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: Megarectosigmoid (MRS) is commonly seen in children with anorectal malformations (ARM) and contributes to the high incidence of constipation. Surgical resection has been advocated by some, whereas others propose intense bowel management as the treatment of choice. The aim of this study was to evaluate outcome of both bowel function and configuration after surgical or conservative treatment of MRS in ARM patients. The study included 79 patients with ARM, excluding perineal fistula, (48 boys, 31 girls) from 1986 to 2007. MRS was diagnosed at colostomy formation or contrast enema performed in the neonatal period. Early in the period, the majority of the patients were treated surgically, whereas in the late 1990s, a conservative approach with intensified bowel treatment was implemented. Contrast enema and bowel function investigations were performed repeatedly during follow-up. MRS, according to radiological criteria, was diagnosed in 26/79 (33%) of the ARM children. Bowel functional outcome was similar regardless of surgical or conservative treatment and comparable to function in ARM children with non-MRS. The radiological signs of rectal dilatation and elongation disappeared after surgical intervention, but normalisation of the rectosigmoidal configuration was also seen with age in the conservative group. Bowel functional outcome in ARM children with MRS was similar after either surgical or conservative treatment during follow-up. The radiological signs of rectal dilatation and elongation disappeared also in the conservatively treated patients over time.Journal of Pediatric Surgery 04/2014; 49(4):564-9. · 1.38 Impact Factor
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ABSTRACT: Anteriorly displaced anus is an anomaly that is debated with regard to its nomenclature, diagnosis and management. To describe MRI anatomy of the anal canal in children with anteriorly displaced anus and its impact on the process of defecation. We prospectively examined ten children (7 girls, 3 boys; age range 7 months to 8 years, mean 3 years) with anteriorly displaced anus between August 2009 and April 2012. Noncontrast MRI examinations were performed on a 1.5-T magnet. T1- and T2-weighted turbo spin-echo images were acquired in axial, sagittal and coronal planes of the pelvis. The anorectal angle and the relative hiatal distance were measured in mid-sagittal images, and compared with those of a control group using the Mann-Whitney test. In children with anteriorly displaced anus, no anatomical abnormality was depicted at the level of the proximal anal canal. However, the distal anal canal was displaced anteriorly, running out its external muscle cuff, which remained un-displaced at the usual site of the anus. This changes the orientation of the central axis of the anal canal by passing across instead of along the fibers of the longitudinal muscle coat. Children with anteriorly displaced anus had a more obtuse anorectal angle (mean 112.1°), which was significantly greater than that of the control group (mean 86.2°). MRI is a valuable tool in studying the anatomy of the anal canal in children with anteriorly displaced anus. The abnormal orientation of the longitudinal muscle across the anal canal can explain the obstructed defecation in these children. Based on this study, it might be of interest to use MRI in studying equivocal cases and children with unexplained constipation.Pediatric Radiology 04/2014; · 1.57 Impact Factor
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ABSTRACT: The aim of this study is to compare the long term outcomes between laparoscopic-assisted anorectoplasty (LAARP) and posterior sagittal anorectoplasty (PSARP) for children with rectobladderneck and rectoprostatic fistula anorectal malformations (ARM). Thirty-two ARM children with rectobladderneck and rectoprostatic fistula who underwent LAARP between October 2001 and March 2012 were reviewed. The outcomes were compared with those of 34 ARM children who underwent PSARP between August 1992 and September 2001. The sacral ratio (SR), age at operation, operative time, postoperative hospital stay and complications were evaluated. Bowel functions were assessed using the Krickenbeck classification. The mean operative time of the LAARP was significantly shorter than that of PSARP group (1.62±0.40 vs 2.13±0.30h). The postoperative hospital stay was significantly shorter in the LAARP group (5.8±0.65 vs 8.4±0.67h). The wound infections (11.8% vs 0%) and recurrent fistula (11.8% vs 0%) were more common in PSARP patients. The overall morbidity rate of PSARP group was significantly higher than that of the LAARP group (35.3% vs 12.5%, p<0.05). However, 7.5% of the LAARP patients developed rectal prolapse. Twenty-four of 32 patients were followed up for more than 3years in LAARP group. The median follow up period was 7.5years (range 4-11) in LAARP patients and 15.5years (range 11-20) in PSARP patients. The rates of voluntary bowel movement, soiling (grade 1, 2 & 3) were similar in both groups. More patients from PSARP group developed grade 2 or 3 constipation (22.5% vs 0%, P<0.01). Compared to PSARP, LAARP is a less invasive procedure. The long term functional outcomes after LAARP were equivalent if not better than those of PSARP.Journal of Pediatric Surgery 04/2014; 49(4):560-3. · 1.38 Impact Factor