Background Congenital nasal pyriform aperture stenosis (CNPAS) is a rare condition that may occur alone or as part of a multi-formative syndrome. Management remains difficult. There is no specific treatment protocol. Traditional surgery would be anachronistic; a non-invasive or minimally invasive therapeutic option is required. However, the rarity of the disease and the infantile context render randomised clinical trials difficult. Case presentation We present the case of a one-month-old Caucasian boy with CNPAS. He presented to the Emergency Department of the Bambino Gesù Pediatric Hospital with nasal obstruction, noisy breathing, feeding difficulties, and suspected sleep apnoea. During hospitalisation, he underwent overnight pulse oximetry, airway endoscopy, and maxillofacial computed tomography (CT); the final diagnosis was CNPAS with moderate obstructive sleep apnoea syndrome. We successfully treated the patient using an innovative strategy that involved collaboration between ear-nose-and-throat surgeons and orthodontists. Conclusions A combination of minimally invasive balloon surgery and placement of a palatal device may successfully treat CNPAS; it may also treat other types of nasal bone stenosis. Future studies may allow the development of practice consensus treatment strategies.

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BackgroundCongenital nasal pyriform aperture stenosis (CNPAS) is a rare condition isolated or inserted in a multi-formative syndrome, which still encounters management difficulties. There are no specific treatment protocols and the use of traditional surgery represents an anachronism since cognitive assumptions and non-invasive or minimally invasive therapeutic innovations would currently be available. However, the rarity of the disease and the infantile context makes RCTs difficult to process. Case presentationWe expose the case of a one-month-old caucasian male infant suffering from CNPAS. The patient presented to the Emergency Department of the Bambino Gesù Pediatric Hospital with a clinical picture characterized by nasal obstruction, noisy breathing, feeding difficulties and suspected sleep apnea. During hospitalization he underwent overnight pulse oximetry, airway endoscopy and maxillofacial CT with a final diagnosis of CNPAS with moderate obstructive sleep apnea. We treated him successfully through an innovative therapeutic strategy that required the collaboration of a team of ENT surgeons and orthodontists.ConclusionsThe combination of minimally invasive balloon surgery and the application of a palatal device may represent a successful strategy for patients with CNPAS, probably also applicable to other types of nasal bone stenosis. Future studies may allow the definition of treatment strategies of these pathologies, we hope in the context of novel practical guidelines.

Background Juvenile ossifying fibromas (JOF) are benign osteofibrous tumors very rarely located in paranasal sinus. The diagnostic and therapeutic tools applied in sinonasal inflammatory pathologies and other tumors fail to address a specific management in this rare pathology. Currently, the only available treatment is surgery. Endoscopic sinus surgery (ESS) allows a complete but minimally invasive approach compared to the open technique, especially for the minor demolition on growing bone structures. Case Description We describe a case of a 13-year-old boy presenting in the Emergency Department for a head trauma. The computerized tomography (CT) revealed an ethmoid-maxillary bony mass suspected to be a juvenile ossifying fibroma. The patient underwent ESS. Histological examination confirmed a juvenil psammomatoid ossifyng fibroma (JPOF). A revision surgery with the same technique was required after 7 months due to relapse of the tumor. He did not develop postoperative complications. The 3-years follow-up did not show any signs of relapse. Conclusions Paranasal JOF is a challenge for the ear-nose-throat (ENT) surgeon. The principles of local disease control for relapse reduction are different from inflammatory diseases and other paranasal tumors. Severe organ damage and recurrences are possible if caution is not observed. Specific guidelines about management are required on the basis of the shared experiences in the few cases reported in literature.

Nasal polyps (NPs) are rarely reported in childhood and usually represent red flags for systemic diseases, such as cystic fibrosis (CF), primary ciliary dyskinesia (PCD) and immunodeficiencies. The European Position Paper released in 2020 (EPOS 2020) provided a detailed classification and defined the correct diagnostic and therapeutic approaches. We report a one-year experience of a multidisciplinary team, made up of otorhinolaryngologists, allergists, pediatricians, pneumologists and geneticists, with the aim of ensuring a personalized diagnostic and therapeutic management of the pathology. In 16 months of activity, 53 patients were admitted (25 children with chronic rhinosinusitis with polyposis and 28 with antro-choanal polyp). All patients underwent phenotypic and endo-typic assessment, using proper classification tools for nasal pathology (both endoscopic and radiological), as well as adequate cytological definition. An immuno–allergic evaluation was carried out. Pneumologists evaluated any lower airway respiratory disease. Genetic investigations concluded the diagnostic investigation. Our experience enhanced the complexity of children’s NPs. A multidisciplinary assessment is mandatory for a targeted diagnostic and therapeutic pathway.