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Split Hand/Foot Malformation about Two Family Cases

Open Journal of Pediatrics 2023年第1期13卷 63-68页

作者： Neli Yvette Ngakengni Bredel Djeri Djor Mabika Gauthier J. Buambo Irene L. P. Ondima Lucie C. Ollandzobo Atipo-Ibara lynda gamo tchidjo Landes C. Togho Abessou Samia M. Oya Angouma Benedicte M. Foueta Moukouba Flora Nombo Mavoungou Corinne Akouango Gnessou Nuptia C. Obengui Dhalia Y. Ngonya Mbongo Rachelle Dusabimana Bowassa Ekouya Gaston Aurore Mbika Cardorelle Faculty of Health Sciences Marien Ngouabi University of Brazzaville (Congo) Brazzaville Congo Neonatology Department University Hospital of Brazzaville (Congo) Brazzaville Congo Stomatology Department University Hospital of Brazzaville (Congo) Brazzaville Congo Obstetrics and Gynecology Department University Hospital of Brazzaville (Congo) Brazzaville Congo Surgery Pediatric Department University Hospital of Brazzaville (Congo) Brazzaville Congo Pediatric Departments University Ce Hospital of Brazzaville (Congo) Brazzaville Congo

Split hand/foot malformation (SHFM), formerly known as ectrodactyly is a rare congénital anomaly, its incidence varies from 1/8.500 to 1/25.000 live birth. It mainly affects the development of the limbs, its clinical variability is standard, can present as an isolated feature or as a syndrome associated with other congenital anomalies. Our objective was to present the two cases of SHFM, and to review the literature on the clinical aspects and discuss a probable origin. The father went to school and is a driver because the malformations concerned only the fingers, were less severe, and did not prevent the realization of certain simple gestures of the daily life. On the other hand, the malformations of the fingers of the newborn were severe and the absence of the thumbs compromised the later prehension function. Also the association of a microglossia and a cleft palate contributed to a weight loss that justified hospitalization. The clinical presentation of split hands and feet is variable and the prognosis depends on the type of anomaly. Familial cases suggest a probable genetic origin. Genetic testing is necessary to establish genetic counseling.

关键词： Split Hand/Foot Malformation Isolated Form Syndromique Form Family

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