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Why should a deformed, anaesthetic, functionless hand be wanted?

A 58-year-old woman presented at a disability assessment tribunal with a severely deformed left forearm. She had broken the arm 25 years previously and the infected open fracture had not healed. There were no sensory or motor functions in the hand, which the patient kept constantly hidden in her garments. The question of an amputation was raised and the advantages of modern prosthetics with respect to cosmesis and function failed to convince the patient that she might benefit from such surgery. One questions why a patient should want to retain this cumbersome, obviously embarrassing, burden and whether the question of surgery should have been raised.

Amputation, Surgical↗

A randomized controlled trial of home exercise on the rheumatoid hand.

OBJECTIVE: To study differing home hand exercise interventions to determine effects on grip strength, and secondarily any immediate or short term effects on range of motion, pain, deformities, hand disease activity, and dexterity. METHODS: Randomized controlled trial of 12 weeks of home hand exercise performed for 10-20 min twice daily. Study exercise interventions were range of motion exercises, balanced resistive exercises, and range of motion plus balanced resistive exercises. RESULTS: Aside from transient, mild to moderate discomfort, exercises were well tolerated. Range of motion exercises were associated with improved right hand joint count. Range of motion plus balanced resistive exercises were associated with increased left hand dexterity. Home hand exercise (exercise groups combined) significantly increased left grip strength. CONCLUSIONS: Temporary use of home hand exercise has acceptable side effects and is an effective means of increasing grip strength.

Ambulatory Care↗

A laser-aligned method for anthropometry of hands.

We have developed a laser-aligned measuring device to facilitate the measurement of linear hand dimensions. Our objective has been to make a simple, clinically applicable device for anthropometry of the hands in patients with arthritis in order to gauge the progression of hand deformities. The system provides an alternative to the use of rules, tapes, photogrammetry and least-squares devices which have previously been applied to hand measurements. The system delivers results which are not significantly different from those measured with rules and callipers and has a similar level of accuracy. The use of laser pointers for alignment facilitates measurements which necessitate picking the highest point of the hand. The device is portable and simple to use in the clinic. We have used it to measure hand dimensions in normal subjects and patients with arthritis. However, the system could equally well be used to facilitate measurement of other objects with an irregular surface profile.

Adult↗

Effectiveness of rheumatoid hand surgery: contrasting perceptions of hand surgeons and rheumatologists.

PURPOSE: Surgical management of rheumatoid hand diseases is controversial with large variation in practice pattern in the U.S. The purpose of this study is to evaluate the attitudes of hand surgeons and rheumatologists toward the effectiveness of rheumatoid hand surgery. METHODS: We designed a survey instrument to examine physicians' opinions about the effectiveness of different surgical treatments for rheumatoid hand deformities. The self-administered survey was mailed to a national random sample of 500 members of the American Society for Surgery of the Hand and 500 members of the American College of Rheumatology. RESULTS: Of survey responders, 82.5% of hand surgeons versus 34.1% of rheumatologists believe metacarpophalangeal joint arthroplasty improves hand function; 93.2% and 54.6%, respectively, believe prophylactic extensor tenosynovectomy prevents tendon rupture; and 52.5% and 12.6%, respectively, believe small joint synovectomy delays joint destruction. CONCLUSIONS: Rheumatologists view rheumatoid hand surgery as significantly less effective than do hand surgeons, which highlights the disagreements between the 2 specialties about the management of this clinical problem.

Arthritis, Rheumatoid↗

Functional assessment of the hand: reproducibility, acceptability, and utility of a new system for measuring strength.

A new system for measuring strength of the hand using a torsion dynamometer linked to a microprocessor is described. The system permits analysis of timed squeezes of both grip and pinch and is adjustable to all sizes of hand and degrees of hand deformity. Results obtained with the system were found to be reproducible, and the rigid device was acceptable to a group of patients with arthritic hands. In rheumatoid arthritis there is a marked reduction in maximum grip and pinch strength, together with a prolongation of the time taken to reach this maximum, and increased fatigue. The limitations of grip strength as a measure of function of the hand are discussed.

Adult↗

Chest wall deformities.

Children present with chest wall deformities to their pediatrician because of physiologic and psychologic causes. We have a 22-year experience in the operative management of more than 300 of these children. Initial evaluation should accurately categorize the deformity, determine the severity, assess for associated problems and refer the child at an appropriate time for further management. Pectus carinatum is the most common deformity that can cause physical pain from an intercostal neuropathy and psychologic pain from the unsightly protuberance. Poland syndrome is managed by early correction of the hand deformity and later correction of the chest wall deformity if necessary for psychologic or functional reasons. Jeune syndrome and failure of sternal fusion may have severe associated cardiopulmonary compromise. Each should be corrected in infancy for the best results.

Adolescent↗

Rheumatoid arthritis and Dupuytren's contracture.

Of four patients with rheumatoid arthritis and Dupuytren's contracture, two were not aware of the presence of Dupuytren's contracture. When both diseases coexist, the presence of rheumatoid hand deformities, especially flexion and ulnar deviation of the metacarpophalangeal joints, may mask the flexion deformity caused by Dupuytren's contracture. Careful clinical examination should rule out the presence of a pathologic fascial cord. When reconstructive surgery is indicated for the rheumatoid hand in the presence of advanced Dupuytren's contracture, staged surgery would be appropriate and reconstruction of Dupuytren's contracture should precede other surgery.

Aged↗

Trisomy 18: behavioral and structural abnormalities. An ultrasonographic case study.

A behavioral and structural analysis using ultrasound of a fetus suffering from Edwards syndrome is described. A number of structural anomalies were observed: the classic hand deformity, extended legs, diamond-shaped head, open mouth and a severe cardiac abnormality. A number of distinctive behavior patterns were observed: vertical eye movements, longitudinal rotation of the trunk, rotation of hands, tremor of extremities and pulsatile body movements, which are not usually seen in the normal fetus. The use of behavioral indicators in the diagnosis of abnormalities is discussed. We conclude that the analysis of behavior may make a significant contribution to the ultrasonographic diagnosis of handicap.

Journal Article↗

The community of human malformation syndromes that shares ectodermal dysplasia and deformities of the hands and feet.

Syndromes of human congenital malformation may be classified be recognizing communities of syndromes that share multiple phenotypic similarities involving their principal diagnostic features. A community of syndromes that shares various expressions of ectodermal dysplasia and various deformities of the hands and feet is proposed; these syndromes are divisible into two classes according to the presence or absence of anomalies in the nasal or labial regions of the face. The dysmorphogenetic validity of the division is supported by the fact that the syndromes without nasal or labial anomalies have a high frequency of sensorineural deafness as one expression of ectodermal dysplasia whereas those without such anomalies do not. The usefulness of such a syndromal community as a base for evolving a taxonomic scheme of dysmorphogenetic relatedness amongst different syndromes is illustrated.

Abnormalities, Multiple↗

Distraction osteogenesis in the hand.

Distraction osteogenesis was used to correct congenital hand deformities in 12 digits. The patients' ages at the time of distraction ranged between 2.5 and 7 years. A total of nine patients underwent lengthening of 12 bones. The average period of distraction was 31.1 +/- 17.6 days. The average length of distraction was 23.6 +/- 7.3 mm. Seventeen percent of the patients after lengthening had an angular deformity that required remodeling of the regenerate. No infections were noted. Distraction osteogenesis provides a useful tool for reliable and predictable correction of osseous defects with vascular bone in patients with congenital anomalies of the hand.

Acrocephalosyndactylia↗

Total joint arthroplasty. The hand.

Joint replacement in the hand has developed on the basis of improved understanding of the pathophysiologic and the biomechanical factors involved in normal and diseased joints. Compromises are required in prosthetic design and surgical reconstruction, but function and appearance in deformed hands are susceptible to surgical improvement.

Arthritis, Rheumatoid↗

Swan neck deformity in rheumatoid arthritis of the hand.

Swan neck deformity is not a single entity. Significantly different types of swan neck deformity are found, each demanding careful clinical evaluation and specialised treatment. This paper discusses the pathomechanics of swan neck deformity and presents a classification upon which rational treatment can be based. The surgical treatment of each variant is briefly outlined.

Arthritis, Rheumatoid↗

Arthropathy of hands and feet in systemic lupus erythematosus.

Hand and foot radiographs of 51 patients with systemic lupus erythematosus (SLE) were examined prospectively and correlations made between radiological, clinical and serological data. At least one abnormality was present in the hands of 53% and in the feet of 66.7% of patients. Jaccoud's type arthropathy affected the hands of 12% and the feet of 8% of patients. Erosions of the ulnar styloids were common (27.5%) but their presence did not correlate with any clinical or laboratory feature of SLE. Antibodies to U1 RNP correlated with hand deformity, while severity of radiological damage related mainly to disease duration.

Adolescent↗

Pediatric mutilating hand injuries.

Mutilating hand injuries in children are a devastating problem. With aggressive efforts at replantation and revascularization, methodic debridement, timely soft tissue coverage, and early mobilization, however, the results in these unfortunate children can be quite rewarding. The child often does well with the functional aspect of recovery and rehabilitation but will probably hide his or her deformed hand from friends and family. These children generally become more shy and reserved. The parents are the key to rehabilitation. A good relationship between the parent, the physician, and the hand therapist is essential for the best result. Interestingly, the parents who are the most demanding on the staff during the initial emergency period are often the most appreciative parents and their children often achieve the best result. Conscientious parents are the best advocates for their children. Obviously, the prevention of these devastating injuries is much preferable to extraordinary heroic reconstruction. Unfortunately, some injuries are inevitable. It is nearly impossible to create an absolutely hazard-free environment for children. Potential injuries can be avoided, however, simply by keeping hazardous machines and equipment out of the reach of the child and by keeping children out of the potentially dangerous workplace.

Amputation, Traumatic↗

Primary surgical management of the deeply burned hand in children.

Primary burn excision with immediate wound closure by skin grafting was used as the initial definitive treatment for deep burns of the hand in 30 consecutive children. The results are compared with an additional group of 30 children whose burns were treated with AgNO3 alone. There is a significant difference in all categories examined between the excised and the nonexcised group. Hand deformities, secondary reconstructive procedures, and morbidity were significantly reduced in the excised group. Excision provided improved functional and cosmetic results.

Adolescent↗

Peripheral nerve involvement in the Rett syndrome.

Clinical and neurophysiological studies were performed on 4 girls aged 9 to 19 years with the Rett syndrome (RS) (stages II and III). In addition to constant foot deformity (pes planus) and brisk tendon reflexes in the lower limbs, they showed muscular atrophy of the calves and mild atrophy of the extensor digitorum brevis muscle. Motor NCVs were within normal values for age, but distal latencies in two girls and compound action potential in one were abnormal, suggesting mild distal, predominantly motor neuropathy. The upper limbs showed nothing abnormal except for mild hand deformities. Neuropathology in 2 autopsy cases (aged 11 and 17 years, stage IV) and sural nerve biopsies in three girls aged 2, 3 and 17 years, all clinical stage II-III without inanition, revealed mild distal axonopathy without demyelination. Selected axons in sural nerve of a girl aged 17 and in the dental pulp of a girl aged 9, both at clinical stage II, showed increased numbers of neurofilaments. Skeletal muscle in two girls aged 11 and 17 years (both stage IV) exhibited mild alterations in the sarcoplasmic reticulum with occasional disordered myofibrils and tubular structures in the Z-filaments, but no neurogenic atrophy or abnormal mitochondria. The reported data do not support spinal denervation (motor neuron degeneration) in RS, but suggest late onset denervation due to mild distal axonopathy of hitherto unknown origin.

Adolescent↗

Congenital constriction band of the upper arm: the role of three-dimensional ultrasound in diagnosis, counseling and multidisciplinary consultation.

Constriction band syndrome represents a sporadic condition that may result in amputations, constrictions and other deformities of the fetal limbs and body. Prenatal diagnosis by two-dimensional ultrasound has been reported. We present a case of constriction band involving the upper arm in which the assessment by three-dimensional ultrasound significantly contributed to the diagnosis and the multidisciplinary counseling. In fact, multiplanar imaging and surface rendering allowed a clear depiction of the extent of the constriction, the club-hand deformity and the relationship between the amniotic band, the cord and the fetal limb. This case represents a unique and effective application of three-dimensional ultrasound in prenatal diagnosis.

Amniotic Band Syndrome↗