Siblings with Acquired Tracheocele: Possible Hereditary Etiopathogenesis?
- PDF / 1,629,879 Bytes
- 5 Pages / 595.276 x 790.866 pts Page_size
- 25 Downloads / 161 Views
CASE REPORTS
Siblings with Acquired Tracheocele: Possible Hereditary Etiopathogenesis? Cecilia Botti1,2 · Francesco Maccarrone1 · Giuditta Bernardelli3 · Massimo Lupi3 · Livio Presutti1 · Francesco Mattioli1 Received: 23 September 2019 / Accepted: 7 October 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Tracheocele is a herniation of the tracheal mucosa through a defect in the tracheal wall. Familiar and/or genetical factors have never been taken into consideration in the etiopathogenesis of acquired tracheocele. Acquired tracheocele occurred in a brother and a sister from India. CT scan of the neck showed an air cyst next to the right tracheal wall. They underwent surgical excision through a transverse cervical incision. Histopathology of the lesion revealed a fibrous sac lined by a pseudostratified columnar respiratory epithelium in both patients. They had no recurrence of tracheocele at 12 months follow up. Genetic counseling did not evidence any significative associated abnormalities. No previous cases of familiar acquired tracheocele have been reported in the literature. Given the rarity of the lesion occurring in more than one member of a family, hereditary factors could be supposed as contributing factors in the etiopathogenesis of acquired tracheocele. Keywords Tracheocele · Acquired tracheocele · Trachea · Tracheal lesion
Introduction Tracheocele is a rare lesion. It can be congenital or acquired. Tracheocele is a herniation of the tracheal mucosa through a defect in the tracheal wall. It was described first by Rokitansky in 1838 in a cadaveric study [1]. Radiologically, it appears as a cystic lesion filled with air or with fluid and air. It arises at the junction between the membranous and the cartilaginous portion of the trachea. They are more common one the right side, probably due to the absence of the esophagus on this side, giving mechanical support only on the left side. About 30 cases of tracheocele are reported in the English literature. They are sporadic cases. At our knowledge there
* Francesco Maccarrone [email protected] 1
Department of Otolaryngology Head Neck Surgery, University Hospital of Modena, Via Largo del Pozzo, 41125 Modena, Italy
2
Clinical and Experimental Medicine, University of Modena and Reggio Emilia, Modena, Italy
3
Department of Pathology, University Hospital of Modena, Modena, Italy
are no previous reported cases of acquired tracheocele in patients of the same family.
Case Report Two cases of acquired tracheocele occurring in a brother and a sister from India were referred to our hospital. They used to live together until their teens and denied excessive exposure to environmental pollution. There had no history of previous surgery on the neck, positive pressure ventilation or smoking habit. They didn’t play wind instruments or glass blow. Their parents were not consanguineous.
Patient 1 A 45-year-old man presented with a latero-cervical rightsided palpable mass with recurrent local infections since 4 years. H
Data Loading...
