Pierre-Aurélien Beuriat1, Isabelle Poirot2, Frederic Hameury3, Alexandru Szathmari1, Christophe Rousselle4, Isabelle Sabatier4, Federico di Rocco1, Carmine Mottolese5. 1. Department of Pediatric Neurosurgery, Hôpital Femme Mère Enfant, Lyon Cedex, France. 2. Department of Pediatric Rehabilitation l'ESCALE, Hôpital Femme Mère Enfant, Lyon Cedex, France. 3. Department of Urologic Surgery, Hôpital Femme Mère Enfant, Lyon Cedex, France. 4. Department of Pediatric Neurosurgery, Hôpital Femme Mère Enfant, Lyon Cedex, France; Department of Pediatric Neurology, Hôpital Femme Mère Enfant, Lyon Cedex, France. 5. Department of Pediatric Neurosurgery, Hôpital Femme Mère Enfant, Lyon Cedex, France. Electronic address: carmine.mottolese@chu-lyon.fr.
Abstract
INTRODUCTION: Myelomeningocele (MMC) is a complex neural tube defect. Few studies report the results of modern postnatal management. The goal of this study was to report the long-term outcome of a multidisciplinary approach of patients with MMC. METHODS: Forty-six MMCs were included. Clinical status was evaluated prospectively. RESULTS: Mean follow-up was 8.1 years. The level of the malformation was sacral or lower lumbar (≤L4) in 27 cases, higher lumbar (between L1 and L3) in 5 cases, and thoracic in 14 cases. A Chiari II malformation was present at birth in 78.3% of the cases. Seventy-six percent of the patients were operated on within the first 24 hours of life. Sixty-one percent needed a cerebrospinal fluid diversion procedure. Seven patients underwent a second surgery for a retethering of the spine. Eighty percent presented with orthopedic problems. Sixty-five percent of our patients were able to walk. Only 13% of patients had a normal urinary elimination. Two groups of patients were identified: one group with a malformation at the level of L4 or below (group 1) and another group with a malformation strictly above L4 (group 2). Group 1 had significantly better outcome. CONCLUSIONS: This series proves that modern multidisciplinary postnatal management of MMC is effective. In the light of these results and of the results of prenatal management of MMC, prenatal surgery seems to be a highly valuable tool to improve the outcome of patients with high lesions (level ≥L3).
INTRODUCTION: Myelomeningocele (MMC) is a complex neural tube defect. Few studies report the results of modern postnatal management. The goal of this study was to report the long-term outcome of a multidisciplinary approach of patients with MMC. METHODS: Forty-six MMCs were included. Clinical status was evaluated prospectively. RESULTS: Mean follow-up was 8.1 years. The level of the malformation was sacral or lower lumbar (≤L4) in 27 cases, higher lumbar (between L1 and L3) in 5 cases, and thoracic in 14 cases. A Chiari II malformation was present at birth in 78.3% of the cases. Seventy-six percent of the patients were operated on within the first 24 hours of life. Sixty-one percent needed a cerebrospinal fluid diversion procedure. Seven patients underwent a second surgery for a retethering of the spine. Eighty percent presented with orthopedic problems. Sixty-five percent of our patients were able to walk. Only 13% of patients had a normal urinary elimination. Two groups of patients were identified: one group with a malformation at the level of L4 or below (group 1) and another group with a malformation strictly above L4 (group 2). Group 1 had significantly better outcome. CONCLUSIONS: This series proves that modern multidisciplinary postnatal management of MMC is effective. In the light of these results and of the results of prenatal management of MMC, prenatal surgery seems to be a highly valuable tool to improve the outcome of patients with high lesions (level ≥L3).