“…Recently Tonoki [1987] and Stensvold et al [1986], cited patients who lived 212 and 169 days, respectively, with death occurring because of respiratory failure. Hydrocephalus may develop beyond the neonatal period [Stensvold et al, 19861, and apneic spells may be an early manifestation of cord compression in the small foramen magnum [Langer et al, 1969;Shah et al, 19731. Management of these complications may not prevent neurological problems that affect development [Tonoki, 19871. Cortical malformations [Tonoki, 19871, polymicrogyria [Shah et al, 19731, neuronal heterotopia, and cerebellar hypoplasia in a small posterior fossa [Shigematsu et al, 19851 have been noted.…”