Communicating hydrocephalus in children with genetic inborn errors of metabolism

The authors have seen eight cases of communicating hydrocephalus in children with genetic metabolic disorders, namely, one mucopolysaccharidosis I (MPS I or Hurler syndrome), one MPS II (Hunter's disease), four MPS III (Sanfilippo syndrome) two of which were siblings, and two achondroplasias. The authors recommend surgical treatment of the latter but are doubtful about the former in which case hydrocephalus is only a contributing cause to severe dementia. In MPS hydrocephalus is due to accumulation of storage material in the piaarachnoid, as indicated by RISA cisternography carried out in six cases.