Department of Pediatric Surgery, Reproductive and Developmental Medicine, Faculty of Medical Sciences, Kyushu University, Fukuoka, Japan.
Department of Pediatric Surgery, Reproductive and Developmental Medicine, Faculty of Medical Sciences, Kyushu University, Fukuoka, Japan.
J Pediatr Surg. 2023 Jul;58(7):1246-1251. doi: 10.1016/j.jpedsurg.2023.01.050. Epub 2023 Feb 16.
The aim of this study was to clarify the appropriate management after birth for congenital biliary dilatation (CBD, choledochal cyst) patients with a prenatal diagnosis.
Thirteen patients with a prenatal diagnosis of CBD who underwent liver biopsy during excision surgery were divided into two groups and retrospectively analyzed: group A, with liver fibrosis above F1 and group B, without liver fibrosis.
Excision surgery was performed earlier in group A (F1-F2), at a median of 106 days old (p = 0.04). There were significant differences between the two groups in the presence symptoms and sludge, the cyst size, and the level of serum bilirubin and gamma glutamyl transpeptidase (GGT) before excision surgery (p < 0.05). Especially, in group A, prolonged serum GGT elevation and larger cysts were consistently observed from birth. The cut-off values of predictions for the presence of liver fibrosis in serum GGT and cyst size were 319 U/l and 45 mm. No significant differences were observed in the postoperative liver function or complications during the follow-up period.
In patients with prenatally diagnosed CBD, the postnatal serial changes of serum GGT values and cyst size, in addition to symptoms, could help to prevent progressive liver fibrosis.
Ⅲ.
Treatment Study.
本研究旨在阐明产前诊断为先天性胆管扩张症（CBD，胆总管囊肿）患者出生后的适当管理方法。
对 13 例产前诊断为 CBD 并在切除术中进行肝活检的患者进行分组并进行回顾性分析：A 组为肝纤维化 F1-F2 期，B 组为无肝纤维化。
A 组（F1-F2）行切除术的时间更早，中位数为 106 天（p=0.04）。两组患者在切除术前的症状和胆泥、囊肿大小、血清胆红素和γ-谷氨酰转肽酶（GGT）水平方面存在显著差异（p<0.05）。特别是在 A 组，从出生起就持续观察到血清 GGT 升高和囊肿增大。预测肝纤维化存在的血清 GGT 和囊肿大小的截断值分别为 319 U/l 和 45 mm。在随访期间，术后肝功能或并发症无显著差异。
对于产前诊断为 CBD 的患者，除了症状之外，血清 GGT 值和囊肿大小的产后连续变化有助于预防进行性肝纤维化。
Ⅲ级。
治疗研究。
