Thoracoscopic resection of a lung keratocyst associated with pulmonary sequestration in a neonate

Abstract Introduction Keratocyst is a cystic lesion that has distinctive histological features composed of an epithelial lining with surface keratinization that is corrugated and predominantly parakeratotic. Although rare, they present in human pathology mainly as odontogenic keratocysts and have occasional association with Gorlin-Goltz syndrome. Case report We present a neonate with an antenatal suspicion of congenital pulmonary airway malformation. While stable and imaging confirmation studies were accomplished, thoracoscopic exploration and excision of a bronchogenic-like lung cyst attached to an extralobar pulmonary sequestration supero-medial to the left upper lobe were performed successfully at day 44 of life. Histopathological study has revealed keratocystic features of the lung cyst attached to a pulmonary sequestration tissue. Discussion and conclusion Up to our knowledge and review of the literature, this is the first case and occurrence of Lung Keratocyst. Long-term follow up is needed to assess the outcome of the thoracoscopic management of such lesion, and its hypothetic relation with odontogenic keratocysts or any associated syndromes.