CAJ | 학술논문

背景:脊椎朗格汉斯组织细胞增生症(LCH)是一种少见病,诊断困难,治疗方法仍有争议. 目的:探讨脊椎LCH的临床和影像学特征及其治疗效果. 方法:回顾性分析1985年10月至2003年10月收治的13例脊椎LCH患者的临床资料,男7例,女6例,年龄1.5~11岁,平均5.5岁.脊椎以外颅骨、腓骨、股骨颈、股骨大转子和坐骨等多发骨骼受累6例;勒-雪病发病1~2年内出现新的骨病变3例;患病处疼痛或放射痛、压痛或畸形;合并四肢轻瘫3例;骶神经受压1例;皮疹、间质性肺炎、肝大、尿崩症等多系统受累5例.对合并四肢轻瘫者行化疗或放疗加手术治疗2例;因骶神经受压行手术减压1例;仅行放疗1例;单用化疗3例;无全身症状,无进行性脊柱畸形者,密切观察不作特殊处理6例.X线、CT的特点有椎旁阴影扩大,椎体溶骨性改变(呈楔形变或致密薄片),病变严重者累及椎弓根或椎板6例.两相邻椎体受累者可见椎间变窄,与椎体炎症难鉴别.经活检或手术确诊7例,根据临床资料、影像学确诊6例. 结果:经治疗4例脊髓及骶神经功能恢复.2例尿崩症随访11年1例无改善,另1例恶化.12例经2~12年随访(平均7.4年)椎体高度不同程度恢复,6例恢复正常,5例轻度后突畸形,1例轻度侧突畸形.失访1例. 结论:根据临床及X线、CT或MRI资料,并结合活检及密切随访,有助于多系统或多发骨骼受累的脊椎LCH的诊断.化疗结合手术可控制脊椎LCH进展.全身无症状的脊椎LCH无进行性畸形者密切观察无需特殊处理.
배경:척추랑격한사조직세포증생증(LCH)시일충소견병,진단곤난,치료방법잉유쟁의. 목적:탐토척추LCH적림상화영상학특정급기치료효과. 방법:회고성분석1985년10월지2003년10월수치적13례척추LCH환자적림상자료,남7례,녀6례,년령1.5~11세,평균5.5세.척추이외로골、비골、고골경、고골대전자화좌골등다발골격수루6례;륵-설병발병1~2년내출현신적골병변3례;환병처동통혹방사통、압통혹기형;합병사지경탄3례;저신경수압1례;피진、간질성폐염、간대、뇨붕증등다계통수루5례.대합병사지경탄자행화료혹방료가수술치료2례;인저신경수압행수술감압1례;부행방료1례;단용화료3례;무전신증상,무진행성척주기형자,밀절관찰불작특수처리6례.X선、CT적특점유추방음영확대,추체용골성개변(정설형변혹치밀박편),병변엄중자루급추궁근혹추판6례.량상린추체수루자가견추간변착,여추체염증난감별.경활검혹수술학진7례,근거림상자료、영상학학진6례. 결과:경치료4례척수급저신경공능회복.2례뇨붕증수방11년1례무개선,령1례악화.12례경2~12년수방(평균7.4년)추체고도불동정도회복,6례회복정상,5례경도후돌기형,1례경도측돌기형.실방1례. 결론:근거림상급X선、CT혹MRI자료,병결합활검급밀절수방,유조우다계통혹다발골격수루적척추LCH적진단.화료결합수술가공제척추LCH진전.전신무증상적척추LCH무진행성기형자밀절관찰무수특수처리.
@@@@Background: Langerhans cell histiocytosis (LCH) of the spine is a rare condition and is difficult to diagnose, and appropri-ate treatment is still controversial. @@@@Objective: To investigate the clinical and imaging features and therapeutic efficacy of spinal LCH. @@@@Methods: Thirteen cases with spine LCH treated from October 1985 to October 2003 were reviewed retrospectively. There were 7 boys and 6 girls with an average age of 5.5 years (range, 1.5 to 11 years). Six cases had extra-spinal skeletal lesions involved in skull, fibula, ischium, pubis, greater trochanter and neck of femur. Among those with Letterer-Siwe disease, new osseous lesions occurred in 3 cases within 1 to 2 years. The symptoms included pain, refered pain, tenderness or abnormali-ties in the focus. Of all cases, there were 3 cases with mild tetraplegia, 1 case with sacral nerve compression, and 5 cases with systemic lesions including rash, interstitial pneumonia, hepatomegaly and diabetes insipidus. Two cases with mild tetra-plegia were treated with radiotherapy (or chemotherapy) plus surgery. One case with sacral nerve compression underwent decompression. One were treated with radiotherapy and 3 treated with chemotherapy only. For six cases without systemic symptoms or progressive spinal deformity, no special treatment was done under close observation. X-ray and CT showed wide paravertebral shadow, osteolysis of the vertebrae with wedge shape or vertebra plana. Serious lesions involved verte-bral pedicles and even vertebral plates in 6 cases. When adjacent vertebral bodies were involved, the narrowed intervertebral space could be seen, which was difficult to be differentiated from vertebral inflammation. Seven cases were diagnosed by bi-opsy or surgery and 6 by clinical and imaging data. @@@@Results: The spinal cord and sacral nerve function recovered in 4 cases after treatment. Two cases with diabetes insipidus were followed for 11 years, in whom one was unchanged and one deteriorated. The vertebral height in 12 cases restored spontaneously to various extent over a follow-up of 2-12 years (mean,7.4 years). Six cases restored completely, and mild ky-phosis was found in 5 cases and mild scoliosis in 1. One case lost contact. @@@@Conclusions: Based on clinical findings, the characteristics of X-ray, CT and MR images combined with biopsy and close follow-up, the diagnosis of spinal LCH with multi-skeletal lesions or multi-systemic lesions can be made. Chemotherapy combined with surgery may control the progression of spinal LCH. For LCH of the spine in the absence of systemic disease, there is no need for special treatment except close follow-up, unless the progressive spinal deformity is found.