The efficacy of treatment with acetazolamide (100 mg/kg/day) without frusemide in arresting post-hemorrhagic ventricular dilatation was evaluated in 3 infants at the Hammersmith Hospital, London, UK. Treatment was begun at 21, 25, and 35 days of age. A decrease in ventricular size occurred after one week in all patients. Dilatation recurred when acetazolamide was withdrawn or reduced. Reintroduction of therapy was less effective and less well tolerated. Treatment was tailed off between 8 and 14 months of age. No patient has required shunting. In 2 additional infants, a severe and treatment-resistant acidosis required discontinuation of therapy after two days. Nephrocalcinosis was not a side-effect with monotherapy, whereas the combination of acetazolamide and frusemide is known to cause kidney damage. [1]

COMMENT. Huttenlocher first described the benefit of acetazolamide in 8 of 15 children with hydrocephalus due to various etiologies. Bergman, Shinnar, and colleagues recommended acetazolamide combined with frusemide. The report of nephrocalcinosis with the combined therapy in 1992 was cause for concern and a return to trials of monotherapy. Acetazolamide may reduce ventricular size and postpone or obviate the need for shunt insertion in neonates with hydrocephalus. The use of acetazolamide in hydrocephalus is discussed by Sarnat HB in Progress in Pediatric Neurology II, 1994, pp277-8.

The long-term prognosis for 42 children, born between 1963 and 1975, who underwent shunting for hydrocephalus, was reported from Oulu University Central Hospital, Finland [2]. Seven had died, 5 were in institutions for the mentally handicapped, one-half of the patients had neurological abnormalities or epilepsy, one-third were receiving vocational training, and one-quarter had no meaningful work. Shunts had been changed 103 times in 29 patients still alive. Encouragement and support for the families seemed essential in improving social development of patients in adolescence.