Ulnar Deficiency Research Articles

Congenital Anomalies of the Elbow Joint: Clinical Features and Classification

AbstractIn order to examine the distribution of different types of congenital anomalies of the elbow joint and discuss their classification, a total of 94 patients, 96 congenital anomalies of the elbow joint, were analyzed. They were classified according to the elbow deformity itself as follows; radio‐ulnar synostosis in 59 cases (61%), radial head dislocation in 23 cases (24%), ankylosis of the elbow joint in eight cases (8%), severe flexion contracture of the elbow joint in three cases (3%), and complete or partial aplasia of the elbow joint in three cases (3%). On the other hand, congenital elbow deformities can be classified into three types according to the associated anomalies. In 78 patients, in which congenital elbow deformity appeared as an independent deformity, there were radio‐ulnar synostosis in 56 patients, radial head dislocation in 19 patients, ankylosis of the elbow joint in two patients, and hypoplasia of trocholea in one patient. In the 13 patients, which congenital elbow deformity appeared as a part of congenital upper limb anomalies, the underlying congenital anomalies of the upper limb were ulnar deficiency in eight patients, radial deficiency in two patients, transverse deficiency in two patients, and phocomelia in one patient. In five patients, elbow deformity appeared as a part of congenital syndromes. Classification according to associated anomalies seems to be more suitable in order to examine the real distribution of different types of congenital deformities of the elbow joint as a part of the upper limb.

Preliminary soft tissue distraction in congenital forearm deficiency

Four patients (five limbs) were treated with preliminary soft tissue distraction using an Orthofix external distractor. The patient's ages ranged from 6 months to 14 years. Forearm deficiencies treated included partial absence of the radius (one patient), radial aplasia (two patients, three limbs), and partial absence of the ulna (one patient). Soft tissue distraction (mean, 55 days) was continued in radial deficiency until the hand could be passively centralized without residual radial deviation, allowing centralization to be accomplished through a single mid-dorsal incision. Mean measurement of distraction for the radial deficiencies was 1 cm. In the ulnar deficiency, soft tissue distraction was carried out until the cut radius was distal to the partially absent ulna (98 days) allowing for the creation of a one-bone forearm using the entire radius. Distraction (1 mm/day) was done by the children's parents with no distractor removed because of patient intolerance. All radial deficiency limbs remained centralized at a mean followup period of 14 months.

Clinical features and treatment of congenital fusion of the small and ring finger metacarpals

Nine cases of congenital fusion of the small and ring finger metacarpals are reported. The underlying etiology of congenital fusion of the small and ring finger metacarpals was ulnar ray deficiency in four hands, cleft hand in two hands, and symbrachydactyly in one hand; two hands were unclassifiable. X-ray films revealed that the small finger was hypoplastic and accompanied by brachymesophalangy in all cases. It was supposed that the main cause of congenital metacarpal fusion was the deficit of mesenchymal cells. Treatment for metacarpal fusion included splitting the metacarpal fusion and autogenous iliac bone graft in two hands, deepening of the interdigital space in two hands, and amputation of the small finger in one hand. Satisfactory improvement in appearance was achieved in three hands.

Clinical and Experimental Studies on Teratogenic Mechanisms of Congenital Absence of Digits in Longitudinal Deficiencies*

ABSTRACT: In order to clarify teratogenic mechanisms of congenital absence of the digits in longitudinal deficiencies, ulnar deficiency, radial deficiency, central polydactyly, osseous syndactyly and cleft hand (central deficiency) were analyzed. Then, the same anomalies induced in rat fetuses with myleran were analyzed. In ulnar and radial deficiencies, the more severe was the degree of finger deficit, the more severe was the arrest of the forearm bone. The critical periods of these anomalies were earlier than those of other anomalies. Ulnar deficiencies were induced only in Gun: Wistar rats and radial deficiencies only in WKAH/Hkm rats. A genetic factor may influence the teratogenesis of ulnar and radial deficiencies. From histological analysis of tibial deficiency, it may be suggested that the cause of absence of digits in longitudinal deficiency is closely related to a deficit of mesenchymal cells. The analysis of skeletal changes of central polydactylies, osseous syndactylies and cleft hands suggested that there seemed to be the cleft hand formation process from osseous syndactylies and central polydactylies. These anomalies were often observed in both hands in various combinations. They could be induced by the same treatment at the same developmental stage in rats. The teratogenic mechanism of cleft hand seemed to be failure of induction of digital rays in the hand plate.

Heterotopic Finger Transfer in Ulnar Ray Deficiency Associated with Contralateral Postaxial Polydactyly: A Case Report

A case of type-I ulnar ray deficiency, combined with postaxial polydactyly of the opposite hand, is described. Heterotopic transfer of the supernumerary finger was performed, in order to improve function in the defective hand and to treat the hexadactyly of the other hand. A combination of these malformations has apparently not previously been reported in the literature.

Arteriographic findings in radial and ulnar deficiencies

The arterial pattern in 13 cases of radial deficiency and six cases of ulnar deficiency was studied by arteriography. The radial artery was absent or hypoplastic in 84.6% of cases of radial deficiency and in 50% of cases of ulnar deficiency. A persistent median artery was seen in 76.9% of cases of radial deficiency and in 16.7% of cases of ulnar deficiency. In 12 hands, the deep palmar arch was small or absent, depending upon the degree of radial arterial dysplasia. The digital artery to the deficient digits was missing in some cases. The persistence of an embryonic vascular pattern in cases of radial deficiency and ulnar deficiency with dysplasia of the ulna suggests that the teratogenic injury may have been simultaneous, whereas ulnar deficiency without dysplasia of the ulna was not associated with arterial abnormalities in the forearm.

The treatment of longitudinal ulnar deficiency

Longitudinal ulnar deficiency, whose detailed anatomy is varied, is often accompanied by other abnormalities and appears as part of several syndromes. The history of its classification is reviewed, and the treatments, which have been offered, described. Follow-up of a series shows that the function achieved is good and is optimised by aids, occupational therapy and some hand surgical procedures. The more elaborate surgical reconstructions are unlikely to be beneficial.

Experimental study of radial ray deficiency

The teratogenic mechanisms of radial ray deficiency were investigated. Radial ray deficiencies were induced in WKAH/Hkm rat foetuses by maternal administration of myleran on day 10, 10.5, or 11 of pregnancy. The skeletal anomalies in rats were similar to those in human beings, suggesting that exposure to some environmental factors at a sensitive period of embryogenesis may cause radial ray deficiency in humans. Ulnar ray deficiency has also been induced in rat foetuses by maternal administration of myleran, so it is suggested that radial deficiency and ulnar deficiency are caused by a common teratogenic mechanism in humans. However, the critical period for radial deficiency is one day later than that for ulnar deficiency in rats, which may contribute to the greater frequency of radial deficiency than ulnar deficiency in clinical cases.

Congenital hand anomalies in Japan: A family study

During the 20-year period from 1968 to 1987, 1024 patients with congenital anomalies of the hand from 1000 Japanese families were examined in our clinic. Of these patients, 204 had relatives with limb anomalies. While postaxial polydactyly showed a 33% level of familial recurrence, symphalangism and Kirner's syndrome showed about 23% recurrence, syndactyly 18%, radioulnar synostosis 9%, radial ray deficiency 8%, cleft hand 7%, and duplicated thumb 5%. None of the relatives had ulnar deficiency or symbrachydactyly.

The carpal bones in congenital hand anomalies: A radiographic study in patients older than ten years

Abnormalities of the carpal bones in 192 anomalous hands of 154 patients older than 10 years were examined. Judging from the time of appearance of the pisiform, there was no delay of carpal bone maturation in these anomalies. There were differences between the carpal bones of three distinct groups: which included central polydactyly, syndactyly, and typical cleft hand; the group which included radial and ulnar deficiency, and the group which included symbrachydactyly and transverse defect. This suggests that there were differences between these groups in the timing and degree of injury to the limb bud or the hand plate in the course of development. It is thought that the first group originates from maldistribution of mesenchymal tissue of the limb bud (or of the apical ectodermal ridge), the second group from defects of that tissue, and the last group from defects of the mesenchymal tissue of the hand plate.

Congenital Ulnar Deficiency

We have reviewed the natural history of congenital ulnar deficiency in 15 limbs in our series and in 185 limbs in the literature. The natural history suggests that there is little progression of deformity. Specific variables for prognosis have not been determined. There are no data that discuss relative ulnar length or relative forearm shortening of the involved extremity. We compared function in our 15 limbs with standards of function in the general population. Based on our findings, treatment must be individualized to maximize function and should be conservative regarding ulnar deviation of the wrist, radial head dislocation, and preservation of forearm pronation and supination. Standard reconstruction of hand anomalies is most rewarding for functional results.

Carpal deviation in congenital ulnar deficiency.

In patients with hypoplasia of the ulna, the role of the fibrocartilaginous anlage of the ulna in the development of deformity is controversial. Between 1971 and 1982, we treated eight such patients with eleven affected extremities. We maintained them on a conservative regimen of static splinting, and surgery was not undertaken unless there was uncorrectable ulnar deviation at the wrist of 30 degrees or more. Resection of the anlage had to be performed in only one patient. Progression of uncorrectable ulnar deviation at the wrist rarely occurs in patients with ulnar deficiency; therefore, routine precautionary excision of the anlage is not justified.

Séquelles fonctionnelles après réparation du nerf cubital: A propos de quarante-neuf cas

The full picture of functional ulnar deficiency, following suture or grafting, was studied and analyzed by two of the authors, who followed this series of patients on a long-term basis. The patients were tested using sphygmometric rubber bulbs in other to determine the overall closing force of the hand (making a fist), and the force of the pinch between the thumb and index ("energetic pinch"). Moreover, radionuclide vascular tests were used in order to find an eventual etiology of the "cold" disease, present in 2/3 of these injured hands. The principal functional deficiencies which bothered the patients and which we were able to demonstrate are as follows: deficient adduction of the thumb: the pinch between the thumb and index is diminished by approximately 50%, impairment, in the spreading of fingers, impairment in the overall force of the grasp, ulnar clawing which may embarrass function of the hand one year after the operation in one fourth of the cases. Therefore it appeared logical to propose an adduction plasty at the same time of repair, whether it was a primary suture or a secondary nerve graft. This adduction plasty uses the flexor digitorum sublimis of the ring finger which is rerouted and passed behind the profondus tendons and brought over to the lateral sesamoid bone of the thumb. This transfer is prolonged and tacked to the extensor pollicis longus in order to prevent the hyperflexion of the proximal phalanx of the thumb of which patients hardly ever complain but which denotes the severeness of ulnar nerve injury. An excellent correlation exists between the severeness of the functional deficiency and the importance of Froment's sign. The study of the other residual deficiencies shows that the restoration of the first dorsal interosseus, the reinforcement of the force of the flexores digitorum profundus of the middle and fourth fingers and of the intrinsics does not seem to be justified on an emergency basis. Zancolli's operation, which has been advocated by certain authors as an emergency procedure, does not seem to be necessary at this stage of repair, but remains a very interesting palliative procedure one year after the nerve repair if still judged necessary. Several clinical examples illustrate the value of long-term follow-up of these operated patients.

Functional status in ulnar deficiency.

Function is the most important consideration in the evaluation of patients with congenital ulnar deficiency. The upper extremity function of 8 patients with ulnar deficiency was evaluated. None of these patients had been treated with surgical procedures directed to the elbow, forearm, or wrist. The functional criteria included: (a) active ranges of elbow, forearm, and wrist motion, (b) power grip, (c) prehension, (d) dexterity, and (e) a patient's activities questionnaire. The averaged total active range of joint motion was 229 degrees or 46% of predicted normal active motion. Power grip averaged 27% of the contralateral extremity, and prehension tests were generally well performed. Timed tests were completed an average of 11.6 s slower than the contralateral control hand. Our patients did not report any deficiencies in bimanual activities. Patients performed most poorly when their congenital anomaly included radiohumeral synostosis or congenital absence, deformity, or contracture of the ipsilateral digits. The radiographic appearances or classification of the ulnar deficiency, in the absence of radiohumeral synostosis, did not correlate well with patient function.

Deformities of the hand and wrist with ulnar deficiency

Deformities of the hand and wrist with ulnar deficiency

Deformities of the hand and wrist with ulnar deficiency

Deformities of the hand and wrist in 26 upper limbs of 20 patients with ulnar deficiency were studied. In eight limbs the ulna was totally absent. In the remainder it was severely or moderately hypoplastic. Abnormalities of the digits were present in all affected limbs. There was ectrodactyly in 25 hands. The thumb was hypoplastic in 21 hands and totally absent in five. Digital deformities also included syndactyly (14 hands) and delta phalanx (six hands). Early syndactyly release, pollicization, and rotational osteotomy of the metacarpals improved hand function in many of these patients. In only three of 26 limbs was there more than 20 degrees of fixed ulnar deviation at the wrist. In 16 patients the distal ulnar "anlage" was not removed. None of these patients developed ulnar deviation of the wrist or progressive deformity at the wrist during growth. Resection of the distal ulnar "anlage" does not appear to be warranted, unless there is progressive deformity of the wrist or elbow or increased bowing of the radius.