Department of Pediatric Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
Department of Pediatric Surgery, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
J Hepatobiliary Pancreat Sci. 2020 Mar;27(3):141-148. doi: 10.1002/jhbp.692. Epub 2019 Nov 26.
This study was performed to assess the effect of portosystemic shunt surgery (PSS) on portal cavernoma cholangiopathy (PCC) in children with extrahepatic portal vein obstruction (EHPVO).
Children with EHPVO and PCC (unfit for Meso-Rex shunt) underwent magnetic resonance cholangiogram (MRC) and magnetic resonance portovenogram (MRPV) before non-selective PSS. PCC was graded by modified Llop classification. Those with patent shunt were re-evaluated at least 6 months after surgery with MRC, MRPV and compared with pre-shunt images.
Twenty-five children underwent PSS (central end to side splenorenal shunt with splenectomy [n = 24], mesocaval shunt [n = 1]). Pre-surgery MRC showed PCC grade I in 11, grade II in 1 and grade III in 13. MRPV showed superior mesenteric vein (SMV) block in 20. Re-assessment for PCC 18 (6 to 54) months after surgery showed grade I in 6 and grade III in 19. Thus, PCC was progressive in 6 and static in 19. Density of peribiliary collaterals decreased in 5 (SMV patent, static PCC), increased in 3 and remained unchanged in 17. Splenomegaly-related problems, gastroesophageal varices and other intra-abdominal (esophageal, perisplenic and perigastric) collaterals ameliorated in all.
Non-selective PSS decompresses esophago-gastro-splenic venous circuit effectively but fails to ameliorate cholangiopathy and peribiliary collaterals. Persistence of cholangiopathy is attributable to SMV block.
本研究旨在评估门体分流术（PSS）对肝外门静脉阻塞（EHPVO）儿童门静脉海绵样变胆管病（PCC）的影响。
EHPVO 伴 PCC（不适合 Meso-Rex 分流术）患儿行磁共振胆胰管造影（MRC）和磁共振门静脉造影（MRPV）检查，并行非选择性 PSS。改良 Llop 分级法对 PCC 进行分级。有分流通道的患儿在分流术后至少 6 个月进行 MRC、MRPV 复查，并与术前图像比较。
25 例行 PSS（脾切除脾肾分流术[24 例]，肠系膜腔静脉分流术[1 例]）。术前 MRC 显示 PCC Ⅰ级 11 例，Ⅱ级 1 例，Ⅲ级 13 例。MRPV 显示肠系膜上静脉（SMV）阻塞 20 例。术后 6 至 54 个月行 PCC 复查 18 例，Ⅰ级 6 例，Ⅲ级 19 例。因此，PCC 进展 6 例，稳定 19 例。5 例（SMV 通畅，PCC 稳定）胆管周围侧支循环密度降低，3 例增加，17 例无变化。所有患者的脾肿大相关问题、胃食管静脉曲张及其他腹腔内（食管、脾肾旁和胃周）侧支循环均得到改善。
非选择性 PSS 可有效减压食管胃脾静脉循环，但不能改善胆管病和胆管周围侧支循环。胆管病持续存在是由于 SMV 阻塞。
