Parry Romberg Syndrome: An Unusual Clinical Diagnosis

JOURNAL TITLE: An International Journal of Otorhinolaryngology Clinics

Author
1. Mary Kurien
2. Naina Picardo
3. Mary John
4. Gaurav Ashish
ISSN
0975-444X
DOI
10.5005/jp-journals-10003-1278
Volume
9
Issue
3
Publishing Year
2017
Pages
3
Author Affiliations
    1. Department of ENT and Head and Neck, Christian Medical College, Vellore, Tamil Nadu, India
    1. Department of ENT, Christian Medical College, Vellore Tamil Nadu, India
    1. Department of ENT, CMC Vellore, Tamil Nadu, India
  • Article keywords
    Blaschko\'s lines, En coup de sabre, Hemifacial atrophy, Parry Romberg syndrome

    Abstract

    Parry Romberg syndrome is a very rare clinical entity, manifested by chronic progressive hemifacial atrophy. The exact pathophysiology is poorly understood; however many hypotheses have been put forward to explain the above condition. It is a self-limiting condition and usually settles down after a few years of active facial resorption. This can clinically overlap with linear scleroderma also known as “en coup de sabre,” which is seen as a diagonal pit in the frontoparietal area. When the active phase is over, various treatment modalities have been employed such as fat, silicone or bone implants and pedicled flaps but with limited success. We present a case of Parry Romberg syndrome with involvement of sagittal sutures of the skull, an association that has been rarely reported. This case is being reported with aim of better understanding of this clinical entity regarding diagnosis and management.

    © 2019 Jaypee Brothers Medical Publishers (P) LTD.   |   All Rights Reserved