Friday, March 28, 2008

Spina Bifida Children and Their Parents: A Population Prone to Family Dysfunction?

Spaulding BR, Morgan SB (1986) Spina Bifida Children and Their Parents: A Population Prone to Family Dysfunction? Journal of Pediatric Psychology 11(3) pp. 359-374, 1986

Spina bifida children of normal intelligence and their parents were compared with a carefully matched control group of nondisabled children and their parents in a hierarchically oriented assessment of individual, marital, and family functioning. Contrary to popular hypotheses that families with a handicapped child are especially vulnerable to stress and psychological problems, results across all measures revealed no differences between the two groups. Further, no differences were found between mothers and fathers on the various measures. These results support the view that nonretarded spina bifida children and their parents have no special psychological characteristics that distinguish them from nonhandicapped children and their parents. The findings also underscore the strengths and coping skills of handicapped children and their families. The tendency of researchers and clinicians to focus on the deviant aspects of these populations may obscure the fact that many may be adapting successfully.

Monday, March 17, 2008

Complications with shunts in adults with spina bifida

Tomlinson P, Sugarman ID. Complications with shunts in adults with spina bifida. British Medical Journal 1995 Jul 29;311(7000):286-7.

OBJECTIVE--To assess the incidence of malfunction of shunts in adults with spina bifida who have shunts to control hydrocephalus.

DESIGN--A retrospective review of the medical notes and contact by questionnaire of adults with spina bifida to assess symptoms, function of shunts, frequency of operative procedures, and follow-up.

SUBJECTS--110 patients with shunts who attended Lord Mayor Treloar College for the physically disabled between 1978 and 1993.

RESULTS--The average (range) number of revisions of shunts per person was 3.6 (0-28). Although 37 patients underwent an emergency operation for revision in their first year of life, there was a continuing low incidence, increasing in the early teenage years, which persisted into the third decade. Intervals between emergency revisions varied: 202/320 occurred within one year of the last shunt operation, 56 occurred after five years, 24 after 10 years, and 15 after 15 or more years. Fifteen patients had chronic intermittent headaches, of whom four died and three suffered severe morbidity. Thirteen died; three had raised intracranial pressure, and four died suddenly; these deaths were presumed to be related to their shunts. Up to the age of 16 there was 100% hospital follow up, but after that only 40% of young adults underwent review, including review of their shunt function.

CONCLUSION--Shunts to control hydrocephalus may fail after many years without symptoms. This is difficult to diagnose and if missed may lead to chronic morbidity and death. As hospital follow up of this group is falling, both general practitioners and hospital doctors must be aware that a shunt may malfunction after prolonged quiescent periods.

PMID: 7633231

Chronic headaches in adults with spina bifida and associated hydrocephalus

Edwards RJ, Witchell C, Pople IK. Chronic headaches in adults with spina bifida and associated hydrocephalus. European Journal of Pediatric Surgery. 2003 Dec;13 Suppl 1:S13-7.

INTRODUCTION: Adults with spina bifida and associated hydrocephalus are exposed to multiple risk factors for the development of chronic headache. The management of these patients can be complex and misdiagnosis can precipitate unnecessary shunt revision. This study aims to evaluate the usefulness of intracranial pressure (ICP) monitoring as a diagnostic tool in these cases and to look at the causes of chronic headaches and treatment outcomes for this patient population.

METHODS: All patients over the age of 18 years with a diagnosis of spina bifida and shunted hydrocephalus who had undergone inpatient or outpatient neurosurgical review within the last 10 years were identified in our hospital database. Case notes were then retrospectively reviewed to identify all patients who had undergone either inpatient or outpatient evaluation of chronic headaches (defined as a headache of at least one month's duration) occurring in the absence of any other symptoms or signs suggestive of raised intracranial pressure (ICP). The incidence, causes, management and outcome of chronic headache in these patients was determined.

RESULTS: 42 patients were identified, mean age 30 years (range 18 - 59). All had undergone lifelong follow-up. All had previously undergone shunt insertion for hydrocephalus. 16 had undergone endoscopic third ventriculostomy (ETV). 11 had undergone choroid plexus coagulation. 55 % (23/42) of patients underwent investigation for 1 or more episodes of chronic headache. Recurrent hydrocephalus due to shunt malfunction or ETV failure was excluded by ICP monitoring using either an intraparenchymal transducer or monitoring via a ventricular access device. All patients underwent repeat imaging, using CT and/or MR imaging. Identified causes of headache included: shunt blockage; shunt overdrainage; ETV failure and symptomatic Arnold-Chiari malformation. A history of choroid plexus coagulation (CPC) as an infant was associated with a decreased risk of chronic headache in later life (p = 0.02). In 8 patients no definite cause for headaches was identified, in 4 of these patients symptoms resolved spontaneously, the remainder required specialist pain management.

CONCLUSIONS: The aetiology of chronic headaches in this patient group is multifactorial. In the absence of other clinical symptoms or signs of raised ICP, ICP monitoring is an invaluable adjunct to management. 10 % of hydrocephalic adult spina bifida patients required specialist pain management for control of chronic idiopathic headache.

PMID: 14758561

Issues of medical management in adults with spina bifida

Related Articles, LinksMcDonnell GV, McCann JP. Issues of medical management in adults with spina bifida. Child's Nervous System. 2000 Apr;16(4):222-7.

The objective of this study was to establish the range of medical issues among those attending a clinic for adults with spina bifida (SB) and hydrocephalus (CASBAH). Owing to improvements in medical care in the past 3-4 decades, an increasing number of SB patients go on to prosper in adult life. Since 1990 there has been a CASBAH service in Belfast serving Northern Ireland on a regional basis. There are now 237 patients with SB remaining on the live register at CASBAH. All records were reviewed with regard to site of lesion, ambulatory ability, shunt placement and regularity of attendance. The records of 193 were also reviewed with reference to musculoskeletal problems, bladder function, bowel function, renal impairment and hypertension, skin breakdown, epilepsy and the incidence of clinically significant Chiari/hydrosyringomyelia. Of the patients on the register, 36% are wheelchair dependent, 8% have some ambulatory capacity but are largely wheelchair dependent, 22% are ambulatory with aid and 34% are independently ambulatory. Bladder function is normal in 8%, whilst 32% describe normal bowel function. Renal impairment is present in 48% of patients, and 15% are on anti-hypertensive therapy. Epilepsy is an active issue in 9%, and intraventricular shunts are in situ in 37% of patients. Scoliosis is present in 50% and 70% have joint deformities or contractures. Five patients have become symptomatic from the Chiari/hydrosyringomyelia complex. These data reflect the considerable range of disability in adult SB patients, the challenges presented in long-term management and the need for organised follow-up.

PMID: 10855520

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