[An unusual form of craniofacial dysmorphism with mechanical respiratory disturbances in newborns (author's transl)].
Gross and histologic findings at autopsy of a one week old newborn baby with respiratory distress and mechanical obstacles to adequate intubation revealed a variation of the craniofacial dysmorphy syndrome: hypoplasia of the mandible combined with dysplastic and hypoplastic tongue and complete situs inversus. The tongue consisted of an hypoplastic smooth anterior part and a hypertrophic posterior part with the usual papillae. Both parts were separated by a deep sulcus across the middle of the tongue with a predominantly fibrous amuscular bottom. Because of this anomaly the posterior part of the tongue was retracted covering the orifice of the larynx and thus causing the respiratory distress. The lesion is discussed in context with data of similar syndromes.
D001344:Autopsy; D003394:Craniofacial Dysostosis / Q000150:complications* / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D012127:Respiratory Distress Syndrome, Newborn / Q000209:etiology* / Q000473:pathology; D014059:Tongue / Q000473:pathology
Eine Sonderform der kraniofazialen Dysmorphie mit mechanisch bedingter Atemstörung bei Neugeborenen.