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Syndromic Piere Robbin Sequence- A Rare Presentation in Association with Multiple Heart Defects and Type III Stickler Syndrome

Pierre Robin Sequence (PRS), a rare congenital disorder, is a triad of micrognathia, glossoptosis, and tongue based airway obstruction (TBSO). It may occur as isolated anomaly (iPRS) or as a part of a syndrome (sPRS), like that seen in association with Stickler Syndrome. Approximately 20% of childre...

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Bibliographic Details
Published in:Indian journal of otolaryngology, and head, and neck surgery and head, and neck surgery, 2024-02, Vol.76 (1), p.1325-1327
Main Authors: Akhtar, Soubia, Wasif, Muhammad, Afzal, Yumna, Shahab, Iqra, Dhanani, Rahim, Shaikh, Abdul Rauf
Format: Article
Language:English
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Summary:Pierre Robin Sequence (PRS), a rare congenital disorder, is a triad of micrognathia, glossoptosis, and tongue based airway obstruction (TBSO). It may occur as isolated anomaly (iPRS) or as a part of a syndrome (sPRS), like that seen in association with Stickler Syndrome. Approximately 20% of children with PRS have congenital heart diseases. To the best of our knowledge this case of a one-day old infant is the first one to be reported as having two heart defects; patent ductus arteriosus and patent foramen ovale in Pierre Robbin Sequence child.
ISSN:2231-3796
0973-7707
DOI:10.1007/s12070-023-04307-x