A 22-year-old female presented with painful, progressive swelling of the right forearm for six months. Physical examination revealed a 7 cm x 5 cm firm, tender soft-tissue swelling over the anterior aspect of the right proximal forearm with normal overlying temperature. Xrays showed increased soft tissue shadow but without any bony involvement. Serum alkaline phosphatase, serum lactate dehydrogenase, and leukocyte count were raised. MRI of the right forearm revealed enhancing soft tissue lesion with internal hemorrhagic and necrotic components involving the flexor carpi radialis muscle. Core biopsy confirmed the diagnosis of extraosseus Ewing's sarcoma. Neoadjuvant chemotherapy, wide local tumor excision, and adjuvant chemotherapy and radiotherapy were done. The patient then lost to follow-up and presented again after six months with a fungating mass and neurovascular involvement for which an above-elbow amputation was done. We, through this case report, aim to discuss the clinical and radiological findings, line of management, and the importance of early detection and treatment and a regular follow-up for extraosseus Ewing's sarcoma of the extremity.
parameters in patients with healed spinal tuberculosis with severe thoracic kyphosis. Overview of Literature: Deterioration of neurological function is an absolute indication of surgical intervention in severe posttubercular kyphosis, but the relationship of compromise in lung function and spinal alignment with severity of kyphosis is still unclear. Methods: Twenty patients (age, 14-60 years) with healed spinal tuberculosis with thoracic kyphosis >50° were included. Lateralview radiography of the whole spine, including both hips, was performed for assessment of kyphotic angle (K angle), sagittal balance, lumbar lordosis, and spinopelvic parameters. Pulmonary function was assessed by measuring the forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), and their ratio (FEV1/FVC) by spirometry. Results: A positive correlation between severity of kyphosis and sagittal imbalance was noted, with compensatory mechanisms maintaining the sagittal balance in only up to 80° of dorsal kyphosis. In >80° of kyphosis, FVC was found to be markedly decreased (mean FVC=50.6%). The mean K angle was lower in subjects with lower thoracic kyphosis. In lower thoracic kyphosis, due to short lordotic and long kyphotic curves, both lumbar lordosis and pelvic retroversion worked at compensation, whereas, in middle thoracic kyphosis, due to long lordotic curve, only lumbar lordosis was required. Normal pulmonary function (mean FVC, 83.0%) and lesser kyphotic deformity (mean K angle in adolescents, 69.8°; in adults, 94.4°) were found in adolescents. Conclusions: In >80° of thoracic kyphosis, there is sagittal imbalance and a markedly affected pulmonary function. Such patients should be offered corrective surgery if they are symptomatic and medically fit to undergo the procedure. However, whether the surgical procedure would result in improved pulmonary function and sagittal balance needs to be evaluated by a follow-up study.
A 25-year-old man presented to us with progressive multiple joint pain, enlargement, and restricted movements. X-rays showed platyspondyly, multiple epiphyseal widening, synovial chondromatosis, and decreased bone stock and cortical thickness. Genetic testing showed biallelic pathogenic variants in CCN6 which confirmed the diagnosis of progressive pseudorheumatoid dysplasia. Supportive care, physical therapy, genetic and psychological counselling were provided to the patient.
Spinal neurenteric cysts are very rare intradural developmental lesions, predominantly localized anterior to the cervical cord and arising from misplaced endodermal cells in the 3rd week of embryonic life. An acute onset of symptoms can occur due to hemorrhage, but has not as yet been reported in relation to infection in the cyst. We report an 18-year-old male who presented with a rapid onset of respiratory distress and quadriplegia over a period of 6 h requiring intubation and ventilatory support. There was no respiratory effort with a dense sensory level from the neck. MRI of the cervical spine revealed an intradural extramedullary ventrally located cystic lesion at the C2–3 level. There was no evidence of systemic infection elsewhere in the body. The lesion was radically excised using an extreme lateral approach as it provided excellent visualization of the cyst, thus permitting safe dissection without retraction of the cord. The cyst was tense and the contents turbid, thick, creamy and mucinous without any evidence of fresh or altered blood. Histopathological examination confirmed the cyst to be a neurenteric cyst. The cystic fluid, which was sent for culture, grew coagulase-negative Staphylococcus aureus. This is the first documented case of rapid neurological deterioration due to infection in a neurenteric cyst. In spite of an early effective surgical management, outcome was poor due to the rapid and profound neurological deficit.
Batheja et al. This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 4.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
A 32-year-old woman presented with progressive pain and swelling of the left wrist for 6 months. Physical examination revealed a firm, tender, oval swelling over the left wrist. X-rays showed a pressure effect on the distal radius and ulna. Magnetic Resonance Imaging (MRI) revealed a well-defined, asymmetrical, dumbbell-shaped soft-tissue lesion involving the interosseous region of the distal forearm and extending until the distal radioulnar joint (DRUJ). Core needle biopsy confirmed the diagnosis of desmoid tumour. Marginal excision of the tumour was done. At the 2-year follow-up, the patient was doing well and had painless and improved left wrist motion. Desmoid tumour involving the DRUJ has not been previously reported. We, through this case, report new observation and discuss the epidemiology, investigation of choice, treatment modalities, and the need for a regular follow-up for appendicular desmoid tumours.
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