L’atrésie congénitale des choanes est une malformation rare mais non exceptionnelle. L’obstruction bilatérale de la région postérieure des cavités nasales est. Disease definition. Arhinia-choanal atresia-microphthalmia is a malformation disorder characterized by complete or incomplete absence of nose (arrhinia). Disease definition. Choanal atresia – deafness – cardiac defects – dysmorphism syndrome, also known as Burn-McKeown syndrome, is an extremely rare.

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Malignant infantile osteopetrosis is a rare genetic disease characterized by increased bone density due to osteoclastic dysfunction.

Orphanet: Arhinie atresie des choanes microphtalmie

We report on the case of a 3-month-old girl who was referred to our hospital by the ENT department for severe anemia in the context of bilateral choanal atresia. Clinical examination showed failure to thrive, anemia, respiratory distress, bilateral choanal atresia, and chest deformation. The abdomen ces soft with large hepatosplenomegaly. We noted a lack of eye tracking, no optical-visual reflexes, and left nerve taresie paralysis.


The blood count showed normocytic normochromic anemia with severe thrombocytopenia.

Journal Tunisien d’ORL et de Chirurgie Cervico-Faciale

The infectious work-up and blood smears were negative. The skeleton X-ray dess diffuse bone densification of the skull, long bones, pelvis, vertebrae, and ribs. The facial bone CT confirmed membranous choanal atresia. The patient had supportive treatment transfusion, oral steroid, vitamin D, oxygen, nutrition. Bone marrow transplantation was indicated but not available.

Malignant infantile osteopetrosis is rare and symptoms are nonspecific. Diagnosis should be considered in young infants presenting refractory anemia, particularly in the context of choanal atresia. Bone marrow transplantation remains the only curative treatment. Dhoanes page Archives Contents list. A case report S.

Informations sur l’atrésie choanale – Unité des voies aériennes – CHUV

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If you are a subscriber, please sign in ‘My Account’ at the top right of the screen. If you want to subscribe to this journal, see our rates You atresiee purchase this item in Pay Per View: Malignant infantile osteopetrosis revealed by choanal atresia: Thiongane aA.


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