Pediatric Ophthalmology

156 8 Congenital Abnormalities of the Optic Nerve

PERIPAPILLARY STAPHYLOMA

Differential Diagnosis ● MGDA ● Optic disc coloboma ● TDS Diagnostic Evaluation

● Peripapillary staphyloma is a generally spo radic, rare, usually unilateral optic disc anom aly characterized by a deep excavation of the area of the fundus surrounding the optic disc. ● Optic disc head sits at the base of the poste rior pole excavation. ● Not associated with glial or vascular abnormali ties of the disc, uveal coloboma, and progression ● Affected eyes typically are emmetropic or slightly myopic, although high myopia has been reported. Etiology ● The etiology is unknown. It appears to arise as incomplete differentiation of sclera. Staphyloma may be the consequence of the development of normal intraocular pressure causing scleral herniation. Symptoms ● Visual acuity is usually mildly or severely reduced. Signs ● Peripapillary staphyloma is usually associ ated with a relatively normal appearance of the optic disc. ● Centrocecal scotomas commonly occur in eyes with decreased vision. ● Associated conditions may include the following: ■ Usually, absence of associated systemic abnormalities or intracranial diseases ■ Peripapillary staphyloma has been reported to be associated with basal encephalocele in patients with midfa cial abnormalities. PHACE syndrome, linear nevus sebaceous syndrome, and 18q-syndrome have been observed in asso ciation with peripapillary staphyloma. ■ Increased risk of RD

● Ocular ultrasound and ERG can help the diagnosis especially in pediatric patients. ● OCT can be used to evaluate a peripapillary staphyloma. ● VF can show a centrocecal scotoma, espe cially in eyes with decreased vision. ● MRI of the brain is indicated for children with midfacial abnormalities. Treatment ● No medical treatment for the primary disorder ● Amblyopia therapy and strabismus surgery as needed ● RD surgery as indicated ● Consider polycarbonate eye glasses. OPTIC DISC DRUSEN (PSEUDOPAPILLEDEMA) ● Optic disc drusen are acellular calcific de posits located within the optic nerve head. ● Optic drusen are typically buried in the optic disc early in life and become more su perficial later. ● Often bilaterally ● Most common form of pseudopapilledema— anomalous elevation of the optic disc unrelated to increased intracranial pressure Etiology ● The etiology is unknown—they are thought to result by a disturbance in axonal metabolism with slowed axoplasmic flow, Prognosis ● Risk of RD

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