Banani S A
Department of Pediatric Surgery, Shiraz University of Medical Sciences, Iran.
J Pediatr Surg. 1998 Mar;33(3):449-53. doi: 10.1016/s0022-3468(98)90086-0.
Nine patients with thalassemia major (8 boys, 1 girl; age 3 to 8 years) who had signs of hypersplenism and required more than one blood transfusion per month (1 to 2 weeks) underwent partial dearterialization of the spleen (PDAS) from April through December 1992. All were on a low transfusion regimen (Hb < 10 g%). Hematologic profile, IgM and splenic dimensions (SD) by sonography were determined preoperatively and postoperatively during follow-up periods. Isotopic scan was also performed a few months and 2 to 4 years after operation. The patients did not receive pneumovax or long-term prophylactic antibiotics. The procedure consists of division of the splenic arterial branches, except the one that supplies the superior pole, along with preservation of all the veins.
Seven patients with SD less than 11 cm (6.9 to 10.8 cm; four with less than 9.5 cm) had uneventful recovery. Two others with SD greater than 13 cm (13.2 to 13.5 cm) required total splenectomy a few days after PDAS because of splenic enlargement associated with high fever. One patient was lost to follow-up after 18 months. The remaining six patients underwent follow-up for 4 to 4.5 years. The hematologic profile (Hb, RBC, platelet counts) showed significant improvement during follow-up period. The blood transfusion requirement in all patients was once every 25 to 32 days except one in which transfusion was increased to every 2 to 3 weeks during the fourth postoperative year. The ultimate magnitude of the hematologic response compared with 15 matched splenectomized patients studied retrospectively in a 4-year period was almost the same in the majority after the first postsplenectomy year. There was no significant change in SD by sonography in four patients compared with preoperative state, but a 30% decrease was seen in the other two during the follow-up period. Isotope scan showed a functional residual spleen in all six, with no remarkable difference in size 4 years after operation, except in the one who relapsed. The IgM level did not fall significantly after operation, nor did signs of infection develop in any patient.
PDAS is a safe and effective mode of therapy in thalassemia major, provided the disease is not advanced and SD does not exceed 11 cm in its greatest diameter.
1992年4月至12月，对9例重型地中海贫血患者（8例男孩，1例女孩；年龄3至8岁）进行了脾部分去动脉化（PDAS），这些患者有脾功能亢进的体征，且每月（1至2周）需要输血不止一次。所有患者均采用低输血方案（血红蛋白<10 g%）。在术前及随访期间术后测定血液学指标、IgM及脾脏超声尺寸（SD）。术后数月及2至4年还进行了同位素扫描。患者未接种肺炎疫苗或长期预防性使用抗生素。该手术包括除供应脾上极的动脉分支外，切断脾动脉分支，同时保留所有静脉。
7例SD小于11 cm（6.9至10.8 cm；4例小于9.5 cm）的患者恢复顺利。另外2例SD大于13 cm（13.2至13.5 cm）的患者因脾肿大伴高热在PDAS术后数天需要行全脾切除术。1例患者在18个月后失访。其余6例患者随访4至4.5年。血液学指标（血红蛋白（Hb）、红细胞（RBC）、血小板计数）在随访期间有显著改善。除1例患者在术后第4年输血频率增加至每2至3周一次外，所有患者的输血需求为每25至32天一次。与回顾性研究的15例匹配的脾切除患者在4年期间的最终血液学反应程度相比，大多数患者在脾切除术后第1年几乎相同。4例患者的超声SD与术前状态相比无显著变化，但另外2例在随访期间SD下降了30%。同位素扫描显示所有6例均有功能性残留脾，术后4年除复发的1例患者外，大小无明显差异。术后IgM水平无显著下降，且无患者出现感染迹象。
对于重型地中海贫血，只要病情未进展且SD最大直径不超过11 cm，PDAS是一种安全有效的治疗方式。
