Synchronous congenital nasal piriform aperture stenosis and atresia : A rare accompaniment of mid-facial dystosis and cause of neonatal upper airway obstruction

Congenital nasal piriform aperture stenosis (CNPAS) is a recently distinguished clinical entity that causes airway compromise in neonate as a result of a narrowing of the nasal piriform aperture. It may be bilateral or unilateral. Often considered as a form of holoprosencaphaly. Being the narrowest part of the nasal airway, any slight reduction in its cross sectional area will profoundly increase the nasal airway resistance with the attendant clinical challenges. Readily making an accurate clinical diagnosis may be very challenging when CNPAS occurs in the setting of a craniofacial dysostosis. This communiqué is aimed at increasing awareness among clinicians involved in paediatric care of the clinical entity congenital nasal piriform aperture stenosis and atresia presenting atypically as synchronous disorder with midfacial dysostosis. Hence, it should be kept in mind as a rare mimicker of bilateral choanal atresia and requires mandatory high resolution computerized tomography to confirm the diagnosis

Rosai-Dorfman Disease: A Diagnostic Albatross in a Female Patient with Bilateral Persistent Neck Masses. A Case Report

RosaiDorfman disease (sinus histiocytosis with massive lymphadenopathy) is an uncommon benign and often self limiting lymphoproliferative disease of unknown aetiology which was first described by Rosai and Dorfman in 1969. It usually presents with a multiple progressive painless bilateral cervical lymphadenopathy, low grade fever, polyclonal hypergammaglobulinemia and raised erythrocyte sedimentation rate. Extranodal diseases are uncommon but have been documented. Due to the rarity of this condition, the diagnosis may not be suspected clinically and fine needle aspiration cytology of neck nodes may miss the diagnosis. In this study, we present a 24 year old female who presented with bilateral cervical lymphadenopathy and right breast lump since childhood