Kim J, Beasley SW, Maoate K. Appendicostomy stomas and antegrade colonic irrigation after laparoscopic antegrade continence enema. Journal of Laparoendoscopic & Advanced Surgical Techniques 2006 Aug;16(4):400-3.
BACKGROUND: Children with spina bifida, high anorectal anomalies, or neuronal intestinal dysplasia who are treated with a laparoscopic antegrade continence enema to achieve a socially acceptable level of fecal continence sometimes have problems with the stoma and its catheterization. The goal of this study was to determine the nature and incidence of these problems, and their relationship to the underlying condition.
MATERIALS AND METHODS: A retrospective review of the hospital case notes of 74 consecutive patients who had a laparoscopic antegrade continence enema was undertaken.
RESULTS: The laparoscopic antegrade continence enema procedure is well tolerated but is associated with a variety of usually minor complications including stomal stenosis, leakage, peristomal infection, granulation tissue overgrowth, mucosal prolapse, abdominal discomfort during irrigation, appendiceal false passage, and ineffective irrigation. Surgical revision of the stoma was required in 19% of the cases in this study, usually because of stenosis and skin overgrowth. Ongoing problems achieving colonic emptying were most likely to occur in patients with an anorectal malformation.
CONCLUSION: Overall, the laparoscopic antegrade continence enema provides an effective method of facilitating emptying of the bowel. However, it has a high incidence of minor troublesome problems, with stenosis and skin overgrowth of the stoma being the most common. Younger children tend to have more complications and difficulty performing effective irrigation. Children with anorectal malformations were more likely to require surgical revision of the stoma, and often needed ongoing manual evacuation and bowel washouts despite the laparoscopic antegrade continence enema.
PMID: 16968193
Wednesday, October 31, 2007
Achieving Fecal Continence in Patients With Spina Bifida: A Descriptive Cohort Study
Velde SV, Biervliet SV, Renterghem KV, Laecke EV, Hoebeke P, Winckel MV. Achieving Fecal Continence in Patients With Spina Bifida: A Descriptive Cohort Study. Journal of Urology. 2007 Oct 16; [Epub ahead of print]
PURPOSE: Fecal incontinence is a major problem in patients with myelomeningocele. We evaluate the results of a stratified approach aimed at obtaining fecal pseudo-continence in patients with myelomeningocele.
MATERIALS AND METHODS: We conducted a cross-sectional descriptive study of last file data in 80 patients 5 to 18 years old with myelomeningocele followed at our center. Beginning at birth patients with myelomeningocele were seen at least annually by the pediatric gastoenterologist, a member of the multidisciplinary "spina team." Constipation was treated with diet and osmotic laxatives. Starting at age 5 years, treatment was targeted at achieving pseudo-continence. A toilet training scheme was started, associated with induced defecation by digital stimulation. Retrograde tap water enemas were used in patients with unsatisfactory results. If retrograde enemas were unsuccessful, an antegrade continence enema procedure was proposed. For children unable to sit on a toilet regular manual evacuation of stools was advised.
RESULTS: Eight of the 80 patients were fecal continent. Pseudo-continence was achieved in 50 of the 72 incontinent patients (69%), including 5 of 5 following only a strict toilet scheme, 21 of 24 (87.5%) performing retrograde enemas, 16 of 20 (80%) performing orthograde enemas through an antegrade continence device and 8 of 10 performing regular manual evacuation of stools. In 4 of the 20 patients (20%) performing orthograde enemas complications led to closure of the antegrade continence device. Treatment failed and was stopped in 17 patients. Success of treatment was not related to level of spinal lesion or degree of mobility.
CONCLUSIONS: Fecal pseudo-continence was achieved in 58 of 80 patients (72.5%) with myelomeningocele.
PMID: 17945290
PURPOSE: Fecal incontinence is a major problem in patients with myelomeningocele. We evaluate the results of a stratified approach aimed at obtaining fecal pseudo-continence in patients with myelomeningocele.
MATERIALS AND METHODS: We conducted a cross-sectional descriptive study of last file data in 80 patients 5 to 18 years old with myelomeningocele followed at our center. Beginning at birth patients with myelomeningocele were seen at least annually by the pediatric gastoenterologist, a member of the multidisciplinary "spina team." Constipation was treated with diet and osmotic laxatives. Starting at age 5 years, treatment was targeted at achieving pseudo-continence. A toilet training scheme was started, associated with induced defecation by digital stimulation. Retrograde tap water enemas were used in patients with unsatisfactory results. If retrograde enemas were unsuccessful, an antegrade continence enema procedure was proposed. For children unable to sit on a toilet regular manual evacuation of stools was advised.
RESULTS: Eight of the 80 patients were fecal continent. Pseudo-continence was achieved in 50 of the 72 incontinent patients (69%), including 5 of 5 following only a strict toilet scheme, 21 of 24 (87.5%) performing retrograde enemas, 16 of 20 (80%) performing orthograde enemas through an antegrade continence device and 8 of 10 performing regular manual evacuation of stools. In 4 of the 20 patients (20%) performing orthograde enemas complications led to closure of the antegrade continence device. Treatment failed and was stopped in 17 patients. Success of treatment was not related to level of spinal lesion or degree of mobility.
CONCLUSIONS: Fecal pseudo-continence was achieved in 58 of 80 patients (72.5%) with myelomeningocele.
PMID: 17945290
Antegrade and retrograde endoscopic dextranomer/hyaluronic Acid bladder neck
Dean GE, Kirsch AJ, Packer MG, Scherz HC, Zaontz MR. Antegrade and retrograde endoscopic dextranomer/hyaluronic Acid bladder neck bulking for pediatric incontinence. Journal of Urology. 2007 Aug;178(2):652-5. Epub 2007 Jun 15.
PURPOSE: Endoscopic bladder neck bulking techniques offer the potential for a relatively noninvasive cure for a difficult clinical problem. We review our experience using antegrade and retrograde approaches.
MATERIALS AND METHODS: A total of 34 children (18 boys and 16 girls, mean age 11.7 years) have been treated since March 2003. Of the patients 28 (82%) had neurogenic bladder and 6 had nonneurogenic sphincteric incontinence. Urodynamics confirmed low detrusor leak point pressures and adequate bladder capacity. Patients were treated with either a retrograde or an antegrade approach. In 82% of patients an antegrade approach was used and a posttreatment suprapubic tube was placed.
RESULTS: Mean followup was 11.7 months (range 3 to 31). Patients averaged 1.47 injections (range 1 to 5). Detailed followup of 19 patients revealed significant improvement in continence in 78% (mean 1.6 injections), with an average followup of approximately 1 year.
CONCLUSIONS: Our 31 months of experience with antegrade/retrograde bladder neck bulking demonstrates that it is a viable therapy for this group of children. While some patients have experienced prolonged success, re-treatment can be beneficial. The antegrade approach offers several advantages, including intraoperative leak point pressures, improved visualization and placement of a suprapubic tube to limit post-procedural remodeling.
PMID: 17574622
PURPOSE: Endoscopic bladder neck bulking techniques offer the potential for a relatively noninvasive cure for a difficult clinical problem. We review our experience using antegrade and retrograde approaches.
MATERIALS AND METHODS: A total of 34 children (18 boys and 16 girls, mean age 11.7 years) have been treated since March 2003. Of the patients 28 (82%) had neurogenic bladder and 6 had nonneurogenic sphincteric incontinence. Urodynamics confirmed low detrusor leak point pressures and adequate bladder capacity. Patients were treated with either a retrograde or an antegrade approach. In 82% of patients an antegrade approach was used and a posttreatment suprapubic tube was placed.
RESULTS: Mean followup was 11.7 months (range 3 to 31). Patients averaged 1.47 injections (range 1 to 5). Detailed followup of 19 patients revealed significant improvement in continence in 78% (mean 1.6 injections), with an average followup of approximately 1 year.
CONCLUSIONS: Our 31 months of experience with antegrade/retrograde bladder neck bulking demonstrates that it is a viable therapy for this group of children. While some patients have experienced prolonged success, re-treatment can be beneficial. The antegrade approach offers several advantages, including intraoperative leak point pressures, improved visualization and placement of a suprapubic tube to limit post-procedural remodeling.
PMID: 17574622
An assessment of health related quality of life
Dodson JL, Diener-West M, Gerson AC, Kaskel FJ, Furth SL. An assessment of health related quality of life using the child health and illness profile-adolescent edition in adolescents with chronic kidney disease due to underlying urological disorders. Journal of Urology. 2007 Aug;178(2):660-5; discussion 665. Epub 2007 Jun 15.
PURPOSE: We examined health related quality of life in adolescents with congenital urological disease causing kidney disease using a generic health related quality of life instrument. We then compared the results to those in adolescents with medical kidney disease and to population based norms.
MATERIALS AND METHODS: The Child Health and Illness Profile-Adolescent Edition was administered to 113 patients 10 to 18 years old with chronic kidney disease. Mean domain and subdomain scores for adolescents with urological disease were compared to those of adolescents with medical kidney disease and to population based norms.
RESULTS: The cohort included adolescents with an underlying diagnosis of congenital urological anomaly (37 patients) or other causes of kidney disease (76). Compared to adolescents with kidney disease caused by other factors, those with congenital urological disease scored statistically significantly better in the Child Health and Illness Profile-Adolescent Edition subdomain of Limitations of Activity (mean 22.3 [SD 2.5] vs 20.4 [SD 5.0], p = 0.04). Compared to population norms, adolescents with congenital disorders scored lower in the Disorders domain (mean 16.5, 95% CI 14.2 to 18.9) but better in the Risks domain (mean 25.9, 95% CI 25.1 to 26.6) and in the Home Safety and Health subdomain (mean 25.2, 95% CI 23.7 to 26.6).
CONCLUSIONS: As assessed by the Child Health and Illness Profile-Adolescent Edition generic health status questionnaire, adolescents with kidney disease due to underlying congenital urological disease had fewer limitations of activity compared to those with underlying medical kidney disease. Except for low scores in the Disorders domain, children with underlying urological disease did not have significant impairments in any other domain compared to population based norms.
PMID: 17574620
PURPOSE: We examined health related quality of life in adolescents with congenital urological disease causing kidney disease using a generic health related quality of life instrument. We then compared the results to those in adolescents with medical kidney disease and to population based norms.
MATERIALS AND METHODS: The Child Health and Illness Profile-Adolescent Edition was administered to 113 patients 10 to 18 years old with chronic kidney disease. Mean domain and subdomain scores for adolescents with urological disease were compared to those of adolescents with medical kidney disease and to population based norms.
RESULTS: The cohort included adolescents with an underlying diagnosis of congenital urological anomaly (37 patients) or other causes of kidney disease (76). Compared to adolescents with kidney disease caused by other factors, those with congenital urological disease scored statistically significantly better in the Child Health and Illness Profile-Adolescent Edition subdomain of Limitations of Activity (mean 22.3 [SD 2.5] vs 20.4 [SD 5.0], p = 0.04). Compared to population norms, adolescents with congenital disorders scored lower in the Disorders domain (mean 16.5, 95% CI 14.2 to 18.9) but better in the Risks domain (mean 25.9, 95% CI 25.1 to 26.6) and in the Home Safety and Health subdomain (mean 25.2, 95% CI 23.7 to 26.6).
CONCLUSIONS: As assessed by the Child Health and Illness Profile-Adolescent Edition generic health status questionnaire, adolescents with kidney disease due to underlying congenital urological disease had fewer limitations of activity compared to those with underlying medical kidney disease. Except for low scores in the Disorders domain, children with underlying urological disease did not have significant impairments in any other domain compared to population based norms.
PMID: 17574620
Monday, October 29, 2007
Do grip and pinch strength predict neurologic complications in children with spina bifida and hydrocephalus
Liptak GS, Fried R, Baltus-Hebert E, Eyer Tierney S, Fucile S, Doremus TL. Do grip and pinch strength predict neurologic complications in children with spina bifida and hydrocephalus? Pediatric Neurosurgery. 2006;42(4):208-13.
BACKGROUND: Neurological complications occur commonly in children with meningomyelocele and can cause significant morbidity and mortality. An earlier study suggested that acute changes in grip and pinch strength could be used to identify individuals at increased risk for developing a neurological complication.
OBJECTIVE: To evaluate the use of grip and pinch measurements to screen for neurological problems in children with spina bifida and hydrocephalus.
DESIGN: A prospective evaluation of screening tests.
PATIENTS: 92 children, born since 1976, who had meningomyelocele and hydrocephalus and were treated at the University of Rochester Medical Center.
METHODS: Grip and pinch strength were measured between July, 1991, and June, 2003. RESULTS: Mean grip and pinch strengths were similar to those found in previous studies of children with meningomyelocele; 58 neurological events occurred in 39 (40%) individuals. These included 31 episodes of ventricular shunt failure and 22 symptomatic tethered cord occurrences. Specificity, sensitivity and likelihood ratios were calculated in multiple ways using different criteria for loss of grip or pinch strength and for interval to neurological event. Sensitivities were low (<0.35) and the highest positive likelihood ratio found, using fall in either lateral pinch with 6 months to neurological event, was 2.3.
CONCLUSIONS: Despite previous recommendations, grip and pinch measurements were not helpful when used as a routine screening test for neurological dysfunction for children with meningomyelocele and hydrocephalus.
PMID: 16714860
BACKGROUND: Neurological complications occur commonly in children with meningomyelocele and can cause significant morbidity and mortality. An earlier study suggested that acute changes in grip and pinch strength could be used to identify individuals at increased risk for developing a neurological complication.
OBJECTIVE: To evaluate the use of grip and pinch measurements to screen for neurological problems in children with spina bifida and hydrocephalus.
DESIGN: A prospective evaluation of screening tests.
PATIENTS: 92 children, born since 1976, who had meningomyelocele and hydrocephalus and were treated at the University of Rochester Medical Center.
METHODS: Grip and pinch strength were measured between July, 1991, and June, 2003. RESULTS: Mean grip and pinch strengths were similar to those found in previous studies of children with meningomyelocele; 58 neurological events occurred in 39 (40%) individuals. These included 31 episodes of ventricular shunt failure and 22 symptomatic tethered cord occurrences. Specificity, sensitivity and likelihood ratios were calculated in multiple ways using different criteria for loss of grip or pinch strength and for interval to neurological event. Sensitivities were low (<0.35) and the highest positive likelihood ratio found, using fall in either lateral pinch with 6 months to neurological event, was 2.3.
CONCLUSIONS: Despite previous recommendations, grip and pinch measurements were not helpful when used as a routine screening test for neurological dysfunction for children with meningomyelocele and hydrocephalus.
PMID: 16714860
Seven distinct coexistent cranial and spinal anomalies
Emmez H, Tokgoz N, Dogulu F, Yilmaz MB, Kale A, Baykaner MK. Seven distinct coexistent cranial and spinal anomalies. Pediatric Neurosurgery. 2006;42(5):316-9.
Existence of multiple cranial and spinal anomalies in spina bifida is well known; however, coexistence of seven different severe anomalies is extremely rare. The location of the anomalies, the patient's age and presentation are other interesting aspects of the presented case.
CASE REPORT: A 1-year-old girl with an enlarged head and big, infected lumbosacral myelomeningocele (MMC) was admitted to the emergency department in a comatose state. Further investigations revealed hydrocephalus, Chiari malformation, syringohydromyelia, split cord malformation, dermal sinus tract, lumbosacral MMC, and tethered cord. Unfortunately, the patient died 3 days after admission because of meningitis and sepsis.
DISCUSSION: Coexistence of seven different craniospinal anomalies is extremely unusual. Further investigations with magnetic resonance imaging for associated anomalies, early shunt placement and MMC repair are required to prevent this life-threatening condition in spina bifida.
PMID: 16902346
Existence of multiple cranial and spinal anomalies in spina bifida is well known; however, coexistence of seven different severe anomalies is extremely rare. The location of the anomalies, the patient's age and presentation are other interesting aspects of the presented case.
CASE REPORT: A 1-year-old girl with an enlarged head and big, infected lumbosacral myelomeningocele (MMC) was admitted to the emergency department in a comatose state. Further investigations revealed hydrocephalus, Chiari malformation, syringohydromyelia, split cord malformation, dermal sinus tract, lumbosacral MMC, and tethered cord. Unfortunately, the patient died 3 days after admission because of meningitis and sepsis.
DISCUSSION: Coexistence of seven different craniospinal anomalies is extremely unusual. Further investigations with magnetic resonance imaging for associated anomalies, early shunt placement and MMC repair are required to prevent this life-threatening condition in spina bifida.
PMID: 16902346
Spina bifida outcome: a 25-year prospective
Bowman RM, McLone DG, Grant JA, Tomita T, Ito JA. Spina bifida outcome: a 25-year prospective. Pediatric Neurosurgery. 2001 Mar;34(3):114-20.
BACKGROUND: Open spina bifida is the most complex congenital abnormality compatible with long-term survival. This report outlines the 20- to 25-year outcome for our original cohort of patients with a myelomeningocele treated in a nonselective, prospective manner.
METHODS: Of the initial 118 children, 71 patients were available for our most recent review. Nineteen patients have been lost to follow-up and 28 patients have died. Data were collected on: motor level, shunt status, education/employment, seizure history, mobility, bladder/bowel continence, tethered cord, scoliosis, latex allergy, posterior cervical decompression, tracheostomy and/or gastrostomy tube.
RESULTS: Mortality (24%) continues to climb into young adulthood. Eighty-six percent of the cohort have cerebrospinal fluid diversion, with 95% having undergone at least one shunt revision. Thirty-two percent have undergone a tethered cord release, with 97% having an improvement or stabilization in their preoperative symptoms. Forty-nine percent have scoliosis, with 43% eventually requiring a spinal fusion. Sixteen patients (23%) have had at least one seizure. Eighty-five percent are attending or have graduated from high school and/or college. More than 80% of young adults have social bladder continence. Approximately 1/3 of patients are allergic to latex, with 6 patients having experienced a life-threatening reaction.
CONCLUSION: At least 75% of children born with a myelomeningocele can be expected to reach their early adult years. Late deterioration is common. One of the greatest challenges in medicine today is establishing a network of care for these adults with spina bifida. Copyright 2001 S. Karger AG, Basel
PMID: 11359098
BACKGROUND: Open spina bifida is the most complex congenital abnormality compatible with long-term survival. This report outlines the 20- to 25-year outcome for our original cohort of patients with a myelomeningocele treated in a nonselective, prospective manner.
METHODS: Of the initial 118 children, 71 patients were available for our most recent review. Nineteen patients have been lost to follow-up and 28 patients have died. Data were collected on: motor level, shunt status, education/employment, seizure history, mobility, bladder/bowel continence, tethered cord, scoliosis, latex allergy, posterior cervical decompression, tracheostomy and/or gastrostomy tube.
RESULTS: Mortality (24%) continues to climb into young adulthood. Eighty-six percent of the cohort have cerebrospinal fluid diversion, with 95% having undergone at least one shunt revision. Thirty-two percent have undergone a tethered cord release, with 97% having an improvement or stabilization in their preoperative symptoms. Forty-nine percent have scoliosis, with 43% eventually requiring a spinal fusion. Sixteen patients (23%) have had at least one seizure. Eighty-five percent are attending or have graduated from high school and/or college. More than 80% of young adults have social bladder continence. Approximately 1/3 of patients are allergic to latex, with 6 patients having experienced a life-threatening reaction.
CONCLUSION: At least 75% of children born with a myelomeningocele can be expected to reach their early adult years. Late deterioration is common. One of the greatest challenges in medicine today is establishing a network of care for these adults with spina bifida. Copyright 2001 S. Karger AG, Basel
PMID: 11359098
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