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Bartsocas-Papas syndrome (BPS) is an autosomal recessively inherited form of the popliteal pterygium syndrome characterized by severe growth retardation, midface hypoplasia, popliteal pterygia, and syndactyly. Almost all affected babies die in utero or infancy. We report the difficulties of reconstruction and ongoing plastic surgical management in an 8-year-old child with BPS. With increasingly sophisticated resuscitation and supportive techniques, it is possible that more patients with BPS will survive beyond the neonatal period. This raises new challenges with reconstruction highlighted by this case with a difficult balance between trying to overcome some of the profound effects of the syndrome versus diminishing quality of life for the child by repeated and often unsuccessful surgical procedures.

Original publication

DOI

10.1097/SAP.0000000000000348

Type

Journal article

Journal

Ann Plast Surg

Publication Date

04/2016

Volume

76

Pages

459 - 462

Keywords

Child, Cleft Lip, Cleft Palate, Eye Abnormalities, Humans, Knee, Male, Reconstructive Surgical Procedures, Syndactyly