Myelocystocele Associated with Hydrocephalus: Case Report

Terminal myelocystocele is an uncommon congenital spinal dysraphism (1–5%) in which early surgical intervention is crucial for optimizing the child's neurological function. A rare complication in post-surgical correction is recurrent hydrocephalus, and its manage still challenging since other cerebral malformations, usually concomitant, are underdiagnosed by traditional methods and corroborate its development. A female child born at term, with a 10-centimeter lumbosacral dysraphism, hip dislocation, and left kidney agenesis, was diagnosed with Waardenburg Syndrome Type I. On the fourth day of life, the dysraphism was surgically addressed, with identification and dissection of a cyst formed by an abnormal tegumentary fascia, draining 120 ml of cerebrospinal fluid from its contents. Additionally, dural malformation with lipomatous content and identification of the nerve root were observed, but no placode was visualized. The dural sac was reconstituted and identified as a myelocystocele. A week later, an MRI identified non-obstructive hydrocephalus, and a flexible neuroendoscopy was performed, identifying the cerebral aqueduct, a large interthalamic adhesion, severe ventricular malformations with a narrow and highly vascularized foramen of Monro. The mammillary bodies were not visualized. The floor of the third ventricle had lateral rotation, which made endoscopic puncture difficult, posing a high risk of basilar artery injury, leading to the placement of a ventriculoperitoneal shunt. Terminal myelocystocele with hydrocephalus is a rare condition, with few studies in literature describing its management. Furthermore, flexible neuroendoscope has a high diagnostic capacity for cerebral and ventricular malformations that are involved with CSF dynamic and therapeutic option for hydrocephalus since it allows a safer and more accurate shunt placement, although other neurosurgery modalities are necessary to correct the malformation.