Intracranial ventricular haemorrhage as a first presentation of haemophilia. A case of successful surgical management.

Between 1957 and 1968, six cases of haemophilia were encountered at the University College Hospital (U.C.H.), Ibadan, Nigeria, of which the last (which forms the subject of this report) was the only one which presented first with intracranial haemorrhage. Haemorrhage of the central nervous system in haemophilia was first described in 1819 (Lane, 1840) and was once regarded as a rare occurrence. Bulloch and Fildes (1911) in their extensive review of the literature found only 13 cases; Aggeler and Lucia (1944) reported 32 cases; Sk6ld (1944) cited another five cases from Scandinavian literature; and Jones and Knighton (1956) found that the total documented cases (including three of their own) of this complication numbered 64 by 1945. However, in the last decade or so, the reported incidence of intracranial bleeding in haemophilia included the 7-2% of Hartmann and Diamond (1957); 6-3% of Silverstein (1960); 13-8% of Kerr (1964); 1-6% of Fessey and Meynell (1966); and the 3-4% of Davies, Turner, Cumming, Gillingham, Girdwood, and Darg (1966). While the bulk of evidence in recent years would indicate that intracranial haemorrhage is not as uncommon as suggested by earlier reviewers, haemorrhage into the ventricular system in haemophilia remains a rarity. Up to 1945 Jones and Knighton (1956) in their review of the literature found no single case of this complication. The present paper describes a patient whose first significant manifestation of a haemophilic state was ventricular haemorrhage which was successfully treated by surgical and medical measures.