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1/7. Optimizing the correction of severe postburn hand deformities by using aggressive contracture releases and fasciocutaneous free-tissue transfers.

    Severe postburn hand deformities were classified into three major patterns: hyperextension deformity of the metacarpophalangeal joint of the fingers with dorsal contracture of the hand, adduction contracture of the thumb with hyperextension deformity of the interphalangeal joint, and flexion contracture of the palm. Over the past 6 years, 18 cases of severe postburn hand deformities were corrected with extensor tenotomy, joint capsulotomy, and release of volar plate and collateral ligament. The soft-tissue defects were reconstructed with various fasciocutaneous free flaps, including the arterialized venous flap (n = 4), dorsalis pedis flap (n = 3), posterior interosseous flap (n = 3), first web space free flap (n = 3), and radial forearm flap (n = 1). Early active physical therapy was applied. All flaps survived. Functional return of pinch and grip strength was possible in 16 cases. In 11 cases of reconstruction of the dorsum of the hand, the total active range of motion in all joints of the fingers averaged 140 degrees. The mean grip strength was 16.5 kg and key pinch was 3.5 kg. In palm reconstruction, the wider contact area facilitated the grasping of larger objects. In thumb reconstruction, key-pinch increased to 5.5 kg and the angle of the first web space increased to 45 degrees. Jebsen's hand function test was not possible before surgery; postoperatively, it showed more functional recovery in gross motion and in the dominant hand. Aggressive contracture release of the bone,joints, tendons, and soft tissue is required for optimal results in the correction of severe postburn hand deformities. Various fasciocutaneous free flaps used to reconstruct the defect provide early motion, appropriate thinness, and excellent cosmesis of the hand.
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2/7. Polyarticular symmetric tophaceous joint inflammation as the initial presentation of gout.

    A 76-year-old woman suffered from bilateral distal index finger pain and swelling. The patient had been initially treated with antibiotics for herpetic whitlow complicated by a secondary bacterial infection. gout was diagnosed through clinical history, physical examination and identification of monosodium urate crystals in the joint aspirate. gout appearing as polyarticular, symmetric tophi involving the periungual region and distal interphalangeal joint has not been previously described.
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3/7. Pseudodiastrophic dysplasia: a distinct newborn skeletal dysplasia.

    Pseudodiastrophic dysplasia is a distinct disorder that differs from diastrophic dysplasia on the basis of clinical, radiographic, and chondro-osseous histopathologic findings. In addition to the rhizomelic shortening of the limbs and severe clubfoot deformity, which suggest the diagnosis of diastrophic dysplasia, distinguishing features are elbow and proximal interphalangeal joint dislocations, platyspondyly, and scoliosis, which are observed in infancy. This disorder has been reported previously in three infants, all of whom died in the first year of life. Two of these were sisters, suggesting autosomal recessive inheritance. We report four new patients with this distinct skeletal dysplasia, including two children now older than 4 years of age. In both of these patients neonatal contractures have improved with physical therapy and scoliosis has progressed significantly.
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4/7. Xanthoma disseminatum: an unusual cause of facial and limb deformity.

    A rare case of xanthoma disseminatum producing deformity of the head, neck, and extremities is reported. This pathologically benign disease, considered to represent one of the histiocyte proliferative disorders, is manifested by numerous cutaneous xanthomas primarily in flexion areas. Progressive enlargement of these lesions accompanied by marked fibrosis has produced the severe cosmetic and functional deformities described in this patient. Treatment in this case involved excision of xanthomatous contraction bands with split-thickness skin grafting, followed by intensive physical therapy.
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5/7. Bejel in Malatya, turkey.

    Bejel is a form of endemic syphilis which is transmitted by non-venereal route. According to our knowledge, no cases of bejel have been reported from turkey for more than 30 years. In this report, three children and their father who suffer from bejel are presented. The history, physical findings, laboratory investigations, treatment and results of the cases are reviewed, and the related literature is discussed.
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6/7. A 47-year-old woman with a 5-year history of bilateral hand deformities.

    The following case is presented to illustrate the roentgenographic and clinical findings of a condition of interest to the orthopaedic surgeon. Initial history, physical findings, and roentgenographic examinations are offered for your consideration. The final clinical and roentgenographic differential diagnoses are presented on the following pages.
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7/7. Unilateral insensate macrodactyly secondary to tuberous sclerosis in a child.

    Primary localized gigantism of the hand is exceedingly rare and has been reported only twice in the literature. Both cases were associated with tuberous sclerosis in which the patients had normal sensation. In this case report, a very unusual combination of insensate and unilateral macrodactyly in a child with tuberous sclerosis is described using both physical and roentgenographic findings.
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