Magnetic resonance imaging of the anal sphincter and spine in patients with anorectal malformations after posterior sagittal anorectoplasty: a late follow-up cross-sectional study.

PURPOSE: We aimed to assess the association of fecal incontinence to the anatomy of the anal sphincter complex and lower bony spinal anomalies as investigated with magnetic resonance imaging (MRI) in adolescents and adults with anorectal malformations (ARM) after posterior sagittal anorectoplasty (PSARP). METHODS: We conducted a cross-sectional study in 20 patients with ARM after PSARP. Anatomy of the anorectum and spine were examined with MRI and functional outcome assessed with the Wexner incontinence score. RESULTS: We included 20 patient (12 males) had a median age of 19.5 years (14-27). One patient was excluded leaving 19 patients for outcome analysis. Fecal incontinence was found in 12 out of 19 patients (63%). Interposed fat was present in 9 patients (47%). The presence (r = 0.597, p = 0.012) and thickness of interposed fat (r = 0.832, p = 0.005) between the anal sphincter complex and bowel were positively correlated to the Wexner fecal incontinence score. No correlation was found between lower bony spinal anomalies and fecal incontinence. CONCLUSIONS: A positive correlation between interposed fat and higher Wexner fecal incontinence score was found indicating a more severe fecal incontinence but no other correlation between anatomy of the anal sphincter complex and neorectum to functional bowel outcome was observed.

Polyethylene Glycol Maintenance Treatment for Childhood Functional Constipation: A Randomized, Placebo-controlled Trial.

OBJECTIVE: The aim of this study was to investigate the long-term efficacy of polyethylene glycol (PEG) during maintenance treatment of childhood functional constipation (FC) in a randomized, double-blinded, placebo-controlled trial. METHODS: Children (2-16 years) with FC according to the Rome III criteria were randomly assigned to maintenance treatment with PEG or placebo for 24 weeks. Children reporting treatment failure before 24 weeks were switched to conventional treatment. Primary outcome was successful treatment, defined as absence of any Rome III criteria with or without use of medication after 24 weeks. RESULTS: A total of 102 children were included: PEG/placebo: 49/53. At 24 weeks, significantly more patients in the PEG group, compared to the placebo group, were successfully treated (33 [67%] vs 19 [36%] hazard ratio (95% confidence intervals) = 3.21 [1.73-5.94]). Significantly fewer children in the PEG group switched to rescue medication (2 [4%] vs 30 [57%], P < 0.001). Time before the change to rescue medication was 13 and 27 days, respectively, for each of the 2 children in the PEG group who required rescue medication. Median time to shift to rescue medication was 27 days (range: 3-64 days) in the placebo group. At 24 weeks after initiation of treatment, 33 children (67%) in the PEG group were successfully treated, compared to 19 children (32%) in the placebo group (hazard ratio (95% confidence intervals) = 3.21 [1.73-5.94]). No serious adverse event related to use of the medication was registered. CONCLUSIONS: Maintenance treatment with PEG is significantly more effective than placebo in preventing relapse of constipation symptoms during long-term maintenance treatment in childhood FC. We therefore recommend that maintenance treatment commence after disimpaction.

Conventional treatment of functional constipation has a positive impact on the behavioural difficulties in children with and without faecal incontinence.

AIM: Constipation studies have only evaluated behavioural difficulties in children with faecal incontinence. This study evaluated changes in behavioural difficulties in childhood with functional constipation (FC) with and without faecal incontinence, based on treatment outcomes. METHODS: Children aged five to 16 years who fulfilled the Rome III criteria for FC received conventional treatment. The Strength and Difficulties Questionnaire was completed at inclusion and at the 12-month follow-up. RESULTS: We included 116 children. The behaviour scores decreased in successfully treated boys (10.3 versus 7.9; p < 0.001) and girls (10.0 versus 7.4; p = 0.0001) with and without faecal incontinence. There was no decrease in the behaviour scores in children with unsuccessful outcomes. Unsuccessfully treated boys had significantly higher behaviour scores than successfully treated boys at inclusion (13.2 versus 10.3; p = 0.006) and after 12 months (11.4 versus 7.9; p = 0.02). No difference was found between unsuccessfully treated and successfully treated girls at inclusion (10.5 versus 10.0; p = 0.77) or after 12 months (10.3 versus 7.4; p = 0.18). CONCLUSION: Our findings indicate that conventional treatment of FC had a positive impact on behavioural difficulties in constipated children with and without faecal incontinence. This study highlights the importance of proactive detection and treatment of FC in paediatric patients.

Low-fat, high-carbohydrate parenteral nutrition (PN) may potentially reverse liver disease in long-term PN-dependent infants.

INTRODUCTION: Parenteral nutrition-associated cholestasis (PNAC) is a complication of long-term parenteral nutrition (PN). Removal of lipids may reverse PNAC but compromises the energy to ensure infant growth. The purpose of this study was to test whether a low-fat, high-carbohydrate PN regimen, which prevents and reverses PNAC in adults, could do the same in infants. This regimen could potentially avoid the problem of diminished energy input after removing nutritional lipids. METHODS: Infants developing PNAC over a 2-year period were started on a low-fat PN regimen with calories primarily from carbohydrates. The fat-free PN, containing 314 kJ/ml, was provided 5-6 times a week and fat, including essential fatty acids and fat-soluble vitamins, 1-2 times a week. Enteral feeding was continued according to individual tolerance. RESULTS: The study included 10 infants with short bowel syndrome (six with intestinal failure due to necrotizing enterocolitis, one with gastroschisis, one with complications due to unrecognized anal atresia and two with midgut volvulus). Median duration of PN with fat before initiating the low-fat regime was 69 days (25-75 % percentile: 41-75 days), and mean s-bilirubin was 139 µmol/l (range 87-323 µmol/l). Median duration with low-fat regimen was 69 days (25-75 % percentile: 18-123 days). Bilirubin reversed to normal (<50 µmol/l) in all infants. Seven children showed catch-up growth. No essential fatty acid deficiency, steatosis or deaths were observed. CONCLUSIONS: A low-fat, high-carbohydrate PN regimen together with enteral feeding is well tolerated and may be used in reversing liver disease in PN-dependent infants without compromising growth.

Identifying faecal impaction is important for ensuring the timely diagnosis of childhood functional constipation.

AIM: Most research on functional constipation has been carried out at a tertiary level. We focused this study on a secondary-level hospital outpatients' department, assessing the distribution of diagnostic criteria for childhood functional constipation and evaluating the consequences of current diagnostic practice based on current guidelines. METHODS: We enrolled 235 children, aged two to 16 years of age, with functional constipation according to the Rome III criteria and assessed them using medical histories and physical examinations, including rectal examinations and ultrasound measurements of rectal diameter. Faecal impaction was a rectal diameter exceeding 3 cm. RESULTS: The most frequently reported symptom was painful bowel movements (72.3%) and 39.1% had faecal incontinence. Rectal examination identified faecal impaction in 149 children (66.2%), and ultrasound showed a mean rectal diameter of 3.4 ± 0.6 cm, compared to 2.2 ± 0.6 cm in children without impaction (p < 0.001). Of the 149 children with faecal impaction, 19 (12.8%) only had one additional Rome III criterion. The rectal diameter exceeded 3 cm in 79.9% of cases. CONCLUSION: Identifying faecal impaction is important for ensuring the timely diagnosis of childhood functional constipation at the secondary care level. Ultrasound examination proved a reliable alternative to rectal examination or abdominal radiography when identifying faecal impaction.

Dyssynergic patterns of defecation in constipated adolescents and young adults with anorectal malformations.

We aimed to evaluate the etiologies of constipation in patients with anorectal malformations having a good prognosis for bowel control but a high risk of constipation. We included twenty-five patients from the Odense university hospital in Denmark. Patients were subjected to colon transit time examination and high resolution anorectal manometry (HRAM). The median age was 18 (14-24) and 48% (12/25) were females. Fifty-two % (13/25) of patients were diagnosed with constipation. Types of anorectal malformation were perineal fistula (9/25), rectovestibular fistula (8/25), rectourethral bulbar fistula (5/25) and no fistula (3/25). No difference in neither total colon transit time nor segmental colon transit times were found based on the presence of constipation. Only four of the constipated patients fulfilled criteria for dyssynergic defecation with a dyssynergic pattern at HRAM and prolonged colon transit time. A Type I dyssynergic pattern was dominant in constipated patients (7/13). A Dyssynergic defecation pattern was due to isolated contraction of puborectalis muscle in 9 out of 13constipated patients. We found a dyssynergic pattern during attempted defecation in patients with anorectal malformations disregarded the presence of constipation. In the majority of constipated patients an isolated contraction of the puborectalis muscle was visualized with HRAM.

Impact of Spinal Defects on Urinary and Sexual Outcome in Adults With Anorectal Malformations-A Cross-sectional Study.

OBJECTIVE: To examine the impact of spinal defects on urinary function, sexual outcome and quality of life in adult patients born with anorectal malformations. MATERIALS AND METHODS: A prospective cohort study at Odense University Hospital in Denmark was conducted. From 1985 to 2000, 93 patients were found eligible for participation. Twenty-seven (29%) consented to participate. One patient refrained from clinical examinations. Patients were examined with magnetic resonance imaging, uroflowmetry, and validated questionnaires on urinary function, sexual function, and quality of life. RESULTS: There were 14 were females and 13 were males, median age of 25 (range 19-31) years and 23 (18-32) years, respectively. The type of anorectal malformations in females were vestibular fistula (n = 6), anocutaneous fistula (n = 4), anal stenosis (n = 3), and cloaca (n = 1). In males the type of malformations were anocutaneous fistula (n = 4), bulbar fistula (n = 4), rectovesical fistula (n = 2), anal stenosis (n = 1), rectal atresia (n = 1), and anal atresia with no fistula (n = 1). Patients with spinal defects had a lower average voiding rate compared to patients with normal spinal anatomy (P .03), a lower voiding-related quality of life (P .02), and a tendency was observed toward a worse total urinary incontinence-related quality of life score (P .06). Moreover in patients with spinal defect a tendency was seen toward a worse general quality of life (P .09). CONCLUSION: Spinal defects detected by magnetic resonance imaging in adults with anorectal malformations were found to be associated with urinary voiding function.

Congenital Abdominal Wall Defects: Staged closure by Dual Mesh.

OBJECTIVE: To evaluate the clinical utility of GORE® DUALMESH (GDM) in the staged closure of large congenital abdominal wall defects. MATERIALS AND METHODS: Data of patients with congenital abdominal wall defects managed with GDM was analyzed for outcome regarding complete fascial closure; mesh related complications; and post-discharge gastrointestinal surgery. RESULTS: GDM was placed in 34 (gastroschisis=27, omphalocele=7) patients during the study period. Complete closure of the fascia was obtained in one patient with omphalocele and in 22 patients with gastroschisis. Mesh related surgical complications were seen in five (15%) children: four had detachment of the mesh and one patient developed abdominal compartment syndrome. Mesh related clinical infection was observed in five children. In hospital mortality occurred in four cases (2 gastroschisis and 2 omphalocele) and was not procedure-related. Of the 30 children discharged, 28 (82%) were still alive. At follow-up, three patients (10%) were operated for a minor ventral hernia and 4 children were operated (laparotomy and adhesionolysis) for adhesive intestinal obstruction. CONCLUSION: Staged closure with GDM is a safe alternative when primary fascial closure is difficult.