Complex bilateral polysyndactyly featuring a triplet of delta phalanges in a syndactylised digit

The delta phalanx is a rare congenital skeletal anomaly. An abnormal C-shaped epiphysis is usually responsible for a progressive angular digital deformity observed either in hands or feet. Solitary delta phalanges are usually described. We report a case of bilateral congenital hand malformations featuring a triplet of delta phalanges affecting a single digit on one hand, together with a concealed central polydactyly on the other.     

Frostbite of the upper and lower limbs in an expert mountain climber: the value of bone scan in the prediction of amputation level

A 38 year old man was admitted to our hospital 10 days after suffering a frostbite injury in hands and feet while practicing mountain climbing, at 8,100 meters of altitude, while he was trying to reach the top of the K2 mountain. A 99mTc-MDP bone scan performed in aseptic conditions showed: in hands: absence of bone uptake in the 3rd phalanx and distal portion of 2nd phalanx of the 5th finger of the left hand, and multiple areas of increased uptake in the distal portion of both hands. In feet: uptake decreases in the 2nd phalanx of the first toe of the left foot, and absence of bone uptake in the 3rd phalanx of the 2nd toe of the left foot, and in 2nd phalanx of the 1st toe and 3rd phalanx of the 2nd, 3rd and 4th toes of the right foot. As in the hands, there were multiple areas of increased uptake in the distal portion of both feet. The phalanges with absence of bone uptake had to be amputated, while those that presented increased uptake recovered with conservative treatment. Bone scan is indicated in the evaluation of frostbite injuries and helps to establish the prognosis early.     

The duplicated longitudinal epiphysis or “kissing delta phalanx”: evolution and variation in three different disorders

Background The delta phalanx, also known as the delta bone, or longitudinal epiphyseal bracket (LEB), has been described in a variety of syndromes and dysplasias. However, the kissing delta phalanx is not as well recognized in the literature; it consists of a duplicated longitudinal bracketed epiphysis, or a complex of two adjacent delta bones, with opposing convex portions facing each other. Magnetic resonance imaging of the kissing delta phalanx has not been previously described.Objective To describe the evolution, variation and associated osseous anomalies of the kissing delta phalanx in three patients with distinct distal limb malformations using both plain films and magnetic resonance imaging.Results Patient 1 had Rubinstein-Taybi syndrome and, in addition to a kissing delta phalanx in both feet, had corresponding delta metatarsals (MT1). Patient 2 had Cenani-Lenz syndactyly with distinct variation of the kissing delta phalanx in each hand. He had a disorganized appearance to the phalanges, metacarpal fusions and carpal coalitions. Patient 3 had an isolated oligosyndactyly of the left hand with metacarpal fusions and carpal coalitions. All three patients were followed over time. We describe two types of kissing delta phalanges: separated (without fusion of the corresponding epiphyseal brackets) and nonseparated (with fusion of the corresponding epiphyseal brackets). Both types can be seen in the same patient and are a reflection of the relative degree of segmentation of the two delta bones.Conclusion The appearance of this rare osseous abnormality changes with time and can be found in a limited number of uncommon disorders. It can also be found in association with other osseous anomalies of the distal extremities; therefore magnetic resonance imaging early in life can greatly assist in surgical planning. Recognition of the kissing delta phalanx may be an important radiological clue to diagnosis of these rare disorders.     

Osteosclerosis of the phalanges in Werner syndrome

Werner syndrome is an autosomal-recessive disease characterized by premature aging, shortness of stature, scleroderma-like skin changes, endocrine abnormalities, and cataracts. Although radiographic findings have been well documented, the presence of distinctive osteosclerotic changes in the phalanges of the hands and feet has not been emphasized in previous publications. The authors' review of radiographs of both hands in nine patients and of both feet in six patients with Werner syndrome documented the frequent occurrence of phalangeal sclerosis related predominantly to endosteal thickening. In the hand, sclerosis was present in every patient, was generally symmetric in distribution, predominated in the distal phalanges, and demonstrated an ulnar predilection. Similar changes in the phalanges of the feet were demonstrated in only two patients. The presence of osteosclerosis in the phalanges of the hand alone or both the hand and foot, when combined with osteoporosis and periarticular calcification, suggests the diagnosis of Werner syndrome.     

Osteo-articular tuberculosis

Various authors have reported differences in the radiological features of skeletal tuberculosis in the coloured patient. We studied retrospectively the radiographs of 71 patients with proven skeletal tuberculosis seen in our hospital, over a 6 year period. Sclerotic bone reactions and periosteal new bone formations were unusual findings but multiple bone involvement occurred about 15% of our patients. There was also a high in incidence of tuberculosis of the ankle and foot compared to that reported in white Caucasian patients.     

Bone excrescence at the medial base of the distal phalanx of the first toe: normal variant,reactive change,or neoplasia?

In a review of 157 anteroposterior (AP) foot radiographs in 117 adult and pediatric patients, we observed a medial excrescence on the plantar aspect of the terminal phalanx of the hallux in 88.5% of the feet. Histologic analysis documented the presence of reactive bone. The bony outgrowth appears after closure of the adjacent physeal plate and is rarely symptomatic. Its high prevalence indirectly implies that it is not specific to any particular type of foot. Although certain types of foot may contribute to its development, we believe that repetitive forces occurring during ambulation induce this reactive bone formation.Supported in part by VA grant SA 306     

毛发-鼻-指(趾)综合征病例分析并文献复习

目的:探讨毛发-鼻-指(趾)综合征(TRPS)的主要临床特征、骨骼X线表现及毛发超微结构特点,提高对该病的认识.方法:用刻度尺在X线片上测量患者手足长骨长度,分析患者手足骨正位片、鼻骨侧位片、脊柱正位片、骨盆正位片,以及髋关节与骶髂关节MRI图像.总结不同类型TRPS的临床特征及鉴别诊断要点.取患者头发于光镜及电镜下观...     

Chondroblastoma of the hands and feet

Objective To review the imaging findings, age and gender distribution of chondroblastoma of the hands and feet.Design and patients Twenty-five cases of pathologically proven chondroblastoma of the hands and feet were reviewed. Available imaging modalities included radiographs (n=24), CT (n=3), MRI (n=5), and radionuclide bone scintigraphy (n=1). The following imaging features for each case were tabulated: location, presence of sclerotic margin, calcification, expansion, presence of fluid/fluid levels on cross-sectional imaging and surrounding edema on MRI. The images were evaluated for skeletal maturity using closure of the physeal plate in the region as a standard.Results The average age at time of diagnosis was 23 years (range 7–57 years). Eighty-four percent (n=21) of the patients were skeletally mature. Males (20 of 25) outnumbered females by a ratio of 5:1. The bones of the foot accounted for 22 cases: calcaneus (n=8), talus (n=8), metatarsals (n=3), and the cuboid (n=3). The bones of the hand accounted for three cases: phalanx (n=1), triquetrum (n=1), and a metacarpal (n=1). Radiographically all lesions were osteolytic with identifiable calcification in 54% (13 of 24). Fluid/fluid levels were seen in four of five cases on MRI. Edema on MR images was seen in 40% (2 of 5). The size of the lesions ranged from 2 to 41 cm².Conclusion Chondroblastomas of the hands and feet share many of the radiographic characteristics seen in the long bones, but manifest in skeletally mature patients with a higher male to female ratio than in long bone chondroblastoma. Talar and calcaneal lesions were encountered only in males. Chondroblastoma of the wrist and hand appears to be exceptionally rare.Presented at the special scientific session of the International Skeletal Society held in San Francisco, California, USA, September 2003     

Radiological analysis of hand and foot injuries after small aircraft crashes

Medico-legal investigation of fatal aviation accidents should contribute to the reconstruction of the accident in addition to providing the usual information about cause and manner of death. In cases with more than one fatality, the question of who was flying the plane at the time of the crash may need to be answered. In such cases the identification of “control injuries” plays an important role. This study aims to investigate whether specific patterns of skeletal hand and foot injuries could assist in the identification of the pilot. The analysis of radiological investigations of hands and feet of 27 fatalities from 18 accidents showed that foot injuries are more frequent than hand injuries in pilots and passengers, dislocations of feet were more frequent in passengers, and right-sided injuries were more frequent in pilots. Injuries of the distal parts of the hand were slightly more frequent in the pilot group. The limited numbers in the study do not allow definitive conclusions and further investigations are needed. However, the study yields interesting results and shows that radiological examination should be included in the medico-legal air crash investigation.     

Skeletal abnormalities of hands and feet in Laurence-Moon-Bardet-Biedl (LMBB) syndrome: a radiographic study

 Objective. To identify radiological changes of the hands and feet in a large group of patients with Laurence-Moon-Bardet-Biedl syndrome. Design. Postero-anterior views of hands and feet were obtained and analysed. Patients. The material consists of 43 Scandinavian patients with the syndrome (24 males and 19 females; age 3 weeks to 57 years, median 23 years at the time of radiological examination). Results and Conclusions. Polydactyly of the hands and feet is one of the main criteria. This was noted clinically in 33 of 43 patients, but all but 3 had been operated on before this radiological study. Remnants of the extirpated finger or toe noted as exostoses, additional joint surfaces of duplication were found in half the hands and feet, while the remainder showed no radiological changes. Other features found were short, broad bones and flat joint surfaces of the metacarpophalangeal or metatarsophalangeal joints. We also found a high frequency of short or long ulna in relation to the radius and Madelung deformity of the wrist in several patients. Thus, the radiographs showed several non-specific normal variations besides remnants or postoperative changes after polydactyly.     

Radiological picture of the hand and foot in systemic lupus erythematosus

A detailed examination of the hands and feet was performed in a group of 34 patients affected by systemic lupus erythematosus (SLE), using low-dose mammographic film and Rank Xerox selenium plate, according to current diagnostic techniques. All patients presented articular symptoms (pain and arthralgia). The high incidence (38,8%) of patients with no radiographic evidence of bone damage-even though articular symptoms are present-is emphasized. In such cases, it is very difficult to distinguish SLE from rheumatoid arthritis, especially as far as therapeutic management and prognosis are concerned. The lack of any "pathognomonic" radiological sign of the lupus arthritis, in the hands as well as in the feet, is then stressed. Nevertheless, arthropathy in SLE is defined as a deforming non-erosive arthritis, with a typical symmetric distribution, affecting most commonly-according to incidence-the proximal interphalangeal and metacarpophalangeal joints. In the hand, arthropathy is referred to as Jaccoud's type arthritis, because it is characterized by joint deformities which can be corrected. In the foot, the main abnormalities include hallux valgus, subluxation of the metatarsophalangeal joints and widening of the forefoot.     

Initial experience with visualizing hand and foot tendons by dual-energy computed tomography

PurposeTo assess the feasibility of visualizing hand and foot tendons by dual-energy computed tomography (CT).Methods and materialsTwenty patients who suffered from hand or feet pains were scanned on dual-source CT (Definition, Forchheim, Germany) with dual-energy mode at tube voltages of 140 and 80 kV and a corresponding ratio of 1:4 between tube currents. The reconstructed images were postprocessed by volume rendering techniques (VRT) and multiplanar reconstruction (MPR). All of the suspected lesions were confirmed by surgery or follow-up studies.ResultsTwelve patients (total of 24 hands and feet, respectively) were found to be normal and the other eight patients (total of nine hands and feet, respectively) were found abnormal. Dual-energy techniques are very useful in visualizing tendons of the hands and feet, such as flexor pollicis longus tendon, flexor digitorum superficialis/profundus tendon, Achilles tendon, extensor hallucis longus tendon, and extensor digitorum longus tendon, etc. It can depict the whole shape of the tendons and their fixation points clearly. Peroneus longus tendon in the sole of the foot was not displayed very well. The distal ends of metacarpophalangeal joints with extensor digitoium tendon and extensor pollicis longus tendon were poorly shown. The lesions of tendons such as the circuitry, thickening, and adherence were also shown clearly.ConclusionDual-energy CT offers a new method to visualize tendons of the hand and foot. It could clearly display both anatomical structures and pathologic changes of hand and foot tendons.     

Greig's syndrome. Neonatal radiologic manifestations

A newborn infant with postaxial polydactyly of the hands, preaxial polydactyly of the feet, syndactyly and craniofacial dysmorphism (Greig's Syndrome) is reported. The radiographic manifestations in the neonatal period are described. The advanced bone age, the unusual shape of the iliac bones and the supernumerary prehallux toe are new radiological findings in Greig's Syndrome at this age. The diffuse distribution of skeletal anomalies suggests a more complex problem of the intrauterine process of ossification.     

Estimation of stature from dimensions of hands and feet in a North Indian population

In medico-legal autopsies, establishing personal identity of the victims is often required. Estimation of stature from extremities and their parts plays an important role in identifying the dead in forensic examinations. The study examines the relationship between stature and dimensions of hands and feet among Rajputs of Himachal Pradesh -- a North Indian endogamous group. The purpose for understanding these examinations was the paucity in the literature of studies that allow the reconstruction of stature from various dimensions of hands and feet amongst Rajputs. Hand length, hand breadth, foot length and foot breadth of 246 subjects comprising 123 males and 123 females ranging in age from 17 to 20 years were taken independently on left and right side of each individual. Statistical analyses indicated that the bilateral variation was insignificant for all the measurements except hand breadth in both the sexes (P<0.01). Sex differences were found to be highly significant for all the measurements (P<0.01). Linear and multiple regression equations for stature estimation were calculated using the above mentioned variables. The correlation coefficients between stature and all the measurements of hands and feet were found to be positive and statistically significant. The highest correlation coefficient between stature and foot length and lowest SEE (standard error of estimate) indicate that the foot length provides highest reliability and accuracy in estimating stature of an unknown individual. The regression equations were checked for their accuracy by comparing the estimated stature and actual stature.     

Congenital skeletal abnormalities: an introduction to the radiological semiology

Despite the recent advances in the molecular diagnosis of congenital abnormalities, the initial identification and the decision to refer a patient for further molecular analysis and expensive genetic tests still relies frequently on clinical and radiological criteria. The radiological identification of syndromes, dwarfs and dysplasias is a difficult task, because there are so many findings to consider and so many syndromes to remember that the problem is overwhelming. There is a definite need for an easy and systematic analysis system, in order to try to categorize a skeletal dysplasia in a certain group. In this brief review, we suggest an approach to the evaluation of skeletal syndromes, based on the analysis of cardinal criteria, from which the most useful information is derived, and additional criteria, making further differentiation possible. Generally, cardinal information is derived from analysis of the long bones, hands, pelvis and the spine, whereas the analysis of other skeletal elements, like the skull, feet, and other flat bones is of additional value.     

Radiographic appearance of Ewing sarcoma of the hands and feet: report from the Intergroup Ewing Sarcoma Study

Review of current data from the Intergroup Ewing Sarcoma Study (IESS) shows that Ewing sarcoma is rare in bones of the hands and feet. The 12 patients from the IESS protocols with hand or foot Ewing sarcoma are comparable to those already reported in the literature. With the exception of lesions in the calcaneus, the prognosis for disease-free survival is excellent. The radiographic features of hand and foot Ewing sarcoma are generally those of classic Ewing sarcoma: permeation, soft-tissue mass, and, often, associated sclerotic reaction. However, with the exception of sclerosis, features suggesting bone reaction and slow tumor growth in these patients were distinctly uncommon compared with Ewing sarcoma in general. Those radiographic features suggesting aggressive behavior were slightly more common than in the general Ewing population. Even so, neither the radiographic appearance nor the greatest dimension of the radiographic lesion can be shown to contribute significantly to prognostication in these patients. Apparently location of the lesion is important, since in the reported cases in the literature and in this series, lesions of the calcaneus fared poorly. The importance of this set of patients therefore relates to awareness and early recognition of an unusual appearance and location of Ewing sarcoma.     

Growing bone cysts in long-term hemodialysis

All patients with chronic renal failure undergoing hemodialysis for more than 10 years in the university hospitals of Leuven were selected for this study. The medical records and radiographs of these 21 patients were studied retrospectively. Skeletal surveys were examined for the presence and location of subchondral cysts. The predialysis films and the films taken after 5, 10, 15 and 20 years of dialysis were reviewed. Subchondral cysts that grew in size and number were found in the wrist, humeral head, hip, and patella. Accurate measurements were made of cysts in the wrist and compared with a control group. In the dialysis group, cystic involvement of the wrist was more common and the size and number of the cysts were larger. Soft tissue swelling was seen in the dialysis group but not in controls. Soft tissue swelling was assessed on shoulder radiographs by measuring the acromiohumeral distance (ACD) and in the knees by ultrasonic measurement of synovial thickness [25]. In 11 patients synovial or bone biopsies or aspirated synovial fluid were available. All these patients had swollen joints and multiple subchondral periarticular cysts. Amyloid deposition was found in ten of these patients, and this proved to be composed of B2 microglobulins in seven (Table 1).     

蜡流样肢骨硬化合并骨斑点症的影像学诊断 (附家族三代3例报告及文献复习)

目的 探讨蜡流样肢骨硬化合并骨斑点症的影像学诊断.方法 对一家族祖孙三代4人进行X线(4例)、MR(1例)检查并作文献复习.结果 发现4例中3例患病,病变均表现双手、足、膝、骨盆、肩、长骨骨骺及骨端,松质骨内呈对称性多发的大小不等的,圆形或椭圆形致密骨斑点病变的阴影,2例发现手、足、股骨皮质蜡流样肢骨硬化.MR扫描可见长T1短T2异常信号.结论 X线、MRI蜡流样肢骨硬化合并骨斑点症在X线、MRI具有典型的特征性征象,可明确本病诊断.     

Feet rolled over by cars: radiological and histological considerations from experiments

This study investigates the question of whether bone structures are injured when a vehicle rolls over a foot. A total of 15 detached feet from deceased persons who had donated their bodies to research were rolled over using a VW Passat station wagon. The feet were enclosed in various types of shoes. The front left tire of the vehicle, inflated to 1.8 bar and driven at walking speed, ran over the feet at a right angle to the long axis. The feet were dissected, and histological and radiological examinations were carried out. The only macroscopically well-defined abrasions of the epidermis were on the back of the foot in the area of contact with the tire and only where the foot had not been covered by a shoe. These abrasions were also well presented histologically. No injuries to the bone structures of the feet, in the form of incomplete fractures, corticalis interruptions or spongiosa compressions were ascertained, either radiologically or microradiologically.     

Bone metastases as the presenting manifestation of rhabdomyosarcoma in childhood

Rarely, rhabdomyosarcoma can present with bone pain and bone lesions on radiographs without evidence of a primary tumor. Of 428 children with biopsyproven rhabdomyosarcoma, four presented with radiographic evidence of bone metastases, but no primary tumor was found on subsequent evaluation. On radiographs, these metastases, located most commonly in the metaphyses of the extremities and in the spine, displayed a destructive or diffusely permeative pattern without sclerotic margins and mimicked the more common neuroblastoma. One patient also had diaphyseal cortical lytic metastases of the tibia. Radiographs defined metastases of the extremities better than the correlative bone scans. In the spine, on T2-weighted magnetic resonance (MR) images, metastases displayed high signal intensity which contrasted with the low-signal-intensity marrow in these pediatric patients. On histopathologic examination, metastatic rhabdomyosarcoma was composed of small cells of variable size, shape, and growth pattern similar to other round cell tumors. A positive desmin immunohistochemical test helped to establish the diagnosis. The radiologist, pathologist, and clinician should be aware of this unusual presentation of rhabdomyosarcoma so that suitable immunohistochemical tests are performed and appropriate chemotherapy given.