Autoimmune hepatitis (AIH) is relatively rare in children. Herein, our case demonstrates a unique presentation of AIH in a previously healthy 18-year-old female presenting with a mild cough, fatigue, and severe anemia (hemoglobin 2.9 g/dL). Initial evaluation revealed jaundice and scleral icterus, prompting transfer of care and further testing, which demonstrated severe microcytic anemia, pancytopenia, elevated liver enzymes, direct hyperbilirubinemia, and marked splenomegaly. Concern for autoimmune hemolytic anemia resulted in a delayed diagnosis. The combination of triple antibody positivity (anti-nuclear antibodies, anti-actin, and anti-liver-kidney microsomal-1) and liver histology findings confirmed the diagnosis of AIH. Intravenous methylprednisolone was initiated to induce remission. Due to pancytopenia and persistently elevated international normalized ratio, tacrolimus was chosen as the maintenance immunosuppression instead of azathioprine. This case highlights several significant considerations for clinicians, including the importance of a timely clinicopathologic diagnosis, the severe anemia presentation secondary to hypersplenism, and the rare finding of triple autoantibody-positive AIH.

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BACKGROUND: Extrahepatic Portal Vein Obstruction is the most common cause of portal hypertension in children. However, it has a very low prevalence. Esophageal varices due to portal hypertension in children can lead to recurrent episodes of upper gastrointestinal bleeding, which can have a sinister outcome if timely diagnosis and treatment are not initiated.
METHODS: A 7-year-old male child presents with recurrent episodes of upper gastrointestinal bleeding for 3 years. Clinical examination reveals pallor and splenomegaly. Laboratory investigations revealed signs of hypersplenism with anemia, leucopenia and thrombocytopenia, and Doppler ultrasonography and CT abdomen and pelvis revealed splenic vein thrombosis with splenomegaly and cavernous transformation of the portal vein. The patient was managed operatively with splenectomy with splenorenal shunting and devascularization of esophagogastric varices.
CONCLUSIONS: Extrahepatic Portal Vein obstruction is the most common cause of noncirrhotic portal hypertension in children. Its occurrence in the pediatric population is very rare. Portal hypertension can lead to variceal bleeding and splenomegaly, which can have a significant impact on a child\'s long-term health. Because of its insidious nature, a meticulous workup is required for its diagnosis, and treatment in the pediatric population is difficult, and appropriate guidelines for its management specifically targeting the pediatric population are lacking.
CONCLUSIONS: Extrahepatic Portal Vein obstruction is rare in children with a difficult diagnosis and management. Despite these hindrances, timely intervention can lift a significant burden of its detrimental outcome off the young children and drastically uplift the quality of life of these patients.

Portomesenteric decompression is often necessary to treat patients with refractory symptoms of portal hypertension. When transjugular or direct intrahepatic portosystemic shunt creation is not feasible or is inadequate, surgical portosystemic shunt creation is considered, which carries significant morbidity and mortality in these high-risk patients. Surgery is further complicated in patients with portomesenteric thrombosis who require concurrent thrombectomy and long-term anticoagulation. In this article, we outline the technique for performing advanced endovascular alternatives to intrahepatic portosystemic shunt creation including mesocaval and splenorenal shunting. We will also discuss some of the clinical considerations for treating these patients with symptomatic portal hypertension and portomesenteric thrombosis.

A 74-year-old patient presented with hematochezia and a history of liver cirrhosis with repeated bleeding from esophageal and rectal varices. Endoscopic examination revealed multiple rectal varices with positive red color signs. Ascites, severe portosystemic thrombosis and a splenorenal shunt were diagnosed on a contrast-enhanced dynamic computed tomography examination. From a transjugular approach, we circumvented thrombosed regions by maneuvering double balloon catheters through the shunt and dilated left colic marginal vein. We managed to successfully obliterate the varices.

The management of portal hypertension complicated by iterative gastro-intestinal bleeding remains challenging, especially in a low-income environment. Interventional radiology and endoscopic treatments are not always accessible, and a definitive surgical option may prove to be lifesaving. We report a new technique of surgical portosystemic shunt that can be performed in all contexts. We describe the surgical technique of a H-shaped splenorenal shunt using autologous rolled up peritoneum as a vascular graft.

This case illustrates adult-onset noncirrhotic hyperammonemic encephalopathy, which is most likely caused by splenorenal shunts and is a rare but potentially fatal cause of altered mentation in the critical care setting. Splenorenal shunts should be considered as a differential in cases of hyperammonemic encephalopathy without liver cirrhosis.

Acquired hepatocerebral degeneration (AHD) is a neurologic syndrome caused by liver dysfunction and long-standing portosystemic shunting. The pathogenesis of the condition is predominantly considered to be related to the deposition of manganese in parts of the brain due to shunting. We report a case of a 25-year-old male who underwent splenectomy and splenorenal shunt for recurrent upper GI bleeding (UGIB) due to esophageal varices caused by non-cirrhotic portal hypertension (NCPH). He presented with bradykinesia, hypophonia, gait instability, and rigidity of the lower extremities 18 months after the procedure was done.

Cavernous transformation of the portal vein (CTPV) is a sequela of extrahepatic and/or intrahepatic portal vein obstruction caused by a combination of local and risk factors. It was ever taken as a relatively rare disease due to its scant literature, which was mainly based on clinical series and case reports. CTPV often manifests as gastroesophageal variceal bleeding, splenomegaly, and portal biliopathy after the long-term insidious presentation. It is unable for CTPV to be recanalized with anticoagulation because it is a complete obstruction of the mesentericoportal axis. Endoscopic therapy is mainly used for temporary hemostasis in acute variceal bleeding. Meso-Rex shunting characterized by portal-flow-preserving shunts has been widely performed in children with CTPV. The multitude of complications associated with CTPV in adults can be effectively addressed by various interventional vascular therapies. With the ubiquity of radiological examinations, optimal treatment for patients with CTPV becomes important. Multivisceral transplantation, such as liver-small intestinal transplantation, may be lifesaving and should be considered for patients with diffuse mesenteric venous thrombosis.

Surgical shunts are commonly used to manage complications resulting from extrahepatic portal vein thrombosis (EHPVT) in children. We describe a single-center experience utilizing a functional Side-to-Side Splenorenal Shunt (fSRS), created using either an enlarged inferior mesenteric vein (IMV) or left adrenal vein (LAV).
Pediatric patients with isolated EHPVT who were poor candidates for a Rex shunt and who underwent a fSRS procedure at our institution between 2003 and 2020 were reviewed. The pre/post shunt portosystemic gradient change, rates of early and late complications, postoperative shunt patency, and mortality were evaluated.
Twelve EHPVT patients (mean age of 6.1 years) underwent a fSRS procedure. The mean portosystemic gradient change for the cohort was -11.7 mmHg (±4.9). There were no cases of recurrent variceal bleeding or episodes of shunt thrombosis reported after fSRS procedures.
Surgical shunts continue to be an important adjunct in the treatment of complications related to EHPVT. The functional Side-to-Side Splenorenal Shunt is a safe alternative that is easy to perform, involves minimal dissection and requires only a single anastomosis.