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.

Highlights

  • Myelomeningocele (MMC) is the most common congenital defect of the spine and spinal cord[1]

  • We present a case report of a child who underwent in utero repair of MMC, who developed bulging of the repair site prenatally despite the partial regression of Chiari malformation (CM) type II

  • The clinical picture of hypertensive hydrocephalus was clear, ventriculoperitoneal shunt (VPS) was indicated and the cerebrospinal fluid (CSF) leakage regressed after the procedure

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Summary

Background

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. After correction of the pseudomenigocele, the neonate showed signs of hipertensive hydrocephalus.

Conclusion
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