(Polenov Research Neurosurgical Institute, Saint Petersburg)
Female patient B., aged 4, was admitted to the Department of Children’s Neurosurgery on March 11, 2004. The complaints were as follows: marked reduction of vision acuity, convulsion episodes, excessive body mass. According to the anamnesis data, there was sharp reduction of vision acuity at the age of 1.5, which developed during a day. At the age of 2 there appeared spontaneous nystagmus. The first episode of convulsions took place in August 2003 (there were 2 more attacks later).
On admission to the Polenov Research Neurosurgical Institute the girl had an excessive weight, nocturnal and diurnal enuresis. The latter was less frequent. Vision acuity was reduced to a great extent (the girl brought things close to her face). The response of pupils to light was reduced and more marked on the right. There were spontaneous nystagmus at a look to the sides and converging nystagmus at a straight look with a rotation component. Neuro-ophthalmologic examination was indicative of sharp reduction of vision acuity (Vis OD=OS£0.1), horizontal nystagmus at a straight look and vertical nystagmus in convergence. Examination of the eye fundus showed simple atrophy of optic nerves. There was a lesion of chiasm.
Craniograms demonstrated a mass of a round shape in the chiasm-sellar region. Its dimensions were 35´37´38 mm. It had a heterogeneous structure with hyperdense areas. There were no peripheral edema and widening of ventricles.
The operation was performed by Professor V.A. Khachatryan on March 15, 2004. It consisted in bifrontal osteoplastic craniotomy and removal of teratoma of the chiasm-sellar region, using a subfrontal approach.
The intraoperative findings were as follows: considerable dislocation of the chiasm and optic nerves in a dorsal-lateral direction, a tumor with a dense capsule. Its dissection revealed a great number of bony formations (137 teeth and 2 flat bones). The bottom of the third ventricle, hypophysis peduncle, both carotid arteries and cavernous sinuses were visualized after complete tumor removal. A postoperative period was characterized by a subfebrile condition, watched during a week and a half.
Control CT and MRI examinations demonstrated a state after total removal of tumor of the chiasm-sellar region.
The demonstration goal was to present information on such rare pathology as teratoma of the chiasm-sellar region, containing a great number of bony formations, and to illustrate possibility of its total removal.