METHODS: Here, we retrospectively describe a 10-month-old infant who was admitted to our hospital due to stubborn jaundice. The patient responded poorly to routine medical treatment and his clinical manifestations and laboratory tests lacked specificity, so we turned to repeated ultrasound scans and other imaging examinations. As both hepatosplenic ultrasonography and diffusion-weighted magnetic resonance imaging demonstrated a space-occupying lesion, an exploratory laparotomy was performed. The final diagnosis made over two mo after the disease onset was infant biliary cirrhosis caused by a biliary IMT, which partially infiltrated into the liver. This infant is the youngest case of biliary IMTs that has been reported till now. The patient underwent an incomplete resection of the mass and Kasai Portoenterostomy. However, because of cirrhosis, he also received a paternal liver transplant. Since some IMTs show malignant properties, we proceeded with a three-year of follow-up; however, no recurrence or metastasis has been noted.
CONCLUSIONS: Neoplastic disease such as IMTs should be considered when routine medical treatment of obstructive jaundice is not successful. Observation of dynamic imaging changes is helpful for diagnosis. Periodic follow-up is necessary for IMTs.
方法:这里,我们回顾性地描述了一名10个月大的婴儿,他因顽固性黄疸入院.患者对常规药物治疗反应不佳,临床表现和实验室检查缺乏特异性,所以我们转向重复的超声扫描和其他成像检查。由于肝脾超声和磁共振弥散加权成像均显示为占位性病变,进行了剖腹探查术.疾病发作后两个月的最终诊断是由胆道IMT引起的婴儿胆汁性肝硬化,部分渗入肝脏.该婴儿是迄今为止报道的最年轻的胆道IMT病例。患者接受了肿块的不完全切除和Kasai门肠造口术。然而,因为肝硬化,他还接受了父亲肝脏移植。由于一些IMT显示出恶性特性,我们进行了为期三年的随访;然而,没有发现复发或转移。
结论:当常规药物治疗梗阻性黄疸不成功时,应考虑肿瘤疾病如IMT。观察动态影像学变化有助于诊断。定期随访对于IMT是必要的。