Ou X, Snow JH, Byerley AK, Hall JJ, Glasier CM. (2011) Decreased activation and increased lateralization in brain functioning for selective attention and response inhibition in adolescents with spina bifida. Child Neuropsychol. 2011 Dec 6.
We used functional magnetic resonance imaging to evaluate functional activity in the brain of adolescents with spina bifida when performing selective attention and response inhibition tasks. We then compared the results to that of age-matched controls. Our results showed that adolescents with spina bifida had decreased frontal and superior parietal activation and more apparently low involvement of left brain hemisphere during these tasks. Our results indicated activation deficits and possibly abnormal functional organization in adolescents with spina bifida and associated pathologies such as hydrocephalus.
PMID: 22145814
Monday, January 9, 2012
Treatment of the neurogenic bladder in spina bifida.
de Jong TP, Chrzan R, Klijn AJ, Dik P. (2008) Treatment of the neurogenic bladder in spina bifida. Pediatr Nephrol. 2008 Jun;23(6):889-96. Review.
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.
PMID: 18350321
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.
PMID: 18350321
Urological follow-up of adult spina bifida patients
Ahmad I, Granitsiotis P. (2007) Urological follow-up of adult spina bifida patients. Neurourol Urodyn. 2007;26(7):978-80. Review.
Department of Urology, Southern General Hospital, Glasgow, Scotland, United Kingdom. imranahmad@doctors.net.uk
AIMS:
The vast majority of the current urological literature understandably has concentrated on the management of children with spina bifida, because in the past the majority did not survive into adulthood. With improvements in the understanding and multidisciplinary care of spina bifida patients it has become a disease of adults. Our aim was to evaluate the current literature to attempt to formulate evidence based guidelines for the management of this difficult group of patients.
METHODS:
We reviewed the literature on adult urological management of spina bifida, all relevant articles which concentrated on adults and long-term management were studied in full.
RESULTS:
Renal function may begin/continue to deteriorate into adulthood, becoming the leading cause of adult death. This is thought to occur because of changes in the adult bladder, with increases in storage pressure. Medical and surgical management should aim to preserve renal function as well as the maintenance of continence in the face of the growing and changing urinary tract. Follow-up should be regular and in the context of a specialist multidisciplinary clinic. Despite being unvalidated in the follow-up of adult spina bifida patient's annual serum creatinine, ultrasound and urodynamics are currently the best tools available.
CONCLUSIONS:
There is no reason why the majority of spina bifida sufferers cannot use their own kidneys for the rest of their lives. This however relies on urological treatment being instigated soon after birth and continuing into adulthood.
PMID: 17626275
Department of Urology, Southern General Hospital, Glasgow, Scotland, United Kingdom. imranahmad@doctors.net.uk
AIMS:
The vast majority of the current urological literature understandably has concentrated on the management of children with spina bifida, because in the past the majority did not survive into adulthood. With improvements in the understanding and multidisciplinary care of spina bifida patients it has become a disease of adults. Our aim was to evaluate the current literature to attempt to formulate evidence based guidelines for the management of this difficult group of patients.
METHODS:
We reviewed the literature on adult urological management of spina bifida, all relevant articles which concentrated on adults and long-term management were studied in full.
RESULTS:
Renal function may begin/continue to deteriorate into adulthood, becoming the leading cause of adult death. This is thought to occur because of changes in the adult bladder, with increases in storage pressure. Medical and surgical management should aim to preserve renal function as well as the maintenance of continence in the face of the growing and changing urinary tract. Follow-up should be regular and in the context of a specialist multidisciplinary clinic. Despite being unvalidated in the follow-up of adult spina bifida patient's annual serum creatinine, ultrasound and urodynamics are currently the best tools available.
CONCLUSIONS:
There is no reason why the majority of spina bifida sufferers cannot use their own kidneys for the rest of their lives. This however relies on urological treatment being instigated soon after birth and continuing into adulthood.
PMID: 17626275
Early Start to Therapy Preserves Kidney Function in Spina Bifida Patients
Dik P, Klijn AJ, van Gool JD, de Jong-de Vos van Steenwijk CC, de Jong TP. (2006) Early Start to Therapy Preserves Kidney Function in Spina Bifida Patients
Eur Urol. 2006 May;49(5):908-13. Epub 2006 Jan 19.
OBJECTIVE:
Renal scarring and renal failure remain life-threatening for children born with spinal dysraphism. We reviewed our data of spina bifida patients to evaluate whether optimal treatment of the neurogenic bladder from birth onwards can preserve kidney function.
METHODS:
We reviewed data on all newborns with spinal dysraphism who were referred to our hospital between January 1988 and June 2001. We looked at their situations at referral and at follow-up: the type of treatment, antimuscarinic agents, clean intermittent catheterisation (CIC), antibiotic prophylaxis, and operations (sling procedures, bladder augmentations, antireflux procedures). Renal function (ultrasound, DMSA scan, serum creatinin, creatinin clearance) and bladder function (urodynamic studies) were evaluated over time.
RESULTS:
Data of 144 children of 176 could be evaluated by the end of the study: 5 patients had pre-existing renal abnormalities, 69 had an overactive sphincter, 27 had reflux, and six had renal scarring. None are currently developing end-stage renal disease. All patients with spina bifida aperta started CIC and antimuscarinic therapy shortly after birth. Five of the six patients with renal scarring were started on therapy with intermittent catheterisation and antimuscarinic therapy several months after birth. Sixty-three of 82 children with spina bifida were dry at school age (age six), although 37 of these had not had an operation.
CONCLUSION:
We show that an early start to therapy helps to safeguard renal function for children born with spina bifida. Our data support other recent reports that children born with spina bifida can probably use their own kidneys for a lifetime, if they are given adequate urological treatment. To protect the upper urinary tract, we need to ensure low intravesical pressure by starting children early on CIC (the preferred treatment); antimuscarinic agents to counteract detrusor instability are indispensable in most cases. Proactive treatment of risks for upper tract deterioration results in a negligible loss of renal function, even when early urinary continence is included in the treatment protocol.
PMID:16458416
Eur Urol. 2006 May;49(5):908-13. Epub 2006 Jan 19.
OBJECTIVE:
Renal scarring and renal failure remain life-threatening for children born with spinal dysraphism. We reviewed our data of spina bifida patients to evaluate whether optimal treatment of the neurogenic bladder from birth onwards can preserve kidney function.
METHODS:
We reviewed data on all newborns with spinal dysraphism who were referred to our hospital between January 1988 and June 2001. We looked at their situations at referral and at follow-up: the type of treatment, antimuscarinic agents, clean intermittent catheterisation (CIC), antibiotic prophylaxis, and operations (sling procedures, bladder augmentations, antireflux procedures). Renal function (ultrasound, DMSA scan, serum creatinin, creatinin clearance) and bladder function (urodynamic studies) were evaluated over time.
RESULTS:
Data of 144 children of 176 could be evaluated by the end of the study: 5 patients had pre-existing renal abnormalities, 69 had an overactive sphincter, 27 had reflux, and six had renal scarring. None are currently developing end-stage renal disease. All patients with spina bifida aperta started CIC and antimuscarinic therapy shortly after birth. Five of the six patients with renal scarring were started on therapy with intermittent catheterisation and antimuscarinic therapy several months after birth. Sixty-three of 82 children with spina bifida were dry at school age (age six), although 37 of these had not had an operation.
CONCLUSION:
We show that an early start to therapy helps to safeguard renal function for children born with spina bifida. Our data support other recent reports that children born with spina bifida can probably use their own kidneys for a lifetime, if they are given adequate urological treatment. To protect the upper urinary tract, we need to ensure low intravesical pressure by starting children early on CIC (the preferred treatment); antimuscarinic agents to counteract detrusor instability are indispensable in most cases. Proactive treatment of risks for upper tract deterioration results in a negligible loss of renal function, even when early urinary continence is included in the treatment protocol.
PMID:16458416
Care coordination in the spina bifida clinic setting: current practice and future directions
Brustrom J, Thibadeau J, John L, Liesmann J, Rose S. (2012) Care coordination in the spina bifida clinic setting: current practice and future directions.
J Pediatr Health Care. 2012 Jan;26(1):16-26. Epub 2010 Jul 10.
INTRODUCTION:
Although the potential benefits of care coordination are widely recognized, little is known about care coordination in the multidisciplinary spina bifida clinic setting. This study examined several aspects of care coordination in this environment.
METHOD:
We conducted semi-structured interviews with clinic staff (N = 43) and focus groups with caregivers (N = 38) at seven spina bifida clinics in the United States.
RESULTS:
Clinic staff described several primary goals of care coordination, including coordinating multiple services during one visit to ease the burden on families. Although the structure of care coordination varied across the clinics, several clinics had a dedicated care coordinator. Barriers and facilitators to care coordination included staffing issues, clinic day logistics, community resources, and family-related concerns. Despite challenges associated with care coordination processes, clinic staff and caregivers alike believed that care coordination is beneficial.
DISCUSSION:
Study findings suggest ways that care might be coordinated optimally in spina bifida clinics. A synthesis of these findings for clinics interested in implementing care coordination or improving the care coordination services they currently offer is provided.
PMID: 22153140
J Pediatr Health Care. 2012 Jan;26(1):16-26. Epub 2010 Jul 10.
INTRODUCTION:
Although the potential benefits of care coordination are widely recognized, little is known about care coordination in the multidisciplinary spina bifida clinic setting. This study examined several aspects of care coordination in this environment.
METHOD:
We conducted semi-structured interviews with clinic staff (N = 43) and focus groups with caregivers (N = 38) at seven spina bifida clinics in the United States.
RESULTS:
Clinic staff described several primary goals of care coordination, including coordinating multiple services during one visit to ease the burden on families. Although the structure of care coordination varied across the clinics, several clinics had a dedicated care coordinator. Barriers and facilitators to care coordination included staffing issues, clinic day logistics, community resources, and family-related concerns. Despite challenges associated with care coordination processes, clinic staff and caregivers alike believed that care coordination is beneficial.
DISCUSSION:
Study findings suggest ways that care might be coordinated optimally in spina bifida clinics. A synthesis of these findings for clinics interested in implementing care coordination or improving the care coordination services they currently offer is provided.
PMID: 22153140
Academic, Physical, and Social Functioning of Children and Adolescents With Chronic Physical Illness: A Meta-analysis.
Pinquart M, Teubert D. (2011) Academic, Physical, and Social Functioning of Children and Adolescents With Chronic Physical Illness: A Meta-analysis.
J Pediatr Psychol. 2011 Dec 15.
OBJECTIVE:
We compared the levels of academic, physical, and social functioning of children and adolescents with chronic physical diseases with those of healthy peers or test norms.
METHODS:
A random effects meta-analysis was computed to integrate the results of 954 studies.
RESULTS:
Impairments of physical functioning (g = 0.82 standard deviation units) were stronger than impairments of academic (g = 0.53) and social functioning (g = 0.43). Children with cerebral palsy and spina bifida showed the largest levels of functional impairments. The levels of functional impairments also varied by year of publication, source of information, ethnicity, country, and sociodemographic equivalence of the compared groups.
CONCLUSIONS:
More efforts are needed to develop and implement measures for the prevention of impaired functioning of children and adolescents with chronic physical illness and for the reduction of already existing impairments of their functioning.
PMID: 22173882
J Pediatr Psychol. 2011 Dec 15.
OBJECTIVE:
We compared the levels of academic, physical, and social functioning of children and adolescents with chronic physical diseases with those of healthy peers or test norms.
METHODS:
A random effects meta-analysis was computed to integrate the results of 954 studies.
RESULTS:
Impairments of physical functioning (g = 0.82 standard deviation units) were stronger than impairments of academic (g = 0.53) and social functioning (g = 0.43). Children with cerebral palsy and spina bifida showed the largest levels of functional impairments. The levels of functional impairments also varied by year of publication, source of information, ethnicity, country, and sociodemographic equivalence of the compared groups.
CONCLUSIONS:
More efforts are needed to develop and implement measures for the prevention of impaired functioning of children and adolescents with chronic physical illness and for the reduction of already existing impairments of their functioning.
PMID: 22173882
Monday, January 24, 2011
Cognitive and functional outcome in spina bifida-Chiari II malformation.
Jenkinson MD, Campbell S, Hayhurst C, Clark S, Kandasamy J, Lee MK, Flynn A, Murphy P, Mallucci CL.
Cognitive and functional outcome in spina bifida-Chiari II malformation.
Childs Nerv Syst. 2010 Dec 31.
PURPOSE: The long-term outcome in spina bifida-Chiari II-hydrocephalus complex is poorly understood. Traditional neurosurgical outcome measures are crude. Neuropsychological testing is increasingly important in outcome assessment. We investigated the health, disability, lifestyle and cognitive function in adults who had myelomeningocoele closure at birth.
METHODS: Adult patients under routine follow-up were assessed in a joint neurosurgery/neuropsychology clinic. Patients completed lifestyle questionnaires, the hydrocephalus outcome questionnaire (HOQ) and underwent cognitive testing. Clinical variables including number of shunt revisions, shunt infection and surgical decompression of foramen magnum, which may influence outcome, were investigated.
RESULTS: Twenty-one adults with a median age of 35 years were investigated. All had treated hydrocephalus, and eight had foramen magnum decompression for headache or progressive brainstem symptoms with stabilisation of symptoms in seven and improvement in one. Only eight patients were living independently, five were in paid employment and five work voluntarily. HOQ scores for cognitive function were lower (0.56 ± 0.20; mean ± standard deviation (SD)) than those for physical (0.64 ± 0.15) and social-emotional (0.65 ± 0.17) health. Cognitive function varied across the cohort with attention most severely affected (73.9 ± 17.0; mean ± SD). Repeated episodes of shunt malfunction or foramen magnum decompression were not associated with a worse cognitive function.
CONCLUSIONS: Despite intervention in childhood and adequate cerebrospinal fluid diversion the prognosis for independent living into adulthood remains poor. All patients have elements of cognitive impairment. Structural brain abnormalities may be more important determinants of cognitive outcome than shunt malfunction.
PMID: 21193992
Cognitive and functional outcome in spina bifida-Chiari II malformation.
Childs Nerv Syst. 2010 Dec 31.
PURPOSE: The long-term outcome in spina bifida-Chiari II-hydrocephalus complex is poorly understood. Traditional neurosurgical outcome measures are crude. Neuropsychological testing is increasingly important in outcome assessment. We investigated the health, disability, lifestyle and cognitive function in adults who had myelomeningocoele closure at birth.
METHODS: Adult patients under routine follow-up were assessed in a joint neurosurgery/neuropsychology clinic. Patients completed lifestyle questionnaires, the hydrocephalus outcome questionnaire (HOQ) and underwent cognitive testing. Clinical variables including number of shunt revisions, shunt infection and surgical decompression of foramen magnum, which may influence outcome, were investigated.
RESULTS: Twenty-one adults with a median age of 35 years were investigated. All had treated hydrocephalus, and eight had foramen magnum decompression for headache or progressive brainstem symptoms with stabilisation of symptoms in seven and improvement in one. Only eight patients were living independently, five were in paid employment and five work voluntarily. HOQ scores for cognitive function were lower (0.56 ± 0.20; mean ± standard deviation (SD)) than those for physical (0.64 ± 0.15) and social-emotional (0.65 ± 0.17) health. Cognitive function varied across the cohort with attention most severely affected (73.9 ± 17.0; mean ± SD). Repeated episodes of shunt malfunction or foramen magnum decompression were not associated with a worse cognitive function.
CONCLUSIONS: Despite intervention in childhood and adequate cerebrospinal fluid diversion the prognosis for independent living into adulthood remains poor. All patients have elements of cognitive impairment. Structural brain abnormalities may be more important determinants of cognitive outcome than shunt malfunction.
PMID: 21193992
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