Yasemin ÖZKALE, Nurcan CENGİZ, Murat DERBENT

Hallermann Streiff Sendromu: Olgu Sunumu Hallermann Streiff Sendromu: Olgu Sunumu

Okülo-mandibulo-dissefali olarak da bilinen Hallermann Streiff Sendromu (HSS) (OMIM 234100) nadir görülen ve esasolarak baş boyun bölgesindeki multiple konjenital anomaliler ile tanı alan bir sendromdur. Genellikle sporadik olarakortaya çıkan bu sendrom, kuşa benzer yüz görünümü, mandibula ve maksilla hipoplazisi, göz ve diş anomalileri, deriatroﬁ si ve ufak vücut yapısı ile karakterizedir. Bu olguların %15inde mental retardasyon görülmektedir. Süt çocuğudöneminde yaşamı tehdit eden solunum problemleri, küçük burun delikleri ya da küçük çeneye ikincil glossoptozis vetrakeomalaziye bağlı solunum yolu tıkanıklığı nedeniyle görülmektedir. Bu yazıda, HSSnun özelliklerini taşıyan 2 aylık birolgu sunulmuştur.

Hallermann Streiff Syndrome: A Case Report

Hallermann Streiff Syndrome (HSS) (OMIM 234100) also known as oculo-mandibulo-dyscephaly is a rare disorder thatis principally diagnosed by multiple congenital anomalies localized in the head and neck region. This syndrome is usuallysporadic and characterized by a bird-like face, mandibular and maxillary hypoplasia, ocular and dental abnormalities,skin atrophy and short stature. Fifteen percent of the patients are mentally retarded. They may have life-threateningrespiratory problems during early infancy because of the small nares and glossoptosis secondary to micrognathiaand tracheomalacia. In this report, we present a 2 -month-old girl with typical clinical features of the Hallermann Streiffsyndrome.

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