Monday, March 17, 2008

Why do adults with spina bifida and hydrocephalus die?

McDonnell GV, McCann JP. Why do adults with spina bifida and hydrocephalus die? A clinic-based study. European Journal of Pediatric Surgery. 2000 Dec;10 Suppl 1:31-2.

AIM: To establish the death rate and factors contributing to death among attenders at a clinic for adults with spina bifida and hydrocephalus (CASBAH).

INTRODUCTION: Due to improvements in medical care in the past 40 years, increasing numbers of spina bifida patients go on to prosper in adult life. Despite this, levels of morbidity are high and services for such patients are uneven and unpredictable.

MATERIALS AND METHODS: Since 1990 there has been a CASBAH service in Belfast operating on a regional basis. Led by a consultant in rehabilitation medicine, it provides a multidisciplinary approach to patient care. Between 1990 and 1999 there have been 280 referrals to the clinic. Charts were reviewed regarding current health status and mode of death where relevant.

RESULTS: There have been 18 deaths during the term of the clinic (6.4%). There were four deaths due to renal failure and two due to cardiac failure. Two deaths occurred following surgery for a Chiari/hydrosyringomyelia complex and one patient died due to shunt malfunction. Three deaths were presumed related to infection. Two deaths were attributable to malignancy (carcinoma of a renal conduit and transitional cell carcinoma of the kidney) and other terminal events in one patient each were: basilar artery aneurysm rupture, post-partum pulmonary embolism, peptic ulcer disease, myocardial infarction and status epilepticus.

CONCLUSIONS: Although certain deaths were unpredictable during the period of review and not directly attributable to spina bifida, the majority reflect many of the related secondary health issues of the condition and indicate the need for organised, medically co-ordinated follow-up of adult patients.

PMID: 11214829

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