Children With Synpolydactyly Type I Homozygous HOXD13 Mutations: Embryology and Surgical Correction of the “Cupping” of the Hands and Feet
Samir O. Alsayegh, Abdullah M. Alghamdi, Mohammad M. Al-QattanPurpose:
Some patients with familial Synpolydactyly Type I have severe flexion deformities of the hands and feet at the metacarpophalangeal and the metatarsophalangeal joints, and the resulting deformity has been named “cupping” of the hands and feet. Severe “cupping” requiring surgical correction has not been previously reported. We explore the embryology and pathogenesis of the phenotype and present the surgical technique and results after the correction of the cupping deformity in a case series of 3 children.
Patients and Methods:
Two children had severe deformity of the postaxial toes, and they were stepping on the dorsal aspect of these rays. Correction was done with dorsal wedge osteotomies of the metatarsals. One child required correction of 1 hand. He underwent syndactyly release, release of the tight volar skin at the metacarpophalangeal joints, and volar plate release of the metacarpophalangeal joints.
Results:
Partial correction of the deformity was achieved in all 3 children. All children were followed up for more than 5 years. The former 2 children were stepping on the plantar aspect of the toes, and the latter child was using his hand well in daily activities. All parents were satisfied with the results.
Conclusion:
Cupping of the hands and feet is severe enough to require surgery in some patients with familial Synpolydactyly Type I. Our surgical technique partially corrects the deformity, but it resulted in a good functional outcome.
Level of Evidence:
Level IV—case series.