placed, the patient's visual acuity was hand motions in the right eye and no light perception in the left eye. The corneal grafts remain clear.

First Page Preview View Large
First page PDF preview
First page PDF preview