Idiopathic subglottic stenosis in a patient with generalized port-wine stain (PWS): A case report and review of literature

Zahra Azizian,Laya Yousefian,Aliakbar Moodi,Elham Behrangi

doi:10.31661/gmj.v4i2.292

Zahra Azizian, Laya Yousefian + Show 2 more

Open Access

https://doi.org/10.31661/gmj.v4i2.292

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Abstract

Background: Subglottic stenosis is defined as inflammatory narrowing of the airway. It can be related to trauma, intubation, autoimmune disorders, vascular malformations, infection and congenital abnormalities. Pot-Wine Stain (PWS) is a small dermis venous malformation which is present at birth. PWS is found on face and neck, but can occur in throat and can cause subglottic stenosis. Case Report: Authors describe an 18-year-old girl with progressive dyspnea misdiagnosed with asthma having no response to medication. She has no histories of trauma, intubation, gastro-esophageal reflux disease (GERD) or allergy symptoms. Conclusion: Evaluations revealed subglottic stenosis in association with generalized PWS. To the best of our knowledge, this finding has not been reported in English medical literature. [GMJ.2015;4(2):126-28]

Highlights

Subglottic stenosis is an inflammatory narrowing of the airway, usually at the level of cricoid cartilage
Pot-Wine Stain (PWS) is a small dermis venous malformation which is present at birth
Pot-wine stain (PWS) or Nevus flammeus is defined as a macular telangiectatic patch which is present at birth and remains throughout life [3]

Summary

Introduction

Subglottic stenosis is an inflammatory narrowing of the airway, usually at the level of cricoid cartilage. Pot-wine stain (PWS) or Nevus flammeus is defined as a macular telangiectatic patch which is present at birth and remains throughout life [3]. It is an uncommon problem encountered in 0.3% of newborns. Symptoms were more severe during two past weeks She was previously misdiagnosed with asthma and received Fluticasone for 4 years. There were no histories of trauma, intubations, gastro-esophageal reflux disease (GERD), or allergy symptoms On examination, she had multiple, well-defined, 2-mm to 3.5-cm, erythematous to violaceous, blanching macules and patches located on both upper and lower limbs (Figure 1, 2). She has had no recurrence after 6 months of follow-up

Discussion

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Conclusion