Unilateral tonsilar hypertrophy in a 4-year-old girl with focal dermal hypoplasia (Goltz syndrome)
Philippine Journal of Otolaryngology Head and Neck Surgery
; : 43-46, 2017.
Article
in English
| WPRIM (Western Pacific)
| ID: wpr-633725
Responsible library:
WPRO
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 ReportSetting:
Tertiary Private Teaching HospitalPatient OneRESULTS:
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.
Full text:
Available
Database:
WPRIM (Western Pacific)
Main subject:
Palatine Tonsil
/
Tonsillectomy
/
Focal Dermal Hypoplasia
/
Adenoidectomy
/
Polysomnography
/
Sleep Apnea, Obstructive
/
Diagnosis, Differential
/
Hypoxia
Type of study:
Diagnostic study
Limits:
Female
/
Humans
Language:
English
Journal:
Philippine Journal of Otolaryngology Head and Neck Surgery
Year:
2017
Document type:
Article