脊椎朗格汉斯组织细胞增生症13例报告

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。     

手术治疗胸椎朗格汉斯组织细胞增多症一例

1 病例介绍 患儿女,6岁10个月.因背部疼痛,活动受限8个月入 院.生长发育史及家族史无特殊.检查:T11棘突处有压痛及 轻叩痛,胸腰段活动受限,病理征(一).     

胸腰椎损伤手术治疗失误原因分析

目的：分析胸腰椎损伤手术治疗失误的原因及对策。方法：1997年5月～2001年5月收治因手术失误而再次手术的63例，通过临床检查结合影像学X线平片、CT或MRI检查，对初次手术失误的原因进行评估分析。结果：63例初次手术失误原因，可分为二类：(1)手术方法选择失误：包括前、后路术式选择不当4例，椎管减压术应用不当8例，内固定器械选择不当5例；(2)手术技术应用不当，包括脊柱骨折复位不良29例，椎管减压不彻底28例，内固定技术不良32例，术中可疑伤及脊髓5例。结论：对胸腰椎损伤手术治疗需正确选择术式及正确应用减压及内固定技术。     

胸腰椎爆裂骨折的手术治疗

胸腰椎爆裂骨折为临床常见脊柱创伤，多由高能量暴力所致，如高处坠落伤、车祸伤等。因椎体爆裂性骨折多伴脊柱中柱结构损害．脊柱稳定性破坏较多．骨折块常侵占椎管从而继发椎管狭窄，治疗目标主要是脊髓及神经根的减压和脊柱稳定性的恢复。我院自1995年10月以来采用前或后入路手术治疗胸腰椎爆裂骨折患者248例，总结如下。     

儿童胸腰椎结核手术治疗的效果及并发症分析

目的 :回顾性分析儿童胸腰椎结核病灶清除、矫形、植骨融合内固定手术治疗的效果和并发症的发生情况及其原因分析。方法:2002年1月~2012年12月收治31例12岁以下儿童胸腰椎脊柱结核患者,男20例,女11例,年龄8.1±2.3岁(4~12岁),胸椎结核14例,胸腰段结核9例,腰椎结核8例。术前Frankel瘫痪分级,B级3例,C级6例,D级11例,E级11例。对仅有1个椎体部分破坏或椎体周围冷脓肿,脊柱稳定者,行前路椎体结核病灶清除植骨融合内固定术,本组3例;对椎体破坏较重或椎体破坏数量2个以上伴较重的后凸畸形者,行经后路前柱结核病灶清除,截骨矫形,钛网/异体或自体骨前柱支撑+后路椎弓根螺钉固定,本组18例;对于结核病灶破坏椎体严重,椎体破坏3个以上,冷脓肿蔓延广泛,同时伴有严重的后凸畸形者,行联合前后路手术,本组5例;对于冷脓肿以及脊柱结核破坏病灶较少,以严重后凸畸形为主要表现者,行后路截骨矫形术,本组4例;对于仅有较大冷脓肿,椎体破坏轻微者,行单纯病灶清除术,本组1例。采用X线、三维CT扫描、MRI等影像学手段评估内固定情况、畸形矫正与丢失、植骨融合效果、邻近节段畸形发生情况以及应用VAS评分、ODI评分、Frankel神经功能分级评价手术效果。随访观察并发症发生情况并分析其相关因素。结果:平均随访时间38±11个月(12~52个月),所有患者均顺利完成手术,无神经功能障碍加重者,末次随访时Frankel分级提高1~2级以上,分别为C级2例,D级3例,E级26例。VAS从术前的7.6±1.1分改善至末次随访时的2.1±1.4分;ODI评分从术前的75.3±12.1改善至末次随访时的26.2±12.4;病椎平均后凸Cobb角从术前的56.0°±2.5°改善至末次随访时的35°±1.9°,上述指标均显著改善(P0.05),所有病例通过二维CT证实全部骨性融合,平均骨愈合时间为6.1±1.1个月。手术相关并发症发生率32%(10/31):其中后凸畸形加重3例,交界性后凸畸形5例,椎弓根切割1例,结核复发1例;并发症病例和无并发症病例比较,其平均年龄较小,病变节段数较多,病程较长。上述病例经延长固定节段和翻修手术全部治愈。结论:手术治疗儿童胸腰椎结核可取得较好效果,主要并发症是出现交界性后凸畸形以及原有固定节段后凸畸形加重,其发生的原因和患儿的年龄、病变节段和范围、病程长短等密切相关。     

胸腰椎爆裂性骨折的手术治疗

胸腰椎爆裂性骨折是临床常见的脊柱损伤，多由高能量致伤力导致，如高处坠落伤，车祸伤等。由于椎体爆裂性骨折多伴有脊柱后结构的损伤，脊柱稳定性破坏较多，并大都有椎管被骨折块侵占，治疗目的主要是减压和稳定。对于胸腰椎爆裂性骨折治疗近期治疗的理念并没有大的改变，但手术方法却有一些变化。     

DRFS在胸腰椎手术中的应用

目的：评价撑开、复位、内固定系统(Distraction Reduce Fixation System DRFS)在胸腰椎手术中的应用价值。方法：对胸腰椎骨折26例，结核10例，腰椎滑脱24例病人均行DRFS内固定及植骨融合术。结果：随访4～36个月，平均14个月，26例胸腰椎骨折生理曲度恢复，伤椎高度恢复90％，神经功能除1例A级的无改善外，其余的均有1～2个级别的提高；结核10例，后突畸形矫正．脊柱稳定，植骨在4～5个月内融合，无1例复发；滑脱24例，20例完全复位占83．3％。无1例出现断钉、折板、螺钉松动。结论：DRFS材料优质、设计合理、操作简单，在术中同时完成撑开、复位、固定作用，效果优良，是胸腰椎手术中理想的内固定物。     

胸腰骶支架治疗胸腰椎稳定性骨折

胸腰椎稳定性骨折保守治疗效果良好，但传统治疗方式卧床时间长、并发症多。我们自2001年1月至2003年1月应用胸腰骶支架（TLSO）治疗此类损伤43例，取得较好效果，报告如下。     

后路手术治疗无神经症状胸腰椎爆裂骨折的临床观察

目的探讨无神经症状的胸腰椎爆裂骨折的最佳治疗方法及其选择依据。方法选取2009年1月至2011年4月我院收治的90例无神经功能损害的胸腰椎爆裂骨折患者为研究对象,知情同意后,遵循自愿原则分为手术组（38例）和保守组（52例）。测量并比较两组的伤椎后凸Cobb角、伤椎前后缘高度、椎管侵占率、卧床时间、治疗费用、复工时间等指标及总体疗效。结果治疗后两组伤椎后凸Cobb角、伤椎前后缘高度、椎管侵占率均有明显改善（P〈0．05）,手术组的卧床时间和复工时间明显短于保守组,而治疗费用明显高于保守组（P〈O．05）;两组均取得较好的优良率,差异无统计学意义（P〉0．05）。结论对于青壮年患者手术治疗可以更快的恢复其家庭和社会功能,并能在最大程度上减少并发症;对于家庭经济条件较差、身体条件和手术适应证不理想的患者采用保守治疗则更为适宜。     

Surgical treatment based on pedicle screw instrumentation for thoracic or lumbar spinal Langerhans cell histiocytosis complicated with neurologic deficit in children

Background contextSurgical indications and procedures for spinal Langerhans cell histiocytosis (LCH) in children are still controversial. Reports containing large samples of surgically treated patients are few in the currently available literature, and the reported operative procedures were also somewhat obsolete. So, further investigation based on large-sample cases and using improved surgical techniques is beneficial and helpful to refine the treatment strategy.PurposeTo recommend a reasonable treatment strategy for thoracic or lumbar spine LCH in children complicated with neurologic deficit.Study design/settingRetrospective/academic medical center.Patient sampleTwelve children aged from 2 to 16 years old with the diagnosis of thoracic or lumbar spinal LCH accompanied by neurologic deficit received surgical treatment from January 2005 to January 2010.Outcome measuresFrankel scale for neurologic function, fusion of the mass, and recurrence of the lesion.MethodsAll 12 patients presented initially with local pain and progressive neurologic detriment. Neurologic evaluation revealed two patients with Frankel Grade B, eight with Grade C, and two with Grade D. Radiographic features were positive for typical vertebra plana, a space-occupying mass in the spinal canal compressing neural elements, and a spinal canal encroachment rate more than 50%. Posterior instrumentation with pedicle screw combined with anterior corpectomy, decompression, and support bone graft was performed in the first seven patients as a one-stage procedure. In the remaining five patients, posterior pedicle screw fixation, laminectomy for decompression (via excision of the tumor-like mass), and repair of laminae with allograft bone block were performed. The collapsed vertebral body was left untouched. No chemotherapy or radiotherapy was administrated postoperatively in any of the cases.ResultsThe mean follow-up duration was 43.3 months. The mean operation time was 330 minutes with combined procedure and 142 minutes with single posterior approach (p=.000). The average blood loss was 933 mL with combined procedure and 497 mL with single posterior approach (p=.039). Three of seven patients who received combined surgery encountered approach-related complications, that is, one with intercostal neuralgia and two with pleural effusion. No severe neurologic deteriorate, instrumentation failure, or disease recurrence was detected at follow-up. Neurologic function completely recovered in all 12 patients from 2 to 12 weeks after surgery. The anterior bone graft fused and shaped well in all seven patients, and allograft bone block for lamina repair also achieved complete fusion in the remaining five patients. The internal fixator was removed at 3 to 5 years (average 4.1 years) after initial operation in six patients. No deformity, including scoliosis and kyphosis, has been identified during follow-up period in both procedures.ConclusionsFor spinal LCH patients, neurologic deficit is a main indication for operative treatment to prevent permanent and serious consequences. Surgery provides an opportunity for rapid recovery of neurologic function. Both combined and single-stage posterior approaches based on pedicle screw instrumentation techniques are similarly effective in relieving neurologic compression. However, single-stage posterior approach is more favorable with less complications, and preserving involved vertebral body is not a latent hazard of recurrence.     

Zoledronic acid in treatment of bone lesions by Langerhans cell histiocytosis

Langerhans cell histiocytosis (LCH) is a rare disease, probably an atypical myeloproliferative syndrome, with variable clinical presentation and behavior. In this report, we focus on bone involvement by LCH and treatment with zoledronic acid in six patients as they progressed after chemotherapy and radiotherapy. Zoledronic acid appeared safe and produced significant relief in pain.     

Langerhans cell histiocytosis of the penis

Summary Involvement of the male genitalia by langerhans cell histiocytosis ist very rare. It has been reported in only three cases yet. We present a patient with recurrent disease of the skin of the penis and by proliferation involvement of the distal urethra and the corpus cavernosum. An overview of the disease and its therapy is given.      

Clinical features and treatment outcomes of Langerhans cell histiocytosis of the spine

Background Context

Langerhans cell histiocytosis (LCH) of the spine is a relatively rare condition with unknown etiology. The diagnosis and treatment protocols for spine LCH remain controversial.

CNS involvement of Langerhans cell histiocytosis. Report of 23 surgically treated cases

We present 23 cases of Langerhans cell histiocytosis with central nervous system (CNS) involvement. The major complaints were a mass on the cranial vault in fifteen (65 %), visual disturbance in four (16 %), polyurea-polydipsia in three (13 %), and progressive weakness in all extremities in one patient (4 %). Neurological examination revealed no abnormality in sixteen patients (70 %), cranial nerve palsy, visual field defect and optic atrophy in six (26 %) and paraparesis in one (4 %). Tumoral mass was found to be located on the cranial vault (65 %), in the suprasellar region (21 %) and in the spinal column (8 %). The cranium and spinal column were both involved in one patient. All patients underwent surgery; craniectomy with grossly total tumor excision plus cranioplasty (65 %), craniotomy with subtotal tumor excision (26 %), and vertebrectomy with grafting (13 %) were performed. The clinical radiological and histopathological features, as well as therapeutical considerations are discussed and the pertinent literature is reviewed.     

Langerhans cell histiocytosis: coexistence of bronchogenic and thymic cysts in the thymus

Langerhans cell histiocytosis (LCH) is a disease caused by the proliferation of Langerhans cells in various tissues or organs. A 43-year-old male patient presented with an anterior mediastinal mass in the thymus. Histological examination after a thymectomy revealed a bronchogenic cyst in the thymus, and multiple LCH and small thymic cysts were also incidentally observed in the thymus. Unifocal LCH in an adult occurring in the thymus is extremely rare. Furthermore, no cases of LCH with the coexistence of bronchogenic and thymic cysts in the thymus have been previously reported.     

Surgical options for osteoporotic vertebral compression fractures complicated with spinal deformity and neurologic deficit

IntroductionThis paper describes surgical options for Osteoporotic vertebral compression fracture (OVCF) with acute flexible or chronic rigid kyphosis, and pseudarthrosis complicated with pain and neurologic deficit.MethodsThis study has two components. a) A prospective clinical study of surgical treatment of 31 patients (age: 69 ± 11 years) with either acute flexible or progressive pseudarthrotic kyphosis manifested with severe pain or neurological deficit between 2010 and 2014. Eleven patients exhibited neurocompression (Frankel B, C, D). Surgery consisted in indirect reduction, kyphoplasty, and short posterior instrumentation in 28 patients and multilevel instrumentation in three. b) The second component involved a literature search of OVCF complicated with acute or painful chronic deformities and neurologic deficit, managed with open surgical approach.ResultsIndirect reduction, kyphoplasty and short posterior stabilization can restore satisfactory anatomic alignment and neurological deficit. Multilevel instrumentation was used for rigid long kyphosis. Complications were related to a) screw pull out and junctional kyphosis (4 patients) one of the patients also developed anterior migration of cement, b) cement leakage (4 patients). L5 radiculopathy occurred in one patient. The others remained asymptomatic. The literature review concluded that corpectomy with anterior, posterior or combined instrumentations is indicated for burst fractures, or rigid kyphosis with neurocompression. Prompt decompression with anatomical alignment may restore paraplegia. Complications were germane to osteoporotic bone predisposing to hardware loosening or cut out and dislodgement of instrumentation.DiscussionNeurologic deficit associated with fractures or progressive pseudarthrotic kyphosis effectively may respond to indirect postural reduction, kyphoplasty and posterior percutaneous short segment transpedicle instrumentation. For burst fractures and rigid chronic kyphosis corpectomy reconstructed with cages and anterior, or posterior or combined instrumentations can restore and maintain normal anatomy. The following guidelines for optimal surgical instrumentation have been established: To prevent screw loosening and junctional kyphosis the instrumentation should not end within the kyphotic segment. Screws for anterior instrumentation should penetrate the contralateral cortex. Multiple site of fixation or combined anterior and posterior instrumentations dissipate stresses at any one site. Augmentation of transpedicle screw fixation with cement is a sound technical principle. Cement should inserted in a doughy state with minimal pressure to prevent cement complications.     

胸、腰段脊柱脊髓损伤外科治疗失误的处理

目的:分析急性胸、腰段脊柱脊髓损伤外科治疗失误的主要原因,探讨处理原则。方法:对104例首次外科治疗失误的急性胸、腰段脊柱脊髓损伤患者的临床资料和影像学资料进行分析,并根据临床具体情况进行外科处理。结果:104例患者中,残存腰痛45例次,脊柱畸形30例次,脊柱不稳35例次,脊髓神经受压8例次,坐位失平衡8例次。经再次手术治疗后脊柱畸形患者明显改善;椎管残存压迫的不完全性脊髓损伤患者椎管减压术后运动指数和感觉指数均增加;30例脊柱不稳定患者重建了脊柱稳定性;45例腰痛患者中40例腰痛消失或基本消失,5例仍有轻度腰痛需应用止痛药物,但不影响轮椅活动。结论:胸、腰段脊柱脊髓损伤手术治疗失误的主要因素包括手术适应证选择不当、手术技术失误和保守治疗不当。再处理方案主要参考首次治疗主要失误因素来确定。     

Langerhans cell histiocytosis and human herpes virus 6 (HHV-6), an analysis by real-time polymerase chain reaction.

Patients with Langerhans cell histiocytosis (LCH) usually present to orthopedic surgeons because this disease most commonly affects bone. The pathogenesis of LCH is unknown, although roles for environmental, infectious, immunologic, and genetic causes have been postulated. More specifically, there is limited data suggesting that human herpes virus 6 (HHV-6) may be a potential etiologic agent. Frozen biopsy material was obtained from 13 patients with LCH and 20 patients without the disease. After ensuring histologic adequacy of the material, the tissue was tested for HHV-6 by qualitative and quantitative real-time TaqMan PCR. Four of 13 patients with LCH had evidence of HHV-6 DNA in their tissue while 7 of 20 control patients tested positive for HHV-6 genome. Viral loads are reported for the positive patients; no statistical difference was observed in the presence or quantity of HHV-6 DNA found in either population, suggesting that the prevalence of HHV-6 in the tissue of LCH patients is the same as that found in tissue from individuals without disease.