Communicating hydrocephalus and raised intracranial pressure in association with multi-systemic smooth muscle dysfunction syndrome (MSMDS).

Multi-systemic smooth muscle dysfunction syndrome (MSMDS) is extremely rare and can manifest in multiple ways. Associated hydrocephalus has not yet been reported. Here, we report a three-year-old girl with communicating hydrocephalus and raised intracranial pressure secondary to MSMDS. Pathological mechanisms are proposed, as is the need to investigate patients diagnosed with MSMDS for ventriculomegaly and raised pressure.