Styles L A, Vichinsky E P
Department of Hematology/Oncology, Children's Hospital Oakland, California, USA.
Am J Hematol. 1996 Jun;52(2):103-7. doi: 10.1002/(SICI)1096-8652(199606)52:2<103::AID-AJH6>3.0.CO;2-Y.
Sickle-cell disease (SCD) is the most common cause of avascular necrosis (AVN) of the hip in childhood. It results in significant physical impairment and chronic pain, and often progresses to require hip replacement. Conservative therapy is ineffective. We evaluated whether core decompression can arrest progression of AVN. We performed 13 coring procedures in 10 patients with SCD and AVN. Patients ranged from age 9-21 years at diagnosis (mean, median age, 15 years); five hips were stage I, six hips were stage II, and two hips were stage III. Mean follow-up on these patients was 3.7 years. Efficacy of the procedure was evaluated by clinical improvement in pain, radiographic progression, and need for further surgery. All 5 stage I patients had substantial improvement in pain, and only one showed X-ray progression. Five of the 6 (83%) stage II patients had improvement in pain, and 2 patients progressed on X-ray. Both stage III patients progressed on X-ray, but one was clinically improved. None of the 10 patients has required further surgery. Our results demonstrate that in early AVN, core decompression was beneficial for almost all patients, even with progression on X-ray. Core decompression should be considered in the management of SCD patients with early AVN.
镰状细胞病（SCD）是儿童期髋关节缺血性坏死（AVN）最常见的病因。它会导致严重的身体功能障碍和慢性疼痛，并且常常进展到需要进行髋关节置换。保守治疗无效。我们评估了髓芯减压术能否阻止AVN的进展。我们对10例患有SCD和AVN的患者进行了13次髓芯减压手术。患者诊断时年龄在9至21岁之间（平均年龄、中位数年龄为15岁）；5个髋关节为I期，6个髋关节为II期，2个髋关节为III期。对这些患者的平均随访时间为3.7年。通过疼痛的临床改善、影像学进展以及是否需要进一步手术来评估该手术的疗效。所有5例I期患者的疼痛均有显著改善，只有1例出现X线进展。6例II期患者中有5例（83%）疼痛得到改善，2例患者出现X线进展。2例III期患者均出现X线进展，但其中1例临床症状有所改善。10例患者中无一例需要进一步手术。我们的结果表明，在早期AVN中，髓芯减压术对几乎所有患者都有益，即使存在X线进展。对于患有早期AVN的SCD患者，在治疗中应考虑髓芯减压术。
