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1/10. Treatment of cervical cord compression, caused by hereditary multiple exostosis, with laminoplasty: a case report.

    STUDY DESIGN: Case report. OBJECTIVES: Successful excision of the exostosis within the spinal canal. SUMMARY OF BACKGROUND DATA: Myelopathy caused by exostosis within the spinal canal developed in a 13-year-old boy with hereditary multiple exostosis. methods: Spinous process-splitting laminoplasty with an ultrasonic knife was performed to remove the mass and minimize the possibility of postlaminectomy kyphosis. RESULTS: The spinal canal exostosis with cervical cord compression was excised successfully with laminoplasty. After surgery there has been no recurrence of tumor, and the stability of the cervical spine has been preserved. CONCLUSION: This is the first report of laminoplasty as a useful surgical approach for intraspinal exostosis to prevent postoperative cervical instability.
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2/10. Tetraparesis due to exostotic osteochondroma at upper cervical cord in a patient with multiple exostoses-mental retardation syndrome (langer-giedion syndrome).

    STUDY DESIGN: Case report of a severe upper cervical cord compression and tetraparesis by a massive cervical exostotic osteochondroma in a patient with multiple exostoses-mental retardation syndrome (langer-giedion syndrome; LGS). OBJECTIVE: To describe this very rare pathological condition and the results of surgical intervention. SETTING: Gifu, japan. methods: A 23-year-old man was referred to our clinic because of progressing tetraparesis. He had previously been diagnosed with hereditary multiple exostoses and mental retardation. As he had not complained of any symptoms, his family only noticed the tetraparesis after advanced deterioration. His face possessed the pathognomic features of LGS. A postmyelogram CT scan demonstrated an exostotic mass arising from the left-side C2 pedicle with associated severe spinal cord compression. He was diagnosed with LGS. Hemilaminectomy on the left side and resection of the osteochondroma were performed. RESULTS: At 5 years postoperatively, a neurological examination showed the full return of all motor functions. The CT scan revealed no intracanalar recurrence of the tumor. CONCLUSION: In this case of severe tetraparesis due to cervical osteochondroma, decompression by hemilaminectomy provided excellent results. In patients with LGS and intracanalar osteochondroma, the neurological deficit may be masked by mental retardation. Hence, awareness of this pathological condition will help clinicians diagnose it at an early stage.
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3/10. Correction and lengthening for deformities of the forearm in multiple cartilaginous exostoses.

    BACKGROUND: Multiple cartilaginous exostoses cause various deformities of the epiphysis. In exostoses of the ulna, the ulna is shortened and the radius acquires varus deformity, which may lead to dislocation of the radial head. In this study, we present the results of exostoses resection, with correction and lengthening with external fixators for functional and cosmetic improvement, and prevention of radial head dislocation. methods: We retrospectively reviewed seven forearms of seven patients who had deformities of the forearm associated with multiple cartilaginous exostoses. One patient had dislocation of the radial head. Operative technique was excision of osteochondromas from the distal ulna, correction of the radius, and ulnar lengthening with external fixation up to 5 mm plus variance. We evaluated radiographs and the range of pronation and supination. Furthermore, we conducted a follow-up of ulnar length after the operation. RESULTS: Dislocation of the radial head of one patient was naturally reduced without any operative intervention. At the most recent follow-up, six of the seven patients showed full improvement in pronation-supination. Ulnar shortening recurred with skeletal growth of four skeletally immature patients; however, it did not recur in one skeletally mature patient. Overlength of 5 mm was negated by the recurrence of ulnar shortening about 1.5 years after the operation. CONCLUSIONS: We treated seven forearms of seven patients by excision of osteochondromas, correction of radii, and gradual lengthening of ulnas with external fixators. The results of the procedure were satisfactory, especially for function of the elbow and wrist. However, we must consider the possible recurrence of ulnar shortening within about 1.5 years during skeletal growth periods in immature patients.
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4/10. osteochondromatosis of the ankle.

    Synovial chondromatosis is a rare condition that presents as juxta-articular masses which arise due to metaplasia of the synovia and formation of cartilaginous nodules. It will present as a monoarticular mass that is a chronically progressive condition without any tendency to resolve spontaneously. The authors present a review of the literature with a rare case study involving the ankle joint of a 29-year-old black male. The clinical presentation, surgical excision, histologic analysis, and postoperative course are discussed.
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5/10. osteosarcoma arising from a multiple exostoses lesion: case report.

    A case of osteosarcoma arising from a multiple exostoses lesion is presented. Poorly differentiated osteosarcoma occurred in a twelve-year-old girl's proximal tibia where an exostosis was confirmed from radiographs. We treated this patient with preoperative chemotherapy, thigh amputation and postoperative chemotherapy, but she died of multiple pulmonary metastases seven months after surgery. The osteosarcoma, complicated by multiple exostoses, had a very poor prognosis because it was resistant to various anticancer agents.
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6/10. The combined tensor fasciae latae/rectus femoris musculocutaneous flap: a possibility for major soft tissue reconstruction in the groin, hip, gluteal, perineal, and lower abdominal regions.

    A 57-year-old man, with a long-lasting multiple hereditary cartilaginous exostoses, presented with a progressive tumor growth in the left iliac wing and in the gluteus maximus muscle. An open biopsy revealed a secondary chondrosarcoma, which had developed from an osteochondroma. A wide surgical resection, sparing the inferior limb, was the option for treatment. The fairly constant and reliable vascular anatomy of the lateral circumflex femoral artery, as confirmed by 27 previous anatomical dissections, gave us the opportunity to repair the wide postoperative defect by means of a single, very large flap, combining the vascular territories of the tensor fasciae latae and rectus femoris musculocutaneous flaps. The postoperative period was uneventful, and 1 year after surgery the man had no sign of local recurrence or metastases, and the flap was fully viable without sign of local complication. A functional evaluation was performed on a Kin-Com II dynamometer, showing major impairment of the limb that had undergone surgery. Despite the severe functional disturbance, the man prefers the impaired status to an amputation after a hemipelvectomy.
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7/10. Cervical cord compression in hereditary multiple exostoses.

    A seven year old girl, known to have hereditary multiple exostoses, developed moderate gait disorders at the age of six years. Deterioration of myelopathy initiated the clinical investigation including spinal computed tomography (CT) and magnetic resonance imaging (MRI). These examinations demonstrated a coneshaped exostosis originating from the posterior arch of C2. The spinal canal was markedly narrowed with significant compression of the spinal cord at the C2 level. The girl underwent laminectomy of C2 with total removal of the exostosis. Postoperative deterioration of neurological symptoms correlated with a hypointense lesion on T1 weighted imaging in the cord at the same level, but no further cord compression on follow-up MRI. The spastic tetraparesis has improved considerably within 12 months thereafter.
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8/10. One-bone forearm as a salvage procedure for recalcitrant forearm deformity in hereditary multiple exostoses.

    Hereditary multiple exostoses commonly affect the forearm and cause significant deformity. The response of this disease to operative intervention is usually gratifying, but in recalcitrant cases salvage procedures may be necessary. We report two patients treated with radial-ulnar fusion, review the technical aspects of the creation of the so-called "one-bone forearm," and discuss the classification and treatment alternatives available to surgeons treating patients with forearm exostoses. Treatment of both forearms resulted in functional, painless extremities at 3- and 14-year follow-up.
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9/10. 3D-spiral CT of multiple exostoses.

    We report pre- and post-operative three-dimensional (3D)-spiral CT images in a patient with multiple exostoses. Images of 3D-CT, which were performed using the integrated 3D software of the CT system, showed the exact shapes and locations of the individual tumors around the knee joint in comparison with the surgical findings and resected specimen. 3D-spiral CT images of multiple exostoses would be useful for the planning of surgical procedure.
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10/10. Cervical laminar exostosis in multiple hereditary osteochondromatosis: anterior stabilization and fusion technique for preventing instability.

    Multiple hereditary osteochondromatosis is a genetically transmitted disorder consisting of multiple projections of bone capped by cartilage, which are called exostoses. spinal cord compression due to expansion of a laminar osteochondroma is rare but well recognized. Surgical decompression usually improves the patient's neurological status but, in cervical exostosis, post-laminectomy kyphosis and instability problems, especially in the high-risk adolescent group, form the most significant potential difficulties in the postoperative period. We report a case of cervical laminar exostosis that was treated by anterior stabilization and fusion and discuss the benefits of this technique.
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