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1/7. Surgical management of tophaceous gout in the hand.

    A patient who suffered from severe deforming arthritis secondary to chronic tophaceous gout with multilobular, solid, tender, enlarged subcutaneous nodules and draining tophi in both hands was evaluated and treated by second ray amputation of the most deformed second finger to provide a more functional result.
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2/7. Anesthetic management of a difficult airway in a patient with epidermolysis bullosa: a case report.

    epidermolysis bullosa is an inherited skin disease that leads to an array of medical problems. patients are susceptible to blistering and scar formation following even minor trauma. These patients may present with scarring, limiting the range of motion of their temporal mandibular joint. This case report describes a 15-year-old patient with epidermolysis bullosa presenting for contracture release, with a difficult airway.
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3/7. Deforming chondrosarcoma of the fingers secondary to a long-term enchondromatosis of 28 years.

    enchondromatosis of the hand in the context of the Oilier disease is an infrequent pathologie associated with a high rate of malignancy. In this work, we present a case with multiple enchondromas affecting mainly the fourth and fifth rays of the hand, and with multiple ipsilateral lesions in the foot, sacroiliac joint and iliac bone. This patient is, in addition, an unusual case for a long-term progression (28 years), serious deforming appearance and for the double malignant degeneration to chondrosarcoma of the affected fingers. Clinical features, tumor factors, criterion of diagnosis and prognosis, surgical strategy and follow-up evaluation are analysed, as well as a review of the literature.
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4/7. Thenar hypoplasia with associated radiologic abnormalities.

    We report on five patients who presented with unilateral or bilateral hypoplasia of the thenar eminence. X rays of the affected limbs showed varying degrees of malformation and underdevelopment of the thumb phalanges and first metacarpal bone, and of the carpal bones at the base of the thumb, indicating a developmental abnormality. Our electrodiagnostic findings were compatible with this interpretation. We suggest that this condition is part of the clinical spectrum of radial club hand (radial dysplasia) and that when seen in adults, its recognition is important in the differential diagnosis of carpal tunnel syndrome.
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5/7. wrist involvement in rheumatoid swan-neck deformity.

    Two observations have led to the consideration of another possible factor in the etiology of rheumatoid swan-neck deformity. Distraction of the rheumatoid wrist with correctable carpal collapse and reversible swan-neck deformities resulted in easier initiation of proximal interphalangeal joint flexion, not found in hands with boutonniere deformity or lack of deformity. review of x-rays of rheumatoid patients with swan-neck deformity indicates that most have evidence of carpal collapse. With carpal collapse and proximal migration of the carpal-hand unit, long or extrinsic motor systems are no longer working at physiological length or optimal efficiency. An imbalance is created, resulting, in effect, in an "extrinsic-minus" phenomena and swan-neck finger deformity.
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6/7. Multifocal plasmacytoma of hand and foot bones.

    Simultaneous occurrence of localized plasmacytomas of both hands and feet has not been reported so far. Here we report a 40-year old female patient, who had at presentation pain and deformity. Of hands and feet, with numerous cystic lytic lesions of phalangeal, metacarpal and metatarsal bones, detected by x-rays. The biopsy of the affected bone showed moderately differentiated plasmacytoma of lambda light chain type (lambda-LC). serum and urine biochemical analysis revealed the existence of lambda LC monoclonal component. The patient was treated by local radiotherapy and subsequent systemic chemotherapy, which consisted of 3 cycles of the M-2 protocol and 7 cycles of melphalan-prednisone. Five years after the diagnosis, the absence of plasmacytoma was confirmed by puncture biopsy of the left hand phalanx. Monoclonal protein in serum and urine was not detected.
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7/7. Juvenile diabetic cheiroarthropathy.

    A peculiar involvement of the interphalangeal joints of both hands with palmar flexion of the fingers has been observed in 11 insulin-treated, nonrheumatoid, juvenile diabetics. The onset of diabetes occurred between 1 and 12 years of age. Painless deformities of the fingers with progressive stiffness and impaired extension started 4 to 10 years later. One patient complained of articular pain and swelling. X-ray and circulatory changes were absent or minimal. Prepubertal patients showed delayed puberty and stunted growth, adult patients had normal sexual development. Rheumatic or rheumatoid signs were absent. electromyography showed minor abnormalities of the motor units, normal or subnormal motor nerve conduction velocity, increased median nerve terminal latency, in the absence of muscular atrophy or thickening of palmar tendons. Vibratory sensitivity was impaired in 1 subject. Juvenile cheiroarthropathy is associated with: a) early onset and poor control of diabetes; b) stunted growth; c) hepatomegaly; d) delayed puberty; e) long standing administration of insulin. The articular changes are distinct from previously known forms of "diabetic hand", such as atrophic neuropathy, osteoarthropathy, Dupuytren's contracture, carpal tunnel syndrome.
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