Surgical Management of Coxa Vara in Cleidocranial Dysostosis (Scheuthauer Marie-Sainton Syndrome): Case Report at French Medical Institute for Mothers and Children (FMIC), Kabul, Afghanistan

Cleidocranial dysostosis was first described by Marie and Sainton in 1898. It is also known as Marie and Sainton disease. Patients may have excessive movement and motion of the shoulder as clavicles are not developed fully. Late closure of the fontanels, open skull sutures and multiple wormian bones are the other finding. Due to having short ribs the thoracic cavity seen like a small Bell shape (1). It is a disorder or abnormality of bones formed by intramembranous ossification (primariltyh e clavicles,
cranium, and pelvis). This is a rare anomaly with the incidence rate of 1/1,000,000, without sex or ethnic group predilection (3). Differential diagnoses of CCD comprise of
Yunis-Varon syndrome, hypophosphatasia (perinatal and infantile forms), osteogenesis imperfect congenita (type 1) and clavicle congenital pseudarthrosis (7). When there is a positive family history of CCD, prenatal ultrasound can help in early diagnosis by showing an abnormal growth of the clavicles (8).

Author(s): Shekaib Rahman Behroz, Mirza Mohammad Nijrabi, Ezatullah Faiz

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