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.

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.

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.

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.

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.

A case report of an asymptomatic necrotic Meckel's diverticulum in an inguinal hernia during elective surgery in a resource limited setting: Littre's hernia.

INTRODUCTION AND IMPORTANCE: Although the common complications of Meckel's diverticulum (MD) are well known, that these congenital intestinal outpouchings may become involved as the content of abdominal hernia sacs is not well appreciated. MD is the most prevalent congenital abnormality of the gastrointestinal tract, but involvement in a hernia, known as Littre's hernia (LH), accounts for less than 1 % of MD cases. Incarcerated LH has been reported sporadically in the literature, with MD found in the sacs of paraumbilical, femoral, inguinal, and incisional hernias. PRESENTATION OF CASE: We report a LH in a 3-year-old male child who was scheduled for elective herniotomy for a reducible left inguinal hernia. Intraoperatively we found the hernia sac contained a necrotic and perforated MD with viable associated bowel loop. The patient was successfully managed by diverticulectomy and primary repair through a trans-inguinal incision and herniotomy was performed. CLINICAL DISCUSSION: LH is a rare presentation of MD, and preoperative diagnosis of LH is challenging. Even in the case of a strangulated MD, a patient may not present with the typical signs and symptoms associated with compromised viscous. Once identified, repair of Littre hernia consists of resection of the diverticulum, or segmental bowel resection if necessary, and herniotomy. CONCLUSION: The finding of a perforated MD during elective hernia repair emphasizes the importance of awareness of unusual variants of inguinal hernia, and the necessity of identifying a MD given the risk of sequelae in the case of necrosis or perforation, if not repaired.

Pediatric abdominal trauma in a National Referral Hospital.

Background: Trauma is a major contributor to pediatric morbidity and mortality. Injury and violence are a major killer of children throughout the world. Unintentional injuries account for almost 90% of these cases. They are the leading cause of death for children aged 10-19 years. More than 95% of all injury deaths in children occur in low income and middle-income countries. Abdominal trauma is present in approximately 25% of pediatric patients with major trauma and is the most common cause of unrecognized fatal injury in children. Objectives: To describe the patterns, the management and outcomes of pediatric abdominal trauma.This was a descriptive retrospective study. Data was extracted from the Pediatric surgery Unit database from January 2012 to July 2019 on all abdominal trauma admissions to the unit. Results: Falls were the commonest (51.3%) mechanism for trauma on the unit. Most (84%) of the admissions had blunt abdominal trauma, with the majority (77%) managed non operatively. Only 16% had penetrating trauma, with the majority (84%) of these managed operatively. The average length of hospital stay for most (71.9%) of the patients was less than 7 days, with 96.1% of all admitted patients being discharged upon recovery. Conclusion: Blunt abdominal trauma is the most common pattern of pediatric abdominal trauma, with majority of these patients being managed non-operatively with good outcomes. Selective non-operative management for penetrating pediatric abdominal trauma has good patient outcomes as well.

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.

Adenomatous Hyperplasia of the Gallbladder in the Setting of Mirizzi Syndrome, Mimicking Adenocarcinoma of the Gallbladder.

BACKGROUND: Adenomatous hyperplasia (AH) is an uncommon benign lesion of the gallbladder (GB), predominantly seen in men. AH is commonly confused with malignant GB neoplasms in the setting of chronic cholecystitis and gallstones. There is a scarcity of published literature on AH, suggesting its rarity and the need for this case report. CASE PRESENTATION: A 24-year-old woman from Western Uganda presented with signs and symptoms consistent with extrahepatic biliary obstruction. Trans-abdominal ultrasound scan revealed cholecystomegaly (13.9 cm by 4.29 cm), thickened GB wall at 5.2 mm, with a poorly defined hypoechoic polypoid solid mass involving the fundal body of the GB. Explorative laparotomy with cholecystectomy and lymph node sampling was performed. Histopathological examination was consistent with adenomatous hyperplasia of the GB. The symptoms and laboratory values improved on follow-up in the clinic after laparotomy. CONCLUSION: Adenomatous hyperplasia may be misdiagnosed as a malignant GB neoplasm, especially in the setting of chronic cholecystitis and gallstones. If a correct histopathological diagnosis is made, no further diagnostic work-up is necessary following surgical interventions.

15-Year-old with neglected recto-vestibular fistula in western Uganda: a case report.

BACKGROUND: Teenage and late presentation of anorectal malformations are not uncommon in developing world. Some of the reasons for late presentation include but not limited to illiteracy, poverty, lack of awareness, and limited trained pediatric surgeons. In rural areas, neonates with ARMs are considered cursed and are marginalized. CASE: 15-Year-old African girl (a munyankole by tribe in Uganda) from western Uganda presented at 15 years of life with colostomy and uncorrected anorectal malformation. Never went to school due to social stigma. CONCLUSION: Due to limited number of trained pediatric surgeons in most of African Countries, many children in addition to living with a colostomy or untreated malformation, may also be undiagnosed with chronic constipation. Improved awareness and advocacy would promote early presentation and treatment.

Implementation of a contextually appropriate pediatric emergency surgical care course in Uganda.

BACKGROUND: Low- and middle-income countries like Uganda face a severe shortage of pediatric surgeons. Most children with a surgical emergency are treated by nonspecialist rural providers. We describe the design and implementation of a locally driven, pilot pediatric emergency surgical care course to strengthen skills of these providers. This is the first description of such a course in the current literature. METHODS: The course was delivered three times from 2018 to 2019. Modules include perioperative management, neonatal emergencies, intestinal emergencies, and trauma. A baseline needs assessment survey was administered. Participants in the second and third courses also took pre and postcourse knowledge-based tests. RESULTS: Forty-five providers representing multiple cadres participated. Participants most commonly perform hernia/hydrocele repair (17% adjusted rating) in their current practice and are least comfortable managing cleft lip and palate (mean Likert score 1.4 ±â€¯0.9). Equipment shortage was identified as the most significant challenge to delivering pediatric surgical care (24%). Scores on the knowledge tests improved significantly from pre- (55.4% ±â€¯22.4%) to postcourse (71.9% ±â€¯14.0%, p < 0.0001). CONCLUSION: Nonspecialist clinicians are essential to the pediatric surgical workforce in LMICs. Short, targeted training courses can increase provider knowledge about the management of surgical emergencies. The course has spurred local surgical outreach initiatives. Further implementation studies are needed to evaluate the impact of the training. LEVEL OF EVIDENCE: V.

Social and financial barriers may contribute to a "hidden mortality" in Uganda for children with congenital anomalies.

BACKGROUND: The true incidence of congenital anomalies in sub-Saharan Africa is unknown. Owing to complex challenges associated with congenital anomalies, many affected babies may never present to a health facility, resulting in an underestimation of disease burden. METHODS: Interviews were conducted with Ugandans between September 2018 and May 2019. Responses from community members versus families of children with congenital anomalies were compared. RESULTS: A total of 198 Ugandans were interviewed (91 family members, 80 community members). All participants (N = 198) believed that seeking surgical care would lead to poverty, 43% (n = 84) assumed fathers would abandon the child, and 26% (n = 45) thought a child with a congenital anomaly in their community had been left to die. Causes of anomalies were believed to be contraceptive methods (48%, n = 95), witchcraft (17%, n = 34), or drugs (10%, n = 19). Of family members, 25 (28%) were advised to allow the child to die. Families with affected children were more likely to have a lower income (P < .001), believe anomalies could be treated (P = .007), but thought that allowing the child to die was best for the family (32% vs 9%; P < .0001). Monthly household income <50,000 Uganda shillings ($13 United States dollars) was a significant predictor of the father leaving the family (P = .024), being advised to not pursue medical care (P = .046), and believing that God should decide the child's fate (P = .047). CONCLUSION: Families face significant financial and social pressures when deciding to seek surgical care for a child with a congenital anomaly. Many children with anomalies may die and never reach a health facility to be counted, thus contributing to a hidden mortality.

Management of a huge symptomatic hemorrhagic mesenteric cyst.

Mesenteric cysts are uncommon benign tumors with atypical clinical presentations. An acute presentation may not necessarily warrant emergency surgery, and planned surgery can achieve excellent results if the patient is hemodynamically stable at presentation.

A Comparison of the Predictive Value of the Glasgow Coma Scale and the Kampala Trauma Score for Mortality and Length of Hospital Stay in Head Injury Patients at a Tertiary Hospital in Uganda: A Diagnostic Prospective Study.

INTRODUCTION: The prevalence rates of head injury have been shown to be as high as 25% among trauma patients with severe head injury contributing to about 31% of all trauma deaths. Triage utilizes numerical cutoff points along the scores continuum to predict the greatest number of people who would have a poor outcome, "severe" patients, when scoring below the threshold and a good outcome "non severe" patients, when scoring above the cutoff or numerical threshold. This study aimed to compare the predictive value of the Glasgow Coma Scale and the Kampala Trauma Score for mortality and length of hospital stay at a tertiary hospital in Uganda. METHODS: A diagnostic prospective study was conducted from January 12, 2018 to March 16, 2018. We recruited patients with head injury admitted to the accidents and emergency department who met the inclusion criteria for the study. Data on patient's demographic characteristics, mechanisms of injury, category of road use, and classification of injury according to the GCS and KTS at initial contact and at 24 hours were collected. The receiver operating characteristics (ROC) analysis and logistic regression analysis were used for comparison. RESULTS: The GCS predicted mortality and length of hospital stay with the GCS at admission with AUC of 0.9048 and 0.7972, respectively (KTS at admission time, AUC 0.8178 and 0.7243). The GCS predicted mortality and length of hospital stay with the GCS at 24 hours with AUC of 0.9567 and 0.8203, respectively (KTS at 24 hours, AUC 0.8531 and 0.7276). At admission, the GCS at a cutoff of 11 had a sensitivity of 83.23% and specificity of 82.61% while the KTS had 88.02% and 73.91%, respectively, at a cutoff of 13 for predicting mortality. At admission, the GCS at a cutoff of 13 had sensitivity of 70.48% and specificity of 66.67% while the KTS had 68.07% and 62.50%, respectively, at a cutoff of 14 for predicting length of hospital stay. CONCLUSION: Comparatively, the GCS performed better than the KTS in predicting mortality and length of hospital stay. The GCS was also more accurate at labelling the head injury patients who died as severely injured as opposed to the KTS that categorized most of them as moderately injured. In general, the two scores were sensitive at detection of mortality and length of hospital stay among the study population.

Prevalence and Predictors of Cancellation of Elective Surgical Procedures at a Tertiary Hospital in Uganda: A Cross-Sectional Study.

Introduction. The cancellation of elective procedures has been shown to waste resources and to have the potential to increase morbidity and mortality among patients. This study aimed to determine the prevalence of the cancellation of elective surgical procedures and to identify the factors associated with these cancellations at Mulago Hospital, a large public hospital in Kampala, Uganda. METHODS: A cross-sectional study was conducted from January 10, 2018, to February 20, 2018. We recruited patients of all ages who were admitted to surgical wards and scheduled for elective surgery. Data on patients' demographic characteristics and diagnosis, as well as the specialty of the surgery, the planned procedure, the specific operating theatre, cancellation, and the reasons for cancellation were extracted and analyzed using logistic regression. RESULTS: Of a total of 400 cases, 115 procedures were canceled-a cancellation prevalence of 28.8%. Orthopedic surgery had the highest cancellation rate, at 40.9% (n = 47). Facility-related factors were responsible for 67.8% of all cancellations. The most common reason for cancellation was insufficient time in the theatre to complete the procedure on the scheduled day. No procedures were canceled because of a lack of intensive care unit beds. There was a significant association between surgical specialty and cancellation (P < 0.05) at multivariate analysis. CONCLUSION: The prevalence of cancellation of elective surgical procedures at Mulago Hospital was 28.8%, with orthopedic surgery having the highest cancellation rate. Two-thirds of the factors causing cancellations were facility-related, and more than 50% of all cancellations were potentially preventable. Quality-improvement strategies are necessary in the specialties that are susceptible to procedure cancellation because of facility factors.

Long term bowel function after repair of anorectal malformations in Uganda.

BACKGROUND: Anorectal malformations (ARMs) are common congenital anomalies cared for at Mulago Pediatric Surgery Unit (PSU), similar to other sites in the region. All patients undergo staged repairs and complete treatment at older ages compared to high-income countries (HICs). This is the first study to examine long-term bowel function in our patients and compare with HICs. METHODS: A retrospective cohort study was conducted of all children 3-12 years old with ARMs who had repair between January 2012 and June 2017 and who completed surgical repair at least 6 months prior to the study. Bowel function was measured using the fecal continence scoring system derived from Rintala and Lindahl (1995). As in prior studies, patients were classified by score into four categories: Poor (6-9); Fair (9-11); Good (12-17); and Normal (18-20). RESULTS: Median follow up was 2 years post stoma closure. Long-term bowel function was Normal/Good in 65% (C.I 27, 45), and Fair/Poor 35% (C.I 55, 73), with soiling in 49% (C.I 40, 59), constipation in 23% (C.I 16, 32); and incontinence in 39% (C.I 30, 39). There was no statistically significant association between bowel function and multiple demographic, social, and clinical factors. Median age at completion of treatment (stoma reversal) was 2.3 years old, and median duration of colostomy (interval between stoma placement and takedown) was 1.8 years. CONCLUSION: Despite definitive repair at older age and almost two years of living with a stoma, our patients achieve fair long-term bowel function. Nonetheless, improved follow up and timely management of complications may improve outcomes soiling, incontinence and constipation. LEVEL OF EVIDENCE: Level II.

Prevalence and risk factors of gallstone disease in patients undergoing ultrasonography at Mulago hospital, Uganda.

BACKGROUND: Gallstone disease (GSD) is the most prevalent medical condition in the pancreatobiliary system. The burden of GSD and its complications are major public health issues globally. It is a common cause of surgical intervention, contributing substantially to health care costs. Most patients are asymptomatic, however 20% become symptomatic after 10 years. Its prevalence differs among populations and remains unknown in Uganda. OBJECTIVE: To determine the prevalence and risk factors of GSD in patients undergoing abdominal ultrasonography at Mulago hospital, Uganda. METHODS: This was a cross-sectional study at the Department of Radiology in Mulago hospital. Convenient sampling was used to recruit individuals having an abdominal ultrasound scan. Questionnaires were used to assess risk factors, and an abdominal exam was performed for individuals with gallstones to assess symptomatology. RESULTS: The prevalence of GSD was 22%. Statistically significant factors associated with GSD were a history of hormonal contraceptive use OR 3.2 (1.88-5.41) and a history of previous biliary symptoms OR 2.9 (1.68-4.91). Ninety-four percent of individuals with gallstones had epigastric/right upper quadrant pain. CONCLUSION: The prevalence of GSD is high in Mulago hospital; use of hormonal contraceptives and a previous history of biliary symptoms were significant risk factors for GSD in this study. Majority of patients with GSD were symptomatic with epigastric pain as the cardinal symptom. We recommend a countrywide screening program using abdominal ultrasonography to determine the prevalence of GSD in the general population. There is need to study further the risk of hormonal contraceptive use and GSD. Women on these contraceptives should be informed of the potential risk, and offered alternative options where feasible.