• Plast. Reconstr. Surg. · Apr 1999

    Case Reports

    Langer-Giedion syndrome associated with submucous cleft palate.

    • D Morioka, T Suse, Y Shimizu, F Ohkubo, and Y Hosaka.
    • Department of Plastic and Reconstructive Surgery at Showa University, Tokyo, Japan.
    • Plast. Reconstr. Surg. 1999 Apr 1;103(5):1458-63.

    AbstractWe report a 4-year-old girl with characteristic features of the Langer-Giedion syndrome (trichorhinophalangeal syndrome type II) who also had submucous cleft palate. When she underwent a palatoplasty, a diagnosis of Langer-Giedion syndrome was made because of the characteristic facial features, multiple exostoses, and partial deletion of the long arm of chromosome 8. This is the first case of trichorhinophalangeal syndrome associated with cleft palate. We review the clinical alterations of trichorhinophalangeal syndromes and differential diagnosis of Langer-Giedion syndrome from trichorhinophalangeal syndrome type I and hereditary multiple exostoses. We also describe the importance of trichorhinophalangeal syndrome in plastic surgery.

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