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Here we report on 11 individuals, including the original four patients plus seven new individuals with three longer\u2010term survivors. The prenatal phenotype included stillbirth, bending of the femora, and a high incidence of polyhydramnios, prematurity, and perinatal death in three of 11 patients in the series. The survivors presented with characteristic radiographic findings that were observed among those with lethality, including bent bones, distinctive (moustache\u2010shaped) small clavicles, angel\u2010shaped metacarpals and phalanges, poor mineralization of the calvarium, and craniosynostosis. Craniofacial abnormalities, hirsutism, hepatic abnormalities, and genitourinary abnormalities were noted as well. Longer\u2010term survivors all needed ventilator support. Heterozygosity for mutations in the gene that encodes Fibroblast Growth Factor Receptor 2 (<jats:italic>FGFR2<\/jats:italic>) was identified in the nine individuals with available DNA. Description of these patients expands the prenatal and postnatal findings of Bent Bone Dysplasia\u2013FGFR2 type and adds to the phenotypic spectrum among all <jats:italic>FGFR2<\/jats:italic> disorders. \u00a9 2016 Wiley Periodicals, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ajmg.a.37772","type":"journal-article","created":{"date-parts":[[2016,5,31]],"date-time":"2016-05-31T04:52:48Z","timestamp":1464670368000},"page":"2652-2661","source":"Crossref","is-referenced-by-count":9,"title":["Clinical and radiographic delineation of Bent Bone Dysplasia\u2010FGFR2 type or Bent Bone Dysplasia with Distinctive Clavicles and Angel\u2010shaped Phalanges"],"prefix":"10.1002","volume":"170","author":[{"given":"Deborah","family":"Krakow","sequence":"first","affiliation":[{"name":"Department of Orthopaedic Surgery University of California Los Angeles California"},{"name":"Department of Human Genetics University of California Los Angeles California"},{"name":"Department of Obstetrics and Gynecology 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