Imperforate Anus Research Articles

Immunohistochemical evaluation of CD34, CD117, and calretinin for diagnosis of hirschsprung’s disease

IntroductionHirschsprung’s disease (HD) is a neurogenic intestinal disorder attributed to incomplete neural crest cell migration during fetal intestinal development, leading to an aganglionic segment of the colon and functional obstruction. Associated malformations like intestinal atresia, hydronephrosis, and imperforate anus can accompany Hirschsprung’s disease. this study aims to evaluate the efficacy of Calretinin and Cajal cells (CD34 and CD117) immunohistochemical staining in improving HD diagnosis.MethodsThe study involved 70 pediatric patients suspected of Hirschsprung’s disease. Clinical, histopathological, and immunohistochemical analyses were conducted, focusing on calretinin, CD34, and CD117 markers to identify ganglion cells and Cajal cells. Data were statistically analyzed using SPSS software.ResultsIn the examination of the samples, the calretinin marker exhibited a consistent accuracy of 100% in diagnosing Hirschsprung’s disease (with sensitivity and specificity both at 100%). Regarding the markers for Cajal cells in cases of Hirschsprung’s disease, an irregularity in the arrangement of Cajal cells was observed, which was absent in normal cases. These markers also demonstrated a specificity and sensitivity of 100% in diagnosing the disease.ConclusionHirschsprung’s disease remains a complex condition with multifaceted pathophysiological mechanisms. Calretinin immunohistochemical staining offers enhanced diagnostic accuracy, while the debate surrounding ICC distribution underscores the need for advanced diagnostic techniques. Further research is warranted to unravel the intricacies of Hirschsprung’s disease and its associated complications.

Anorectal Malformations (ARM) and associated maternal factors among children at Tikur Anbessa Specialized Hospital and St. Paul’s Hospital Millennium Medical College, Addis Ababa, Ethiopia: An unmatched case-control study

Anorectal Malformations (ARM) and associated maternal factors among children at Tikur Anbessa Specialized Hospital and St. Paul’s Hospital Millennium Medical College, Addis Ababa, Ethiopia: An unmatched case-control study

Letter to the Editor: Congenital Heart Defects in Patients with Anorectal Malformations: A Retrospective Cohort Study of 281 Patients

Letter to the Editor: Congenital Heart Defects in Patients with Anorectal Malformations: A Retrospective Cohort Study of 281 Patients

ERN eUROGEN Guidelines on the Management of Anorectal Malformations Part III: Lifelong Follow-up and Transition of Care

Abstract Introduction Anorectal malformations (ARMs) are complex congenital anomalies of the anorectal region, oftentimes also affecting the genitourinary system. Although successful surgical correction can often be achieved in the neonatal period, many children will experience functional problems in the long term. The European Reference Network for rare and complex urogenital conditions (eUROGEN) assembled a panel of experts to address these challenges and develop comprehensive guidelines for the management of ARM. Methods The Dutch Quality Standard for ARM served as the foundation for the development of guidelines applicable on a European level. Literature was searched in Medline, Embase, and Cochrane. The ADAPTE method was utilized to incorporate the newest available evidence. A panel of 15 experts from 7 European countries assessed currency, acceptability, and applicability of recommendations. Recommendations from the Dutch Quality Standard were adapted, adopted, or rejected and recommendations were formed considering the current evidence and/or expert consensus. Results Lifelong follow-up, integration, and transition of care were assessed. A total of eight new studies were identified. The panel adapted 18 recommendations, adopted 6, and developed 6 de novo. Overall, the level of evidence was considered low. Conclusion Successful lifelong follow-up and transition of care require a dedicated team of pediatric and adult specialist and an individually tailored patient-centered approach. This guideline summarizes the best available evidence on follow-up of ARM patients and provides guidance for the development of structured transition programs.

European Reference Network eUROGEN Guidelines on the Management of Anorectal Malformations, Part II: Treatment

Abstract Introduction Anorectal malformations (ARMs) are rare birth defects affecting the anorectum and oftentimes the genitourinary region. The management of ARM patients is complex and requires highly specialized surgical and medical care. The European Reference Network eUROGEN for rare and complex urogenital conditions aimed to develop comprehensive guidelines for the management of ARM applicable on a European level. Methods The Dutch Quality Standard for ARM served as the basis for the development of guidelines. Literature was searched in Medline, Embase, and Cochrane. The ADAPTE method was utilized to incorporate the newest available evidence. A panel of 15 experts from seven European countries assessed currency, acceptability, and applicability of recommendations. Recommendations from the Dutch Quality Standard were adapted, adopted, or rejected and recommendations were formed considering the current evidence, expert opinion, and the European context. Results Surgical and medical treatment of ARM, postoperative instructions, toilet training, and management of fecal and urinary incontinence were addressed. Seven new studies were identified. The panel adapted 23 recommendations, adopted 3, and developed 8 de novo. The overall level of newly found evidence was considered low. Conclusion Treatment of ARM patients requires a multidisciplinary team and expertise about anatomical and surgical aspects of the disease, as well as long-term follow-up. This guideline offers recommendations for surgical and medical treatment of ARM and associated complications, according to the best available evidence and applicable on a European level.

Primary Anterior Sagittal Anorectoplasty Shift from Staged to Single-stage Procedure for Low Anorectal Malformations in Female Children: Our Initial Experience in Single Centre.

Anorectal malformation (ARM) is a common congenital anomaly found in the paediatric age group. Previously, the repair of the vestibular fistula (VF) was performed as three-stage procedure with initial colostomy followed by a definite procedure and, lastly, colostomy closure. At present, a single-stage procedure is preferred, especially in lower anomalies owing to the convenient and time-saving approach. The clinical profile and outcome of ASARP for treatment of ARM in females (vestibular anus and perineal fistula). A retrospective analytical study was conducted at Government Medical College, Jammu. A total of 60 patients were included in the study. Most of the patients were in the age group of 1-5 years (30%), followed by 6 months-1 year age group (25%). Forty-five (75%) females had VF while 15 (25%) had perineal fistula. Intraoperative complication was vaginal tear seen in 6 (10%) patients followed by rectal tear seen in 3 (5%) patients. Early post-operative complications were seen in 9 (15%) patients. Wound infection was the most common complication seen in 5 (8.3%) patients, wound dehiscence in 3 (5%) patients and retraction of rectum in 1 (1.6%) patient. Late post-operative complications were seen in 24 (40%) patients. Perineal excoriation was the most common complication seen in 6 (10%) patients, constipation seen in 5 (8.3%) patients, anal stenosis in 4 (6.6%) patients and mucosal prolapse in 4 (6.6%) patients. The single-staged ASARP procedure resulted in satisfactory outcomes. It is an excellent procedure for females with VF and perineal fistula. Appropriate selection, preparation of patient, optimal correction with minimal sphincter damage and needful post-operative care of wound give excellent cosmetic and functional outcomes in terms of continence. It also decreases the burden on treating the surgeon, family and psychological stress on the patients and parents.

Classic divided sigmoidostomy vs loop sigmoidostomy in anorectal malformations: time for change?

Divided sigmoidostomy (DS) is the classic stoma for patients with anorectal malformations (ARM). Loop sigmoidostomies (LS) in ARM are associated with a higher risk of stoma prolapse and urinary tract infections (UTI). This is not clearly supported by literature. We compared our experience with both techniques. Retrospective study of ARM patients who underwent DS or LS between 2013 and 2023. We analysed demographics, associated malformations, intraoperative variables, oral intake and stoma functioning times, hospital stay, complications, prolapses, and UTI. Of 40 patients, 29 underwent open DS and 11 laparoscopic LS. Demographics, malformation type, associated anomalies, surgical time, intraoperative and anaesthetic complications were comparable. Postoperative complications were higher in DS than LS [14(48.3%) vs 1(9.1%), (p = 0.02)], mostly due to wound complications [12(41.3%) vs 0(0%), (p = 0.01)]; with 3 dehiscenses and 3 strictures reintervened. The hours to oral intake and stoma functioning were higher for DS [48(39-90) and 48(24-48) vs 24(24-48) and 24(24-24), (p < 0.05)], with more days of hospital stay [36(19-60) vs 8(5-10), (p = 0.001)]. Prolapses [1(3.4%) vs 1(9.1%)] and UTIs [3(10.3%) vs 1(9.1%) (p > 0.05)] were comparable. LS in ARM patients have no higher risk of prolapse or UTI than DS. DS had more complications, mostly wound infections, strictures and dehiscenses.

Rethinking Fistula Preservation in Anorectal Malformation Surgery: A Histopathological Perspective.

This study aims to assess the histopathological characteristics of the fistulous tissue in males with anorectal malformation (ARM) to determine its suitability for use in constructing a neoanus. This prospective observational study included male ARM patients with recto-urethral fistula. All other types were excluded. The tissue specimens comprised 0.5-2.0 cm of the most distal part of the rectal pouch and the fistulous tissue. Hematoxylin and eosin-stained sections were prepared. Histological features viz. internal sphincter, anal grand and crypts, ganglion cells, presence of subepithelial fibrosis, thickened nerve trunks, and metaplasia were evaluated. Of 65 patients of ARM admitted, 24 met the inclusion criteria. Gross and microscopic internal sphincter was not found in any sample. The anal mucosa was visualized in all cases; however, crypts were irregular and distorted architecture was seen in 4 (16.67%) samples. Urothelial metaplasia was identified in 10 (41.67%) cases. Adequate ganglion cells were seen only in 6 (25%) samples. Significant submucosal fibrosis was seen in all samples. Thickened nerve trunks were identified in 4 (16.67%) samples. All normal anal histological features could not be found together in the fistula tissue. With the absence of normal features, such as internal anal sphincter muscles, and the presence of abnormal histopathological features, such as subepithelial fibrosis, thickened nerve trunks, and metaplasia there are high chances of abnormal bowel function like constipation if the fistulous tissue is used for neoanus construction. It may have an impact on the quality of life of patients.

Evaluation of Anal Position Indexes from Anal Dimple and Neoanus at Sphincter Muscle Complex in Children with High Anorectal Malformations in Kano, Nigeria.

Anorectal malformations (ARMs) comprise a spectrum of congenital anomalies that continue to present a challenge to patients, caregivers and paediatric surgeons. The main objective of this study was to determine the anal position index (API) from anal dimple and neoanus at the sphincter muscle complex in children with high ARM. Using a cross-sectional study design, the API was assessed from anal dimple and neoanus at the sphincter muscle complex in children with high ARM. All eligible children with high ARM who presented to AKTH during the study period were recruited into the study. Forty eligible patients with an age range of 8 months to 108 months were recruited. Twenty-five were males (62.5%) with a male: female ratio of 1.7:1. The mean pre-operative API using anal dimple was 0.47 ± 0.05, while that of the post-operative API using neoanus was 0.53 ± 0.04. There was a statistically significant difference between the two indices (t = -11.8, P < 0.01). There was also a statistically significant difference amongst male and female indices P < 0.05, but there were no significant differences between different age groups. This study has shown that there was a statistically significant difference between the pre-operative API and post-operative API; thus, the position of the anal dimple does not significantly predict the central position of the sphincter muscle complex.

Decisions in Diversion: Enterostomy vs. Primary Anastomosis for Colonic Atresia

BackgroundColonic atresia (CA) is associated with Hirschsprung disease (HD) in up to 10% of cases. Therefore, some surgeons elect to complete proximal diversion at the initial operation. We sought to better define the incidence of concurrent HD and evaluate practice patterns regarding diversion for CA. MethodsThe Pediatric Health Information System (PHIS) database was used to identify patients with CA from 2013 to 2022. Patients with small bowel atresia, anorectal malformation, gastroschisis, or first operation after 14 days of age were excluded. Index and subsequent operations were defined. Complications, time to enterostomy closure, and unplanned operations were evaluated. ResultsHD was diagnosed in 8 (9.5%) patients and 7 of these were initially diverted. Diverted and anastomosed patients were demographically similar. In the 58 (69%) patients initially diverted, 19 (33%) had an ileostomy. Continuity was restored with an ileo-colic anastomosis in 63% of diverted and 27% of primarily anastomosed patients. Of those initially managed with a colostomy, 53% ultimately had a colo-colonic anastomosis. Patients with primary anastomoses had fewer operations and received more days of parenteral nutrition. Other outcomes did not vary. ConclusionIn a large population of infants with CA, 9.5% had concurrent HD. Almost 70% of CA patients underwent initial diversion and only one with HD had a primary anastomosis. Patients managed with a primary anastomosis were substantially more likely to retain the proximal colonic segment, but had a similar incidence of complications. When intraoperative colonic biopsies are obtained, primary anastomosis is a safe and effective strategy for CA. Level of Evidence3.

Infracoccygeal/transperineal window: new method to prenatally diagnose and classify level of anal atresia.

To introduce a two-dimensional sonographic method to assess the fetal anus, and to evaluate the feasibility of this method to diagnose anal atresia prenatally and identify the presence or absence of anoperineal fistula (in males) and anovestibular fistula (in females). This was an observational study of suspected cases of anal atresia referred to a single center in Israel between August 2018 and October 2023. In addition to conventional evaluation of the perineum in the axial plane, fetuses referred to our center for suspected malformation were scanned with a new method termed the 'infracoccygeal/transperineal window'. This window consisted of a midsagittal view of the fetal pelvis, including the distal rectum and the anal canal. Normal anatomy was confirmed when the anal canal was continuous with the rectum and terminated at the expected location on the perineum. In female fetuses, the normal anal canal runs parallel to the vaginal canal and diverges posteriorly, terminating at the perineal skin, distant from the vestibule. In male fetuses, the normal anal canal diverges posteriorly in relation to the corpora cavernosa, terminating at the perineal skin, distant from the scrotum. High anal atresia was identified when a blind-ending rectal pouch was demonstrated in the pelvis without a fistula to the perineum or vestibule. Low anal atresia was determined when a rectal pouch was continuous with an anteriorly deflected fistula. In females, the fistula converges with the vaginal canal, terminating at the vestibule; in males, the fistula deflects anteriorly, terminating at the base of the scrotum. Postnatally, the diagnosis and type of anal atresia were confirmed through physical examination with direct visualization of the fistula, radiographic studies, surgical examination and/or postmortem autopsy. Of the 16 fetuses diagnosed prenatally with anal atresia, eight were suspected to have low anal atresia and eight were suspected to have high anal atresia. The median gestational age at diagnosis was 23 (range, 14-37) weeks. All cases showed additional structural malformation. Eleven patients opted for termination of pregnancy, of which four had low anal atresia and seven had high anal atresia. Postnatal confirmation was not available in four cases due to curettage-induced mutilation or in-utero degradation following selective termination of the affected twin, leaving 12 cases for analysis, of which seven were diagnosed with low anal atresia and five with high anal atresia. In these 12 cases, all prenatal diagnoses were confirmed as correct, rendering 100% sensitivity and 100% specificity in this high-risk fetal population. The infracoccygeal/transperineal window is an effective method to detect and classify the level of anal atresia prenatally. This may improve prediction of postnatal fetal continence and optimize prenatal counseling. © 2024 International Society of Ultrasound in Obstetrics and Gynecology.

Adult obstetricians and gynecologists lack knowledge of anorectal malformations – a call for action: Gynecologists lack knowledge of anorectal malformations

ObjectivePatients with anorectal malformations (ARMs) may have concurrent gynecologic abnormalities. As patients grow, they typically transition from pediatric subspeciality care and seek adult OB/GYN related services. We aimed to assess adult OB/GYN physicians’ knowledge, competency, and comfort meeting the sexual and reproductive health care needs of patients with ARM. MethodsWe performed a cross-sectional observational survey-based study of graduates from a single academic OB/GYN residency program from 2013-2022. Physicians were surveyed on experience, comfort, and challenges caring for patients with ARMs and given a knowledge assessment. Descriptive and comparative statistics between those who did and did not complete a pediatric and adolescent gynecology (PAG) rotation were generated. ResultsThere were 59 respondents (53.6%). Fewer than half (39.0%) report caring for a patient with ARM, an appendicovesicostomy (12.3%) or an appendicostomy (5.4%). Most felt uncomfortable (80.4%) or felt they lacked competence caring for these patients (81.8%). The majority (64.3%) felt ARMs should be discussed in residency. Only one physician (1.7%) answered all questions in the knowledge assessment correctly; 33.9% did not answer any question correctly. On subgroup analysis, more physicians completing a PAG rotation recalled learning about ARMs (83.3 vs 51.9%, p=0.03); however, there were no differences in experience, comfort, competence, or willingness to learn. ConclusionOB/GYN providers report lack of knowledge and comfort in caring for patients with ARMs. Development of a standardized OB/GYN residency curriculum and education for practicing OB/GYN physicians is necessary to allow access to knowledgeable sexual and reproductive health for this patient population.

Economic Impact of Daily Bowel Management Regimens

BackgroundChronic constipation and fecal incontinence are devastating problems for patients with anorectal malformations, Hirschsprung's disease, idiopathic constipation, and spina bifida/spinal cord injuries. Finding the proper regimen allows these patients to be out of diapers and free of stool accidents. A typical bowel management regimen consists of daily laxatives or enemas; because these products are sold over the counter, insurance companies do not cover them. This study reviews the cost of bowel regimens and analyzes their economic impact on the families we treat. MethodsA retrospective review of patients undergoing bowel management between January 2016 and September 2023 was done. The mean annual income of families was calculated using their zip codes. ResultsUpon review, 430 patients met inclusion criteria; 167 were on laxatives, and 263 were using enemas. There was significant variation in the cost of medications based on the distributors they were bought from and the dose of the medication in their regimen. The cost of laxatives ranged from $15.70 to $2938.10 annually. The cost of enemas containing glycerin ranged from $29.20 to $4380.00 annually. In comparison, diapers/incontinence briefs ranged from $131.40 to $4343.50 annually. The median annual income of patients across 41 states ranged from $32,192 to $225,119. ConclusionsOur findings emphasize the importance of proper counseling families regarding medication costs in the market, intending to promote long-term treatment adherence. They also serve as a data source to advocate for improved insurance coverage of the medications required to manage these chronic conditions successfully. Level of EvidenceIV.

What Happens Post-Malone? An Investigation of Long-Term Postoperative Management of Antegrade Continence Enemas

BackgroundAn option for medically refractory fecal incontinence and/or constipation is the antegrade continence enema (ACE). We investigated ACE usage and its perceptions, including whether patients were able to discontinue use of the appendicostomy/cecostomy tube. MethodsPatients who underwent appendicostomy creation or cecostomy tube placement at two institutions between 2012 and 2021 were reviewed. Patients or parents/guardians were contacted for completion of a survey. Summary statistics for clinical data were tabulated and associations were evaluated with chi-square analysis. ResultsA total of 165 patients were included, including 92 (55.8%) males. Eighty-two (49.7%) surveys were completed. Most patients (51.5%) presented with fecal incontinence; 38 (23.3%) presented with constipation. More patients had a primary underlying diagnosis of anorectal malformation (39.4%), followed by functional constipation (21.2%), Hirschsprung disease (18.8%), and spinal malformation (17.6%). Thirty-six (21.8%) patients discontinued flushes by time of contact, with switch to laxatives being the most common reason (19%), followed by appendicostomy stricture/obstruction/closure (17%), switch to ileostomy/colostomy (17%), and patient preference (14%). There was no difference in patients’ ability to stop using flushes based on underlying diagnosis (p = 0.31). The majority (84.1%) of respondents were “very likely” to recommend antegrade enemas to other children with similar diagnosis and 76.8% reported being “very satisfied” that the operation was done. ConclusionsThere remains a high degree of satisfaction with antegrade continence enemas for children with constipation and fecal incontinence; some children may be able to stop using antegrade enemas with varied mechanisms including patient/family weaning versus with assistance and laxative trials. Type of StudyRetrospective cohort study. Level of EvidenceIII.

Impact of Epithelial Claudin-4 and Leukotriene B4 Receptor 2 in Normoganglionic Hirschsprung Disease Colon on Post Pull-through Enterocolitis

PurposeTo investigate whether Leukotriene B4 receptor 2 (BLT-2), an upstream regulator of tight junction protein (TJP) Claudin-4, and TJPs could be etiologic factors in Hirschsprung-associated enterocolitis (HAEC) after pull-through (PT) for Hirschsprung disease (HD). MethodsNormoganglionic colon (HD-N) and aganglionic rectum (HD-A) specimens from rectal/rectosigmoid (R/RS) or descending/transverse (D/T) HD were assessed using quantitative polymerase chain reaction (qPCR) for Occludin, TJP-1, TJP-2, Junctional adhesion molecule (JAM)-1, JAM-2, Claudin-1, Claudin-3, Claudin-4, and BLT-2 and immunoblotting for Claudin-4 using fresh specimens obtained intraoperatively (2021–2024; n = 17; R/RS = 15 and D/T = 2). Claudin-4 immunohistochemistry was also evaluated quantitatively using preserved (n = 29; R/RS = 20 and D/T = 9; 2009–2021) and fresh HD specimens for comparison with anorectal malformation patients having colostomy closure as controls (n = 42) and between HD-A versus HD-N, R/RS versus D/T, and HAEC (+) versus HAEC (−). Technically inadequate or transitional zone PT were excluded. ResultsSubjects were 123 PT cases. Mean ages at PT/colostomy closure (years) were R/RS: 2.7 ± 2.9, D/T: 1.6 ± 2.2, and controls: 1.4 ± 0.7. Postoperative HAEC occurred 18 times in 14 PT cases (grade I = 5, grade II = 13). Post-PT HAEC was significantly more frequent in D/T (50.0% versus 6.4%; p < 0.001); Claudin-4 was significantly lower in HD-N from post-PT HAEC cases, especially D/T (p < 0.05) on immunohistochemistry. Claudin-4 was significantly lower in HD-N/HD-A compared with controls on immunoblotting (p < 0.05) and immunohistochemistry (p < 0.001). qPCR showed TJP-1, TJP-2, JAM-1, JAM-2, Claudin-4, and BLT-2 were significantly lower in HD-N/HD-A compared with controls. ConclusionsLower Claudin-4 and BLT2 in post-PT HAEC HD-N (especially D/T) suggests generalized epithelial barrier derangement with possible etiologic implications for HAEC. Level of EvidenceⅡ

Intra-amniotic delivery of tropomodulin 3 rescues cell apoptosis induced by miR-200b-3p upregulation via non-canonical nuclear factor kappa B pathways in ethylene thiourea induced anorectal malformations fetal rat

Ethylene thiourea (ETU), a metabolite of the fungicide ethylene bisdithiocarbamate (EBDC), has received great concern because of its harmful effects. ETU-induced anorectal malformations (ARMs) in rat models have been reported and widely used in the study of ARMs embryogenesis. Dysplasia of the lumbosacral spinal cord (LSSC), pelvic floor muscles (PFMs), and hindgut (HG) during intrauterine life affects postoperative defecation in patients with ARMs. However, the underlying toxic effects of ETU and pathological mechanisms in the three defecation-related tissues of fetuses with ARMs have not been reported. Thus, this study aimed to elucidate the molecular mechanisms involved in ARMs, with a focus on the dysregulation of miR-200b-3p and its downstream target tropomodulin 3 (TMOD3). The mRNA and protein levels of miR-200b-3p and TMOD3 in LSSC, PFMs, and HG of fetal rats with ARMs were evaluated by reverse transcription quantitative polymerase chain reaction and Western blotting (WB) on embryonic day 17 (E17). Further, a dual-luciferase reporter assay confirmed their targeting relationship. Gene silencing and overexpression of miR-200b-3p and TMOD3 were performed to verify their functions in HEK-293 T cells. Fetal rats with ARMs also received intra-amniotic microinjection of Ad-TMOD3 on E15, and key molecules in nuclear factor kappa (NF-κB) signaling and apoptosis were evaluated by WB on E21. Abnormally high levels of miR-200b-3p inhibited TMOD3 expression by binding with its 3′-untranslated region, leading to the activation of the non-canonical NF-κB signaling pathway, which is critical in the maldevelopment of LSSC, PFMs, and HG in ARMs rats. Furthermore, miR-200b-3p triggered apoptosis by directly targeting TMOD3. Notably, intra-amniotic Ad-TMOD3 microinjection revealed that the upregulation of TMOD3 expression mitigates the effects of miR-200b-3p on the activation of non-canonical NF-κB signaling and apoptosis in fetal rat model of ARMs. A novel miR-200b-3p/TMOD3/non-canonical NF-κB signaling axis triggered the massive apoptosis in LSSC, PFMs, and HG of ARMs, which was restored by the intra-amniotic injection of Ad-TMOD3 during embryogenesis. Our results indicate the potential of TMOD3 as a treatment target to restore defecation.

Massive sigmoid megacolon due to giant fecaloma: A case report of anal stricture

BackgroundA fecaloma is a mass of hardened feces in the colon or rectum, more solid than typical impacted feces, leading to severe intestinal distension. It predominantly affects elderly, frail individuals, children with anorectal deformities, and patients with conditions such as Chagas disease, Hirschsprung's disease, and spinal injuries. Case ReportWe report the case of a 34-year-old man with a history of anal imperforation and subsequent anal stricture, who presented with a massive megacolon due to a giant fecaloma. The patient experienced progressive abdominal distension, colicky pain, and an inability to pass stool for 15 days. Physical examination revealed a tender, distended abdomen and impacted feces in the rectum. Laboratory tests and imaging studies confirmed the diagnosis. An emergency laparotomy revealed a 30 × 30 cm dilated sigmoid colon filled with 15–25 kg of fecal material. The fecaloma was removed via enterectomy, and a Hartmann's procedure with colostomy was performed. Histopathological examination showed no evidence of malignancy. Post-operatively, the patient recovered well and was discharged in good condition. ConclusionThis case highlights the presentation and surgical management of a giant fecaloma causing megacolon. Prompt surgical intervention is critical in managing severe fecaloma to prevent life-threatening complications.

Current practice of diagnosis and treatment for rectourethral fistula in male patients with anorectal malformation: a multicenter questionnaire survey in Japan

PurposeSurgical procedures for anorectoplasty for anorectal malformations (ARMs), particularly rectourethral fistula (RUF), depend on the institution. We investigated the diagnosis and treatment of RUF in male patients with ARMs in Japan using a questionnaire survey.MethodsAn online survey inquiring about the diagnosis and treatment (diagnostic modalities, surgical approaches, fistula dissection devices, and fistula closure techniques) of each type of ARM in male patients was conducted among institutional members of the Japanese Study Group of Anorectal Anomalies. Fisher’s exact test was used to compare surgical methods between posterior sagittal anorectoplasty (PSARP) and laparoscopy-assisted anorectoplasty (LAARP).ResultsSixty-one institutions (100%) completed the survey. LAARP was the preferred approach for high-type ARM (75.4%). PSARP was preferred for intermediate-type ARM (59.0%). Monopolar devices were most commonly used (72.1%) for RUF dissection. Blunt dissection was more frequent in the PSARP group (PSARP vs. LAARP: 55.6 vs. 20.0%, p < 0.005). Cystoscopy/urethroscopy to confirm the extent of dissection was used more frequently in the LAARP group (70.0% vs. 25.0%, p < 0.005). Clips and staplers were used more frequently in the LAARP group (p < 0.05).ConclusionDistinct fistula management strategies for PSARP and LAARP were revealed. Further studies are needed to investigate the postoperative outcomes associated with these practices.

Applications of Indocyanine Green (ICG) fluorescence in pediatric colorectal conditions

Postoperative success in pediatric colorectal surgery hinges on achieving adequate perfusion during complex reconstructive procedures. This review synthesizes findings from recent publications regarding the application of Indocyanine Green (ICG) in various surgical interventions for conditions such as Hirschsprung disease, anorectal malformations, cloacal reconstructions, and others.Preliminary evidence suggests that ICG fluorescence enhances intraoperative decision-making by improving vascular visualization and assessing tissue perfusion. Compared to conventional methods, ICG may offer superior evaluation of intestinal perfusion.While initial reports indicate a favorable safety profile for ICG use in pediatric patients, further prospective studies are needed to confirm these observations, quantify benefits, and assess its impact on surgical outcomes. By providing real-time perfusion data, ICG holds promise for enhancing surgical precision and improving patient outcomes in pediatric colorectal surgery.

Identification of urological anomalies associated with anorectal malformation in southwestern Uganda: Limitations and opportunities

Anorectal malformations (ARMs) may be associated with congenital anomalies affecting other body parts namely vertebral, anorectal, cardiac, tracheoesophageal, renal, and limb (VACTERL) with varying incidences of 7%-60% . Genitourinary defects might occur approximately in 50% of all patients with anorectal malformations hence patients should be evaluated from birth to rule out these defects. To identify urological anomalies associated with anorectal malformation in southwestern Uganda. This was a descriptive retrospective cohort study conducted at our regional referral hospital in Southwestern Uganda involving patients who have undergone surgical correction of ARMs between June 2021 and July 2023. The overall prevalence of renal anomalies in our study patient population was 18.05%. Of those with ARM-associated renal anomalies, Specific anomalies included; renal agenesis (6.8%), hydronephrosis, (4.5%), duplex collecting system (3.8%), crossed fused kidney (1.5%), and ectopic kidney (0.75%). (Table) DISCUSSION: We found that the prevalence of ARM-associated renal anomalies was 18.05%, and the commonest anomaly was unilateral agenesis (6.8%) similar to other studies. Previous data have shown renal anomalies are common anomalies in ARM. While the exact values vary across studies, they all concluded that the rate of associated anomalies is extremely high in ARMs and warrants a thorough preoperative investigation once the ARMs are detected. This finding therefore underscores the importance of thorough evaluation and a multidisciplinary approach of care and follow-up system for ARM management including urologists even when the children are asymptomatic now. The main limitation of our study was missing information on patients' charts, we were not able to get the diagnosis since most patients didn't have their discharge forms at the time of evaluation. ARM associated with renal anomalies may remain undiagnosed and asymptomatic. Those identified as asymptomatic need to be followed in a multidisciplinary fashion including pediatric urologists.