Gillian M Hunt

Open spina bifida: outcome for a complete cohort treated unselectively and followed into adulthood.

Between 1963 and 1970, 117 consecutive cases of open spina bifida were treated unselectively from birth, following a full neurological examination. The author reviews the outcome 16 to 20 years later. 48 died before their sixteenth birthday. Of the 69 survivors, 60 had had a shunt inserted and two were blind following shunt dysfunction; 22 were mentally retarded (IQ<80); 35 were wheelchair‐dependent; 52 were incontinent, and 32 of these continued to need help with its management; nine weighed >75kg; one‐third of both sexes had a precocious puberty; three had insulin‐dependent diabetes; 12 continued to need anticonvulsant drugs; 32 had suffered from pressure sores; 33 were unable to live without help or supervision and only 17 were capable of open employment. These findings demonstrate the wide range of disabilities implied by the diagnosis of open spina bifida. The neurological findings recorded at birth were reflected in the mortality and disability of the survivors. The sensory level represented a yardstick for predicting the likely range of handicap into adult life, provided no additional impairment resulted from serious complications: such damage occurred in seven of the survivors.

Open spina bifida: a complete cohort reviewed 25 years after closure.

This study summarises the long‐term results of non‐selective treatment of open spina bifida. Every member of a consecutive series of 117 cases was ascertained 22 to 28 years after closure of the back. 56 of the cohort had died. The condition of the 61 survivors ranged from normal to severe disability. Only 33 of the survivors were capable of living independently, 11 required supervision and some help, and 17 needed daily care which was generally provided by the parents. The two main determinants of disability and dependency were the extent of the neurological deficit and IQ. Improvements in the management of patients with open spina bifida have greatly reduced mortality, but they are less likely to influence long‐term disability since that is dependent on the severity of the neurological deficit.

Link between the CSF shunt and achievement in adults with spina bifida

OBJECTIVES A few enterprising adults with shunt treated spina bifida live independently in the community, have a job in competitive employment, and drive to work in their own car. By contrast others with similar disability but lacking their motivation remain dependent on care and supervision. The aim of this study was to identify events in the history of their shunt which may have influenced their subsequent achievement. METHODS Between June 1963 and January 1971 117 babies born in East Anglia with open spina bifida had their backs closed regardless of the severity of their condition. When reviewed in 1997 every case was ascertained. Sixty had died and the 57 survivors had a mean age of 30. These were assigned to two groups: achievers and non-achievers, according to their attainments in independence, employment, and use of a car. RESULTS Of the 57 survivors nine had no shunt and eight of these were achievers. All were of normal intelligence (IQ⩾80) and only one was severely disabled. Of the 48 with shunts only 20 were achievers (OR 11.2, 95% confidence interval (95% CI) 1.3–96.8). Lack of achievement in these 48 was associated with revisions of the shunt, particularly when revisions were performed after the age of 2. Sixteen patients had never required a revision and 11 (69%) were achievers; 10 had had revisions only during infancy and five (50%) were achievers; 22 had had revisions after their second birthday and only four (18%) were achievers (p<0.001). Elective revisions were not performed in this cohort and in 75% of patients revisions had been preceded by clear symptoms of raised intracranial pressure. CONCLUSION Revisions of the shunt, particularly after the age of 2, are associated with poor long term achievement in adults with spina bifida.

Expectation of life and unexpected death in open spina bifida: a 40‐year complete, non‐selective, longitudinal cohort study

Aim  The aim of our study was to investigate survival and causes of death in a complete cohort of open spina bifida at the mean age of 40 years.

Some Factors Relating to Intelligence in Treated Children with Spina Bifida Cystica

The 83 survivors of a consecutive series of children with spina bifida cystica, born between 1963 and 1971 and treated non‐selectively since birth, were assessed by intelligence and developmental testing. In nine of the children who had not required shunts the distribution of intelligence was within the normal range. The need for a shunt was significantly related to the presence of craniolacunae and to the sensory level of the lesion recorded at birth. Seven of the 74 shunt‐treated children had suffered ventriculitis, meningitis or septicaemia, and their intelligence was significantly worse than the others. In the 67 shunt‐treated children who had not suffered infection, intelligence was significantly related to the thickness of the pallium when the shunt was inserted during the first four weeks of life, and to the sensory level of the lesion recorded at birth. Intelligence was not related to the function of the shunt at time of assessment, to the number of revisions of the shunt, or to the rate of increase in head size during the first four weeks of life. It is concluded that the best indication of later intelligence can be gained at birth from the thickness of the pallium and the sensory level of the lesion.

Spina bifida: implications for 100 children at school.

One hundred children with spina bifida were examined at home and reviewed at the schools they attended.

THE PATTERN OF CONGENITAL RENAL ANOMALIES ASSOCIATED WITH NEURAL-TUBE DEFECTS

This is a retrospective study of 190 patients with neural‐tube defects for whom there were precise data, both on sensory levels (to pin‐prick) and on the presence or absence of congenital renal anomalies. There were 17 renal anomalies, of which three were renal agenesis, five horseshoe kidneys, eight ureteral duplications and one simple ureterocele. There was a consistent pattern of congenital renal anomalies in relation to the extent of the neurological lesion, as measured by the sensory level. Renal agenesis was associated with a sensory level in the dermatomes T5 to T8, horseshoe kidneys with a T9 to LI level and ureteral duplications predominantly with a sensory level in the sacral dermatomes.

Intermittent catheterisation: simple, safe, and effective but underused.

Twenty five years ago Lapides and coworkers showed that intermittent catheterisation was a safe and effective way of managing patients with urinary retention or incontinence due to neuropathic or hypotonic bladder.1 2 This technique has transformed the lives of people rendered housebound by bladder problems and has preserved the kidneys of children with spina bifida3 4 5 6 7 8 9 10 and of adults with spinal cord injury.11 12 13 14 15 Intermittent catheterisation is a clean but not sterile technique. It entails inserting a catheter into the bladder, emptying out all the urine, and then removing the catheter. This is performed several times a day by the patient or carer. It puts the patient in control of emptying the bladder and safeguards the kidneys by providing drainage. #### Summary points Incomplete emptying of bladder—A bladder that retains a large volume of residual urine soon fills to capacity, causing frequency, urgency, nocturia, or incontinence. Urinary stasis leads to urinary tract infections and calculi. By eliminating residual urine, intermittent catheterisation enables the bladder to function more effectively as a reservoir, relieves symptoms, and reduces the incidence of symptomatic urinary infections.3 9 16 22 25 28 Obstructive uropathy—Many patients with a neuropathic bladder have an overactive sphincter, causing obstruction of the bladder outlet and dilatation of the upper urinary tracts. A combination of a contracting urethral sphincter with hyperreflexic bladder contractions (detrusor-sphincter dyssynergia) accelerates the damage. If the obstruction of the bladder outlet is overcome by intermittent catheterisation then dilatation of the upper urinary tracts is reduced and renal function improved.6 22 Reflux nephropathy—Vesicoureteric reflux associated with neuropathic bladder often causes renal damage. The risk is increased by obstruction of the bladder outlet, high intravesical pressure, or urinary infection. Intermittent catheterisation can reduce or eliminate …

Open spina bifida: birth findings predict long-term outcome

Objectives To investigate if lifestyle in spina bifida at age 40±3 years, relates to neurological deficit in infancy or cerebrospinal fluid shunt history. Design Prospective cohort study with 100% ascertainment. Setting Community. Participants 117 consecutive cases of open spina bifida whose backs were closed non-selectively at birth. In 2007, all 46 (39%) survivors and/or carers were surveyed by postal questionnaires and telephone interviews. Results Of the 38 children with absent sensation only below the knee (sensory level below L3), 23 (61%) survived of whom 14 (61%) were community walkers and only 5 (22%) needed daily care. But in 42 babies with absent sensation up to the umbilicus (sensory level above T11) only seven (17%) survived, none could walk and five (71%) needed daily care. Survivors with no shunt revisions were more likely to walk, live independently and drive a car. Conclusion Mobility and the need for care at 40 can be predicted from the neurological deficit.

Intermittent catheterisation in the management of children with neuropathic bladder.

Twenty-three children with neuropathic bladder have been treated by intermittent catheterisation during the past 3 years. Twelve are now practically dry, nine are drier than before, and two have remained wet. Urinary infections have not been a major problem, but it is too soon to assess the long-term effects on the upper renal tract. Intermittent catheterisation lightens the burden for the mothers. Four children catheterise themselves and two of them are now independent.