Syringomyelia secondary to posthemorrhagic hydrocephalus in a preterm infant

Syringomyelia is often associated with hydrocephalus, especially in Chiari malformations, but it has never been described as a complication of posthemorrhagic hydrocephalus after preterm birth. We report on a premature infant who presented this exceptional association. He was born at 29 weeks of gestational age and suffered a grade 3 intraventricular hemorrhage. Progressive ventricular dilatation developed despite repeated lumbar punctures, and a ventricular reservoir had to be inserted for cerebrospinal fluid drainage. Two weeks later he presented a flaccid, areflexic paralysis of his left upper limb. Magnetic resonance imaging disclosed a remarkable tetraventricular hydrocephalus and a cervical hydrosyringomyelia expanding from the C(5) to T(1) segments. After shunt surgery, the cephalic perimeter stabilized, and the infant began to move his arm. On follow-up, a minimal paresis of the left hand persisted. This case highlights an unreported outcome of posthemorrhagic hydrocephalus. In this context, syringomyelia should be included in the differential diagnosis of any infant with who presents segmental signs of acute or progressive onset.