gms | German Medical Science

62nd Annual Meeting of the German Society of Neurosurgery (DGNC)
Joint Meeting with the Polish Society of Neurosurgeons (PNCH)

German Society of Neurosurgery (DGNC)

7 - 11 May 2011, Hamburg

Open spina bifida – an analysis of 5 fatal courses of the disease

Meeting Abstract

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  • D. Class - Klinik für Neurochirurgie und Wirbelsäulenchirurgie, Universitätsklinikum Magdeburg, Magdeburg
  • A. Franke - Klinik für Neurochirurgie und Wirbelsäulenchirurgie, Universitätsklinikum Magdeburg, Magdeburg
  • R. Firsching - Klinik für Neurochirurgie und Wirbelsäulenchirurgie, Universitätsklinikum Magdeburg, Magdeburg

Deutsche Gesellschaft für Neurochirurgie. Polnische Gesellschaft für Neurochirurgen. 62. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), Joint Meeting mit der Polnischen Gesellschaft für Neurochirurgen (PNCH). Hamburg, 07.-11.05.2011. Düsseldorf: German Medical Science GMS Publishing House; 2011. DocDI.08.02

DOI: 10.3205/11dgnc158, URN: urn:nbn:de:0183-11dgnc1584

Published: April 28, 2011

© 2011 Class et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.en). You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.


Outline

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Objective: Due to the better medical care in the western world, more than 90% of the newborn children with open spina bifida now reach adulthood and the prognosis for long life-expectancy is basically good. Despite this tremendous progress, there are ongoing risks associated with this congenital malformation with potential morbidity and also mortality even in later life. We investigated the question of possible risk factors and the causes of death in 5 patients with a fatal course of the disease.

Methods: We reviewed a group of 70 patients with open spina bifida treated between 1974 and 2010 with special focus on the patients, who are known to have died. Before 1995, these patients was treated mainly by pediatric surgeons. Clinical data and follow-up information were collected from own medical reports and from those given to us by the parents of the patients.

Results: During a time period between 1984 and 2009, 5 patients out of a group of 70 patients seen regularly in the clinic for follow-up care died, 1 child was treated in another unit and the caring physicians and parents turned to our department for a second opinion. 4 patients had died in childhood already and one patient died later as an adult due to shunt failure. Causes of death were cardio-pulmonary failure in 2 patients and neurological complications including Chiari malformation and shunt failure in 3 patients. Survival time varied from 97 days up to 22 years.

Conclusions: Patients with open spina bifida carry a life-long lasting risk as to morbidity and mortality. Very little data exists about late and possibly life-threatening complications in adult life to be taken into account with planning the care of these patients. Close neurosurgical follow-up is mandatory including regular testing of hindbrain function, including a focus on central sleep apnea and surveillance of hydrocephalus drainage in the first place.