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PubMed2007

Radiographic method to assess the prevalence of sacral spina bifida occulta.

Spina bifida occulta of the sacrum is the most common type of spinal deformity. Many authors have published data on the frequency of spina bifida occulta, with varying results. Some possible reasons for this variability could include the differing methods used to gather data and differing ways of classifying the condition. This study attempts to develop an X-ray method to study sacral spina bifida occulta in a standardized fashion, using an angulated antero-posterior technique. This technique is then used to estimate the frequency of sacral spina bifida occulta in an Australian sample. The sacra of 53 cadavers were X-rayed and the level of closure of the sacral spinal canal recorded. The X-ray technique was validated by open dissection of six of the cadavers studied and was shown to be accurate to half a sacral segment. No sacra with a completely open sacral canal were found, two sacra (4%) were open from S2 down to S5 and ten sacra (19%) were open from S3 down to S5. The most common condition (43%) recorded was where S4 and S5 only were open. Eighteen cadavers (34%) showed only S5 open, and interestingly, no sacra were recorded as having the dorsal sacral arch completely closed. A study of a larger sample will follow using the validated X-ray technique.

Adult↗
PubMed1983

Coxa vara in spina bifida.

In spina bifida the femoral neck can develop either the well-known coxa valga or the hitherto unreported coxa vara. Twenty-three cases of coxa vara in spina bifida are reported. These result from spontaneous separation of the upper femoral epiphysis (10 cases), spontaneous fracture of the femoral neck (three cases) and iatrogenic avascular necrosis of the upper femoral epiphysis (10 cases).

Child↗
PubMed2002

Needs assessment in a spina bifida program: a comparison of the perceptions by adolescents with spina bifida and their parents.

The primary purpose of this study was to describe and compare medical, psychosocial, and economic needs of adolescents and young adults with a neural tube defect and their parents. A secondary aim of the study was to establish the psychometric properties of a previously published needs assessment tool. Forty-nine adolescent and 59 parents receiving services from a comprehensive spina bifida program at a large children's hospital completed a needs assessment survey designed to measure perceived needs for health and social services. The Needs Assessment Questionnaire includes 8 subscales (Accessibility/Transportation/Independence, Financial Concerns, Medical Resources, Communication/Acceptance of individuals with spina bifida, Family/Socialization, Education of others regarding spina bifida, School Services, and Vocational Training). Factors related to perceived needs and differences between adolescents and parents were explored. There was a significant difference ( <.05) between parents and adolescents in 6 of the 8 subscales. On the remaining 2 subscales, differences between parents and adolescents approached significance. In all areas of needs for services, parents were less satisfied with the delivery of services and reported that needs were not being met. A significant negative correlation between age of the adolescent and perceived needs related to Transportation/Independence ( = -0.35) and School Services ( = -0.31) was found. Parents with more than a high school education perceived significantly greater needs for Vocational Training than those with less education ( = -2.1, =.04). Identification of medical, psychosocial, and economic needs of adolescents with a neural tube defect and their families has implications for quality improvement and program development.

Adolescent↗
PubMed1987

MRI CT, and plain film appearance of anterior spina bifida.

Anterior spina bifida or butterfly vertebral body has a well known and characteristic appearance on plain film and CT. Its appearance on magnetic resonance imaging also appears to be characteristic and should not be mistaken for more serious abnormalities.

Humans↗
PubMed1996

The cost of preventable conditions in adults with spina bifida.

Adults with spina bifida are at risk of developing secondary health conditions that are causally related to the primary condition and are to some degree preventable. By investigating preventable secondary conditions in adults with spina bifida, we can determine the burden of care that might be reduced if preventive strategies were developed. We audited hospitalizations of adults with spina bifida to gain insight into serious secondary conditions in this population. We reviewed all admissions of adults with spina bifida to a large American tertiary care center (Johns Hopkins Hospital; Baltimore, Maryland) over 11 years. We determined the discharge diagnoses for each admission and the hospital costs associated with admission. We defined preventable secondary conditions in this adult population and calculated the percentage of total admissions and of total costs for these secondary conditions. The patient population consisted of spina bifida patients 18 years and over admitted from January 1982 to December 1992, including 59 females and 39 males. 77 of our patients were identified as white, 20 as black, and 1 as "other". Patient age ranged from 18 to 61 years with an average age of 25.8 years. There were 353 admissions and admissions per patient ranged from 1 to 25 over the 11-year period. The average number of admissions per patient was 3.6 and the average length of stay for all admissions was 11.2 days. Of the 353 total admissions, 166 (47.0%) were due to potentially preventable secondary conditions such as serious urologic infections, renal calculi, pressure ulcers, and osteomyelitis. For admissions due to these secondary conditions, the average length of stay was 12.5 days. The total hospital costs, excluding professional fees, were $175,885, $247,355, and $437,262 for 1990, 1991, and 1992 respectively. Permanent loss of function resulting from secondary conditions, not investigated in this study, can also be a source of long-term financial and psychological cost to the patient. This study found a high number of patients with multiple admissions and that many admissions qualified as potentially preventable. More information is needed if we are to understand the factors associated with secondary health conditions in adults with spina bifida. We need to investigate the roles played by physician and patient knowledge, attitudes, and behaviors in contributing to patient outcome.

Adolescent↗
PubMed2006

Quality of life and continence in patients with spina bifida.

PURPOSE: Spina bifida (SB) is the most common congenital cause of incontinence in childhood. This study attempts to determine the relationships between urinary/faecal incontinence, methods of management, and Health Related Quality of Life (HRQoL) in people with SB. PATIENTS AND METHOD: A total of 460 patients (300 adults and 160 adolescents) from six centres in France have taken part in this cross-sectional study. Clinical outcome measures included walking ability, urinary/faecal continence, and medical management. HRQoL was assessed using the SF36 in adults and the VSP in adolescents and their parents. Univariate and multivariate analysis was used to determine the relationships between clinical parameters and HRQoL. RESULTS: HRQoL were significantly lower than in the general population. Adult women had significantly lower scores than men, and adolescent females had significantly lower scores for psychological well being. We did not found strong relationship between incontinence and HRQoL in this population. Moreover patients surgically managed for urinary/fecal incontinence did not show significantly higher scores of HRQoL. CONCLUSION: Using generic HRQoL measures, urinary/faecal incontinence and their medical management may not play a determinant role in HRQoL of persons with SB. However many other factors affect HRQoL in these patients. A longitudinal study design is recommended to assess whether incontinence management is associated with improved HRQoL.

Adolescent↗
PubMed1995

Helping to solve problems associated with spina bifida: 1. Presentation of the TRS Project: organizing services for low frequency diagnostic groups and 2. Cognitive deficits often seen in young adults with spina bifida: effects in the school and work place.

1. A Presentation of the TRS Project: Counselling and (Re)habilitation Center. A Model Project in Organizing Services for Low Frequency Diagnostic Groups. Persons with low frequency disabilities often require services from a number of professions. The patient organizations claim that the patients themselves often have to coordinate their own treatment. The TRS project has been established as one of the initiatives under the Norwegian Government's Plan of Action for the Disabled. The project is one of three national projects designed to develop models for the coordination of services for persons with low frequency congenital disabilities. The TRS project deals with the following five diagnoses: Marfan syndrome, arthrogryphosis multiplex congenita, myelomeningocele/spina bifida, osteogenesis imperfecta and congenital limb deficiency. The project is based on patient (user) participation. The five patient organizations are represented on the board where they are in the majority. Patients along with professionals give lectures during group stays. The project offers its services to persons with the diagnosis from all parts of Norway (4.3 mill. inhabitants). Persons with spina bifida over 16 years are included in the project, as well as persons with the other diagnoses at all ages. We present the organization of the project and the repertoire of services that are on offer. 2. Cognitive Deficits Often Seen in Young Adults with Spina Bifida: Effects in the School and Work Place. As survival rates continue to increase with the use of shunting procedures for persons with spina bifida (SB), the need for improved educational and vocational planning also increases. Orbeck and Schanke reported that the cognitive deficits of young adults with SB have not received enough attention in educational and vocational planning. A thorough description of these deficits will aid in developing more effective individualized planning. With improved planning, insight into alternative methods for improving support may also then be investigated. The purpose of this study is to better define and describe the cognitive deficits often encountered while planning support in the school and work place for this group. Neuropsychological examinations are the basis for this description of observed cognitive deficits. The study included 46 young adults with SB aged 15 to 38 living in Norway. All persons were given a neuropsychological test battery which included tests for attention, memory, speed of information processing, visual perception and visual constructive function, arithmetic, fine motor coordination, and verbal functioning. The results indicated deficits in fine motor coordination, speed of information processing, and a slow learning curve. The consequences of these deficits in educational and vocational planning are discussed and guidelines for further studies are suggested.

Abnormalities, Multiple↗
PubMed1991

Origin and treatment of fractures in spina bifida.

Children with spina bifida cystica have a high risk of fracturing their paraplegic legs. During the last fifteen years we observed 261 fractures and epiphyseal injuries in 173 children out of 1,400 (12.2%) patients with spina bifida. The increased risk of fracture seems to be due to reduced muscle activity in the paralysed limb with insufficient axial loading of the legs. A large proportion of fractures occurs after orthopedic interventions. Fractures are less common after urologic or neurosurgical procedures. Breaks are extremely frequent after operations in association with cast immobilization. Early standing and short immobilization times are the best defence mechanisms against fractures. If plasters are needed postoperatively one should preferably use the "Max and Moritz" standing cast. Fractures in spina bifida children heal quickly as compared to those in non-paralyzed children. In 30% excessive callus is seen. Immobilization for fracture care can be done in a standing cast even in the very early phase of treatment. In spite of the swelling and elevated temperature axial loading in the standing cast should continue. Splints and braces can be used instead of the plaster cast. By any means, the vicious circle of Fracture-Plaster-Fracture-Plaster should be avoided. Repeated stress on the growth plate causes a mechanism of loosening. The most common site is the distal tibia and femur, less often loosenings occur in the proximal tibia and proximal femur. Epiphyseal loosening is a nosological entity seen in spina bifida and also in congenital absence of sensation. Distal tibial epiphyseal loosening is frequently seen in adolescents who have learned to walk only after muscle balancing procedures in the hips.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed2000

Ventricular shunts and the prevalence of sensitization and clinically relevant allergy to latex in patients with spina bifida.

Patients with spina bifida represent the highest-risk group for developing hypersensitivity to latex. Recognized risk factors for these patients are repeated surgery and an atopic disposition. Our aim was to study the influence of a ventricular shunt as an independent risk factor. One hundred and sixty-one patients with spina bifida (median age 10 years) were investigated for the presence of a shunting device and the number, type and date of previous surgical interventions. Additionally, skin-prick tests and provocation tests were performed in order to classify sensitized and symptomatic latex-allergic individuals. Eighty-eight patients (54.7%) were sensitized; 55 (34.2%) were provocation-positive. Patients with a shunt system had undergone a significantly higher number of surgical procedures (p < 0.0001) and showed significantly higher levels of immunoglobulin E (IgE)-antibodies to latex (p < 0.0001) than patients without a shunting device. The total number of operations correlated significantly with the level of IgE-antibodies to latex in serum (p < 0.0001), whereas the number of shunt operations in patients with a ventricular shunt did not significantly correlate with the degree of sensitization. In conclusion, patients with spina bifida who have a ventricular shunt are at particularly high risk because they undergo a significantly higher number of operations than patients without a ventricular shunt. However, the ventricular shunt does not seem to be an independent risk factor. Furthermore, this study emphasizes that individuals at high risk, such as patients with spina bifida, should be handled latex-free from the very beginning of life.

Adolescent↗
PubMed1997

Embryogenesis. Why do we need a new explanation for the emergence of spina bifida with lipoma?

Spina bifida with lipoma is a human malformation that most often affects the lumbosacral area. It is a complex morphological type. Its origin is controversial, and none of the previous hypotheses can be retained in view of the most recent advances in experimental embryogenesis. Contrary to earlier opinions, adipocytes cannot arise from meninges, vessels or glial cells and spina bifida cannot be explained by incarceration of mesodermal tissues during primary neurulation or developmental defect at the level of the tail bud. Spina bifida with lipoma, which actually involves all the derivatives of the so-called dorsal mesoderm, must therefore result from abnormal development of this mesoderm, which is induced by the dorsal neural tube. The location of the primary defect (dorsal mesoderm or neural tube) remains to be established.

Embryonic Induction↗
PubMedDate not supplied

Rehabilitation of patients with spina bifida.

Patients with spina bifida present with multifarious problems requiring multi-disciplinary approach for their rehabilitation. 172 patients of spina bifida attending the Department of Physical Medicine and Rehabilitation at A.I.I.M.S., New Delhi, were studied retrospectively to ascertain the problems posed by them while presenting for rehabilitation. Male:female ratio was 1.23:1. Lumbo-sacral lesion comprised 41.3% followed by lumbar (26.1%) and sacral (22.1%). Common spinal deformities observed were Kyphoscoliosis (62.2%) and increased lumbar lordosis (19.2%). Clinically, active signs and symptoms of hydrocephalus were found in 47.7% associated with mental subnormality in 22.1%. Varying degrees of weakness of the muscles of lower limbs were detected in 98.3% cases, spasticity was found in 10.5% and ataxia in 2.9%. Improvement of muscle power was noticed in 45.3% during first three years and deterioration in 10.5% due to various complications. Foot deformities were commonest amongst deformities. Mobility was affected in 55.8% while 62.2% could attain independence. Bladder symptoms were present in 75% cases and bowel symptoms in 26.7%. Anxiety and guilt amongst parents was high in the families. Main hindering factors in vocational rehabilitation were bladder control, ambulation and pressure sores.

Activities of Daily Living↗
PubMed1979

Spina bifida in the dog.

Spina bifida was diagnosed in four English Bulldogs and one Collie dog. These cases and the examination of records from the Veterinary Medical Data Program suggested a high incidence of spina bifida in the English Bulldog. Urinary and fecal incontinence was the most common reason for initial examination for the five dogs. Radiographs and myelography confirmed the diagnosis of spina bifida. Blood, cerebrospinal fluid and urine analyses did not aid diagnosis but urocystitis was identified. In addition to spina bifida, clinical signs and morphologic changes in the spinal cords of all five dogs fulfilled the criteria for an additional diagnosis of spinal dysraphism. A common pathogenesis for the two conditions is suggested.

Animals↗
PubMed2002

[Spina bifida. Congenital malformation].

Spina bifida is the most common central nervous system defect and it occurs at a very early stage in an embryo, beginning at two weeks of gestation. The causes which produce this defect are varied and its consequences depend on the level at which the spinal lesion occurs. The authors describe the most common nursing and physiotherapeutic operations available for all those who suffer spina bifida, covering aspects such as neonatal care, treatment of anaesthetic skin, management of incontinence in the sphincters, care for deformities, ambulatory treatment, etc. These aspects are necessary in order to establish a good therapeutic plan, always as a part of an integrated treatment plan which facilitates the greatest degree of autonomy possible to these patients.

Humans↗