BACKGROUND:Little is known about how patients with anorectal malformations (ARMs), their caregivers and healthcare providers perceive and experience transition from pediatric to adult care (transition of care) in low- and middle-income countries. This study aimed to explore the perceptions and experiences of young adults, adolescents, their caregivers, and healthcare providers regarding transition of care, as well as their perceptions of an ideal transition of care at the Johannesburg Pediatric Colorectal Clinic. METHODS:A qualitative, exploratory study was conducted employing rich pictures. A workshop was held for each of the four stakeholder groups. Participants were asked to draw a rich picture illustrating their perceptions and experiences of the transition of care, followed by a second picture depicting the ideal transition of care. Data were analyzed using Braun and Clarke's thematic analysis. RESULTS:Four overarching themes were identified: (1) Born to shine-living with ARM as a lifelong condition that shapes, but does not define, identity; (2) Golden gloves-pediatric services as trusted, emotionally safe spaces, contrasted with fear and uncertainty regarding adult care; (3) Growing up, letting go-transition experienced as both developmental progression and relational loss; and (4) Overwhelmed joint passion-system fragmentation, limited adult expertise in congenital colorectal conditions, poor information transfer, and reliance on informal pediatric workarounds. Transition of care was experienced as a fragile, relational and system-level process rather than a discrete transfer event. It was marked by the loss of trusted pediatric relationships, uncertainty regarding adult expertise, and fragmented information transfer. These experiences shaped participants' perceptions of an "ideal transition" as one that is relationally anchored, developmentally appropriate, and coordinated across services. Participants identified feasible, low-resource strategies, including: adolescent-focused clinics, joint pediatric adult consultations to build trust, identifiable adult "champions," and structured information-handover tools. CONCLUSION:Co-designed transition pathways offer a pragmatic opportunity to strengthen lifelong care for patients with ARMs in resource-constrained settings.

Antegrade continence enema (ACE) appendicostomy is widely used in children with complex colorectal and neurogenic bowel conditions, yet data from resource-constrained settings remain limited. We aimed to describe the experience of a single-center pediatric cohort in a low-middle-income country (LMIC). A retrospective review of all ACE procedures performed between 2012 and 2026 was conducted. Demographics, diagnoses, indications, complications, surgeries, and functional outcomes (assessed with Milan Bowel Function Questionnaire) were collected from clinical and operative records. Descriptive statistics were applied. Forty-five children (80

Background: Anorectal malformations (ARMs) are congenital defects requiring lifelong management, including surgeries and bowel management strategies. Transitioning from pediatric to adult care presents challenges, especially in low- and middle-income countries (LMICs) like South Africa, where fragmented healthcare systems complicate the process. This study examines the management of ARM patients transitioning to adult care at a Paediatric Colorectal Clinic in South Africa, emphasizing the need for structured transition programs. Methods: We reviewed the demographics, anatomical anomalies, surgical interventions, and outcomes of patients aged 9 years and older at the Johannesburg Paediatric Colorectal Clinic. Data was extracted from patient files and the clinic's database. Seventy patients met the inclusion criteria, and descriptive statistics were used for analysis. Results: Of 277 ARM patients, 70 (25%) met the inclusion criteria. The majority were male (64%), with a median age of 13 years (range: 10 to 32). Common malformations included recto-vestibular fistula (15.7%) and cloaca (12.9%). A total of 315 procedures were performed, with a mean of 4.5 operations per patient. Functional outcomes showed 89% achieved voluntary bowel control; however, 64% of patients aged 16 or older required antegrade continence enema. Forty percent of these patients needed ongoing nephrology and urology care. Conclusion: The findings highlight the complexity of ARM management, underscoring the need for a structured transition program to ensure continuity of care. Such programs are vital in low- and middle-income countries to improve long-term outcomes and quality of life for patients.

Delayed diagnosis of Hirschsprung disease (HD), defined as diagnosis after 12 months of age, is usually uncommon and, according to some authors, associated with a worse outcome. We aim to report our experience in a tertiary hospital in a Low-and-Middle-Income Country (LMIC). A retrospective analysis was conducted using the RedCap® Database of the Johannesburg Paediatric Colorectal Clinic, which includes data from all HD patients since 2017. Patients who had pull-through surgery in other hospitals were excluded. The remaining patients were divided into delayed and non-delayed. Demographic and clinical data, including the number and type of surgeries, complications, and functional outcomes, were collected and compared between the two groups using the Mann-Whitney U and Fisher’s Exact Test. Of 80 patients with HD, 26 (32.5

A posterior cloacal variant is a congenital malformation where a urogenital sinus terminates anterior to a normally placed anus. These are rare malformations with highly variable anatomy. We report on three cases of a novel phenotype of posterior cloaca encountered at our institutions between October 2021 and November 2023. Three newborn girls were referred with ambiguous external genitalia and an anorectal malformation. In all cases, a midline sac, which is likely fused labioscrotal folds, replacing the clitoris was noted anterior to the perineal orifices. Two of the three patients demised as a result of renal failure. The third patient underwent reconstruction and is well. This posterior cloacal phenotype appears to be frequently associated with severe renal insufficiency. In survivors of the neonatal period, a good cosmetic outcome is achievable. Functional outcomes remain to be assessed.

In low-and-middle-income countries (LMIC), a non-negligible number of cases of paediatric perineal trauma is observed. Trauma can arise from less conventional mechanisms such as traditional enemas and flame burns, alongside blunt and penetrating injuries. The data on surgical management and long-term outcomes are limited. This study aims to evaluate surgical treatment and continence outcomes post-reconstruction. A retrospective review was conducted at Johannesburg’s Chris Hani Baragwanath Academic Hospital (2018–2025). Cases of sexual abuse were excluded. Patients were stratified based on whether they required surgical reconstruction. Data analysed included demographics, injury cause, procedures, and long-term continence via the Krickenbeck questionnaire. Of 20 patients, 10 underwent reconstruction. In the conservative group, 7 (70

To compare functional bowel outcomes between patients followed-up at two referral centers in a high-income country and a low/middle-income country after anorectal malformation repair (ARM). Cross-sectional study using Krickenbeck, Rintala and Baylor Continence Scale questionnaires. Patients below three years of age, with developmental delay, or incomplete questionnaires were excluded. Associated malformations, surgical notes, toilet training, treatment and toilet setting were considered. Results were analyzed according to age and type of ARM. 68 patients from Milan and 65 from Johannesburg were included. Johannesburg population was younger (mean age 10.7 ± 6.1 years old vs 7.1 ± 4.7, p < 0.001). Perineal fistula was the most common ARM type in Milan (32/68, 47

BACKGROUND:In low- and middle-income countries (LMICs), pediatric colostomy care is associated with significant clinical, social, and economic challenges that negatively impact patient outcomes. This study aimed to identify key barriers to pediatric colostomy care and to develop a scoring system for barriers to colostomy care. METHODS:A modified Delphi study was conducted, involving caregivers of pediatric patients with colostomies and health care professionals managing such patients in Southern Africa. Forty individuals were invited to participate (20 caregivers and 20 health care professionals). In Round 1, participants were asked to list barriers to colostomy care via an online REDCap survey. Three authors (GB, CB, JS) thematically grouped identified barriers. In Round 2, participants rated the relevance of each statement: ≥ 75% agreement defined consensus. A virtual Round 3 refinement meeting was held with an expert panel to finalize the scoring system. RESULTS:Of the 40 invited individuals, 23 (57.5%) participated in Round 1: 12 health care professionals (9 doctors, 2 nurses, and 1 with an unknown profession) and 11 caregivers. Sixteen participants completed Round 2, with consensus reached on all barrier statements. In Round 3, 4 nurses and 4 doctors reviewed and refined the statements, resulting in a final 36-item JPCC barriers to colostomy care scoring system. CONCLUSION:This study presents the first scoring system specifically designed to measure barriers to pediatric colostomy care in Southern Africa. The scoring system offers a practical framework for research, clinical assessment, and advocacy. Further multicenter validation is recommended to assess its applicability across diverse settings.

Acquired rectovaginal fistulae (RVF) are a complication of paediatric HIV infection. We report our experience with the surgical management of this condition. We retrospectively reviewed the records of paediatric patients with HIV-associated RVF managed at Chris Hani Baragwanath Academic Hospital (2011–2023). Information about HIV management, surgical history, and long-term outcomes was collected. Ten patients with HIV-associated RVF were identified. Median age of presentation was 2 years (IQR: 1–3 years). Nine patients (9/10) underwent diverting colostomy, while one demised before the stoma was fashioned. Fistula repair was performed a median of 17 months (IQR: 7.5–55 months) after colostomy. An ischiorectal fat pad was interposed in 5/9 patients. Four (4/9) patients had fistula recurrence, 2/9 patients developed anal stenosis, and 3/9 perineal sepsis. Stoma reversal was performed a median of 16 months (IQR: 3–25 months) after repair. Seven patients (7/9) have good outcomes without soiling, while 2/9 have long-term stomas. Failure to maintain viral suppression after repair was significantly associated with fistula recurrence and complications (φ = 0.8, p < 0.05). While HIV-associated RVFs remain a challenging condition, successful surgical treatment is possible. Viral suppression is a necessary condition for good outcomes.

Background. In our clinical setting, a three-staged approach is consistently employed to manage patients with anorectal malformations (ARMs). Objective. To evaluate the safety and feasibility of end-colostomies, in particular subtypes of ARMs. Methods. The medical records of babies born with an ARM treated between 1 January 2017 and 31 December 2022 were reviewed. Information regarding the type of ARM, type of colostomy, complications during colostomy formation, posterior sagittal anorectoplasty (PSARP) and colostomy closure was recorded. Results. Ultimately, 194 patients were included. Of those, 137 patients had divided colostomies with distal mucus fistulas (DC) and 57 had end-colostomies (EC). Ninety-seven patients (50%) had perineal and vestibular fistulas, including 40 (41%) patients with DC and 57 (59%) with EC. For post-colostomy formation, eight (20%) complications were recorded in the DC group and nine (16%) in the EC group (p=0.78). Wound sepsis presented in six patients with DC and four with EC (p=0.3). Nineteen percent (16/85) of patients who had PSARP developed complications, including 4/37 (11%) with DC and 12/48 (25%) with EC (p=0.16). Of the 72 patients who underwent stoma reversal, six (8%) had complications post-operatively: three in the DC group and three in the EC group. Conclusion. For patients with rectoperineal and vestibular fistulas, where divided colostomies are not warranted, end colostomies provide a safe alternative.

A fetiform sacrococcygeal teratoma (homunculus) is a highly differentiated subgroup of mature cystic teratoma that resembles a malformed fetus. These tumours originate at the base of the coccyx and may vary in their intrapelvic and extrapelvic extent and location. It is important to differentiate this anomaly from fetus-in-fetu which have a higher degree of structural organisation. A 5-day old neonate presented with a type II sacrococcygeal fetiform teratoma. The mass contained both cystic and solid components. Upon surgical excision and coccygectomy, fully formed bowel was found inside the mass, as well as bones and other well- defined structures. The tumour was confirmed to be fully excised and no malignant or immature features were found on histopathological examination. The patient was last seen growing well with an AFP of 3.5 μg/l, 14 months after resection.

BACKGROUND:The diagnosis of Hirschsprung's disease (HD) by rectal suction biopsy (RSB) has cost implications that could be reduced by ascertaining the optimal number of specimens required. The aim was to audit our experience to optimise cost-effectiveness.METHODS:Medical records of all patients who underwent an RSB between January 2018 and December 2021 were reviewed. In 2020, we transitioned from using the Solo-RBT to the rbi2 system (requiring single-use cartridges). Descriptive statistics were reported and a comparative analysis of the diagnostic efficacy of the Solo-RBT versus the rbi2 system was performed. The cost of consumables was calculated according to the number of specimens submitted.RESULTS:Of 218 RSBs, 181 were first and 37 were repeat. The mean age at biopsy was 62 days (IQR 22-65). An average of two tissue specimens were obtained per biopsy. Of the 181 first biopsies, 151 were optimal and 30 suboptimal. HD was confirmed in 19 (10.5%) of the patients. Amongst biopsies where a single specimen was obtained, 16% were inconclusive, compared to 14% with two specimens and 5% with three specimens. The cartridges for the rbi2 system cost R530. If two cartridges are used at initial biopsy the total cost is double of a single tissue specimen sent for initial biopsy, and two specimens sent for repeat biopsies.CONCLUSION:In a low-resource setting, selecting the appropriate RSB system and obtaining a single specimen is sufficient to diagnose HD. Patients with inconclusive results should undergo a repeat biopsy where two specimens are obtained.

Objective A relative oversupply of pediatric surgeons led to increasing difficulties in surgical training in high-income countries (HIC), popularizing international fellowships in low-to-middle-income countries (LMIC). The aim of this study was to evaluate the benefit of an international fellowship in an LMIC for the training of pediatric surgery trainees from HICs. Methods We retrospectively reviewed and compared the prospectively maintained surgical logbooks of international pediatric surgical trainees who completed a fellowship at Chris Hani Baragwanath Academic Hospital in the last 10 years. We analyzed the number of surgeries, type of involvement, and level of supervision in the operations. Data are provided in mean differences between South Africa and the respective home country. Results Seven fellows were included. Operative experience was higher in South Africa in general (Delta x-=381; 95% confidence interval [CI]: 236-656;p<0.0001) and index cases (Delta x=178; 95% CI: 109-279;p<0.0001).In South Africa, fellows performed more index cases unsupervised (Delta x=71; 95% CI: 42-111;p<0.0001), but a similar number under supervision (Delta x=-1; 95% CI: -25-24;p=0.901). Fellows were exposed to more surgical procedures in each pediatric surgical subspecialty. Conclusion An international fellowship in a high-volume subspecialized unit in an LMIC can be highly beneficial for HIC trainees, allowing exposure to higher caseload, opportunity to operate independently, and to receive a wider exposure to the different fields of pediatric surgery. The associated benefit for the local trainees is some reduction in their clinical responsibilities due to the additional workforce, providing them with the opportunity for protected academic and research time.

Background: The purpose of this study is to describe all published studies of single-stage procedures for anorectal malformations and to perform a meta-analysis of studies that compared single-stage to staged procedures.Methods: Searches were conducted in Pubmed, Medline, Embase and CENTRAL. Meta-analysis was per-formed in RevMan and expressed as forest plots with odds ratios (OR) and 95% confidence intervals (CI).Results: Thirty-eight studies were included in the narrative synthesis. Nine studies were included in the meta-analysis, representing 537 patients. The majority (70%) of patients included in this meta-analysis had either perineal or vestibular fistulas. Surgical site infection (SSI) was defined as any reported in-fection involving the neoanus (both superficial infection and dehiscence) and occurred in 51 of the 291 patients who underwent single-stage procedures, and 26 of the 244 patients who underwent staged pro-cedure. Meta-analysis showed a 2.2 times higher risk of surgical site infection (SSI) amongst patients who undergo single-stage procedures (OR 2.22, 95% CI 1.26, 3.92). Six of the 293 patients (2%) who underwent single-stage procedures required a rescue ostomy for wound dehiscence. In LMIC the risk of wound dehis-cence was three-fold higher in single-stage (36/202) compared to staged procedures (12/126) (OR 3.07, 95% CI 1.42, 6.63). In HIC there was no evidence of an increased risk of wound dehiscence in patients who underwent a single-stage (15/91) compared to a staged procedure (14/118) (OR 1.51, 95% CI 0.65, 3.51). There is no evidence of a difference between single-stage versus staged procedures with regards to functional outcomes including voluntary bowel movements (79/90 versus 111/128), soiling (24/165 versus 20/203) or constipation (27/90 versus 36/128).Conclusion: This systematic review provides further evidence that single-stage procedures for selected patients with anorectal malformations are safe. Whilst there is evidence of an increased risk of SSI, this did not translate to a significant difference in long-term functional outcomes.Levels of evidence: Level II (c) 2022 Elsevier Inc. All rights reserved.

Background: Anogenital Condylomata Acuminata (AGCA) are caused by Human Papilloma Virus (HPV), which is one of the most common sexually transmitted illnesses in adults. Although commonly seen in the paediatric population, especially in the setting of immunocompromise, literature regarding transmission, viral type and management in this population is scant. The aim of this study was to assess the profile of patients presenting with anogenital warts in light of associated immunocompromise with Human Immunodeficiency Virus (HIV).Methods: Three years of patient records from Chis Hani Baragwanath Academic Hospital were reviewed (January 2017 - December 2019). Information collected included: gender, age of presentation, age at intervention, type and duration of medical treatment, type and number of surgical interventions, HIV status, and histology results. Fisher's and Pearson's test were used to assess correlation between immune status and surgical interventions necessary.Results: In the time frame considered, we treated 66 patients with AGCA . The average age was 4 years old (1-14). HIV status was recorded in 30 patients (15 positive and 15 negative). Only one patient out of 66 had a history of sexual abuse. Whilst the proportion of patients who required surgical intervention in the HIV negative and HIV positive groups was equal (2:1), the total number of surgical interventions needed to achieve clearance was significantly more in those with HIV ( p = 0.03).Conclusions: HIV positive patients with AGCA require more surgical interventions compared to HIV negative individuals. Further research will be conducted to ascertain the sub-type of HPV infection in this subset of patients and to assess if this impacts follow-up for future malignancy. Further research also needs to be conducted to ascertain whether surgical intervention should be instituted earlier in the treatment protocol for HIV positive children.(c) 2021 Elsevier Inc. All rights reserved.

Currarino syndrome (CS) is a rare condition that presents with any combination of a sacral defect, a presacral mass, and an anorectal malformation. This collection, referred to as Currarino's triad, may not necessarily present as all three abnormalities in the diagnosis of the syndrome. Anal canal duplication (ACD) is an even rarer occurrence. We present a case that lies on the CS spectrum with an associated ACD and discuss a complex surgical challenge that necessitated a customized management plan, devised through a multidisciplinary approach.

BACKGROUND:The precise burden of paediatric surgical care in South Africa is unknown. In the absence of epidemiological data, hospital-based study is a first step to gauge the burden and profile of paediatric surgical disease. We aim to describe the profile of pathology, pattern of referrals, and complications of paediatric surgical care at Chris Hani Baragwanath Academic Hospital (CHBAH).METHODS:A 1-year retrospective record review for the period 3/1/2019 to 1/1/2020 was conducted by evaluation of the morbidity and mortality databases of the Department of Paediatric Surgery (DPS). Number of admissions, consultations, complications, and surgeries performed were analysed and classified.RESULTS:A total of 11,932 unique patient encounters occurred. Emergencies (79%, 1841/2329) accounted for the majority of admissions. Trauma accounted for 49% (896/1841) of emergency admissions. Elective surgery constituted 52% (1202/2316) and emergency surgery 48% (1114/2316) of all procedures performed. The emergency department (55%, 1271/2329), outpatients department (19%, 447/2329), and peripheral hospitals (16%, 378/2329) were the source of the majority of admissions. A complication rate of 9% (208/2316) was observed.CONCLUSION:The high-volume subspecialist environment at CHBAH presents the ideal environment for delivery of specialist paediatric surgical services and training. Injury prevention, optimal use of existing resources, and additional physical, human and financial resources are required to meet the existing and predicted future burden of paediatric surgical disease.

We present a case and discuss the management of a posterior cloacal variant not as yet described in the literature. A 5-week-old infant presented to our institution with a posterior cloacal variant and transposition of the clitoris and labia. After initial radiological investigations, staged operative intervention was performed over a 1-year period. This included an initial laparotomy (with drainage of hydrocolpos and formation of a colostomy), a left ureteric reimplantation and a posterior sagittal anorectoplasty due to a rectoperineal fistula. The child is under continued long-term follow-up by our specialist pediatric surgical team.

Constipation and fecal incontinence in pediatric patients are conditions due to either functional or organic bowel dysfunction and may represent a challenging situation both for parents, pediatricians, and pediatric surgeons. Different treatments have been proposed throughout the past decades with partial and alternant results and, among all proposed techniques, in the adult population the Transanal Irrigation (TAI) has become popular. However, little is known about its efficacy in children. Therefore, a group of Italian pediatric surgeons from different centers, all experts in bowel management, performed a literature review and discussed the best-practice for the use of TAI in the pediatric population. This article suggests some tips, such as the careful patients’ selection, a structured training with expert in pediatric colorectal diseases, and a continuous follow-up, that are considered crucial for the full success of treatment.

Purpose The neonatal period is the most vulnerable period for a child. There is a paucity of data on the burden of neonatal surgical disease in our setting. The aim of this study was to describe the frequency with which index neonatal surgical conditions are seen within our setting and to document the 30-day outcome of these patients. Methods This was a single-centre prospective observational study in which all neonates with paediatric surgical pathology referred to the paediatric surgical unit with a corrected gestational age of 28 days were included. Results Necrotising enterocolitis was the most frequent reason for referral to the paediatric surgical unit ( n = 68, 34.34%). Gastroschisis was the most frequent congenital anomaly referred ( n = 20, 10.10%). The overall morbidity was 57.58%. Surgical complications contributed to 18.51% of morbidities. The development of gram negative nosocomial sepsis was the most frequent cause of morbidity ( n = 98, 50.78%). Mortality at 30 days was 21.74% ( n = 40). Sepsis contributed to mortality in 35 patients (87.5%), 16 of which had gram negative sepsis. Conclusion Gram-negative sepsis was a major contributing factor in the development of morbidity and mortality in our cohort. Prevention and improvement in infection control are imperative if we are to improve outcomes in our surgical neonates.