Non-Cirrhotic Portal Hypertension in Turner's Syndrome: A Case Report

Abstract

Introduction: Involvement of the gastrointestinal system is less common in Turner's syndrome. Hepatic derangements have been reported in individuals with Turner's syndrome due to nonalcoholic steatosis, steatohepatitis, and less commonly due to viral hepatitis and alcoholic hepatitis. Portal hypertension is typically associated with cirrhosis; however, in a minor fraction of individuals, it occurs in the absence of cirrhosis. Portal hypertension is rare in Turner's syndrome and is even more rarely observed in the absence of cirrhosis in individuals with Turner's syndrome.

Case presentation: Herein, we report a case of liver biopsy-proven non-cirrhotic portal hypertension due to portosinusoidal vascular disease.

Conclusion: High index of clinical suspicion can lead to early diagnosis and treatment of portal hypertension in individuals with Turner's syndrome, reducing the burden of complications of portal hypertension.

Keywords: Portal hypertension; Portosinusoidal vascular disease; Turner’s syndrome.

Fig. 1.

Computed tomography of the abdomen and pelvis. a Coronal view of computed tomography illustrates an enlarged spleen measuring 20.2 × 11.6 × 20.4 cm. The liver measures 6.8 cm craniocaudally with mild atrophy of the right lobe and mild hypertrophy of the left lobe. The gallbladder, bile ducts, and pancreas are unremarkable, and there is no evidence of portal vein thrombosis. b Axial view of computed tomography demonstrates a lack of surface nodularity of the liver.

Fig. 2.

Upper gastrointestinal endoscopy findings. Esophagogastroduodenoscopy demonstrates grade II esophageal varices at gastroesophageal junction.