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腹腔镜下脾血管结扎术治疗儿童遗传性球形红细胞增多症

Laparoscopic ligation of splenic vessels for the treatment of hereditary spherocytosis in children.

作者信息

Zhang Jin-Shan, Li Long

机构信息

Department of General Surgery, Capital Institute of Pediatrics, No. 2 Yabaolu Rd., Beijing, 100020, People's Republic of China.

出版信息

Pediatr Surg Int. 2020 Mar;36(3):365-371. doi: 10.1007/s00383-020-04623-1. Epub 2020 Jan 25.

Abstract

BACKGROUND

Total splenectomy is the most effective surgical treatment for hereditary spherocytosis (HS). Nevertheless, post-splenectomy sepsis and hypoimmunity can pose a great risk to children. Some alternative treatments have been proposed to avoid the post-splenectomy complications. In this study, we propose such a procedure (laparoscopic ligation of splenic vessels, L-LSV) for the treatment of HS in children and investigate its effectiveness and feasibility.

MATERIALS AND METHODS

A total of 17 children with HS who underwent the L-LSV at our hospital between May 2015 and Apr 2018 were enrolled in the current study. All patients were followed-up for 3-38 months (mean 19.8 months). The volume of spleen was preoperatively and postoperatively measured using the AW VolumeShare5. The size of functional spleen and the condition of splenic infarction were evaluated using ultrasound and computed tomography (CT) after surgery. The routine blood, biochemistry and coagulation tests were carried out after surgery.

RESULTS

The L-LSV was successfully performed in all patients. The average operative time was 115 min (range 60-180 min). No patients underwent blood transfusion during surgery, and the length of hospital stay varied 5-9 days after surgery (mean 7 days). Postoperatively, the red blood cells, platelet and hemoglobin were significantly increased (P < 0.05). The postoperative volume of functional spleen was significantly smaller than preoperative volume of spleen (307.393 ± 177.634 cm vs. 581.242 ± 270.260 cm, P = 0.000). The recent volume of functional spleen was significantly bigger than the postoperative 1 month volume of functional spleen in ten children who were followed-up for more than 1 year (P = 0.004). The index of splenic infarction (the proportion of the postoperative 1-month volume of splenic infarction in the preoperative volume of spleen) was 0.31-0.99 (mean 0.53). There were no patients undergoing the blood transfusion after surgery.

CONCLUSIONS

The L-LSV is an effective treatment for HS in children; however, future studies should re-evaluate the long-term prognosis.

摘要

背景

全脾切除术是治疗遗传性球形红细胞增多症(HS)最有效的手术方法。然而,脾切除术后败血症和免疫功能低下会给儿童带来很大风险。有人提出了一些替代治疗方法以避免脾切除术后并发症。在本研究中,我们提出了一种治疗儿童HS的手术方法(腹腔镜脾血管结扎术,L-LSV),并研究其有效性和可行性。

材料与方法

选取2015年5月至2018年4月在我院接受L-LSV手术的17例HS患儿纳入本研究。所有患者均随访3 - 38个月(平均19.8个月)。术前及术后使用AW VolumeShare5测量脾脏体积。术后通过超声和计算机断层扫描(CT)评估功能性脾脏大小及脾梗死情况。术后进行血常规、生化及凝血检查。

结果

所有患者L-LSV手术均成功完成。平均手术时间为115分钟(范围60 - 180分钟)。术中无患者输血,术后住院时间为5 - 9天(平均7天)。术后红细胞、血小板及血红蛋白显著升高(P < 0.05)。术后功能性脾脏体积明显小于术前脾脏体积(307.393±177.634 cm³对581.242±270.260 cm³,P = 0.000)。在随访超过1年的10例患儿中,近期功能性脾脏体积明显大于术后1个月的功能性脾脏体积(P = 0.004)。脾梗死指数(术后1个月脾梗死体积占术前脾脏体积的比例)为0.31 - 0.99(平均0.53)。术后无患者输血。

结论

L-LSV是治疗儿童HS的有效方法;然而,未来研究应重新评估其长期预后。

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