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Purpose: To evaluate bowel function in patients with anorectal malformations (ARM) comparing existing scoring systems., Methods: Parents of ARM patients treated at our institution were asked to fill in Holschneider, Kricknebeck, and Rintala questionnaires. Scores obtained from the questionnaires were expressed per cent and analyzed depending on the age and type of ARM according to Krickenbeck classification. Patients younger than 3 years of age or with developmental delay were excluded., Results: Eighty patients (42 males: 52%) were included. Median age was 7.6 years (range 3-22). Twenty eight patients (35%) had perineal fistula, 13 (16%) bulbar, 7 (9%) prostatic, 5 (6%) rectobladder neck, 15 (19%) vestibular, 7 (9%) had a cloaca and 5 (6%) imperforate anus without fistula. Using Holschneider, Krickenbeck, and Rintala, average scores were respectively 72, 71 and 73 (p = 0.4 with ANOVA). Using the three questionnaires patients with perineal fistula scored 82, 76 and 84 respectively (p = 0.003), with bulbar 70, 71, 73 (p = 0.8), with prostatic 52,69,59 (p = 0.06), with bladder neck 56, 80, 57 (p = 0.004), with vestibular 75,67,75 (p = 0.02), with cloaca 64, 67, 65 (p = 0.9), and with imperforate anus without fistula 61,49, 53 (p = 0.12). Patients from 3 to 6 years of age scored 74,72 and 76 (p = 0.37), from 7 to 12: 70,71 and 71 (p = 0.87), and older than twelve: 74,66 e 73 (p = 0.08)., Conclusion: The scores obtained using Holschneider, Rintala, and Krickenbeck questionnaires are significantly lower with increasing severity of the ARM. For each type of ARM there are some differences in the results obtained using the three questionnaires. In general, Krickenbeck and Peña questionnaires tend to give lower scores in patients with ARMs that have good prognosis, and higher scores for ARMs with poor prognosis. Age is not significantly related to the score obtained., Level of Evidence: III TYPE OF STUDY: Diagnostic study., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Aim: In paediatric and adult patients with neurogenic bowel, transanal irrigation (TAI) of the colon has gained popularity due to the introduction of a specifically designed device. The aim of this pilot study was to present the results of TAI using the Peristeen(®) TAI system in a group of paediatric patients with anorectal malformation (ARM) and congenital or acquired spinal cord lesions (SCLs)., Method: Eight Italian paediatric surgery and spina bifida centres participated in the study. The inclusion criteria were age between 6 and 17 years, weight above 20 kg and unsatisfactory bowel management. Patients with chronic inflammatory bowel disease, mental disability and surgery within the previous 3 months were excluded. At the beginning of treatment (T0) and after 3 months (T1) the Bristol scale, a questionnaire assessing bowel function, and two questionnaires on quality of life (QoL) for patients aged 6-11 years (CHQ-pf50) and 12-17 years (SF36) were administered., Results: Eighty-three patients were enrolled, and seventy-eight completed the study (41 ARMs, 37 SCLs). At T1, constipation was reduced in ARMs from 69% to 25.6% and in SCLs from 92.7% to 41.5%, faecal incontinence in ARMs from 50% to 18.6% and in SCLs from 39% to 9.8% and flatus incontinence in ARMs from 20.9% to 9.8% and in SCLs from 31.7% to 10%. At T0, the Bristol Stool Scale types were 1-2 in 45% of ARMs and 77.5% of SCL patients, whereas at T1 types 1-2 were recorded in only 2.5% of SCL patients. QoL improved in both groups. In the younger group, a significant improvement in QoL was recorded in ARM patients for eight of nine variables and in SCL patients for seven of nine variables., Conclusion: This study showed that Peristeen TAI resulted in a significant time reduction in colonic cleansing, increased independence from the carer and improved QoL in paediatric patients with ARMs and SCLs., (Colorectal Disease © 2015 The Association of Coloproctology of Great Britain and Ireland.)

Background/purpose: Anorectal malformations (ARM) are a spectrum of defects ranging from the very minor ones, with an excellent functional prognosis, to those that are more complex, often associated with other anomalies, difficult to manage and with a poor functional outcome. A significant number of these children suffer from fecal and urinary incontinence despite major advances in the management of ARM patients have significantly improved the quality of life. The role of sacrovertebral anomalies/dysraphism (SD) and neurospinal cord anomalies/dysraphism (ND) associated with ARM on the continence of these patients is still controversial. The authors made a review of their experience in a period of 5 years, focusing on the role of neurospinal cord anomalies in patients with ARM., Materials: At colorectal clinic of our department of pediatric surgery 215 patients who underwent a procedure of posterior sagittal anorectoplasty for ARM are followed-up in a multidisciplinary clinic. Among them 60 patients with either SD or ND were documented. In 37 patients the anomaly involved the spinal cord (ND). 12 of these 37 patients underwent neurosurgical treatment and 25 were managed conservatively. Data collected from their follow-up were analyzed and compared, focusing on their bowel and urinary continence., Results: All 37 patients acquired regular bowel movements with an appropriate bowel management according to Peña's protocols. Urinary incontinence required clean intermittent catheterization in four cases. None of the patients who did not receive neurosurgical treatment developed acute complications due to the progression of the neurospinal anomaly, like acute urinary retention, orthopedic and motility problems or acute hydrocephalus. From literature review we were unable to find good evidence that the presence of ND worsens the functional prognosis of patients with ARM. We were also unable to find convincing evidence to support the practice of prophylactic neurosurgical procedures., Conclusions: The present study supports the theory that for ARM patients the prognosis in terms of continence depends mainly on the type of malformation and is not complicated by the association with ND. In our series neurosurgical treatments did not have any effect in improving the continence of ARM patients and a conservative management of ND did not expose the patients to the sequelae of progressive deterioration, reported elsewhere, requiring rescue neurosurgery. We believe that the correct practice of pediatric surgeons following-up ARM patients is a protocol which includes appropriate investigations to detect the presence of a SD or ND and, once these entities are detected, it is mandatory to manage the patient with a multidisciplinary team, where a conservative non-operative management is initially justified and advocated in the absence of neurosensorymotor symptoms.