Preliminary report on the International Conference for the Development of Standards for the Treatment of Anorectal Malformations.
ABSTRACT Anorectal malformations (ARM) are common congenital anomalies seen throughout the world. Comparison of outcome data has been hindered because of confusion related to classification and assessment systems.
The goals of the Krinkenbeck Conference on ARM was to develop standards for an International Classification of ARM based on a modification of fistula type and adding rare and regional variants, and design a system for comparable follow up studies.
Lesions were classified into major clinical groups based on the fistula location (perineal, recto-urethral, recto-vesical, vestibular), cloacal lesions, those with no fistula and anal stenosis. Rare and regional variants included pouch colon, rectal atresia or stenosis, rectovaginal fistula, H-fistula and others. Groups would be analyzed according to the type of procedure performed stratified for confounding associated conditions such as sacral anomalies and tethered cord. A standard method for postoperative assessment of continence was determined.
A new International diagnostic classification system, operative groupings and a method of postoperative assessment of continence was developed by consensus of a large contingent of participants experienced in the management of patients with ARM. These methods should allow for a common standardization of diagnosis and comparing postoperative results.
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ABSTRACT: PURPOSE: Longitudinal follow-up of changes in bowel function in children with anorectal malformations (ARMs) with or without spinal cord pathology and neurogenic bladder dysfunction (NBD) as they grow. Another purpose was to identify predictors influencing bowel functional outcome. MATERIAL AND METHODS: The study included 41 patients with ARM, excluding perineal fistula (21 boys and 20 girls). Bowel function was evaluated at ages 5, 10 and 15years using a structured questionnaire and a three-week registration of number and time of bowel movements, episodes of fecal leakage and soiling. Additional bowel treatment with enemas and stool softeners and use of diapers were recorded. A group of 52 healthy boys and girls was used as control. RESULTS: A successive improvement in functional outcome with age in children with ARM and normal spinal cord was seen with respect to continence, soiling and constipation. Continence was achieved earlier in girls than in boys (at 10years: girls 80%, boys 36%). Soiling and constipation decreased with age both in grade and frequency (at 10years low grade soiling: girls 53%, boys 64%). Boys with spinal cord malformation with NBD in combination with prostatic/bladder neck fistula (PRF/BNF) and sacral agenesis had the worst functional outcome with minimal possibility of improvement over time. Functional outcome in girls with NBD and tethered cord did not differ significantly from those without NBD and with a normal spinal cord. Psychosocial co-morbidity, neuropsychiatric disorders, developmental delay and megarectosigmoid were also risk factors impeding the functional outcome. CONCLUSION: In the present study there was a successive improvement in bowel function during childhood and adolescence in ARM children, but they did not achieve the level of healthy children. NBD, spinal cord malformation, sacral malformation and PRF all were negative predictive factors for bowel score at 5years.Journal of Pediatric Surgery 03/2013; 48(3):597-606. · 1.38 Impact Factor
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ABSTRACT: The goal of surgery for anorectal malformations (ARM) is to achieve good bowel, urinary, and sexual functions, as well as the ability for children to become healthy adults. Various surgical procedures and surgical management protocols have been explored or devised by pediatric surgeons. These are described in this review. Making a correct type classification by invertography, fistelography and urethrography in the neonatal period allows pediatric surgeons to select an appropriate surgical strategy. Surgery for low-type malformations is principally neonatal perineoplasty, while that for intermediate- or high-type malformations is colostomy, followed by a pull-through operation during infancy. Posterior sagittal anorectoplasty or laparoscopy-assisted surgery has recently been accepted as alternative procedures. Fecal incontinence represents a devastating problem that often prevents a patient from becoming socially accepted and may cause serious psychological sequelae. One-third of adult patients with high- or intermediate-type malformations occasionally complain of fecal incontinence after surgery. Most patients with ARM have normal urinary function if they do not have urinary tract or sacral anomalies. These associated anomalies also influence the prognosis for sexual function, especially in males. Some female patients have experienced normal vaginal delivery and had children. In patients with cloacal malformation, however, fertility or sexual problems are also often present. Based on this information, it is clear that only well-planned and systemic treatments can provide a good functional prognosis after making a correct classification in the neonatal period.Surgery Today 11/2012; · 0.96 Impact Factor
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ABSTRACT: BACKGROUND: Anorectal malformations are often associated with rectal pouch fistulas. Surgical correction requires accurate evaluation of the presence and position of such fistulas. Fluoroscopy is currently the chosen modality for the detection of fistulas. The role of MRI is unexplored. OBJECTIVE: To compare the diagnostic accuracy of MR versus fluoroscopic fistulography in the pre-operative evaluation of infants with anorectal malformation. MATERIALS AND METHODS: We conducted a pilot study of infants requiring defunctioning colostomy for initial management of anorectal malformation. Dynamic sagittal steady-state free-precession MRI of the pelvis was acquired during introduction of saline into the mucous fistulas. Findings were compared among MR fistulography, fluoroscopic fistulography and intraoperative inspection. RESULTS: Eight children were included. Median age at fistulography was 15 weeks, inter-quartile range 13-20 weeks; all were boys. There was full agreement among MR fistulography, fluoroscopic fistulography and surgical findings. CONCLUSION: The pilot data suggest that MR fistulography is promising in the pre-operative evaluation of children with anorectal malformation.Pediatric Radiology 03/2013; · 1.57 Impact Factor