Subhasis Roy Choudhury, Rajiv Chadha, Kumar Sharma Akshay, Kumar Anand Vinod, Kumar Patwari Ashok
Department of Pediatric Surgery, Lady Hardinge Medical College, New Delhi 110001, India.
Surg Today. 2007;37(1):19-23. doi: 10.1007/s00595-006-3333-3. Epub 2007 Jan 1.
Massive splenomegaly with severe hypersplenism can occur as a late complication of portal hypertension (PH) caused by extrahepatic portal venous obstruction (EHPVO) in children. Severe hypersplenism is often refractory to treatment with endoscopic sclerotherapy (EST) and shunt surgery. We report our experience of managing this disorder surgically.
We performed splenectomy and esophagogastric devascularization via laparotomy in 14 children with an average age of 9.7 years. Upper gastrointestinal endoscopy had shown esophageal varices of varying grade, and EST had been done for patients with a history of bleeding. The indications for surgery were pain and discomfort caused by a large spleen greater than 15 cm below the costal margin, and intractable symptomatic hypersplenism with a total leukocyte count <2500/mm3 and a platelet count <50,000/mm3, or both.
Postoperative recovery was uneventful and the leukocyte and platelet counts reverted to normal. After follow-up for 1-5 years, all 14 children were asymptomatic, with improved growth and nutrition and no reported episodes of gastrointestinal bleeding, sepsis, or encephalopathy.
Splenectomy with devascularization is effective for children with massive splenomegaly and severe hypersplenism secondary to EHPVO.
巨大脾肿大伴严重脾功能亢进可作为儿童肝外门静脉阻塞（EHPVO）所致门静脉高压（PH）的晚期并发症出现。严重脾功能亢进通常难以通过内镜硬化治疗（EST）和分流手术治愈。我们报告我们通过手术治疗这种疾病的经验。
我们对14名平均年龄为9.7岁的儿童进行了剖腹脾切除术和食管胃去血管化手术。上消化道内镜检查显示有不同程度的食管静脉曲张，有出血史的患者已接受EST治疗。手术指征为脾脏肿大超过肋缘下15 cm引起的疼痛和不适，以及难治性症状性脾功能亢进，白细胞总数<2500/mm³且血小板计数<50,000/mm³，或两者兼有。
术后恢复顺利，白细胞和血小板计数恢复正常。随访1至5年后，所有14名儿童均无症状，生长和营养状况改善，未报告有胃肠道出血、败血症或脑病发作。
去血管化脾切除术对EHPVO继发的巨大脾肿大和严重脾功能亢进的儿童有效。
