Two infants are described with intractable, drug-resistant seizures from birth associated with an enlarged cerebral hemisphere. The first died at sixteen months and demonstrated arrested head growth. Histology of the abnormal hemisphere showed disturbed cortical architecture, and subcortical heterotopias with multinucleate cells resembling tuberous sclerosis cells. Continuous fitting persisted in the second infant following callosal section. Hemispherectomy was undertaken in an attempt to preserve function of the "normal" hemisphere. Seizures ceased and head growth velocity accelerated. The neuropathology of the excised hemisphere is compared with the first case. From the literature this is the first report of a favourable outcome after hemispherectomy for hemimegalencephaly. The finding of an echodense enlarged hemisphere may allow presumption of this diagnosis in a neonate with unilateral seizures. After CT scan the diagnosis may be confirmed histologically providing that a precisely orientated frontal biopsy is submitted to the neuropathologist. We suggest that very early hemispherectomy may be the treatment of choice for this condition.
A 10-year-old girl was examined in Casualty complaining of a torticollis with her head looking downwards and to the right, which had developed suddenly that morning as she was drying herself after a bath. She had no neurological signs or symptoms. Interpretation of her cervical spine radiographs was difficult owing to her inability to move her neck for proper radiographic positioning (Fig. 1). She was given a soft collar and sent home.
Fifty-four neonates transferred to the neonatal surgical unit because of acute necrotising enterocolitis (N.E.C.) are presented. Eleven died (20.4%), of whom seven were treated conservatively and four by operation. Ten patients required surgical exploration because of intestinal perforation, obstruction or suspected perforation. Another five neonates presented with intestinal perforation and were treated by percutaneous catheter drainage of the peritoneal cavity because of their extreme prematurity. Nine patients developed a colonic stricture diagnosed between six weeks and a year after the onset of the disease. Another six patients showed a segment of colonic narrowing detected on barium enema radiography without clinical evidence of bowel obstruction. They were treated conservatively and showed complete resolution at follow up studies.
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