Cases reported "Spinal Dysraphism"

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1/7. A child with spina bifida, cerebral palsy and juvenile rheumatoid arthritis: rehabilitation challenge.

    PURPOSE: A child with spina bifida, cerebral palsy and juvenile rheumatoid arthritis is presented, and strategies to approach a patient with multiple paediatric onset disabling conditions with possible overlaps are discussed. CONCLUSION: The value of multidisciplinary team approach including physiatrist, physical therapist, occupational therapist, rehabilitation nurse, prosthetist-orthotist, psychologist, speech-language pathologist, paediatric rheumatologist, social worker, kinesiotherapist, dietitian, recreation therapist, dentist and other disciplines as required is emphasized.
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keywords = physical
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2/7. Caring for a child with spina bifida: understanding the child and carer.

    Spina bifida is a common congenital abnormality, which causes significant physical and psychological morbidity in affected children and which also affects their carers. This small-scale study looked at the health problems of a child with a myelomeningocoele. It also addresses the psychosocial problems that his mother, his main carer, faced and the social networks involved in his care. The evidence supporting various aspects of spina bifida prevention and management is explored. Furthermore, a literature review is included, with regards to physical and psychological issues for child and carer. This study aims to raise awareness of the problems faced by children with myelomeningocoele and their families. In particular, we aim to educate health care professionals on the importance of perceived stress by carers of such children, and suggest ways to reduce psychosocial morbidity.
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keywords = physical
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3/7. Pseudo-tumours of the urinary tract in patients with spinal cord injury/spina bifida.

    OBJECTIVE: To raise awareness of pseudo-tumours of urinary tract, as pseudo-tumours represent benign mass lesions simulating malignant neoplasms. Accurate diagnosis helps to avoid unnecessary surgery in spinal cord injury patients. SETTING: Regional spinal injuries Centre, Southport, UK case reports: Pseudo-tumour of kidney: A 58-year-old man with tetraplegia developed a right perirenal haematoma while taking warfarin; ultrasound and CT scanning showed no evidence of tumour in the right kidney. The haematoma was drained percutaneously. After 8 months, during investigation of a urine infection, ultrasound and CT scan revealed a space-occupying lesion in the mid-pole of the right kidney. CT-guided biopsy showed features suggestive of an organising haematoma; the lesion decreased in size over the next 13 months, thus supporting the diagnosis. Pseudo-tumour of urinary bladder: A frail, 34-year-old woman, who had spina bifida, marked spinal curvature and pelvic tilt, had been managing her neuropathic bladder with pads. She had recurrent vesical calculi and renal calculi. CT scan was performed, as CT would be the better means of evaluating the urinary tract in this patient with severe spinal deformity. CT scan showed a filling defect in the base of the bladder, and ultrasound revealed a sessile space-occupying lesion arising from the left bladder wall posteriorly. Flexible and, later, rigid cystoscopy and biopsy demonstrated necrotic slough and debris but no tumour. Ultrasound scan after 2 weeks showed a similar lesion, but ultrasound-guided biopsy was normal with nothing to explain the ultrasound appearances. A follow-up ultrasound scan about 7 weeks later again showed an echogenic mass, but the echogenic mass was seen to move from the left to the right side of the bladder on turning the patient, always maintaining a dependent position. The echogenic bladder mass thus represented a collection of debris, which had accumulated as a result of chronic retention of urine and physical immobility. CONCLUSION: Recognising the true, non-neoplastic nature of these lesions enabled us to avoid unnecessary surgical procedures in these patients, who were at high risk of surgical complications because of severely compromised cardiac and respiratory function.
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ranking = 1
keywords = physical
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4/7. Premeditated assaults on young boys by a man with spina bifida and hydrocephalus--a cognitive-behavioural approach to treatment.

    This paper describes the case of a 25-year-old man with spina bifida and hydrocephalus, who had at least a ten-year history of physically assaulting young boys. Anti-social behaviour is reported as comparatively rare in people with this disability, and the case study illustrates how psychological assessment and treatment techniques were successfully applied. Information from his personal history plus a functional analysis of the assaults suggested that a combination of existing psychological difficulties and learning experiences contributed to the development of his behaviour. A behavioural model of the psychological mechanisms underlying the behaviour was suggested and treatment objectives defined. Cognitive and behavioural treatment techniques were adapted where necessary, with a good outcome. A gap in services for physically handicapped people with behavioural problems is noted and briefly discussed.
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keywords = physical
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5/7. spinal dysraphism: a comprehensive diagnostic approach.

    Twelve patients with the clinical findings of spinal dysraphism form the basis for this report. In eight patients, physical findings, plain x-rays, and unenhanced spinal computed tomography allowed for precise diagnosis and subsequent therapy, without contrast myelography. All patients in the study had intravenous pyelography, and this revealed evidence of renal deterioration in two. The other 10 patients had radiographically normal upper urinary tracts. Urodynamic assessment was performed in seven; three were normal and four were abnormal. The pattern of the abnormal studies (three "flaccid type" with an adequate urethral pressure profile and one with detrusor-sphincter dyssynergia) allowed for appropriate therapy to be employed for bladder emptying and continence. Both spinal computed tomography and urodynamic testing serve as noninvasive studies that can be employed in the follow-up and management of patients with spinal dysraphism.
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keywords = physical
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6/7. Hemifacial microsomia with vertebral anomalies: case report.

    A case of hemifacial microsomia with vertebral anomalies is reported. The patient, a 15-year-old girl, shows the typical physical and radiologic findings of the syndrome; in addition a severe spine involvement is present.
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7/7. Omphalocele-exstrophy-imperforate-anus-spina bifida (OEIS) complex in a male prenatally exposed to diazepam.

    A male clinically affected by the OEIS complex was studied. His mother, aged 30 years, has an affective disorder and ingested 30 mg of diazepam daily, from 3 months previous to the gestation and during the entire pregnancy. At birth, a closure during the entire pregnancy. At birth, a closure defect of the anterior abdominal wall, exstrophy of hemibladders, exposure of intestinal epithelium, abnormal pelvis, imperforate anus, and bifid penis were noted. birth weight was 3600 g and other measurements were not recorded. colostomy was performed in the postnatal period followed by partial closure of the abdominal wall defect, and iliac osteotomies. At six years, 6 months of age, physical examination showed somatometric measurements around the third percentile (height 109 cm, weight 17 kg, cephalic circumference 48.5 cm). Clinically he presented mild mental retardation, functional colostomy, incomplete closure of the vesical exstrophy, imperforate anus, bifid penis and scrotum, descended testes, diastasis of pubis, lumbosacral scoliosis and shortening of the left leg (clinical photograph of the external features is not included as we were not able to obtain authorization to do so). Radiological studies (Figure 1) revealed wide separation of the ischiopubic bones; lumbosacral region with rotoscoliosis, platyspondyly and dysraphism; left coxa valga, and right coxa vara. The abdominal ultrasonographic studies showed unilateral renal agenesis (left). Chromosomal analysis (GTG bands) in peripheral blood lymphocyte cultures demonstrated a normal 46,XY constitution. Exposure to other substances, particularly alcohol, were excluded with the study of the mother's medical history and through information obtained from relatives.(ABSTRACT TRUNCATED AT 250 WORDS)
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