High-type Anorectal Malformations Research Articles

Laparoscopic-assisted distal colon excision and proximal colon pull-through anorectoplasty for anorectal malformation.

During the second stage surgery for anorectal malformations (ARM), patients whose distal intestine of the colostomy is particularly short underwent laparoscopic-assisted distal colon excision and proximal colon pull-through anorectoplasty (PCPARP). This study aimed to discuss the outcomes of PCPARP after colostomy in patients with ARM. This is a single-center propensity score-matched (PSM) study which was retrospectively initiated patients with intermediate- or high-type ARM who underwent laparoscopic surgery from June 2007 to December 2018. These patients were divided into PCPARP group and conventional laparoscopic-assisted anorectoplasty (LAARP) group according to specific surgical methods. The general data, surgical data, postoperative complications, and functional results were evaluated. In total, 216 patients were included in this study: 190 (88.0%) undergoing LAARP approach and 26 (12.0%) undergoing PCPARP approach. After PSM, two well-balanced groups of 26 patients were analyzed and showed the postoperative complications (P = 0.126) and bowel function (P = 0.809) were similiar between the two groups. The curative effect of PCPARP after colostomy is similar to that of classic LAARP surgery, which can be used for ARM patients with a very short and abnormal distal intestine of the stoma.

Short and Long-Term Outcomes of PSARP versus LAARP and Single versus Staged Repair for Infants with High-Type Anorectal Malformations: A Systematic Review and Meta-Analysis.

We aimed to compare among patients with high-type anorectal malformations (ARM): (i) short- and long-term outcomes of laparoscopic-assisted anorectoplasty (LAARP) compared to classic posterior sagittal anorectoplasty (PSARP) and (ii) the results of single-stage versus staged PSARP. Using a defined search strategy, two independent investigators systematically reviewed the English literature. PRISMA guidelines were followed, and meta-analysis was performed using RevMan5.3. Of 567 abstracts screened, 7 papers have been included (254 pts; 121 PSARP, 133 LAARP) in the first systematic review and meta-analysis. The length of hospitalization was shortened in LAARP versus PSARP (10.9 versus 14.4 days; p < 0.0001). PSARP and LAARP were comparable in terms of early postoperative complications (28.9% versus 24.7%; p = ns) and rectal prolapse (21.6% versus 17.5%; p = ns). At long-term follow-up, the presence of voluntary bowel movements (74.0% versus 83.5%; p = ns) and the incidence of soiling (45.5% versus 47.6%; p = ns) were similar in both PSARP and LAARP. Six papers (297 pts) were included in the second systematic review, with three comparative studies included in the meta-analysis (247 pts; 117 one-stage, 130 staged procedures). No significant difference in terms of presence of voluntary bowel movements after single-stage versus staged procedures (72.6% versus 67.3%; p = ns) has been detected. LAARP seems to be a safe and effective procedure, showing short- and long-term outcomes similar to PSARP. One-stage PSARP could be a safe alternative to the classic three-stage procedure, even for those infants with high-type ARM. Further and larger comparative studies would be needed to corroborate these partial existing data.

Prevalence and Factors Associated with Post-operative Strictures in Anorectal Malformations

Objective: Rectal strictures are a serious complication following operation for anorectal malformations (ARM). The purpose of this study was to determine the factors affecting rectal strictures following surgical treatments for ARM. Materials and Methods: Retrospective chart reviews of 204 patients with ARM who underwent surgical treatment at Siriraj Hospital between January 2003 and December 2019 were carried out. Results: Overall, the prevalence of post-operative rectal stricture was 19.6% (40/204). The higher types of ARM had higher rectal stricture rates. In low type ARM, the stricture rate following surgery for perineal fistula, vestibular fistula was 4.1% and 14.7%, respectively. Recto-bulbar urethral fistula and recto-prostatic urethral fistula had stricture rates of 19.2% and 26.7%, respectively, but in higher types, the stricture rates were above 70%. Complications such as wound infection, dehiscence, retraction, colonic necrosis and recurrent fistula all affected the post-operative stricture rate (p = 0.029, p = 0.01, p = 0.01, p = 0.042 and p = 0.002, respectively). The operation for low type ARM using local tissue flap, such as YV and cutback anoplasty, had low complications. More complicated operations were performed for higher type ARM. The higher the complication rate, the higher the post-operative rectal stricture. Routine rectal dilatation by parents seemed to prevent rectal strictures (p = 0.056). The surgical treatments for rectal strictures composed of 57.5% anoplasty, 17.5% PSARP, 15% abdo-assisted pull-through and 10% abdo-assisted PSARP. Conclusion: Post-operative rectal stricture occurred because of complications following complicated operations for high type ARM. A meticulous operative technique is crucial.

Vertebral anomalies associated with anorectal malformations incidentally found on contrast studies: a case series

Background: Spinal congenital malformations are closely associated with other congenital anomalies, are usually asymptomatic and be found incidentally. Moreover, spine abnormalities such as hemivertebra may cause further complications if left untreated. Among patients with VACTERL anomalies, spine anomalies account for 60-95% of them. This report aimed to describe rare and interesting cases of spinal malformation in children to provide further information on the importance of a proper approach in such cases. Case Presentation: We present three pediatric cases of vertebral anomalies associated with anorectal malformations incidentally found in contrast studies. All patients have a history of anorectal abnormalities at birth and are referred to the hospital for contrast studies. The first case found semi-segmented hemivertebra at T8-9 levels, cleft at T10 level and high-type anorectal malformations (ARMs). The second case showed unsegmented hemivertebra at the level of L1 and butterfly vertebra at T10, approximately at the same level as the skin lesion, with the possibility of occult spina bifida and high-type ARMs. The third case revealed bilateral vesicoureteral reflux (VUR), grade 1 right hydroureteronephrosis and left hydroureter, with semi-segmented hemivertebra at the level of L3, unsegmented hemivertebra at L4-5 and an extra lumbar vertebra, possibly a lumbosacral transitional vertebra (LSTV). Conclusion: Thorough screening and other examinations such as contrast studies could reduce their morbidity as more sophisticated imaging may be done to better visualize the anatomical malformation.

Pattern of anatomic disorder and surgical management of anorectal prolapse in anorectal malformation.

Due to the paucity of data and controversy regarding the etiology and surgical approach for managing anorectal prolapse (ARP) after anorectoplasty, we sought to investigate the underlying anatomic disorder and the surgical outcome in managing this challenging complication. We performed a retrospective study on 83 patients with ARP related to anorectal malformations (ARM). Logistic regression analyses were performed to detect the risk factors for the ARP severity. Surgical procedures were stratified according to identified anatomical abnormalities and surgical outcomes were analyzed. 50 patients (62.7%) had high-type ARM. The original anorectoplasty had a higher rate of ARP in laparoscopic-assisted anorectoplasty (n = 49, 59.0%) versus posterior sagittal anorectoplasty (n = 11, 13.3%). ARP was associated with rectal fat hyperplasia (67.5%), dilated muscular tunnel (79.5%), longitudinal muscle (LM) discontinuity (16.9%), rectal dilation (22.9%), mislocated anus (7.2%), and excessive mobile mesorectum (3.6%). Based on the ARP severity, the patients were divided into a severe group (Group 1, n = 38) and a moderate group (Group 2, n = 45). Binary logistic regression analysis showed that hyperplasia rectal fat (OR 4.55, 95% CI 1.16-17.84), rectal dilation (OR 4.21, 95% CI 1.05-16.94), and high-type ARM (OR 2.90, 95% CI 1.14-7.39) were independent risk factors for the development of severe ARP. Complications after stratified surgical repair included wound infection in six patients (7.2%), anal stenosis in one patient (1.2%), and ARP recurrence in two patients (2.4%). Twenty-six patients without colostomy before prolapse repair were followed up for 2 to 12years. All the patients maintained voluntary bowel movements. Following ARP repair, there was an overall higher rate of no soiling or grade 1 soiling (88.5 vs. 65.4%), but 3 of 12 patients with grade 2 constipation were upgraded to grade 3. Our study shows that ARM-related anorectal prolapse is associated with excessive rectum, hyperplasia of rectal fat, mobile mesorectum, loose muscular tunnel, LM discontinuity, and anal mislocation. Surgical repair with techniques stratified according to the patients' underlying risk factors is effective to prevent recurrence and improve the soiling continence.

Laparoscopic-assisted anorectal pull-through vs. posterior sagittal anorectoplasty for treating high anorectal malformations: a single-center experience

Background/purposeSurgical management of high-type anorectal malformations (ARM) has evolved over the years with the widespread of the laparoscopic approach over the last two decades. This study aims to compare the outcome of patients with high anorectal malformation (ARM) managed with laparoscopic-assisted anorectal pull-through (LAARP) vs. the open posterior sagittal anorectoplasty (PSARP) at a single Institution.MethodsA retrospective chart review of pediatric patients, who were managed for high-type ARM at our institution, was performed for the period 2000–2015. Nine were excluded because of a lack of important data. Demographic data, surgical approach, associated anomalies, and complications were collected. Functional outcome was measured using the modified clinical scoring for the defecation function of the Japanese study group of anorectal anomalies. Comparison between groups was done using the T test for continuous variables and the Fisher-exact test for proportions. P < 0.05 is considered statistically significant.ResultsThere were 82 patients with high type imperforate anus (59 males and 23 females) 73% had laparoscopy vs. 27% open. Type of fistulae were rectourethral (36), rectovesical (12), no fistula (19), and (15) cloaca anomalies. Patients underwent surgery at a mean age 8.4 (laparoscopy) vs. 10.1 (open) months (P value = 0.14). There was no difference between the two groups regarding weight at the surgery or associated anomalies, except for VACTRAL and genitourinary malformations which were more among the laparoscopic group (8 vs. 1).There was no statistical significance for all elements of defecation function score: sensation, constipation, and soiling (P values 0.17, 0.6, 0.07, respectively). There was no difference between the two groups regarding rectal prolapse (P = 0.06), whereas rectal stenosis is more in PSARP compared to LAARP (P = 0.03). The hospital stay was significantly shorter in LAARP 7.1 vs. 9.4 (P = 0.006).ConclusionsThe defecation function and complication rate for both laparoscopic and open groups were almost similar. This may justify the preference of the laparoscopic approach over the open one, given its minimally invasive nature.

Levator ani asymmetry and deviation in high-type anorectal malformation evaluated by magnetic resonance imaging.

The levator ani (LA) complex in high-type imperforate anus (H-IA), low-type imperforate anus (L-IA), and Hirschsprung's disease (HD) patients as controls were documented using magnetic resonance imaging (MRI) and compared for symmetry. Mean left:right LA thickness ratio (LA ratio), and deviation of the LA from the pubococcygeal line (PCL; LA angle) were calculated from thin-slice MRI images (axial 2mm, coronal 2mm, and sagittal 3mm) of the puborectalis and pubococcygeus taken parallel to the PCL under sedation in H-IA (n=14), L-IA (n=16), and HD (n=9). MRI scans were performed between January 2018 and June 2021. LA were significantly thinner in H-IA (1.78±0.46mm) compared with L-IA (2.97±0.55mm) and controls (2.87±0.32mm), p<0.0001. LA ratio was significantly lower in H-IA (0.71±0.15) compared with L-IA (0.93±0.04), and controls (0.91±0.06), p<0.0001. Mean LA-angle was significantly different in H-IA, 10.8° (range 6°-19°), versus L-IA and controls, both zero degrees (range 0°-5°), p<0.0001, respectively. LA was confirmed to be significantly asymmetric in H-IA. Because outcome of surgical repair involving a midline incision, such as posterior sagittal anorectoplasty could be impaired, pediatric surgeons are advised to plan surgical intervention for H-IA carefully and appropriately.

Pelvic Floor Muscles Contribution in Surgical Outcome of Children with High-type Anorectal Malformations.

As a consequence of high-type anorectal malformations (ARMs) pathogenesis, the pelvic floor muscles remain severely underdeveloped or hypoplastic, the rectal pouch is located at the level or above the puborectalis sling, and the bowel terminates outside the sphincter muscle complex support. For children with high-type ARMs the ultimate objective of therapy is mainly to grow up having bowel continence function that is compatible with a good quality of life, and the final prognosis depends significantly on the grade of development of pelvic floor muscles and the successful entering of the anorectum fully within the support of the external anal sphincter due to intraoperative conservation of the puborectalis sling. Pelvic magnetic resonance imaging (MRI) has recently become the preferred imaging study for prediction of functional outcomes, since it can define the anatomy and evaluate the development of the sphincteric muscles before and after surgical correction. Based on recent literature and our clinical experience, we will discuss the relevance of pelvic floor muscles MRI to the clinical outcome of children with high type ARMs.

Impact of "Lateralizing" the Sigmoid Colostomy on Port Ergonomics in Laparoscopic Anorectoplasty for High Anorectal Malformations.

The high-type anorectal malformations (ARM) are conventionally managed by an initial left iliac fossa sigmoid colostomy, followed by laparoscopic anorectoplasty (LARP). Such a stoma occupies left half of the infraumbilical region and hinders the LARP ergonomics, leading to the surgeon's discomfort. We studied the outcome and impact of "lateralizing" (shifting laterally in the abdominal wall) the colostomy on port ergonomics. This prospective study was conducted in the pediatric surgery unit of a medical college in central India between March 2014 and June 2017 in two parts. In the first part of the study, neonates with high ARM were block randomized in two groups: conventional colostomy (CC) in left iliac fossa and lateral colostomy (LC, laterally placed abdominal colostomy). Outcomes of colostomy were compared among these two groups. In the second part of the study, 40 consecutive infants from both types of colostomy groups underwent ergonomic comparison and assessment of the surgeon's discomfort during LARP (CC-LARP and LC-LARP). Standard statistical tests were used for comparison. In the first part of the study, 203 ARM cases were included in this study; 100 underwent CC and 103 underwent LC. Colostomy prolapse, excoriation, reversed stoma, and short distal limb were significantly higher in the CC group. In the second part of the study, the LC-LARP group showed many significant advantages over the CC-LARP group, including less peri-stomal adhesions, better vision, shorter operative time, and better ergonomics (better manipulation, elevation, and azimuth angles). The LC-LARP also significantly reduced the surgeon's discomfort. Lateralization of high-sigmoid colostomy should be preferred over left iliac fossa sigmoid colostomy for ARM, as it improves the port ergonomics and reduces the surgeon's discomfort for second stage LARP.

Long-term follow-up in surgical newborns: A single-institution experience

BackgroundTo assess the actual experiences of long-term follow-up and discuss ways to improve care during the period from childhood to adulthood in newborns who have undergone surgery. MethodsA total of 306 patients with congenital anomalies requiring newborn surgery who survived to discharge from 1994 to 2013 were eligible for inclusion. Survivors with severe chromosomal and cardiac anomalies were excluded. Patients with myelomenigocele, urogenital anomalies and miscellaneous diagnoses were also excluded. Patients with Hirschsprung’s disease were excluded since many of them underwent surgery after the neonatal period. Patients with hypertrophic pyloric stenosis were also excluded since their duration of follow-up was too short for this study. ResultsAccording to the follow-up status, survivors were categorized into 4 groups: under follow-up as an outpatient (UF, n = 67), moved (MV, n = 60), follow-up suspended by doctor (Sus, n = 87), and lost to follow-up (LF, n = 92). The incidence of active medical problems was high, and the duration of follow-up was significantly longer in the survivors with esophageal atresia, congenital diaphragmatic hernia and high-type anorectal malformations than in those with other anomalies. Survivors followed by pediatric surgeons alone, free from active medical problems or free from adverse events during the initial hospitalization were at risk of being LF. ConclusionsMore than 30% of the surgical newborn cases were LF. Disease-specific and standardized multidisciplinary follow-up programs that increase both children’s and parents’ satisfaction and compliance are needed. (230/250 words).

Laparoscopic surgical technique to enhance the management of anorectal malformations: 330 cases' experience in a single center.

Laparoscopic-assisted anorectoplasty (LAARP) is considered to benefit the patients with vesico-prostatic fistula. The aim of this study is to present the details of our LAARP technique for improving the short- and long-term outcomes in the patients with high and intermediate types of anorectal malformations (ARMs). 330 patients with high-type (174 cases) and intermediate-type (156 cases) anorectal malformation (aged 8days to 15years) underwent LAARP from 2001 to 2019. LAARP was performed for full mobilization and resection of the dilated rectum, intra-rectal closure of the fistula, visualization, and enlargement of the center of the longitudinal muscle tube (LMT) from pelvic and perineal aspects. LAARP was performed in all patients and no patient was converted to open procedure. The urethral diverticulum was found in three patients (1.02%, 3/294) according to postoperative protocol voiding cystourethrogram but was not associated with any symptoms such as urinary tract infection and dysuria. Rectal prolapse requiring surgical intervention developed in 25 (7.6%) of 330 patients. Anal stricture occurred in three patients and re-do anoplasty was performed 5months after LAARP. Anal retraction occurred in two patients and re-pull-through was conducted at 5 and 6days, respectively, after LAARP. 228 patients who were older than 3years were followed up. The median follow-up period was 5.8years (range 3-15years). 217 patients (95.2%) had voluntary bowel movements; 202 patients (88.6%) were free from soiling or with grade 1 soiling; 30 patients (13.6%) and 25 patients (11.3%) suffered from grade 1 and grade 2 constipation, respectively, while no patient had grade 3 constipation. Our experience demonstrates that the LAARP has advantages on rectal mobilization and resection, intra-rectal fistula closure and accurate tunnel formation in the LMT with minimal trauma. The improvement of the short-term and long-term outcomes after LAARP has been shown not only for high-type ARM but also for intermediate-type ARM.

Follow-Up Outcomes of Laparoscopic-Assisted Anorectal Pull Through for Anorectal Malformations of High Type.

Background: Laparoscopic-assisted endorectal pull-through (LAEPT) procedure in the management of high-type anorectal malformations (ARMs) was first introduced in 1998 and is quickly accepted worldwide. However, evidence on long-term outcomes of this technique is constrained. This study aims to evaluate the long-term outcomes of LAEPT for high-type ARMs in Vietnamese pediatrics. Materials and Methods: A longitudinal study was carried out from January 2009 to July 2014 in 56 patients <3 years old. Variables included age of operation, associated anomalies, type of fistula, the duration of hospital stay, complications, and long-term functional outcomes (Krickenbeck modified standards were used for children ≥3 years). Results: There were 56 patients including 48 males and 8 females. The mean age at operation was 3.7 months, the mean hospital stay was 4.6 days. The mean operative time was 76.7 minutes. The mean follow-up time was from 38 to 104 months (mean follow-up: 71.5 months). There were 46 (82.1%) patients having feeling of urge, 42 (75.0%) patients having capacity to verbalize, and 40 (71.4%) patients having hold the bowel movement. Conclusion: LAEPT is feasible, safe, and effective in the management of high-type ARMs.

Loop versus Divided Colostomy in Patients with High and Intermediate Anorectal Malformations: Randomized Controlled Study

Background: Colostomy is performed as a step of staged management of high-type anorectal malformations in pediatrics. Creating a colostomy, by itself, is a minor surgical procedure, but it has many complications. Aim: The aim of the study was to compare the outcome results of loop versus divided colostomies in patients with high and intermediate anorectal malformations. Methods and Material: This randomized prospective study was performed on 34 patients with imperforate anus. Patients were divided into 2 groups (17 patients each); group A were subjected to loop colostomy and group B were subjected to divided colostomy. Results: There was no significant difference between both groups as regards to demographic data (gestational age, sex and birth weight), age of first presentation, associated congenital anomalies and types of fistula. The mean operative time was 42.05 ±6.19 min in group A and 51.76 ±8.21 min in group B. The difference between both groups in this point was statistically significant (P value =0.04). The hospital stay and postoperative complications showed insignificant difference between both groups. Patients with loop stomas were significantly more likely to develop prolapse (P=0.033). Conclusions: Both techniques of colostomy are easy to perform and safe in the anorectal malformation (ARM) patients. Loop colostomy had a shorter operative time, but the complications rates, especially prolapse and urinary tract infection (in cloaca patients), are significantly higher in the loop colostomy when compared to the divided colostomy.

Case series: review of several types fistulas of anorectal malformation on distal loopography

Introduction: Anorectal malformation (ARM) is a wide spectrum of congenital malformations involving the distal rectum and anus as well as the urinary and/or gynaecological systems with an estimated incidence ranging between 1 in 2000 and 1 in 5000 live. Types of fistulas are essential to diagnose to decide the management and surgical repair. We review several fistula types of anorectal malformation based on distal loopography examination. Case Presentation: Review of 5 patients with distal loopography in Sanglah Hospital was performed. All patients were neonate with absence of the anus without any clues of fistulas.  Plain abdominal x-ray and knee-chest position showed distended loops of bowel and blind-ending of distal rectum with various distance from anal dimple. Two cases were confirmed as high type of ARM with recto-vesica fistula, where the contrast filled rectum and the bladder which decreased significantly after micturition. The two other cases were confirmed as high type ARM with recto-urethrae fistula, and the last one as low type of ARM with recto-perineal fistula. All patients undergoing surgical management for fistula repair and anorectal reconstruction.Discussion: Anorectal malformation forms a heterogeneous group of anomalies, most cases showed imperforated anus and blind-ending of distal enteric component (atresia) with or without fistula. Our cases were diagnosed by plain x-ray and contrast study. High or low type was evaluated from the distance rectal pouch to anal dimple. Distal loopogram was showed low type of ARM with recto-perineal fistula and high type of ARM with fistula extended anteriorly as recto-vesica fistula and recto-urethrae fistula, as The Krickenbeck classifications.Conclusion: Distal loopography is an important diagnostic investigation to delineate the altered anatomy of anorectal malformations, the location fistula and the spectrum of associated fistula between the blind rectum and the bladder, urethra, perineum, scrotal and vagina. Radiology interpretation is indeed important for clinician to establish patient management.Â

Anorectal malformations: Early outcome analysis from a high-volume tertiary care institute

Context: Associated malformations in anorectal malformation (ARM) are of important implication with regard to final outcomes since the anorectal lesions that cause intestinal obstruction are correctable with operative intervention. Aims: To study the presentation, types of anomalies, associated malformations, and procedures performed in relation to the type of anomaly and early outcomes analysis of ARM patients in the neonatal period. Settings and Design: A retrospective study was performed from January 2016 to December 2016. Subjects and Methods: The study included all patients with ARM admitted in the Neonatal Intensive Care Unit. Results: There were 216 neonates having ARM with 173 males and 43 females (m:f = 4:1). High-type ARM was seen in 177 (81.94%) cases, while low type in 39 (18.06%) neonates. Associated malformations were documented in 67 (31.02%) neonates. Esophageal atresia (EA) was the most common (51) associated anomaly. Among the 200 procedures undertaken for ARM, left transverse loop colostomy was the most common (132). Only 83 (38.42%) neonates were diagnosed on the 1st day of life. There were 67 (31.02%) deaths, 145 (67.13%) survivors, and 4 (1.85%) cases left against medical advice. There were only 9 (17.65%) survivors among those associated with EA. Cardiovascular was the most common (39) cause of mortality followed by septicemia (30). Conclusions: An overall high mortality rate of 31.02% and an extremely high rate (74.51%) among those associated with EA were present. In spite of the progress made in the field of neonatal care, associated malformations compounded with a high volume of patients in our resource-limited institution negatively influence the outcome of ARM in the newborn period. Strict infection control measures for prevention of septicemia and investigations for other associated malformations are recommended to improve the outcomes.

Ultrasound imaging of the anorectal malformation during the neonatal period: a comprehensive review.

Anorectal malformation (ARM) is classified as low, intermediate, or high; accurate diagnosis of the type during the early neonatal period is important to determine the appropriate initial surgical approach. This review assesses the role of ultrasound examination in the classification of ARM during the neonatal period, with a focus on landmarks on the sonogram, the approach used for sonography, and the optimal examination timing. The following three factors on the sonogram are used for the classification: location of the fistula, the distance between the distal rectal pouch and the anal dimple (perineum) (P-P distance), and the relationship between the puborectalis muscle and the distal rectal pouch. Three approaches can be used to evaluate ARM by ultrasonography, namely, suprapubic, perineal, and infracoccygeal approaches. Each approach has its own advantages and disadvantages. Optimal timing of the ultrasound examination is also important with respect to each factor to classify ARM. We have described the pitfalls of ultrasound in diagnosis of cases, namely ARM with Down syndrome (which tends to be without fistula), ARM with low birth weight, ARM with unusual location of fistula, ARM with opened fistula (where the P-P distance is unreliable), and cloacal malformation (variation of the high-type ARM).

Diagnostic suggestion and surgical consideration for Hirschsprung’s disease associated with high anorectal malformation

Object: The objective of this study were to highlight the finding of vasa recta (tortuous) on the colonic wall as a diagnostic clue for suspecting an associated Hirschsprung’s disease (HD) and to draw attention to the importance of preserving the aganglionic rectum plus a retrorectal pull through in these cases. Background: The association of HD with anorectal malformation (ARM) is both diagnostically and surgically challenging. Patients and methods: Records of cases with ARM treated over 15 years were examined. Among these, five children with an associated HD were analyzed with regard to their clinical, radiological, surgical, histopathological findings, and outcome. Relevant literature was also reviewed. Results: HD was present in 1.26% cases of ARM. All were men with high-type ARM. Two groups were identified. In group 1 (two patients), associated HD was suspected after completion of all stages of ARM repair. They reported prolonged postoperative constipation, abdominal distention, and enterocolitis. Moreover, they endured additional surgeries for HD. In group 2 (three patients), HD was suspected at the time of initial colostomy for ARM in two patients on visualizing tortuous (cork screw) vasa recta on the sigmoid colon surface in a region similar to the transition zone in HD. Biopsy from the site confirmed HD. In the third patient, these vessels were visualized on the colostomy loop at the time of laparoscopic pull through for ARM. Preservation of the aganglionic rectum (to function as a fecal reservoir) as well as a retrorectal pull through was done in both groups. Conclusion: Finding prominent corkscrew vessels on the colonic surface could serve as a clinical clue for the presence of HD in cases of ARM. Moreover, preserving the aganglionic rectum and performing a Duhamel pull through helps provide acceptable continence. Keywords: anorectal malformation, Hirschsprung’s disease, rectal reservoir, tortuous vasa recta

Left transverse loop colostomy versus high sigmoid loop colostomy for high-type anorectal malformations: Early outcome analysis

Background: High sigmoid (divided) colostomy has been recommended by various studies for the management of high-type anorectal malformation (ARM). It is postulated to be associated with fewer complications. Aims: To compare the complications related to left transverse loop colostomy and high sigmoid loop colostomy done in male patients with high-type ARM and share our experience. Materials and Methods: A prospective study on all male neonates admitted with high-type ARM at our high-volume tertiary care institute from January 2014 to March 2016 was carried out. The patients were randomized and divided into two groups (group A: left transverse loop colostomy and group B: high sigmoid loop colostomy). The patient's clinical presentation, operative course, and complications were recorded. Results: Of 158 male neonates who were subjected to loop colostomy, 93 (58.9%) were in group A and 65 (41.1%) in group B. Mortality was higher in group B (16.9%) than group A (8.6%), but none directly related to the type of colostomy. Intraoperative complications (technical difficulties) such as difficulty in approaching the sigmoid colon with dilated small bowel loops and identifying the proximal and distal parts of sigmoid colon along with handling of the bowel (including serosal tears) were significantly more in group B (20%) than group A (1.07%) where transverse colon was easily exteriorized. The average time taken for the procedure, pericolostomy excoriation, and bowel obstruction was more in group B when compared with group A. Conclusion: Left transverse loop colostomy works well to relieve the obstruction and is less time-consuming, particularly with late presentation. We recommend left transverse loop colostomy in cases of high-type male ARM because of the ease of procedure and comparatively low complication rates.

Nonfluoroscopic well-tempered pressure augmented distal colostogram in high-type anorectal malformation: Our experience

Background: Accurate imaging of rectourethral fistula is essential for optimal surgical management of infants with high-type anorectal malformation (ARM). The aim of this study was to study the role of nonfluoroscopic well-tempered pressure-augmented distal colostogram in high-type ARM in demonstrating rectourinary fistula, bladder anomalies, and for the presence of vesicoureteral reflux (VUR). Patients and Methods: We present a prospective study of 10 male infants who underwent a diverting colostomy in the neonatal period from January 2011 to December 2012. Our method of performing a colostogram is similar to conventional colostogram, but involves (a) meticulous bowel preparation preoperatively, ensuring that while doing the last distal colostomy wash before colostogram there is passage of clear saline from the urethral meatus to establish the presence of rectourinary fistula; (b) nonfluoroscopic study (c) performed with gentle and sustained pressure of contrast media, maintained over the period of the investigation; (d) bladder is completely filled with contrast media through rectourinary fistula and it is confirmed clinically; and (e) radiographic films in addition are taken in the micturating phase. Results: Rectourinary fistula was demonstrated in all (100%) cases. Rectoprostatic urethral fistula was present in three (30%) infants, rectobulbar urethral fistula in six (60%) patients, and one (10%) infant had type 4 congenital pouch colon (CPC) with colovesical fistula. Also three (30%) patients demonstrated unilateral primary vesicoureteral reflux (VUR), which was previously undiagnosed in all of them, as micturating cystourethrogram (MCU) was not attempted. Conclusions: Our study confirmed the level of anomaly and accurately demonstrated the type of fistula in all patients. It also detected the undiagnosed VUR in three (30%) cases. It obviated the need for a MCU.

Combined Laparoscopically Assisted and Anterior Sagittal Anorectoplasty for Imperforate Anus with Rectobulbar Urethral Fistula: A Case Report

Abstract Introduction: Laparoscopically assisted anorectoplasty (LAARP) was introduced in 2000,1 and the number of hospitals adopting it for the treatment of high-type anorectal malformation (rectovesical or rectoprostatic fistula) is increasing. However, the application of LAARP for rectobulbar urethral fistula is controversial because precise division of the fistula in the deep pelvic cavity is difficult, and there is a potential risk of posterior urethral diverticulum.2–4 In this video, we introduce a novel procedure for an imperforate anus with a rectobulbar fistula involving precise ligation of the fistula and appropriate placement of the rectum in the center of the sphincter using combined laparoscopically assisted and anterior sagittal approaches. Case Report: A boy weighing 2220 g was born at a gestational age of 37 weeks and diagnosed with an imperforate anus immediately after birth. No associated malformations, including neurological abnormalities and sacral deformities, were noted. Initially, a...