血友病性假性肿瘤1例

患者 男，17岁。自幼轻微外伤即引起瘀斑、青紫或出血不止，8岁时确诊为血友病。半年前摔倒后出现右膝关节疼痛、肿胀、功能受限。查体：右膝关节肿胀，皮肤紧张发亮，触硬，皮温不高，无静脉曲张。血液检查：凝血时间延长约20min，血小板第Ⅷ因子缺乏。X线示：右股骨髁间凹变深，右膝关节呈退行性骨关节病样改变，右胫骨上端前侧巨大囊状透亮区，边缘硬化，骨皮质膨胀。部分缺如，周围软组织明显肿胀。CT示：右胫骨上端前侧见巨大骨质破坏区，     

血友病性假肿瘤和关节炎

血友病引起的骨关节和软组织改变，文献上论述较多者为膝关节，其他较少，现将我们对45例所观察到的一些X线征象综合报道如下。     

血友病性假瘤的MDCT表现

目的提高对血友病性假瘤多层螺旋CT(MDCT)表现特点的认识。方法回顾性分析16例经临床证实的血友病性假瘤患者的CT影像学特点。16例均行MDCT检查,并行冠状位及矢状位重组,着重分析假肿瘤的部位、大小、形态、密度、强化方式及骨质改变。结果 16例经实验室检查确诊为血友病A型12例,血友病B型4例。16例中12例有外伤史。主要影像学改变为溶骨性破坏14例,软组织肿胀、肿块13例。结论血友病性假瘤影像学特征取决于病变的首发部位和出血程度。CT对血友病性假瘤的诊断具有一定的临床实用价值,对临床治疗具有重要的指导意义。     

血友病性假肿瘤1例

患者男性,31岁。1岁时因碰撞后出现肌肉、关节内出血于当地医院诊断为血友病A,当时予以输注Ⅷ因子治疗后好转,但此后上述症状反复出现,予以对症治疗。20余年前患者出现左下肢大腿肿胀,伴行走困难,并逐渐增大,曾外院CT检查提示左股骨膝关节面下骨质破坏伴肿块形成。现患者述双下肢活动障碍,发涨感明显。查体：四肢关节畸形,不能行走,左大腿扪及30cm×30cm大小肿块,质中等硬,表面皮肤紧张,无红肿,无压痛。     

血友病左侧腹膜后血肿1例

患者　男 ,16岁。原患血友病甲 ,2年前曾发生膝关节腔出血 ,并行滑膜清除术。本次以头痛、头晕、发热 ,腹胀 ,腰背不适 3天入院 ,Bp2 0 0 / 14 0mmHg ,T38 5℃ ,APTT 6 5s,SPT18s。体检 :谵妄状 ,左下腹扪及一约 10cm× 2 0cm类圆形肿物 ,质韧 ,位置固定 ,轻压痛 ,左髋关节外旋     

多骨骺血友病性假肿瘤1例

血友病性假肿瘤(ＨｅｍｏｐｈｉｌｉｃＰｓｅｕｄｏｔｕｍｏｒ)是血友病在骨骼系统的一种少见并发症,国内已有不少报道,但同时累及关节多个骨骺者相当罕见,现报道1例如下。患者,男,13岁。左膝关节肿痛3年,加重1个月。患者自5岁起,多次鼻衄及皮肤紫斑。近3年来,左膝关节肿痛,伴活动障碍。患者其弟有类似病史。查体:营养状况差,左膝关节明显肿胀,质软,有明显压痛,活动障碍,无发热及浅表静脉曲张。实验室检查:ＷＢＣ7.5Ｇ/Ｌ,ＲＢＣ2.96Ｔ/Ｌ,ＨＧＢ67ｇ/Ｌ,ＨＣＴ0.226Ｌ/Ｌ,ＰＬＴ667Ｇ/Ｌ,ＣＴ2ｍｉｎ,ＢＴ2ｍｉｎ。Ｘ线检查　左膝关节骨骺…     

血友病关节病变的X线、CT和MR影像比较分析

目的 比较观察血友病患者关节病变的X线、CT和MR影像显示差异。方法 选取14例血友病患者41个症状关节的X线、CT和MR影像。每个关节的3种影像学检查均在1d内完成。各关节病变程度均行X线分级[将关节病变严重者（5级）除外]。影像观察分别选用软组织肿胀或关节积液、骨质疏松、骨骺增大、关节侵蚀、关节面下囊肿、关节间隙狭窄、骨髓水肿、关节内出血、滑膜增厚及髁间窝增大和前、后十字韧带病变（仅用于膝关节MRI评估）等征象进行X线、CT和MR影像比较分析。结果 14例血友病患者41个症状关节X线分级中0级5个，1级7个，2级6个，3级8个，4级15个。软组织肿胀或关节积液X线显示有33个关节，CT和MRI均为34个；骨侵蚀病变MRI检出34个关节，Cr33个关节，X线20个关节。关节面下囊肿MRI显示21个关节，CT显示18个关节，X线显示9个关节。χ^2值检验表明MRI和CT分别与X线比较差异均有统计学意义（P值均〈0．05），MRI与CT比较差异无统计学意义（P〉0．05），但MRI检出骨侵蚀和关节面下囊肿病灶数均多于CT和X线；MRI显示骨髓水肿者14个关节，关节内出血者34个关节，滑膜增厚者27个关节。结论 在显示血友病关节炎病变方面，MR／优于CT和传统X线检查，可作为血友病关节炎患者的首选影像学方法。     

肾上腺孤立性纤维性肿瘤1例

<正>患者女,37岁。体检发现左侧肾上腺占位性病变1月余。偶有腹痛、多汗症状。CT检查：左侧肾上腺区见一较大混杂密度肿块,与左侧肾上腺内侧支分界不清,病灶大小约5.0 cm×7.0 cm,其内见多房囊性低密度区,增强扫描病灶实性部分及分隔呈持续渐进性强化,皮质期CT值101 HU,髓质期CT值120 HU,排泄期CT值116 HU,而低密度区未见明显强化（图1～5）。腹腔镜下行左肾上腺肿瘤切除术,术中见肿瘤大小约6.0 cm×7.0 cm,呈囊实性,     

血友病性假骨肿瘤一例

患者男、15岁2个川前人小腿前缘被硬物碰掩后出现厂If一球大小肿块．呈逐渐增大趋势．最近ZL来明l｛增大，什活动受限一体公：慢州。病容、贫m貌、个什分前中卜段软组织肿块，￥拳头人小，质中偏硬，无压病，无打肿热感。实验室检开：白细胞66XI（）9／I，i-f细胞374X10’／l，血小板314XI（）9／l。，凝血因十IX活性测定40％（正常值ho％-140％）。X线平片示左胜骨中上段骨质破坏、骨膜反应和软组织肿块（图1）。MHI图！X线平片，小人昨分中卜段计质破坏，人计明Q…和软纠织肿块国2＊m义状村Tm，。广引子骨中上段骨内及软纣1幻I肿…     

Magnetic resonance imaging in hemophilic arthropathy of the knee

The MR findings at the examination of 16 knees in 9 hemophilic patients were reviewed. The hemophilic arthropathy was demonstrated in great detail. The state of the joint cartilage, menisci, posterior cruciate ligaments and synovial tissue could be assessed. The presence of intraarticular hemorrhage and cyst fluid in bone cavities was recorded. MRI may become a valuable tool for assessment of early treatment of hemophilic arthropathy.     

Radiosynoviorthesis in hemophilic arthropathy: pathologic blood pool imaging on pre-therapeutic bone scintigraphy is not a predictor of treatment success

Purpose

Increased articular ^99mTc MDP uptake on blood pool imaging (BPI) of patients with rheumatologic conditions is indicative of active inflammatory changes, and has been suggested as a strong predictor of response to radiosynoviorthesis (RSO). In this study, we aimed to assess the value of pretreatment BPI positivity (i.e. scintigraphic-apparent hyperemia) for successful RSO in hemophilic arthropathy.

Methods

Thirty-four male patients with painful hemophilic arthropathy underwent RSO after failure of conservative treatment. Treated joints comprised the knee in eight, elbow in five, and ankle in 21 patients. Pretreatment triple-phase bone scintigraphy showed hyperemic joints (pathologic BPI) in 17 patients, whereas 17 patients had no increased tracer uptake on BPI. Response to RSO was evaluated 6 months post-treatment by measuring changes in intensity of arthralgia according to the visual analog scale (VAS), bleeding frequency, and range of motion. The association between hyperemia (pathologic BPI) and treatment outcome was examined using nonparametric tests for independent samples.

Results

Clinically evident pain relief occurred in 26 patients (76.5 %), and the mean VAS decreased from 7.7?±?1.1 to 4.6?±?2.7 (p?<?0.001). Joint bleeding frequency (hemarthrosis) decreased from 4.5?±?0.6 to 2.1?±?0.4 during the first 6 months after RSO (p?<?0.001). For both parameters (pain relief and bleeding frequency), patients experienced a similar benefit from RSO regardless of pretreatment BPI: arthralgia (p?=?0.312) and frequency of hemarthrosis (p?=?0.396). No significant improvement was observed for range of motion, but it was significantly more restricted in hyperemic joints both before (p?=?0.036) and after treatment (p?=?0.022).

Conclusions

Hemophilic arthropathy can be effectively treated with RSO regardless of pre-therapeutic BPI. Patients in whom articular hyperemia is not detectable by scintigraphy may have similar (outstanding) outcomes, and thus should not be excluded from treatment.

     

Fabella fracture with CT imaging: a case report

Fracture of the fabella is rare, may be easily overlooked, and can be a clinically important cause of posterolateral knee pain following traumatic injury or total knee arthroplasty. To date, nine case reports of fabella fracture with radiographic documentation have been reported in the literature. This report documents a 55-year-old male pedestrian who was struck by an automobile and presented with radiographs demonstrating depressed lateral tibial plateau and proximal fibula fractures. Computed tomography (CT) was performed for surgical planning and demonstrated the additional finding of a radiographically occult nondisplaced fabella fracture. To the best of our knowledge, this is the first case in which CT documentation of a fabella fracture is reported. Fracture of the fabella is a rare but important clinical entity which may be overlooked clinically and radiographically. Clinical information can provide a high index of suspicion, and when coupled with radiographic and CT findings, may lead to the correct diagnosis. CT imaging of the knee may confirm a suspected fabella fracture or may help detect a radiographically occult fracture.     

Hemophilic arthropathy: assessment with MR imaging

Fifteen patients with hemophilia, 14 of whom had hemophilic arthropathy, were examined with magnetic resonance (MR) imaging to determine if it could be used to assess hemophilic arthropathy, especially synovial hypertrophy and the status of the articular cartilage. Thirty-five joints of the appendicular skeleton were imaged. Four joints in two patients were clinically normal. Synovial hypertrophy was detected in 28 joints and appeared as areas of low to intermediate signal intensity on T1- and T2-weighted images, with foci of increased signal intensity on T2-weighted images (presumed to be due to areas of fluid or inflammation) in 16 joints. Abnormal articular cartilage was demonstrated in 26 joints; bone lesions, fluid collections, and joint space narrowing could also be seen. MR imaging appears to be useful in depicting the components of hemophilic arthropathy.     

Recurrent osteoid osteoma: a case report with imaging features

We present a case of a 14-year-old boy, who presented with pain in the left thigh region for 1 year. The pain exacerbated at night, waking up the child and was relieved by salicylates. On the basis of clinical history, conventional radiography, computerized tomography and bone scintigraphy, a diagnosis of osteoid osteoma was made. The tumor was completely excised, and there was complete remission of symptoms. Six months following surgery, the pain recurred in the same region. Conventional radiography revealed dense sclerosis at the surgical site. Three-phase skeletal scintigraphy showed a focal area of increased blood pool activity followed by intense focal uptake in delayed images, characteristic of osteoid osteoma. Computerized tomography confirmed the findings of skeletal scintigraphy.     

膝关节血友病性关节炎1例

血友病性关节炎(hemophilic arthritis)临床较为少见。现报告1例并讨论其MRI表现,以提高对该病的认识。患者男性,18岁。反复出现左膝关节肿胀、疼痛,关节活动障碍4年。近来症状加重,患者疼痛难忍,来我院就诊。查体:患者体温、呼吸、脉搏均无明显异常,左膝关节肿大、僵硬、局部压痛,关节强直,活动受限,浮髌试验阳性。实验室检查:凝血时间:16min25s(试管法)、部分凝血活酶时间     

粘多糖病影像学的多系统表现1例

患者男,5岁。自幼肝、脾大,智力发育迟缓就诊。体检：身材矮小、头大、前额突出,脊柱后突,腹膨隆,肝肋下5cm,脾脐下可触及。实验室检查：尿粘多糖测定为阳性。腹部超声：肝脏上界位于右锁中线第7肋间,肋下长5．2cm,脾肋间厚约2．5cm,肋下长约4．3cm。X线检查：     

Arthroscopic treatment of shoulder ochronotic arthropathy: a case report and review of literature

Alcaptonuria is an inherited hereditary metabolic disorder, which is associated with various systemic abnormalities and related to the accumulation of homogentisic acid and a derived melanine-like pigment in the connective tissues; the latter is termed ochronosis. We present the arthroscopic findings in the shoulder of a 58-year-old female with ochronotic arthropathy and discuss the role of arthroscopy in the diagnosis and management of this rare metabolic disorder.     

MR imaging in a child with scurvy: a case report.

Scurvy is very rare disease in industrialized societies. Nevertheless, it still exists in higher risk groups including economically disadvantaged populations with poor nutrition, such as the elderly and chronic alcoholics. The incidence of scurvy in the pediatric population is very low. This study reports a case of scurvy in a 5-year-old girl with cerebral palsy and developmental delay based on MRI findings.