Multiple frontobasal meningoencephaloceles in neurofibromatosis

A patient is reported who has multiple osseomeningeal defects and bilateral frontobasal encephaloceles in the context of a neurofibromatosis. After frontobasal revision with plastic closure of a cerebrospinal fluid fistula, the rhinoliquorrhea cleared, but there was an aggravation of a preexisting internal hydrocephalus with signs of raised intracranial pressure. The conditions were stabilized after various shunt operations. Additional diagnoses included a cyst (possibly a colloid cyst) in the anterior third ventricle and partial aplasia of the anterior trabecula. The particular problems in this case in relation to the spinal meningoceles already known in neurofibromatosis and spheno-orbital encephaloceles are discussed.