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The typical hand deformity of Parkinsonism consists of metacarpophalangeal joint flexion and interphalangeal joint extension. An unusual case of a claw hand deformity in a patient with Parkinson's disease is described with hyperextension in the metacarpophalangeal joints and flexion in the interphalangeal joints. The operative technique to improve the marked limitation in hand function is described.
12 hands with congenital short finger in 11 patients were treated with various types of metacarpal bone lengthening. These included three patients with brachydactyly, seven with transverse deficiency, and one with constriction ring syndrome. All cases involved metacarpal lengthening and surgery was performed 16 times in 15 digits. Single-stage lengthening was performed in seven cases, on-top plasty in three cases, and distraction lengthening in six cases. The length gained ranged from 2 to 10 mm in single-stage lengthening, 3 to 17 mm in on-top plasty, and 12 to 30 mm in distraction lengthening. Delayed union and malunion occurred in single-stage lengthening or on-top plasty. After metacarpal lengthening, pinch function was improved in seven out of eight patients with either transverse deficiency or constriction band syndrome, and aesthetic improvement was achieved in three patients with brachydactyly.
The claw hand deformity, resulting from low ulnar or combined low ulnar and median nerve palsy, is an incapacitating situation. The splint described herein reverses the clawing by substituting for the lumbricals and interossei. If started early, it not only prevents the permanent stiffness of fingers in the claw position, but also effectively restores function without hampering day to day work because it is a surface splint.
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Hand deformities cause severe discomfort and functional limitations to patients with Parkinson disease (PD) and related disorders. In clinical practice the problem is often overlooked or misdiagnosed. This paper reports on four cases whose characteristics are discussed.
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We report the prevalence of sensory loss and hand deformity in 63 patients with Hansen's disease in American Samoa. Open ulceration, the most common deformity, was present in 41% of patients; sensory abnormalities were present in 54% and were bilateral in 65%. The presence of abnormal sensibility correlated with a high percentage of other deformities. Hand abnormalities were most prevalent in lepromatous patients and were related to a prolonged duration of disease. Nerve thickening did not appear to be a helpful clinical finding to assess the degree of sensory loss.
OBJECTIVES: To evaluate patients with mutilans-like hand deformities in chronic inflammatory joint diseases and to determine radiographic scoring systems for arthritis mutilans (AM). METHODS: A total of 52 patients with severe hand deformities were collected during 1997. A Larsen hand score of 0-110 was formed to describe destruction of the hand joints. Secondly, each ray of the hand was assessed individually by summing the Larsen grade of the wrist and the grades of the MCP and PIP joints. When the sum of these grades was > or = 13, the finger was considered to be mutilated. A mutilans hand score of 0-10 was formed according to the number of mutilans fingers. Surgical treatment and spontaneous fusions were recorded. RESULTS: The study consisted of 22 patients with juvenile rheumatoid arthritis (JRA), nine with rheumatoid factor (RF) positive and 13 with RF negative arthritis, 27 patients with RF positive RA, and three adult patients with other diagnoses. The mean age of patients with adult rheumatic diseases was 27 years at the onset of arthritis. The mean disease duration in all patients was 30 years. The mean Larsen hand score was 93. Four patients had no mutilans fingers and in 15 patients all 10 fingers were mutilated. The Larsen hand score of 0-110 and the mutilans hand score of 0-10 correlated well (rs = 0.90). Fourteen patients showed spontaneous fusions in the peripheral joints. A total of 457 operations were performed on 48 patients. CONCLUSION: Both the Larsen hand score of 0-110 and the mutilans hand score of 0-10 improve accuracy in evaluating mutilans-like hand deformities, but in unevenly distributed hand deformities the mutilans hand score is better in describing deformation of individual fingers.
The therapy for congenital hand malformations, especially in symbrachydactyly and constriction ring syndromes, is challenging. Between 1975 and 1995, 20 children with congenital hand deformities underwent reconstruction by 56 nonvascularized free toe phalanx transfers. The average age at initial surgery was 4.8 (range, 0.5 to 22) years. Retrospectively, the children were examined after an average of 3.5 (range, 1.5 to 17.6) years for function of the hand, transplanted phalanx growth, assessment for the epiphyseal plate, and assessment for psychologic performance with their parents. Donor-site morbidity was determined according to measured growth deficit, observing the child's gait, and toe function. In the younger patients (up to 1.5 years), the grafts were well tolerated and showed good growth and only few resorptions. In the age group from 1.5 to 4 years, the grafts showed no growth. In the age group older than 4 years, the grafts were mostly resorbed. The clinical reexamination revealed in most cases only passive motion in the joints, but the function of the hand was improved, with only few problems of the donor site. Most patients and their parents reported a positive effect of the phalanx transfer.
Eighteen adult patients with spastic hand deformities underwent release of the flexor-pronator origin and step-cut lengthening of the flexor pollicis longus to improve prehensile hand function. The goal of surgery was to provide active grasp release in order to improve hand function. Using a previously published grading scale, patients were graded before and after operation. At follow-up after an average of 35 months (range 24-64 months), all had improved by at least two functional levels.
This study examined radiographically the prevalence of arthritis mutilans hand deformities in an inception cohort of 68 rheumatoid arthritis (RA) patients. Hand deformities of 103 RF-positive RA patients were evaluated after 8 years, 83 patients after 15 years and 68 patients 20 years after entry. The grade of destruction in the hand joints was assessed by the Larsen method and Larsen scores of 0-50 were determined for both PIP (+IP) and MCP joints. At the end point, 3 patients had Larsen scores > or =40 for both PIP and MCP joints. These three patients had severe resorption in most of the finger joints, but did not demonstrate classical opera-glass hand. The prevalence of mutilans-like hand deformities with RA was 3/68 (4.4%) in a prospective 20-year study.
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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.
A 23-year-old woman from Honduras was diagnosed to have hepatoerythropoietic porphyria. She had photosensitive skin of early onset, hypertrichosis, and severe scleroderma-like lesions of the hands. Erythrocyte uroporphyrinogen decarboxylase activity was reduced to about 10% of the normal activity.
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Explore the source record for details and available documents.