Classification And Treatment Of The Congenital Split Hand Malformation

Objective To report the clinical classification and treatment of anunusual anomaly of congenital cleft hand or congenital split hand.Method 6 cases of congenital cleft hand or congenital split hand in ourhospital from April 1998 to April 2003 were reviewed. According to theprinciple that we regarded the closure of the ugly fissure and the functionalreconstruction of the forefinger as the center of treatment we classified thecongenital cleft hand or congenital split hand into two types : Simplicity andMultiplicity. The importance of its treatment is the closure of the ugly fissureand the functional reconstruction of the index finger.Results According to the principle that we regarded the closure of theugly fissure and the functional reconstruction of the forefinger as the center oftreatment we classified the congenital cleft hand or congenital split hand intotwo types : Simplicity and Multiplicity.In all the patients the lost function was restored and the deformity wascorrected after the operations.Discussion Split hand is frequently seen in combination with split foot,called split hand/split foot malformation: SHFM. SHFM is a rare clinical entityand usual existent with other malformations. The most common mode ofinheritance is autosomal-dominant with reduced penetrance. Anticipation hasbeen suggested in some families. Autosomal-recessive and X-linked formsoccur more rarely. A large number of human gene defects can cause SHFM. Themost common and best known human SHFM syndrome is EEC syndrome,which is caused by missense mutations in the TP63 gene. There also are fourSHFM loci that have been mapped, including 2q31,7q21,10q25 and Xq26.Duijf PH etc. have reported that pathogenesis of split hand is related withchromosomal rearrangement, such as: chromosomal deficiency and shifting.Genetic defects, as well as environmental factors, may cause ectrodactyly byinterfering with AER function or maintenance, which affect the function ofSignals from the AER. This theory has been confirmed by the mouse mutantswith AER disruption. The functional reconstruction of index finger and theclosure of the ugly fissure can achieve the functional restoration. The result issatisfactory.Conclusion According to the principle that we regarded the closure ofthe ugly fissure and the functional reconstruction of the forefinger as the centerof treatment we classified the congenital cleft hand or congenital split hand intotwo types : Simplicity and Multiplicity. The importance of its treatment is theclosure of the ugly fissure and the functional reconstruction of the index finger.Prospect In the future, we can exploit the techniques of molecular biologyto research the pathogenesis of SHFM in China, and then map the concreteSHFM loci that is not ever found by the researchers.