Lal Richa, Behari Anu, Sarma Moinak S, Yachha Surender K, Mandelia Ankur, Srivastava Anshu, Poddar Ujjal
Department of Pediatric Surgical Superspecialties, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, 226014, India.
Department of Surgical Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, 226014, India.
J Clin Exp Hepatol. 2023 Nov-Dec;13(6):997-1007. doi: 10.1016/j.jceh.2023.06.001. Epub 2023 Jun 9.
This exclusively surgical series on pediatric extrahepatic portal venous obstruction (EHPVO) defines surgical indications beyond endoscopic eradication of esophageal varices (EEEV), the selection of an appropriate surgical procedure, and the long-term post-surgical outcome.
EHPVO management protocol at the reporting institute has been endotherapy until esophageal variceal eradication and surgery for select adverse sequelae manifesting after EEEV.
One hundred and thirty-nine EHPVO cases underwent surgery for the following indications in combination: i) massive splenomegaly with severe hypersplenism ( = 132, 95%); ii) growth retardation (GR, = 95, 68%); iii) isolated gastric (IGV) and ectopic varices ( = 49, 35%); iv) Portal cavernoma cholangiopathy (PCC) ( = 07, 5%). A portosystemic shunt (PSS) was performed in 119 (86%) cases. Types of PSS performed were as follows: central end-to-side splenorenal shunt with splenectomy ( = 104); side-to-side splenorenal shunt ( = 4); mesocaval shunt ( = 1); inferior mesenteric vein (IMV) to left renal vein shunt ( = 2); IMV to inferior vena cava shunt ( = 3); H-graft interposition splenorenal shunt ( = 1); spleno-adrenal shunt ( = 3); makeshift shunt ( = 1). Esophagogastric devascularization ( = 20, 14%) was opted for only for non-shuntable anatomy. At a median follow-up (FU) of 41 (range: 6-228) months, PSS block was detected in 13 (11%) cases, with recurrent variceal bleeding in 4 cases. PCC-related cholestasis regressed in 5 of 7 cases. Issues of splenomegaly were resolved, and growth z-scores improved significantly.
Endotherapy for secondary prophylaxis until EEEV has resulted in a shift in surgical indications for EHPVO. Beyond EEEV, surgery was indicated predominantly for non-variceal sequelae, namely massive splenomegaly with severe hypersplenism, GR, and PCC. Varices warranted surgery infrequently but more often from sites less amenable to endotherapy, i.e., IGV and ectopic varices. The selection of PSS was tailored to anatomy and surgical indications. On long-term FU post surgery, PSS block was detected in 13% of patients. PCC-related cholestasis regressed in 71%, and issues of splenomegaly resolved with significantly improved growth Z scores.
本项关于小儿肝外门静脉阻塞（EHPVO）的纯手术系列研究明确了内镜下根除食管静脉曲张（EEEV）之外的手术指征、合适手术方式的选择以及术后长期疗效。
报告机构的EHPVO治疗方案是先进行内镜治疗直至根除食管静脉曲张，对于EEEV后出现的特定不良后遗症则进行手术治疗。
139例EHPVO患者因以下指征联合接受手术：i）巨脾伴严重脾功能亢进（n = 132，95%）；ii）生长发育迟缓（GR，n = 95，68%）；iii）孤立性胃静脉曲张（IGV）和异位静脉曲张（n = 49，35%）；iv）门静脉海绵样变胆管病（PCC）（n = 7，5%）。119例（86%）患者进行了门体分流术（PSS）。实施的PSS类型如下：中心端侧脾肾分流术联合脾切除术（n = 104）；侧侧脾肾分流术（n = 4）；肠系膜上腔静脉分流术（n = 1）；肠系膜下静脉（IMV）至左肾静脉分流术（n = 2）；IMV至下腔静脉分流术（n = 3）；H型移植脾肾分流术（n = 1）；脾肾上腺分流术（n = 3）；临时分流术（n = 1）。仅对无法进行分流术的解剖结构选择了食管胃去血管化术（n = 20，14%）。中位随访（FU）41个月（范围：6 - 228个月）时，13例（11%）患者检测到PSS阻塞，4例出现复发性静脉曲张出血。7例PCC相关胆汁淤积患者中的5例病情缓解。脾肿大问题得到解决，生长Z评分显著改善。
在达到EEEV之前进行二级预防的内镜治疗导致了EHPVO手术指征的转变。除了EEEV之外，手术主要针对非静脉曲张后遗症，即巨脾伴严重脾功能亢进、GR和PCC。静脉曲张很少需要手术，但更多是针对内镜治疗效果欠佳的部位，即IGV和异位静脉曲张。PSS的选择是根据解剖结构和手术指征量身定制的。术后长期随访中，13%的患者检测到PSS阻塞。71%的PCC相关胆汁淤积病情缓解，脾肿大问题得到解决，生长Z评分显著改善。
