Pull-Through for Hirschsprung's Disease: Insights for Limited-Resource Settings From Mbarara.

INTRODUCTION: In many resource-limited settings, patients with Hirschsprung's Disease (HD) undergo initial diverting colostomy, followed by pull-through, and finally, colostomy closure. This approach allows for decompression of dilated and thickened bowel and improved patient nutritional status. However, this three-stage approach prolongs treatment duration, with significant stoma morbidity, costs, and impact on quality of life. Our aim was to determine whether pull-through for HD can safely be performed with simultaneous stoma closure, reducing treatment approach from three to two stages. METHODS: Children with HD and diverting colostomy were prospectively followed as they underwent pull-through with simultaneous stoma closure. Their in-hospital course and 3-mo outpatient course were assessed for postoperative complications. Patients with total colonic HD, redo pull-through, and residual dilated colon were excluded from the study. RESULTS: Of the 20 children, 17 were male (n = 17, 85%). All patients had rectosigmoid HD. The median weight, age at colostomy formation, and age at pull-through were 11.05 kg (interquartile range [IQR] 10-12.75), 0.9 y (IQR 0.25-2.8), and 2.08 y (IQR 1.28-2.75), respectively. Mean duration with colostomy before pull-through was 1.1 y (standard deviation 1.51). Median hospital length of stay was 6 d (IQR 5-7). Early complications included anastomotic leak (n = 1), perianal skin excoriation (n = 2), surgical site skin infection (n = 3), and fascial dehiscence (n = 1). Longer-term complications included stricture (n = 1, 5%) and enterocolitis (n = 2, 10%). CONCLUSIONS: In this small case series, we have demonstrated that pull-through with simultaneous stoma closure can be safely performed in resource-constrained settings. Further studies are needed to understand the quality of life and economic impact of this change in management for HD patients.

Understanding the Burden of Pediatric Traumatic Injury in Uganda: A Multicenter, Prospective Study.

INTRODUCTION: Traumatic injury is responsible for eight million childhood deaths annually. In Uganda, there is a paucity of comprehensive data describing the burden of pediatric trauma, which is essential for resource allocation and surgical workforce planning. This study aimed to ascertain the burden of non-adolescent pediatric trauma across four Ugandan hospitals. METHODS: We performed a descriptive review of four independent and prospective pediatric surgical databases in Uganda: Mulago National Referral Hospital (2012-2019), Mbarara Regional Referral Hospital (2015-2019), Soroti Regional Referral Hospital (SRRH) (2016-2019), and St Mary's Hospital Lacor (SMHL) (2016-2019). We sub-selected all clinical encounters that involved trauma. The primary outcome was the distribution of injury mechanisms. Secondary outcomes included operative intervention and clinical outcomes. RESULTS: There was a total of 693 pediatric trauma patients, across four hospital sites: Mulago National Referral Hospital (n = 245), Mbarara Regional Referral Hospital (n = 29), SRRH (n = 292), and SMHL (n = 127). The majority of patients were male (63%), with a median age of 5 [interquartile range = 2, 8]. Chiefly, patients suffered blunt injury mechanisms, including falls (16.2%) and road traffic crashes (14.7%) resulting in abdominal trauma (29.4%) and contusions (11.8%). At SRRH and SMHL, from which orthopedic data were available, 27% of patients suffered long-bone fractures. Overall, 55% of patients underwent surgery and 95% recovered to discharge. CONCLUSIONS: In Uganda, non-adolescent pediatric trauma patients most commonly suffer injuries due to falls and road traffic crashes, resulting in high rates of abdominal trauma. Amid surgical workforce deficits and resource-variability, these data support interventions aimed at training adult general surgeons to provide emergency pediatric surgical care and procedures.

Pediatric Emergency Surgery Course in Uganda: Long-Term Follow-Up and Insights From Further Dissemination.

INTRODUCTION: Approximately 170 pediatric surgeons are needed for the 24 million children in Uganda. There are only seven. Consequently, general surgeons manage many pediatric surgical conditions. In response, stakeholders created the Pediatric Emergency Surgery Course (PESC) for rural providers, given three times in 2018-2019. We sought to understand the course's long-term impact, current pediatric surgery needs, and determine measures for improvement. METHODS: In October 2021, we distributed the same test given in 2018-2019. Student's t-test was used to compare former participants' scores to previous scores. The course was delivered again in May 2022 to new participants. We performed a quantitative needs assessment and also conducted a focus group with these participants. Finally, we interviewed Surgeon in Chiefs at previous sites. RESULTS: Twenty three of the prior 45 course participants re-took the PESC course assessment. Alumni scored on average 71.9% ± 18% correct. This was higher from prior precourse test scores of 55.4% ± 22.4%, and almost identical to the 2018-2019 postcourse scores 71.9% ± 14%. Fifteen course participants completed the needs assessment. Participants had low confidence managing pediatric surgical disease (median Likert scale ≤ 3.0), 12 of 15 participants endorsed lack of equipment, and eight of 15 desired more educational resources. Qualitative feedback was positive: participants valued the pragmatic lessons and networking with in-country specialists. Further training was suggested, and Chiefs noted the need for more trained staff like anesthesiologists. CONCLUSIONS: Participants favorably reviewed PESC and retained knowledge over three years later. Given participants' interest in more training, further investment in locally derived educational efforts must be prioritized.

The creation of a pediatric surgical checklist for adult providers.

PURPOSE: To address the need for a pediatric surgical checklist for adult providers. BACKGROUND: Pediatric surgery is unique due to the specific needs and many tasks that are employed in the care of adults require accommodations for children. There are some resources for adult surgeons to perform safe pediatric surgery and to assist such surgeons in pediatric emergencies, we created a straightforward checklist based on current literature. We propose a surgical checklist as the value of surgical checklists has been validated through research in a variety of applications. METHODS: Literature review on PubMed to gather information on current resources for pediatric surgery, all papers on surgical checklists describing their outcomes as of October 2023 were included to prevent a biased overview of the existing literature. Interviews with multiple pediatric surgeons were conducted for the creation of a checklist that is relevant to the field and has limited bias. RESULTS: Forty-two papers with 8,529,061 total participants were included. The positive impact of checklists was highlighted throughout the literature in terms of outcomes, financial cost and team relationship. Certain care checkpoints emerged as vital checklist items: antibiotic administration, anesthetic considerations, intraoperative hemodynamics and postoperative resuscitation. The result was the creation of a checklist that is not substitutive for existing WHO surgery checklists but additive for adult surgeons who must operate on children in emergencies. CONCLUSION: The outcomes measured throughout the literature are varied and thus provide both a nuanced view of a variety of factors that must be taken into account and are limited in the amount of evidence for each outcome. We hope to implement the checklist developed to create a standard of care for pediatric surgery performed in low resource settings by adult surgeons and further evaluate its impact on emergency pediatric surgery outcomes. FUNDING: Fulbright Fogarty Fellowship, GHES NIH FIC D43 TW010540.

Hirschsprung disease: a cost analysis study of the direct, indirect costs and financial coping strategies for the surgical management in Western Uganda.

BACKGROUND: Surgical management of Hirschsprung disease (HD) in low- and middle-income countries is typically a staged procedure, necessitating multiple hospitalizations and clinic visits increasing family financial burden. Currently, there is limited information on the costs borne by caretakers of children with Hirschsprung disease seeking surgical intervention. This study seeks to measure the costs and economic burden of surgical treatment for Hirschsprung disease in western Uganda. METHODS: A cross-sectional study using cost analysis was conducted among caretakers of patients who completed surgical treatment of HD between January 2017 and December 2021 at two hospitals in western Uganda. The average direct and indirect costs incurred by caretakers presenting at a public and private hospital were computed. RESULTS: A total of 69 patients (M: F = 7:1) were enrolled in the study. The median age at diagnosis was 60.5 (IQR 3-151.25) days for children and two-staged pull-through procedure was the common surgery performed. The mean overall cost for treatment was US $960 (SD = $720), with the majority of costs coming from direct medical costs. Nearly half (48%) of participants resorted to distress financing to finance their child's surgical care. The overwhelming majority of patients (n = 64, 93%) incurred catastrophic expenditure from the total costs of surgery for HD, and 97% of participants fell below the international poverty line at the time treatment was completed. CONCLUSION: Despite the availability of 'free care' from government hospital and non-profit services, this study found that surgical management of Hirschsprung disease imposed substantial cost burden on families with Hirschsprung disease patients.

One year overall survival of wilms tumor cases and its predictors, among children diagnosed at a teaching hospital in South Western Uganda: a retrospective cohort study.

BACKGROUND: Wilms tumor (WT) is the second most common solid tumor in Africa with both low overall survival (OS) and event-free survival (EFS) rates. However, no known factors are predicting this poor overall survival. OBJECTIVE: The study was to determine the one-year overall survival of WT cases and its predictors among children diagnosed in the pediatric oncology and surgical units of Mbarara regional referral hospital (MRRH), western Uganda. METHODOLOGY: Children's treatment charts and files diagnosed and managed for WT were retrospectively followed up for the period between January 2017 to January 2021. Charts of children with histologically confirmed diagnoses were reviewed for demographics, clinical and histological characteristics, as well as treatment modalities. RESULTS: One-year overall survival was found to be 59.3% (95% CI: 40.7-73.3), with tumor size greater than 15 cm (p 0.021) and unfavorable WT type (p 0.012) being the predominant predictors. CONCLUSION: Overall survival (OS) of WT at MRRH was found to be 59.3%, and predictive factors noted were unfavorable histology and tumor size greater than 115 cm.

Met and Unmet Need for Pediatric Surgical Access in Uganda: A Country-Wide Prospective Analysis.

INTRODUCTION: Children's surgical access in low and low-middle income countries is severely limited. Investigations detailing met and unmet surgical access are necessary to inform appropriate resource allocation. MATERIALS AND METHODS: Surgical volume, outcomes, and distribution of pediatric general surgical procedures were analyzed using prospective pediatric surgical databases from four separate regional hospitals in Uganda. The current averted burden of surgical disease through pediatric surgical delivery in Uganda and the unmet surgical need based on estimates from high-income country data was calculated. RESULTS: A total of 8514 patients were treated at the four hospitals over a 6-year period corresponding to 1350 pediatric surgical cases per year in Uganda or six surgical cases per 100,000 children per year. The majority of complex congenital anomalies and surgical oncology cases were performed at Mulago and Mbarara Hospitals, which have dedicated pediatric surgical teams (P < 0.0001). The averted burden of pediatric surgical disease was 27,000 disability adjusted life years per year, which resulted in an economic benefit of approximately 23 million USD per year. However, the average case volume performed at the four regional hospitals currently represents 1% of the total projected pediatric surgical need. CONCLUSIONS: This investigation is one of the first to demonstrate the distribution of pediatric surgical procedures at a country level through the use of a prospective locally created database. Significant disease burden was averted by local pediatric and adult surgical teams, demonstrating the economic benefit of pediatric surgical care delivery. These findings support several ongoing strategies to increase pediatric surgical access in Uganda.

Pediatric Surgical Waitlist in Low Middle Income Countries During the COVID-19 Pandemic.

INTRODUCTION: Coronavirus disease-19 led to a significant reduction in surgery worldwide. Studies, however, of the effect on surgical volume for pediatric patients in low-income and middle-income countries (LMICs) are limited. METHODS: A survey was developed to estimate waitlists in LMICs for priority surgical conditions in children. The survey was piloted and revised before it was deployed over email to 19 surgeons. Pediatric surgeons at 15 different sites in eight countries in sub-Saharan Africa and Ecuador completed the survey from February 2021 to June 2021. The survey included the total number of children awaiting surgery and estimates for specific conditions. Respondents were also able to add additional procedures. RESULTS: Public hospitals had longer wait times than private facilities. The median waitlist was 90 patients, and the median wait time was 2 mo for elective surgeries. CONCLUSIONS: Lengthy surgical wait times affect surgical access in LMICs. Coronavirus disease-19 had been associated with surgical delays around the world, exacerbating existing surgical backlogs. Our results revealed significant delays for elective, urgent, and emergent cases across sub-Saharan Africa. Stakeholders should consider approaches to scale the limited surgical and perioperative resources in LMICs, create mitigation strategies for future pandemics, and establish ways to monitor waitlists on an ongoing basis.

Pilot bowel management program at Mbarara Hospital, Uganda.

PURPOSE: Children with Hirschsprung's disease (HD) and anorectal malformations (ARM) may benefit from a bowel management program (BMP) to treat constipation and fecal incontinence. This study describes a pilot BMP in Uganda. METHODS: Patients treated for HD or ARM were recruited for the BMP. Local staff underwent training and progressively took over decision-making. The rates of patient involuntary bowel movements (IBMs) and provider confidence were evaluated pre- and post-BMP with questionnaires. The results were compared with Fischer's exact test. RESULTS: Ten staff-2 surgeons, 6 nurses and 2 physiotherapists-and 12 patients participated. Patient median age was 4.5 years (IQR 3-6.6) and ten were male. Ten reported at least daily IBMs prior to the BMP. All patients underwent a clean-out. The parents were then taught to perform daily enemas or irrigations. Specific regimens were determined by patient history and imaging and titrated throughout the BMP. There were differences in the rates of both daytime and nighttime IBMs before and after the BMP (p = 0.0001 and 0.002, respectively). All staff reported increased confidence. CONCLUSIONS: We describe the first BMP in Uganda. BMPs can successfully treat constipation and fecal incontinence in low-income countries, although there are challenges with resources and follow-up.

Burden and Outcomes of Neonatal Surgery in Uganda: Results of a Five-Year Prospective Study.

BACKGROUND: Ninety-four percent of congenital anomalies occur in low- and middle-income countries. In Uganda, only three pediatric surgeons and three pediatric anesthesiologists serve more than 20 million children. This study estimates burden, outcomes, coverage, and economic benefit of neonatal surgical conditions in Uganda. METHODS: A prospectively collected database was reviewed for neonatal surgical admissions from January 1, 2012, to December 31, 2017, at the only two sites with specialist pediatric surgical coverage. Outcomes were compared with high-income countries. Met and unmet need were estimated using disability-adjusted life years. Economic benefit was estimated using a value of statistical life-year approach. RESULTS: For 1313 neonatal admissions, the median age of presentation was 3 d, overall mortality was 36%, and median distance traveled was 40 km. Anorectal malformations were most common (18%). Postoperative mortality was 24%. Mortality was significantly associated with surgical intervention (P < 0.0001). Met need was 4181 disability-adjusted life years per year, which corresponds to a $3.5 million net economic benefit to Uganda, with a potential additional benefit of $153 million if unmet need were fully addressed. Approximately 2% of the total need is met by the health care system. CONCLUSIONS: Neonatal surgery is associated with improved survival for most conditions. Despite increases in workforce and infrastructure, a limited proportion of the need for neonatal surgery is currently being met. This is multifactorial, including lack of access to surgical care and severe shortages of workforce and infrastructure. Current and potential economic benefit to Uganda appears substantial.

Ugandan Medical Student Career Choices Relate to Foreign Funding Priorities.

INTRODUCTION: The surgical workforce in sub-Saharan Africa is insufficient to meet population needs. Therefore, medical students should be encouraged to pursue surgical careers and "brain drain" must be minimized. It is unknown to what extent foreign aid priorities influence medical student career choices in Uganda. METHODS: Medical students in Uganda completed an online survey examining their career choices and attitudes regarding career opportunities and funding priorities. Data were analyzed using descriptive statistics, and responses among men and women were compared using Fisher's exact tests. RESULTS: Ninety-eight students participated. Students were most influenced by inspiring role models, employment opportunities and specialty fit with personal skills. Filling an underserved specialty was near the bottom of the influence scale. Women placed higher importance on advice from mentors (p = 0.049) and specialties with lower stress burden (p = 0.027). Men placed importance on opportunities in non-governmental organizations (p = 0.033) and academia (p = 0.050). Students expressed that the most supported specialties were infectious disease (n = 65, 66%), obstetrics (n = 15, 15%) and pediatrics (n = 7, 7%). Most students (n = 91, 93%) were planning a career in infectious disease. Fifty-three students (70%) indicated plans to leave Africa for residency. Female students were more likely to have a plan to leave (p = 0.027). CONCLUSION: Medical students in Uganda acknowledge the career opportunities for physicians in specialties prioritized by the Sustainable Development Goals. In order to avoid "brain drain" and encourage students to pursue careers in surgery, career opportunities including surgical residencies must be prioritized and supported in sub-Saharan Africa.

Unifying Children's Surgery and Anesthesia Stakeholders Across Institutions and Clinical Disciplines: Challenges and Solutions from Uganda.

BACKGROUND: There is a significant unmet need for children's surgical care in low- and middle-income countries (LMICs). Multidisciplinary collaboration is required to advance the surgical and anesthesia care of children's surgical conditions such as congenital conditions, cancer and injuries. Nonetheless, there are limited examples of this process from LMICs. We describe the development and 3-year outcomes following a 2015 stakeholders' meeting in Uganda to catalyze multidisciplinary and multi-institutional collaboration. METHODS: The stakeholders' meeting was a daylong conference held in Kampala with local, regional and international collaborators in attendance. Multiple clinical specialties including surgical subspecialists, pediatric anesthesia, perioperative nursing, pediatric oncology and neonatology were represented. Key thematic areas including infrastructure, training and workforce retention, service delivery, and research and advocacy were addressed, and short-term objectives were agreed upon. We reported the 3-year outcomes following the meeting by thematic area. RESULTS: The Pediatric Surgical Foundation was developed following the meeting to formalize coordination between institutions. Through international collaborations, operating room capacity has increased. A pediatric general surgery fellowship has expanded at Mulago and Mbarara hospitals supplemented by an international fellowship in multiple disciplines. Coordinated outreach camps have continued to assist with training and service delivery in rural regional hospitals. CONCLUSION: Collaborations between disciplines, both within LMICs and with international partners, are required to advance children's surgery. The unification of stakeholders across clinical disciplines and institutional partnerships can facilitate increased children's surgical capacity. Such a process may prove useful in other LMICs with a wide range of children's surgery stakeholders.

Out-of-Pocket and Catastrophic Expenses Incurred by Seeking Pediatric and Adult Surgical Care at a Public, Tertiary Care Centre in Uganda.

BACKGROUND: Surgical care is critical to establish effective healthcare systems in low- and middle-income countries, yet the unmet need for surgical conditions is as high as 65% in Ugandan children. Financial burden and geographical distance are common barriers to help-seeking in adult populations and are unmeasured in the pediatric population. We thus measured out-of-pocket (OOP) expenses and distance traveled for pediatric surgical care in a tertiary hospital in Mbarara, Uganda, as compared to adult surgical and pediatric medical patients. METHODS: Patients admitted to pediatric surgical (n = 20), pediatric medical (n = 18) and adult surgical (n = 18) wards were interviewed upon discharge over a period of 3 weeks. Patient and caregiver-reported expenses incurred for the present illness included prior/future care needed, and travel distance/cost. The prevalence of catastrophic expenses (≥10% of annual income) was calculated and spending patterns compared between wards. RESULTS: Thirty-five percent of pediatric medical patients, 45% of pediatric surgical patients and 55% of adult surgical patients incurred catastrophic expenses. Pediatric surgical patients paid more for their current treatment (p < 0.01)-specifically medications (p < 0.01) and tests (p < 0.01)-than pediatric medical patients, and comparable costs to adults. Adult patients paid more for treatment prior to the hospital (p = 0.04) and miscellaneous expenses (e.g., food while admitted) (p = 0.02). Patients in all wards traveled comparable distances. CONCLUSIONS: Seeking healthcare at a publicly funded hospital is financially catastrophic for almost half of patients. Out-of-stock supplies and broken equipment make surgical care particularly vulnerable to OOP expenses because analgesics, anaesthesia and preoperative imaging are prerequisites to care.

The prevalence and factors associated with malnutrition among infants with cleft palate and/or lip at a hospital in Uganda: a cross-sectional study.

BACKGROUND: To determine the prevalence and factors associated with malnutrition among infants with Cleft palate and/or cleft lip (CP+/-L) at Comprehensive Rehabilitation for Uganda Hospital (CoRSU) in Uganda. METHODS: This was a cross-sectional study done on infants with CP+/-L and their caretakers admitted between November 2013 and October 2014 at CoRSU hospital which was the study setting. A questionnaire was answered by the infants' caretakers. The main outcome measure, malnutrition was defined and classified based on Z-scores obtained using the W.H.O Z-calculator in which weights of the infants in kilograms and lengths in centimeters respectively were placed. The values obtained were expressed as a proportion using all enrolled infants with CP+/-L as denominator. Multivariable analysis was used to determine the risk factors. RESULTS: A total of 44 infants with CP+/-L were enrolled. Of these, 77% were below 4 months of age and 97.7% were immunized. The male-to-female ratio was 1.06:1. About 59% had unilateral CP+/-L. A total of 93.2% were delivered at term with 69.4% having a birth weight greater than 3 kg. Generally, 68% were malnourished, with the highest burden among females (71.4%), infants below 4 months (73.5%) and those with unilateral CP+/-L (77%). About 57% had moderate-to-severe malnutrition. There was delayed supplementation to breast milk, with cow-milk as the main supplemental feed for all the infants. In the multivariable analysis, factors associated with malnutrition included, having caretakers lacking nutritional information post-delivery (OR = 3.8, p = 0.17), low birth weight (OR = 3.4, p = 0.20), and having less than 10 feeds/day (OR = 4.9, p = 0.09). CONCLUSION: CP+/-L infants born in Uganda suffer a high-burden of malnutrition. Preventional strategies are needed with focus on proper feeding information. Research on cost-effective feeds, feeding methods and reasons behind gender disparities in these infants is also required.

The Pediatric Emergency Surgery Course: Impact on Provider Practice in Rural Uganda.

PURPOSE: The Pediatric Emergency Surgery Course (PESC) trains rural Ugandan providers to recognize and manage critical pediatric surgical conditions. 45 providers took PESC between 2018 and 2019. We sought to assess the impact of the course at three regional hospitals: Fort Portal, Kabale, and Kiwoko. METHODS: We conducted a retrospective cohort study. Diagnosis, procedure, and patient outcome data were collected twelve months before and after PESC from admission and theater logbooks. We also assessed referrals from these institutions to Uganda's two pediatric surgery hubs: Mulago and Mbarara Hospitals. Wilcoxon rank-sum and Pearson's chi-squared tests compared pre- and post-PESC measures. Interrupted time-series-analysis assessed referral volume before and after PESC. RESULTS: 1534 admissions and 2148 cases were documented across the three regional hospitals. Kiwoko made 539 referrals, while pediatric surgery hubs received 116 referrals. There was a statistically significant immediate increase in the number of referrals from Fort Portal, from 0.5 patients/month pre-PESC to 0.8 post-PESC (95 % CI 0.03-1.51). Moving averages of the combined number of pyloromyotomy, intussusception reductions, and hernia repairs at the rural hospitals also increased post-course. Neonatal time to referral and referred patient age were significantly lower after PESC delivery. CONCLUSION: Our data suggest that PESC increased referrals to tertiary centers and operative volume of selected cases at rural hospitals and shortened time to presentation at sites receiving referrals. PESC is a locally-driven, validated, clinical education intervention that improves timely care of pediatric surgical emergencies and merits further support and dissemination. TYPE OF STUDY: Retrospective Cohort Study. LEVEL OF EVIDENCE: III.

Pediatric Surgery Collaboration in Uganda, the Benefits of Long Term Partnerships at Regional Referral Hospitals.

Background: In 2022 there were only seven pediatric surgeons in Uganda, but approximately 170 are needed. Consequently, Ugandan general surgeons treat most pediatric surgical problems at regional hospitals. Accordingly, stakeholders created the Pediatric Emergency Surgery Course, which teaches rural providers identification, resuscitation, treatment and referral of pediatric surgical conditions. In order to improve course offerings and better understand pediatric surgery needs we collected admission and operative logbook data from four participating sites. One participating site, Lacor Hospital, rarely referred patients and had a much higher operative volume. Therefore, we sought to understand the causes of this difference and the resulting economic impact. Methods: Over a four-year period, data was collected from logbooks at four different regional referral hospitals in Uganda. Patients ≤ 18 years old with a surgical diagnosis were included. Patient LOS, referral volume, age, and case type were compared between sites and DALYs were calculated and converted into monetary benefit. Results: Over four sites, 8,615 admissions, and 5,457 cases were included. Lacor patients were younger, had a longer length of stay, and were referred less. Additionally, Lacor's long-term partnerships with a high-income country institution, a missionary organization, and visiting Ugandan and international pediatric surgeons were unique. In 2018, the pediatric surgery case volume was: Lacor (967); Fort Portal (477); Kiwoko (393); and Kabale (153), resulting in a substantial difference in long-term monetary health benefit. Conclusion: Long-term international partnerships may advance investments in surgical infrastructure, workforce, and education in low- and middle-income countries. This collaborative model allows stakeholders to make a greater impact than any single institution could make alone.

3-year-old with Mayer-Rokitansky-Küster-Hauser syndrome and anorectal malformation: A case report.

INTRODUCTION AND IMPORTANCE: Anorectal malformation is associated with congenital anomalies affecting other body systems, including vertebral, anorectal, cardiac, tracheoesophageal, renal and limb defects. Mayer-Rokitansky-Küster-Hauser syndrome is a collection of several congenital defects in the female reproductive system, including congenital agenesis of the uterus, cervix, and upper two-thirds of the vagina, that could be associated with anorectal malformation. CASE PRESENTATION: A 3-year old female initially treated for vestibular fistula as she had only two orifices, who intra-operatively was discovered to have Mayer-Rokitansky-Küster-Hauser syndrome associated with cardiac, skeletal (foot), and anorectal anomalies. CLINICAL DISCUSSION: The diagnosis Mayer-Rokitansky-Küster-Hauser syndrome should be considered in a female patient presenting with anorectal malformation especially when two orifices are encountered in the perineum. Mayer-Rokitansky-Küster-Hauser syndrome can be associated with vestibular or rectovaginal fistula, of which the former is the most common type that coexists. Treatment involves surgical and nonsurgical approaches, Surgically a new cavity is created to replace the vagina with a mucous membrane lined canal such as a bowel segment. CONCLUSION: This case demonstrates that anorectal malformation with Mayer-Rokitansky-Küster-Hauser syndrome can be mistaken for a vestibular fistula, even in specialized centers like ours. Therefore, a high index of suspicion should be noted especially when examination under anesthesia confirms only two orifices in the perinium.

The Creation of a Pediatric Surgical Checklist for Adult Providers.

Purpose: To address the need for a pediatric surgical checklist for adult providers. Background: Pediatric surgery is unique due to the specific needs and many tasks that are employed in the care of adults require accommodations for children. There are some resources for adult surgeons to perform safe pediatric surgery and to assist such surgeons in pediatric emergencies, we created a straightforward checklist based on current literature. We propose a surgical checklist as the value of surgical checklists has been validated through research in a variety of applications. Methods: Literature review on PubMed to gather information on current resources for pediatric surgery, all papers on surgical checklists describing their outcomes as of October 2022 were included to prevent a biased overview of the existing literature. Interviews with multiple pediatric surgeons were conducted for the creation of a checklist that is relevant to the field and has limited bias. Results: 42 papers with 8529061 total participants were included. The positive impact of checklists was highlighted throughout the literature in terms of outcomes, financial cost and team relationship. Certain care checkpoints emerged as vital checklist items: antibiotic administration, anesthetic considerations, intraoperative hemodynamics and postoperative resuscitation. The result was the creation of a checklist that is not substitutive for existing WHO surgery checklists but additive for adult surgeons who must operate on children in emergencies. Conclusion: The outcomes measured throughout the literature are varied and thus provide both a nuanced view of a variety of factors that must be taken into account and are limited in the amount of evidence for each outcome. We hope to implement the checklist developed to create a standard of care for pediatric surgery performed in low resource settings by adult surgeons and further evaluate its impact on emergency pediatric surgery outcomes. Funding: Fulbright Fogarty Fellowship, GHES NIH FIC D43 TW010540.

Etiology, Clinical Presentations, and Short-Term Treatment Outcomes of Extrahepatic Obstructive Jaundice in South-Western Uganda.

Background: The diagnosis of extrahepatic obstructive jaundice (EHOJ) remains a challenge and is often made late in low-resource settings. Systematic data are limited on the etiology and prognosis of patients with obstructive jaundice in Uganda. The objective of this study was to determine the etiology, clinical presentations, and short-term treatment outcomes of patients managed for EHOJ at Mbarara Regional Referral Hospital (MRRH) in south-western Uganda. Methods: Between September 2019 and May 2020, we prospectively enrolled a cohort of patients who presented with EHOJ at MRRH. A pretested, semi-structured data collection tool was used to abstract data from both the study participants and their files. Results: A total of 72 patients, 42 (58.3%) of whom were male with a median age of 56 (range of 2 months to 95 years) were studied. Forty-two (58.3%) participants had malignancies: Pancreatic head tumors 20 (27.8%), cholangiocarcinoma 13 (18.1%), duodenal cancers 5 (6.94%), and gall bladder cancer 4 (5.6%). The remaining 30 (41.7%) participants had benign etiologies: choledocholithiasis 10 (13.9%), biliary atresia 7 (9.7%), pancreatic pseudo cyst 6 (8.3%), Mirizzi syndrome 5 (6.9%) and 1 (1.4%) each of chronic pancreatitis and choledochal cyst. Sixty-seven (93.1%) patients presented with right upper quadrant tenderness, 65 (90.3%) abdominal pain and 55 (76.3%) clay-colored stool. Cholecystectomy 11 (25.6%) and cholecystojejunostomy + jejunojejunostomy 8 (18.6%) were the commonest procedures performed. Twelve (17.0%) of cases received chemotherapy (epirubicin/cisplatin/capecitabine) for pancreatic head tumors and (gemcitabine/oxaliplatine) for cholangiocarcinoma. Mortality rate was 29.2% in the study, of which malignancy carried the highest mortality 20 (95.24%). Conclusion: Malignancy was the main cause of EHOJ observed in more than half of the patients. Interventions aimed at early recognition and appropriate referral are key in this population to improve outcomes.

Hirschsprung's disease in low- and middle-income countries.

Hirschsprung's disease (HD) is one of the most common causes of pediatric bowel obstruction in low- and middle-income countries (LMICs). This paper describes the unique aspects of presentation, diagnosis, management and post-operative care and outcomes of HD in LMICs. In LMICs, patients with HD are much more likely to present in a delayed fashion with subsequent increased morbidity and mortality including higher rates of chronic obstruction, malnutrition with failure to thrive, complete obstruction and perforation. There are multifactorial causes for delay, with opportunities to improve initial timely diagnosis and referral, support families to address socioeconomic and cultural barriers, and improve workforce and infrastructure resources to provide definitive care. In LMICs, the diagnosis is often made based on clinical presentation and radiographic findings as pathological services may be limited. Initial diversion with multi-stage procedure, instead of a single-stage pull-through, predominates. This is also a result of multifactorial causes, including initial presentation to general surgeons at first-level hospitals instead of pediatric surgeons, delayed presentation with sick, malnourished children with significantly distended bowel, and a lack of fresh-frozen pathological services to guide the extent of resection. Post-operatively, HD patients in LMICs experience higher complication and mortality rates - likely stemming from sicker baseline presentations and more limited resources. Significant recent advances in care have occurred for patients with HD in LMICs, while opportunities to continue to improve care remain.