“…The findings in these cases included: hypoplastic nails, gingival hyperplasia and bifid tongue [1 case from Cortina et al, 1979]; overlapping findings of EvC [Brueton et al, 1990]; small abnormal teeth, and postaxial polydactyly of the hands [Casteels et al, 2000]; overgrowth of membranous bone [Edelson et al, 1974]; facial dysmorphism and broad thumbs [Sankar and Phadke, 2006]; microcephaly, preaxial polydactyly of feet and postaxial polydactyly of unilateral hand, lobulation of the tongue and gingiva [Tahernia and Stamps, 1977]; cleft palate and prominent frenula (1st case), and tetralogy of Fallot with polydactyly (2nd case) [2/7 cases from Turkel et al, 1985]. Application of the diagnostic criteria led to the designation of 118 cases (79%) of Jeune syndrome from the original total [Jeune et al, 1955;Herdman and Langer, 1968;Say et al, 1969;Cremlin, 1970;Russell and Chouksey, 1970;Kozlowski and Masel, 1976;Elejalde et al, 1985;Giorgi et al, 1990;Aronson et al, 1999;Tongsong et al, 1999;Poggiani et al, 2000;den Hollander et al, 2001;Kaddoura et al, 2001;Das et al, 2002;Norberg, 2002;Hall et al, 2003;Haycraft et al, 2005;Huang et al, 2008;O'Connor et al, 2008;Phillips and van Aalst, 2008]. Table I provides a summary of the findings of our patients, and the frequency of findings in the 118 cases in the literature.…”