Tubular Bones Research Articles

Пересадка васкуляризованого епіфізарного трансплантату проксимального відділу малогомілкової кістки в дитини із саркомою Юінга (клінічний випадок)

Bone reconstruction after complete surgical resection of a tumor in children with a growing skeleton is a surgical problem. A special problem of periarticular reconstruction of the proximal femur and acetabulum is a special structure and function that provides movement and resistance, so transplantation of only vascularized autograft of the tibia is not enough. The use of free bone revascularized autografts makes it possible to replace significant defects of tubular bones. Clinical case. The case of transplantation of vascularized epiphyseal graft of the proximal tibia in a child diagnosed with Ewing's sarcoma of the proximal femur, after tumor resection in the Department of Reconstructive and Plastic Microsurgery National Children's Specialized Hospital MOZ of Ukraine «OHMATDYT». Conclusions. Transplantation of a vascularized epiphyseal graft of the proximal tibia with preserved articular surface after removal of the tumor together with the affected fragment of the femur, restores joint mobility and, most importantly, growth potential, which is undeniable in children. The treatment tactics proved to be effective and saved the child's life. The research was carried out in accordance with the principles of the Helsinki declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: vascularized epiphyseal graft transplantation, children, clinical case.

Classification of vascularized fibular flap hypertrophy based on X-ray evaluation.

The aim of this study was to analyze and classify hypertrophy seen in vascularized fibula flaps used for reconstruction of tubular bone defects. Thirty-three patients who underwent a vascularized fibula flap for the reconstruction of massive bone defects of the upper or lower extremity long bones were retrospectively reviewed and included in this study. There were 24 lower extremities (21 tibial and 3 femoral) and 9 upper extremities (4 humeral, 2 radial and 3 ulnar) reconstructions in this series. The mean age was 32.7 (range= 10- 59) years. The mean length of bony defect following initial debridement was 10.3 (range= 4-25) cm. The fibula was inserted as a single strut in 29 patients, and as a double barrel construct in 4 patients. The degree of fibular hypertrophy was calculated based on anteroposterior (AP) and lateral X-ray measurements of fibular flaps at an average postoperative period of 52 months. The difference in thickness between the initial and final x- ray measurements were expressed as percentage of hypertrophy. The variances seen in this period were defined and classified. When bony consolidation of the 33 cases were examined in detail, 4 different modes of flap hypertrophy were defined: type 0- absence of hypertrophy, type 1- limited hypertrophy, type 2- marked hypertrophy triggered by stress fracture, and type 3- massive hypertrophy enhanced by peripheral bone production. Fibular hypertrophy follows different modes based on vascularity of the flap, amount of stress imparted on the flap, site of reconstruction, and whether the periosteal sleeve is retained at the reconstruction site. Determination of these factors at the initial period may help the surgeons to predict the final hypertrophy that will be seen at the end of flap maturation Level of Evidence: Level IV, Therapeutic Study.

Correlation of Body Length and Certain Linear Parameters of the Tubular Bones of the Hand in Tajik Children and Adolescents

The intensifying integration processes between the Russian Federation and the Republic of Tajikistan update creation of databases and development of regression models that allow determining the body length by individual bones in representatives of both genders of this ethnic group.The aim of the study was to verify application of regression equations to determine the body length in Tajik children and adolescents, aged 6–17, according to the linear parameters of the short tubular bones of the hand.Material and methods. There were performed osteometric studies of 251 X-ray images of the right hand to determine the body length of the examined individuals. Linear regression analysis was carried out to identify dependence of the body length on linear parameters of the short tubular bones of the hand. The quality of the regression was assessed through the coefficient of determination R2 and significance by the F-criterion. There were considered coefficients of determination higher than 0.8.Results. It was found that for boys the linear parameter of the length of the tubular bones of the hand was the best to predict the body length. When applying regression equations to determine the body length according to the length of the hand rays in boys, it was determined that the best results were obtained when applying a model developed for the bones of the 2nd and 4th hand rays. For Tajik girls, regression models that included parameters of the length and width of the tubular bones of the hand were more reliable. More reliable results for identifying the body length of Tajik children were obtained when regression models were developed separately for individuals of different genders.Conclusion. The results obtained demonstrated that osteometric parameters of the short tubular bones of the hand can be used to assess the body length of Tajik children and adolescents of both genders.

State of the Art and New Concepts in Giant Cell Tumor of Bone: Imaging Features and Tumor Characteristics.

Simple SummaryThe 2020 World Health Organization classification of soft tissue and bone tumors classified the giant cell tumor of bone (GCTB) as an intermediate malignant tumor, with locally aggressive behavior and high recurrence rate. Imaging plays a pivotal role in the assessment of GCTB, and this review tries to summarize the main concepts about GCTB histopathogenesis and new biomarkers, describing those GCTB imaging findings which could possibly be explained by tumor molecular alterations. We have illustrated pre-operative imaging features related to prognosis and radiological findings for response evaluation after surgical treatment and denosumab administration. We have also reported the results described in literature regarding the role of radiomics in aiding GCTB diagnosis, predicting possible post-treatment recurrence and providing a quantitative assessment of the response to denosumab treatment.Giant cell tumor of bone (GCTB) is classified as an intermediate malignant tumor due to its locally aggressive behavior, burdened by high local recurrence rate. GCTB accounts for about 4–5% of all primary bone tumors and typically arises in the metaphysis and epiphyses of the long tubular bones. Mutation of gene H3F3A is at the basis of GCTB etiopathogenesis, and its immunohistochemical expression is a valuable method for practical diagnosis, even if new biomarkers have been identified for early diagnosis and for potential tumor recurrence prediction. In the era of computer-aided diagnosis, imaging plays a key role in the assessment of GCTB for surgical planning, patients’ prognosis prediction and post treatment evaluation. Cystic changes, penetrating irregular margins and adjacent soft tissue invasion on preoperative Magnetic Resonance Imaging (MRI) have been associated with a higher rate of local recurrence. Distance from the tumor edge to the articular surface and thickness of unaffected cortical bone around the tumor should be evaluated on Computed Tomography (CT) as related to local recurrence. Main features associated with local recurrence after curettage are bone resorption around the graft or cement, soft tissue mass formation and expansile destruction of bone. A denosumab positive response is represented by a peripherical well-defined osteosclerosis around the lesion and intralesional ossification. Radiomics has proved to offer a valuable contribution in aiding GCTB pre-operative diagnosis through clinical-radiomics models based on CT scans and multiparametric MR imaging, possibly guiding the choice of a patient-tailored treatment. Moreover, radiomics models based on texture analysis demonstrated to be a promising alternative solution for the assessment of GCTB response to denosumab both on conventional radiography and CT since the quantitative variation of some radiomics features after therapy has been correlated with tumor response, suggesting they might facilitate disease monitoring during post-denosumab surveillance.

APPROXIMATION OF GROWTH INDICATORS AND ANALYSIS OF INDIVIDUAL GROWTH CURVES BY LINEAR DIMENSIONS OF TUBULAR BONES IN CHICKENS OF MEAT PRODUCTION DIRECTION DURING POSTNATAL PERIOD OF ONTOGENESIS

Medical and biological sciences, including morphology, now require the introduction of the latest information technologies and mathematical methods to process the obtained and accumulated research results. To study the growth dynamics of body weight in domestic birds, classical growth models, Gompertz, were used for the purpose of quantitative description of the growth processes in biological objects, in particular for the growth and development of birds – Von Bertalanffy, Richards, and hyperbolastic models. The research material was tubular bones of the thoracic (humerus, ulna, and radius) and pelvic (femur, tibia, and tarsometatarsus) limbs in birds of meat production (broiler chickens and laying hens from the parent broiler flock of Cobb-500 strain) of different age groups during the postnatal period of ontogenesis. An appropriate regression analysis of experimental data based on known growth models was performed to solve the goal of obtaining growth curves and identifying special points (extremes, inflections, etc.), to build a picture of the overall development of the body as a whole and individual bones of the extremities. The most biologically suitable growth models for describing the growth dynamics of the body as a whole and individual studied bones were determined. The absence of a unified growth model of linear parameters of different tubular bones in meat producing chickens during the postnatal period of ontogenesis was established. This implies the need for a clear selection of growth models taking into account age, species, breed, keeping and feeding conditions of domestic birds. The growth model that best describes the body weight dynamics of broiler chickens is the hyperbolastic growth model of the H3 type, and in laying hens from the parent broiler flock – the Brody growth model.

Clinical expert case with a combination of the Saul–Wilson and Rubinstein–Taybi syndromes

SaulWilson syndrome, like RubinsteinTaybi syndrome, is classified as a rare genetic disease according to modern classification. So, to date, there are descriptions of 16 patients from different countries in literary sources.
 The SaulWilson syndrome is characterized by a combination of nanism, pathology of long tubular bones, the spine and the organ of vision, typical dysmorphic facial features and radiological data; RubinsteinTaybi syndrome has various skeletal defects (low height, dilation of the phalanges of the fingers, polydactyly on the legs, deformities of the face and skull, high arched palate, changes in the shape and position of the auricles, dilated phalanges of the fingers, lordosis, kyphosis, scoliosis), strabismus, anomalies refraction, disorders of internal organs, disorders of intellectual and physical development.
 The article presents a complex clinical and expert case with a combination of two genetic diseases ― SaulWilson and RubinsteinTaybi syndromes, typical manifestations of which were visible craniofacial dysmorphia, changes in the musculoskeletal system in the form of progressive bilateral coxarthrosis, instability of the thoracic spine, deformities of the feet, accompanied by pronounced disorders of neuromuscular, skeletal and statodynamic functions. The multi-system lesion of the body caused difficulties for specialists during the medical and social examination. These nosological forms are absent in the current classifications and criteria for establishing disability, and therefore the quantitative assessment of persistent functional disorders was carried out individually ― according to the degree of severity of impaired functions, their combination and influence on each other.
 The use of modern diagnostic methods, including genetic studies, in hereditary diseases allows to establish a diagnosis at an early stage, to determine the tactics of managing children in order to ensure timely pathogenetic treatment, including surgical, prevention of the development of severe complications, functional disorders. The successes of medicine can not only significantly improve the quality of life of this category of children, but also increase their life expectancy. Since in most cases hereditary diseases involving the musculoskeletal system are accompanied by persistent disorders of the bodys functions and lead to disability, classifications and criteria for establishing disability should include a quantitative assessment of the degree of impaired functions in congenital anomalies and hereditary diseases.

Bone ice skates in the medieval Novgorod (based on archaeological research of the Institute of Archaeology RAS in 2018–2019)

The article summarizes available information on bone ice skates and presents the results of research and classification of the collection of skates of the 11th–15th centuries found by the Novgorod Expedition of the Institute of Archaeology RAS. Medieval ice skates are small bone runners made from the tubular bones of large domestic animals. All objects show traces of the specific processing of original bones: cut off epiphyses and a flattened plantar side (sliding surface). The amount of accumulated archaeological material, instrumental study of wear pattern on the working surface, experiments in the use and manufacture of skates, numerous ethnographic parallels in the use of bone skates in a number of countries almost up to the present day, as well as the fact of skating on bone shoes recorded in a 12th century source, make it safe to say that, in functional terms, ice skating was one of the forms of winter pastime and was a part of the Novgorod dwellers’ everyday life.

Clinical Images: Spotted bone: Incidental detection of a rare genetic disease.

The patient, a 27-year-old woman with a history of Hashimoto thyroiditis, presented to the rheumatology clinic for upper and lower extremity swelling. A physical examination demonstrated hand swelling and flesh-colored lichenified papules on her sacrum, neck, and ankles. Results of laboratory workup showed weakly positive antinuclear antibodies (1:320) with a nuclear fine-speckled pattern but otherwise no autoantibodies. A skin biopsy specimen demonstrated thickened dermal collagen consistent with connective tissue nevus. We obtained anterior–posterior (AP), axial, and Grashey views on radiographs of both shoulders (A); AP, lateral, and oblique radiographs of both feet (B); and posterior–anterior, lateral, and oblique radiographs of both hands (C). Each view demonstrated diffusely distributed small sclerotic densities overlying the normal osseous structures consistent with scattered enostoses. The differential diagnosis included sclerosing bony dysplasias (sporadic or inherited), sclerotic metastases, osteomas, and osteosarcomas (1). Genetic testing revealed a heterozygous mutation in LEMD3. Her constellation of findings was consistent with Buschke-Ollendorff syndrome, an autosomal dominant condition in which diagnostic features include osteopoikilosis, connective tissue nevi, an LEMD3 mutation, and a family history (2). Loss-of-function mutations in LEMD3, a gene involved in bone morphogenic protein signaling, have been previously described in cases of Buschke-Ollendorff syndrome, although the exact relationship is yet unknown (3). Imaging features that may help differentiate osteopoikilosis from other malignant disorders include its preponderance for the epiphyseal regions of short tubular bones, lesion size less than 1 cm, homogenously radiodense lesions with spiculated margins, and lack of uptake on bone scintigraphy (1, 4). Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

Analysis of errors and prevention of complications after perosseous osteosynthesis

Distal fractures rank a special place among all fractures of the long tubular bones of the extremities. Complications are an inevitable companion of any surgical intervention, as well as an integral part of the work of every surgeon. Objective: To analyze the outcomes of treatment of distal fractures of the long bones of the extremities, to identify the causes of errors and the frequency of complications after perosseous osteosynthesis, and to outline ways for their prevention. Patients and methods: 1723 patients with distal fractures of the long bones of the extremities and further perosseous osteosynthesis have been under study. Distal fractures of the humerus were registered in 647 patients, those of the femur – in 392, and the bones of the lower leg were damaged in 684. Results: The most common complication of perosseous osteosynthesis is inflammation of the soft tissues near the pins - 21.8%. Non-infectious complications (secondary displacement of fragments, contractures, impaired innervation and trophism) were observed in 18.2% of cases. Residual effects of neuritis of the radial and peroneal nerves amounted to 0.1%. In the long-term period there were 2.1% of the knee and elbow joints contractures. Conclusions: Complications observed in the process of treatment of distal fractures of long bones of the extremities with the use of perosseous osteosynthesis are not inherent this method of treatment, since they are the result of medical errors in the choice of indications for its use, violations of osteosynthesis’ technique and postoperative management of patients, mainly in the period of minimally invasive technology of surgical intervention development.

Academician Gavriil Abramovich Ilizarov (1921–1992) (on the 100th anniversary of the birth)

Professor Gavriil Abramovich Ilizarov was born on June 15, 1921 in the town of Bialowiez in the Bialystok Voivodeship of the Polish Republic. Gavriil was able to go to school only at the age of 11, but his level of education allowed him to pass the exams for the elementary grades, and he was enrolled immediately in the fourth grade. He graduated from the eight-year school with excellent marks and in 1938 continued his studies at the medical faculty in the city of Buinaksk, Dagestan ASSR. In 1939, Gavriil Abramovich entered the Crimean State Medical Institute, after graduating from which in 1944 he worked as a doctor in Polovinskaya and then Kosulinskaya district hospitals of the Kurgan region. At this time, Ilizarov came up with a brilliant idea for the treatment of complex fractures of long tubular bones with the fixation of their fragments with a special metal controlled device instead of blind and static plaster immobilization. In 1955, G. A. Ilizarov was appointed head of the Traumatological and Orthopedic department of the Kurgan Regional Hospital for the Disabled of the Great Patriotic War, where he began to apply his invention. The first official scientific recognition of G. A. Ilizarov received his dissertation «Transosseous compression osteosynthesis by the author’s apparatus (experimental and clinical study)» in September 1968 in Perm. In 1966, on the basis of the 2nd city hospital of Kurgan, the problem laboratory of the Sverdlovsk Research Institute of Traumatology and Orthopedics was organized, the head of which was Gavriil Abramovich, and in 1969 the laboratory was transformed into a branch of the Leningrad Research Institute of Traumatology and Orthopedics named after R. L. Vreden and its director was appointed G. A. Ilizarov. Since 1982, the triumphant introduction of the Ilizarov method into the practice of leading foreign countries began, and the press gave the author the enthusiastic title of «Michelangelo in orthopedics». Gavriil Abramovich successfully engaged in public activities, was elected deputy of the district and regional Councils of Workers‘ Deputies, deputy of the Supreme Soviet of the RSFSR, People’s Deputy of the USSR. For his great achievements in medical science and outstanding services to the national and world health care, G. A. Ilizarov was awarded the highest state awards, academic and honorary titles of the USSR and many foreign countries. Academician Gavriil Abramovich Ilizarov died suddenly of heart failure on July 24, 1992 in Kurgan and was buried in the central alley of the new Riabkovskое cemetery in Kurgan.

Giant Cell Tumors of Bone in Patients Aged 18 Years Old or Younger: Imaging Features and Tumor Characteristics.

ObjectiveThe majority of giant cell tumors of bone (GCTB) occur in adult patients, especially between the ages of 20 and 40. This study aims to investigate the imaging features of GCTBs in pediatric patients and compare their characteristics with adult cases.MethodsFifty-seven cases of patients aged 18 years old or younger were retrospectively analyzed, accounting for 12.8% of GCTBs in the First Affiliated Hospital of Zhengzhou University from 2001 to 2019. One hundred twenty-six adult patients (19 years of age and older) with GCTB occurring in long tubular bones were also included in this study. The following clinical information was identified from the medical records: age, sex, and follow-up data. Imaging features were reviewed by two musculoskeletal radiologists. Patient characteristics and imaging features between the two groups were compared.ResultsA total of 57 patients (32 females, 25 males) were included in the study. The patients’ ages ranged from 9 to 18 (median = 17 y). The majority of tumors occurred in tubular bones (n = 38, 66.7%) and the pelvis (n = 8, 14.0%). Imaging features were identified in GCTB cases occurring in the long tubular bones. Compared with adult GCTB patients, pediatric GCTB patients had a larger superior–inferior (SI) diameter (P = 0.005) and smaller left-to-right diameter/SI diameter ratio (P = 0.001). Epiphyseal involvement was relatively less common in pediatric patients with GCTBs than in adult patients (P = 0.009). The median age of patients without epiphyseal involvement was lower than the median age of patients with epiphyseal involvement (11 vs 17 y).ConclusionGCTB in the pediatric age group is rare. This study has found that, in pediatric patients with GCTBs, the epiphysis is relatively less involved, and the tumor is more likely to grow longitudinally. These findings are helpful in the diagnosis of GCTBs in the pediatric population.

Морфологічні аспекти репаративної регенерації кісткової тканини в умовах впливу протипухлинної хіміотерапії

The occurrence of fractures in the development of cancer in the body is due to changes in bone metabolism in the form of osteoporosis and metastatic bone damage. Their appearance leads to the postponement or cessation of treatment of cancer, which affects the life expectancy of such patients and the chances of recovery. Antitumor chemotherapy, as one of the main methods of cancer treatment, is prescribed for long-term courses and affects the healing of fractures. However, according to the literature, the effect of antitumor chemotherapeutics on reparative regeneration is poorly understood today. The purpose of the work is to study the morphological features of reparative osteogenesis under the influence of antitumor chemotherapy. Materials and methods. The study was performed on 96 white laboratory male rats 7 months of age weighing 230±10 g. All animals had a perforated defect with a diameter of 2 mm spherical cutter to the bone marrow canal in the middle third of the femoral shaft. Animals were divided into control (n = 24, without chemotherapy) and three experimental groups (I, II, III, n = 72), which after injury and every 21 days of the study were administered intraperitoneal anticancer chemotherapeutics: I (n = 24) – doxorubicin (60 mg / m²), II (n = 24) – 5-fluorouracil (600 mg / m²), III (n = 24) – methotrexate (40 mg / m²). On the 15th, 30th, 45th, 60th days after injury, the animals were removed from the experiment, followed by removal of the injured long tubular bones. Histological preparations stained with hematoxylin-eosin, followed by their morphometry, scanning electron microscopy with the method of X-ray energy dispersion spectroscopy, immunohistochemical examination were performed. Results and discussion. Antitumor chemotherapeutics causes delayed callus formation, which is manifested by an increase in the area of connective and reticulofibrous bone tissue in the regenerate, along with the slow formation of lamellar bone tissue. Chemotherapy leads to disorders of phosphorus-calcium metabolism both in the regenerate and in the maternal bone in the form of reducing the intensity of mineralization of the newly formed bone matrix and slowing down the remodeling activity of the maternal bone. Chemotherapy is accompanied by an increase in the expression of the bone resorption marker cathepsin K and a decrease in the expression of the osteopontin bone marker, which indicates a delay in the formation of regenerate in the area of injury and a decrease in the rate of reparative regeneration. Conclusion. The most pronounced delay in the processes of remodeling of bone regenerate was found with the use of doxorubicin and methotrexate, while 5-fluorouracil showed less inhibitory effect on these processes

Growth Layers and Its Complex Structure in a Common Species Under Uncommon Conditions: <i>Pelophylax ridibundus</i> in the Talysh Mountains

In the Talysh Mountains, the marsh frog, Pelophylax ridibundus (Pallas, 1771), increases its distribution due to human activity. In the mountain forest belt, frogs inhabit flowing ponds with cold spring water. These reservoirs are characterized by a stable temperature regime: the water in them is kept at the level of 10 – 12°C in winter and does not fall below 6°C, and in summer does not rise above 18°C. Probably, the cold flow water of ponds in the mountain-forest belt of Talysh can cause some features of growth and maturation for P. ridibundus. The aim of our work was to study the features of the structure of growth layers including the variation in degree of expression of lines of arrested growth (LAGs) of the marsh frogs in ponds with cold flow water. Frogs were collected in the upper part of the Tangeru River gorge in Sym village of Astara District of Azerbaijan (480 m a.s.l.) in August 2018. In total, we studied skeletochronologically 8 females and 9 males. In contrast to the results of the tubular bones studies in the marsh frog presented in earlier works, the studied individuals are characterized by a more complex and diverse structure in the cross section of shin bones. All the revealed diversity of this structure can be divided into three groups. To the first group are relatively rare cases in which there are growth layers with wintering LAGs and additional growth layers but without additional LAGs. The second group includes more frequent cases in which there are growth layers not only with wintering LAGs but with additional LAGs differing discretely from wintering LAGs. The third group includes the rarest cases where there are growth layers with wintering LAGs and with additional LAGs without discrete difference from wintering LAGs. The revealed high variable structure of growth layers and distinctiveness of LAGs in P. ridibundus can be explained by unusual temperature regime in habitat of studied population and the existence of several activity period during a year. The most continuous period of low temperatures (February and March) corresponds to formation hibernation LAGs and dark rings in growth layers. Besides, period from early December to late January may be so cold that frog growth retard sufficiently or stop completely. The formation of more dark parts of growth layers or even several additional LAGs can correspond to this period.

The problem of dating fractures: A retrospective observational study of radiologic features of fracture healing in adults

Accurate dating of bone fractures can be crucial in the context of forensic trauma analysis since it may provide essential information for the corroboration or contradiction of statements by victims or suspects in cases of domestic abuse or torture. The different stages of bone healing have been well described in the existing literature, and some previous studies attempted to define related timelines particularly in the paediatric population. However, the bone healing process can be very variable and despite the importance of the topic in the forensic field, so far little is known about the radiological appearance of bone fractures at different healing stages, and how this correlates with time, especially in an adult population.The aim of this retrospective observational study was to describe the temporal aspect of pre-defined healing stages as they appear on radiographs of tubular bone fractures in adults, and explore the effect of potential cofounding variables. A dynamic nomogram was developed as a user-friendly tool to be eventually applied in clinical or medico-legal settings. This study showed that the posttraumatic time interval (PTTI) increased progressively with the pre-defined healing stages. However, confounding factors, such as patients’ age, sex, and location of the fracture need to be accounted for in the final estimation model.Further studies are needed to explore more potentially confounding variables to refine the presented outcomes. Better knowledge of the effect of different confounding variables in the dating of fracture healing will contribute to greater accuracy of PTTI estimation of bone fractures in adults.

308 The Effect Of COVID-19 On Hand Trauma Management: A Single-Centre Correlation Study, Inter-Rater Agreement and Functional Outcomes

IntroductionManagement of hand trauma has evolved to incorporate assessment, treatment and rehabilitation of patients in a ‘one-stop’ clinic on initial presentation. Our aim was to evaluate the effect of COVID-19 on hand trauma management using inter-rater agreement between hand surgeons.MethodPatients with hand fractures were consecutively assessed from March to May 2020, with atleast 6-month follow-up outcomes. Two experienced hand surgeons blinded to management and outcomes independently reviewed radiographic images and clinical history to provide their opinion on optimal treatment. Weighted-kappa analysis was performed for concordance and inter-rater agreement between the two surgeons and actual management.Results268 patients were identified, 82 had fractures involving tubular bones of the hand. 62 were male and 20, female. Mean age was 40.3 (SD 19.7). Mechanism of injury was secondary to home-related injuries (34%) and falls (28%). Fractures involving the metacarpals (n = 24) and distal phalanx (n = 20) were most commonly seen. 35 patients underwent surgery whereas 47 were managed conservatively. Overall agreement between actual management and both consultant-1 and consultant-2 was moderate (κ = 0.55, p < 0.0001 and κ = 0.63, p < 0.0001, respectively). Subgroup analysis showed a weak agreement between actual management of metacarpal fractures and both consultant-1 and consultant-2 (κ = 0.22, p = 0.29 and κ = 0.47, p = 0.02, respectively). Inter-rater agreement was substantial for management of metacarpal fractures (κ = 0.73, p < 0.0001) whilst functional outcomes were comparable as reported by patients.ConclusionsOur study has shown that overall management of hand fractures remained optimized throughout the pandemic. However, lack of concordance was observed in the management of metacarpals through substantial inter-rater agreement but overall poor agreement.

Safety of Tenofovir Alafenamide in People With HIV Who Experienced Proximal Renal Tubulopathy on Tenofovir Disoproxil Fumarate

Proximal renal tubulopathy (PRT) is an infrequent complication of tenofovir disoproxil fumarate (TDF). It remains to be established whether tenofovir alafenamide (TAF) can be safely administered to individuals who experienced PRT on TDF. Individuals with a history of TDF-associated PRT and current estimated glomerular filtration rate (eGFR) over 30 mL/min/1.73 m2 initiated TAF and were followed for 96 weeks. The primary outcome of interest was recurrent PRT. Secondary outcomes were changes in kidney biomarkers, bone biomarkers, and bone mineral density (BMD). Data were analyzed using multilevel mixed-effects linear regression models. The trial was registered under EudraCT 2016-003345-29. All 31 participants [median age 55 (inter-quartile range 51, 60) years, 97% men, 87% White ethnicity] remained on TAF at week 96, and none developed glycosuria or recurrent PRT. Participants experienced small declines in eGFR-creatinine [-1.9 (95% confidence interval: -3.5 to -0.3) mL/min/1.73 m2/yr; P = 0.024], but not in eGFR-cystatin C [-0.9 (-2.1 to 0.4) mL/min/1.73 m2/yr; P = 0.16]. Ten (32%) and 5 (16%) participants experienced rapid (>5 mL/min/1.73 m2/yr) decline in eGFR-creatinine and eGFR-cystatin C. No significant change in other kidney biomarkers, bone turnover, or BMD was observed (P > 0.2). In individuals with a history of PRT on TDF, 96 weeks of TAF was not associated with recurrent PRT or adverse effects on renal tubular function, bone turnover, or BMD. These data suggest that TAF is a treatment option for this vulnerable population.

A CASE REPORT OF HYPERTROPHIC OSTEOARTHROPATHY LEADING TO DIAGNOSIS OF PULMONARY EPITHELIOID HEMANGIOENDOTHELIOMA

TOPIC: Lung Cancer TYPE: Medical Student/Resident Case Reports INTRODUCTION: Hypertrophic Osteoarthropathy (HOA) is a condition characterized by abnormal proliferation of skin and osseous structures of extremities. Periostitis is the imaging hallmark of HOA and most commonly manifests symmetrically along the shaft of tubular bones. Incidence of HOA in patients with intrathoracic malignancy is 5-10%. CASE PRESENTATION: A 49-year-old male presented to his primary care physician for bilateral knee pain with associated swelling. After failing conservative management, bilateral knee x-rays were performed which revealed a thin periosteal reaction in both femoral shafts, prepatellar soft tissue swelling and small bilateral joint effusions.The radiologist recommended a chest x-ray for further evaluation, which showed subtle soft tissue fullness at the right lung base. Chest CT was then performed revealing a 4.2 x 2.2 x 4.7 cm spiculated soft tissue lung mass of the right lower lobe with extension into the mediastinum and right hilar, paratracheal and subcarinal lymph nodes.The patient underwent bronchoscopy with FNA of the right lower lobe mass and station 7 and 11 lymph nodes. Highly atypical cells suspicious for malignancy were noted, but analysis was limited by scant material. IR guided biopsy was subsequently performed revealing patchy proliferation of epithelioid cells with ample eosinophilic cytoplasm, occasional cytoplasmic vacuoles and necrosis. Immunohistochemically, the tumor cells stained positive for ERG, CD31, and CD24. The cytomorphologic and immunohistochemical features support the diagnosis of epithelioid hemangioendothelioma (EHE). DISCUSSION: This case illustrates EHE's heterogeneous presentations. The primary lesion of EHE has been documented in numerous locations throughout the body, but most commonly arises in the liver, lungs, and bones. HOA as the presenting sign in pulmonary EHE is not well documented in the literature and periosteal reaction is considered rare. Digital clubbing was not present in this case and the only known location of periosteal reaction was the femoral shafts. While the mechanism of EHE is not well understood, it is believed that angiogenic stimulators, such as vascular endothelial growth factor (VEGF), may act as promoters of endothelial cell proliferation.The proposed pathogenesis of HOA has been linked to hypersecretion of vasoactive agents such as VEGF, platelet derived growth factor (PDGF), and prostaglandin E2 (PGE2). The proposed pathogenesis of secondary HOA has been linked to hypersecretion of vasoactive agents such as VEGF, PDGF, and PGE2. These agents are released both systemically in response to hypoxemia and by the tumor itself. CONCLUSIONS: Pulmonary Epithelioid Hemangioendothelioma can also present with Hypertrophic Osteoarthropathy.Bone X- ray can lead to the diagnosis of Hypertrophic Osteoarthropathy if careful evaluation is done. Periostitis is frequently a sign of pulmonary and systemic pathology. REFERENCE #1: Chakraborty, Rebanta K. "Secondary Hypertrophic Osteoarthropathy." StatPearls [Internet]., U.S. National Library of Medicine, 1 Mar. 2021, www.ncbi.nlm.nih.gov/books/NBK513342/. REFERENCE #2: Sardaro, Angela, et al. "Epithelioid Hemangioendothelioma: an Overview and Update on a Rare Vascular Tumor." Oncology Reviews, PAGEPress Publications, Pavia, Italy, 13 Oct. 2014, www.ncbi.nlm.nih.gov/pmc/articles/PMC4419652/. REFERENCE #3: Silveira LH;Martínez-Lavín M;Pineda C;Fonseca MC;Navarro C;Nava A; "Vascular Endothelial Growth Factor and Hypertrophic Osteoarthropathy." Clinical and Experimental Rheumatology, U.S. National Library of Medicine, pubmed.ncbi.nlm.nih.gov/10728444/. DISCLOSURES: No relevant relationships by Julia Lawinger, source=Web Response No relevant relationships by Raul Mendoza-Ayala, source=Web Response

VP16.14: Diagnosis of short rib polydactyly syndrome type IV (Beemer‐Langer syndrome): a case report

Short rib-polydactyly syndrome (SRPS) is a rare congenital autosomal recessive inherited disease classified in four subtypes. Prenatal ultrasonography findings and postnatal examinations may help classify in one of four subtypes. We present a case of an infant who presented multiple congenital anomalies and the postnatal radiography helped to classify the SRPS as type IV (Beemer-Langer). A 23-year-old was referred for prenatal ultrasound which showed cystic hygroma, small omphalocele and choledocal cyst. Chromosomal analysis revealed a normal male karyotype. Morphological scan revealed small thorax, short ribs, micromelic shortening of limbs with the tibia slightly longer than the fibula, unilateral clubfoot, hidrocephalus and renal hiperechogenicity. Magnetic Resonance performed at 35 weeks revealed micrognathia and mesenteric lymphangioma. The infant delivered at 38 weeks of gestation and died within 60 minutes due to respiratory failure. Postmortem radiography showed marked shortening of all long bones, strikingly narrow thorax with short and horizontal ribs, highly placed clavicles, small scapulae, small ilia and the tibia was longer then the fibula. Identification of the SRPS subtype can be difficult due to overlap of clinical and pathologic findings. This case may help understanding Beemer-Langer type's phenotypic features such as micrognathia, cleft lip, hydrops, hydrocephalus and omphalocele. The most common radiological findings include narrow thorax with short ribs, short tubular bones, highly placed clavicle, small scapulae and ilia and bowing of radius and ulna with the tibia being longer than the fibula. The prognosis of Beemer-Langer syndrome is poor as infants usually die shortly after birth because of respiratory failure secondary to marked pulmonary hypoplasia.

Natural history of facial and skeletal features from neonatal period to adulthood in a 3M syndrome cohort with biallelic CUL7 or OBSL1 variants

3M syndrome is characterized by severe pre- and post-natal growth restriction, typical face, slender tubular bones, tall vertebral bodies, prominent heels and normal intelligence. It is caused by biallelic variants of CUL7, OBSL1 and, more rarely, CCDC8. The aim of this study is to evaluate facial and skeletal findings in 3M patients from neonatal period to adulthood. A total of 19 patients with a median age of diagnosis of 9.2 months were included in this study and were followed for two to 20 years. CUL7 and OBSL1 variants were found in 57.9% and 42.1% of patients, respectively, five of which are novel. Most of patients had triangular face, frontal bossing, short fleshy nose, full fleshy lower lip, transverse groove of rib cage, hyperlordosis and prominent heels. Three new early-diagnostic signs were observed in infants; two were infraorbital swelling of the lower lid and facial infantile hemangioma, both of which became less pronounced with aging. The third was the central tubercle of the upper lip that became more prominent with in time. While slender long bones did not change with aging, the tall vertebral bodies became more prominent radiologically. The mean birth length in patients was −4.3 SDS. Eight patients reached a mean final height of −4.9 SDS. Despite described growth hormone (GH) insensitivity in 3M syndrome, 12 patients either with GH deficiency or with normal GH levels were treated with GH; seven patients responded with an increase in height SDS. This study not only provided early diagnostic signs of the syndrome, but also presented important follow-up findings.

New radiation techniques in controlling bone regeneration in fractures

Objective: comparative study of the possibilities of radiation methods in control of bone regeneration in fractures.Materials and methods. A study of bone calluse formation was conducted in 116 patients with broken tubular bones of the upper and lower limbs from the first day of the fracture to the complete consolidation of the fractures. In X-rays and CT determined the mineral density of bone calluses, with ultrasonic elastography shear wave and compression elastography determined the rigidity of bone calluses in kPa.Results. X-ray bone corn was determined at the end of the second – the beginning of the third stage of bone calluse formation. On CT bone corn density (in the HU), its structure and the condition of bone breaks were determined in some patients in all stages of bone regenerate formation. In ultrasound examination in the multiparametric mode studied the rigidity, structure and vascularization of bone calluses, the correct comparison of bone breaks from the first day of the fracture to their complete fusion.Conclusions. Ultrasonic studies in multiparametric mode can be used to control bone regeneration in fractures. Ultrasonic elastography shear wave, in determining the rigidity of bone calluse, in sensitivity and specificity exceeds the indicators of ultrasonic compression elastography at all stages of bone regenerate formation.