Portal de Periódicos da CAPES

Ectopic Brain Tissue in a Limbal Dermoid Associated by a Scleral Staphyloma

1973; Elsevier BV; Volume: 76; Issue: 6 Linguagem: Inglês

10.1016/0002-9394(73)90094-9

1879-1891

David S. Hutchison, William R. Green, Charles E. Iliff,

Dermatological and Skeletal Disorders

A white 4-month-old female infant had a whit­ ish mass on the left eye, which had been present without change since birth. The child was the term product of a normal pregnancy and delivery and had no other apparent congenital abnormality. On examination the patient had good ocular motility and was able to follow a light with either eye. The pupils were normally reactive and there was a good red reflex bilaterally. A whitish-yellow mass with surface hair was noted from 6:30 to 8:30 o'clock at the limbus of the left eye. There was a slight full­ ness of the left lower eyelid nasally. We made a diagnosis of a limbal dermoid, and the child was scheduled for an examination under anesthesia, but she was not brought back until four months later. The lesion was unchanged. The child was again seen at IS months (Fig. 1) . The mother asked that the dermoid be removed for cosmetic reasons. At operation the senior staff surgeon remarked that he had once encountered a large scierai staphy­ loma while dissecting a limbal dermoid, and he rec­ ommended a cautious approach. A round whitishyellow mass, 6 mm in diameter with surface hair, was dissected free of the underlying sciera. This lesion extended 2 mm onto the cornea. Just poste­ rior to the dermoid, we found a large, multilobulated staphyloma, about IS mm in diameter (Fig. 2 ) . It began 4 mm posterior to the limbus at the posterior border of the dermoid and extended for approximately 12 mm. The staphyloma was rein­ forced with a large scierai patch graft. Indirect ophthalmoscopy then revealed an infolding of the retina near the ora in the area of the staphyloma. There was some vitreous reaction overlying the ret­ ina but no hemorrhage. Figure 3 shows the area six months after surgery. A discrete retinal detachment with evidence of vitreous traction developed in the area of the scierai