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Laryngeal findings in short rib polydactyly syndrome: Case report and embryological correlations
Author(s) -
Knapp Calvin H.,
SantinHodges Antonio,
Cole Randolph R.
Publication year - 1990
Publication title -
the laryngoscope
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.181
H-Index - 148
eISSN - 1531-4995
pISSN - 0023-852X
DOI - 10.1288/00005537-199006000-00005
Subject(s) - polydactyly , medicine , hypoplasia , larynx , anatomy , rib cage , dysostosis , syndactyly , autopsy , surgery , congenital disease , pathology
Short Rib Polydactyly Syndrome (SRPS) is a group of rare congenital disorders characterized by polydactyly, short limbs, and short ribs. Infants with type I SRPS (Majewski syndrome) may exhibit dysgenesis of the larynx, which is manifested by epiglottic hypoplasia. Photographic documentation of laryngeal findings obtained at autopsy in an affected infant is provided. To our knowledge, such photographic documentation has not been previously published. Normal laryngeal development is reviewed, based on the Carnegie system of staging. The developmental errors leading to SRPS are thought to occur at 33 to 48 days of fetal life (Carnegie stages 15 through 19).