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

Year 2020, Volume: 23 Issue: 2, 149 - 152, 30.06.2020

Abstract

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.

Supporting Institution

Mashhad University of Medical Sciences

References

  • 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.
Year 2020, Volume: 23 Issue: 2, 149 - 152, 30.06.2020

Abstract

References

  • 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.
There are 10 citations in total.

Details

Primary Language English
Subjects Health Care Administration
Journal Section Case Reports
Authors

Ali Bagherpour 0000-0002-6281-1867

Shahin Moeını 0000-0001-5251-6218

Publication Date June 30, 2020
Submission Date December 24, 2019
Published in Issue Year 2020Volume: 23 Issue: 2

Cite

EndNote Bagherpour A, Moeını S (June 1, 2020) Agenesis of Frontal Sinuses in Association with Kindler Syndrome: A Rare Case Report Running title: Agenesis of frontal sinuses in Kindler Syndrome. Cumhuriyet Dental Journal 23 2 149–152.

Cumhuriyet Dental Journal (Cumhuriyet Dent J, CDJ) is the official publication of Cumhuriyet University Faculty of Dentistry. CDJ is an international journal dedicated to the latest advancement of dentistry. The aim of this journal is to provide a platform for scientists and academicians all over the world to promote, share, and discuss various new issues and developments in different areas of dentistry. First issue of the Journal of Cumhuriyet University Faculty of Dentistry was published in 1998. In 2010, journal's name was changed as Cumhuriyet Dental Journal. Journal’s publication language is English.


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