Angiodysgenetic necrotising encephalopathy : presentation as an intrauterine death

The various morphological manifestations of intracranial vascular anomalies include the rare diffuse meningo-cerebral angiomatosis, sometimes termed "angiodysgenetic necrotising encephalopathy" on account of the severe associated hypoxic damage to the cerebral cortex and white matter. It may occur in any age group and displays both sporadic and familial forms. A further case is reported which is apparently unique in presenting as an intrauterine death with extensive subarachnoid haemorrhage. Associated pathological abnormalities of the adrenal glands indicate that cerebral damage may have commenced from as early as the 20th week of pregnancy. Possible relationships of this entity to other central nervous system vascular malformations are discussed.