Agenesis of Frontal Sinuses in Association with Kindler Syndrome: A Rare Case Report Running title: Agenesis of frontal sinuses in Kindler Syndrome
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.
Keywords
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.
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.
Details
| Primary Language | English |
|---|---|
| Subjects | Health Care Administration |
| Journal Section | Case Reports |
| Authors | |
| Publication Date | June 30, 2020 |
| Submission Date | December 24, 2019 |
| Published in Issue | Year 2020Volume: 23 Issue: 2 |
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