后路减压联合不同融合节段固定治疗退变性脊柱侧凸的临床研究

目的比较后路减压联合短节段融合与长节段融合治疗退变性脊柱侧凸的临床疗效。方法回顾性分析50例采用后路减压联合椎弓根钉固定融合治疗的退变性脊柱侧凸患者,按融合范围分为短节段组（28例）和长节段组（22例）。收集患者术前、术后1周影像学指标（侧凸Cobb角、Cobb角矫正率）、ODI评分以及并发症发生率。结果短节段组融合节段2．4个（1～3个）,长节段组5．7个（4～7个）,两组之间差异有统计学意义（P〈0．05）。术前短节段组侧凸Cobb角为（21．4±3．7）°,术后（13．6±214）。,矫正率36．4％;长节段组术前（32．9＋6．2）。,术后（11．3＋3．5）。,矫正率65．7％,两组手术前后差异均有统计学意义,且两组侧凸Cobb角纠正率之间差异有统计学意义。短节段组6例发生并发症,长节段组10例发生并发症,长节段组术后并发症发生率较短节段组高。两组患者ODI评分差异无统计学意义。结论后路加压联合椎弓根螺钉固定融合治疗退变性脊柱侧凸疗效肯定;侧凸Cobb角较小、脊柱平衡保持较好者可以选择短节段融合,侧凸严重甚至侧方严重滑脱者宜选用长节段融合。     

Imaging in adult scoliosis: preoperative assessment and postoperative complications

Adult scoliosis rates range from 2 to 32%. Surgery for scoliosis is common. Accurate and surgically relevant information should be provided to the referring surgeon from pre- and postoperative imaging. There are various methods to correct scoliosis surgically with the end points correction of the curve and relief of symptoms. This is achieved through the placement of spinal instrumentation with a goal of osseous fusion across the instrumented levels. There are many potential postoperative complications. The initial and postoperative imaging, types of surgery, and hardware are reviewed along with the common early and late complications with relevant illustrations.     

特发性脊柱侧弯后路矫形植骨融合内固定围术期护理

目的 探讨脊柱侧弯后路矫形植骨融合内固定术围术期的护理方法.方法 对31例特发性脊柱侧弯患者采用后路矫形钉、钩棒系统内固定选择性脊柱融合术,对于重度患者采用广泛后路松解或前路松解后矫形融合治疗;术前给予心理护理、肺功能训练、自我伸长训练、唤醒试验训练、自我形象鉴定、床上生活能力训练;术后密切观察神经系统变化,正确搬运和翻身,给予最佳卧位,加强引流管护理、呼吸道护理、功能锻炼指导和出院指导.结果 31例患者均顺利完成手术.术后身高增加2～15 cm.本组未发生螺钉及棒的松动、断裂及神经系统损伤、切口感染,压疮等.结论 术前充分的准备,术后精细的护理是手术成功及患者尽早康复的重要保证.

Abstract：

Objective To probe into the perioperative nursing method during osterior fusion and fmation for idiopathic scoliosis. Methods The study involved 31 patients with idiopathic scoliosis treated with posterior correction nails and selective spinal fusion with hook-rod system fixation.The severe patients were treated with extensive posterior release or anterior correction and fusion posterior to release.Before the operation,the patients were administrated with mental nursing,lung function exercise,self-elongation exercise,wake-up pilot training,self-image appraisement and bed and life skills exercise.After the operation,an observation was done on the change of nervous system,propor turning the body over best clinostatism,drainage tube care,respiratory passage care,functional exercise instruction and discharge instruction. Results The operation was accomplished successfully in all the patients,which showed that the body height was increased 2-15 cm.There found no complications including loosening or breakage of the nails or sticks,nervous system injury,incisional wound infection or press~e sore. Conclusion Preoperative sufficient preparation and fine postoperative nursing are important guarantee to successful operation and fast rehabilitation.     

Spinal curves and scoliosis

Scoliosis, an abnormal side-to-side curve of the spine with associated vertebral rotation, affects as many as 4% of all adolescents. Several different categories of scoliosis exist, and treatment can range from observation and follow-up to bracing and surgical correction. This article discusses special imaging series for scoliosis and emphasizes the need for proper radiation protection techniques for patients with scoliosis, most of whom are girls in their early to mid-teens.     

退行性脊柱侧凸后路三维矫形术后伤口感染的处理

 目的 探讨退行性脊柱侧凸后路三维矫形术后伤口感染的诊断和处理方法.方法 回顾性分析10例退行性脊柱侧凸后路三维矫形术后伤口感染患者的治疗经过.2例为浅层伤口感染,采用单纯清创换药;8例为深部伤口迟发性感染,其中5例采用伤口清创,置管冲洗引流;3例植骨已获得融合的患者行内置物取出、病灶清除、灌洗引流术,Ⅰ期关闭切口.结果 10例患者中的8例经过上述治疗后获得治愈,2例深部伤口感染患者经伤口清创、置管冲洗引流治疗,分别于治疗后4个月、8个月再次发生深部伤口感染;复查X线片,植骨均获得融合,给予内置物取出、病灶清除、灌洗引流术,Ⅰ期关闭切口,深部感染获得痊愈.结论 退行性脊柱侧凸后路三维矫形术后伤口感染是一种严重的并发症,伤口浅层感染可经过单纯清创换药获得治愈;对于深部伤口迟发性感染,伤口清创,置管冲洗引流是一种有效的方法,可为早期感染植骨融合赢得时间;内固定取出为非必要条件.     

The prevalence of disc aging and back pain after fusion extending into the lower lumbar spine. A matched MR study twenty-five years after surgery for adolescent idiopathic scoliosis

PURPOSE: To determine the long-term outcome after fusion for adolescent idiopathic scoliosis in terms of degenerative disc findings diagnosed using MR imaging and to elucidate the clinical consequences. MATERIAL AND METHODS: Thirty-two patients with adolescent idiopathic scoliosis, who had undergone spinal fusion using Harrington rods to the lower lumbar spine with one or two unfused discs below the fusion, were re-examined 25 years after the fusion. The re-examinations included validated questionnaires, clinical examination, full standing frontal and lateral radiographs and MR examination of the lower lumbar region. Curve size and degenerative findings on MR images were evaluated by two unbiased radiologists, blinded to the clinical findings. A matched control group of 32 persons without scoliosis was subjected to the same examinations. RESULTS AND CONCLUSION: There were significantly more degenerative disc changes (p<0.0001), disc height reduction (p=0.0010) and end-plate changes (p<0.0001 for both upper and lower end-plates) in the lowest unfused disc in the patient group compared with the control group. The MR findings in the lowest unfused disc, but not the one above, in the patient group correlated to lumbar pain intensity as well as to the diminished lumbar lordosis.     

Treatment planning in severe scoliosis: the role of MRI

The use of magnetic resonance imaging (MRI) in the preoperative investigation of children with idiopathic scoliosis is controversial. Syringomyelia and other intraspinal lesions may be risk factors for neurological injury during surgical correction. Our purpose was to investigate whether pathology of the neuraxis is associated with scoliosis and to detect lesions which may threaten neurological sequelae during distraction and instrumented correction. We obtained T1- and T2-weighted images of 40 children (28 girls, 12 boys), mean age 12.7 years with severe idiopathic scoliosis (Cobb angle 50–70 °) obtained in coronal, sagittal and axial planes from the posterior cranial fossa to the sacrum, and these were assessed by two neuroradiologists and an orthopaedic surgeon prior to further treatment planning. Abnormalities of the neuraxis were found in 24 patients (60 %); five (12 %) had two or more lesions. No abnormalities of the neuraxis were found in 16 patients (40 %). There were 15 patients (38 %) with intraspinal abnormalities who deteriorated clinically and nine (22 %) who showed no clinical changes. We transferred 16 patients (40 %) from the orthopaedic to the neurosurgical department for further assessment. Our results suggest that one should investigate the neuraxis with MRI before contemplating orthopaedic surgical correction of severe idiopathic scoliosis, because the findings may lead to a change of procedure. Received: 14 July 2000 Accepted: 5 September 2000     

Scoliosis after thoracotomy for esophageal atresia

Progressive scoliosis was seen in eight children after thoracotomy for esophageal atresia. Postoperatively, all had had severe mediastinitis and empyema secondary to dehiscence of the esophageal anastomosis and required reoperation. Healing was accompanied by marked scarring and rib fusion; with growth, a scoliosis developed with the concavity toward the thoracotomy site. Most spinal curvatures appeared years after the thoracotomy and progressed rapidly at the time of adolescent growth spurt. Excision of fused ribs, spinal fusion, and instrumentation led to improvement in four patients.     

特发性脊柱侧凸不同固定术式疗效分析

目的 分析治疗特发性脊柱侧凸的不同手术方法和效果.方法 对1989年～2005年收治的34例特发性脊柱侧凸术前、术后临床资料进行分析,总结所采用不同术式的治疗效果.结果 34例分别采用了Harrington术式、联合Harrington-Luque(H-L)术式、Cotrel-Dubousset(C-D)双棍法及Texas Scottish-Rite Hospital(TSRH)三维矫正,平均矫正率分别为36.42%、45.18%、55.68%、63.28%,采用H-L、C-D和TSRH手术方法的矫正度均高于Harrington方法,其中以TSRH、C-D三维矫正系统效果最好.结论 手术矫正特发性脊柱侧凸效果是明显的,尤以近年运用的三维矫正系统效果更为优良.     

Variation in Cobb angle measurements in scoliosis

In order to determine the reliability of the Cobb angle measurement as it is used in the clinical management of scoliosis, a methodological survey was carried out. In the measurement of a Cobb angle two phases can be distinguished: (a) the production of a spinal radiograph and (b) the measurement of the angle itself. In respect of the first phase, the variation in production of the radiographs was calculated on Cobb angle measurements made by one investigator on serial radiographs of patients who underwent spinal fusion for scoliosis and therefore had a fixed spinal curvature. For the second phase, the accuracy of Cobb angle measurement was investigated by comparing measurements on the same radiographs of 46 scoliosis patients obtained by three investigators, namely two orthopaedic surgeons and an orthopaedic fellow who was assigned to a school screening project. Results were expressed as a Spearman correlation coefficient and a standard deviation of the differences. The Spearman correlation coefficient was 0.98 for the repeated radiographs (production variation) and also 0.98 for the repeated measurements on one radiograph (interobserver measurement variation). The standard deviation of the differences in Cobb angle for the repeated radiographs amounted to 3.2° and for the repeated measurements on one radiograph it was 2.0°. Although there is a good reproducibility of the Cobb angle measurement between different investigators, the variation in production of a spinal radiograph is an important source of error. This should be taken into account when making decisions in scoliosis management.We regret to inform the reader of the sudden and unexpected death on 4 December 1993 of our colleague, Dr. W. Keessen, initiator of this study.     

Three-dimensional computed tomography in the assessment of congenital scoliosis

Objective. Patients with congenital vertebral anomalies frequently are afflicted with kyphoscoliosis, with the curvatures often being severe and progressive. Spinal fusion almost always is the treatment of choice in such patients. This report examines the use of three-dimensional computed tomography (3D CT) in the preoperative investigation of patients with congenital scoliosis. Design and patients. Twelve spinal CT examinations on 11 pediatric patients with congenital scoliosis underwent image processing to produce 3D images. The 3D images were compared with both the axial sections from the CT examinations and multiplanar reformations with regard to the detection of malformations liable to cause progression of scoliosis (i. e., hemivertebrae and unsegmented bars). Results and conclusions.In six of the 12 cases, the 3D images provided improved depiction of the congenital anomalies and their interrelationships compared with planar CT images. This work suggests that 3D CT can be a useful tool in the assessment of patients with congenital scoliosis. Received: 16 February 1999 Revision requested: 19 May 1999 Revision received: 10 June 1999 Accepted: 11 June 1999     

手术治疗青少年特发性脊柱侧弯体会

目的探讨应用椎弓根内固定技术对青少年特发性脊柱侧弯（Adolescent Idiopathic Scoliosis,AIS）的手术治疗效果。方法对32例AIS患者,在严格掌握手术适应证的前提下,全部采用插管全麻,一期后路手术,选择性植入椎弓根螺钉,进行侧弯矫形及预弯、旋棒后凸旋转矫形,并选择性节段丰富植骨融合。结果全组病例Cobb角术前平均52°,术后平均16°,矫正率69.2%,随访时间12～22月,平均18月,Cobb角平均17°。结论应用椎弓根内固定技术治疗青少年特发性脊柱侧弯,矫形力量强大,可以获得一个稳定的、平衡的脊柱系统,疗效确切。     

I期后路全脊椎切除治疗重度胸腰椎畸形的神经系统并发症

目的 探讨应用I期后路全脊椎切除治疗重度胸腰椎畸形的神经系统并发症,并分析相关危险因素.方法 2000年2月-2010年9月接受I期后路全脊椎切除治疗的重度胸腰椎畸形患者67例,男29例,女38例;年龄14～62岁,平均31.4岁.其中青少年(年龄<18岁)21例,成人(年龄≥18岁)46例.侧凸畸形11例,平均冠状面主弯Cobb角90.4°;侧后凸畸形25例,冠状面主弯Cobb角94.5°,后凸角度平均65.5°;角状后凸畸形28例,平均后凸角74.3°;圆弧状后凸3例,平均后凸角91.1°.初次手术患者59例,翻修患者8例.采用主弯区顶椎全脊椎切除,全节段椎弓根螺钉内固定矫形和360°植骨融合术,统计神经系统并发症的发生情况.结果 平均随访时间14个月(3～69个月),出现神经系统并发症者共8例(11.9%),其中严重神经并发症3例,发生率4.5%,包括1例大量失血血容量灌注不足导致完全性脊髓损伤.轻度神经并发症患者5例,发牛率7.5%.胸椎全脊椎切除的神经损伤发生率要明显高于腰椎(P<0.05).多个椎体切除的并发症发牛率显著增加(P<0.05).术前已经伴有或者不伴有神经损害表现患者的神经并发症发生率分别为33.3%和7.3%(P<0.05),翻修手术的并发症发生率明显增加(P<0.05).差异虽无统计学意义(P>0.05),但出现神经系统并发症的8例患者术前均合并有严重的后凸畸形(>60.).结论 I期后路全脊椎切除是外科治疗重度胸腰椎畸形有效手术方式,但神经并发症应引起关注.相关神经损伤危险因素包括术中操作不当、大量失血、术前已经有神经受损表现、胸段截骨、多个椎体切除、翻修手术和严重后凸.

Abstract：

Objective To analyze the neurological complications in treatment of severe thoracolumbar spinal deformity with one stage posterior vertebral column resection (pVCR) and discuss the related risk factors. Methods There were 67 patients with severe thoracolumbar spinal deformity who underwent one-stage pVCR from February 2000 to September 2010.There were 29 males and 38 females at an average age of 31.4 years old(range,14-62 years).There were 21 patients at age less than 18 years old and 46 at age more than 18 years old.Patients were divided into four pathological types:severe scoliosis group(n=11,mean Cobb angle 90.4°),kyphoscoliosis group(n=25,mean scoliosis 94.5°,and mean kyphosis 65.5°),angular kyphosis group(n=28,mean kyphosis 74.3°)and global kyphosis group(n=3,mean kyphosis 91.1°).of all the patients,59 patients underwent primary surgery and eight underwent revision surgery.Surgical methods included posterior apex vertebral column resection,segemental pedicle screw fixation and correction as well as 360° bone fusion.Neurological complication was statistically analyzed. Results The average follow-up was 14 months (range,3-69 months),which showed severe neurologic complication in eight patients(11.9%)after surgery.Severe neurologic complication occurred in three patients (4.5%),among whom one patient presented delayed complete paraplegia 23 hours after surgery.Five patients had mild neurologic deficits(7.5%),the incidence of which was higher than 23.1%for thoracic osteotomy (P<0.05).Multilevel pVCR had high rate of neurological complications (P<0.05).The incidence rate was 33.3% for patients with preoperative neurologic compromise and 7.3%for patients mthom preoperative neuroiogic compromise (P<0.05).The incidence rate was increased in the revision surgery (P<0.05).Eight patients with neurological deficits had kyphotic angle of raore than 60°although there was no statistical difference (P>0.05). Conclusions pVCR is an effective surgical method for the correction of severe thoracolumbar spinal deformity.The neurological complications,however,should be paid attention to the surgeons.The risk factors for neurologic complications include improper manipulation,massive blood losing,preoperative neurologic compromise,osteotomy at thoracic rein,multi-level vertebrectomy,revision surgery and severe kyphosis.     

Imaging of painful scoliosis

Scoliosis is defined as a lateral deviation of the spine from the normal plumb line. Commonly, there is a rotational component and deviation also in the sagittal plane (kyphosis or hyperlordosis). When scoliosis presents in adults, it is often painful. In contrast, back pain in a child is considered rare, and serious underlying pathology should be excluded, particularly since idiopathic scoliosis is typically painless. A painful scoliosis in a child or adolescent, especially if the patient has a left-sided curve, should be examined thoroughly. The aim of this review is to illustrate the causes of a painful scoliosis in children, adolescents and adults.     

Correction of an image size difference between positron emission tomography (PET) and computed tomography (CT) improves image fusion of dedicated PET and CT

AIM: Clinical work in software positron emission tomography/computed tomography (PET/CT) image fusion has raised suspicion that the image sizes of PET and CT differ slightly from each other, thus rendering the images suboptimal for image fusion. The aim of this study was to evaluate the extent of the relative image size difference between PET and CT and the impact of the correction of this difference on the accuracy of image fusion. METHODS: The difference in real image size between PET and CT was evaluated using a phantom study. Subsequently, 13 patients with cancer in the head/neck area underwent both CT and [(18)F]fluorodeoxyglucose PET in a custom-made mask for external beam radiotherapy, with multimodality markers for positional reference. The image size of PET relative to CT was determined by evaluating the distances between the markers in multiple directions in both scans. Rigid-body image fusion was performed using the markers as landmarks, with and without correction of the calculated image size difference. RESULTS: Phantom studies confirmed a difference in real image size between PET and CT, caused by an absolute error in PET image size calibration. The clinical scans demonstrated an average relative difference in image size of 2.0% in the transverse plane and 0.8% along the longitudinal axis, the PET images being significantly smaller. Image fusion using original images demonstrated an average registration error of 2.7 mm. This error was decreased to 1.4 mm after size correction of the PET images, a significant improvement of 48% (P<0.001). CONCLUSIONS: A significant deviation in PET image size may occur, either as a real image size deviation or as a relative difference from CT. Although possibly not clinically relevant in normal diagnostic procedures, correction of such a difference benefits image fusion accuracy. Therefore, it is advisable to calibrate the PET image size relative to CT before performing high-accuracy rigid-body image fusion.     

Correction of nonuniform attenuation and image fusion in SPECT imaging by means of separate X-ray CT

Improvements in image quality and quantitation measurement, and the addition of detailed anatomical structures are important topics for single-photon emission tomography (SPECT). The goal of this study was to develop a practical system enabling both nonuniform attenuation correction and image fusion of SPECT images by means of high-performance X-ray computed tomography (CT). A SPECT system and a helical X-ray CT system were placed next to each other and linked with Ethernet. To avoid positional differences between the SPECT and X-ray CT studies, identical flat patient tables were used for both scans; body distortion was minimized with laser beams from the upper and lateral directions to detect the position of the skin surface. For the raw projection data of SPECT, a scatter correction was performed with the triple energy window method. Image fusion of the X-ray CT and SPECT images was performed automatically by auto-registration of fiducial markers attached to the skin surface. After registration of the X-ray CT and SPECT images, an X-ray CT-derived attenuation map was created with the calibration curve for 99mTc. The SPECT images were then reconstructed with scatter and attenuation correction by means of a maximum likelihood expectation maximization algorithm. This system was evaluated in torso and cylindlical phantoms and in 4 patients referred for myocardial SPECT imaging with Tc-99m tetrofosmin. In the torso phantom study, the SPECT and X-ray CT images overlapped exactly on the computer display. After scatter and attenuation correction, the artifactual activity reduction in the inferior wall of the myocardium improved. Conversely, the incresed activity around the torso surface and the lungs was reduced. In the abdomen, the liver activity, which was originally uniform, had recovered after scatter and attenuation correction processing. The clinical study also showed good overlapping of cardiac and skin surface outlines on the fused SPECT and X-ray CT images. The effectiveness of the scatter and attenuation correction process was similar to that observed in the phantom study. Because the total time required for computer processing was less than 10 minutes, this method of attenuation correction and image fusion for SPECT images is expected to become popular in clinical practice.     

Computed tomographic myelography in the investigation of childhood scoliosis and spinal dysraphism

The combined investigations of positive contrast myelography and computed tomographic (CT) myelography were performed on 53 consecutive children. Thirty-eight (72%) of these investigations were performed as a routine pre-operative procedure to identify occult spinal dysraphism in patients with childhood scoliosis; the remainder were because of the "orthopaedic syndrome", cervical radiculopathy, back pain and patients with clinical findings to suggest spinal dysraphism. In the 20 patients (38%) with idiopathic scoliosis, there was no case of spinal dysraphism and CT myelography provided no additional information to the myelogram. In the seven patients with spinal dysraphism the plain radiographic abnormalities identified were lumbar vertebral abnormalities (five), thoracic vertebral abnormalities (one), and sacral agenesis (one). Diastematomyelia was found in four patients, a low tethered cord and lipoma in two patients and a large lipoma in one patient. In all of these cases the myelogram indicated the intraspinal abnormalities; however, CT myelography provided more precise anatomical detail. We conclude that CT myelography is not indicated in the initial preoperative assessment of idiopathic scoliosis but should be reserved for patients with congenital or complicated scoliosis where the association with dysraphism is well recognised.     

Morphological characteristics of the young scoliotic dancer

BackgroundThe literature lacks important data about the relationship between scoliosis and growth process, scoliosis and intensive exercise, scoliosis and morphological characteristics, and scoliosis and injuries, among young dancers.ObjectiveThe aims of the present study were to determine the extent to which dance experience, body structure, anatomical anomalies and injuries are associated with scoliosis, and to identify variables able to discriminate between scoliotic and non-scoliotic female dancers at time of screening.DesignCross-sectional cohort study.MethodsOne thousand two hundred and eighty-eight non-professional female dancers, aged 8–16 years, were screened for the current study. We determined their morphometrical profile (height, weight, BMI), dance discipline (as hours of practice per week), manifestation of anatomical anomalies, and existing injuries. All dancers were clinically examined for presence of scoliosis.ResultsThree hundred and seven of the 1288 dancers (23.8%) were diagnosed as having scoliosis. Dance experience and body structure were similar for dancers with or without scoliosis. Scoliotic dancers presented a significantly higher prevalence of anatomical anomalies (such as genu varum, and hallux valgus). Back injuries were more common among scoliotic dancers compared to non-scoliotic dancers.ConclusionScreening and identifying the young scoliotic dancers prior to their advancing to higher levels of exercise is recommended. The scoliotic dancers should realize that there might be a connection between the presence of scoliosis and increased incidence of anatomical anomalies and back pain, hence, it should be suggested they seek help with an adequate assessment and exercise rehabilitation program.     

Spinal osteoblastoma: relationship between paravertebral muscle abnormalities and scoliosis

 Objective. To illustrate the variety of paravertebral muscle abnormalities as seen on computed tomography (CT) or magnetic resonance imaging (MRI) in association with spinal osteoblastomas and correlate the findings with the presence of scoliosis. Design. In a retrospective study the clinical notes, plain radiographic, CT and/or MRI features were reviewed for the presence of scoliosis and paravertebral muscle abnormality (either inflammation or atrophy). Patients. Twelve patients (7 male, 5 female) with a mean age of 17 years were studied. Three lesions occurred in the cervical spine, five in the thoracic spine and four in the lumbar spine. Results and conclusions. Nine patients had scoliosis. All patients with a thoracic or lumbar lesion and scoliosis (n=8) had an associated abnormality of the paravertebral muscles (usually atrophy with fatty replacement). One patient with a lumbar lesion and no scoliosis had normal paravertebral muscles. One patient with a cervical lesion had thoracic scoliosis but no muscle abnormality in the cervical region, while two patients with cervical lesions and no scoliosis showed muscle abnormalities. The results support the clinical hypothesis that scoliosis in patients with spinal osteoblastoma is due to paravertebral muscle spasm, although this would not appear to be the case for cervical lesions.     

The musculoskeletal manifestations of epidermolysis bullosa: an analysis of 19 cases with a review of the literature.

Epidermolysis bullosa encompasses a group of rare disorders characterized by marked skin fragility and blister formation. In patients with dystrophic epidermolysis bullosa, skeletal and soft-tissue abnormalities are an important feature. An analysis of the musculoskeletal manifestations in 19 patients is presented. In the hands and feet, features included generalized osteoporosis, wedge-shaped thinning and hooking of distal phalanges, overconstricted bones, acro-osteolysis, flexion contractures, metatarsal and metacarpal subluxation, distal trophic changes, webbing of digits, encasement of the whole extremity in a pouch of skin, soft-tissue calcification and retarded skeletal maturity. Previously undescribed findings in the hands and feet are bony ankylosis of the proximal interphalangeal joints, resorption of the metatarsal and metacarpal heads, shortened metatarsal bones, carpal and tarsal fusion and destruction, and cystic changes of the distal radius and ulna. In the remainder of the skeleton, hip dysplasia with premature osteoarthritis, knee joint bony ankylosis and thoracic and thoraco-lumbar scoliosis are other undescribed findings.