The Natural History of Spina Bifida Cystica: Detailed Analysis of 407 Cases

Laurence, K. M.

doi:10.1136/adc.39.203.41

Cited by 88 publications

(30 citation statements)

References 24 publications

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“…Clinical data show that the majority of the sample (53.8%) have several associated neurological conditions (hydrocephalus, Arnold-Chiari syndrome, radiological or clinical tethered cord syndrome), as confirmed by literature and clinical practice data (25)(26)(27)(28)(29). The dominance of psychological problems, such as anxiety and depression that can be classified as internalizing disturbances, is confirmed in the literature (2).…”

Section: Discussionmentioning

confidence: 70%

“…The SF-36 quality of life data are reported in Table 5. As regards clinical data, it can be noticed that most of the sample patients (53.8%) are affected by several associated neurological disorders (hydrocephalus, Arnold-Chiari syndrome, radiological or clinical tethered-cord), as confirmed by literature and clinical practice data (25)(26)(27)(28)(29). The majority of subjects in the sample either walk unaided or use a wheelchair, while only 10% of them use walking orthoses.…”

Section: Resultsmentioning

confidence: 80%

See 1 more Smart Citation

Adolescents and young adults with spina bifida: psycho-pathological risks and quality of life

Gatta¹,

Pertile²,

Zotto³

et al. 2014

PER

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Background: youths with Spina Bifida (SB) experience a variety of urological, neurological and orthopaedic problems, as well as varying degrees of limitations in mobility and autonomy which affect their psycho-affective state and the quality of their life (QOL). So far the psycho-relational functioning of SB subjects has been little studied in terms of psychological problems and psycho-pathological risks, especially in children and teenagers patients. The multidisciplinary team taking care of individuals with SB must have medical competences associated to familiarity with the psychological and social problems of patients and their families. We analized the quality of life as perceived by adolescents and young adults with Spina Bifida not only in relation to their physical skills but also to their psychosocial health and well-being. The starting hypothesis is that greater physical disability and the presence of psychopathology are associated to a lower quality of life. Objectives: in light of the limited literature on Spina Bifida and psychopathological risks, this study is aimed to assess the presence of psychopathological behaviour in the current clinical sample. Methods: the sample included 42 subjects, 20 males (47.6%) and 22 females (52.4%), average age 19 years ± 3.5. The following tests were administered: Barthel Index, SF-36, SCL-90 R, YSR 11-18 and CBCL 6-18.Results: the psychopathology results indicate a relevant percentage of pathological behaviour, mostly internalizing problems. In particular, psychopathological elements are associated to more serious physical disability. As regards the relationship between quality of life, physical disability and psychic disability, the study revealed that psychopathology is generally associated to a lower quality of life (both in physical and emotional aspects), whereas physical dysfunction is related to quality of life in different ways. The fact that adolescents with fewer physical problems have a worse emotional self-concept than adolescents with severe disability is discussed. Conclusions: this trend does not make it possible to predict the evolution of psychic disturbance and suggests that, for the treatment and for a good quality of life, it is important to consider each patient's personal history and life setting in terms of culture, education and family history, with particular attention to how this condition is perceived.KEY WORDS: spina bifida, adolescence, quality of life, psychopathology.

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Section: Discussionmentioning

confidence: 70%

Section: Resultsmentioning

confidence: 80%

Adolescents and young adults with spina bifida: psycho-pathological risks and quality of life

Gatta¹,

Pertile²,

Zotto³

et al. 2014

PER

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“…Of these neurofibromatosis was recorded in 70 %, though it was thought that the proportion would RL TRIPLOWELIA have been higher had they been re-assessed at a later date. It is interesting that by far the commonest he embryonic form of meningocele unassociated with neurofibromatosis is a dorsal protrusion of meninges fan abnormal through a defect of the spinal laminae, and the !y inhibit tube thoracic region is a comparatively uncommon site ernatively the for this (Ingraham and Matson, 1954;Doran and tly, producing Guthkelch, 1961;Laurence, 1964). Indeed only one case of meningocele outside the thoracic region with rat neuroecto-neurofibromatosis has been recorded (Sammons and lht side to an Thomas, 1959).…”

Section: Discussionmentioning

confidence: 99%

Neurofibromatosis with congenital malformation of the spinal cord

Barson¹,

Cole²

1967

Journal of Neurology, Neurosurgery & Psychiatry

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“…Esta constatação simplesmente reflete a evolução dos conceitos relacionados à postura médica frente a portadores de mielomeningocele, não mais se justificando a adoção de critérios seletivos, conforme os propostos pelo grupo de Sheffield há aproximadamente 20 anos 10 . A análise de uma série de 407 casos de mielomeningocele publicada por Laurence, em 1964 9 , revela que o número de pacientes que teve o defeito corrigido correspondia a pouco mais de uma terça parte da população estudada, que a maioria morria de infecções intracranianas e que a possibilidade de um recém-nascido alcançar os 12 anos de idade era de 29%. A introdução de antibióticos mais potentes, bem como o desenvolvimento e posterior aperfeiçoamento dos sistemas de derivação ventricular, aumentou consideravelmente os índices de sobrevivência destes pacientes, sendo parcialmente responsáveis pela popularização dos chamados critérios de seleção, cujo objetivo era oferecer tratamento apenas àqueles pacientes considerados de bom prognóstico.…”

Section: Acompanhamentounclassified

Acompanhamento ambulatorial de pacientes com mielomeningocele em um hospital pediátrico

Salomão

Leibinger

Carvalho

et al. 1995

Arq. Neuro-Psiquiatr.

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RESUMO -A mielomeningocele constitui a mais freqüente malformação congênita do sistema nervoso e, a despeito de sua complexidade e acometimento de múltiplos órgãos, é compatível a sobrevida prolongada. Por esses motivos, o acompanhamento de pacientes com essa malformação assume vital importância no que diz respeito à qualidade de sua sobrevida. Com o objetivo de avaliar a qualidade do acompanhamento de portadores de spina bifida cística tratados em um hospital pediátrico, 54 pacientes foram examinados e seus familiares entrevistados. A inexistência de um centro multidisciplinar de tratamento de portadores de defeito de fechamento do tubo neural na cidade do Rio de Janeiro obrigou os pacientes a se deslocarem para locais geograficamente distantes entre si em busca de tratamento complementar em várias especialidades correlatas, com reflexos negativos na qualidade de sobrevida. Desta maneira, apenas uma quarta parte dos pacientes era capaz de se locomover e a chamada deambulação comunitária era praticamente nenhuma. Apenas 2/3 se submetiam regularmente a fisioterapia e apenas a metade era regularmente acompanhada por ortopedistas. Quase 50% dos pacientes não foram orientados a procurar assistência urológica e 75% apresentavam incontinência urinária. A incidência de infecções urinárias de repetição foi 72,2%. As complicações e intercorrências neurocirúrgicas propriamente ditas tiveram incidências comparáveis às observadas na literatura. Concluímos que a qualidade de sobrevida dos pacientes estudados é significativamente afetada por fatores sócio-econômicos e pela ausência de centros multidisciplinares de tratamento. PALAVRAS-CHAVE: spina bifida, mielomeningocele, disrafismo espinhal, clínica multidisciplinar. Follow-up of myelomeningocele patients in the outclinic of a pediatric hospitalSUMMARY -Myelomeningocele is the most common congenital malformation of the nervous system and despite its complexity and involvement of multiple organs is compatible with long survival. The peculiar characteristics of this malformation expose myelomeningocele patients to acute and chronic care problems with effects in quality of survival. In order to evaluate the quality of the follow-up of spina bifida patients in a pediatric hospital, the authors examined 54 patients attending the neurosurgical outpatient unity of a pediatric hospital. The lack of a multidisciplinary spina bifida clinic in Rio de Janeiro forced the patients to pursuit for complimentary medical and paramedical care outside the hospital with significant effects in the quality of survival. In consequence, only 25% of the patients were able to walk and community ambulation was nearly absent. Only 66.6% had a regular rehabilitation program and nearly 50% had routine orthopedics consultations. Almost half of the patients had no urological referral at all and 75% were incontinent, with recurrent urinary infections ranging 72.2%. The rates of neurosurgical complications were similar to those observed in the literature. We concluded that the quality of survival of patients...

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The Natural History of Spina Bifida Cystica: Detailed Analysis of 407 Cases

Cited by 88 publications

References 24 publications

Adolescents and young adults with spina bifida: psycho-pathological risks and quality of life

Adolescents and young adults with spina bifida: psycho-pathological risks and quality of life

Neurofibromatosis with congenital malformation of the spinal cord

Acompanhamento ambulatorial de pacientes com mielomeningocele em um hospital pediátrico

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