A rare case of neonatal-onset hepatic sinusoidal obstruction syndrome
WANG Zi-Ming, DENG Ying-Ping, SHI Yuan-Mei, WANG Lai-Shuan, SUN Song, XIE Xin-Bao, ZHAO Lu, WANG Hong-Sheng, LI Zhi-Hua
Department of Neonatology, Children's Hospital of Fudan University/National Children's Medical Center/National Health Commission Key Laboratory of Neonatal Diseases, Shanghai 201102,China
Abstract A male infant, aged 1 month and 14 days, was admitted to the hospital due to abdominal distension lasting for 2 weeks and worsening for 3 days. The infant had a history of omphalitis. Physical examination revealed severe abdominal distension, prominent abdominal wall veins, hepatosplenomegaly, and massive ascites. There was a slight elevation in liver transaminase levels. Liver ultrasound and CT scans demonstrated the absence of visualization of the intrahepatic segment of the portal vein and the left, middle, and right veins of the liver, indicating occlusion of these vessels, along with surrounding fibrous hyperplasia. The clinical diagnosis was hepatic sinusoidal obstruction syndrome resulting from omphalitis. A large amount of bloody ascites developed after 12 days of hospitalization, resulting in hypovolemic shock and respiratory failure. The infant passed away following the family's decision to discontinue treatment. This article focuses on the diagnostic approach and multidisciplinary management of neonatal-onset hepatic sinusoidal obstruction syndrome, as well as provides insights into the differential diagnosis of hepatomegaly and ascites.
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