Year 2020, Volume 23 , Issue 2, Pages 149 - 152 2020-06-30

Agenesis of Frontal Sinuses in Association with Kindler Syndrome: A Rare Case Report Running title: Agenesis of frontal sinuses in Kindler Syndrome

Ali BAGHERPOUR [1] , Shahin MOEINI [2]


Kindler syndrome, as a rare subtype of Epidermolysis Bullosa, sets in motion a series of genetic conditions causing minor traumas and blisters on skin and making the skin susceptible to sunburn. The present study presented a case report of a 32-year-old female diagnosed with Kindler syndrome. Coronal and axial cone-beam computed tomography (CBCT) images clearly exhibited the agenesis of frontal sinuses. The condition was completely obvious in the images, which is a rare occurrence and has not been previously reported. The underdevelopment or aplasia of the paranasal sinuses is a rare phenomenon, which relates primarily to the frontal sinuses (12%) and secondarily to the maxillary sinuses (5-6%). Similarly, the agenesis of the sphenoid sinuses is an extremely rare condition. Therefore, raising our awareness about the paranasal sinus anomalies associated with the Kindler syndrome can lead to new discoveries about this syndrome. Further, with respect to the other patients suffering from Kindler syndrome, obtaining the basis of such knowledge together with evaluating CBCT or CT images in order to detect abnormalities can facilitate the management of the problems arising from paranasal sinus abnormalities.

Frontal sinus, Epidermolysis Bullosa, Syndrome
  • Lai-Cheong JE, Parsons M, Tanaka A, et al. Loss-of-function FERMT1 mutations in kindler syndrome implicate a role for fermitin family homolog-1 in integrin activation. Am J Pathol 2009; 175:1431-41.
  • Kindler T. Congenital poikiloderma with traumatic bulla formation and progressive cutaneous atrophy. Br J Dermatol 1954; 66: 104–11.
  • Lai-Cheong JE, McGrath JA. Kindler syndrome. Dermatol Clin. 2010; 28:(1):119-24.
  • Keskin G, Ustundag E, Ciftci E. Agenesis of sphenoid sinuses. Surg Radiol Anat.2002; 24(5):324-6.
  • Haktanir A, Acar M, Yucel A, Aycicek A, Degirmenci B, Albayrak R. Combined sphenoid and frontal sinus aplasia accompanied by bilateral maxillary and ethmoid . sinus hypoplasia. Br J Radiol.2005; 78(935):1053-6.
  • Guven DG, Yilmaz S, Ulus S, Subasi B. Combined aplasia of sphenoid,frontal, and maxillary sinuses accompanied by ethmoid sinus hypoplasia. J Craniofac Surg. 2010; 21(5):1431-3.
  • Aydinliog˘lu A, Kavakli A, Erdem S. Absence of frontal sinus in Turkish indivudials. Yonsei Med J. 2003; 30:44(2):215-8.
  • Ozgursoy OB, Comert A, Yorulmaz I, Tekdemir I, Elhan A, Kucuk B. Hidden unilateral agenesis of the frontal sinus:human cadaver study of a potential surgical pitfall. Am J Otolaryngol. 2010; 31(4):231–4.
  • Spaeth J, Krugelstein U, Schlondorf G The paranasal sinuses in CT-imaging: development from birth to age 25. Int J Pediatr Otorhinolaryngol. 1997; 14:39(1):25-40.
  • M. Pifferi, A. Bush, D. Caramella, Eur Respir J . Agenesis of paranasal sinuses and nasal nitric oxide in primary ciliary dyskinesia 2011; 37: 566–571.
Primary Language en
Subjects Health Care Sciences and Services
Journal Section Case Reports
Authors

Orcid: 0000-0002-6281-1867
Author: Ali BAGHERPOUR
Institution: Mashhad University of Medical Sciences
Country: Iran


Orcid: 0000-0001-5251-6218
Author: Shahin MOEINI (Primary Author)
Institution: Mashhad University of Medical Sciences
Country: Iran


Supporting Institution Mashhad University of Medical Sciences
Dates

Application Date : December 24, 2019
Acceptance Date : April 1, 2020
Publication Date : June 30, 2020

EndNote %0 Cumhuriyet Dental Journal Agenesis of Frontal Sinuses in Association with Kindler Syndrome: A Rare Case Report Running title: Agenesis of frontal sinuses in Kindler Syndrome %A Ali Bagherpour , Shahin Moeını %T Agenesis of Frontal Sinuses in Association with Kindler Syndrome: A Rare Case Report Running title: Agenesis of frontal sinuses in Kindler Syndrome %D 2020 %J Cumhuriyet Dental Journal %P 1302-5805-2146-2852 %V 23 %N 2 %R %U