The surgical treatment of congenital hand deficiency.

This review describes the two types of congenital cleft hand deformity, stressing the important ways in which they differ and summarises the surgical techniques that may be used in their t reatment . Introduction The two conditions are frequently grouped together under such titles as lobster-claw hand, ectrodactyly, oligodactyly and split hand. These terms are confusing, sometimes hurtful to parents and best discarded. Lange (1936) and Barsky (1964) have differentiated the typical from the atypical cleft hand and the two conditions are distinct, imposing a different surgical approach while sharing similar functional problems. Central longitudinal failure of development: the typical cleft hand Barsky (1964) credits Hartsinck (1770) with the first description of a case and Birch-Jensen (1949) claims that the first use of the term cleft hand was by Kummell (1895). The deformity (Fig. 1) has been described by Flatt (1977) as being "a functional tr iumph and a social disaster". It is rare and has been estimated at invovling 0.4 (Rogala, et al., 1974) to 0.14 (Birch-Jensen, 1949) per 10,000 live births. Cases occur sporadically but the condition may be familial and is inherited as an autosomal dominant with mixed penetrat ion (Graham and Badgley, 1954; David, 1974). Recessive inheritance may occur (Flatt, 1977). Both hands and feet are frequently involved and the cleft is 'V ' shaped. Other anomalies sometimes affect the limb and the condition may be associated with abnormalities in various organs. Genetic counselling is particularly important for these patients (David, 1974). In the common form the middle finger is missing, with or without the metacarpal , and suppression of more digits in a radial direction occurs in other cases. Ring finger absence alone is rare and when the digits are so severely suppressed as to produce a single digital variety this is the little finger. Syndactyly frequently affects the digits bordering the cleft and where the thumb is involved this can severely affect function. Several metacarpal variations are described. This bone may be totally missing in the cleft or two metacarpals may seem to support one finger which then resembles severe compound syndactyly on radiographs. Alternatively a bifid metacarpal may support two digits. Several subclassifications have been proposed (Watari and Tsuge, 1979; Nut t and Flatt , 1981; Tada et al., 1981). The cause of the defect is probably due to an abnormality in the apical ectoderm of the limb bud which produces a wedge shaped gap in the hand (Muller, 1937). The much quoted centripetal theory (Maisels, 1970) at tempts to explain a progression of clefting from a simple central soft tissue gap to complete suppression of all digits. Management The first question is: do these patients need Fig. 1. Typical cleft hands All correspondence to be addressed to P. J. Sykes F.R.C.S., St. Lawrence Hospital, Chepstow, Gwent NP6 5YX, UK.