Functional Outcomes of Patients Who Underwent Anorectal Malformation Repair Using MRI Guidance.

BACKGROUND: Despite the initiation of minimally invasive laparoscopic techniques, the majority of patients who undergo anorectal malformation repair still experience functional bowel issues in childhood, including constipation and fecal incontinence. In this study, we evaluate the functional outcomes of a procedure in which magnetic resonance imaging guidance is used during initial laparoscopic repair to better locate the epicenter of the sphincter muscle complex and pelvic floor with the goal of more accurate placement of the neoanus and improved functional outcomes. METHODS: A retrospective chart review evaluated demographic, operative, and outcome details for patients who underwent this procedure. A telephone survey was employed to determine levels of social continence using the validated Baylor Continence Scale and to determine what type of bowel management is used. RESULTS: Twenty-six patients were included. Median age at operation was 7 months, and median age at follow-up was 4 years old, with a range of 1-9. Bowel management regimen results revealed that 19 % (n = 5) use no bowel management regimen, 58 % (n = 15) use laxatives only, and 23 % (n = 6) use enemas. Enema use was not associated with different spine or sacral anomalies (p = 0.77). Fifteen patients (58 %) answered the Baylor Continence Scale questions and had a median score of 14. No difference was found in scores when accounting for lesion level (p = 0.43), quality of needle placement (p = 0.46), or quality of sphincter muscles (p = 0.75). CONCLUSIONS: Using MRI guidance in the repair of anorectal malformations shows promise in both the qualitative and quantitative functional outcomes of this complex patient population. LEVEL OF EVIDENCE: Level III.

Rectal atresia and rectal stenosis: the ARM-Net Consortium experience.

PURPOSE: To assess the number, characteristics, and functional short-, and midterm outcomes of patients with rectal atresia (RA) and stenosis (RS) in the ARM-Net registry. METHODS: Patients with RA/RS were retrieved from the ARM-Net registry. Patient characteristics, associated anomalies, surgical approach, and functional bowel outcomes at 1 and 5-year follow-up were assessed. RESULTS: The ARM-Net registry included 2619 patients, of whom 36 (1.3%) had RA/RS. Median age at follow-up was 7.0 years (IQR 2.3-9.0). Twenty-three patients (63.9%, RA n = 13, RS n = 10) had additional anomalies. PSARP was the most performed reconstructive surgery for both RA (n = 9) and RS (n = 6) patients. At 1-year follow-up, 11/24 patients with known data (45.8%, RA n = 5, RS n = 6) were constipated, of whom 9 required stool softeners and/or laxatives. At 5-year follow-up, 8/9 patients with known data (88.9%, RA n = 4, RS n = 4) were constipated, all requiring laxatives and/or enema. CONCLUSION: RA and RS are rare types of ARM, representing 1.3% of patients in the ARM-Net registry. Additional anomalies were present in majority of patients. Different surgical approaches were performed as reconstructive treatment, with constipation occurring in 46% and 89% of the patients at 1 and 5-year follow-up. However, accurate evaluation of long-term functional outcomes remains challenging.

A case of Currarino syndrome in an adult female presenting with refractory chronic constipation.

We present the case of a 25-year-old female who presented due to refractory chronic constipation and fecal incontinence. She had bowel movements every 7-30 days with an increased consistency (1-2 Bristol type stools), together with soiling and passive fecal incontinence (Wexner Scale: 12/20). She had previously undergone surgery shortly after birth for an anorectal malformation repair. The colonoscopy and histological study of the rectum were normal. A pelvic magnetic resonance imaging (MRI) was performed, which showed a right pararectal mass that compressed the rectum without invading it. This mass was compatible with a presacral teratoma or hamartoma. MRI also revealed coccyx agenesis and hypoplasia of the last sacral vertebrae (Image 1), which were consistent with Currarino syndrome (CS). The patient received 14 sessions of transcutaneous electrostimulation of the posterior tibial nerve, resulting in an increase in bowel movements (every 3 days) and a reduction in fecal incontinence. She was then referred to surgery for presacral mass removal.

Treatment of megarectum in anorectal malformation with emphasis on preventive aspects: 17 years experience.

PURPOSE: Megarectum in anorectal malformation (ARM) causes severe morbidity. To compare conservative management (CM) of megarectum with excision (EX), to propose a new classification and to analyse management strategies. METHODS: Between 2000-2016, we reviewed all ARM to identify megarectum, defined by radiological recto-pelvic ratio > 0.61. A new classification was proposed: primary megarectum (PM) pre-anorectoplasty, and secondary megarectum (SM) post-anorectoplasty, with sub-types. Complications and Krickenbeck bowel function were compared between CM and EX. RESULTS: Of 124 ARM, 22 (18%) developed megarectum; of these, 7 underwent EX. There was no difference in functional outcomes when comparing CM vs EX-voluntary bowel movement (both 86%), soiling (40% vs. 57%) and constipation (both 86%). However, EX was associated with major complications (43%) and the requirement for invasive bowel management, compared to CM (85% vs. 27%, P = 0.02). 6/7 EX needed antegrade continence enema (ACE), one of these has a permanent ileostomy. With strategic changes, incidence of megarectum reduced from 20/77 (26%) to 2/47 (4%) after 2013 (P = 0.002). CONCLUSION: EX did not confer benefit in the functional outcome but carried a high risk of complications, often needing ACE or stoma. By adhering to strategies discussed, we reduced the incidence of megarectum and have avoided EX since 2013.

Diagnosis of Hirschsprung Disease.

Diagnosis or exclusion of Hirschsprung disease (HSCR) is a frequent exercise in any pediatric hospital. Although HSCR may present at different ages and with varied clinical findings, the most common presentation is a neonate with severe constipation or signs of intestinal obstruction. A variety of diagnostic tests including contrast enema and anorectal manometry may be used as diagnostic screens, but diagnosis ultimately rests upon histopathological evaluation of a rectal biopsy. For the experienced pathologist, conventional hematoxylin-and-eosin-stained sections often suffice to exclude HSCR or establish the diagnosis. However, ancillary diagnostic tests such as acetylcholinesterase histochemistry or calretinin immunohistochemistry are complementary and extremely helpful in some cases. In this Perspectives article, we review the clinical and pathological features of HSCR, highlight those that are found in most patients, and discuss how to address particularly challenging aspects of the diagnostic workup.

Quality of life and defecative function 10 years or longer after posterior sagittal anorectoplasty and laparoscopic-assisted anorectal pull-through for anorectal malformation.

PURPOSE: In our center, patients with anorectal malformation, including males with recto-vesical (RV)/recto-bladder neck (RBN)/recto-prostatic urethral (RU) fistulas, and females with recto-vaginal (RV) fistulas have been treated by posterior sagittal anorectoplasty (PSARP) before 2000, and by laparoscopic-assisted anorectal pull-through (LAARP) thereafter. We would like to compare the quality of life (QOL) and long-term defecative function between these two groups of patients 10 years after reconstructive surgery. METHODS: Patients who underwent LAARP between 2001 and 2005 were compared with historical controls treated with PSARP between 1996 and 2000. Degrees of continence were graded by the Krickenbeck classification and Kelly's score. QOL was assessed by Hirschsprung's disease/Anorectal Malformation Quality of Life (HAQL) questionnaire. Results were compared using Chi-square test and t test. RESULTS: There were 14 LAARP and 7 PSARP patients. All attained voluntary bowel movements. Moderate to severe soiling (Krickenbeck Grade 2 and 3) was found in 3/14 LAARP (21.4%) and 1/7 PSARP (14.3%) patients, p = 1.00. Constipation requiring use of laxatives was present in 3/14 LAARP (28.6%) and 1/7 PSARP (14.3%) patients, p = 0.62. Mean Kelly's scores were 3.79 ± 0.98 (LAARP) and 4.71 ± 1.25 (PSARP), p = 0.12. No patient required Malone antegrade continence enema (MACE). The QOL scores based on the HAQL questionnaire were comparable between the two groups in all areas except social functioning, in which the LAARP patients attained a significantly lower mean score (26.4 vs 71.7, p = 0.0001). CONCLUSION: The 10-year outcome between LAARP and PSARP patients in terms of QOL and defecative function is comparable. Impairment in social functioning in these patients is reflected by the self-reported lower level of functioning.

Outcomes of transanal endorectal pull-through for rectal atresia.

Rectal atresia is a rare anorectal malformation, and it has been reported to represent 1%-2% of all anorectal malformations. We report three newborns who were admitted to the neonatal intensive care unit for abdominal distention, bilious vomiting and failure to pass meconium. The external anus and genitalia were normal and well formed. Digital rectal examination showed a blind-ending anal canal. All three infants were initially managed with diverting colostomy and then transanal resection of the rectal atresia with primary anastomosis, followed by colostomy closure. All patients eventually developed normal bowel habits and gained complete bowel control at 3-5 years of age, with mild constipation managed with laxatives. Contrast enema in a newborn with distal bowel obstruction is helpful to delineate the anatomy to show the gaps and to facilitate the procedure. In conclusion, transanal endorectal pull-through is a feasible and safe procedure with satisfactory clinical outcomes.

Peristeen(®) transanal irrigation in paediatric patients with anorectal malformations and spinal cord lesions: a multicentre Italian study.

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.

Parental risk factors of anorectal malformations: Analysis with a regional population-based control group.

BACKGROUND: Adequate evidence on environmental risk factors for anorectal malformations (ARMs) is very limited. We assessed maternal body weight and several prenatal exposures of the parents to tobacco, pregestational diabetes, chronic cardiovascular and respiratory diseases, periconceptional folic acid and multivitamin intake. METHODS: Data from the German Network for Congenital Uro-REctal malformations (CURE-Net) were compared with data from the Malformation Monitoring Centre Saxony-Anhalt of the Otto-von-Guericke University in Magdeburg, Germany. Controls were matched to cases by gender and birth year of the child. Crude and adjusted odds ratios (95% confidence intervals) were calculated for potential risk factors using multivariable logistic regression. RESULTS: In total, 158 ARM patients and 474 healthy infants born between 1993 and 2008 in Germany were included. Maternal age at birth of ARM cases and birth plurality were significantly higher and gestational age and weight significantly lower compared with controls (p < 0.0001). We observed significantly increased risks for ARMs associated with maternal smoking before conception and the first trimester of pregnancy (odds ratio = 2.23, 95% confidence interval 1.04-4.79, p = 0.039) and maternal chronic respiratory diseases (odds ratio = 29.25, 95% confidence interval 8.22-104.14, p < 0.0001). No statistically significant increased risk or protective effect was found for the other investigated factors. CONCLUSION: This study suggests an association between the occurrence of ARMs in the offspring and periconceptional maternal smoking as well as maternal chronic respiratory diseases. In addition, there might be a sign of an association for maternal diabetes, although not statistically significant. It can be assumed that the power is far too low to provide reliable estimates.

Laparoscopic Cecostomy Tube Placement.

Fecal incontinence is a debilitating problem for many children, especially those with anorectal malformations. Historically, surgical options have included the Malone antegrade continence enema, using an appendicostomy for antegrade colonic enemas. Since the development of this procedure, multiple alternatives have been developed, including the Chait cecostomy tube. Here, we present our technique for laparoscopic cecostomy tube placement. We find that this approach has several advantages, including mobilization of the cecum to allow the tube to be placed below the waistline for optimal cosmesis and comfort, increased safety provided by direct vision of needle access to the cecum, and increased security of the cecum to the abdominal wall allowing for safer tube replacement should it become dislodged in the early postoperative period.

Cloacal reconstruction after a complex treatment of perineal haemangioma in a variant of PELVIS syndrome.

BACKGROUND: PELVIS is an acronym defining the association of perineal hemangioma, malformations of external genitalia, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus and skin tag. Eleven cases have been reported according to the Orphanet data. Acronyms of LUMBAR and SACRAL syndrome have been used and most probably represent a spectrum of the same entity. Very little is known about the success and timing of cloacal reconstruction after the treatment of hemangioma. We present a variant of PELVIS syndrome and discuss the possibilities and optimal timing of surgical reconstruction. CASE PRESENTATION: Female infant was born with persistent cloaca and multiple hemangiomas of genitals, perineal area and left thigh. Colostomy was performed after birth. In order to treat hemangioma and to make the reconstruction of cloaca possible, corticosteroid treatment orally and multiple laser treatments were performed alternating Nd:YAG laser and pulsed dye laser therapy. Cystoscopy confirmed hemangiomatosis in the mucosa of the common channel, bladder neck and septate vagina. Oral propranolol treatment was started at the age of 18 months and continued for 1 year. It induced rapid improvement of hemangiomas. Two more pulsed dye laser treatments were performed to remove residuals of hemangiomas from the perineum and genital area. Posterior sagital reconstruction by separation of the rectum, mobilization of urogenital sinus and vaginal reconstruction was performed with no major bleeding at the age of 4 years. Postoperatively, after a period of progressive rectal dilatation colostomy was closed. Girl is now 6 years old, dry day and night without residual urine and normal upper tracts. Rectal calibration is normal, fecal continence is still to be evaluated but constipation is easily manageable. CT of the spine and the perineum showed sacral dysplasia and spina bifida with lumbo-sacral lipoma and tethering of terminal filum without neurological deterioration at the moment but requiring close neurological monitoring. CONCLUSIONS: Large perineal hemangiomas are commonly associated with extracutaneous abnormalities. Successful reconstructive surgery is possible after significant reduction of hemangioma by complex treatment.

Prognostic evaluation of biofeedback response in patients treated for anorectal malformation.

PURPOSE: Functional bowel outcome in patients with anorectal malformation often is poor. For fecal incontinence resulting from sphincter dysfunction, biofeedback (BFB) training appears to be effective. The aim of study was to investigate the bowel function in incontinent children treated for ARM, using a clinical score, a manometric and pelvic magnetic resonance evaluation, in order to establish predictive parameters of response after BFB. METHODS: 25 children (median age of 6.5 years) with true fecal incontinence were evaluated by clinical score, anorectal manometry and magnetic resonance imaging (MRI). According to these evaluations patients were divided in 4 groups: group 1 (favorables manometry and MRI); group 2 (favorable manometry and unfavorable MRI); group 3 (unfavorable manometry and favorable MRI); group 4 (unfavorables manometry and MRI). All groups started a cycle of BFB and six months after end of BFB, were reevaluated by clinical score and manometry. RESULTS: The overall response to BFB was excellent in 44%, discrete in 40% and poor in 16%; a better response was found in groups 1 and 2 than groups 3 and 4. The differences between groups before BFB proportionally correlated with values after BFB; a correlation with genitourinary and spinal anomalies was found. CONCLUSIONS: Our results showed that BFB is an effective for fecal incontinence when the assessment pretreatment (functional and morphologic) is favorable; the manometry can evaluate the potential sphincterial recovery after BFB with a further prognostic benefit if correlated to morphologic evaluation with MRI.

Dynamic article: composite antropyloric valve and gracilis muscle transposition for total anorectal reconstruction: a preliminary report.

BACKGROUND: Technique and functional outcomes of anorectal reconstruction using an antropyloric graft have been reported previously. This technique had reasonable initial outcomes but lacked voluntary function. OBJECTIVE: We hereby report the initial results of patients who underwent gracilis muscle wrapping around the perineally transposed antropyloric valve in an attempt to improve voluntary fecal control. SETTING: This study was conducted at a single tertiary care institution. PATIENTS: Eight adult patients (7 men and 1 woman) with a median age of 38 years (range, 19-51 years) underwent this procedure. Seven patients already had anorectal reconstruction with a transposed antropyloric valve, and 1 patient with severely damaged anal sphincter complex underwent single-stage composite antropylorus transposition with a gracilis muscle wrap. MAIN OUTCOME MEASURES: The primary outcome measures were anatomical integrity and functional status of the composite graft in the perineum. RESULTS: No operative mortality or serious procedure-related morbidity occurred in any patient. The median postoperative resting pressure was 29 mmHg (range, 22-38 mmHg) and squeeze pressure was 72.5 mmHg (range, 45-267 mmHg). There was a significant improvement in the squeeze pressure following surgery (p = 0.039). Also, the St. Mark's incontinence scores significantly improved in all patients and varied between 7 and 9 (p = 0.003). The ability to defer defecation and the reduced frequency of leakage accidents were the prime reasons for improved postgraciloplasty outcomes in these patients. On personal interviews, all patients who underwent this procedure were satisfied with the results of their surgery. LIMITATIONS: A longer follow-up with a larger sample size is required. Quality-of-life data have not been evaluated in this study. CONCLUSIONS: Gracilis muscle wrapping around a perineally transposed antropyloric valve is possible and improves the voluntary control and overall functional outcomes in a select group of patients with end-stage fecal incontinence requiring anal replacement (Supplemental Digital Content 1, http://links.lww.com/DCR/A173).

[Descending perineum syndrome in children: Pathophysiology and diagnosis].

Objective: To propose a safer, simpler, and more exact method for the diagnosis of descending perineum syndrome (DPS). Material and Methods: A total of 194 patients aged 5 days to 15 years were examined and divided into 2 groups: Group 1 consisted of 65 patients without anorectal anomalies (AA); Group 2 comprised 129 patients, including 66 children with functional constipation, 55 with AA and visible fistulas, who were preoperatively examined, and 8 patients with anorectal angle (ARA), who were postoperatively examined. All the patients underwent irrigoscopy that was different from standard examination in the presence of X-ray CT contrast marker near the anus. Results and Conclusion: DPS is caused by puborectalis muscle dysfunction. A method was proposed to evaluate the status of the puborectalis muscle from the distance between the position of the ARA and the marker near the anus. This not only promotes an exacter estimate of DPS, but also allows refusal of defecography. The use of a barium enema with the minimum number of X-ray films decreases dose of ionizing radiation hazard and permits the use of this procedure not only in adults, but also in children with chronic constipation, fecal incontinence, and in AA for both pre- and postoperatively assessment of the causes of complications.

Currarino's syndrome in twins presenting as neonatal intestinal obstruction--identical presentation in non-identical twins.

We report a case of non-identical twins who presented with identical neonatal intestinal obstruction with features of anorectal stenosis, presacral mass and sacral anomaly consistent with Currarino's syndrome or triad. Plain sacral radiograph, contrast enema and MRI were diagnostic. Initial management involved a defunctioning colostomy followed by a posterior sagittal anorectoplasty with excision of the teratoma ± anterior sacral meningocele and finally closure of colostomy in a staged multidisciplinary approach. The twins' father is also affected with features of Currarino's syndrome but was diagnosed during family screening. Currarino's syndrome presenting with identical neonatal low intestinal obstruction in a non-identical set of twins is rare and interesting. Antenatal diagnosis of Currarino's syndrome is difficult and may prove to be a challenge even in the postnatal period. Sacral spine radiograph, contrast enema and MRI are diagnostic. Management requires high index of suspicion, low threshold for MRI and multidisciplinary staged approach.

Effect of surgical techniques on long-term outcome in congenital pouch colon: A tertiary care centre experience.

BACKGROUND: The objective of the following study is to assess effect of a novel surgical technique on long-term outcome in operated cases of congenital pouch colon (CPC). PATIENTS AND METHODS: We retrospectively analysed our surgical neonatal records from June 2002 to May 2012. Out of 477 cases of anorectal malformations, CPC was found in 73 (15%) cases. Out of 73 cases of CPC, 39 (53.4%) were complete pouch and 34 (46.6%) were incomplete. In addition to invertogram, an erect skiagram was done in all cases to confirm diagnosis. Patients were operated either by single stage pull-through or by staged procedure using conventional abdominoperineal (AP) pull-through or by our new hanging bowel technique. All patients were put on bowel management programme starting 1 month post-surgery until 5 years of life. RESULTS: Children presented to us at median age of 2.1 days. Mean weight at time of presentation was 2.74 kg. In our study, group median age at time of initial procedure was 2.7 days. In staged procedures definitive surgery was done after 6 months. Hospital stay in single stage procedure using the hanging bowel technique was 9.7 days while 17.4 days in conventional AP pull-through. Complication rate were high in conventional pull-through when compared to hanging bowel technique as shown by the significant P < 0.05. CONCLUSION: Our novel surgical technique for pull-through is suitable for both single stage as well as staged pull-through. Bowel enema programme should be an integral part of management of CPC.

No major role for periconceptional folic acid use and its interaction with the MTHFR C677T polymorphism in the etiology of congenital anorectal malformations.

BACKGROUND: Both genetic and nongenetic factors are suggested to be involved in the etiology of congenital anorectal malformations (ARM). Maternal periconceptional use of folic acid supplements were inconsistently suggested to play a role in the prevention of ARM. Therefore, we investigated independent associations and interactions of maternal periconceptional folic acid supplement use and the infant and maternal MTHFR (methylenetetrahydrofolate reductase) C677T polymorphisms with the risk of ARM and subgroups of ARM. METHODS: A case-control study was conducted among 371 nonsyndromic ARM cases and 714 population-based controls born between 1990 and 2012 using maternal questionnaires and DNA samples from mother and child. Cases were treated for ARM at departments of Pediatric Surgery of the Radboud university medical center, Sophia Children's Hospital-Erasmus MC Rotterdam, and the University Medical Center Groningen in The Netherlands and hospitals throughout Germany. RESULTS: No association with folic acid use was present (odds ratio = 1.1; 95% confidence interval: 0.8-1.4) for ARM as a group. Infant and maternal MTHFR C677T polymorphisms were weakly associated with isolated ARM in particular. Lack of folic acid supplement use in combination with infants or mothers carrying the MTHFR C677T polymorphism did not seem to increase the risk of ARM or subgroups of ARM. The relative excess risks due to interaction did not clearly indicate interaction on an additive scale either. CONCLUSION: This first study investigating interactions between periconceptional folic acid supplement use and infant and maternal MTHFR C677T polymorphisms in the etiology of ARM did not provide evidence for a role of this gene-environment interaction.

El colostograma distal a presión en el manejo radiológico de las malformaciones anorrectales / Augmented-pressure colostogram in the radiological assessment of anorectal malformations

Objetivos. Presentar nuestra experiencia en la detección de las fístulas asociadas a las malformaciones anorrectales (MAR), utilizando como método diagnóstico el colostograma distal a presión y su posterior correlación entre los hallazgos radiológicos y quirúrgicos. Material y métodos. Se han revisado retrospectivamente, en un periodo de 17 años, 43 pacientes con diagnóstico de malformación anorrectal, de los cuales 34 fueron remitidos al Servicio de Radiología para la realización de un colostograma distal a presión antes de la cirugía. Resultados. De los 34 casos en los que se realizó el colostograma distal a presión, en 26 se objetivó la existencia de una fístula y en los 8 casos restantes no. En todos los casos nuestros hallazgos radiológicos fueron confirmados posteriormente en la intervención quirúrgica. Conclusiones. El colostograma distal a presión es una prueba diagnóstica sencilla y precisa para definir la anatomía alterada de las MAR, conocer la distancia entre el bolsón rectal y el margen anal, y la localización anatómica de las fístulas asociadas. Es la exploración diagnóstica más fiable para la elección de la vía de abordaje quirúrgica por parte del cirujano, condicionada por la existencia o no de una fistula

Objectives. To present our experience in detecting the existence of a possible associated fistula between the pouch colon and the urogenital tract in patients with anorectal malformations by carrying out an augmented-pressure colostogram, and its subsequent correlation between radiological and surgical findings. Materials and methods. A 17-year retrospective revision of 43 patients with anorectal malformations was performed. 34 of them were referred to the Radiology Department in order to carry out an augmented pressure distal colostogram prior to surgery. Results. A fistula was demonstrated in 26 of the 34 patients who had an augmented-pressure distal colostogram done. In the remaining 8 patients, this technique failed to demonstrate a fistula. The radiological findings were confirmed during the surgery in each case. Conclusion. The augmented-pressure distal colostogram is a simple and accurate study to delineate the altered anatomy of anorectal malformations, to define the distance between pouch colon and perineum, and to identify the localization of any associated fistulous communication. It is the most dependable test for a surgeon in order to choose the type of surgical approach, which depends on the presence or absence of an associated fistula

A new job for an old device: a novel use for nerve stimulators in anorectal malformations.

Muscle stimulation of the perineum is a crucial step in the repair of anorectal malformations. This allows the surgeon to assess muscle function and locate precisely the sphincter muscles during a pull-through operation. Presently, the device commonly used is very expensive. In searching for a cheaper and amenable device we explored utilizing the nerve stimulator MiniStim (model MS-IIIA, Life-Tech, Inc., Houston, TX) normally used for the "train of four" sign in assessing paralysis during general anesthesia. We have used this device in seven consecutive posterior sagittal anorectoplasties and compared its effectiveness with the regular muscle stimulator. In our experience, the nerve stimulator is easier to work with and is a common device in the operating theater. It gave us information that was at least equal to the regular muscle stimulator.

Peristeen integrated transanal irrigation system successfully treats faecal incontinence in children.

OBJECTIVE: Faecal incontinence secondary to myelomeningocele, Hirschsprung disease, and anorectal anomalies remains a significant and common problem. We aimed to report our 5-year experience with the Peristeen trans-anal irrigation system (TAIS) to manage such children. PATIENTS AND METHOD: This study was a combination of a retrospective case note review and assessment using a validated quality of life questionnaire (QOL) to determine pre- and post-TAIS bowel function and continence. QOL scores and functional outcomes before and during TAIS use were compared using Wilcoxon matched pairs test (p < 0.05 significant). RESULTS: Twenty-four children (median age 6 years) were managed with the TAIS 2006-2011 to treat faecal incontinence. Three did not tolerate the system. Median QOL scores in 20 out of 21 patients using TAIS demonstrated significant improvement in bowel management and continence. Two discontinued use due to failure to improve continence; one underwent the Malone antegrade continence enema (MACE) procedure and one returned to oral/rectal medications. Nineteen of 24 patients (79%) continue to use TAIS. CONCLUSIONS: The Peristeen TAIS is an effective, safe, non-operative alternative to MACE in children with faecal incontinence, if initial compliance can be achieved.