Long Bones Of Lower Extremities Research Articles

Erdheim–Chester disease: An elusive diagnosis in a 50‐year‐old Ethiopian man presenting with diffuse sclerotic bone lesion

Key Clinical MessageDiagnosis of Erdheim–Chester disease (ECD) requires the clinician to be familiar with its various manifestations, classic radiologic and histologic features. This case highlights the significance of considering ECD in any patient presenting with bone pain and symmetric osteosclerosis of long bones of extremities to allow for early diagnosis and treatment.AbstractErdheim–Chester disease (ECD) is a rare non‐Langerhans histiocytic disorder with diverse clinical manifestations, ranging from indolent, localized presentation to life‐threatening, multi‐systemic disease. Delayed or erroneous diagnosis is common. The presence of classic radiographic finding along with foamy histiocytes that is positive for CD68 but negative for CD1a on histologic examination establishes the diagnosis. We report a second case of ECD from Ethiopia. A 50‐year‐old Ethiopian man presented with a 13‐year history of bilateral lower leg bone pain, cold intolerance, somnolence, constipation, impotence, decreased libido, and secondary infertility. The diagnosis was suspected when skeletal X‐ray revealed bilateral symmetric sclerosis of metadiaphysis of femur, tibia, and humerus. The demonstration of foamy histiocytes that were positive for CD68 but negative for CD1a on histologic examination with immunohistochemical staining confirmed the diagnosis. Evaluation for the extent of the disease revealed coated aorta sign, hairy kidney sign, and cystic lesion with ground glass opacity of lung, primary hypothyroidism, and hypergonadotropic hypogonadism. ECD is rare histiocytic neoplasm with wide range of clinical features which often delay the diagnosis. Clinician should be mindful of the various presentations and the classic radiographic and histologic features of ECD. This case highlights the significance of entertaining ECD in any patient presenting with lower leg bone pain and symmetric osteosclerosis of long bones of lower extremities to allow for early diagnosis and treatment.

RECOVERY OF STABILIZING MUSCLES THAT PROVIDE A VERTICAL POSITION OF THE TRUNK IN PATIENTS WITH POST-TRAUMATIC DEFORMITIES OF THE LONG BONES OF THE LOWER LIMBS IN THE DISTANT PERIOD

Rehabilitation treatment in the case of posttraumatic deformities of long bones of lower extremities (PDLBLE) in the distant period after trauma is aimed at the restoration of support ability of lower extremity while walking and standing. Objective. To work out the set of the exercises targeted on the restoration of muscle-stabilizer function in patients with (PDLBLE) Methods. Research group — 40 patients who received closed injuries of the long bones of the lower limbs, initial trauma occured 6–12 months ago. Among them were 21 women. (52.5 % of persons; age 27–73 years) and 19 men (47.5 % of people, age 29–77 years). The comparison group included 40 healthy volunteers, among which there were 12 women (30 % of persons; age 24–50 years) and 28 men (70 % of people, age 31–49 years). The assessment was carried out before and after rehabilitation for 2 weeks. Parameters evaluated: pain scores on VAS, the results of restoring the support ability using the scale of Tyazhelov O. A. Results. The results of observations are given, where in addition to descriptive statistics (minimum, maximum, average meanings), parameters of support ability and VAS, in patients and volunteers before and after treatment, cumulative group indicators (CG) were calculated for each group at the beginning (CG0) and 2 weeks after rehabilitation (CG1); the «rehabilitation» coefficient as a ratio of group indicators before and after treatment was calculated as well. Cumulative group indicator after rehabilitation (CG1) increased to 2 995 points, which indicates an increase in support ability. Cumulative group VAS score after rehabilitation (CG1) decreased to 116 points from 200 points before rehabilitation — reduction of pain in patients. After rehabilitation, volunteers showed a slight increase in the cumulatory group indicator (CG1) to 3 917 points. Conclusions. The system of rehabilitation of patients with PDLBLE in the remote period after injuries proved its effectiveness in restoring muscle-stabilizers vertical position of the trunk, pelvis, lower limbs and pain reduction.

A Case of Intraosseous Sphenoid Lipoma Presenting as Cluster Headache

Intraosseous lipoma is an extremely rare benign primary bone tumor, most commonly found in the long bones of lower extremities and calcaneus. Very few cases have been reported in the skull base, and mostly noninvasive follow-up is proposed due to its indolent growth and the low possibility of malignant transforming. Surgical biopsy or treatment is needed only when the patient’s symptom is correlated with the location of the lesion. We report a rare case of symptomatic intraosseous sphenoid lipoma which presented ipsilateral retroorbital pain accompanied by watering eye and runny nose on the same side. Surgical excision led to immediate resolution of the symptoms and the tumor was pathologically confirmed. This case highlights the radiographic differential diagnosis based on the review of literature.

OSTEOMA IN MANDIBULAR SUBCONDYLAR REGION: CASE REPORT

Osteoid osteoma is a benign bone-forming tumor, first described by Bergstand in 1930. It can involve every bone but is more common in long bones of lower extremities and rarely occurs in the jaw. A 14-year-old male patient complained of increased volume in the left preauricular region and had a history of condylectomy surgery in the region 3 months before the first evaluation. He presented a computed tomography report prior to the surgical procedure compatible with a nodular lesion with an exophytic ossified aspect and without cleavage plans, with the mandibular condylar process, occupying a parotid region, of approximately 21 × 16 mm. The removed lesion was submitted to histopathologic analysis with a diagnosis of a mandibular tumor compatible with osteoma. The patient was followed for 12 months, with periodic imaging exams, which revealed a spontaneous bone neoformation of the condylar region, without clinical and imaginologic signs of lesion recurrence.

The concept of postural pattern formation in patients with posttraumatic diaphyseal deformities of the long bones of lower extremities

Objective. To invent the concept of new postural pattern formation in the consequence of severe injury of lower extremities associated with the development of «syndrome of posttraumatic diaphyseal deformities of lower extremities». Methods. The classic model of the development of transformation «health–disease», that was initially worked out in 1960th years, has been used to build up a concept of the returning of patient from trauma to relative health. The idea of this approach was to make analogy of regulatory process under the conditions of disease with emergency control insophisticated technical systems of automatic control. The results of instrumental investigation of patients and mathematic modelling was used. Results. In the case of the development of «syndrome of posttraumatic diaphyseal deformities of lower extremities» in addition to decreased muscular strength, joints stiffness and limb lengthening the complex of following complications occurs: anatomical disarrangements in adjusted joints and muscular imbalance. If the deformity development is accompanied by the saved weightbearing function of the limb a compensatory mechanism in length of time and in a process of walking capacity restitution creates new «body scheme» and stereotypes of walking and standing. Inherent program of movement substitutes with acquired one. If the deformity occurs just on one lower extremity the anatomical disarrangements of the whole skeletal system take place: the tiltof pelvis and spine, hip abduction/adduction contracture, varus/valgus deformity in knee joint, the disturbance of weightbearing capacity of feet with different derangements in ankle and subtalar joints (including varus/valgus deformities). The severity of these secondary changes depends upon the value of deformity, general and psychological status of patient. Outcomes. Invented concept of new postural pattern formation in consequence of severe injuryof lower extremities associated with the development of «syndrome of posttraumatic diaphyseal deformities of lower extremities» allows to assess and understand a locomotor behavior of patient and take it into consideration in treatment planning.

The clinical significance of posttraumatic malunions of lower extremities long bones

The treatment of patients with long bones fractures, despite the development and improvement of treatment methods and fixing devices in certain cases can lead to unwanted results such as a formation of posttraumatic deformity — malunion. Objrctive. To provide modern approach regarding clinical significance and necessity of correction of secondary changes in moving segment that appeared as a result of formation of lower extremity malu­nion. Methods. The results of treatment of 196 patients were analyzed (89 men, 107 women; age from 19 to 76 years). All the patients had the malunion of the long bones of lower extremities and have been treated from 2004 to 2019 years. Analysis of special literature and own experience was performed to identify the effects of malunions. Results. It was shown that formed malunion lead to tangible shortening of injured extremity, joints overloading and muscle function violation. Malunions are compensated by the means of changing in movement arch and gait pattern. While it is noted that unphysiological load on joints, that predispose the development of degenerative changes, and muscle fatigue and pain are the results of muscle work to support inadequate position of the joints. Malunions on the level of femur and tibia became clinically significant on condition of severe restriction of extremi­ty function, thus its restoration is the goal of treatment. Diffe­rent cases of malunion development were considered: varus, valgus, antecurvatio, recurvatio, translation, torsion malalignment. Clinical examples are given. Conclusions. Clinical significance of different malunions depend upon their localization, presence and severity of secondary changes, status of injured extremity and physical condition of the patient, his or her individual demands. Secondary changes can play the role of compensation and spread on injured extremity, sacro-iliac joints, lumbar spine, contralate­ral extremity. The assessment of secondary changes of musculoskeletal system is mandatory for the patients with malunions during the planning of treatments measures. The indication for surgical correction should be defined with taking into considera­tion possible development of malunion complications in future.

Epidemiological Study of Adamantinoma from US Surveillance, Epidemiology, and End Results Program: III Retrospective Analysis.

Objective Adamantinomas are rare low-grade malignant bone tumors. This study aims to describe the demographic characteristics and survival rates of patients suffering from adamantinomas. Methods The National Institute of Cancer Surveillance, Epidemiology, and Recent Results (SEER) database was used, and patients diagnosed with adamantinoma between 1973 and 2016 were screened. Patients were classified according to sex, age, race/ethnicity, and marital status, and also tumors were classified according to year of diagnosis, laterality, type of treatment, and follow-up. Results The mean age of patients was 30.8 ± 16.7 (range: 4–75). A total of 92 patients were identified; of these, 43 were females and 49 were males. The mean follow-up period was 138.1 ± 90.3 (range: 1–156) months. Mean survival duration was 287.8 ± 15.4 (95% CI: 257.7–317.9) months. Five- and ten-year survival rates were 98.8% and 91.5%, respectively. Besides, survival time was also observed to be independent of gender, age groups, race, marital status, tumor location, and year of diagnosis. Conclusion Adamantinoma is a very rare bone tumor that affects the long bones in lower extremities and is more common in men. Five- and 10-year survival prognoses are reasonably satisfactory. Also, survival time is independent of variables such as gender, age, and tumor location.

Triquetral Osteoid Osteoma: A Diagnostic Dilemma

Background: Osteoid osteoma (OO) is a benign osteoblastic neoplasm, mostly occurring in long bones of lower extremity. Its manifestation in unusual locations can be a diagnostic challenge and distressing for patients. OO in carpal bones is uncommon; its occurrence in triquetrum is even more so. Case Presentation: This study reports on a nineteen-year-old female with history of wrist pain for 4 years. She had sustained trauma to the same wrist before commencing of pain. Patient's pain was unresponsive to conservative treatments. Her routine x-rays were mostly interpreted as normal and obtaining further imaging helped us in diagnosis of OO of triquetrum. Surgical resection of the nidus was performed. Conclusions: Manifestation of OO in small carpal bones may not be typical; identifying the lesion or nidus could be challenging. Whatever unusual the location or presentation of such lesions may be, obtaining advanced imaging can be of significant help; therefore, we suggest clinicians to have low threshold in ordering advanced imaging specifically in patients suffering from chronic pain. Also, persistent history of nocturnal pain is a sign which is worth attention.

Juvenile Paget's Disease From Heterozygous Mutation of SP7 Encoding Osterix (Specificity Protein 7, Transcription Factor SP7).

Juvenile Paget's disease (JPD) became in 1974 the commonly used name for ultra-rare heritable occurrences of rapid bone remodeling throughout of the skeleton that present in infancy or early childhood as fractures and deformity hallmarked biochemically by marked elevation of serum alkaline phosphatase (ALP) activity (hyperphosphatasemia). Untreated, JPD can kill during childhood or young adult life. In 2002, we reported that homozygous deletion of the gene called tumor necrosis factor receptor superfamily, member 11B (TNFRSF11B) encoding osteoprotegerin (OPG) explained JPD in Navajos. Soon after, other bi-allelic loss-of-function TNFRSF11B defects were identified in JPD worldwide. OPG inhibits osteoclastogenesis and osteoclast activity by decoying receptor activator of nuclear factor κ-B (RANK) ligand (RANKL) away from its receptor RANK. Then, in 2014, we reported JPD in a Bolivian girl caused by a heterozygous activating duplication within TNFRSF11A encoding RANK. Herein, we identify mutation of a third gene underlying JPD. An infant girl began atraumatic fracturing of her lower extremity long-bones. Skull deformity and mild hearing loss followed. Our single investigation of the patient, when she was 15 years-of-age, showed generalized osteosclerosis and hyperostosis. DXA revealed a Z-score of +5.1 at her lumbar spine and T-score of +3.3 at her non-dominant wrist. Biochemical studies were consistent with positive mineral balance and several markers of bone turnover were elevated and included striking hyperphosphatasemia. Iliac crest histopathology was consistent with rapid skeletal remodeling. Measles virus transcripts, common in classic Paget's disease of bone, were not detected in circulating mononuclear cells. Then, reportedly, she responded to several months of alendronate therapy with less skeletal pain and correction of hyperphosphatasemia but had been lost to our follow-up. After we detected no defect in TNFRSF11A or B, trio exome sequencing revealed a de novo heterozygous missense mutation (c.926C>G; p.S309W) within SP7 encoding the osteoblast transcription factor osterix (specificity protein 7, transcription factor SP7). Thus, mutation of SP7 represents a third genetic cause of JPD.

Особливості клініко-рентгенологічного перебігу різних форм фіброзної дисплазії

The aim of the study: to improve the diagnosis of forms of fibrous dysplasia by investigating clinical and radiological features, depending on the disease form, age and sex of the patient. Materials and methods. The research was conducted by analyzing results of examination and treatment of 80 patients with different forms of fibrous dysplasia at Kyiv Institute of Traumatology and Orthopedics by NAMS of Ukraine from 2000 to 2019. The diagnosis was made on the basis of studying features of the medical history of the disease, its clinical course and radiological examination method. There were 56 patients with monoosal disorder, 14 with polyosal, and 10 with Albright syndrome. Clinical-radiological method of the research was used to clarify the diagnosis of the disease and to study features of the disease. The radiological examination was performed on the Multix UP in the department of functional diagnostics «ITO NAMSU» according to the standard technique in direct posterior and lateral projections with capture of adjacent joints. Localization, volume of lesion and condition of cortical layer at pathological dysplastic process of long bones of lower extremities were evaluated. Results . The article specifies clinical-radiological features revealed in various forms of fibrous dysplasia and shows that pathological fractures of the long bones and their deformation depend on the form of the disease, age and sex of patients. The incidence of pathological fractures of long bones of lower extremities was determined depending on the localization, form of the disease and sex of patients with different forms of fibrous dysplasia: femur – 72.3% (proximal part – 76.7%); tibia bones – 27.3% (diaphyseal part – 78.3%); it was found that the number of fractures was significantly greater (49.4%) with the monoosal form of fibrous dysplasia than with the polyosal form (30.1%, p<0.05) and Albright syndrome (20.5%, p<0,5); in males with monoosal form and Albright syndrome, the number of fractures was significantly greater (p<0.05) than in females. Conclusions . Features of the course of clinical and radiological manifestations depending on the form of the disease, age and sex of the patient have been covered in the article, which will allow reducing and eliminating diagnostic errors related to this pathology and create a basis for improvement of the system of orthopedic treatment of this nosology. The research was conducted in accordance with principles of the Declaration of Helsinki. The research protocol was approved by institution’s Local Ethics Committee. Informed consent was obtained from parents of children (or their guardians) for the research.

Value of 68Ga-DOTA-TATE Positron Emission Tomography/Computed Tomography in the Localization of Culprit Tumors Causing Osteomalacia with Negative 99mTc-HYNIC-TOC Single Photo Emission Computed Tomography

Objective To analyze 68Ga-DOTA-TATE positron emission tomography/computed tomography (PET/CT) imaging features of tumor-indud osteomalacia (TIO) patients with negative 99mTc-HYNIC-TOC single photo emission computed tomography (SPECT) findings and to investigate the value of 68Ga-DOTA-TATE PET/CT in accurate localization of culprit tumors.Methods We retrospectively analyzed 68Ga-DOTA-TATE PET/CT imaging features including location,size,density,the maximum and mean standardized uptake value in 37 TIO patients with negative 99mTc-HYNIC-TOC SPECT findings.Results Totally 37 solitary TIO tumors,including 35 phosphaturic mesenchymal tumors and 2 spindle cell tumors confirmed by pathological examinations,were detected via 68Ga-DOTA-TATE PET/CT scans in the included 37 cases. These 37 TIO tumors showed obviously increased activities,with an maximum standardized uptake value of 7.2±4.3 and mean standardized uptake value of 4.3±2.4. The average maximum diameter was (1.9±0.7) cm. The majority of the tumors occurred in the lower extremities (19/37),followed by the trunk (11/37),maxillary/mandibular bone (5/37),and upper extremities (2/37). In addition,24 bone lesions were located in long bones of lower extremities (13/24),most of which demonstrated eccentric growth (8/13). Osteolytic changes (14/24) were observed mainly in the lesions via the corresponding CT imaging;meanwhile,sclerotic changes presented in nine cases. Of the 13 soft-tissue lesions,the majority (10/13) showed well-circumscribed isodense or hypodense nodules on the CT images,with spot calcification in one lesion located in the pleura.Conclusions 68Ga-DOTA-TATE PET/CT scans can detect the TIO culprit tumors miss-diagnosed by 99mTc-HYNIC-TOC SPECT. Somatostatin-receptors highly expressed lesions with focal osteolytic or osteosclerotic change in bone and isodense or hypodense nodules in soft tissue will favor the diagnosis of TIO tumors.

Lower-extremity growth patterns and skeletal maturation in children with unilateral fibular hemimelia.

Fibular hemimelia is the most common congenital long-bone deficiency. It is usually unilateral and results in a limb-length discrepancy. The literature generally subscribes to the concept of constant inhibition, a process by which limb-length ratios between the shorter and longer extremity remain constant throughout growth, but scientific data supporting this concept are sparse. Additionally, recent literature suggests that these children have abnormal skeletal maturation. To elucidate the lower-extremity long-bone growth patterns and skeletal maturation of children with unilateral fibular hemimelia. We reviewed medical records of children with unilateral fibular hemimelia seen at a large pediatric hospital over a 17-year period. Inclusion criteria were: at least two scanograms prior to any shortening/lengthening procedure, and no other congenital or acquired disorders. We collected the study cohort's femoral and tibial lengths (scanogram reports), plotted them against patient chronological ages and compared them to published growth standards. When these children's bone ages (Greulich and Pyle) were available, we plotted them against the children's chronological ages. Twenty-three children were included (total=115 scanograms). At least 1 bone-age assessment was performed in 19 children (total=84 bone ages). All bone growth curves were within normal growth standards for the femur and tibia. Length ratios between shorter and longer limbs remained constant. Skeletal maturation was within two standard deviations of normal in 90% of bone ages. Lower-extremity long bones of children with unilateral fibular hemimelia have relatively normal growth curves, supporting and confirming the concept of constant inhibition. Most children show normal skeletal maturation.

Role of flexible elastic nails in diaphyseal fractures of long bones of lower extremities in children of 5-14 years of age

Femoral and tibial fractures are common long-bone injuries in children. Above five years of age all such fractures, when treated conservatively could lead to loss of reduction, malunion, intolerance and complications associated with plaster. The goals are to stabilize the fracture, to control length and alignment. Flexible Elastic Nailing has become the choice of stabilization in paediatric long bone fractures. The aim of our study was to see the outcome of Flexible elastic nailing system in diaphyseal fractures of children of 5-14 years age. The study included total of 50 children treated with flexible elastic nailing system and they were followed up at 3, 6, 12 and 24 weeks. The average duration of callus formation was 3.8weeks (4.2weeks in Tibia & 3.4 weeks in femur). Radiological union was seen in a mean time of 9.65 weeks. Full weight bearing was possible in a mean time of 10 weeks. According to flynn’s scoring criteria, excellent and satisfactory results were in 90% and 10% respectively. There were 2 cases of post-operative superficial infection which resolved with regular dressing and no cases of physeal injury and implant failure. Flexible elastic nail is a safe and satisfactory mode of treatment and is relatively easy to perform in disphyseal fracture of femur and tibia in children. It avoids the chances of physeal injury, infection and offers early mobilisation and rapid healing.

Surgery and Functional Results of Pathological Fractures of Long Bones of Lower Extremities in Malignant Tumors

Surgery and Functional Results of Pathological Fractures of Long Bones of Lower Extremities in Malignant Tumors

Explore the method of repairing bone defect after resection of lower extremity long bone structure fiber

Objective To analyze the repair methods of bone defects caused by the harmful removal of lower extremity long bone segmental bone fibrous structure, and to provide reference for clinical treatment. Methods Six cases of patient with adverse fibrous structure,3 cases with the jnjury of backbone of the tibia and 3 cases of femoral shaft damage were conducted as the research objects.Bone defect length after tumor resection was(17.3±4.2)cm,in order to coinside with the peroneal vessels in the other side, fibulas with peroneal vessels from both sides should be cut down.Dual fibula medial periosteal was stripped from the central longitudinal incision to the bone crest,folding fibula two-stage to make the front sides face to face, and then fixed the ends.The peroneal vascular was looped on one side of graft to form a U shape. The vessels out of damage region was dissected, and was inserted fibula between the ends of fracture, as the case wire or screws may be used.The peroneal vessels had to coinside with those in damage region,and imaging assessment should be taken to evaluate the healing of bone joints after surgery,at the same time,assessing postoperative function by functional scores of Bone Cancer Society of the United States was also neccessary, finally vascular anastomosis through the bleeding condition of the grafts were detected. Results All patients were followed up for 5-8 years,the bone healed 1 year later, and the transplanted bone marrow cavity got recanalization 5 years later,at the same time, there was no tumorigenesis in the double fibula, and x-ray showed the good healing. All bones turned to be normal ones without nonunion, infection and other complications. Final follow-up score of lower limbs was 91.2%. Conclusion It is effective and feasible to use a combination of double fibula to repair lower extremity long bone defects caused by large bone fibrous dysplasia in the periosteum. Key words: Fibrous dysplasia of bone; Bone defect; Bone transplantation operation

Ilizarov bone lengthening and bone transport technique for treatment of chronic posttraumatic osteomyelitis of lower-extremity long bones

Objective To investigate the therapeutic effect of chronic posttraumatic osteomyelitis of lower-extremity long bones treated by bone lengthening and bone transport with the Ilizarov method. Methods From January 2005 to December 2012, 42 cases of chronic posttraumatic osteomyelitis of lower-extremity long bones were treated using Ilizarov bone lengthening and bone transport technique, including 13 cases of femur infection and 29 cases of tibia infection. Thirty cases were treated by debridement, compression of the fracture end and bone lengthening and 12 cases by debridement and bone transport. Results All the patients received a follow-up of 12 to 48 months [(15.0 ± 4.5) months]. Infection obtained one-stage control in 38 cases, but the infection was not controlled in 4 cases until 1-3 more debridement. For the 30 cases undergoing compression-distraction, 26 healed in one-stage and 4 nonunion healed after additional autogenous bone grafting. Twelve cases treated by bone transport healed after additional surgery of fracture gap cleaning and autogenous bone grafting.Length of bone lengthening was 2.5-11.0 cm (mean, 4.5 cm). After bone healing, shortening of the lower limb compared to the normal side was less than 2.5 cm in 36 cases, 2.5-3.0 cm in 4 cases, and 3.0-4.5 cm in 2 cases. Eight cases were detected with pin-tract infection, but healed by drainage or replacement of external fixation pins. There was no further bone infection. Three cases had loosening of external fixation pin and the pins were replaced. Conclusion Ilizarov bone lengthening and bone transport technique have advantages in the treatment of chronic posttraumatic osteomyelitis of lower-extremity long bones, but postoperative active rehabilitation and close observation are needed to prevent complications of joint dysfunctions, pin tract infection and pin loosening. Key words: Osteomyelitis; Ilizarov technique; Bone transport

Evaluation of the Etiologies of Implant Fracture in Patients With Fractures of the Implants of Lower Limbs' Long Bones.

Background:Orthopedics implants are important tools for treatment of bone fractures. Despite available recommendations for designing and making the implants, there are multiple cases of fracture of these implants in the body. Hence, in this study the frequency of failure of implants in long bones of lower extremities was evaluated.Methods and Materials:In this cross-sectional study, two types of fractured implants in the body were analyzed and underwent metalogical, mechanical, and modeling and stress-bending analysis.Results:The results revealed that the main cause of fractures was decreased mechanical resistance due to inappropriate chemical composition (especially decreased percentages of Nickel and Molybdenum).Conclusions:It may be concluded that following the standard chemical composition and use of optimal making method are the most important works for prevention of failure of implants.

English

BACKGROUND: Flexible intramedullary nails have gained widespread popularity for stabilization of pediatric fractures of long bones of lower extremity. AIMS: Purpose of our study was to evaluate safety and efficacy of titanium elastic nailing system (TENS) for paediatric lower extremity long bone fractures. METHODS: All children operated for lower extremity long bone fractures at our tertiary care centre (Sri Aurobindo Institute of Medical Sciences, Indore) between Jan 2012 to Dec 2013 who underwent fixation with TENS were reviewed. Out of 57 cases identified, 4 cases were lost to follow-up. Of the 53 cases, 58.49 per cent were males (boys 31 and girls 22). The average age of patients was 10.8 years (range 6- 14 years). The mean weight was 28.8 kg (range 16 kg - 42 kg). All fractures were reduced by closed manipulation. Outcomes were classified as excellent, satisfactory or poor according to Flynn flexible nail fixation classification. RESULTS: As per Flynn classification, we had 36 excellent, 14 good and 1 poor result. All patients attained complete healing at mean of 11.2 weeks (Range 7 - 16 weeks). Mean duration of hospital stay was of 5 days (Range 3 - 16 days). CONCLUSION: On the basis of results obtained TENS is an effective method for management of unstable paediatric fractures of long bones of lower extremity allowing rapid

A case of campomelic dysplasia in whom a new mutation was found in the SOX9 gene

Campomelic dysplasia (CD, OMIM #114290) is a rare autosomal dominant disease characterized with bending and shortness in the long bones of the lower extremities, typical facial features, hypoplastic scapula, costa defect, narrow thorax and pes equinovarus. Campomelic dysplasia occurs with heterozygous mutations in the SOX9 gene in the 17q24 chromosome. The main findings of our four-day old patient included typical facial features, risomelic extremity shortness, angular bending in the long bones of bilateral lower extremities and pes equinovarus. On direct graphies, costa defect and scapula hypoplasia were noted. We showed a missense mutation (c.473C>T [p.A158V]) in the SOX9 gene which had not been reported before in our patient who had the typical clinical findings of CD. The family of the patient was informed about potential future pathologies of this disease and received genetic counseling.

Nonossifying Fibroma of Rib

Nonossifying fibroma is usually an asymptomatic benign lesion seen in the first 2 decades of life, occurring commonly in the long bones of lower extremities. Biopsy is required for symptomatic lesions and for those that develop in unusual sites and outside the usual age of presentation [1]. Histologically, there are no neoplastic features. It is considered to be a developmental bony defect that is usually self-limiting, with spontaneous resolution [2].