Multiple odontogenic keratocysts in Ehlers\u2013Danlos syndrome: a rare case report

Anna Starzyńska,Barbara Alicja Jereczek-Fossa,Piotr Wychowański,Aleksandra Sejda,Michał Studniarek,Paulina Adamska,Łukasz Adamski

doi:10.1186/s12903-021-01472-9

Abstract

BackgroundAn odontogenic keratocyst is a lesion characterized by aggressive and infiltrative growth. The lesion is characterized by the existence of satellite microcysts (microtumours) and frequent recurrence (up to 30%). Ehlers–Danlos syndrome is a condition in which collagen production or its post-translational modifications are affected. Defects in connective tissues cause symptoms, which range from mild joint hypermobility to life-threatening complications.Case presentationWe present an extremely rare case of an 11-year old girl with Ehlers–Danlos syndrome and coexistence of multiple odontogenic keratocysts.ConclusionsThis case shows mainly atypical or rare association between multiple odontogenic keratocysts and Ehlers–Danlos syndrome.

Highlights

An odontogenic keratocyst is a lesion characterized by aggressive and infiltrative growth
Odontogenic keratocyst (OKC according to WHO 2017 classification; from 2005 to 2017 classified as keratocystic odontogenic tumour, Keratocystic odontogenic tumour (KCOT)) is a benign lesion of aggressive growth, capable of infiltrating soft tissues, with frequent existence of satellite microcysts and common recurrences
Lesions localised in the maxilla (16%) present more aggressive growth compared to the mandible

Summary

Background

Odontogenic keratocyst (OKC according to WHO 2017 classification; from 2005 to 2017 classified as keratocystic odontogenic tumour, KCOT) is a benign lesion of aggressive growth, capable of infiltrating soft tissues, with frequent existence of satellite microcysts (microtumours) and common recurrences (up to 30%). It is localised mainly in the mandible (84%) with the prevalence of the angle and the ramus. The treatment of choice should be done with complete enucleation of the lesion with possible extension of the procedure to peripheral bone curettage, decompression, marsupialisation, application of Carnoy’s fluid or cryodestruction. Congenital anomalies—cleft lip-palate, coarse face, hypertelorism, frontal bossing

Bifid or combined costae

Findings

Discussion and conclusions