Peripapillary Staphyloma Research Articles

Optic disc anomalies and frontonasal dysplasia

AIMSTo document the optic disc abnormalities in patients with frontonasal dysplasia in association with basal encephalocele.METHODSNames and hospital numbers of patients with midline clefts were obtained from the ophthalmology and...

Peripapillary Staphyloma

To report a rare case of peripapillary staphyloma. Case report. A 5-month-old infant was examined for esotropia associated with a retinal and optic nerve abnormality of the left eye. Detailed clinical examination along with diagnostic ultrasonography and electroretinography established a diagnosis of a peripapillary staphyloma of the left eye measuring 9.77 mm in depth. The patient's right eye was entirely normal, and she showed no systemic abnormalities. Although extremely rare, the peripapillary staphyloma may cause severe visual impairment of the affected eye in an otherwise healthy individual unassociated with systemic abnormalities.

Oculocerebral Dysgenesis in the Linear Nevus Sebaceous Syndrome

The authors document the association of peripapillary staphyloma and an atypical variant of hemimegalencephaly with the linear nevus sebaceous syndrome. Linear nevus sebaceous syndrome is an uncommon neurocutaneous disorder that has a propensity to involve the eyes. Clinical, histopathologic, and neuroimaging findings are examined in a child with linear nevus sebaceous syndrome. In addition to bilateral peripapillary staphylomas, ophthalmologic abnormalities included a corneal dermoid, a complex conjunctival choristoma, macular hypoplasia, and optic nerve hypoplasia with contralateral optic atrophy. Magnetic resonance imaging disclosed a rare form of hemimegalencephaly characterized by hypoplasia of an optic radiation within the enlarged, dysgenetic cerebral hemisphere. The spectrum of oculocerebral dysgenesis in the linear nevus sebaceous syndrome can be expanded to include peripapillary staphyloma and atypical hemimegalencephaly with hypoplasia of an optic radiation.

Bilateral Peripapillary Staphyloma with Normal Vision

Bilateral Peripapillary Staphyloma with Normal Vision

Peripapillary Staphyloma by Respiratory Pulsation

Peripapillary Staphyloma by Respiratory Pulsation