ABSTRACT
Introduction: Cleft hand or Ectrodactyly or Split Hand is a rare form of congenital hand disorder in which there is deficiancy of one or more central rays of the hand. The hand presents with a V-shaped gap situated in the centre of the palm. The condition may occur alone or maybe associated with anomalies of foot, syndactly, polydactyly, triphalangeal thumb, transverse bones in the hand or maybe part of Ectrodactyly Ectodermal dysplasia Cleft (ECC) Syndrome. Aims and Objectives: To find out the sex ratio and incidence of laterality (unilateral or bilateral) of cleft hand in North East population of Assam. Subjects and Methods: This study included 31 children between age group of 3 years to 8 years with hand deformities who were brought to the Plastic Surgery Department, Gauhati Medical College, Guwahati for aesthetic correction within a time span of 2 years. Only those cases who had central defects of hand were included & those with other syndromic anomalies were excluded. For this detailed clinical and radiological examination was done to evaluate the extent of bone or tissue involvement and to find out any syndromic association of the condition. Results: Out of 31 cases, 10 cases were had Unilateral and 21 cases had Bilateral Cleft Hands. It was seen that out of 31 cases, 22 were males and only 09 were females indicating that male to female ratio of the deformity is 2.44 .Thus, males were more affected than females. Conclusion: Most of the cases presented with absence of the middle finger and central tissue of the affected hand since birth. The embryological basis has been highlighted to throw light into such a catastrophe where both males and females were affected and showed unilateral or bilateral involvement of the hand leading to cosmetic and functional debility. Therefore, the study revealed that males were more affected than females and bilateral involvement of the hands were common than unilateral involvement
ABSTRACT
Through a congenital cleft hand cleft foot line analysis of clinical manifestations, the mode of inherit-ance, clinical type of 15 patients. It was found that the pedigrees of congenital foot deformity in patients with cleft hand cleft hand split foot crack were typical, and non-syndromic. The disease presents the typical pedigree body autosomal dominant mode of inheritance, clinical manifestations of the great differences between patients, the pres-ence of significant genetic heterogeneity.
ABSTRACT
The variation of cleft hand deformity is so wide that there are many synonyms and classifications for it. Among them the classification of Manske based on the condition of first web space is mostly important in functional perspectives. In type IIb or III of the Manske's classification, in which the thumb web space is narrowed or syndactylized, release and re-creation of the thumb web space is extremely important for functional and aesthetic reconstruction. A representative procedure for correction of the type IIb or III cleft hand is Snow-Littler procedure in which the cleft is released as a palmar-based flap and then transferred to the released first web space for creation of the commissure. During the last 10 years, the authors experienced 38 cases of cleft hand deformity in 30 patients. Among them type IIb or III cleft hand was 5 cases in 4 patients. In all patients, 3rd digit was missing except 1 case and the first web spaces were completely syndactylized. We applied the Snow-Littler procedure for correction of these 5 cases. The functional outcomes and patients' satisfaction were followed up postoperatively. Thumb abduction was possible in range of 59.6 +/- 12.3 degrees. Intermetacarpal stabilities were well maintained and average pinch power was 2.3 +/- 0.8 Kg. In one case partial necrosis of the palmar-based flap tip was occurred. Careful design and preservation of the intrinsic blood supply of the palmar-based flap is extremely important for viability of the flap and successful results.
Subject(s)
Humans , Classification , Hand Deformities , Hand , Necrosis , ThumbABSTRACT
Being a rare disease entity, there have been few references about the cleft hand in Korea. We evaluated the postoperative cosmetic results of 17 cleft hands in 14 patients, who were treated surgically at the Department of Orthopedic Surgery of Seoul National University Hospital, from 1982 to 1993. Among 14 patients, 8 were males. Bilateral cleft hands were in five patients and unilateral in 9. Three of five patients who had bilateral cleft hands, were treated surgically on both hands. Remaining two patients had an operation on one hand which affected more severely. Average age at the time of operation was 2 years and 5 months(5 months-10years). Average duration of follow-up was 34 months(1 year 1 month-7years 5 months). By Lange's classification, typical patterns were 16 hands and atypical pattern one. By Flatt's classification, there were 8 hands of I b, 7 I c, 1 I a, 1 II. One patient had family history whose father had both cleft hands and feet. Congenital anomalies other than cleft hand were associated in 10 of 14 patients. Two patients had 3 associated anomalies, 4 patients 2, and 4 patients one. Syndactyly was the most common associated anomaly of affected cleft hand. Central cleft was closed by Snow-Littler technique in 4 hands(24%), Barsky method in 12(71%) and others in 1(6%). Metacarpal bone was treated by intermetacarpal ligament reconstruction in 1 hand(6%), excision only 7(41%), transfer of 2(rd) metacarpal to 3(rd) metacarpal base after excision 5(29%), and 2(rd) metacarpal corrective osteotomy 3(18%). Complications were in 2 hands; one wound dehiesence of cleft, and one partial necrosis of skin. The cosmetic results of surgical treatment were graded into good, fair or poor by the parents-Good ; when parents were satisfied with the result, Fair; improved cosmesis but not satisfied, and Poor; no improvement after surgery. Good was in 16 hands and fair in one.