Unilateral Tonsillar Hypertrophy in a 4-Year-Old Girl with Focal Dermal Hypoplasia (Goltz Syndrome)

Authors

  • John Emmanuel L. Ong Department of Otorhinolaryngology – Head and Neck Surgery Manila Central University – Filemon D. Tanchoco Medical Foundation Hospital
  • Emmanuel Tadeus S. Cruz Department of Otorhinolaryngology – Head and Neck Surgery Manila Central University – Filemon D. Tanchoco Medical Foundation Hospital; Department of Otorhinolaryngology – Head and Neck Surgery Quezon City General Hospital
  • Clydine Maria Antonette G. Barrientos Department of Otorhinolaryngology – Head and Neck Surgery Manila Central University – Filemon D. Tanchoco Medical Foundation Hospital; Department of Otorhinolaryngology – Head and Neck Surgery Makati Medical Center

DOI:

https://doi.org/10.32412/pjohns.v32i2.97

Keywords:

Focal Dermal Hypoplasia, Unilateral Tonsillar Hypertrophy, Goltz Syndrome, Goltz-Gorlin Syndrome

Abstract

Objective: To report a case of unilateral tonsillar hypertrophy resulting in severe Obstructive Sleep Apnea in a 4-year-old girl with focal dermal hypoplasia (FDH, Goltz or Goltz-Gorlin) Syndrome.

Methods:

Design:           Case Report

Setting:           Tertiary Teaching Hospital

            Subject:          One

Results: A 4-year-old girl with Goltz Syndrome (classical features of cutaneous and osteopathic disorders since birth) and unilateral tonsillar hypertrophy manifested with snoring and apneic episodes at two years of age. Polysomnography revealed severe Obstructive Sleep Apnea, and Arterial Blood Gases revealed metabolic acidosis with hypoxemia. A tonsillectomy and adenoidectomy improved breathing, appetite and sleep with resolution of snoring and apneic spells and final tonsil histopathology revealed lymphoepithelial polyp.

Conclusion: A 4-year-old child with Goltz syndrome, who developed severe obstructive sleep apnea due to tonsillar hypertrophy was presented.  Otolaryngologists should be aware of this syndrome, which may manifest with oral and mucosal lesions.   Although rare, Goltz syndrome may be considered in the differential diagnosis of tonsillar hypertrophy especially in the presence of the inherent clinical features. Physicians should educate patients and address the co-morbidities associated with it through individualized treatment.  

 

Keywords: Focal Dermal Hypoplasia, Unilateral Tonsillar Hypertrophy, Goltz Syndrome, Goltz-Gorlin Syndrome

 

Downloads

Download data is not yet available.

Downloads

Published

2018-07-24

How to Cite

1.
Ong JEL, Cruz ETS, Barrientos CMAG. Unilateral Tonsillar Hypertrophy in a 4-Year-Old Girl with Focal Dermal Hypoplasia (Goltz Syndrome). Philipp J Otolaryngol Head Neck Surg [Internet]. 2018 Jul. 24 [cited 2024 Jun. 23];32(2):43-6. Available from: https://pjohns.pso-hns.org/index.php/pjohns/article/view/97

Similar Articles

You may also start an advanced similarity search for this article.

Most read articles by the same author(s)