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Jaccoud's syndrome. A nosographic entity associated with systemic lupus erythematosus.

Six Scandinavian cases of Jaccoud's syndrome, resulting from systemic lupus erythematosus (SLE) are described; 1 patient suffered from both SLE and rheumatoid arthritis (RA). Clinically, the patients showed hand deformities similar to those of RA with pronounced ulnar deviation and swan-neck configuration. Clinically, however, the hands in cases of Jaccoud's syndrome differ from those in RA because the deformities develop more slowly, without much pain, and hand function is well-preserved. Radiologically, subluxation develops in the metacarpophalangeal joints without the typical rheumatoid joint-associated osseous destruction. The presence of erosions and hooks are exceptional and, if present, they are not typical of RA. It is important that Jaccoud's syndrome be recognized and differentiated from RA because an incorrect diagnosis could lead to a choice of therapy inappropriate for patients with SLE.

Adult↗

Allopurinol-associated hand and foot deformities in chronic tophaceous gout.

Telescoped digits of the hands and feet developed in a 69-year-old male with severe chronic tophaceous gout during allopurinol treatment. Extensive osseous tophi, resorbed rapidly during therapy with this xanthine oxidase inhibitor and not replaced by new bone matrix, were responsible for the deformity. Several hypotheses are advanced to explain the failure of bone erosions to heal.

Aged↗

Development of a plastic surgical teaching service in a women's correctional institution.

A plastic surgical teaching service has been organized at a women's correctional institution to provide a previously unrecognized medical need and to serve a valuable educational function for our residents in training. A total of 177 surgical candidates demonstrating a wide range of reconstructive problems including tattoos, scars, keloids, neoplasms of the skin and hands, deformities of the face and breasts, and numerous disabilities of the hands were identified among 241 inmates requesting examination. Thus far, 116 operative procedures have been performed on 101 patients. Patient acceptance has been high, and the support of prison authorities has been enthusiastic. Persistent efforts to convince legislators of the wisdom of supporting this program financially have only been partially successful and will require further accumulation of sociologic data bearing on the rehabilitative potential of the detained patient with a correctable deformity. Meanwhile, residents in training gain maturity as they provide a very "private" type of service for what has traditionally been considered a very "public" population of patients.

Consumer Behavior↗

Surgical treatment of thoracic deformity in Poland's syndrome.

In 1841, Poland described congenital deficiency of the pectoralis major and minor muscles associated with syndactyly. This syndrome is a spectrum, often involving chest wall and breast deformity as well. Identification of the various musculoskeletal components involved permits optimal thoracic reconstruction in the small proportion of patients who will require it. From 1955 to 1988, 75 patients (40 males and 35 females) with Poland's syndrome were treated or evaluated. Patients with isolated deficiencies of the pectoral muscles, breast, or hand deformity were excluded. The complex was right-sided in 44 patients, left-sided in 30, and bilateral in one. The pectoralis minor and the costal portion of the pectoralis major muscle were absent in all patients. Hand anomalies were present in 50 patients. Athelia and/or amastia were noted in 37 patients. In ten patients, the rib cage deformity required reconstruction, and in three cases, rib or cartilage grafts were needed for complete repair. Often unappreciated in these cases is the significant rotation of the sternum toward the involved side and contralateral carinate deformity. Correction is achieved by bilateral subperichondrial costal cartilage resection and sternal osteotomy (seven of ten patients), thus allowing anterior displacement and orthorotation of the sternum. Chest wall reconstruction must be tailored to the requirements of each patient. No intraoperative or postoperative complications occurred in these ten patients. In males without rib cage deformity, generally no treatment is required to replace the absent pectoral muscles, although in two cases rotation of the latissimus dorsi muscle was performed. In all females, reconstruction of the ipsilateral breast is required at full development.(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormalities, Multiple↗

An unusual unilateral deformity of the hand: a case report.

A rare case of a congenital unilateral deformity of the right upper extremity is described consisting of: absence of the carpal bones except the capitate and hamate, absence of the first three metacarpal bones and phalanges and a thumb-like extension to the palm of the dislocated radius. No hereditary family history was found.

Child↗

Early surgical intervention in Apert's syndactyly.

The hand deformities in Apert's syndrome are among the most complex congenital hand problems to reconstruct due to the skeletal malalignment, symphalangism, and progressive functional deficits with growth. Ten children with Apert's syndactyly underwent early surgical intervention (mean age 8.4 months) as part of a staged program of digital separation leading to completion of both hands by the age of 2 years. The incidence of major complications was low, rate of revision (18 percent) acceptable, and the aesthetic result very satisfactory. Long-term functional results tend to reflect the effect of progressive intrinsic disease despite preservation of skeletal alignment and digital form with growth. However, the aesthetic improvement and potential for greater functional gains at a young age support the concept of early surgical intervention in this syndrome.

Acrocephalosyndactylia↗

Acrocephalosyndactyly -- the coalesced hand.

Described nearly a century ago, Apert's Syndrome (A.S.) is a rare disorder affecting 1/80,000 children, the mode of inheritance is autosomal dominant or sporadic. Apart from the more obvious skull malformations, extensive and complex hand deformities create several problems for the child's interaction with its environment, preventing normal intellectual development and generating psychological problems as severe as those caused by the craniofacial abnormalities. The deformities seen here are some of the most complex of all congenital hand malformations. Between January 1987 and January 1999, 71 children presenting with hand syndactyly were treated at the Hospital de Dona Estefania (H.D.E.), a total of 177 syndactylies. Of those, 51 had no associated illnesses, 14 had other conditions and six had A.S. All six cases were sporadic; one child had a cleft palate and another suffered from epilepsy. The timing at which the various surgeries were performed was diverse, however, there has been a recent trend towards earlier interventions in order to obtain a functional hand before the age of two. Aesthetic results vary a lot depending on the complexity of the lesions and we sometimes accept a three or four-digit, but functional hand. We achieved hands with a function of 40 to 65 % in all our patients before school age. Due to A.S.' rarity, an effective use of resources and good results can only be obtained in a specialised centre with a multidisciplinary team.

Acrocephalosyndactylia↗

Preventive surgery for congenital deformities of the hand.

Science is on the verge of major changes in the treatment of congenital deformities of the hand. The first changes will be technical and will involve the increasing use of microsurgery, laser surgery, electricity, and fiber optics. Improved imaging techniques will permit earlier and more precise diagnosis of tissue abnormalities. The second phase will be antenatal preventive surgery, first on the fetus and later on the embryo. The third phase will be the prevention of congenital deformities by bioengineering.

Abnormalities, Drug-Induced↗

Flexor digitorum superficialis tendon transfer for intrinsic replacement. Long-term results and the effect on donor fingers.

This study is a review of 127 hands in 100 patients in whom one or two FDS tendons were used to correct claw-hand deformity and/or loss of opposition of the thumb. In lumbrical replacement the results were graded as excellent in 16 hands (21%) and good in 43 hands (57%). For opponensplasty the results were excellent in 26 hands (32%) and good in 42 hands (51%). Possible defects that can develop in the donor finger are: swan-neck deformity, flexion posture of the DIP joint, not as part of the swan-neck deformity, check-rein deformity or flexion contracture, and insufficient finger flexion. Of the 158 fingers swan-neck deformity was seen in 15%, DIP flexion in 29%, check-rein deformity in 26% and insufficient finger flexion in 18%. The latter occurred with another defect. In 48 fingers (30%) no defects were observed.

Adult↗

New instrumental set for the assessment of the hand functionality.

Hand motor deficits have been widely investigated, and several devices have been proposed for the selective and accurate study of specific hand motor tasks. Most studies have focused on the four, long fingers. The thumb function, although extremely important for the performance of most daily activities involving the hand, has scarcely been documented. A set of general-purpose, instrumental measuring devices has been designed and constructed at the authors' laboratory to measure and monitor the force each finger exerts separately, under isometric conditions, during pressing tasks. More generally, it is meant for the functional evaluation of the normal hand in different postures, but it also provides reliable measurements of the injured or deformed hand. The instrumental set is suitable for both biomechanical research and clinical applications. Effectively integrated with a visual feedback tool, it could be exploited in delivering and monitoring custom-designed rehabilitation programmes. The characteristics of the force transducers (range 0-100 N) were: inter-axis crosstalk < 4%; non-linearity < +/- 0.4% f.s.; hysteresis < 0.3% f.s.; overall accuracy +/- 1% f.s. The overall measurement system resolution was better than 0.1 N, and the keys response to the mechanical shock (acquired at 10 kHz) showed a resonance frequency of about 1 kHz. It was observed that the thumb contributed more than 30% of the overall pressing force.

Adult↗

Bilateral absence of the ulna in twins as a manifestation of the split hand--split foot deformity.

Bilateral absence of the ulna and ulnar rays in twin female infants are described as a manifestation of the split hand--split foot deformity (ectrodactyly). Family history revealed the father had a unilateral split hand. Given the wide variability of expression of this disorder and the availability of prenatal diagnosis, there is a need for obstetricians and pediatricians to recognize parents with this malformation and appreciate the potential for severely affected offspring.

Diseases in Twins↗

Clinical application of posterior calf fascio-cutaneous pedicle flap in leprosy foot ulcer.

Lower extremities of the leprosy patients are characterized by the decreased sensation of the foot from peripheral nerve damage. As a result, repetitive foot ulcers are frequently seen. When these repetitive shallow ulcers become infected, they result in deep soft tissue ulcers and even osteomyelitis. The treatment of these ulcers in leprosy is a challenging problem both to the patient himself and to medical personnel. Conventional treatment methods for these ulcers include, according to the wound condition, skin graft, local flap, long-term cast immobilization, wedge resection of the joint and even amputation in severe cases. But all these methods have the disadvantages of frequent recurrences, long hospitalization periods and permanent foot deformities. Recently in Korea, despite the decreased occurrences of new patients and the decreased frequencies of facial and hand deformity formation due to the active early diagnosis and treatment, there still exists a large number of foot deformities resulting from decreased foot sensation. Moreover, treatment modalities are not definitely established. In fact, there are many environmental difficulties in applying the free flap transfer to the foot ulcer of leprosy patients in leprosy sanatorium as opposed to treating the non-leprosy patients. And so, from 1990 to 1993, we performed 6 superior-based posterior calf cross leg flap transfers and 1 distal-based flap transfer, under spinal anesthesia, referred by the leprosy sanatorium for deep foot ulcers. We obtained favorable results in 6 patients and partial necrosis in a patient who received distal based flap transfer.

Aged↗

[Study of erythrocyte deformability using visco-diffractometry (ektacytometry) in hereditary hemolytic anemias].

Deformability is a basic characteristic of red blood cells; its regulating factors are presently well known (surface volume ratio, internal viscosity, viscoelastic properties of the membrane). The contribution of each of these factors in the pathogeny of hemolytic anemias has not been fully established. We report 64 hereditary hemolytic anemias in which we studied the deformability, using a visco-diffractometric method (ektacytometer) on whole blood and various subpopulations separated by density. Comparison of the response of the cells submitted to isotonic and hypotonic media allows the determination of the respective contribution of viscosity and surface volume ratio. Deformability is always decreased in red blood cell membrane diseases and in homozygous or double heterozygous hemoglobinopathies; on the other hand, deformability is normal in all the enzymopathies studied. This decrease could be due either to one regulation factor (increase of internal viscosity easily demonstrated by this method in hereditary xerocytosis and hemoglobin CC) or to several parameters: increase of internal viscosity and decrease of surface volume ratio in hereditary spherocytosis.

Anemia, Hemolytic, Congenital↗

Transverse deficiency of the hand in one of monozygotic twins.

In this paper, we report on one male infant of twins with a left hand anomaly. The hand deformities were diagnosed as so called "congenital amputation" which is a synonym of "transverse deficiency" in Swanson's classification. The left hand showed spontaneous amputation at the level of the palm. There were two rudimentary digits on the stump, and an extremely hypoplastic thumb and little finger. The other twin had no anomaly. The results of laboratory findings on blood groups and HLA types suggest that these twins are monozygotic. From these facts, we considered that environmental factors are more important than genetic ones in the pathogenesis of transverse deficiency and symbrachydactyly of the hand.

Blood Grouping and Crossmatching↗

Homodigital flaps--especially for treatment of the burned hand.

Burn deformities involving the hand can cause a devastating impairment that often requires ingenious solutions to improve function. Fortunately, a skin shortage usually can be expeditiously replaced by autogenous skin grafts; otherwise, a vascularized flap may be imperative. If that is so, use of a donor site from the digit itself would always be preferable, because normal sensation potentially could be maintained with similar thin, extensile, and durable tissue. The sides of the finger often have been protected from the thermal injury and are available for use as homodigital flaps. Although technically this form of homodigital flap is an axial flap because it incorporates dorsal digital artery branches, the proper digital artery is always spared. The immediate transfer of a homodigital flap completes the desired reconstruction in a single stage, which allows early initiation of hand mobilization and rehabilitation.

Burns↗