Pseudomeningocele after in utero repair of myelomeningocele as an early sign of hypertensive hydrocephalus – a case report and review of the literature

Abstract

Background Myelomenigocele (MMC) is the most common congenital defect of the spine. The Management of Myelomeningocele study (MOMS trial) demonstrated that the prenatal repair decreased shunt implant, reversal of hindbrain herniation and better neurologic function compared to postnatal repair. Several ultrasound findings can predict the risk of postnatal hydrocephalus after intrauterine MMC repair. This report shows a prenatal pseudomenigocele after intrauterine correction of MMC as an early sign of hydrocephalus. Method A 34-year-old female G2P1 with a prenatal diagnosis of MMC with anatomical level L4 and ventricular enlargement was submitted to open surgery intrauterine repair. Follow up ultrasound showed regression of the lemon sign, partial regression of hindbrain herniation and a progressive increase in the wound with local bulging characterizing a pseudomingocele. In the postnatal period, after correction of the pseudomenigocele, the neonate showed signs of hipertensive hydrocephalus. After ventriculoperitoneal shunt, the patient was discharged. Conclusion Presence of pseudomeningocele prenatally after in utero repair of MMC may represent an early sign of hypertensive hydrocephalus.

Keywords

• myelomeningocele
• hydrocephalus
• ventriculoperitoneal shunt
• arnold-chiari malformation
• prenatal diagnosis
• fetal surgery