Search Results (3)

Background: Congenital nasal pyriform aperture stenosis (CNPAS) is a rare disorder characterized by a narrowed pyriform aperture and nasal obstruction. Children with CNPAS often exhibit a bony ridge on the underside of the secondary palate, a solitary central incisor, and a triangular-shaped palate. Due to its rarity, limited research exists, and there is no established treatment algorithm, which complicates management, particularly in craniofacial treatments and long-term follow-up. Aims: This study aimed to develop a comprehensive treatment algorithm based on long-term follow-up, focusing on maxillofacial deformities, dental considerations, and upper airway assessment. Methods: We conducted a retrospective study of four CNPAS patients treated at our institute. We performed a literature review, and a comparison was executed with our data in order to introduce our novel, age-specific clinical treatment framework. Results: A literature review was conducted, and different clinical parameters were examined. Previously published data were compared to our patients-related findings to develop our clinical treatment algorithm based on patients’ age. Patients were monitored for respiratory distress during the first two years of life. Children with cyclic cyanosis underwent surgical widening of the pyriform aperture through bony excess removal and nasal tubing to ensure airway patency. For those with ongoing desaturation events, neonatal palatal expansion was performed. At ages 10–12, additional evaluations using polysomnography and orthodontic assessments were conducted. Based on these findings, patients received surgically assisted rapid palatal expansion (SARPE) and further surgical widening of the pyriform aperture if obstructive sleep apnea (OSA) was present. Subsequent treatments included orthodontic care and restoration of the missing central incisor. Conclusions: We propose an age-based clinical treatment algorithm for CNPAS tailored to address individual patient needs throughout their growth. Full article

Holoprosencephaly is a complex human brain malformation resulting from incomplete cleavage of the prosencephalon into both hemispheres. Congenital nasal pyriform aperture stenosis (CNPAS) is sometimes found in patients with mild forms of holoprosencephaly. Surgical treatment is required. Low-invasive surgical approaches involve balloon dilation of the pyriform opening. We present the case of an 8-day-old girl diagnosed with holoprosencephaly, CNPAS, and the presence of a solitary median maxillary central incisor. Once examined by neonatologist, geneticist, pneumologist, otolaryngologist, and pediatric dentist, a combined otolaryngological–orthodontic approach was used. The obstruction of the right nasal cavity was treated by widening the nasal cavities and stabilizing them with a balloon dilation technique. After surgery, the respiratory space was increased by applying a neonatal palatal expander plate (NPEP) considering the palatal deformity: ogival shaped, anterior vertex growth direction, reduction of transverse diameters. The NPEP promoted distraction of the median palatine suture and assisted the nasal dilation. Therefore, after the insertion of NPEP, the physiological sucking–swallowing mechanism was activated. In infants with CNPAS, NPEP can be useful to ensure the safe stability of nasal dilation. A multidisciplinary approach is fundamental. In our experience, the close collaboration between an otolaryngologist and orthodontist is essential for the management of the patient with CNPAS. Full article

Since newborns are obligatory nasal breathers, upper respiratory tract problems may sometimes be life threatening. The most common pathology causing dyspnea and stridor in newborns is laryngomalacia. Nasal cavity pathologies that risk the neonatal airway are more rarely met. These anomalies may be seen either as solitary anomalies or as a part of a syndrome. While choanal atresia is one of the best-known nasal cavity anomalies, choanal stenosis, congenital nasal mid-line masses, congenital nasal pyriform aperture stenosis, and nasal tip anomalies are more rarely seen structural pathologies. Choanal atresia may be present either as an isolated congenital anomaly or as a part of CHARGE syndrome. Some rare chromosome anomalies may also cause significant problems during nasal respiration in newborns. With this study, we presented a case series of newborns with pathologies that affected nasal respiration. Although the diagnosis and treatment of choanal atresia and congenital dacryocystocele are well known, the information on the diagnosis and treatment of the other two uncommon cases are limited. With this study, we aimed to contribute to the literature by presenting our approach in six cases having congenital pathologies that cause nasal respiratory obstruction. Full article