El-Shabrawi Mortada H F, Eltagy Gamal, Qinawy Mohamed, Oshi Mohammed Am, Algethami Amjad, Alhujayri Nawaf A, Alharthi Shaker S, Alelyani Abdullah M, Kamal Naglaa M
Professor of Pediatrics, Pediatric Hepatology Unit, Kasr Al Ainy Faculty of Medicine, Cairo University, Cairo, Egypt.
Professor of Pediatric Surgery, Kasr Al Ainy Faculty of Medicine, Cairo University, Cairo, Egypt.
J Int Med Res. 2025 Feb;53(2):3000605241311115. doi: 10.1177/03000605241311115.
Extrahepatic biliary atresia (EHBA) is a leading cause of neonatal cholestasis, often resulting in end-stage cirrhosis and portal hypertension without early diagnosis and treatment. This report highlights the importance of timely intervention, describing a 6-day-old male newborn diagnosed with EHBA who underwent successful Kasai portoenterostomy at 9 days of age. While the procedure is typically performed within the first 60 days of life, this exceptionally early intervention led to significantly improved outcomes. Postoperative recovery was marked by bilirubin normalization within 2 months and steady improvement in liver function tests, demonstrating the advantages of early surgery. The procedure involved creating a Roux-en-Y hepatic portojejunostomy to restore bile flow, preventing progression to biliary cirrhosis. Early intervention achieved effective bile drainage and substantial clinical improvement. At the 1-year follow-up, the infant displayed normal growth and liver function. This case supports the hypothesis that performing Kasai portoenterostomy earlier than current guidelines recommend may lead to better outcomes. It underscores the need for vigilant neonatal care to recognize early signs of cholestasis and enable prompt surgical intervention. Early diagnosis and intervention can preserve liver function, potentially delaying or preventing the need for liver transplantation.
肝外胆管闭锁(EHBA)是新生儿胆汁淤积的主要原因,若不及早诊断和治疗,常导致终末期肝硬化和门静脉高压。本报告强调了及时干预的重要性,描述了一名6日龄男性新生儿被诊断为EHBA,并在9日龄时成功接受了Kasai肝门空肠吻合术。虽然该手术通常在出生后的前60天内进行,但这种异常早期的干预导致了显著改善的结果。术后恢复的标志是2个月内胆红素正常化,肝功能检查稳步改善,证明了早期手术的优势。该手术包括创建一个Roux-en-Y肝门空肠吻合术以恢复胆汁流动,防止发展为胆汁性肝硬化。早期干预实现了有效的胆汁引流和显著的临床改善。在1年的随访中,该婴儿生长和肝功能正常。本病例支持这样的假设,即比当前指南建议的时间更早进行Kasai肝门空肠吻合术可能会带来更好的结果。它强调了需要警惕的新生儿护理,以识别胆汁淤积的早期迹象并及时进行手术干预。早期诊断和干预可以保留肝功能,有可能延迟或避免肝移植的需要。