Barf HA, Post MW, Verhoef M, Jennekens-Schinkel A, Gooskens RH, Prevo AJ. Life satisfaction of young adults with spina bifida. Developmental Medicine and Child Neurology. 2007 Jun;49(6):458-63.
This study concerns life satisfaction and its determinants in Dutch young adults with spina bifida (SB). Data on life satisfaction (Life Satisfaction Questionnaire [LiSat-9]) were related to hydrocephalus, lesion level, disabilities, and demographic variables. In total, 179 young adults with SB participated (41% male, age range 16-25y; 79% SB aperta, 67% hydrocephalus [HC], 39% wheelchair-dependent). Most were satisfied with their life as a whole (24% dissatisfied). No difference was found from a population reference group (28% dissatisfied). Highest proportions of dissatisfaction were found for financial situation (44%), partnership relations (49%), and sex life (55%). Least dissatisfaction was found for contact with friends (17%) and families (15%). Young adults with SB and HC were more satisfied with their financial situation and family life but were less satisfied with self-care ability and partnership relations than those without HC and the reference group. However, except for self-care ability, relationships between life satisfaction and having SB were weak. In conclusion, self-care ability and partnership relations were rated least favourable and may need more attention from care providers. Overall, SB does not seem to be an important determinant of life satisfaction.
PMID: 17518933
Friday, September 19, 2008
Lightweight and ultralight wheelchairs: propulsion and preferences of two young children with spina bifida.
Meiser MJ, McEwen IR. Lightweight and ultralight wheelchairs: propulsion and preferences of two young children with spina bifida. Pediatric Physical Therapy. 2007 Fall;19(3):245-53.
PURPOSE: This study compared the influence of two wheelchair styles, an ultralight rigid frame and a lightweight folding frame, on preference and propulsion in young children with spina bifida.
PROCEDURE: Speed, distance, energy expenditure, and perceived exertion of two girls were studied in an A-B-A single subject design. Questionnaires were used after the three phases to obtain parent and child preference.
RESULTS: Visual inspection of the data favored the ultralight wheelchair for all variables, except the first child's speed with classmates and perceived exertion. Parents and children indicated preference for the ultralight wheelchair on all questions but one by Child 1. Using two standard deviation band width (2SDBW) analyses, 12 of 16 comparisons between the phases differed significantly. 2SDBW comparison was not used in the other four phase comparisons because of autocorrelations.
CONCLUSION: This study presents initial evidence supporting the use of ultralight rigid-framed wheelchairs by young children.
PMID: 17700354
PURPOSE: This study compared the influence of two wheelchair styles, an ultralight rigid frame and a lightweight folding frame, on preference and propulsion in young children with spina bifida.
PROCEDURE: Speed, distance, energy expenditure, and perceived exertion of two girls were studied in an A-B-A single subject design. Questionnaires were used after the three phases to obtain parent and child preference.
RESULTS: Visual inspection of the data favored the ultralight wheelchair for all variables, except the first child's speed with classmates and perceived exertion. Parents and children indicated preference for the ultralight wheelchair on all questions but one by Child 1. Using two standard deviation band width (2SDBW) analyses, 12 of 16 comparisons between the phases differed significantly. 2SDBW comparison was not used in the other four phase comparisons because of autocorrelations.
CONCLUSION: This study presents initial evidence supporting the use of ultralight rigid-framed wheelchairs by young children.
PMID: 17700354
Factors affecting shoulder pain in adolescents and young adults with spina bifida.
Roehrig S, Like G. Factors affecting shoulder pain in adolescents and young adults with spina bifida. Pediatric Physical Therapy. 2008 Fall;20(3):224-32.
PURPOSE: This study was designed to determine whether factors affecting shoulder pain in adults with spinal cord injury also affected adolescent and young adult manual wheelchair users with spina bifida.
METHODS: Forty-one participants with spina bifida rated their pain using the Wheelchair User's Shoulder Pain Index.
RESULTS: Results showed no significant relationship between shoulder pain and age or duration of wheelchair use but significant differences in pain between age groups. Participation in a sports program and level of lesion were not factors in pain intensity. Propelling up an incline was the activity for which the highest intensity of pain was reported.
CONCLUSION: Shoulder pain in adolescents and young adults with spina bifida is not as great as previously reported in adults with spinal cord injuries but older subjects had greater pain than younger subjects.
PMID: 18703959
PURPOSE: This study was designed to determine whether factors affecting shoulder pain in adults with spinal cord injury also affected adolescent and young adult manual wheelchair users with spina bifida.
METHODS: Forty-one participants with spina bifida rated their pain using the Wheelchair User's Shoulder Pain Index.
RESULTS: Results showed no significant relationship between shoulder pain and age or duration of wheelchair use but significant differences in pain between age groups. Participation in a sports program and level of lesion were not factors in pain intensity. Propelling up an incline was the activity for which the highest intensity of pain was reported.
CONCLUSION: Shoulder pain in adolescents and young adults with spina bifida is not as great as previously reported in adults with spinal cord injuries but older subjects had greater pain than younger subjects.
PMID: 18703959
Wheelchair propulsion demands during outdoor community ambulation
Wendy J. Hurd, Melissa M.B. Morrow, Kenton R. Kaufman, Kai-Nan An Wheelchair propulsion demands during outdoor community ambulation Journal of Electromyography and Kinesiology, . 2008 Jun 28.
Abstract
Objective: Quantify manual wheelchair propulsion effort during outdoor community ambulation.
Design: Case series.
Subjects: Thirteen individuals (12 with SCI, 1 with spina bifida) who were experienced manual wheelchair users and had no current upper extremity injury or pain complaints.
Methods: Measurements were obtained from instrumented wheelchair rims during steady-state propulsion as subjects traversed outdoor concrete sidewalk terrain that included smooth level, aggregate level, and a ramp with a smooth surface. Propulsion effort was assessed using the average propulsion moment, average instantaneous power, and work for both upper extremities.
Results: Propulsion effort, captured by the propulsion moment, work and power, varied across ground conditions (p < 0.001). Propulsion effort was greater as the rolling resistance increased (i.e., smooth versus aggregate surfaces) and as the inclination angle progressed from level to inclined surfaces. There were no side-to-side differences across ground conditions for the propulsion moment or work. Power generation was significantly greater on the dominant compared to the non-dominant extremity during the more challenging aggregate surface and ramp conditions.
Conclusions: Propulsion effort varies with demands imposed by different ground conditions. Quantification of wheelchair propulsion demands provides rehabilitations specialists with objective information to guide treatment of patients adapting to manual wheelchair use.
Keywords: Wheelchair; Propulsion; Mobility; Rehabilitation; Biomechanics
PMID: 18590967
Abstract
Objective: Quantify manual wheelchair propulsion effort during outdoor community ambulation.
Design: Case series.
Subjects: Thirteen individuals (12 with SCI, 1 with spina bifida) who were experienced manual wheelchair users and had no current upper extremity injury or pain complaints.
Methods: Measurements were obtained from instrumented wheelchair rims during steady-state propulsion as subjects traversed outdoor concrete sidewalk terrain that included smooth level, aggregate level, and a ramp with a smooth surface. Propulsion effort was assessed using the average propulsion moment, average instantaneous power, and work for both upper extremities.
Results: Propulsion effort, captured by the propulsion moment, work and power, varied across ground conditions (p < 0.001). Propulsion effort was greater as the rolling resistance increased (i.e., smooth versus aggregate surfaces) and as the inclination angle progressed from level to inclined surfaces. There were no side-to-side differences across ground conditions for the propulsion moment or work. Power generation was significantly greater on the dominant compared to the non-dominant extremity during the more challenging aggregate surface and ramp conditions.
Conclusions: Propulsion effort varies with demands imposed by different ground conditions. Quantification of wheelchair propulsion demands provides rehabilitations specialists with objective information to guide treatment of patients adapting to manual wheelchair use.
Keywords: Wheelchair; Propulsion; Mobility; Rehabilitation; Biomechanics
PMID: 18590967
Outcome of untethering for symptomatic spina bifida occulta with lumbosacral spinal cord tethering in 31 patients: analysis of preoperative prognostic
Jen-Ho Tseng, Meng-Fai Kuo, Yong Kwang Tu, Ming-Yuan Tseng Outcome of untethering for symptomatic spina bifida occulta with lumbosacral spinal cord tethering in 31 patients: analysis of preoperative prognostic factors The Spine Journal, Volume 8, Issue 4, July-August 2008, Pages 630-638
BACKGROUND CONTEXT: The most important goal for treating symptomatic lumbosacral spinal cord tethering is early untethering.
PURPOSE: To investigate preoperative symptoms that may have affected the outcome.
STUDY DESIGN: Patients with or without improvement and with or without favorable outcome after untethering were compared retrospectively by chart and image review.
PATIENT SAMPLE: Thirty-one patients (age between 2 days to 25 years) with spina bifida occulta and symptomatic cord tethering were analyzed. Presenting symptoms (neurological deficits, urological dysfunction, and lower limb deformities) were assessed before and after untethering.
OUTCOME MEASURES: Favorable outcome was defined as complete relief of symptoms or mild symptoms whereby patients are able to look after their own personal care without assistance. Unfavorable outcome was defined as moderate or severe disability whereby patients are unable to attend to their own bodily needs without assistance, are bedridden, or require constant nursing attention.
METHODS: Differences in patient characteristics and presenting symptoms were compared between those with and without clinical improvement and favorable outcome. Multivariate logistic regression was used to identify prognostic factors affecting the outcome.
RESULTS: The average age at surgery was 7.2 years, with a male-to-female ratio of 1.2. The average follow-up time was 4 years. At least one of the following symptoms was present in all patients: neurological deficits (83.9%), urological dysfunction (77.4%), or limb deformities (38.7%). After untethering, all patients had either symptoms stabilized (14 patients, 45.2%) or improved (17 patients, 54.8%), and 14 patients (45.2%) achieved total resolving of symptoms. Logistic regression confirmed that younger age (#2 years, odds ratio [OR] 22.0, p5.026), lipomas of filum terminale (OR 25.6, p5.042), and a poor anal tone (OR 10.4, p5.061) were positive prognostic factors for the improvement in symptoms. The functional outcome was determined by the age at surgery (OR 0.9 per year since 1 year old, p5.04) and the presence of limb deformities (OR 0.06, p5.017).
CONCLUSIONS: In conclusion, our study suggests that untethering should be performed immediately once the patient shows evidence of symptomatic lumbosacral cord tethering, irrespective of age. Untethering can interrupt progression of symptoms, but sphincter dysfunction and muscle weakness are more likely to improve or resolve. Benefits can be seen in all patients, but young children (before 2 years old) have a higher chance to gain favorable outcome. Retethering is a main concern during follow-up, particularly for the more complicated lipomyelomeningoceles. Investigations using electrophysiologic and urodynamic studies are helpful for early detection of subtle symptomatic cord tethering or retethering.
Keywords: Lipoma; Lumbosacral; Spina bifida occulta; Tethered cord; Untethering
BACKGROUND CONTEXT: The most important goal for treating symptomatic lumbosacral spinal cord tethering is early untethering.
PURPOSE: To investigate preoperative symptoms that may have affected the outcome.
STUDY DESIGN: Patients with or without improvement and with or without favorable outcome after untethering were compared retrospectively by chart and image review.
PATIENT SAMPLE: Thirty-one patients (age between 2 days to 25 years) with spina bifida occulta and symptomatic cord tethering were analyzed. Presenting symptoms (neurological deficits, urological dysfunction, and lower limb deformities) were assessed before and after untethering.
OUTCOME MEASURES: Favorable outcome was defined as complete relief of symptoms or mild symptoms whereby patients are able to look after their own personal care without assistance. Unfavorable outcome was defined as moderate or severe disability whereby patients are unable to attend to their own bodily needs without assistance, are bedridden, or require constant nursing attention.
METHODS: Differences in patient characteristics and presenting symptoms were compared between those with and without clinical improvement and favorable outcome. Multivariate logistic regression was used to identify prognostic factors affecting the outcome.
RESULTS: The average age at surgery was 7.2 years, with a male-to-female ratio of 1.2. The average follow-up time was 4 years. At least one of the following symptoms was present in all patients: neurological deficits (83.9%), urological dysfunction (77.4%), or limb deformities (38.7%). After untethering, all patients had either symptoms stabilized (14 patients, 45.2%) or improved (17 patients, 54.8%), and 14 patients (45.2%) achieved total resolving of symptoms. Logistic regression confirmed that younger age (#2 years, odds ratio [OR] 22.0, p5.026), lipomas of filum terminale (OR 25.6, p5.042), and a poor anal tone (OR 10.4, p5.061) were positive prognostic factors for the improvement in symptoms. The functional outcome was determined by the age at surgery (OR 0.9 per year since 1 year old, p5.04) and the presence of limb deformities (OR 0.06, p5.017).
CONCLUSIONS: In conclusion, our study suggests that untethering should be performed immediately once the patient shows evidence of symptomatic lumbosacral cord tethering, irrespective of age. Untethering can interrupt progression of symptoms, but sphincter dysfunction and muscle weakness are more likely to improve or resolve. Benefits can be seen in all patients, but young children (before 2 years old) have a higher chance to gain favorable outcome. Retethering is a main concern during follow-up, particularly for the more complicated lipomyelomeningoceles. Investigations using electrophysiologic and urodynamic studies are helpful for early detection of subtle symptomatic cord tethering or retethering.
Keywords: Lipoma; Lumbosacral; Spina bifida occulta; Tethered cord; Untethering
Spinal hemorrhages are associated with early neonatal motor function loss in human spina bifida aperta
D.A. Sival, R.J. Verbeek, O.F. Brouwer, K.M. Sollie, A.F. Bos, W.F.A. den Dunnen Spinal hemorrhages are associated with early neonatal motor function loss in human spina bifida aperta Early Human Development, Volume 84, Issue 7, July 2008, Pages 423-431
Background: In spina bifida aperta (SBA), leg movements caudal to the meningomyelocele are present in utero, but they disappear shortly after birth. It is unclear whether leg movements disappear by impact of the neuro-developmental malformation or by superimposed traumatic damage. If superimposed traumatic damage is involved, targeted fetal intervention could improve motor outcome.
Aim: To characterize neuromuscular pathology in association with perinatal motor function loss in SBA.
Patients/methods: In fetal SBA (n=8; 16–40 weeks GA), themedian time interval between ultrasound registrations of fetal motor behavior and post-mortem histology was 1 week. Histology was assessed cranial, at and caudal to the meningomyelocele and compared with findings in fetal controls (n=4).
Results: Despite fetal movements caudal to the meningomyelocele (5/6), histology indicated muscle fiber alterations (6/6) that concurred with neuro-developmental and traumatic spinal defects [Neuro-developmental defects: spinal ependymal denudation (3/8), reduced amount of (caspase3-negative) lower motor neurons (LMNs; 8/8), aberrant spinal vascularization (8/8). Traumatic defects: gliosis (7/8), acute/fresh spinal hemorrhages near LMNs (8/8)].
Key words: Spina bifida; Histology; Motor neuron; Spinal hemorrhage; Ependymal denudation; Fetal movement
Background: In spina bifida aperta (SBA), leg movements caudal to the meningomyelocele are present in utero, but they disappear shortly after birth. It is unclear whether leg movements disappear by impact of the neuro-developmental malformation or by superimposed traumatic damage. If superimposed traumatic damage is involved, targeted fetal intervention could improve motor outcome.
Aim: To characterize neuromuscular pathology in association with perinatal motor function loss in SBA.
Patients/methods: In fetal SBA (n=8; 16–40 weeks GA), themedian time interval between ultrasound registrations of fetal motor behavior and post-mortem histology was 1 week. Histology was assessed cranial, at and caudal to the meningomyelocele and compared with findings in fetal controls (n=4).
Results: Despite fetal movements caudal to the meningomyelocele (5/6), histology indicated muscle fiber alterations (6/6) that concurred with neuro-developmental and traumatic spinal defects [Neuro-developmental defects: spinal ependymal denudation (3/8), reduced amount of (caspase3-negative) lower motor neurons (LMNs; 8/8), aberrant spinal vascularization (8/8). Traumatic defects: gliosis (7/8), acute/fresh spinal hemorrhages near LMNs (8/8)].
Key words: Spina bifida; Histology; Motor neuron; Spinal hemorrhage; Ependymal denudation; Fetal movement
Dynamic assist by carbon fiber spring AFOs for patients with myelomeningocele
Sebastian I. Wolf, Merkur Alimusaj, Oliver Rettig, Leonhard Döderlein Dynamic assist by carbon fiber spring AFOs for patients with myelomeningocele Gait & Posture, Volume 28, Issue 1, July 2008, Pages 175-177
Abstract
Patients with calf muscle insufficiency and a calcaneus gait are often dependent on ankle-foot orthoses (AFO). The orthosis is intended to improve walking and posture and should prevent structural deformities. AFOs are often manufactured with a dorsiflexion stop. The design of this type of orthosis has been investigated in several previous studies. In the current study, orthoses with a dorsal carbon fiber spring were compared with the classic design. Five patients with Spina Bifida took part in the current study. All participants underwent a 3D gait analysis including kinematic (VICON infrared cameras) and kinetic (Kistler force plates) data collection.
The measurements showed that the carbon spring was able to support the patient during the complete stance phase. It was found that the use of a carbon fiber spring significantly increases the energy return during the 3rd rocker, simulating the natural push-off action ( p < 0.05). Via a simple mechanical test, the contribution of the carbon spring to the overall kinetics could be estimated proving that the spring does assist the patient for push-off.
The more physiological ankle and knee kinematics implies a functional improvement from the carbon springs compared to classic orthosis. This investigation showed, further, that in the fitting process a neutral alignment with the shoe wear has to be carefully checked since the spring kinematics and kinetics during stance phase were influenced significantly by the alignment. Further studies are needed to assess the clinical outcome and to prove the functional benefit of this kind of orthosis.
Keywords: Spina Bifida; Gait analysis; AFO; Carbon fiber spring; Orthosis
Abstract
Patients with calf muscle insufficiency and a calcaneus gait are often dependent on ankle-foot orthoses (AFO). The orthosis is intended to improve walking and posture and should prevent structural deformities. AFOs are often manufactured with a dorsiflexion stop. The design of this type of orthosis has been investigated in several previous studies. In the current study, orthoses with a dorsal carbon fiber spring were compared with the classic design. Five patients with Spina Bifida took part in the current study. All participants underwent a 3D gait analysis including kinematic (VICON infrared cameras) and kinetic (Kistler force plates) data collection.
The measurements showed that the carbon spring was able to support the patient during the complete stance phase. It was found that the use of a carbon fiber spring significantly increases the energy return during the 3rd rocker, simulating the natural push-off action ( p < 0.05). Via a simple mechanical test, the contribution of the carbon spring to the overall kinetics could be estimated proving that the spring does assist the patient for push-off.
The more physiological ankle and knee kinematics implies a functional improvement from the carbon springs compared to classic orthosis. This investigation showed, further, that in the fitting process a neutral alignment with the shoe wear has to be carefully checked since the spring kinematics and kinetics during stance phase were influenced significantly by the alignment. Further studies are needed to assess the clinical outcome and to prove the functional benefit of this kind of orthosis.
Keywords: Spina Bifida; Gait analysis; AFO; Carbon fiber spring; Orthosis
Impact of cecostomy and antegrade colonic enemas on management of fecal incontinence and constipation: Ten years of experience in pediatric population
Andrew L. Wong, Dragan Kravarusic, Sarah L. Wong Impact of cecostomy and antegrade colonic enemas on management of fecal incontinence and constipation: Ten years of experience in pediatric population Journal of Pediatric Surgery, Volume 43, Issue 8, August 2008, Pages 1445-1451
Abstract
Background: In childhood and adolescence, fecal soiling represents a psychologically devastating problem. Physical and emotional distress associated with daily rectal enemas is minimized by the introduction of a cecostomy tube for colonic cleansing with antegrade colonic enemas (ACEs).
Patients and Methods: Over a period of 10 years (1997-2007), we performed “button” cecostomies in 69 pediatric patients with fecal soiling secondary to a variety of disorders; laparoscopic procedures were performed in 40 and open procedures in 29. Mean postoperative follow-up was 4.03 SD ± 1.76 years. Cleansing protocols differed between patients.
Results: We adopted a standardized questionnaire concerning management of incontinence/intractable constipation before and after button cecostomy insertion to assess the long-term impact of ACE on symptom severity and quality of life. Complications included tube dislodgement (n = 9), development of granulation tissue (n = 11), decubitus ulcer (n = 5), and infection (n = 3). Patient/parents satisfaction (appraisal scale 1-3) and improvement of quality of life achieved statistical significance for both (P b .001).
Conclusions: Since button cecostomy and ACE were introduced in our institution as a management option, the treatment of fecal incontinence and intractable constipation significantly improved in terms of efficacy and patient compliance and also resulted in greater patient and parent satisfaction.
© 2008 Elsevier Inc. All rights reserved.
Key Words: Cecostomy; Antegrade colonic enema; Laparoscopy; Fecal incontinence; Spina bifida
Abstract
Background: In childhood and adolescence, fecal soiling represents a psychologically devastating problem. Physical and emotional distress associated with daily rectal enemas is minimized by the introduction of a cecostomy tube for colonic cleansing with antegrade colonic enemas (ACEs).
Patients and Methods: Over a period of 10 years (1997-2007), we performed “button” cecostomies in 69 pediatric patients with fecal soiling secondary to a variety of disorders; laparoscopic procedures were performed in 40 and open procedures in 29. Mean postoperative follow-up was 4.03 SD ± 1.76 years. Cleansing protocols differed between patients.
Results: We adopted a standardized questionnaire concerning management of incontinence/intractable constipation before and after button cecostomy insertion to assess the long-term impact of ACE on symptom severity and quality of life. Complications included tube dislodgement (n = 9), development of granulation tissue (n = 11), decubitus ulcer (n = 5), and infection (n = 3). Patient/parents satisfaction (appraisal scale 1-3) and improvement of quality of life achieved statistical significance for both (P b .001).
Conclusions: Since button cecostomy and ACE were introduced in our institution as a management option, the treatment of fecal incontinence and intractable constipation significantly improved in terms of efficacy and patient compliance and also resulted in greater patient and parent satisfaction.
© 2008 Elsevier Inc. All rights reserved.
Key Words: Cecostomy; Antegrade colonic enema; Laparoscopy; Fecal incontinence; Spina bifida
The Malone Antegrade Continence Enema: Single Institutional Review
Ahmad H. Bani-Hani, Mark P. Cain, Martin Kaefer, Kirstan K. Meldrum, Shelly King, Cynthia S. Johnson, Richard C. Rink The Malone Antegrade Continence Enema: Single Institutional Review The Journal of Urology, Volume 180, Issue 3, September 2008, Pages 1106-1110
Purpose: We report the long-term outcome of the Malone antegrade continence enema procedure in the treatment of chronic constipation and fecal incontinence in children with neuropathic bowel.
Materials and Methods: We performed a retrospective chart review of 256 Malone antegrade continence enema procedures. Patient age at surgery, bowel segment used, location of stoma, number and type of revisions required, and fecal continence
were documented and a database was created.
Results: A total of 236 patients with at least 6 months of followup are included in this report. There were 172 in situ appendicocecostomies, 23 split appendix channels, 9 appendicocecostomies with cecal extension, 22 Yang-Monti ileocecostomies and 10 colon flap channels performed. A total of 112 males (47%) and 124 females (53%) were included in the study. Median patient age at surgery was 9 years (range 2 to 36) and median followup for the entire cohort was 50 months (6 to 115). A total of 56 surgical revisions were performed in 39 patients (17%). Median time to first revision was 9.5 months (range 1 to 105). Stomal stenosis occurred in 32 patients (14%). Overall, surgical revisions were required in 14% of in situ appendix channels (24 of 172), 22% of split appendix channels (5 of 23), 33% of appendix channels with cecal extension (3 of 9), 18% of Yang-Monti Malone antegrade continence enema channels (4 of 22) and 30% of colon flap channels (3 of 10). Independent of channel technique, surgical revisions were required in 15% of umbilical stomas and 18% of lower quadrant stomas (p = 0.516). Two patients had minimal stomal leakage, and 94% (221 of 236) achieved fecal continence with irrigations.
Conclusions: The long-term results of the Malone antegrade continence enema channel in a large cohort of patients with neuropathic bowel and chronic constipation are encouraging. Of the patients 17% will require revision surgery, and patients/parents should be counseled accordingly. In our series in situ appendicocecostomy had the lowest revision rate on long-term followup, although the difference was not statistically significant (p = 0.226).
Key Words: appendix, constipation, enema, spina bifida cystica, surgical stomas
Purpose: We report the long-term outcome of the Malone antegrade continence enema procedure in the treatment of chronic constipation and fecal incontinence in children with neuropathic bowel.
Materials and Methods: We performed a retrospective chart review of 256 Malone antegrade continence enema procedures. Patient age at surgery, bowel segment used, location of stoma, number and type of revisions required, and fecal continence
were documented and a database was created.
Results: A total of 236 patients with at least 6 months of followup are included in this report. There were 172 in situ appendicocecostomies, 23 split appendix channels, 9 appendicocecostomies with cecal extension, 22 Yang-Monti ileocecostomies and 10 colon flap channels performed. A total of 112 males (47%) and 124 females (53%) were included in the study. Median patient age at surgery was 9 years (range 2 to 36) and median followup for the entire cohort was 50 months (6 to 115). A total of 56 surgical revisions were performed in 39 patients (17%). Median time to first revision was 9.5 months (range 1 to 105). Stomal stenosis occurred in 32 patients (14%). Overall, surgical revisions were required in 14% of in situ appendix channels (24 of 172), 22% of split appendix channels (5 of 23), 33% of appendix channels with cecal extension (3 of 9), 18% of Yang-Monti Malone antegrade continence enema channels (4 of 22) and 30% of colon flap channels (3 of 10). Independent of channel technique, surgical revisions were required in 15% of umbilical stomas and 18% of lower quadrant stomas (p = 0.516). Two patients had minimal stomal leakage, and 94% (221 of 236) achieved fecal continence with irrigations.
Conclusions: The long-term results of the Malone antegrade continence enema channel in a large cohort of patients with neuropathic bowel and chronic constipation are encouraging. Of the patients 17% will require revision surgery, and patients/parents should be counseled accordingly. In our series in situ appendicocecostomy had the lowest revision rate on long-term followup, although the difference was not statistically significant (p = 0.226).
Key Words: appendix, constipation, enema, spina bifida cystica, surgical stomas
The Experience of Finding an Effective Bowel Management Program for Children With Spina
Sawin KJ, Thompson NM, The Experience of Finding an Effective Bowel Management Program for Children With Spina Bifida: The Parent's Perspective Journal of Pediatric Nursing, In Press
Many families who have a child with spina bifida identify establishing an effective bowel program as one of the major challenges they face; yet, we know little about a family's experience with this process. The purpose of this qualitative study was
to describe the parent's experience. Seven parents were interviewed by telephone. Content analysis yielded four themes: (a) long complicated journey, (b) the impact of the journey on the child, (c) the family struggle, and (d) the promise of the future. The major contribution of this study is a compelling description of one of the most difficult areas for these children and their families. A better understanding of this issue enhances the health care providers' ability to deliver optimal health care.
Key words: Bowel management; Spina bifida; Children; Family struggle
Many families who have a child with spina bifida identify establishing an effective bowel program as one of the major challenges they face; yet, we know little about a family's experience with this process. The purpose of this qualitative study was
to describe the parent's experience. Seven parents were interviewed by telephone. Content analysis yielded four themes: (a) long complicated journey, (b) the impact of the journey on the child, (c) the family struggle, and (d) the promise of the future. The major contribution of this study is a compelling description of one of the most difficult areas for these children and their families. A better understanding of this issue enhances the health care providers' ability to deliver optimal health care.
Key words: Bowel management; Spina bifida; Children; Family struggle
Autoantibodies to folate receptor during pregnancy and neural tube defect risk
Robert M. Cabrera, Gary M. Shaw, Johnathan L. Ballard, Suzan L. Carmichael, Wei Yang, Edward J. Lammer, Richard H. Finnell Autoantibodies to folate receptor during pregnancy and neural tube defect risk Journal of Reproductive Immunology, In Press, Corrected Proof, Available online 18 September 2008
Abstract
Periconceptional folic acid can reduce the occurrence of neural tube defects (NTDs) by up to 70%, and autoantibodies for folate receptors (FRs) have been observed in serum from women with a pregnancy complicated by an NTD. This population-based cohort
study has examined serum from pregnant mothers for autoantibodies to FRs, antibodies to bovine folate binding protein (FBP), and inhibition of folic acid binding to FR and FBP in association with NTD risk. The mid-gestational maternal serum specimens
used for this study were collected during the 15–18th week of pregnancy. Samples were obtained from the California Birth Defects Monitoring Program; 29 mothers had a pregnancy complicated by spina bifida and 76 mothers had unaffected children. The presence of IgG and IgM antibodies to human FR, bovine FBP, and inhibition of folic acid binding to FR and FBP was determined. Higher activity of IgM to FBP in cases verses controls was observed (P = 0.04). Higher activity of IgM and IgG autoantibodies to FR was observed (P < 0.001 and P = 0.04, respectively). Risk estimates at two standard deviations above average control antibody
concentrations were OR= 2.07 (CI = 1.02, 4.06) for anti-FBP IgM, OR= 2.15 (CI = 1.02, 4.69) for anti-FR IgG and OR= 3.19 (CI = 1.47, 6.92) for anti-FR IgM. These data support the hypothesis that high titers of antibodies and blocking of folic acid binding to FRs by maternal serum should be regarded as risk factors for NTDs.
© 2008 Elsevier Ireland Ltd. All rights reserved.
Keywords: Folate receptor; Autoantibodies; Pregnancy; Neural tube defects
Abstract
Periconceptional folic acid can reduce the occurrence of neural tube defects (NTDs) by up to 70%, and autoantibodies for folate receptors (FRs) have been observed in serum from women with a pregnancy complicated by an NTD. This population-based cohort
study has examined serum from pregnant mothers for autoantibodies to FRs, antibodies to bovine folate binding protein (FBP), and inhibition of folic acid binding to FR and FBP in association with NTD risk. The mid-gestational maternal serum specimens
used for this study were collected during the 15–18th week of pregnancy. Samples were obtained from the California Birth Defects Monitoring Program; 29 mothers had a pregnancy complicated by spina bifida and 76 mothers had unaffected children. The presence of IgG and IgM antibodies to human FR, bovine FBP, and inhibition of folic acid binding to FR and FBP was determined. Higher activity of IgM to FBP in cases verses controls was observed (P = 0.04). Higher activity of IgM and IgG autoantibodies to FR was observed (P < 0.001 and P = 0.04, respectively). Risk estimates at two standard deviations above average control antibody
concentrations were OR= 2.07 (CI = 1.02, 4.06) for anti-FBP IgM, OR= 2.15 (CI = 1.02, 4.69) for anti-FR IgG and OR= 3.19 (CI = 1.47, 6.92) for anti-FR IgM. These data support the hypothesis that high titers of antibodies and blocking of folic acid binding to FRs by maternal serum should be regarded as risk factors for NTDs.
© 2008 Elsevier Ireland Ltd. All rights reserved.
Keywords: Folate receptor; Autoantibodies; Pregnancy; Neural tube defects
Parents' personality and parenting stress in families of children with spina bifida
Vermaes IP, Janssens JM, Mullaart RA, Vinck A, Gerris JR. Parents' personality and parenting stress in families of children with spina bifida.
Child: Care Health & Development. 2008 Sep;34(5):665-74.
BACKGROUND: Spina bifida (SB) can place parents at risk for increased levels of parenting stress. Little is known, however, about the role of parents' intrapersonal resources. Therefore, based on ideas of the Disability-Stress-Coping Model, relations between the severity of SB, parents' personality traits and parenting stress were examined.
METHODS: Forty-six mothers and 37 fathers of children with SB (6-14 years) participated. Severity of SB (physical dysfunctions and cognitive functions), parental personality (Big Five) and parenting stress (Parenting Stress Index) were measured. Multiple regression analyses were performed.
RESULTS: The severity of the child's physical dysfunctions was positively associated with parenting stress. Extraversion (mothers only), emotional stability and agreeableness (fathers only) were negatively related to parenting stress. In the final model, 64% of the variance in mothers' and 67% of the variance in fathers' levels of parenting stress was explained. Parents' personality traits explained the largest proportions of variance in parenting stress.
CONCLUSION: Mobility, bladder and bowel dysfunctions in school-aged children with SB represent ongoing stressors for parents. Parents' intrapersonal resources of positive affectivity, however, are more important determinants of parental adjustment to SB than the child's physical dysfunctions.
PMID: 18796058
Child: Care Health & Development. 2008 Sep;34(5):665-74.
BACKGROUND: Spina bifida (SB) can place parents at risk for increased levels of parenting stress. Little is known, however, about the role of parents' intrapersonal resources. Therefore, based on ideas of the Disability-Stress-Coping Model, relations between the severity of SB, parents' personality traits and parenting stress were examined.
METHODS: Forty-six mothers and 37 fathers of children with SB (6-14 years) participated. Severity of SB (physical dysfunctions and cognitive functions), parental personality (Big Five) and parenting stress (Parenting Stress Index) were measured. Multiple regression analyses were performed.
RESULTS: The severity of the child's physical dysfunctions was positively associated with parenting stress. Extraversion (mothers only), emotional stability and agreeableness (fathers only) were negatively related to parenting stress. In the final model, 64% of the variance in mothers' and 67% of the variance in fathers' levels of parenting stress was explained. Parents' personality traits explained the largest proportions of variance in parenting stress.
CONCLUSION: Mobility, bladder and bowel dysfunctions in school-aged children with SB represent ongoing stressors for parents. Parents' intrapersonal resources of positive affectivity, however, are more important determinants of parental adjustment to SB than the child's physical dysfunctions.
PMID: 18796058
Risk and protective influences in the lives of siblings of youths with spina bifida
Bellin MH, Kovacs PJ, Sawin KJ. Risk and protective influences in the lives of siblings of youths with spina bifida. Health & Social Work. 2008 Aug;33(3):199-209.
The impact of childhood chronic health conditions like spina bifida (SB) is a shared family experience. However, the lived experience of siblings is not well known. One hundred and fifty-five brothers and sisters of a child with SB responded to an open-ended question included in an anonymous self-administered mail questionnaire designed to enhance awareness of how adolescent siblings experience this chronic condition. Content analysis performed by interdisciplinary authors identified four domains--Rewards and Consequences of Spina Bifida, Journey Toward Acceptance of Spina Bifida, Emotional Climate of Siblings, and Qualities of the Social Environment--that depict emotional complexity in response to the diverse risk and protective influences present in the lives of siblings. By bolstering the siblings' strengths and resources found in their adaptive coping mechanisms, spirituality, cohesive family ties, and supportive peer friendships, social workers and other health care professionals may help them to successfully navigate the intense and confusing emotions that may naturally spring from the challenges and opportunities associated with SB.
PMID: 18773795
The impact of childhood chronic health conditions like spina bifida (SB) is a shared family experience. However, the lived experience of siblings is not well known. One hundred and fifty-five brothers and sisters of a child with SB responded to an open-ended question included in an anonymous self-administered mail questionnaire designed to enhance awareness of how adolescent siblings experience this chronic condition. Content analysis performed by interdisciplinary authors identified four domains--Rewards and Consequences of Spina Bifida, Journey Toward Acceptance of Spina Bifida, Emotional Climate of Siblings, and Qualities of the Social Environment--that depict emotional complexity in response to the diverse risk and protective influences present in the lives of siblings. By bolstering the siblings' strengths and resources found in their adaptive coping mechanisms, spirituality, cohesive family ties, and supportive peer friendships, social workers and other health care professionals may help them to successfully navigate the intense and confusing emotions that may naturally spring from the challenges and opportunities associated with SB.
PMID: 18773795
Health related quality of life in adolescents with abnormal bladder function: an assessment using the Child Health and Illness Profile-Adolescent Edit
Dodson JL, Furth SL, Hsiao CJ, Diener-West M, Levey EB, Wu AW, Gearhart JP. Health related quality of life in adolescents with abnormal bladder function: an assessment using the Child Health and Illness Profile-Adolescent Edition.
Journal of Urology. 2008 Oct;180(4 Suppl):1846-51; discussion 1851.
PURPOSE: We studied the impact of abnormal bladder function due to congenital urological disorders on health related quality of life in children. A reliable patient based method is needed to assess the impact of these conditions in children and the interventions used to treat them.
MATERIALS AND METHODS: Participants 11 to 17 years old with bladder exstrophy-epispadias complex, spina bifida or other causes of abnormal bladder function self-administered the Child Health and Illness Profile-Adolescent Edition, a generic health related quality of life instrument. They also responded to questions about incontinence, catheterization status and bother level. Mean scores on the profile were compared to population based norms.
RESULTS: Mean age of the 50 participants was 14.9 years, 62% were male and 82% were white. Diagnoses included bladder exstrophy-epispadias complex in 37 patients, spina bifida in 10 and other in 3. The mean +/- SD score on the disorders domain of 14.2 +/- 6.3 was significantly worse than the population norm of 20. Mean scores on the satisfaction, discomfort, resilience, risks and achievement domains were comparable to or better than the population based norm of 20. A total of 29 participants reported incontinence and 31 performed catheterization.
CONCLUSIONS: In this study of adolescents with congenital causes of abnormal bladder function Child Health and Illness Profile-Adolescent Edition generic health related quality of life scores were significantly worse in the disorders domain but largely comparable to or better than those of the general population in other domains. This suggests that the profile may discern between adolescents with structural urological disease and norms but it may not be sensitive enough to fully detect the impact of the condition. Alternatively adolescents may adapt well to the challenges of urological disease.
Key Words: urinary bladder, quality of life, questionnaires, abnormalities, adolescent
PMID: 18721969
Journal of Urology. 2008 Oct;180(4 Suppl):1846-51; discussion 1851.
PURPOSE: We studied the impact of abnormal bladder function due to congenital urological disorders on health related quality of life in children. A reliable patient based method is needed to assess the impact of these conditions in children and the interventions used to treat them.
MATERIALS AND METHODS: Participants 11 to 17 years old with bladder exstrophy-epispadias complex, spina bifida or other causes of abnormal bladder function self-administered the Child Health and Illness Profile-Adolescent Edition, a generic health related quality of life instrument. They also responded to questions about incontinence, catheterization status and bother level. Mean scores on the profile were compared to population based norms.
RESULTS: Mean age of the 50 participants was 14.9 years, 62% were male and 82% were white. Diagnoses included bladder exstrophy-epispadias complex in 37 patients, spina bifida in 10 and other in 3. The mean +/- SD score on the disorders domain of 14.2 +/- 6.3 was significantly worse than the population norm of 20. Mean scores on the satisfaction, discomfort, resilience, risks and achievement domains were comparable to or better than the population based norm of 20. A total of 29 participants reported incontinence and 31 performed catheterization.
CONCLUSIONS: In this study of adolescents with congenital causes of abnormal bladder function Child Health and Illness Profile-Adolescent Edition generic health related quality of life scores were significantly worse in the disorders domain but largely comparable to or better than those of the general population in other domains. This suggests that the profile may discern between adolescents with structural urological disease and norms but it may not be sensitive enough to fully detect the impact of the condition. Alternatively adolescents may adapt well to the challenges of urological disease.
Key Words: urinary bladder, quality of life, questionnaires, abnormalities, adolescent
PMID: 18721969
Development and validation of the fecal incontinence and constipation quality of life measure in children with spina bifida
Nanigian DK, Nguyen T, Tanaka ST, Cambio A, DiGrande A, Kurzrock EA. Development and validation of the fecal incontinence and constipation quality of life measure in children with spina bifida. Journal of Urology. 2008 Oct;180(4 Suppl):1770-3; discussion 1773.
Purpose: Fecal incontinence and constipation in children with spina bifida are recognized to impact quality of life. Most
disease specific quality of life instruments on fecal incontinence target adults and/or children without neuropathic bowel. We developed an instrument to evaluate bowel function and its impact on quality of life in children with spina bifida and their caregivers.
Materials and Methods: A 51-item questionnaire termed the FIC QOL (Fecal Incontinence and Constipation Quality of Life) survey was developed from expert opinion, patient interviews, and modification of previously published adult and pediatric studies for nonneuropathic bowel dysfunction. The items are divided into 7 quality of life factor groupings, including bowel program, dietary management, symptoms, travel and socialization, family relationships, caregiver emotional impact and financial impact. The questionnaire was given to caregivers of children with and without spina bifida. Discriminant validity was evaluated by comparing the spina bifida and control groups. Test-retest reliability was evaluated by having 41 patients complete 2 surveys within 4 to 6 weeks.
Results: Comparing questionnaires from 92 index patients and 52 controls showed a statistically significant difference for all
7 quality of life factor groupings. The FIC QOL instrument objectively demonstrated the negative impact of fecal incontinence and constipation on quality of life in these families. Comparing 82 questionnaires at 2 time points demonstrated the
reliability of all FIC QOL questions.
Conclusions: The FIC QOL instrument provides a valid and reliable measure of the effect of fecal incontinence and constipation on the quality of life of caregivers and their children with spina bifida.
Key Words: abnormalities, spinal dysraphism, quality of life, questionnaires, fecal incontinence
PMID: 18721959
Purpose: Fecal incontinence and constipation in children with spina bifida are recognized to impact quality of life. Most
disease specific quality of life instruments on fecal incontinence target adults and/or children without neuropathic bowel. We developed an instrument to evaluate bowel function and its impact on quality of life in children with spina bifida and their caregivers.
Materials and Methods: A 51-item questionnaire termed the FIC QOL (Fecal Incontinence and Constipation Quality of Life) survey was developed from expert opinion, patient interviews, and modification of previously published adult and pediatric studies for nonneuropathic bowel dysfunction. The items are divided into 7 quality of life factor groupings, including bowel program, dietary management, symptoms, travel and socialization, family relationships, caregiver emotional impact and financial impact. The questionnaire was given to caregivers of children with and without spina bifida. Discriminant validity was evaluated by comparing the spina bifida and control groups. Test-retest reliability was evaluated by having 41 patients complete 2 surveys within 4 to 6 weeks.
Results: Comparing questionnaires from 92 index patients and 52 controls showed a statistically significant difference for all
7 quality of life factor groupings. The FIC QOL instrument objectively demonstrated the negative impact of fecal incontinence and constipation on quality of life in these families. Comparing 82 questionnaires at 2 time points demonstrated the
reliability of all FIC QOL questions.
Conclusions: The FIC QOL instrument provides a valid and reliable measure of the effect of fecal incontinence and constipation on the quality of life of caregivers and their children with spina bifida.
Key Words: abnormalities, spinal dysraphism, quality of life, questionnaires, fecal incontinence
PMID: 18721959
Neural stem cell delivery to the spinal cord in an ovine model of fetal surgery for spina bifida
Fauza DO, Jennings RW, Teng YD, Snyder EY. Neural stem cell delivery to the spinal cord in an ovine model of fetal surgery for spina bifida. Surgery. 2008 Sep;144(3):367-73. Epub 2008 Jul 25.
Department of Surgery and Advanced Fetal Care Center, Children's Hospital Boston, Boston, MA 02115, USA. dario.fauza@childrens.harvard.edu
BACKGROUND: We introduce the notion of prenatal neural stem cell (NSC) delivery to the spinal cord as an adjuvant to fetal repair of spina bifida.
METHODS: Fetal lambs with experimental myelomeningocele (MMC; n = 25) were divided in 3 groups: group I, no repair; group II, standard surgical MMC coverage; and group III, MMC coverage plus delivery of a murine NSCs clone into the spinal cord defect. Donor cells constitutively expressed lacZ encoding for Escherichia coli beta-galactosidase, yet they were further labeled by exposure to either BrdU and/or to the fluorescent membrane dye PKH-26. Blinded initial clinical evaluations and multiple spinal cord analyses were undertaken soon after birth.
RESULTS: Both survival and the incidence of major paraparesis were significantly worse in group I compared with groups II and III. In group III, NSC density was highest within the most damaged areas of the spinal cord, with selective engraftment within those regions. Donor NSCs retained an undifferentiated state in vivo, producing neurotrophic factors within the defect. No animals in group III had a worsened condition following this intervention.
CONCLUSIONS: Neural stem cells retain an undifferentiated state and produce neurotrophic factors in the short term after delivery to the fetal spinal cord, in the setting of experimental MMC. Further scrutiny of NSC delivery to the spinal cord as a therapeutic strategy against spina bifida is warranted.
PMID: 18707035
Department of Surgery and Advanced Fetal Care Center, Children's Hospital Boston, Boston, MA 02115, USA. dario.fauza@childrens.harvard.edu
BACKGROUND: We introduce the notion of prenatal neural stem cell (NSC) delivery to the spinal cord as an adjuvant to fetal repair of spina bifida.
METHODS: Fetal lambs with experimental myelomeningocele (MMC; n = 25) were divided in 3 groups: group I, no repair; group II, standard surgical MMC coverage; and group III, MMC coverage plus delivery of a murine NSCs clone into the spinal cord defect. Donor cells constitutively expressed lacZ encoding for Escherichia coli beta-galactosidase, yet they were further labeled by exposure to either BrdU and/or to the fluorescent membrane dye PKH-26. Blinded initial clinical evaluations and multiple spinal cord analyses were undertaken soon after birth.
RESULTS: Both survival and the incidence of major paraparesis were significantly worse in group I compared with groups II and III. In group III, NSC density was highest within the most damaged areas of the spinal cord, with selective engraftment within those regions. Donor NSCs retained an undifferentiated state in vivo, producing neurotrophic factors within the defect. No animals in group III had a worsened condition following this intervention.
CONCLUSIONS: Neural stem cells retain an undifferentiated state and produce neurotrophic factors in the short term after delivery to the fetal spinal cord, in the setting of experimental MMC. Further scrutiny of NSC delivery to the spinal cord as a therapeutic strategy against spina bifida is warranted.
PMID: 18707035
Subscribe to:
Posts (Atom)
