RESUMEN

PURPOSE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: LS in ARM patients have no higher risk of prolapse or UTI than DS. DS had more complications, mostly wound infections, strictures and dehiscenses.

Asunto(s)

RESUMEN

Anorectal malformations (ARM) encompass a spectrum of rare congenital defects of the rectum and anus, requiring specialized reconstructive surgery. To improve epidemiological and clinical research in rare diseases such as ARM, collaborative efforts and patient registries are key. This retrospective study pools clinical data over a 30-year period from two ARM patient registries (The Royal Children's Hospital (RCH) in Melbourne, Australia, and the ARM-Network Consortium in Europe). It aims to draw comparisons on demographics, management, and outcomes between ARM patients in Australia and Europe. A total of 2947 ARM patients were included in the analyses. The RCH cohort had more complex ARM types (including rectal atresia and recto-vaginal fistula) and more associated anomalies, specifically skeletal, cardiac, and/or trachea-esophageal, than ARM-Net patients. Other patient characteristics were similar. Treatments clearly differed between the groups. European surgeons favoured the PSARP approach for both less complex and more complex ARM types, where Australian surgeons opted more often for cutback surgery in less complex, and laparoscopic assistance in more complex types. Complications were differently distributed, with less complications after LAARP and more after PSARP at RCH, compared to ARM-Net. While RCH patients more often required a redo, ARM-Net patients more commonly underwent anal dilatations. Anorectal malformation patients in Australia and Europe had minor differences in disease characteristics, and both operative and medical approaches differed. Joint efforts such as the present study emphasize the importance of collaboration to elucidate areas of improvement where surgeons may learn from each other across the world, ultimately improving patient outcomes. TYPE OF STUDY: Original Research. LEVEL OF EVIDENCE: III.

RESUMEN

Although 40 years have passed since the first case of DiGeorge's syndrome was described, and the knowledge about this disorder has steadily increased since that time, 22q11.2 deletion syndrome (DS) remains a challenging diagnosis because its clinical presentation varies widely. We describe an infant with 22q11.2 DS who presented with annular pancreas, anorectal malformation, Morgagni-type congenital diaphragmatic hernia, and ventricular septal defect. This constellation of anomalies has never been described in DiGeorge's syndrome. Here, we provide a case presentation and a thorough review of the literature.

RESUMEN

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.

RESUMEN

Congenital pouch colon (CPC) is a rare congenital abnormality, in which a pouch-like dilatation partially or completely replaces the colon, creating a fistula with the urogenital. Congenital colonic pouch is an extremely rare congenital disease mainly reported in India, and to date there are no reported cases in Lebanon. In this paper, we present a case of full-term male neonate diagnosed with a congenital colon pouch in Lebanon. A full-term neonate presented with imperforate anus, abdominal distention, and vomiting. Diagnostic assessments revealed a well-encapsulated mass compressing the intestines and ureter. Surgical intervention identified a type I CPC with a meconium-filled pouch directly connected to the small intestine, and an absent ileocecal valve, prompting an ileostomy. Following the surgery, no postoperative complications were noted. Early imaging can help address the diagnosis to start the appropriate management, plan for surgery to prevent the development of a megacolon and therefore, perforation. The objective is to be aware and consider congenital colonic pouch diagnosis after encountering newborns with such clinical presentation in order to direct clinical investigations toward the diagnosis and treatment of the case early, thus reducing the risk of complications and improving the patient's quality of life.

RESUMEN

In patients born with anorectal malformations (ARM), additional congenital heart defects (CHD) can occur. We aimed to provide an overview on disease and treatment details of CHD identified in patients born with ARM, from a unique large cohort of a very rare disease. We performed a retrospective single-center cohort study between January 2000 and July 2023. All consecutive patients with ARM were included. Outcomes were the number of patients with CHD, and screening percentage and percentage of patients diagnosed with CHD over 3 time periods (2000-2006, 2007-2014, 2015-2023). We used uni- and multi-variable logistic regression analyses to search for associations between CHD present and baseline characteristics. In total, 281 patients were included. Some 241 (85.8%) underwent echocardiography, of whom 80 (33.2%) had CHD. Screening percentage with echocardiography increased (74.1% vs. 85.7% vs. 95.9%, p < 0.001) and percentage of patients diagnosed with CHD remained similar over time (30.2% vs. 34.5% vs. 34.0%, p = 0.836). Atrial and ventricular septal defects (n = 36, n = 29), and persistent left superior vena cava (n = 17) were most identified. The presence of VACTERL-association or a genetic syndrome was independently associated with the presence of CHD. CHD were present in 33% of patients with ARM that underwent echocardiography. Over time, the number of CHD identified through screening remained similar. Patients with the presence of VACTERL-association or a genetic syndrome had a higher risk of having CHD. Therefore, acknowledging the potential presence of CHD in patients with ARM remains important.

RESUMEN

OBJECTIVE: To develop consensus on diagnostic criteria for LUMBAR syndrome, the association of segmental infantile hemangiomas that affect the Lower body with Urogenital anomalies, Ulceration, spinal cord Malformations, Bony defects, Anorectal malformations, Arterial anomalies and/or Renal anomalies. STUDY DESIGN: These diagnostic criteria were developed by an expert multidisciplinary and multi-institutional team based on analysis of peer-reviewed data, followed by electronic-Delphi consensus of a panel of 61 international pediatric specialists. RESULTS: After 2 Delphi rounds, a 92% or higher level of agreement was reached for each Delphi statement. 98% of panelists agreed with the diagnostic criteria, and 100% agreed the criteria would be useful in clinical practice. The diagnosis of LUMBAR requires the presence of a segmental, or patterned, infantile hemangioma of the lumbosacral, sacrococcygeal, or pelvic cutaneous regions plus one additional criterion of the urogenital, spinal, bony, anorectal, arterial, or renal organ systems. CONCLUSIONS: These diagnostic criteria will enhance clinical care by improving screening, detection, and overall awareness of this poorly understood neurocutaneous disorder. The criteria can be utilized by a wide variety of pediatric subspecialists. In addition, formal criteria will improve phenotypic uniformity among LUMBAR syndrome cohorts and a patient registry, allowing investigators to assess clinical features, long-term outcomes, and results of genetic sequencing in a standardized manner. Finally, these criteria will serve as a starting point for prospective studies to establish formal screening and management guidelines.

Asunto(s)

RESUMEN

BACKGROUND: Anorectal malformation (ARM) or Hirschsprung's disease (HD) in children impact on parents' burden of care and quality of life (QoL). The aim of this study was to investigate the relationship between caregiver burden and QoL in parents of children with ARM or HD. DESIGN AND METHODS: This cross-sectional study was conducted with 51 parents who completed the Zarit Burden Inventory (ZBI) and World Health Organization Quality of Life Scale-Short Form Turkish Version (WHOQOL-BREF-TR). RESULTS: The mean (±SD) ZBI score was 33.6 (±12.7), and 47.1% of parents (n = 24) perceived their caregiver burden as mild, 31.4% (n = 16) as moderate, and 3.9% (n = 2) as severe. According to the multivariate linear regression, associated anomalies (ß1 = 5.912), family income (ß1 = -6.007), stoma care (ß1 = 8.287), and diagnosis were identified to be significant determinants of caregiver burden. A negative, moderate, and significant relationship was identified between the ZBI scores and the physical domain (r = -0.417, p < .01), psychological domain (r = -0.421, p < .01), social relations domain (r = -0.398, p < .01), and environmental domain (r = -0.495, p < .01) scores of the WHOQOL-BREF-TR. CONCLUSIONS: The mothers perceived their caregiver burden as mild. However, a significant number of parents suffer from moderate to heavy caregiver burden. An increase in the caregiver burden of parents reduces their quality of life. PRACTICE IMPLICATIONS: Heightened awareness of the potential for caregiver burden and its association with quality of life among parents of children with ARM and HD may contribute to improved.

Asunto(s)

RESUMEN

The treatment of patients with colorectal disorders requires care from a wide variety of medical and surgical specialties over the course of their lifetime. This is ideally handled by a collaborative center which facilitates the assessment and development of patient care among multiple specialties which can enhance the quality and implementation of treatment plans, improve communication among different specialties, decrease morbidity, and improve patient satisfaction and outcomes. This collaborative approach can serve as a model for other parts of medicine requiring a similar multi-disciplinary and integrated method of care delivery. We describe the process, as well as the lessons learned in developing such a program.

RESUMEN

Anorectal malformations (ARMs) are congenital diseases that lead to postoperative fecal incontinence, constipation, and soiling, despite improvements in surgery; however, their pathological mechanisms remain unclear. Here, we report the role of microRNA-141-3p in maintaining homeostasis between apoptosis and autophagy in the lumbosacral defecation center of fetal rats with ARMs. Elevated microRNA-141-3p expression inhibited YIN-YANG-1 expression by binding its 3' UTR, and repressed autophagy and triggered apoptosis simultaneously. Then, adenylate cyclase 3 was screened to be the downstream target gene of YIN-YANG-1 by chromatin immunoprecipitation sequencing experiments, and Yin Yang 1 could positively activate the transcription of adenylate cyclase 3 by directly interacting with the motif GAGATGG and ATGG in its promoter. Intraamniotic microinjection of adeno-rno-microRNA-141-3p-sponge-GFP in fetal rats with ARMs on embryonic day 15 restored apoptosis-autophagy homeostasis. These findings reveal that microRNA-141-3p upregulation impaired homeostasis between apoptosis and autophagy by inhibiting the YIN-YANG-1/adenylate cyclase 3 axis, and that intraamniotic injection of anti-microRNA-141-3p helped maintain homeostasis in the lumbosacral defecation center of ARMs during embryogenesis.

RESUMEN

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

RESUMEN

We present a simple surgical technique aiming to improve urine outflow through the common urogenital sinus in cloaca and facilitate drainage of existing hydrocolpos. The study included three cases of cloaca with associated hydrocolpos that were operated during the period 2022 through 2023. The patient is placed in the prone position for a standard posterior sagittal anorectoplasty. The distal rectal fistula is severed flush with the vagina/sinus leaving an open defect in the posterior wall of the vagina/sinus. The defect is then widened distally via a vertical incision (∼1 cm) through the posterior wall of the common urogenital sinus toward but not reaching the perineum. This vertical defect is then closed horizontally displacing the posterior vaginal wall downwards toward the perineum (posterior sinuplasty). The postoperative recovery was uneventful in the three cases. Adequate drainage of hydrocolpos was confirmed by imaging at follow-up, as well as improvement of upper urinary tract dilatation. In selected cases of cloaca, posterior sinuplasty is a simple procedure that can be applied during anorectoplasty to provide effective drainage of associated hydrocolpos.

RESUMEN

BACKGROUND: Urinary tract drainage is necessary to facilitate the maneuver in laparoscopic-assisted anorectoplasty (LAARP). Male patients with Anorectal malformations (ARM) are often encounter difficult catheterization during surgery. OBJECTIVE: We pioneered the urinary catheterization through the urachus in ARM patients and evaluated the efficacy. METHOD: Fourteen ARM patients undergoing urinary catheterization through urachus between September 2015 and September 2023 were reviewed. RESULTS: The median age at the time of surgery was 0.06 months. Nine neonatal patients underwent the one-stage LAARP, while five patients underwent staged procedure. Four ARM patients with rectoprostatic fistula, while ten with rectobulbar fistula. All patients successfully underwent transurachal catheterization. No incidents of catheter blockage, accidental removal, or urinary tract infection symptoms were detected during hospitalization. CONCLUSIONS: Transurachal catheterization is a safe, effective, and cosmetic procedure for ARM patients with difficulty urethral catheterization. LEVEL OF EVIDENCE: IV.

RESUMEN

OBJECTIVE: Congenital heart diseases (CHD) are the most frequently associated anomalies with anorectal malformations (ARM). Nevertheless, many specific aspects of CHD in ARM patients have yet to be studied. The aims of this study were to evaluate the prevalence and distribution of CHD in neonates-infants with ARM, and to explore whether the severity of ARM, and the presence of VACTERL association, had an impact on CHD rate, severity, and timing at first cardiac surgery. STUDY DESIGN: All consecutive newborn-infants with ARM managed in our tertiary center (January-1999; December-2021) were collected from a prospective database and retrospectively analyzed. Prevalence and distribution of CHD in ARM patients were assessed. Patients were divided into groups depending on ARM severity and presence of VACTERL association. Pairwise comparison for CHD prevalence, severity, and timing at first cardiac surgery was performed between groups. RESULTS: Of 396 ARM patients identified, those with severe ARM showed a higher number of overall CHD compared to patients with non-severe ARM (36.7 % vs. 25.2 %, p = 0.032). VACTERL + patients had a significantly higher prevalence of CHD (73.4 % vs. 16.4 %; p <0.001) and major CHD (51.7 % vs. 26.9 %; p = 0.008) when compared with VACTERL-patients. Furthermore, VACTERL + patients underwent first cardiac surgery at a significantly younger age than VACTERL-patients (5.2 ± 15.2 months vs. 11.9 ± 6.3, p = 0.039). CONCLUSIONS: Patients with severe ARM had a higher number of CHD compared to patients with non-severe ARM. VATERL + patients had significantly more CHD and more severe CHD than VACTERL-patients. Early screening for CHD is strongly recommended in all newborns diagnosed with ARM before surgery. LEVEL OF EVIDENCE: III retrospective comparative study.

Asunto(s)

RESUMEN

In children with anorectal malformations (ARMs), it is essential to have a diagnostic imaging method that helps with the evaluation of the internal anatomy. In patients with a persistent cloaca, an ARM variant, in which the measurement of the urethral channel and common channel determines surgical management, there are multiple options for imaging. Magnetic resonance imaging (MRI) is an excellent method for this purpose, from which accurate measurements of channel length can be obtained. Additionally, the use of volumetric/isotropic sequences allows multiplanar reformatting. We present our experience with pelvic MRI and intracavitary non-paramagnetic contrast (MR genitography). This method uses volumetric T2-weighted images and the instillation of saline solution as a contrast agent to distinguish the common channel, length of the urethra, anatomy of the vagina, and presence and location of the rectal fistula. We believe this technique to be particularly useful for those working in settings with limited MRI resources.

Asunto(s)

RESUMEN

AIM: Compare the laparoscopic treatment (LT) and the posterior sagittal anorectoplasty treatment (ST) of the rectovaginal fistulas (RvaF) in a single center. We have previously reported feasibility and results of LT in this rare variety of anorectal malformations (ARM) [1-3]. MATERIAL AND METHODS: 19 patients were treated between February 2000 and November 2020. Nine underwent a LT and 10 a ST. Both surgical techniques were previously described. [2][4][5] The distal posterior wall of the vagina was kept intact in the LT. A technical change was introduced in the ST for that purpose. The fistula was treated from the inside of the rectum, avoiding the opening of the distal vagina as described for the treatment of a urethra rectal bulbar fistula in males. Age at operation, associated anomalies, sacral ratio index (SR), complications, urinary continence, presence of spontaneous intestinal movements, constipation, soiling and requirements of bowel management program (BMP) were analyzed. RESULTS: Associated anomalies occurred in 17 patients (89.5 %), 63 % of which were urological. Five (26 %) had a SR below 0.4; 4 in the LT group and 1 in the ST group. The mean age at the time of operation was 23.2 (8-59) in ST and 17.6 months (4-32) in LT. Average operative time was 190.4 min for ST (120-334) and 195.8 min (90-270) for LT (p 0.13). One patient in the LT group presented a mild rectal prolapse and 2 a partial wound dehiscence after the ST. Only 15 patients were evaluable for functional results (8 in ST and 7 in LT). Mean follow up was 83 months (12-197). All patients are clean with a bowel management program. Five of the 7 patients undergoing a LT had a bad prognosis (SR < 0,4). Three (43 %) are clean with diet or any treatment, 3 (43 %) using laxatives or enemas and 1 (14 %) with a trans anal irrigation system. Only 1 of the 8 patients in the STgroup had a bad prognosis. Six (40 %) needed a diet; 4 (50 %) laxatives or enemas and 1 (10 %) a cecostomy button for antegrade enemas. CONCLUSIONS: Patients with RvaF had a high index of associated anomalies. The difference of operative time was not statistically significative. No differences in functional results between both groups were observed. LT is a valid option to treat RvaF.

Asunto(s)

RESUMEN

During cloacal reconstruction, we consider both common channel (CC) and urethral length when deciding between total urogenital mobilization (TUM) and a urogenital separation (UGS). Our concern about TUM in the case of a short urethra is resultant bladder neck incompetence, while conversion to UGS after completing the TUM dissection may result in urethral injury. Use of an interposition flap harvested from the ischiorectal fossa is another measure we use to prevent potential complications.

RESUMEN

Objective To investigate the relationship between the development of terminal rectal ganglion and spinal cord/sacral abnormalities in boys with complex anorectal malformations(ARMs)in order to improve the understanding of rectal ganglion development abnormalities in ARMs patients.Methods A retrospective trial was conducted on the male patients with complex ARMs admitted to our hospital from 2015 to 2021.The terminal rectal specimens were taken from them during anoplasty.According to the findings on development of terminal rectal ganglion after HE staining,the patients were classified into G1 group(ganglion cells observed)and G2 group(no ganglion cells observed).Imaging techniques were used to evaluate whether there were abnormalities in the spinal cord and sacrum,and their correlation with the terminal rectal ganglion development was analyzed.Results A total of 139 patients were enrolled,and their median age at anoplasty was 5.77(4.57,6.97)months.There were no significant differences between the G1(n=80,57.6％)and G2(n=59,42.4％)groups in ARMs pathological type(P=0.706)and age at surgery(P=0.140).Radiological findings showed there were 48 cases(34.5％)of spinal cord anomalies(SCA),25 cases(18.0％)of sacral abnormalities and 18 cases(12.9％)of coccyx abnormalities.No significant differences were observed in the incidences of SCA and sacral abnormalities between the G1 and G2 groups(P＜0.05).Moreover,the differences of fatty filum terminale and syrinx were statistically significant(P＜0.05).In addition,the ratio of sacrum to coccyx between the G1 and G2 groups were 0.72±0.10 vs 0.67±0.12(P＜0.05)of the anteroposterior position and 0.77±0.09 vs 0.72±0.09(P＜0.05)of the lateral position.Multivariate logistic regression analysis showed that sacral abnormalities,fatty filum terminale and syrinx were independent predictors of rectal terminal ganglion absence in male patients with complex ARMs.Conclusion The development of terminal rectal ganglia in male patients with ARMs is closely associated with the abnormalities of spinal cord and sacrum.Sacral abnormalities,fatty filum terminale and syrinx are independent predictors of rectal terminal ganglion absence in male patients with complex ARMs.

RESUMEN

BACKGROUND: Female anorectal malformation is a correctable congenital defect. Delayed manifestations in patients with anal deformities are uncommon, especially after adolescence. CASE SUMMARY: The clinical data of a 19-year-old adult female patient with congenital anal atresia accompanied by rectovestibular fistula as the main manifestation was retrospectively analyzed. Diagnosis was made based on the patient's clinical symptoms, signs, imaging showing the fistula, X-ray and magnetic resonance imaging results. The preoperative examination was improved. Anorectoplasty was performed. The patient exhibited an improvement in quality of life and presented no evidence of fecal incontinence during the 6-mo follow-up. CONCLUSION: Transfistula anorectoplasty is a reasonable and reliable surgical method for the treatment of adult congenital anal atresia and rectovestibular fistula.

RESUMEN

The clinical presentation, diagnosis and management of anorectal malformation has been well described in the literature, however the experience with these conditions in low-and middle-income countries is often shaped in unique ways due to the social, cultural and economic factors at work in these regions. This leads to adaptation of modifications in management options for these babies that usually present as delayed cases with added poor prognostic factors like sepsis leading to need for emergency resuscitation and overall increased morbidity and mortality. This article explores the anomaly from a global surgery lens and outlines the spectrum of the anomaly, burden faced in the resource constrained environment and the management options adopted for successful management under the available circumstances.

Asunto(s)