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.