Familial hydrocephalus

Congenital hydrocephalus without spina bifida is a major malformation with heterogeneous aetiology. It may result both from genetic and non-genetic factors. Familial congenital hydrocephalus may be inherited by sporadic, x-linked recessive or autosomal recessive fashion. Because of a high recurrence risk and deleterious long-term effects, proper genetic counselling should be offered to the parents of an affected child. Prenatal ultrasonography may help early detection of foetal hydrocephalus and associated anomalies indicative of a poor prognosis. Recent discovery of the locus for x-linked variety on chromosomal band xq28 may allow both prenatal and carrier detection. A family with four affected siblings is reported here alone with a literature review

Surgical implications of the haemolytic-uraemic syndrome in children

The Haemolytic-Uraemic Syndrome [HUS] in children is a heterogeneous condition consisting of microangiopathic haemolytic anaemia, thrombocytopenia and acute renal failure. Complications requiring surgical intervention are rare but potentially lethal and usually involve the colon. The surgeon may face diagnostic and therapeutic dilemmas. The decision to perform a laparotomy must be individualized. Furthermore, the problem of renal involvement, if not recognised, may lead to serious fluid and electrolyte imbalance. Two patients with HUS developed surgical complications. One had total colonic gangrene with perforation and the other had segmented ischaemic colitis