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Washington University Experience | NEOPLASMS (EMBRYONAL) | Medulloblastoma, Histologically Defined | Classic Subtype | 7A0 Case 7 History
Case 7 History ---- This 8 1/2 year old female was initially admitted to SLCH in 11/78 for evaluation of papilledema, increased head circumference and delayed developmental milestones. The child was a 7 lb. 3 oz. product of a 30 year old grava 2 para 1 female who had a normal pregnancy and delivery. She had normal developmental milestones during the first 6 months of life. Over the two months prior to admission she had occasional intermittent episodes of lower extremity stiffening unaccompanied by any loss of consciousness or evidence of seizure activity. In the 1 month prior to admission she had increased difficulty sitting without assistance and developed titubation. The family then moved to St. Louis and consulted a local physician approximately 1 wk prior to admission. He noted a marked increase in head circumference and referred the patient to the neurological clinic. On the day of admission, the parents noted the child had an episode of poorly reacting pupils which were unequal. The child was brought to the SLCH ER where physical exam revealed papilledema and confirmed enlarged head circumference. The anterior fontanelle was expanded. Eye exam confirmed blurred disc margins. EOMs were full and the child appeared to fix and follow to objects. Deep tendon reflexes were increased in the lower extremities bilaterally. She showed intermittent extensor posturing of both lower extremities with toe pointing occurring approximately once every 2-3 minutes. An EMI scan performed in 11/1978 revealed marked hydrocephalus of the lateral and 3rd ventricles with a blunt cut off at the posterior portion of the 3rd ventricle. The 4th ventricle was not seen and a midline cerebellar mass was noted. A VP shunt was placed but a metrizamide ventriculogram revealed the persistence of large lateral ventricles and a mass apparently pushing against the pineal recess upward. The aqueduct was blocked. On 11/20/78 a posterior fossa craniotomy was performed revealing a malignant tumor involving the 4th ventricle and superior aspect of the cerebellum. The mass of tumor was removed grossly. Postoperatively the child remained lethargic. She showed no purposeful movements. Serial skull xrays revealed air in the lateral ventricles, and persistent enlargement of the lateral ventricles. On 11/24 the ventricular peritoneal shunt was removed because of an area of scalp necrosis overlying the shunt tubing distal to the valve in the scalp. The child was discharged home on 1/7/79. The now 11 month old child was readmitted on 2/2/79 with a 3 wk history of nausea and vomiting and decreased PO intake. On admission the child's physical exam revealed a chronically ill cachectic child. The head circumference was enlarged to 51 cm. She lay quietly, appeared to be unaware of surroundings, and did not fix and follow with her eyes. The child had no head control and had periodic extensor posturing of the lower extremities. She had bilateral papilledema. Her OCN's appeared to be full and random. She had increased tone in her legs and arms. The fontanelle was tense and there was bulging over the suboccipital craniotomy defect. Over the next 10 days the child had a progressive downhill course with poor PO intake and markedly abnormal respiratory pattern. She had a respiratory arrest and expired.
