We report two 11 and 14 year old girls with bilateral ULNAR CLUB HAND deformity, who have the familial form of the disease. Their mother and 8 other family members are affected by the same deformity, the congenital form of deformity was first reported by Robert in 1886 and since then, one more case has been reported in the literature. Our report to our knowledge is the first that reports the case in 11 members of 3 generations of a family.

Background: ULNAR CLUB HAND (ULNAR deficiency) is a rare congenital disorder of the upper extremity. In the Flatt series among 2758 congenital disorders of upper extremity only 28 cases of ULNAR deficiency were reported. Due to its rarity and variations in presentation current data in the management of the deformity is very limited. Here we present our experience and results in comparing management of ULNAR deficiency.Methods: We include all of the ULNAR CLUB HAND patients (five boys and one girl with seven involved extremities) from 1993 to 2006. After recognizing the type of deformity the classic management approach was performed that was splinting in corrective position until six months of age and then anlage resection. Syndactyly release was done in appropriate age according to involved rays, other operations for restoration of apposition was done after 18 months of age. Two of our patients were neglected, the first one was a 12 years old boy without any ULNAR deviation but with syndactyly of the remaining rays and the other was a 32 years old male with severe ULNAR deviation and partial syndactyly who is a skillful worker. We determined the effect of anlage resection on ULNAR deviation of the wrist and restoration of opposition and syndactyly release on function of the limb.Results: In short term follow up, anlage resection was effective in prevention and correction of ULNAR deviation, however the deformity was partially recurred later. Surprisingly, the function of the limb was not significantly affected with the extent of the deformity. On the other HAND, the operations used for opposition of the thumb like first metacarpal rotational osteotomies and tendon transfers for powerful opposition, were more effective in the HAND and also limb function compared with anlage resection alone.Conclusions: Due to our observation of the neglected cases, the most important factor in the function of the HAND is the function of the thumb, thus we believe that restoration of opposition and syndactyly release may be more effective than anlage resection on limb function in ULNAR CLUB HAND patients.

A 17- year's old woman with ULNAR dysmelia presented with right elbow, forearm, and wrist deformity. Xray revealed a partial absence and deficiency in longitudinal development of the distal half of her right ulna associated with dislocated radial head and bowed radius toward the ulna. She had a functional elbow, but her HAND function due to severe ULNAR deviation was compromised. In order to increase her limb function and improve the appearance, the forearm was reconstructed by one bone forearm procedure. Cosmetic and functional improvement was achieved in spite of limitation of pronation - supination in forearm.

Congenital radial CLUB HAND is an intercalary or terminal deficiency characterized by radial deviation of the HAND, marked shortening of the forearm, and generalized underdevelopment of the extremity. We performed centralization of the ulna in 12 forearms of 10 patients with severe congenital radial CLUB HANDs. The mean follow up was 42 months after operation (24 to 68 months). The mean age at the operation was 16.8 months (7 to 42 months). The mean angular deformity before operation was 100 degrees (75 to 135). The angular deformity was corrected in all patients initially, but in the latest follow up the mean residual angular deformity was 19.58 degrees (5 to 45). The mean correction of angular deformity was 79.57 degrees (55 to 120). Range of motion in the wrist increased significantly after operation in both flexion-extension range and radial-ULNAR deviation range (P <0.05).Stability of the wrist was acceptable in the latest follow up in all patients. We recommend that correction of congenital radial CLUB HAND by centralization should be performed in three stages. The first is preoperative splinting and corrective casting, the second centralization of the ulna and correction of angular deformity, and the third which may be longer than previous stages is postoperative splinting and bracing. This stage of treatment should be continued till skeletal maturity.

BACKGROUND: Radial CLUB HAND includes wide spectrum of malformations that involve radial side of forearm. Conservative treatments are recommended to the milder types and surgical interventions specially centralization for the severe forms. Recurrence after centralization is one of the challenging problems in this anomaly. In this study, we assessed the recurrence rate of centralization in radial CLUB HAND surgery in long term follow up.METHODS: In this retrospective study, we reviewed the records and radiographies of 9 patients with radial CLUB HAND who underwent centralization to correct the deformity in the HAND surgery department. Patients' age, type of the thumb anomaly, associated anomalies in other systems, involved side, type of radial CLUB HAND, HAND-forearm angle preoperative, post operative and in the last follow up visit, and centralized wrists motion range in the final follow up visit were all assessed and reported.RESULTS: Eleven cases of radial CLUB HAND in nine patients underwent centralization. Mean age at the time of the centralization was 17 (6-72) months and mean follow up was 90 (48-170) months. Preoperative HAND-forearm angle was 75 (30-110) degrees, immediate postoperative angle was 25 (15-35) degrees and in the last follow up visit it was 52 (40-60) degrees. The amount of correction was 66% and loss of correction in long term was 54%.CONCLUSION: Centralization still can be a standard procedure in treatment of patients with radial CLUB HAND with acceptable results.

Background: Congenital radial CLUB HAND (RCH), as a rare congenital deformity of the upper extremity, is characterized by a wide spectrum of malformations including radial deviation. Centralization surgery is the standard treatment for severe cases that have been associated with a high rate of recurrence. This study reports the long-term results and recurrence rate of radial deviation following the centralization surgery of RCH. Methods: The medical records of 13 congenital RCH patients (16 HANDs), who underwent centralization surgery, were reviewed retrospectively. HAND-forearm angle (HFA), HAND-forearm position (HFP), and ULNAR bow (UB) were used to assess forearm angles. Results: The mean age of the patients was 19. 4± 8. 9 months, and their mean follow-up was 62. 1± 39. 9 months. The mean HFA correction was 29. 4° ± 23. 9° , the mean HFA recurrence was 13. 3° ± 13. 7° , the mean correction of HFP was 13. 4± 7. 3 mm, and the mean recurrence of HFP was 1. 4± 2. 8 mm. The mean UB showed 7. 6° ± 12. 5° correction immediately after surgery and a further 3. 6° ± 7. 3° at the last follow-up (overall 11. 2° ± 17. 6° ). A number of 12 out of 13 parents were completely satisfied with the results. Conclusion: According to our results, an acceptable long-term result is expected after the centralization surgery of RCH. However, the risk of the recurrent radial deviation is high and needs to be optimized in future investigations.

Background: Two main muscles studied in the HAND for evaluation of median nerve injuries are opponens pollicis (OP) and abductor pollicis brevis (APB). However, Riché-Cannieu communicating branch (RCCB) may limit the use of these muscles in electrodiagnosis. This condition is confusing in the case of median nerve injuries. This study was conducted to evaluate the prevalence of RCCB. Methods: Twenty-three consecutive cases of complete median nerve injury were studied. Evoked responses via stimulation of median and ULNAR nerves in the wrist and recording with needle in the thenar area were studied. Results: Of the patients, 82. 6% exhibited RCCB. In 14 (60. 8%) cases the OP and in 19(82. 6%) cases APB was supplied by the ULNAR nerve. Conclusion: RCCB was detected to be 60. 8% in OP and 82. 6% in APB, so OP is preferable to APB in the study of median nerve.