Case Report: Congenital nasal pyriform aperture stenosis with holoprosencephaly: Diagnosis and surgical management

Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of neonatal nasal obstruction that clinically mimics choanal atresia. It occurs as a result of abnormal development of primary palate and maxilla, either in isolation or in association with other anomalies. Differentiation between CNSPAS and choanal atresia is critical as management approachers are quite different from one another. Diagnosis can be made clinically and with characteristics CT findings. Management options include both conservative and surgical approaches depending on the patient’s initial condition. Here is reported a case of 33 days infant who was admitted to our NICU with a working diagnosis of CNPAS after she presented with two days of fast breathing and failure to suckle, managed surgically and discharged improved.

Balloon dilation and rapid maxillary expansion: a novel combination treatment for congenital nasal pyriform aperture stenosis in an infant

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.

Assessment of the anterior superior alveolar nerve and its impact on surgery of the lateral nasal wall

Background: The anterior superior alveolar nerve (ASAN) plays a major role in innervation of the lateral nasal wall. Its damage during nasal surgery can cause dental paraesthesia and numbness around the upper lip. Methodology: Retrospective evaluation of the computed tomographic (CT) scans of 50 consecutive patients analysing 100 sides. We measured the mean distance from the shoulder of the inferior turbinate to the descending portion of the anterior superior alveolar nerve, to the anterior superior alveolar canal and the anterior-posterior distance between the “shoulder” of the inferior turbinate and the pyriform aperture. Results: The mean distance from the shoulder of the inferior turbinate to the descending portion of the anterior superior alveolar nerve was 6.4 ± 2.33 mm, with no difference between sides The mean relative height of the shoulder in relation to the anterior superior alveolar nerve canal was 4.78± 2.31mm with no significant difference between the two sides. The anterior-posterior distance between the “shoulder” of inferior turbinate and the pyriform aperture was 6.96± 2.28mm, with no significant difference between the two sides. Conclusions: We found the anterior superior alveolar nerve to be a constant landmark in the lateral nasal wall. Therefore, the course of the ASAN should be assessed on a CT scan when a surgical approach through the pyriform aperture or anterior medial wall of the maxillary sinus is planned.

Congenital Bilateral Dacryocystocele with Nasal Pyriform Aperture Stenosis

Congenital bilateral dacryocystocele with concomitant congenital nasal pyriform aperture stenosis is a very rare case we seen in the paediatric age group. We report a case referred by the paediatric department to rule out choanal stenosis. The referring department had difficulty to insert a feeding tube to the patient. Nose and throat examination showed reduce misting on cold spatula test and unable to introduce nasoendoscope into both nostrils, which indicates obstruction. Radiological investigation revealed as congenital dacryocystocele with nasal pyriform aperture stenosis. The patient was managed surgically with good recovery.

Novel maxillary enlargement technique in congenital nasal pyriform aperture stenosis: a case report and literature review

Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of neonate nasal obstruction. Because newborns are natural nasal breathers, urgent treatment may be needed. CNPAS is diagnosed by clinical symptoms and signs, and CT. In severe cases, surgical approach is needed. The most often used approach is widening of pyriform aperture via sublabial incision with nasal tubing. We report a novel surgical approach as a curative treatment for CNPAS by immediate transverse enlargement of the maxilla. We also review current literature of the condition focusing on surgical management of the disease.

CT findings of congenital neonatal pyriform aperture stenosis

ABSTRACT Congenital neonatal pyriform aperture stenosis (CNPAS) is a rare but potentially lethal condition that causes respiratory distress. The characteristic narrowing of the pyriform aperture along with other associated craniofacial dysmorphism is diagnosed using cross-sectional imaging such as computed tomography (CT) and magnetic resonance imaging. CT scan is the imaging of choice for confirming and characterizing CNPAS. Infants are obligate nasal breathers in the first 5 months of life. Hence, a high degree of clinical suspicion, prompt imaging diagnosis and adequate respiratory support is critical to help reduce the morbidity of this condition.