儿童Kasabuch—Merritt综合征手术治疗探讨

目的评价儿童Kasabuch—Merritt综合征（Kasabuch—Merritt svndrome，K—M综合征）的手术治疗效果，探讨K—M综合征患儿的治疗方法。方法回顾性分析本院2001年7月至2008年7月经手术治疗的14例K—M综合征患儿的临床资料。患儿血小板最低值为3～29×10^9／L。均先采取保守治疗，疗效欠佳，而转手术切除瘤体。结果瘤体完整切除8例，部分切除6例。手术后1周，9例血小板计数〉100×10^9／L，3例为70～100×10^9／L，1例为58×10^9／L。1例术后因多器官功能衰竭死亡。对13例存活患儿随访6个月至5年，患儿血小板均恢复并保持正常。5例肿块部分残留者肿块逐渐消退。结论对瘤体巨大、药物治疗难以维持血小板正常的K—M综合征患儿，如病变部位及全身状况允许，应尽早手术治疗，完整或部分切除瘤体，可达到理想的治疗效果。

Surgical management of Kasabuch-Merritt syndrome in children (report of 14 cases)

Objective To discuss surgical options of Kasabuch-Merritt syndrome （KMS）, and evaluate the effect of surgical treatment in severe refractory KMS. Methods A retrospective review of 14 cases of intractable KMS managed by surgical treatment from July 2001 to July 2008 in our hospital was conducted. All patients had undergone unsuccessful conservative treatment before undergoing surgical resection of tumor. Results Complete resection of tmnor was carried out in 8 patients, and partial resection in 6. 7 days after the operation, the platelet counts were higher than 100 ×10^9/ L in 9 cases, between 70×10^9/ L and 100×10^9/L in 3; and 58 ×10^9 / L in 1. 1 patient died due to multiple organ failure. 13 survivors were followed up at period of 6 months to 5 years and the platelet count recovered and remained normal in all patients, 5 eases of residual tumor reeeeded gradually. Conclusions For severe intractable KMS case with enormous tumor, it is difficult to maintain normal platelet count by conservative treatment. And with permission by diseased region of tumor and condition of patient, tumor resection should be clone as early as possible. All patients can achieve desired therapeutic effect by complete or partial resection.