Caroli disease is a rare congenital malformation that predisposes to segmental cystic dilatation of the intrahepatic bile ducts. Banti syndrome is characterized by persistent splenomegaly due to chronic congestion, resulting in a low hematocrit and ultimately leading to pancytopenia. In this report, we describe a 29-year-old woman who presented with a >20-year history of hepatitis B surface antigen positivity and a >1-year history of recurrent fatigue and malaise. On examination, the patient had abdominal distension with marked splenomegaly (7 cm below the ribs) and ascites with tenderness of the abdominal muscles to palpation. A complete blood count showed a low white blood cell count, red blood cell count, and hemoglobin concentration. During the course of treatment, the patient developed multiple symptoms of pancytopenia and concomitant splenomegaly, and she was discharged after total splenectomy with good recovery. The combination of Banti syndrome and Caroli disease results in severe symptoms of portal hypertension.

UNASSIGNED: Splenectomy is the most frequently performed procedure as definitive management or as part of shunt surgery or devascularization in portal hypertension. Splenectomy is technically challenging because of the frequent coexistence of multiple collateral varices, splenomegaly, poor liver function, and thrombocytopenia. Early arterial ligation and late mobilization (EALDEM) is the traditional method for splenectomy in portal hypertension. Early spleen mobilization offers good control of the hilum. We aim to compare the effect of the early mobilization and delayed arterial ligation (EMDAL) technique with that of the conventional splenectomy technique in patients with portal hypertension.
UNASSIGNED: During the study period from September 2011 to September 2022, 173 patients underwent surgical intervention for portal hypertension at our institution. Among these patients, 114 underwent the conventional method of splenectomy (early arterial ligation and late splenic mobilization) while 59 underwent splenectomy with the EMDAL technique. Demographics were compared between the two groups. Intraoperative and postoperative outcomes were analyzed using the Mann-Whitney test in each group. A minimum follow-up of 12 months was performed in each group.
UNASSIGNED: Demographics and type of surgical procedure were comparable in the two surgical method groups. Median blood loss was higher in the conventional group than in the EMDAL method. The median duration of surgery was comparable in the two surgical procedures. Clavien-Dindo grade III/IV complications were reported more frequently in the conventional group.
UNASSIGNED: The splenic hilum can be controlled well and bleeding can be minimised with early mobilization and delayed arterial ligation.

Oral contraceptive pills (OCPs) have a known prothrombotic effect. Obliterative portal venopathy (OPV) can be seen in patients with underlying hypercoagulability. We present a case of a 19-year-old female patient taking OCPs who presented with obstructive jaundice. Her main concern was pruritis. An extensive workup was done to reach a diagnosis but it came back negative. A liver biopsy showed OPV. This was thought secondary to her OCP use. Her OCPs were discontinued which resulted in a complete resolution of her symptoms and laboratory abnormalities. Cases with a direct relationship between OPV and OCP use are extremely rare. More studies are required to establish a correlation between OPV and OCPs. OPV should be considered in the differential diagnosis among patients with obstructive jaundice without an obvious cause, especially in patients taking OCPs. Treatment is stopping the OCPs with close follow-up to confirm disease resolution.

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Hepatoportal sclerosis (HPS) is defined as sclerosis of portal areas in the absence of cirrhosis. There is little information about HPS in children in the literature. The aim of this study was to describe the clinical presentation, associated disorders, laboratory characteristics and outcome of children who were diagnosed as HPS. This study included 12 children diagnosed as HPS by the Pathology Department between 2005 and 2011. Data were collected from the gastroenterology clinic charts retrospectively, including demographics, presentation characteristics, laboratory data and recent status of patients. Twelve patients were enrolled (6 girls, 6 boys). The median age of patients was 13.5 yr. Median age at the time of biopsy was 11 yr. Four patients had splenomegaly, 3 had esophageal varices, one had hepatopulmonary syndrome and had been transplanted. Smooth muscle antibody was found positive in 4 patients, without autoimmune hepatitis findings in liver biopsy. One patient had celiac disease and another patient had positive celiac disease serology but pathology findings. Another patient had Turner\'s syndrome. Mean follow-up time was 39 months (3.3 yr) after biopsy. Hepatoportal sclerosis does not necessarily present with portal hypertension in children.