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The musculoskeletal manifestations of the Coffin-Lowry syndrome.

Coffin-Lowry syndrome (CLS) is a rare genetic disorder characterized by craniofacial abnormalities, mental retardation, short stature, and hypotonia. Patients with CLS may present with multiple musculoskeletal abnormalities. The purpose of this study was to identify and characterize the musculoskeletal findings in 10 patients with CLS. Eight patients presented with thoracolumbar kyphosis or kyphoscoliosis, with a mean Cobb angle of 45 degrees in the coronal plane and 31 degrees of thoracolumbar kyphosis. These may be progressive and difficult to treat, needing early surgical treatment. Close follow-up of the spinal deformities is strongly recommended to document progression. Sixty percent of the patients presented with bilateral flexible and painless planovalgus deformities. Hypoplasia of the ilium and hand deformities are common but do not seem to cause any functional problems. Observation is recommended for these asymptomatic hand, foot, and pelvic findings.

Adolescent↗

Limited joint mobility in diabetes.

Cheiroarthropathy is a syndrome usually associated with diabetes mellitus that involves mainly the small joints of the hand. It is generally painless and seldom treated until hand deformity is severe enough to interfere with daily life. Because cheiroarthropathy is correlated with microvascular complications of diabetes, patients with hand symptoms should be carefully investigated for neuropathy, retinopathy, and nephropathy. Improved control of diabetes is the cornerstone of management, but surgery and corticosteroid injection may alleviate severe symptoms in some patients.

Contracture↗

[Occupational therapeutic measures before and after surgical correction of hands with congenital malformations].

The task of the occupational therapist following surgical procedures on deformed hands is discussed. In the child with congenital malformations, we must not only be concerned with treatment of the deformity but we must be concerned about the psychic problems which can develop following prolonged hospitalization or overprotection. One must be keenly observant to detect these problems early. Preoperative testing utilizing a standardized gripping test is carried out by the occupational therapist. The necessity for this is demonstrated by the example of the radial clubhand. In this way complications such as correction of clubhand in the face of a stiff elbow or pollicisation in the presence of inadequate clubhand correction can thus be avoided. After operation training in the new functions is begun as early as possible; preferably with individual supervision. Daily repetative and progressive exercises should result in maximum utilization of gripping ability provided by the operative procedure. The treatment program also includes the adaptation of clothing, the production of appliances and splints, in co-operation with the orthopedic technician, as well as specially designed functional exercises. All these factors are as important as the operative procedure itself in contributing to maximum benifit. All essential findings and their variations must be written down. Also the photographic documentation of the functions is the job of the occupational therapist. Sufficient time should be allowed prior to discharge to inform the parents about the newly acquired functions and the use of the applicances. The maintenance and further development of the newly acquired functions is insured through the continued ambulatory supervision by the doctor and the occupational therapist. The occupational therapist thus has a significant job as a member of the team in a special unit for children with congenital malformations.

Child↗

A review of the management of the hand in dystrophic epidermolysis bullosa.

The hand deformities associated with dystrophic epidermolysis bullosa lead to a mitten-like hand with significant loss of function. The pattern of their development is discussed. Although the digits can be released and divided surgically, the deformity is recurrent. This study provides a review of the literature on the management of the hand in this disease and a discussion of the role of therapy in maintaining hand function.

Epidermolysis Bullosa Dystrophica↗

Functional disability in the rheumatoid hand.

The investigation of sixty-one rheumatoid patients relates their hand function to measured hand deformity. Certain deformities are related to a reduction in function; others are not. The dominant hand, used more frequently, deteriorates more quickly than the non-dominant. The benefit on hand function of synovectomy is limited in duration and should be accompanied by some procedure to defer or correct deformity. Study of the metacarphophalangeal joints by radiography and at operation shows that degeneration starts where the metacarpal head first loses articulation with the base of the phalanx, and spreads out from there across the metacarpal head as deformity increases.

Adult↗

Anomalous hands with cleft formation between the fourth and fifth digits.

Three similar cases of cleft hand deformities between the fourth and fifth digits accompanied by hypoplasia of the small finger are reported. After analyses of clinical photographs, radiographs, angiograms, and dermatoglyphic prints of these abnormal hands, it is difficult to fit them into either the ulnar ray deficiency or cleft hand categories of the system of classification adopted by the International Federation of Societies for Surgery of the Hand.

Child, Preschool↗

Management of advanced foot deformities in dystrophic epidermolysis bullosa.

Treatment for the characteristic hand deformities of dystrophic epidermolysis bullosa has been well described in the literature. We present our first experience delineating the surgical treatment for advanced foot deformities in this disease. This case emphasizes the need for release of contractures, the use of split-thickness skin grafts, and the need for rigid fixation to maintain surgical correction until healing is complete.

Adolescent↗

Cleft hand and pectoral aplasia.

An association of congenital musculoskeletal anomalies is described. This association is characterized by ipsilateral absence of pectoral muscles, generalized mild hypoplasia of the upper extremity, and severe cleft hand deformities. The importance of recognizing this association lies mainly in genetic counseling.

Abnormalities, Multiple↗

Hand abnormalities in pygmy chimpanzees (Pan paniscus).

Hand deformities in 2 wild-born pygmy chimpanzees were investigated through dissection, linear measurements, bone mineral analysis and X-rays. Weight of tissue (skin, muscle, bone) was determined for each body segment (hand, forearm, upper arm), permitting comparison between the abnormal and normal upper limbs. In one individual, the right and left forearms and hands differed in tissue weights and bone mineral content. In the other, the hands differed in tissue composition, but not the forearms.

Animals↗

[Long-term follow-up of the ulnar nerve repair: report of 42 cases].

A long term follow-up for 42 cases of ulnar nerve repair was reported in this article, and the reason of bad function were analysed. They are: 1. As long as the injured ulnar nerve was sutured perfectly and without delay, the result would be satisfying. 2. The follow-up examination of the repaired nerve showed all the paralysed intrinsic muscles would recover in certain extent. Abductor digiti quinti, adductor pollicis and flexor digiti quinti brevis usually restored easily. Among the intrinsic muscles the best result of the first dorsal interosseous and the first palmar interosseous muscles were obtained. The functional recovery of the second and third palmar interosseous muscles usually was poor. 3. After repairing of the injured ulnar nerve, recovery of the claw hand deformity usually takes long time, so the operative correction of the deformity should not be performed until 2.5 years.

Adolescent↗

Hand strength measurement instruments.

A set of instruments based on strain-gage application has been designed for hand strength measurement. The reduction in size, variation in position, and ease of calibration offer a wide range of functional evaluation without causing discomfort to the subjects being examined. The strength during the entire period of functional activity can be continuously monitored. The measurement signal from the device in analogue form makes computer-aided strength evaluation possible. Above all, the construction of the devices also allows easy adaptation to different hand deformities. The instruments can be useful tools for both research and clinical evaluation.

Hand Deformities, Acquired↗

Modulan grip-aids for leprosy patients.

Made-to-measure Modulan grip-aids were fitted to 755 articles for 155 patients with hand deformities due to leprosy. The acceptance of the grip-aids was, in general, good. No instance of contact dermatitis or skin irritation was reported. These grip-aids facilitated a normal grip with crippled hands, and thus considerably improved the quality of the patient's personal and working life. They increased the patient's self-esteem and self-confidence because he/she could handle everyday objects or tools without the help of others and could do his/her job--an important step toward social and economic rehabilitation.

Epoxy Resins↗

The windblown hand - diagnosis, clinical picture and pathogenesis.

The syndrome of the windblown hand deformity is a complex constellation of malformations affecting not only the head and the feet but also the hands in a quite distinct manner. In the hand, it involves congenital bilateral flexion contracture with ulnar deviation of the metacarpophalangeal joints. The thumb is characteristically adducted (reaching the palm; "thumb-in-palm deformity") with flexion of the MP joint and hyperextension of the IP joint. The etiology is basically unknown. We present two theories based on knowledge derived from the disciplines of evolution biology and embryology. We believe that the atavistic appearance of phylogenetically primitive muscle groups in conjunction with an impaired rotation of the extremities during embryological development account for this malformation syndrome.

Abnormalities, Multiple↗

Relationship between finger and wrist deformities in rheumatoid arthritis.

Ulnar deviation of fingers in rheumatoid arthritis is known to be very frequently associated with the radial deviation of the hand. The present study was undertaken to test the hypothesis that there is a significant correlation between the position of the wrist and metacarpophalangeal angulation in common rheumatoid hand deformities. Measurements were carried out on standard radiographs of the hands from two comparative groups: 50 adult patients (100 hands) with classical or definite rheumatoid arthritis and the control group of 40 adults (80 hands) suffering from no rheumatoid arthritis but coming for routine check up relating to some degenerative diseases. The results showed a statistically significant relationship between finger and wrist deformities in patients with rheumatoid arthritis but not in normal controls.

Adult↗

A comparative study of EFMT and sublimis transfer operations in the claw hand.

Two hundred cases each of EFMT and sublimis transfer operations performed for correction of claw hand deformity following Hansen's disease were studied and the results compared. The cases were selected by systematic sampling. The incidences of reclawing in the index, long, and ring fingers were found to be more in the EFMT series. Also the postoperative intrinsic plus deformity was seen more often in the ring and little fingers in the EFMT series. The occurrence of a sublimis minus deformity following removal of the sublimis tendon is recognized and described. Both operative procedures produced 95% satisfactory results.

Hand Deformities, Acquired↗

Total palmar resurfacing with scapular free flap in a 26-year contracted hand.

The scapular free flap offers a large-sized and well-vascularized coverage for variable defects. It is not regarded, however, as a favorite tool for palmar resurfacing because of its bulky and rigid nature. A 28-year-old man had sustained a contact dermal injury at the age of 2 years that resulted in a severely deformed hand. The total palmar defect was resurfaced by surgical intervention with a scapular free flap. After secondary procedures, the final result was promising. The present report focuses on the secondary procedures, which make a more functional and aesthetically pleasing hand after coverage with the scapular flap. Also, our result suggests that even finger joints contracted for 26 years can recover motion if they have not been directly damaged.

Adult↗