El-Shabrawi Mortada H F, Abdel Sattar Ayman Hussein, Hassanin Fetouh, Sheba Maha Fathy, Elhennawy Ahmed, Kamal Naglaa M, Oshi Mohammed A M, Algarni Ali, Marzouk Seham Anwar Emam
Professor of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt.
Professor of Pediatric Surgery, Faculty of Medicine, Cairo University, Cairo, Egypt.
Medicine (Baltimore). 2025 Jan 31;104(5):e41238. doi: 10.1097/MD.0000000000041238.
Umbilical vein catheterization (UVC) is a common procedure in neonatal intensive care units (NICU) but carries risks of severe complications such as portal vein thrombosis (PVT). Extrahepatic biliary atresia (EHBA), a leading cause of neonatal cholestasis, often progresses to end-stage liver disease. This case report discusses the rare coexistence of PVT and EHBA in a 3-month-old infant, highlighting the critical need for timely diagnosis and intervention.
A 3-month-old female presented with jaundice, dark-colored urine, and clay-colored stools. She had a history of NICU admission for neonatal sepsis during which a UVC was inserted.
Physical examination revealed jaundice and hepatosplenomegaly. Abdominal ultrasonography identified hepatosplenomegaly, mild ascites, and portal cavernoma. A liver biopsy confirmed a diagnosis of EHBA.
The patient underwent Kasai portoenterostomy. Postoperatively, she developed complications including ascites, systemic hypertension, and hyperammonemia. Initial improvements were observed with decreased bilirubin levels.
Despite initial stabilization, the patient's condition deteriorated, and she succumbed on day 15 postoperation.
This case underscores the significant risks of PVT associated with UVC and the importance of monitoring NICU graduates for early detection of complications. The early onset of portal hypertension and esophageal varices in this case challenges existing beliefs about EHBA's clinical progression. Greater awareness and routine follow-up imaging are essential to improve outcomes in similar scenarios.
脐静脉插管（UVC）是新生儿重症监护病房（NICU）的常见操作，但存在诸如门静脉血栓形成（PVT）等严重并发症的风险。肝外胆管闭锁（EHBA）是新生儿胆汁淤积的主要原因，常进展为终末期肝病。本病例报告讨论了一名3个月大婴儿中PVT和EHBA罕见的共存情况，强调了及时诊断和干预的迫切需求。
一名3个月大的女性出现黄疸、深色尿液和陶土样大便。她有因新生儿败血症入住NICU的病史，期间插入了UVC。
体格检查发现黄疸和肝脾肿大。腹部超声检查发现肝脾肿大、轻度腹水和门静脉海绵样变性。肝活检确诊为EHBA。
患者接受了Kasai肝门空肠吻合术。术后，她出现了包括腹水、全身性高血压和高氨血症在内的并发症。胆红素水平下降，观察到初步改善。
尽管最初病情稳定，但患者的病情恶化，术后第15天死亡。
本病例强调了与UVC相关的PVT的重大风险以及监测NICU出院患儿以早期发现并发症的重要性。该病例中门静脉高压和食管静脉曲张的早期出现挑战了关于EHBA临床进展的现有观念。提高认识和进行常规随访影像学检查对于改善类似情况下的结局至关重要。
