Pierre Robin sequence and keratoconus, a rare association

Abstract Pierre Robin sequence (PRS) is an inherited disorder that affects one in between 8,500 and 14,000 people and is characterized by a triad of clinical signs. These include micrognathia, glossoptosis and obstruction of the upper airway, typically associated with palatal cleft. PRS has also been associated with various ocular complications, including high congenital myopia, congenital glaucoma, and retinal detachment. Because of the clinical importance of PRS, it is critical to illustrate the features of the Robin sequence to clearly define its primary and secondary clinical signs. We describe a patient with PRS who developed keratoconus as a rare manifestation of the disease and its management.

Saved in:
Bibliographic Details
Main Authors: Hernández-Cerdá,Jorge, Alegre-Ituarte,Víctor, González-Ocampo,Samuel
Format: Digital revista
Language:English
Published: Hospital San Pedro 2023
Online Access:https://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S2695-50752023000100006
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Abstract Pierre Robin sequence (PRS) is an inherited disorder that affects one in between 8,500 and 14,000 people and is characterized by a triad of clinical signs. These include micrognathia, glossoptosis and obstruction of the upper airway, typically associated with palatal cleft. PRS has also been associated with various ocular complications, including high congenital myopia, congenital glaucoma, and retinal detachment. Because of the clinical importance of PRS, it is critical to illustrate the features of the Robin sequence to clearly define its primary and secondary clinical signs. We describe a patient with PRS who developed keratoconus as a rare manifestation of the disease and its management.