蜡泪样骨病1例

蜡泪样骨病较少见 ,多侵犯一侧肢体 ,好发于四肢的长骨 ,亦见于短骨及扁骨 ,颅骨、脊柱和肋骨较少累及。我们遇1例 ,并经 2 2年随访观察 ,特报告如下。患者　男 ,70岁。腰背部疼痛 ,活动后减轻 ,近年来腰背部疼痛加重 ,呈持续性 ,腰椎活动受限 ,伴右下肢疼痛、麻木 ,于 1997年 2月 2 1日就诊。自 1975年起感腰背部疼痛曾来院检查。体检 :腰椎向右侧弯曲 ,右第十一肋处可见沿肋骨走行的皮肤隆起 ,肤色正常 ,触诊表面凹凸不平、坚硬。T11～L2 棘突右侧可见皮肤隆起 ,肤色正常 ,触诊坚硬、固定。实验室检查无特殊。经皮穿刺右侧第十一肋远端并…     

蜡泪样骨病1例报告

蜡泪样骨病又称肢骨纹状增生症、单肢型骨质增生症、Peri病。本病为一种罕见的骨质硬化性疾病 ,我们遇到 1例 ,现报告如下。1　病例介绍患者 ,男 ,2 0岁 ,学生 ,以右踝外伤 1h之主诉来我院就诊。体检 :双下肢对称 ,右下肢皮肤无肿、热及颜色改变 ,右髋及膝关节活动正常 ,右踝关节活动受限 ,软组织肿胀。2　X线检查右股骨及胫腓骨皮质条状增生 ,呈斑点、片状高密度骨质硬化 ,骨皮质增厚 ,骨髓腔变窄 ,右足距骨、楔骨、骰骨内见团块、结节状骨质增生、硬化 ,骨轮廓无明显改变。各关节面光滑 ,关节间隙无狭窄 ,增生的骨质与正常骨质界线清晰 (…     

蜡泪样骨病1例报告

患者男,35岁。自述11年前发现右第4、5手指逐渐肿大,皮肤表面无红肿,右手用力后胀痛。近2个月感右手掌外侧区疼痛,呈间断性胀刺痛,用力后加剧。体检:右第4、5手指不规则肿大,触之有骨质硬化感,皮肤变硬,无发热红肿。掌指关节、指节关节活动受限,腕关节活动自如。X线平片(图1):右第4、5掌骨及指骨骨皮质增厚,表面骨质堆积,呈象牙质样,状如烛泪,遍及骨干全长,骨干不规则增粗,尤以掌骨和近节中节指骨较显著。骨小梁、骨髓腔大部分被病骨替代。各关节无受累,关节间隙清晰,附近软组织中无骨质沉着。加拍右肱骨、尺桡骨正侧位及左手正位均未见异…     

蜡泪样骨病1例报告

1968年瑞典Elekta公司研制成世界上第一台γ-刀，30余年来，全球已陆续建立起130个左右治疗研究中心。国内16台旋转式头部γ-刀累计治疗病人万例以上。这种立体定向放射技术的推广，避免了手术之“短”，发扬了手术之“长”，不必开刀，胜似开刀，两全其美。本文从以下几个方面谈谈体会。     

蜡泪样骨病一例报告

患者男性，62岁、20年前始发现右手中指中节末端有一黄豆大的结节，边界欠清，表面欠光，质硬，不活动，无压痛，不影响关节活动，未予特殊治疗。以后结节缓慢生长，逐渐累及中指近节指骨、掌骨、近年来中指已明显增粗，中节指骨桡侧尤为明显，手指尺侧偏畸形，刺痛减退、食指以近节指骨尺侧增粗为主，压痛明显，手指活动度明显减退。X线表现：右腕舟骨、月骨、大多角骨及头状骨有骨内致密斑，无轮廓改变。右手第2、3骨掌、右食指近节、中指近节及中节、环指近节偏侧性骨组织不规则增生，呈滴注状，并累及邻近关节（见图）。手术所见：食指…     

单侧下肢蜡泪样骨病1例

患者男,28岁,车祸入院。查体患肢皮肤损伤,无畸形,无深压痛,活动度正常。患者日常活动正常,经常从事体育活动,患肢无任何不适,否认具有家族史。影像所见:右侧下肢整侧性受累,右耻骨、右股骨干、右胫骨干及足部跗骨增     

蜡泪样骨病的影像诊断

目的：探讨蜡泪样骨病(Melorheostosis)的X线表现特点。方法：结合临床病例和献资料分析本病的X线表现征象。结果：病变常选择性分布于肢体的同一侧。本病可单发或多发,多侵犯一侧肢体的数个骨结构,其典型影像特征为受累的肢骨皮质呈不规则的波浪状骨质增生硬化,增生的骨质形似蜡烛表面向下流淌的溶蜡柱,多跨越关节,个别病例关节周围软组织中可以见到骨化或钙化。结论：本病多具有典型的影像表现,常规X线检查可以提供满意的诊断信息。     

右下全肢蜡泪样骨病1例

患者 女性，12岁。外伤后右膝关节持续性钝痛6天。查体：右膝关节皮肤光滑，无红、肿、热及功能障碍。     

蜡泪样骨病的影像学诊断

目的:探讨蜡泪样骨病的影像学表现,分析其影像学特征,提高对本病的认识。方法:结合文献,回顾性分析28例蜡泪样骨病的影像学表现,其中22例行X线检查,6例行CT检查,1例行SPECT-CT检查,2例行MRI检查。结果:28例中,单肢26例,双肢2例;单骨15例,同肢多骨11例,双肢多骨2例。X线表现:病变局限性于皮质内侧3例,弥漫性流注19例;骨皮质肥厚21例,骨髓腔变窄18例;跨关节生长11例;3例关节周围软组织出现团状不规则形钙化影。混合型15例,皮质外型4例,皮质内型3例。CT表现:骨皮质肥厚隆起、骨髓腔变窄,骨端松质骨或骨髓腔内团状高密度影,CT值约1 000HU,边界清楚。SPECT-CT表现:腓骨病变区明显异常放射性核素浓聚,跟骨病灶未见明显异常放射性核素浓聚。MRI表现:骨皮质增厚,骨髓腔狭窄,病灶呈长T1、短T2均匀或略混杂信号,与正常组织界限清楚,1例病灶周围软组织轻度水肿改变。结论:蜡泪样骨病具有特征性影像学表现,X线检查结合临床可作出明确诊断;CT和MRI有助于观察病灶的形态、密度及软组织情况;SPECT-CT为本病活跃性的判定提供了有力的参考依据。     

蜡泪样骨病（附1例报告）

患者女23岁。以“左髋部疼痛一年”为主诉入院，PE：体温37．312，血压120／65mmHg，脉搏60次份，心肺正常，四肢活动正常，左髋部托马斯征阳性。X线表现：左股骨头见结节状宽度增高，关节面清晰，左髋臼见斑片状，密度增高，关节间隙正常，关节腔内见多发性游离体，左股骨上段内侧缘见局限性皮质增厚，骨膜增生硬化，髓腔密度正常，诊断：蜡油样骨病，病人于住院期间行关节腔内游离体摘除术，术后病理报告，骨软骨病。     

蜡油样骨病1例报告

患者女,38岁。右手及前臂肿痛20余年,劳累后加剧,近来手指疼痛加重。查体:右手、前臂桡侧肿胀、隆起,皮肤色泽正常。触之表面高低不平,坚硬如石,边界清楚,轻度压痛。诸关节活动受限。X线检查:右肩胛骨上缘示“团块状”、“象牙质”样高密度骨化影,关节盂见斑点状致密影。右肱骨外侧骨皮质内缘骨质增厚、硬化,呈“蜡油样”流注,髓腔变窄,关节内亦见斑点状致密影,骨干外形轮廓无明显改变(图1)。右手第1、2指,掌骨骨皮质增厚、硬化,呈“象牙质”样改变,髓腔趋于消失。右手第3近节、中节指骨皮质内缘骨质增厚、硬化,髓腔变窄,骨干外形无改变。…     

肢骨纹状增生症1例报告

患者 ,男 ,15岁。自述 4年前一次轻微外伤后 ,出现右下肢跛行至今 ,症状逐渐加重 ,现髋关节疼痛 ,功能受限 ,尤以活动后加剧。检查 :右髋关节屈曲、内收及旋转均受限 ,无感觉异常。实验室检查无特殊发现。X线表现 :右下肢自髂骨直至右足均可见条状、斑点状骨质增生。髂骨以髋臼上方为中心呈扇形条状增生 ,向下滴注状增生的骨质累及髋关节。右股骨及胫腓骨沿外侧皮质条状增生 ,骨外形增粗 ,髓腔变窄。右足跖趾骨内膜呈扁丘状或条块状增生 ,髓腔变窄或消失 ;外膜改变轻微。各骨骺及足跗骨内呈斑点状增生 ,而轮廓无改变。诸骨增生的骨质致密如…     

Melorheostosis with scleroderma

The association of linear scleroderma with melorheostosis is very rare. Until now, only a few cases have been reported in the English literature. We describe a case of melorheostosis of a rib associated with overlying skin changes.     

Melorheostosis: a review of 23 cases

The aim of this study was to review clinical and radiological signs of melorheostosis in a large series of cases. Family history, patient history, clinical data and radiological features of 23 consecutive cases of melorheostosis were investigated. Criteria for establishing the diagnosis “melorheostosis” were defined. Sixteen patients (mean age 34 years, equal ratio between genders) had chronic pain in the affected limb(s) and/or subcutaneous fibrosis and/or various skin lesions. Number of involved bones: one bone (n = 10); two bones (n = 4); three or more bones (n = 9). Anatomic distribution: upper extremity (n = 5); lower extremity (n = 16); upper and lower extremity (n = 1); sacrum (n = 1). Radiologic pattern: osteoma-like (n = 7); classic candle wax appearance (n = 5); myositis ossificans-like (n = 1); osteopathia striata-like (n = 6); mixed pattern (n = 4). Patterns different from the appearance formerly judged to be “classic” prevail. The standard concept of disease manifestation has to be adjusted. Pathogenesis remains unclear. The classic theory claims the presence of an early embryonic infection of a sensory nerve inducing changes in the respective sclerotome, but we propose the concept of mosaicism as a better explanation for the sporadic occurrence, the asymmetric “segmental” pattern with variable extent of involvement and equal gender ratio of the disease. Received: 2 March 2000 Revised: 1 June 2000 Accepted: 6 June 2000     

右胫骨蜡油样骨病1例报告

女 ,3 9岁 ,右胫骨中、上段包块 10余年 ,近日来增大较快。患肢局部皮肤凸起 ,触诊表面凹凸不平 ,边界较清晰 ,有轻压痛。余各骨关节未见明显异常。Ｘ线检查示 :右膝关节腔内髌骨后下方可见斑片状钙化影 ,密度较高 ,边界清楚。右胫骨中、上段前缘骨轮廓不规则 ,表面呈骨堆积状凹凸不平 ,似“溶蜡样”自上而下。骨髓腔变窄 ,膝关节间隙无异常 (图 1)。Ｘ线诊断 :蜡油样骨病。讨论 :蜡油样骨病是一种罕见的骨质硬化性疾病。病因不明 ,多认为是一种先天骨骼发育障碍性疾病。好发于四肢长骨 ,亦可见于短骨及扁骨。多侵犯一侧肢体 ,双侧少见 ,男…     

Melorheostosis: unusual presentation in a girl

Melorheostosis is a rare bone disease of unknown etiology. It is characterized by tense and erythematous skin, contractures and shortening of the affected limbs. Radiographs reveal hyperostosis in long bones resembling melting wax, and spotty or patchy endostic deposits in short bones. An 11-year-old girl showed, since birth, contractural deformities in the right hand without bone abnormalities. At the age of 12 months, she complained of soft tissue tightness in the right buttock. At 3 years, limitation of flexion and extension of the right knee was evident. Radiographs revealed findings typical of melorheostosis.     

Melorheostosis and the sclerotomes: A radiological correlation

Melorheostosis is characterised radiologically by hyperostotic linear densities in bone. These densities have a peculiarly segmental distribution which does not correspond with the anatomical course of blood vessels or mixed nerve roots of the limbs. So far this distribution has lacked any valid explanation, although it has been suggested to be a developmental error as a result of an embryonic metameric disturbance. Inman and Saunders in 1944 described a sensory nerve supply to skeletal structures with sclerotomes representing the zones of the skeleton supplied by individual spinal sensory nerves. Radiographs of 30 cases from the Radiological Museum of the Institute of Orthopaedics, London, have been reviewed and an attempt has been made to correlate the sclerosing lesions with the sclerotomes. The investigation was handicapped by paucity of films and clinical information, but in 19 cases the skeletal abnormalities were considered to correspond with a single sclerotome or part thereof. These studies were convincing when films of an affected hand or foot were available. In the remaining 11 cases multiple sclerotomes appeared to be involved and the clinical manifestations were correspondingly more severe. It is proposed that melorheostosis may be the late result of a segmental sensory nerve lesion, to account for its sclerodermal distribution. The association with linear scleroderma is discussed, since it has been suggested that these cutaneous lesions are related to the same nerve segment. Eight cases showed para-articular ossification of soft tissues which may be related to involvement of a corresponding myotome.