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1.  Some sources of stress found in mothers of spina bifida children. 
The scores on a malaise inventory for 51 mothers of spina bifida children born between 1956 and 1962 in South Wales revealed significantly higher scores when the child was incontinent, had a severe locomotor disability, had an IQ less than 80, and was attending a special school rather than a normal school. No difference in scores was found between the sexes or for size of family, and only a slight difference occurred between the malaise scores of mothers of mildly and moderately handicapped children.
PMCID: PMC478883  PMID: 124188
2.  Natural History of Spina Bifida Cystica and Cranium Bifidum Cysticum 
Archives of Disease in Childhood  1971;46(246):127-138.
The 425 cases of spina bifida cystica and cranium bifidum cysticum born in a population of 850,000 between 1956 and 1962 (with an incidence of 4·12 per 1000 births) were followed. Most did not receive the modern treatment for the condition. Follow-up in 1968 showed that 25% were stillborn, 13% died during the first week of perinatal causes, a further 47% died mostly of the complications of the condition, and 15% are still alive. Life table survival figures suggest for the liveborns a 12·8% life expectancy to the 11th birthday. The series included 18 cases of meningocele (4·2% of the total), diagnosed largely by exclusion; all are surviving and are largely free from physical and mental handicap. 26 cases of encephalocele include 8 survivors who are all retarded but not much crippled. Of the 381 myeloceles, 37 survive who though not much affected mentally are severely handicapped physically with limb paralysis and incontinence, the pattern of which was largely determined by the level of the lesion. There is a close relation between degree of hydrocephalus and severity of mental retardation. Boys surviving show better performance than girls. The incontinent, whether treated or untreated, show no more emotional disturbance than the continent.
This series provides a `baseline' allowing modern and surgical treatment to be evaluated. 60% of the total (or 85% of those liveborn) should be regarded as potentially salvageable. Nearly all cases should probably have the `open' lesion closed as a surgical emergency, but thereafter no further procedure should be undertaken on those with severe hydrocephalus, established renal damage, persistent intracranial infection, or severe kyphosis, or if other severe malformations are present.
PMCID: PMC1647472  PMID: 4930541
3.  Handicap and social status of adults with spina bifida cystica 
The present physical handicaps and social status of 202 survivors of spina bifida cystica attending The Hospital for Sick Children, Great Ormond Street before 1954 are described. Ninety-three of the patients, mostly with uncomplicated meningocele, had escaped serious disability and were living normal lives. But 109 had a serious disability, including three who are mentally retarded.
Some of the difficulties of living with spina bifida are described, and a record of work, marriage, and reproduction in the whole group of survivors is shown. Of the 106 with serious locomotor problems and/or incontinence, 58 work regularly, 17 have long spells off work, and 31 have never worked at all; 24 have married, 16 have 26 children none of whom has spina bifida. Of the 96 without physical handicap, 93 work regularly, 47 are married, and 34 have 75 children one of whom has spina bifida cystica and one anencephaly.
PMCID: PMC478843  PMID: 4604518
4.  Health care of physically handicapped young adults 
Little is known about the health needs of physically handicapped young adults after they become too old for the paediatric service. To assess these needs 104 young people with physical handicaps were given a medical examination and interviewed. They had diverse physical handicaps, many of which were rare (41 subjects); 45 had cerebral palsy and 18 spina bifida. Mental handicap (42 subjects) and a need for special education (28) were also diagnosed. The state of health of all subjects was generally poor. Sixty one subjects had contractures of the lower joints and almost a quarter contractures of the upper joints; 26 had deformed feet. Consequently, many subjects (41) could not walk; only 13 could walk without difficulty. Urinary and bowel incontinence were prevalent (58 and 55 subjects, respectively) and with contractures and pressure sores resulted in skin problems (35 subjects). Kyphoscoliosis was present in 30 subjects, particularly those with spina bifida (10 out of 18), of whom one third had respiratory problems and almost half cardiac problems. Thirty one subjects had epilepsy. Many subjects (49) had difficulty communicating or some visual defect (27).
Although over half the subjects had health problems that were severe enough to warrant intervention, less than a third were receiving any form of regular hospital care; regular contact with physiotherapists, speech therapists, and dentists was also poor. Moreover, the original diagnoses and prescriptions had not been reviewed regularly. Teams for handicapped adults should be set up in all district health authorities to provide a clinical and coordination service for all adults with physical and mental handicaps.
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PMCID: PMC2545621  PMID: 2967728
5.  Survival and handicap of infants with spina bifida. 
Archives of Disease in Childhood  1980;55(11):845-850.
A follow-up study was carried out on 213 infants born with spina bifida cystica (including encephalocele and occipital meningocele) from 1965 to 1972 to women resident in Oxfordshire and the western part of Berkshire. The 5-year survival rate was 36% (39/107) for those with open lesions, 60% (30/50) for those with closed ones, and 18% (10/56) for those with lesions which could not be classified (not known) but which were probably nearly all open. The extent of handicap among these survivors was assessed by means of criteria described by Lorber; among those with open lesions (including 'not known') 84% (41/49) were severely handicapped, 10% (4/49) were moderately handicapped, and only 6% (3/49) had no handicap; among those with closed lesions, 37% (11/30) were severely handicapped, 33% (10/30) were moderately handicapped, and the remaining 30% (9/30) were not handicapped. Closed head lesions (encephalocele or occipital meningocele) were more often associated with severe handicap (6/8; 75%) than were closed spinal lesions (5/22; 23%). The children with open lesions who survived for at least 5 years spent, on average, at least 6 months in hospital during the first 5 years of their life and had, on average, at least 6 major surgical operations. In comparison, those with closed lesions spent one-third less time in hospital, and had fewer than half as many operations. During the period of the study a selective treatment policy was adopted typical of that commonly practised now, and all the infants were born before antenatal screening had been introduced. Our results therefore may be helpful in assessing the benefits to be expected from antenatal screening for open spina bifida.
PMCID: PMC1626957  PMID: 7436454
6.  Spina Bifida Children Attending Ordinary Schools 
British Medical Journal  1975;1(5950):142-145.
From September 1971 to September 1973 a policy was actively followed in South Gloucestershire of placing spina bifida children in ordinary schools wherever possible. This was achieved successfully in 14 cases out of a possible 24. Special attention was paid to the selection of children and schools. The main contraindications to attendance at ordinary school were: below average intelligence, special perceptual problems, the need for intensive physiotherapy, and problem incontinence—particularly infaecal continence. Success often depended on special provisions—for example, transport, aids to mobility, peripatetic physiotherapists. Personal attendants (welfare assistants) could be allocated to each of the more severely handicapped children. The lack of facilities for handicapped pupils in comprehensive schools is a matter needing urgent review.
PMCID: PMC1671998  PMID: 1089442
7.  Adult Consequences of Spina Bifida: A Cohort Study 
Background
Patients with spina bifida potentially experience social isolation with limited employment opportunities and restriction of independent living, neurologic and intellectual impairment, and orthopaedic and urologic consequences of their condition. However, the degree of disability as these individuals age into adulthood has not been completely delineated.
Questions/purpose
In adults with spina bifida we determined the cause of death for those who had died, IQ, level of education, work history, independent living status, physical disabilities, and urologic status; we then identified surgical procedures that led to better adult function.
Methods
We retrospectively reviewed 84 individuals with myelomeningocele, examining social, cognitive, and physical disabilities. The minimum age at followup was 20 years (mean, 31 years; range, 20–64 years).
Results
Forty-two percent had normal IQs. Seventy percent never needed a neurosurgical shunt and those without shunts had higher IQs. Forty-four percent had regular education and 8% achieved college degrees. Fifty-six percent were unemployed. Thirty percent lived independently. Twenty-three percent were either married or divorced with nine normal offspring. Eighty-five percent dressed themselves, 65% shopped independently, 54% drove. Thirty-one percent were at the thoracic neurologic level (all used wheelchairs), 12% were at L1–L3 (all used a wheelchair except one), 33% were at L4–L5 (78% used a wheelchair at least part-time), and 24% were at S1 and below (all walked). Fifty-four percent experienced decubiti and as a consequence, four required major extremity amputations. Spinal fusions protected sitting balance, but hip surgery did not produce congruent hips and occasionally resulted in debilitating stiffness. Pressure sores resulted in partial foot amputations despite plantigrade feet.
Conclusions
Our observations confirm the consequences associated with the physical and cognitive disabilities of patients with spina bifida. Many of these consequences can likely be mitigated by modern multidisciplinary care and special education to increase independence.
Level of Evidence
Level IV, observational study. See Guidelines for Authors for a complete description of levels of evidence.
doi:10.1007/s11999-010-1594-z
PMCID: PMC3069297  PMID: 20878278
8.  Neuropsychological profile of young adults with spina bifida with or without hydrocephalus 
Objectives: To determine the relative impact of hydrocephalus and spinal dysraphism in young adults on intellectual and cognitive functioning. Sub-groups of patients with congenital hydrocephalus and/or spina bifida were assessed between 1995 and 2003. The entry criteria were that individuals should have (i) intact global function, (ii) average verbal intelligence (or above), and (iii) should not have clinical depression. There were three sub-groups: patients with hydrocephalus and spina bifida, patients with hydrocephalus without spina bifida, and patients with spina bifida without hydrocephalus.
Methods: Patients were neuropsychologically assessed as part of their normal clinical assessment during their annual medical review. Each individual completed a screening battery assessing global functioning, verbal intelligence, and mood. In addition they completed additional tests including measures of emotional intelligence, memory, attention, and executive function. Results were analysed to compare the performance of the patient sub-groups and to compare them to a healthy control group.
Results: Patients with hydrocephalus (with or without spina bifida) were significantly impaired on the vast majority of all test scores as compared to patients with spina bifida and healthy controls. They were particularly poor on measures assessing executive function. By contrast for patients with spina bifida with no associated hydrocephalus, the significant majority of all test scores fell within the average range or above.
Conclusions: The neuropsychological profile of patients with hydrocephalus is one of relative impairment and this is so whether or not spina bifida is present. In spina bifida alone, in the absence of hydrocephalus, cognitive function is relatively spared.
doi:10.1136/jnnp.2003.029058
PMCID: PMC1739163  PMID: 15258211
9.  Adolescents with spina bifida. How they see their situation. 
Archives of Disease in Childhood  1976;51(6):439-444.
Forty-six adolescents with spina bifida were interviewed at home to find out how they felt about their situation. Some degree of misery was very common indeed but was more likely to be severe in girls than in boys. About half the girls had, on some occasion in the past year, felt that life was hopeless or not worth living. Since these feelings are related to their social isolation outside school, they might be alleviated by improved opportunities for contract with peers. This seems particularly important in view of the capacity of most teenagers to make reasonable relationships where the opportunity exists, e.g. at school or within the family. Over half those who had left school were either unemployed or very dissatisfied with their job. In addition to worries about work, preoccupations about sexual relatinships, sexual function, and marriage were common. About two-thirds of the teenagers hoped to get married and half of these thought they could have children. Girls were particularly worried about their capacity to conceive and boys had understandable concerns about potency. Very few had consulted anyone about this or had had advice about the genetic aspects of the condition and there is a clear need for adequate counseling to help such teenagers to distinguish between real and imagined fears about the consequences of their condition.
PMCID: PMC1546008  PMID: 782378
10.  Maternal occupational exposure during pregnancy and the risk of spina bifida. 
OBJECTIVES: A case-control study was carried out to explore associations between spina bifida and occupational exposure of the mother. METHODS: The cases were children with spina bifida aperta born between 1980 and 1992 from nine hospitals in the Netherlands. The controls were children born healthy in the same period as the cases, from hospitals and from the general population. Data collection was carried out in two steps. Firstly, postal questionnaires were sent to all the parents of cases and controls to gather information on occupations and potential confounders. In the second phase of the study, information on specific exposures was collected by means of job and task specific personal interviews. Interviews were performed with 55 case mothers and 66 control mothers who had occupations with a potential for chemical or physical exposure. Those exposures were assumed to be negligible for--for example, teachers and secretaries, so personal interviews were not indicated for these women. Information was collected on specific tasks in the period just after conception, and on the associated use of chemical or physical agents, frequency of exposure, and use of protective equipment. RESULTS: The analyses of occupation showed an increased risk for women working in agricultural occupations (OR = 3.4, CI:1.3-9.0), and, although less distinct, for cleaning women (OR = 1.7, CI:0.9-3.4). Only a few women seemed to be occupationally exposed to chemical or physical agents. No differences in occurrence of specific exposures could be detected between cases and controls. Besides, no differences were seen in pesticide or disinfectant exposure among case and control mothers in agricultural occupations. CONCLUSIONS: Occupational exposures of the mother during pregnancy were infrequent and did not seem to play an important part in the aetiology of spina bifida in this study. The association found between spina bifida and maternal agricultural occupations could not be explained by the use of pesticides by the mother or by any other occupational exposure.
PMCID: PMC1128418  PMID: 8777455
11.  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.


PMCID: PMC1736621  PMID: 10519863
12.  Treatment of the neurogenic bladder in spina bifida 
Renal damage and renal failure are among the most severe complications of spina bifida. Over the past decades, a comprehensive treatment strategy has been applied that results in minimal renal scaring. In addition, the majority of patients can be dry for urine by the time they go to primary school. To obtain such results, it is mandatory to treat detrusor overactivity from birth onward, as upper urinary tract changes predominantly start in the first months of life. This means that new patients with spina bifida should be treated from birth by clean intermittent catheterization and pharmacological suppression of detrusor overactivity. Urinary tract infections, when present, need aggressive treatment, and in many patients, permanent prophylaxis is indicated. Later in life, therapy can be tailored to urodynamic findings. Children with paralyzed pelvic floor and hence urinary incontinence are routinely offered surgery around the age of 5 years to become dry. Rectus abdominis sling suspension of the bladder neck is the first-choice procedure, with good to excellent results in both male and female patients. In children with detrusor hyperactivity, detrusorectomy can be performed as an alternative for ileocystoplasty provided there is adequate bladder capacity. Wheelchair-bound patients can manage their bladder more easily with a continent catheterizable stoma on top of the bladder. This stoma provides them extra privacy and diminishes parental burden. Bowel management is done by retrograde or antegrade enema therapy. Concerning sexuality, special attention is needed to address expectations of adolescent patients. Sensibility of the glans penis can be restored by surgery in the majority of patients.
Electronic supplementary material
The online version of this article (doi:10.1007/s00467-008-0780-7) contains supplementary material, which is available to authorized users.
doi:10.1007/s00467-008-0780-7
PMCID: PMC2335291  PMID: 18350321
Spina bifida; Surgical treatment; Incontinence; Renal function; Bladder augmentation; Bladder-neck sling; Dryness; Antimuscarinic
13.  Results of selective treatment of spina bifida cystica. 
Archives of Disease in Childhood  1981;56(11):822-830.
The results of selective treatment in 120 infants with open spina bifida, admitted between May 1971 and December 1976, were prospectively studied. Seventy-one infants had adverse criteria at birth and were not treated. They all died, more than 90% of them within 6 months of birth. Seven had meningocele. All were treated and survived without handicap. Forty-two infants with myelomeningocele were actively treated. Thirty-six survive at follow-up after 3 to 9 years. The quality of survival is much better than when selection was not used but 8 children have moderate or severe handicaps. The parents were fully informed and consulted at every decision-making step; they fully supported the principle of selection and the action taken on behalf of their own child.
PMCID: PMC1627397  PMID: 6458248
14.  Complications with shunts in adults with spina bifida. 
BMJ : British Medical Journal  1995;311(7000):286-287.
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.
PMCID: PMC2550354  PMID: 7633231
15.  Perineal sensation: an important predictor of long‐term outcome in open spina bifida 
Objectives
To see if perineal sensation in infants with open spina bifida is associated with a better long‐term outcome, particularly in terms of survival, renal‐related deaths and incontinence.
Methods
We conducted a prospective cohort study on a complete cohort of 117 consecutive patients with open spina bifida, whose backs were closed non‐selectively at birth between 1963 and 1971. A meticulous neurological examination in infancy showed that 33 (28%) of them had perineal sensation, defined as intact sensation to pinprick in at least one dermatome on one side in the saddle area (S2–4). Data recorded within 48 h of birth and during six reviews between 1972 and 2002 were used. Details of deaths were obtained from medical records and from the Office of National Statistics.
Results
By December 2005, 57% (67/117) of the cohort had died. There were 50 survivors with a mean age 38 years (range 35–41). More of those with perineal sensation survived than those without (23/33 v 27/84, p<0.001). This difference was mainly caused by 19 renal deaths in those lacking perineal sensation. Crucially there were no renal‐related deaths in those with perineal sensation (0/33 v 19/84, p = 0.003). Among the survivors, those with perineal sensation were more likely than the remainder to be continent of urine and faeces (10/23 v 1/27, p<0.001 and 18/23 v 9/27, p = 0.002 respectively). They were also more likely to be able to walk at least 50 m (11/23 v 5/27, p = 0.027) and never to have had pressure sores (15/23 v 9/27, p = 0.025).
Conclusions
A simple assessment of perineal sensation in infancy predicts long‐term outcome in terms of survival, renal prognosis and incontinence in open spina bifida.
doi:10.1136/adc.2006.102079
PMCID: PMC2083152  PMID: 16943260
16.  Marjolin's ulcers: theories, prognostic factors and their peculiarities in spina bifida patients 
Background
Due to improved care, more and more children born with spina bifida in rural Kenya are surviving into adulthood. This improved survival has led to significant challenges in their lifestyles, especially the need to ensure pressure ulcer prevention and treatment. Malignant degeneration of pressure ulcers in spina bifida patients is very rare. The author describes the clinical presentation of two pressure ulcer carcinomas that are at variance from classical descriptions.
Materials and methods
An internet/Medline/PubMed search of English literature for theories on Marjolin's ulcer evolution and prognostic features of Marjolin's ulcers was performed.
A chart review of two young adults with spina bifida who had presented to the author's hospital between 2004 and August 2010 with chronic pressure ulcers found to be Marjolin's ulcers on histo-pathological examination was performed, and the clinical features are reported.
Results
The two ulcers appeared clinically benign: one was a deep ulcer, while the other was shallow; both had normal, benign-appearing edges, and a foul smelling discharge. The two ulcers were surrounded by induration and multiple communicating sinuses, with no evidence of chronic osteomyelitis. The internet search revealed a total of nine theories on Marjolin's ulcer development, as well as seven clinical and four histological prognostic features.
Discussion
The multifactorial theory, a coalescence of a number of proposed theories, best explains the evolution of Marjolin's ulcers. Poor prognostic features include pressure ulcer carcinomas, lesions and location in the lower limbs/trunks, all present in the two patients making their prognosis dim: this is despite the surgical margins being clear of tumor. Benign appearance, induration and presence of multiple communicating sinuses are features that have not been previously described as presenting features of pressure ulcers carcinomas.
Conclusion
There is need for spina bifida patients and their guardians/caretakers to receive a close follow-up throughout life; health education focused on pressure ulcer prevention as well as early treatment of pressure ulcers when they occur, will avert the development of Marjolin's ulcers, and save lives.
doi:10.1186/1477-7819-8-108
PMCID: PMC3014936  PMID: 21129225
17.  Impact of functional severity on self concept in young people with spina bifida. 
This study examines the relationship between medical and functional severity of disability and levels of self esteem and self concept in 79 young people with spina bifida. Greater feelings of global self worth and of self esteem in physical appearance were associated with greater severity of disability. This was only in part an effect of lower IQ among the most disabled young people. Many of the least disabled had marked impairment of self esteem. Analysis of the impact of individual aspects of disability confirmed the association between increased self esteem in physical appearance and global self worth, and diminished functional ability. Academic self ratings, however, were higher in the less disabled. Hydrocephalus and continence appeared to have minimal effect on self esteem. The relationship between severity of disability in spina bifida and self concept is complex and mediated by a range of factors. It is incorrect to assume that the psychological impact is less in the mildly disabled young person.
PMCID: PMC1511168  PMID: 7639550
18.  Arnold‐Chiari‐II malformation and cognitive functioning in spina bifida 
Spina bifida is a multifaceted neurological condition with complex neuropsychological sequelae. The cognitive outcome in spina bifida has frequently been attributed to the severity of the hydrocephalus. However, because of complex neuropathology, the influence of hydrocephalus alone does not sufficiently explain the deficits in the cognitive profile in spina bifida. To date, little is known of the role of Arnold‐Chiari‐II malformation (ACM) in the cognitive profile of these patients. Aim of the current study is to delineate the specific contribution of the ACM in spina bifida by comparing children with ACM and those without ACM. 46 children between 6 and 15 years of age underwent a neuropsychological assessment covering intelligence and a wide range of cognitive functions, such as visuo‐motor processing, attention, memory, word fluency and speed of information processing. Comparisons were made between patients with ACM (ACM+) and those without ACM (ACM−); all children with ACM+ also had hydrocephalus. Confounding effects of global cognitive impairment were excluded, such that groups were matched on verbal IQ. Because of complex neuropathology, which is inherent to spina bifida, the method applied was based on a comparison of cognitive profiles of the study group with profiles of patients with cerebellar damage and hydrocephalus found in the literature. Impaired visual analysis and synthesis, verbal memory, and verbal fluency, even after correction for global cognitive impairment, were observed in children with ACM. The hypothesis that in addition to impairment in visual analysis and synthesis, which are related to both hydrocephalus and ACM, specific deficiencies in verbal memory and fluency may be attributed to ACM is supported.
doi:10.1136/jnnp.2005.075887
PMCID: PMC2077736  PMID: 16690692
19.  Long-term outcome in open spina bifida. 
BACKGROUND: Doctors need reliable data on outcome in order to help parents faced with difficult decisions about termination of an affected pregnancy or treatment after birth. AIM: To determine survival, health and lifestyle at the mean age of 30 years in a complete cohort of adults born with open spina bifida. DESIGN OF STUDY: Prospective cohort study. PARTICIPANTS: Well-documented cohort of 117 consecutive cases of open spina bifida whose backs were closed non-selectively at birth between 1963 and 1971. METHOD: Survivors (age range = 26 to 33 years) were surveyed by postal questionnaire and telephone interview. The main outcome measures were the health, independence and lifestyle of the survivors in terms of living in the community, driving a car and working in open employment. RESULTS: Ascertainment was 100%. Sixty (51%) had died, mainly the most disabled. Of the 57 survivors, 84% had a cerebrospinal fluid (CSF) shunt, 70% had an IQ of 80 or more, 37% lived independently in the community, 39% drove a car, 30% could walk more than 50 metres and 26% were in open employment. However one-third (19) still needed daily care, three were on respiratory support, two were blind, two had diabetes mellitus, and one was on dialysis. Mortality, disability and achievement reflected the neurological deficit that had been recorded in infancy in terms of sensory level. Attainment and independence were reduced in those who had needed revision of CSF shunt. CONCLUSION: The survivors in this unselected cohort showed a wide range of outcome from apparent normality to very severe disability. This reflected both the extent of their original neurological deficit and events in the history of their CSF shunt.
PMCID: PMC1314678  PMID: 14601340
20.  Prevalence of spina bifida occulta in patients with functional disorders of the lower urinary tract and its relation to urodynamic and neurophysiological measurements. 
BMJ : British Medical Journal  1989;298(6670):357-359.
OBJECTIVE--To determine the relation between neurophysiological abnormalities and the radiological detection of spina bifida occulta in patients with dysfunction of the lower urinary tract. DESIGN--Blind assessment and subsequent decoding of mixed batch of abdominal radiographs from patients with and without urological symptoms for evidence of spina bifida occulta and comparison of results with those of previous control series. SETTING--Review study among tertiary referrals to an incontinence clinic of a city hospital. PATIENTS--One hundred and thirty eight adults with proved urodynamic abnormalities in whom neurophysiological measurements were available. INTERVENTIONS--None. END POINT--Correlation of neurophysiological abnormalities in lower urinary tract dysfunction with presence and type of spina bifida occulta and level of opening of posterior sacral arcs. MEASUREMENTS AND MAIN RESULTS--On decoding radiographs those from patients without urological symptoms showed a similar prevalence of spina bifida occulta to that in the control series (631/2707 controls; 23%). By contrast, patients with urological symptoms had a significantly increased prevalence of spina bifida occulta at S1 and S2 and a higher level of opening of posterior sacral arcs. The increased prevalence of the bony defect was particularly striking in men with urgency and instability and in women with stress incontinence. No significant correlation was found between any particular neurophysiological abnormality and the presence of spina bifida. CONCLUSIONS--In patients with dysfunction of the lower urinary tract neurophysiological abnormalities may be associated with congenital dysraphic lesions in the lower lumbar spine and sacrum. There appears to be no direct causal relation between the radiological and neurophysiological abnormalities but the findings suggest a common aetiological factor.
PMCID: PMC1835713  PMID: 2493933
21.  How Does Participation of Youth With Spina Bifida Vary by Age? 
Background
Youth with disabilities are at risk for decreased participation in community activities. However, little is known about participation at different developmental periods of childhood and adolescence among youth with spina bifida (SB) or whether child, family, and SB-associated factors influence participation.
Questions/purposes
Our cross-sectional study examined participation among youth with SB and assessed how participation differs between youth ages 2–5, 6–12, and 13–18; how participation relates to child (gender) and family (caregiver marital status, education, and employment) characteristics; and how participation relates to SB-related factors (motor level, hydrocephalus, ambulation, medical issues, and bladder/bowel needs).
Patients and Methods
Sixty-three youth ages 2–18 years and/or their caregivers completed age-appropriate measures of participation for youth with disabilities. The patients had an average age of 9.52 years (SD = 5.22), 83% had a shunt, 34% had a motor level of L2 or higher, and 66% L3 or lower.
Results
A comparison of youth ages 2–5 (n = 19), 6–12 (n = 21), and 13–18 (n = 23) revealed older youth participated less in recreational, physical, and skill-based activities. Caregiver employment facilitated participation in social activities. Youth who did not have a shunt participated more often in physical and skill-based activities. Youth without recent major medical issues participated more often in physical and social activities. More caregivers reported bladder and bowel needs as barriers to participation for youth ages 6–12 than those ages 2–5 or 13–18.
Conclusions
Participation of youth with SB varies by age and across child and caregiver factors and should be understood in a developmental and situational context.
doi:10.1007/s11999-010-1693-x
PMCID: PMC3069299  PMID: 21116755
22.  Adults with spina bifida and/or hydrocephalus. 
Postgraduate Medical Journal  1995;71(831):17-21.
Recent advances in medical technology enable many children with complex disabilities to survive into adulthood and to have certain expectations of life. One of these expectations is the continuity of specialist health care in an adult setting. This paper describes a new out-patient service which aims to provide optimum care, continuity and consistency of service for adults with spina bifida and/or hydrocephalus. The need for specialist health input into this service, in order to monitor the neurological, urological and psychosocial complications often associated with spina bifida and/or hydrocephalus is recognised. In one year (1992), 86 young adults with spina bifida and/or hydrocephalus attended for annual or more frequent assessment, either independently or with their families or carers. A variety of health and social problems were treated. In response to demand, a multi-disciplinary assessment unit, which includes the services of both medical and nursing specialists, occupational and physiotherapists, psychologists and access to specialist surgical opinions has recently opened at the Chelsea and Westminster Hospital. This new service attempts to meet some of the needs described in the outpatient audit. Adults with other disabilities are requesting to use this service. A longitudinal study to monitor quality, and outcome is indicated from this initial survey.
PMCID: PMC2397906  PMID: 7708585
23.  The Impact of Family, Peer, and School Contexts on Depressive Symptoms in Adolescents with Spina Bifida 
Rehabilitation psychology  2010;55(4):340-350.
Objective
Based on social ecological theory, this study examined the joint relations among adolescents’ family, peer, and school contexts and depressive symptoms in youth with spina bifida using cumulative, protective, and specific effects models.
Method
Sixty families of adolescents with spina bifida and 65 comparison families reported on adolescent’s positive experiences within these contexts and on depressive symptoms when youth were 14–15 and 16–17 years old.
Results
Adolescents with spina bifida had fewer total positive contexts and less positive experience within peer and school contexts, as compared to typically developing adolescents. Greater total number of positive contexts and higher levels of positive experiences within family and school contexts were associated with fewer depressive symptoms for both groups; peer positive experiences were related to lower depressive symptoms for typically developing adolescents only.
Conclusion
Adolescents with spina bifida have fewer positive contexts, which may place them at risk for higher levels of depressive symptoms.
doi:10.1037/a0021664
PMCID: PMC3075551  PMID: 21171793
Spina bifida; adolescence; depression; family functioning; school functioning; peers
24.  Early Results of Selective Treatment of Spina Bifida Cyctica 
British Medical Journal  1973;4(5886):201-204.
The results of a policy of “selection” in treating infants with spina bifida cystica over 21 months are presented. Of 37 newborn infants referred on the first day of life 25 were not treated because of the severity of their condition, as defined by exact criteria. All died under 9 months of age. In contrast, only one treated infant died and the rest are either normal or moderately handicapped. Selection for treatment is offered as the best but not a good solution to an insoluble problem.
PMCID: PMC1587325  PMID: 4586035
25.  Incidence, Prevalence, and Characteristics of Fractures in Children, Adolescents, and Adults With Spina Bifida 
The Journal of Spinal Cord Medicine  2007;30(Suppl 1):S5-S9.
Objective:
To determine the age-specific incidence, prevalence, and characteristics of fractures in persons with spina bifida.
Design:
Year-long historical cross-sectional study.
Subjects:
Two hundred twenty-one consecutive patients aged 2–58 years evaluated in 2003 at a regional referral center. Twenty percent (n=44) were children age 2–10 years; 30% (n=68) were adolescents age 11–18 years; and 50% (n =109) were adults age 19– 58 years. Fifty-five percent (n =121) were female; 64% (n = 141) had shunted hydrocephalus. Fifty-eight percent (n = 129) were community ambulators. Defect levels included 14% (n=31) thoracic; 37% (n=81) mid-lumbar; 35% (n=79) low-lumbar; and 14% (n=30) sacral.
Methods:
Chart review of 221 consecutive children, adolescents, and adults enrolled in a spina bifida program in Syracuse, New York, was used to determine incidence and prevalence rates. Chi-square was used for subgroup analyses, and linear regression was used to examine independent association of motor level, functional independence (Functional Independence Measures score), body mass index (BMI), shunted hydrocephalus, epilepsy, and/or other congenital anomalies with fractures, controlling for insurance status, race/ethnicity, age, and sex.
Results:
Annual incidence of fractures among children, adolescents, and adults was 23/1000; 29/1000; and 18/1000, respectively. Overall prevalence was 200/1000. One in 4 patients with fractures reported multiple fractures. Median age at first fracture was 11 years. Most fractures involved the femur or tibia. Comparisons between adult- and childhood-onset fractures were not significant for difference in sex, BMI, defect level, functional independence, shunted hydrocephalus, epilepsy, or other congenital anomalies. In regression models only defect level RR =1.646 (P =0.019; 95% CI 1.085–2.498) and age RR =1.033 (P =0.036; 95% CI 1.002–1.065) were independently associated with fractures.
Conclusions:
Fractures in persons with spina bifida are most common during early adolescence. Environmental modifications may be more effective than pharmacological treatment in reducing the prevalence of fractures in this population.
PMCID: PMC2031989  PMID: 17874679
Spina bifida; Fracture; Child; Adolescence; Adult; Bone loss

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