Spinal Manifestation of Sickle Cell Disease. A Report On 35 Patients with Literature Review

Furat Hassan Shalal, T. A. Hamdan, R. Chasib, Darren F. Lui
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Abstract

Background: Sickle cell disease is a hereditary hemoglobinopathy affecting the spine in a wide range of pathology. Despite the several pathological involvements, some patients remain asymptomatic even with positive imaging findings. Objectives: Theme of this study is to report the various pathological disorders involving the spine of patients with sickle cell disease in Basra “south of Iraq” Patients and method: 35 Homozygous and heterozygous sickle cell patients were included in this study. 22 Males and 13 females, their age range from 6 to 69 years they were evaluated clinically, and also by laboratory imaging studies like plain radiography, computerized tomography, magnetic resonance imaging and mineral bone density “DEXA scan “ Results: 54% of the patients were homozygous, and the remaining were heterozygous, 66% of them were asymptomatic, back pain was the complaint in 60% while spine tenderness was reported in 40%, 31% had tenderness in the sacroiliac region and only 6% had neurological claudication and focal neurological deficit.  Biconcave vertebra was confirmed in 72% of the patients and mainly localized in the lumbar Spine, MRI findings of vertebral infarction were observed in 58% of the studied patients while sacroiliac joint changes appeared in 60%, 60% of those who had DEXA scan showed low mineral bone density in the lumbar spine Conclusion: Sickle cell disease involve the whole Spine in a wide range of pathological process, some show clinical symptoms, and some remain asymptomatic in spite of changes. The structural changes are more obvious in the homozygous group (55) the lumber spine is dominantly involved, disc prolapse, and neurological deficit is rare while the low mineral changes is common. We did not report a case of spondylodiscitis in this series, though it is very much expected, so routine screening of the spine in sticklers is mandatory
镰状细胞病的脊柱表现。35 例患者的报告及文献综述
背景:镰状细胞病是一种遗传性血红蛋白病,影响脊柱的病理范围很广。尽管存在多种病理病变,但一些患者即使在影像学检查结果呈阳性的情况下仍无症状:本研究的主题是报告 "伊拉克南部巴士拉 "镰状细胞病患者脊柱的各种病理紊乱:本研究共纳入 35 名同种和异种镰状细胞病患者。22 名男性和 13 名女性,年龄从 6 岁到 69 岁不等,他们接受了临床评估和实验室影像学检查,如X光平片、计算机断层扫描、磁共振成像和矿物质骨密度 "DEXA 扫描":54%的患者为同卵双生,其余为异卵双生,66%的患者无症状,60%的患者以背痛为主诉,40%的患者脊柱有压痛,31%的患者骶髂区域有压痛,只有6%的患者有神经性跛行和局灶性神经功能缺损。 72%的患者确诊为双凹椎体,主要位于腰椎,58%的患者在核磁共振成像中发现椎体梗塞,60%的患者出现骶髂关节病变,60%接受 DEXA 扫描的患者显示腰椎矿物质骨密度较低:镰状细胞病的病理过程涉及整个脊柱,有些患者会表现出临床症状,有些患者尽管有病变但仍无症状。结构性改变在同卵双生组(55 例)中更为明显,腰椎主要受累,椎间盘脱出和神经功能缺损罕见,而低矿物质改变则常见。尽管脊柱盘炎很有可能发生,但我们在本系列中并未报告一例脊柱盘炎病例,因此必须对顽固患者的脊柱进行常规筛查。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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