Contents

SynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferences

View all Images (9)

Parry-Romberg syndrome in Child
Other Resources UpToDate PubMed

Parry-Romberg syndrome in Child

Contributors: Haya Raef MD, Arin Isenstein MD, David Dasher MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Parry-Romberg syndrome (PRS), also known as progressive facial hemiatrophy or idiopathic hemifacial atrophy, is a rare disorder that presents as slowly progressive atrophy of one half of the face. Skin and subcutaneous tissue atrophy may be accompanied by atrophy of muscle, cartilage, or bone. PRS commonly involves one or more of the trigeminal nerve branches. Severe disease is characterized by atrophy affecting the structures supplied by all 3 branches. Rare bilateral involvement and extrafacial involvement (such as the arm, leg, or trunk) has been reported.

The tongue and salivary glands may be affected. Associated neurologic and ophthalmic involvement may include migraine headaches, seizures, trigeminal neuralgia, enophthalmos, and eyelid dysfunction.

PRS typically develops in the first or second decade and is more common in females. It occurs spontaneously with no known preceding exposures or genetic predisposition, and is believed to be mediated by autoimmune factors. This syndrome may have an insidious onset and is slowly progressive, ultimately stabilizing after 2-20 years.

Codes

ICD10CM:
G51.8 – Other disorders of facial nerve

SNOMEDCT:
718224004 – Progressive hemifacial atrophy

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

To perform a comparison, select diagnoses from the classic differential

Subscription Required

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Reviewed:07/20/2021
Last Updated:07/20/2021
Copyright © 2024 VisualDx®. All rights reserved.
Parry-Romberg syndrome in Child
A medical illustration showing key findings of Parry-Romberg syndrome : Enophthalmos, Muscle atrophy, Unilateral distribution, Teeth abnormalities
Copyright © 2024 VisualDx®. All rights reserved.