Re-thinking congenital piriform aperture stenosis: Modern imaging demonstrates narrowing of the full nasal cavity length.

OBJECTIVE: Measure the width of the nasal cavity in cases of piriform aperture stenosis using computerised tomography scans. METHODS: Retrospective study performed at a paediatric tertiary referral centre in Australia. Comparison nasal cavity widths (measured on computerised tomography scans in the axial plane) at the piriform aperture, choana and points 25, 50 and 75% along the length of nasal cavity, between cases with piriform aperture stenosis and controls. RESULTS: In cases of piriform aperture stenosis the piriform aperture along with the nasal cavity is statistically significantly narrower than controls, measured at 25, 50 and 75% along the distance between the piriform aperture and the choana. CONCLUSION: This modern imaging review has illustrated the need for a change in the treatment paradigm for neonates with this condition, suggesting the need for further investigation of techniques that treat narrowing beyond the aperture.

Congenital Nasal Pyriform Aperture Stenosis: Successful Management of Restenosis After Primary Surgery by Stent Placement.

Congenital nasal pyriform aperture stenosis (CNPAS) is a rare anomaly causing respiratory distress in newborns. While the primary surgical technique is well established, the timing of the removal of the stents and the management of restenosis remain a matter of debate. We report a case of a female newborn affected by CNPAS with the recurrence of respiratory distress after primary surgery due to the early removal of nasal stents, causing an overgrowth of granulation tissue. This report notes that restenosis was successfully managed by repeating the procedure over a 14-day period, with soft polyvinyl chloride uncuffed tracheal tubes acting as nasal stents.

Congenital inferior turbinate hypertrophy: an overlooked entity in newborns and review of the literature.

BACKGROUND: Neonatal nasal obstruction may result in respiratory distress, feeding difficulties, sleep apnoea and failure to thrive; hence, it requires thorough evaluation and prompt intervention. Congenital inferior turbinate hypertrophy is relatively uncommon, and its presentation can mimic other congenital nasal anomalies. RELEVANCE: This paper reports two cases of congenital inferior turbinate hypertrophy in neonates that resulted in significant respiratory distress, feeding difficulties and sleep disturbance. Both patients were successfully treated surgically by endoscopic nasal dilatation and stenting. A literature search was performed to identify articles on congenital inferior turbinate hypertrophy in neonates and its management. CONCLUSION: Albeit rare, congenital inferior turbinate hypertrophy should be considered a differential diagnosis in newborns presenting with respiratory distress at birth.

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.

Novel use of high-flow nasal cannula therapy in the management of pyriform aperture stenosis: case report.

BACKGROUND: Pyriform aperture stenosis is a rare form of congenital nasal obstruction; it poses a management dilemma for otolaryngologists and physicians alike. It can result in poor weight gain and potentially life-threatening airflow obstruction. The challenge lies in the difficulty to predict which patients will require invasive operative management versus conservative therapy alone. CASE REPORT: This case demonstrates the successful use of high-flow nasal cannula therapy in a young child with pyriform aperture stenosis.

Congenital nasal piriform aperture atresia: A case report and novel finding.

Congenital nasal piriform aperture stenosis and choanal atresia are types of nasal obstructions that can be life threatening to infants if left untreated. While there has been numerous reports on both of them there has not been a single reported case of congenital nasal piriform aperture atresia. Here, we present the first case of piriform aperture atresia that includes the diagnostic and clinical approach.

Congenital nasal pyriform aperture stenosis: Diagnosis, management and technical considerations.

BACKGROUND: Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of nasal obstruction in neonates with respiratory distress manifestations. Diagnosis is made with craniofacial computed tomography, prompt and precise treatment creates good outcomes in these patients. AIM: To present our experience in diagnosis and management considerations with this rare pathology and a case series of our surgically managed patients. METHODS: A retrospective, analytical study of CNPAS patients surgically managed over a period of seven years. Evaluation and follow up was reviewed. RESULTS: Thirteen patients were evaluated; pyriform aperture mean width was 5.5mm. Of these patients, 31% also had Congenital Midnasal Stenosis. Medical treatment failed for all the patients and they required surgical enlargement of the pyriform aperture. No complications were seen and all patients improved in symptoms and development. CONCLUSIONS: Congenital nasal obstruction can be fatal in new-borns, CNPAS is a rare differential diagnosis that must be completely evaluated, properly treated with conservative management and if it fails, with well-planned and executed surgery. Follow-up shows high rates of success.

Congenital nasal pyriform aperture stenosis: Analysis of twenty cases at a single institution.

OBJECTIVES: Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of neonatal respiratory distress that is difficult to treat. The primary objective of this study was to identify factors that predict the need for initial and revision surgery for CNAPS. The secondary objective is to identify risk factors in maternal history associated with the development of CNPAS. METHODS: Infants with CNPAS between 2010 and 2017 were identified by ICD- 9 and 10 codes. Demographics, maternal history, anatomic features on imaging and medical and/or surgical management were reviewed. Frequencies, means and standard deviations were calculated. A p-value <.05 was considered significant. RESULTS: Twenty infants were included. All underwent flexible nasal endoscopy with inability to pass the scope in either nostril in 65% of infants. Nineteen had a CT scan and 13 had a MRI with midline defects in 76.3% and 53.8%, respectively. Solitary central mega-incisor was present in 65%. Half underwent surgical intervention at a mean age of 74.8 days, with 90% requiring revision surgery. There was no difference in pyriform aperture distance in the surgical and non-surgical patient subgroups (5.4 mm and 5.2 mm, p = .6 respectively). No specific variables were predictive of need for initial or revision surgery. Maternal diabetes mellitus (MDM) was found in 55% of mothers of infants with CNPAS. CONCLUSION: Pyriform aperture distance was not a predictor of surgical intervention. MRI should be considered in all infants with CNPAS as the rate of intracranial complications is high. MDM may be a risk factor for CNPAS.

Pediatric Rhinoplasty: A national surgical quality improvement program analysis.

OBJECTIVES: Rhinoplasty is commonly performed in children with congenital anomalies and resultant nasal deformity causing airway obstruction. Little is known regarding patient factors or perioperative sequelae. We define demographic characteristics and perioperative complications for children undergoing rhinoplasty within a large national cohort. STUDY DESIGN: Retrospective cohort study of children aged ≤ 18 years undergoing rhinoplasty utilizing data from the 2012 to 2015 American College of Surgeons National Surgery Quality Improvement Program-Pediatric public use file. METHODS: All children who underwent rhinoplasty were identified. Postoperative complications were defined as 30-day postoperative infection, unplanned readmission and reoperation, and death. Multivariate logistic regression was used to identify predictors of complications. Subgroup analysis was performed based on child age (age < 5 years vs. 5-13 years vs. ≥ 14 years). RESULTS: Of 1,378 children undergoing rhinoplasty, 21(1.52%) children experienced complications, with the most common being unplanned readmission. Younger children were more likely to experience complications (3.79% aged < 5 years vs. 0.66% aged ≥ 14 years; P = 0.001). Using multivariate logistic regression analysis, we observed a 61% decreased odds of complication with each age group (odds ratio 0.39, 95% confidence interval 0.19, 0.77; P = 0.007). Younger children were more likely to be male (56.2% male aged < 5 years vs. 46.6% male aged ≥ 14 years; P = 0.011), have developmental delay (11.7% aged < 5 years vs. 3.65% aged ≥ 14 years; P < 0.001), and have craniofacial abnormalities (73.2% aged < 5 years vs. 42.1% aged ≥ 14 years; P < 0.001). CONCLUSION: Children undergoing rhinoplasty experience few major complications, with the most common being unplanned readmission. Younger children are at greater risk and are more likely to be male with craniofacial abnormalities. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:494-499, 2019.

Extramucosal pyriplasty without stenting for management of pyriform aperture stenosis.

PURPOSE: The current management options of congenital pyriform aperture stenosis (CNPAS) are either conservative measures awaiting further growth of the bony nasal framework or surgical intervention that focuses on bone removal from the margin of the pyriform aperture (PA) without exposure of the nasolacrimal duct (NLD) followed by stenting. Recently, CT measurements of the nasal cavity in CNPAS have shed light that the site of maximal bony obstruction corresponds to the bony buttress encasing the NLD rather than the margin of the PA as initially thought. Herein, we present an extramucosal pyriplasty technique that can be used without stenting to enlarge the PA and achieve immediate and sustained relief of nasal obstruction. METHODS: Retrospective chart review of 4 patients with radiologically confirmed CNPAS who had undergone extramucosal pyriplasty without stenting during the period from 2012 to 2016. RESULTS: Three patients were full term without any clinically detectable congenital anomaly. The fourth patient was preterm infant who needed ICU management. On computerized tomography scan, the PA width ranged from 5.8 to 7.1 mm with a mean of 6.4 mm while site of maximal stenosis ranged from 5.4 to 6.8 with a mean of 6 mm. Extramucosal pyriplasty relieved nasal obstruction and restored normal oral feeding in all patients. Postoperative follow-up endoscopy revealed an adequately patent airway with no scarring, granulation or restenosis. CONCLUSIONS: Extramucosal pyriplasty with decompression of the NLD without stenting is a treatment modality for CNPAS that provides prompt sustainable relief of nasal obstruction and avoids the drawbacks of stenting and shortcomings of the current conservative methods.

The use of nasal trumpet as a non-invasive treatment method in congenital nasal stenosis.

Kemal Ö, Atmaca S, Bel-Çeçen A, Düzgün B, Aygün HC. The use of nasal trumpet as a non-invasive treatment method in congenital nasal stenosis. Turk J Pediatr 2017; 59: 210-213. Newborns and infants are dependent on nasal respiration and therefore, nasal obstructions may lead to life-threatening outcomes in this age group. Although the most common cause of nasal obstruction in newborns are choanal atresia, soft tissue edema, congenital nasal bone anomalies such as narrowness of the apertura piriformis and midnasal stenosis should be kept in mind. A 5-day-old infant was referred to our clinic with the complaints of respiratory problems. Physical examination revealed nasal bone hypoplasia and saddle nose deformity. In the endoscopic examination, the septum was seen to be deviated in an `S` shape and the nasal passages were almost completely closed. The paranasal CT examination reported significant narrowing of the airway in the central part of the nasal cavity. After application of nasal trumpet to both nasal passages, clinical recovery was determined in the patient and no pathology was determined during the follow-up period.

Pyriform Aperture Stenosis: A Novel Approach to Stenting.

OBJECTIVES: Congenital nasal pyriform aperture stenosis (CNPAS) is one of several causes of neonatal respiratory distress. Congenital nasal pyriform aperture stenosis can be diagnosed by clinical presentation and evaluated by computed tomography for degree of stenosis. Surgical management of indicated cases involves drillout of pyriform aperture with placement of stents. The following case presents a novel approach to choice of stent in these patients. CASE PRESENTATION: We report the case of an infant diagnosed with CNPAS who underwent surgical correction at 9 days of life, with placement of mometasone fuorate stents. DISCUSSION: Although relatively uncommon, CNPAS is a type of airway obstruction that causes cyclic cyanosis and failure to thrive in affected infants. If uncorrected medically, surgical interventions can successfully restore patency of the nasal cavity. While traditional stents can result in undesired complications, the use of mometasone fuorate stents presents an alternative without the typically associated risks. CONCLUSION: The use of mometasone fuorate stents may be a helpful option for otolaryngologists looking for the potential to avoid restenosis, plugging, and nasal alar necrosis in patients undergoing surgical treatment of CNPAS.

The effects of septoplasty surgery on serum oxidative stress levels.

The aim of this study is to compare the total antioxidant status (TAS), total oxidant status (TOS), and paraoxonase (PON1) serum levels in patients who have undergone septoplasty (NSD). Forty-six patients (mean age 33.7) aged between 18 and 50 years who were diagnosed with nasal septal deviation (NSD) were included in the study. The TAS, TOS, and PON1 levels were compared in the serum samples obtained 1 month before the septoplasty and 3 months after surgery. There was a significant increase in mean TAS after septoplasty (1.041 vs. 1.124 mmol/L, p = 0.011). The mean TOS decreased significantly after septoplasty (20.631 vs. 5.946 mmol/L, p = 0.011). The mean pre- and postoperative PON1 levels were similar (326.93 ± 215.22 vs. 275.20 ± 167.76 U/L, p = 0.253). Increased TAS and decreased TOS after septoplasty show that patients with NSD are under oxidative stress that is relieved following septoplasty.

Pyriform aperture enlargement in all aspects.

BACKGROUND: The pyriform aperture comprises the central area of facial bone structure. It is formed by the free corners of the nasal bone and the frontal processes of the maxillae, which articulate with each other at the nasomaxillary suture lines. Congenital nasal pyriform aperture stenosis might be linked to various craniofacial problems. This review presents all aspects of pyriform aperture stenosis and enlargement. METHODS: A literature search was conducted. Pyriform aperture definition, nasal development, congenital nasal pyriform aperture stenosis and pyriform aperture enlargement were reviewed. RESULTS: One of the most common abnormalities is holoprosencephaly, which is a midline developmental deficiency that may also be present in combination with facial clefting. The aetiology of nasal pyriform aperture stenosis remains unclear. When diagnosed, the choice of treatment is between non-surgical and operative methods, depending on the seriousness of the problem. Provided the sufferer can maintain a secure air passage with the help of specialised medical procedures and respiratory tract adjuvants, operative therapy may be delayed. CONCLUSION: The operative outcomes are extremely good, and the prognosis relies mainly on coexisting neural and endocrine problems. This paper evaluates the nasal pyriform aperture in detail.

Congenital nasal cavity stenosis in children with craniosyntosis: A report of 4 rare cases.

Congenital bony nasal stenosis (CBNS) is a very rare but life-threatening cause of airway obstruction in neonates and infants. This review aims to assess the presentation and early airway management of 4 new cases of craniosynostosis with bilateral nasal cavity stenosis. Patients were treated with endoscopic endonasal widening of the nasal cavity and stenting. All patients were extubated well post-operatively with resolution of symptoms. They remained asymptomatic with stents in situ for at least 6 months with no complications reported. Minimally invasive endoscopic endonasal widening of the nasal cavity with stenting is an effective and safe way of addressing nasal cavity stenosis.

Congenital midnasal stenosis - A novel technique for management.

Neonates are obligate nasal breathers and nasal obstruction in a neonate is an emergency. Here we report two cases of congenital mid-nasal stenosis, discuss its presentation and diagnosis with description of a novel method of management.