Fumino Shigehisa, Higuchi Koji, Aoi Shigeyoshi, Furukawa Taizo, Kimura Osamu, Tajiri Tatsuro
Department of Pediatric Surgery, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, 465 Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, 602-8566, Japan,
Pediatr Surg Int. 2013 Nov;29(11):1097-102. doi: 10.1007/s00383-013-3368-7.
Although most patients with choledochal cyst (CC) have a favorable prognosis with prompt treatment, some of them are complicated with liver fibrosis, resulting in prolonged liver dysfunction even after definitive surgery. The aim of this study was to distinguish the high-risk group of liver fibrosis in patients with CC.
Fifteen patients who underwent liver biopsy during surgery for CC from 1981 to 2012 were enrolled in this study. Liver histology with H&E staining was graded according to Ohkuma's classification, and the correlation with the clinical characteristics was retrospectively assessed.
Their median age at biopsy was 13.4 months (range 1-42 months), and there were 7 in Ia, 2 in Ic, and 6 in IVa of the Todani classification. There were no significant differences in serum liver function test among those cyst types. The histological grades of liver fibrosis were as follows: grade 0 (no fibrosis) in 7 patients, grade 1 (mild) in 2, grade 2 (moderate) in 3, grade 3 (severe) in 2, grade 4 (cirrhosis) in 1. All 3 patients with grade 3 and 4 had CC with IVa and were under 18-month old. A 3-month-old girl with cirrhosis presented with severe jaundice resulting in living-donor liver transplantation despite bile drainage. A 16-month-old girl with grade 3 suffered from prolonged liver dysfunction and intractable ascites after surgery.
The progression of liver fibrosis is likely to be correlated with IVa, and the postoperative course might be unsatisfactory in advanced cases. The prompt surgical intervention is recommended especially for neonatal and infantile cases with type IVa cyst because irreversible liver cirrhosis could occur as early as in the infantile period.
尽管大多数胆总管囊肿（CC）患者经及时治疗后预后良好，但部分患者会并发肝纤维化，即使在确定性手术后仍会导致肝功能长期异常。本研究旨在鉴别CC患者中肝纤维化的高危人群。
选取1981年至2012年间因CC接受手术并在术中进行肝活检的15例患者。肝组织学采用苏木精-伊红（H&E）染色，根据大隈分类法进行分级，并回顾性评估其与临床特征的相关性。
活检时的中位年龄为13.4个月（范围1 - 42个月），按照托达尼分类法，Ia型7例，Ic型2例，IVa型6例。这些囊肿类型之间的血清肝功能检查无显著差异。肝纤维化的组织学分级如下：0级（无纤维化）7例，1级（轻度）2例，2级（中度）3例，3级（重度）2例，4级（肝硬化）1例。所有3例3级和4级患者均为IVa型CC且年龄小于18个月。一名3个月大的肝硬化女童尽管进行了胆汁引流，但仍因严重黄疸接受了活体肝移植。一名16个月大的3级女童术后出现肝功能长期异常和顽固性腹水。
肝纤维化的进展可能与IVa型相关，晚期病例术后病程可能不理想。尤其对于IVa型囊肿的新生儿和婴儿病例，建议尽早进行手术干预，因为在婴儿期就可能发生不可逆的肝硬化。
