A choledochal cyst type IVa in a child treated with Roux-en-Y hepaticojejunostomy

A choledochal cyst is a bile duct anomaly that disrupts the transportation of bile from the liver to the gallbladder and small intestine. Choledochal cysts are rare, occurring in approximately one out of every 100,000 to 150,000 children in Western countries, with a girls-to-boys ratio of 4:1. Immediate surgery to excise the cyst and construct a biliary-enteric continuity is necessary to treat this condition. This case-report aimed to present a child with choledochal cyst type IVa who underwent a Roux-en-Y hepaticojejunostomy. A 3-year-11-month-old girl with an abdominal mass experienced jaundice, nausea, and vomiting over the past two years, which worsened in the last month. Abdominal ultrasonography indicated intrahepatic biliary dilatation. Abdominal computed tomography (CT) scan results confirmed a choledochal cyst type IVa, characterized by fusiform cyst dilatation at the bilateral intrahepatic bile duct, common hepatic duct, cystic duct, and common bile duct. The cyst exerted pressure on the pancreas and small intestine. Before the surgery, the patient was treated with ceftriaxone 100 mg/kg/day and gentamicin 5 mg/kg/day. Roux-en-Y hepaticojejunostomy was performed, involving the complete excision of the extrahepatic bile duct to reconstruct the biliary system. During the surgery, a retroperitoneal cyst measuring 20 cm x 10 cm with a volume of 200 cc was discovered. Following the surgery, the patient showed clinical improvement. Patient follow-ups indicated that no complications such as wound infection, acute pancreatitis, and the formation of pancreatic or biliary fistula occurred. This case highlights that Roux-en-Y hepaticojejunostomy proves to be an effective surgical approach for managing choledochal cyst type IVa in children, helping to prevent further complications

Herlyn-Werner-Wunderlich syndrome: Challenges in diagnosis and management

Herlyn-Werner-Wunderlich syndrome (HWWS), also known as OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly) is a complex congenital malformation characterized by a triad including uterine didelphys, hemivaginal obstruction, and ipsilateral renal agenesis. In this case report, we present a case of HWWS along with the challenges in diagnosis and multi-step treatment processes. A 25-year-old woman presented to Dr. Zainoel Abidin Hospital in Banda Aceh, Indonesia with a chief complaint of lower back pain for the past six months (two months after the marriage). The patient also complained of late menstruation, followed by thick yellow vaginal discharge resembling malodorous pus occurring after menstruation. Additionally, a history of fever and pain during intercourse was reported. Vaginal examination revealed purulent fluid emerging from a pinpoint hole on the right vaginal wall. Vaginal palpation revealed a two-branched uterus, along with a palpable cystic mass on the right vaginal wall. Ultrasound examination indicated the presence of the right and left hemi-uteri, measuring 4.7x1.35 cm and 5.7x1.26 cm in size, respectively with both ovaries appearing normal. Fluid accumulation was observed in the right hemivagina, while the left kidney exhibited normal features, and the right kidney showed signs of hypoplasia. The patient was diagnosed with uterus didelphys, fluid accumulation in the hemivagina, and right kidney hypoplasia. Magnetic resonance imaging (MRI) confirmed uterus didelphys and revealed narrowing of the right hemivagina, suggestive of HWWS. Hysteroscopy was performed to resect the vaginal septum with laparoscopic guidance, along with drainage of hematocolpos, pyocolpos and placement of an intracervical mold. Despite HWWs having conservative gradual management, it was opted to perform vaginal septum resection, hematocolpos and pyocolpos drainage and placement of an intracervical mould in this case, in order to relieve symptoms and restore the reproductive and sexual functions