Congenital unilateral upper limb muscular hypertrophy associated with contracture of an extrinsic extensor tendon

We report a case of congenital unilateral upper limb muscular hypertrophy which presented with loss of extensor tendon excursion of a single digit in late adolescence. The reason for the problem arising is not clear and is not clearly explained by the underlying congenital pathology.     

Recording congenital differences of the upper limb

Consistent classification of congenital differences of the upper limb is of paramount importance for the study of the pathogenesis. To overcome the inconsistencies of present classifications, a non-classifying recording method has been developed. This method records individual aberrations, including bone and soft-tissue defects. Between 1996 and 1998, a prospective study was performed to validate the method. Two hundred and thirty-one patients with upper limb differences were assessed, and all individual aberrations were recorded. These data can be transferred to any classification. It is concluded that the presented method will allow consistent grouping of patients without losing details about simple and complex differences.     

The prognosis in congenital lower limb hypertrophy.

Twenty-eight patients with congenital total hypertrophy and ten patients with lower limb hypertrophy with congenital vascular abnormalities are reviewed. The pattern of increase in leg length discrepancy during growth and its influence on surgical management is discussed and the clinical features of the affected limbs are described. In congenital total hypertrophy the maximal increase in leg length discrepancy of more than 2.5 cm at age 4 years are likely to develop significant limb overgrowth that will require eventual surgical correction. In patients with congenital vascular abnormalities the change of leg length discrepancy was variable in degree and unpredictable in pattern, even in those with similar venous anomalies. The outcome for the limb was detergiography.     

A new prosthesis for the upper limb

We describe the development of a body-powered upper limb prosthesis fitted with a cosmetic functioning hand. The features and advantages of the new design--the PMR (Princess Margaret Rose) prosthesis--are discussed and the clinical results in 15 patients fitted with the prosthesis after above-elbow amputation are described.     

The anatomical base of unilateral external fixation in the upper limb

Unilateral external fixation requires an anatomically sound implantation of screws into the upper extremity. Detailed knowledge about the anatomical situation in the areas of pin implantation is of great importance. This paper focuses on relevant anatomical landmarks when implanting screws for external fixation in the humerus, the elbow, the forearm and the hand by studying anatomical specimen.     

Congenital isolated upper limb hypertrophy with hand abnormality--a report of 2 cases

Two cases of congenital isolated hypertrophy of the left upper limb with different hand deformities are described. A 4-year-old girl had splayed fingers and an abducted thumb due to anomalous muscles. Excision of these muscles corrected the deformity. The other, an 8-year-old boy, had severe ulnar drift of the fingers (windblown-like hand). He had corrective osteotomies of the second and third metarcarpals and reconstruction of the collateral ligaments. The deformity was corrected and at the latest follow up there was with no recurrence of the deviation. Both cases regained good hand function.     

An unusual association of the windblown hand with upper limb hypertrophy.

An eight-year-old boy presented with a congenital windblown hand deformity associated with partial hypertrophy of that arm. Many of the classical features of the windblown hand syndrome described in previous papers were absent. In this case, the primary cause of the ulnar-drift deformity was an anomalous epiphysis at the base of the second metacarpal. Surgical correction was readily obtained by osteotomy at the base of the metacarpal combined with soft tissue re-alignment.     

Abnormalities of the spine in relation to congenital upper limb deficiencies

A retrospective study was performed to determine the incidence of spinal abnormalities in patients with skeletal defects of the upper extremities. The incidence of scoliosis in the patient population studied was 16%, and spinal abnormalities of all types were present in 18% of the population. Patients with bilateral amelia had a 100% incidence of scoliosis. Those patients with unilateral amelia had a 50% incidence of scoliosis. Patients with ulnar and radial hemimelias had an incidence of scoliosis of 20 and 13%, respectively. The transverse complete and partial hemimelics had an 18% incidence of scoliosis and the phocomelic group had an incidence of 11%. The patient population studied had an increased incidence of scoliosis compared with the general population. The patients with amelia had a particularly high incidence of scoliosis. There was no relationship between the side of the deficiency and the direction of the curve. The age of onset was not found to be related to the progression of the curve. The results of bracing were poor, due to patient rejection of the brace. All patients with upper limb deficiencies warrant close observation throughout growth for the development of scoliosis.     

Treatment of congenital unilateral upper eyelid retraction with a marginal myotomy procedure

Two children with congenital unilateral upper eyelid retraction were treated successfully with surgery at 2 years of age. Excellent results were obtained using a modification of Grove's marginal myotomy procedure for lengthening the levator palpebrae superiorus muscle. To our knowledge, early surgical treatment for this rare idiopathic disorder has not been reported previously.     

An estimate of the population frequency of congenital malformations of the upper limb

The first report, based on information from members of the Congenital Malformations Committee of the International Federation of Societies for Surgery of the Hand, is presented.     

Preliminary assessment of three new designs of prosthetic prehensors for upper limb amputees

At present, upper-limb amputees have a basic choice of a hook or a hand for the prosthetic prehensor. In the USA about two-thirds of upper-limb amputees wear hooks and about one-third wear hands. Either of these options represents a compromise. The hook is more functional and the hand is more cosmetic. Some amputees solve the dilemma by having one of each and interchanging them as work and social situations dictate. However, they would prefer to have one acceptable prehensor, and they want one which is functional, is attractive, and does not necessarily have to look like a hand. In an attempt to meet the desires of upper-limb amputees, three prosthetic prehensors or "terminal devices" have been designed and developed into models. This article describes the preliminary assessment of these new designs.     

The Krukenberg procedure: a method of treatment for unilateral anomalies of the upper limb in Chinese children

Eight Chinese patients with congenital absence of the hand were treated with the Krukenberg procedure to provide a pinching function of the forearm bones. The rehabilitation program was simple and the functional results were satisfactory. We have selected three cases to illustrate the Krukenberg procedure and the indications for this operation, a procedure that is particularly suitable for the Chinese who are culturally adapted to chopstick functions.     

Gross measures of exercise-induced muscular hypertrophy

STUDY DESIGN: Pretest-posttest, single factor design. OBJECTIVES: To compare several indices that might be used to depict muscle size. BACKGROUND: The particular strategy used during heavy-resistance training may determine the magnitude of hypertrophic adaptations. At the same time, assorted measures supposedly reflecting muscle size may provide different results. METHODS AND MEASURES: Four groups of men (n = 38, mean age = 21.1 years, SD = 2.1) were exposed to conditions designed to elicit differential hypertrophic adaptations following 21 sessions of squat training. Three of the groups performed 4 sets of multiple repetitions maximum (RM): group I, 3-5 RM; group II, 13-15 RM; and group III, 23-25 RM. A control (C) group did no formal physical training. Tests used to represent muscle size included body weight, thigh girth, net thigh girth, and quadriceps femoris and hamstring thicknesses via B-mode ultrasound. RESULTS: Changes in the groups subsequent to training were similar for body weight and hamstring thickness. Results differed for the remaining 3 dependent variables (mean +/- SD): thigh girth was greater in groups II (1.42 +/- 1.00) and III (1.35 +/- 1.16) than in group C (0.24 +/- 0.69); net thigh girth was greater in groups II (1.33 +/- 0.77) and III (1.40 +/- 1.03) than in group C (0.10 +/- 0.84); and quadriceps femoris thickness was greater in all 3 training groups (I, 0.61 +/- 0.54; II, 0.43 +/- 0.30; III, 0.55 +/- 0.39) than in group C (0.05 +/- 0.11). CONCLUSIONS: Observed muscle mass change following heavy-resistance training is dependent upon both the training intervention and tool used for measurement.