gms | German Medical Science

59th Annual Meeting of the German Society of Neurosurgery (DGNC)
3rd Joint Meeting with the Italian Neurosurgical Society (SINch)

German Society of Neurosurgery (DGNC)

1 - 4 June 2008, Würzburg

Personal experience in the long-term management of 211 patients with myelomeningocele

Meeting Abstract

  • corresponding author G. Talamonti - Department of Neurosurgery, Niguarda Ca’ Granda Hospital, Milan, Italy
  • G. D’Aliberti - Department of Neurosurgery, Niguarda Ca’ Granda Hospital, Milan, Italy
  • M. Collice - Department of Neurosurgery, Niguarda Ca’ Granda Hospital, Milan, Italy

Deutsche Gesellschaft für Neurochirurgie. Società Italiana di Neurochirurgia. 59. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie e.V. (DGNC), 3. Joint Meeting mit der Italienischen Gesellschaft für Neurochirurgie (SINch). Würzburg, 01.-04.06.2008. Düsseldorf: German Medical Science GMS Publishing House; 2008. DocMO.05.07

The electronic version of this article is the complete one and can be found online at:

Published: May 30, 2008

© 2008 Talamonti et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.



Objective: The Authors report their series of 236 consecutive myelomeningoceles (MMC) surgically repaired during the period 1980-2007. Conditions were considered too severe to indicate surgery in 5 cases, so that 231 underwent surgical repair that was performed as soon as possible and only 14 patients (6%) were repaired beyond 3 days of birth. Enlarged ventricles were evident at birth in 212 patients (92%) but hydrocephalus had to be treated just in 182 cases (79%). The vast majority of patients (178) underwent ventriculoperitoneal shunting (VPS), while endoscopic 3rd ventriculostomy (ETV) was rarely used (4 cases) and presently completely abandoned owing to high failure rate. Within the first month, the VPS was complicated by 5 cases of ventriculitis (2.7%) and 21 cases of shunt failure (11.5%). Early brain stem dysfunction related to Chiari malformation was observed in 7 infants (3%): 2 improved by simple VPS whereas 5 required foramen magnum decompression (FMD). Overall neonatal mortality consisted of 5 cases (2%), whereas 226 patients (98%) were eventually discharged.

Fifteen patients were lost at follow-up, but a total of 211 patients (93.3%) remained in our outpatient program for patients with Spina Bifida. Follow-up ranged from 12 months to 27 years (mean 10.1 years). During this period, 107 patients (60% of 178 shunted patients) required re-operation for shunt malfunction; 16 (7.5% of 211 followed patients) experienced late brain stem dysfunction due to Chiari malformation, which could be managed by ventricular drainage in 8 cases whereas 8 patients required cranio-cervical decompression; 140 patients (66%) had magnetic resonance evidence of hydro-syringomyelia, but only 6 required surgical treatment; 43 patients (20.3%) presented signs or symptoms of tethered cord, but surgical untethering was considered indicated just in 24 (11.3%) patients.

During the follow-up, mortality consisted of 6 patients (2.8%) and was related to shunt malfunction (3 cases), general conditions (2 cases), prion spongiform encephalopathy (1 case). Severe adjunctive neurological morbidity was reported in 18 patients (8.5%): 12 cases were due to shunt malfunction; 5 patients with tethered cord and 1 with syringomyelia kept on worsening in spite of repeated surgeries. “Social” results have been retrospectively evaluated by extrapolating from the global series all patients beyond 15 years of age and with initial lesion below L2. There were 41 patients fulfilling these criteria: 33 (80.4%) were able to walk with orthoses, but useful community ambulation was reported just in 19 (46.3%); 40 (97.5%) were fully independent in daily activities, had achieved “social urinary continence”, and had attended at normal schools; 39 (95%) had completed both Primary and Secondary Schools, 32 (78%) had entered 3rd Level Schools. Presently, 19 have obtained the Advanced Level Certificate of Education, 11 have become university students, and 2 have graduated. A total of 17 patients have completed their individual Education Course: 11 have a job, 5 are unemployed but are looking for a job, and 1 is charged to parents and social services.

In conclusion, many MMC patients now reach adulthood and social continence and self care may be expected in a large percentage of cases. Nevertheless, there are so many associated neurological conditions which have to be faced that a coordinated network of care remains necessary throughout the life. Moreover, these patients often present so many peculiarities that indications for treatment, choice of proper techniques, and obtainable results are not always clear and evident.