A giant choledochal cyst in a 17 year old female managed in a resource limited setting: A case report.

INTRODUCTION AND IMPORTANCE: Choledochal cysts are rare congenital bile duct anomalies that lead to cystic dilatations of the biliary tree. This condition is very rare in Africa. When these cysts exceed 10 cm in diameter, they are referred to as giant choledochal cysts, which are much rarer. Giant choledochal cysts present both a diagnostic and surgical challenge. We present a case of a giant Choledochal cyst surgically managed in a resource limited setting with excellent outcome. CASE PRESENTATION: A 17-year-old female presented with 4 months history of progressive abdominal distension associated with abdominal pain, yellow discoloration of eyes, and occasional constipation. Abdominal CT-scan revealed a huge cystic mass in the right upper quadrant extending inferiorly to the right lumbar region. Complete excision of a type IA choledochal cyst was done plus cholecystectomy in addition to bilioenteric reconstruction. The patient recovered uneventfully. DISCUSSION AND CONCLUSION: To the best of our knowledge, this is the largest giant Choledochal cyst reported in literature. Even in a resource limited settings, sonography and a CT scan may be all that is required to make a diagnosis. During surgical excision, the surgeon should take extra caution to carefully dissect the adhesions off the giant cyst for a successful complete excision.

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.

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.

Urinary bladder cavernous hemangioma in a 3-year-old: A rare case report.

Cavernous hemangioma (CH) of urinary bladder occurs relatively infrequently, accounting for 0.6% of all bladder tumors. This tumor may occur sporadically or coexist with other benign and malignant vascular lesions. In this report, we present a rare case of CH in a 3-year-old Ugandan girl. A 3-year-old girl was referred to Mbarara Regional Referral Hospital (MRRH) for urological evaluation following a 3-year history of intravaginal swelling, dysuria, and heavy hematuria resulting in anemia. Imaging was consistent with polypoid bladder mass arising from the bladder trigone. Embryonal rhabdomyosarcoma was suspected based on clinical eyeballing. She was worked up for chemotherapy and received 26 cycles of vincristine sulfate, actinomycin-d, and cyclophosphamide (VAC). Biopsy and fulguration were performed after optimizing the patient. Histopathology confirmed CH. The surgery was uneventful and resulted in complete cure. CH should be considered in the differential diagnosis of childhood genitourinary masses. It is a rare entity in the real-life clinical practice and therefore can be overlooked. Excision biopsy and histology should be performed before initiating the patients to chemotherapy. CH is very insensitive to chemotherapy and therefore surgery maybe adequate in resource-limited settings.

Hair-on-End Sign in a 9-Year-Old Girl Presenting with Acute Stroke in Sickle Cell Disease.

BACKGROUND: The hair-on-end (HOE) sign is a rare finding seen in the diploic space on skull radiographs, computed tomography (CT) and magnetic resonance imaging (MRI) with the appearance of long, thin vertical striations of calcified spicules perpendicular to the bone surface that looks like hair standing on end. It is classically seen in children/adolescents with hemolytic anemias, in particular, thalassemia major and sickle cell disease. Here, we present a 9-year-old Ugandan girl who presented with stroke in whom head CT demonstrated cerebral intraparenchymal hemorrhage and multiple infarcts on the left with HOE sign. Hb electrophoresis confirmed the diagnosis of sickle cell anemia. CASE SUMMARY: We present a 9-year-old Ugandan girl who presented with an unexplained stroke that preceded an episode of acute headache, vomiting, followed by focal convulsions and altered consciousness. Clinical findings revealed severe pallor of the conjunctivae and mild scleral icterus. CT demonstrated right cerebral intraparenchymal hemorrhage, multiple high cerebral infarcts on the right and evidence of extra medullary hematopoiesis with a classical HOE sign. Hemoglobin (Hb) electrophoresis confirmed sickle cell disease (SCD). The child was then initiated on hydroxyurea, antibiotics, analgesics and intravenous fluids. She improved and was discharged 16 days later. Follow-up of the child was uneventful. CONCLUSION: HOE sign is a complication of chronic hemolysis usually seen in patients with thalassemia and sickle cell anemia. It is a rare finding that clinicians should be well conversant with, especially in Africa where sickle cell disease is common.

Giant Choledochal Cyst in an Adult at a Teaching Hospital in South-Western Uganda: A Case Report.

BACKGROUND: Choledochal cysts are rare congenital malformations characterized by cystic dilatations of the biliary tree. They are more prevalent in East Asian populations, but uncommon in western countries, and scarcely reported in African nations. They are classically diagnosed in children, and only 20-30% of cases are diagnosed in adulthood. Giant choledochal cysts are those with sizes exceeding 10cm, and are extremely rare with a few reported in the literature, thus the need for this case report. CASE: A 25-year-old Ugandan woman presented with an 8-month history of abdominal pain, jaundice and progressively increasing right upper quadrant abdominal mass. She underwent trans-abdominal ultrasonography that revealed a large, well-defined, echo-free mass in the right upper quadrant, extending to the lumbar region. Abdominal computed tomography (CT) revealed a huge thin-walled, cystic mass rising from the right wall of common hepatic duct, displacing the surrounding structures and no visible common bile duct. Complete cyst excision of a giant choledochal cyst type IVa was performed without decompression, in addition to cholecystectomy and reconstruction with hepaticojejunostomy and jejunojejunostomy anastomoses. CONCLUSION: Giant choledochal cysts are a very rare pathology worldwide. Diagnosis can still be made using ultrasound and CT where more advanced imaging modalities like endoscopic retrograde cholangiopancreatography (ERCP) are not available. Complete surgical resection of giant choledochal cysts is surgically challenging, but essential to relieve symptoms and prevent malignant transformation. To the best of our knowledge, this is the first reported case in the literature, of a giant choledochal cyst that was successfully treated in an African nation.

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.