Congenital Pyriform Aperture Stenosis Research Articles

Heated humidified high-flow nasal cannula: a new conservative approach for neonatal nasal stenosis.

The aim of this study is to evaluate the efficacy of heated humidified high flow nasal cannula (HFNC) therapy as a conservative treatment option for newborns suffering from nasal stenosis, a condition that often leads to respiratory distress and feeding difficulties. Given the increasing utilization of HFNC in various upper and lower respiratory tract indications, characterized by its flow-based mechanism and minimal mucosal damage, we seek to investigate its potential benefits in this specific patient population. A retrospective chart review of newborns with congenital nasal stenosis treated with HFNC for respiratory distress or feeding difficulties in a pediatric tertiary center between 2014 and 2022. Data were collected for demographic characteristics, clinical presentation and ventilatory requirements, pre and post HFNC application. Six infants with nasal stenosis were included in the study cohort. Five were diagnosed with congenital pyriform aperture stenosis, three of whom had additional midnasal stenosis. One patient had nasal synechiae. Two patients had failed surgical treatment and all patients failed conservative treatment prior to HFNC treatment. Following HFNC use, improvement was noted in oxygen saturations, heart and respiratory rates, meal volumes and weight. None of the patients required any additional sinonasal surgical treatment. No complications were observed. In this case series, we present the first documented use of HFNC treatment for nasal stenosis, showing favorable results. Further studies with a larger cohort, wider range of conditions and extended follow-up periods are needed to establish the risks and benefits of HFNC for neonatal nasal stenosis.

Endoscopic management of infantile non atretic nasal obstruction

Objective of the study: Evaluate some of the non atretic indications for endoscopic infantile nasal surgery regarding surgical technique, safety, and success.Patients and Methods: Retrospective analysis of 14 infants required endoscopic nasal surgery for severe non atretic nasal blockage in ORL department mansoura university hospital between 2010 and 2017, their age ranged between 1 month and 19 months, 8 female and 6 males. The indications were nasal obstruction due to craniofacial anomaly (Cruozon syndrome) 3 cases, post traumatic nasal adhesions 10 cases, and congenital pyriform aperture stenosis in 1 case. Endoscopic nasal surgery was conducted to perform widening of the nasal airway through bilateral partial inferior turbinectomy in Cruozon syndrome, nasal adhesolysis with or without stenting in post traumatic nasal adhesions, or trans nasal correction of pyriform aperture stenosis.Results: All patients showed postoperative improvement without restenosis or complications during follow up period in all study groups, only one patient of nasal adhesions group showed restenosis and didn’t complete the follow up.Conclusion: TVarious non atretic indications for infantile endoscopic surgery as nasal adhesions, nasal obstruction in crouzon syndrome and CPAS could properly managed with endoscopic surgery. Careful management of nasal obstruction during neonatal care with delicate handling is very important to avoid unnecessary complications as nasal adhesions. Proper knowledge and experience with endoscopic surgery in this age is mandatory regarding the preoperative preparation and planning, operative technique, and post operative follow up. The technique is challenging and required good experience yet it is rewarding and mostly successful.

Congenital nasal pyriform aperture stenosis 5.7 mm or less is associated with surgical intervention: A pooled case series

ObjectivesCongenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of upper airway obstruction in the newborn. CNPAS is diagnosed clinically and confirmed with CT scanning. Early diagnosis and management is essential for this potentially life-threatening condition. Patients can be managed conservatively or surgically. Surgical treatment is usually reserved for those patients that fail conservative treatment. Our objective was to provide a radiologically-measured pyriform aperture (PA) width that predicts the need for surgical intervention. MethodsThis study was a retrospective chart review of patients treated in a tertiary paediatric hospital as well as a review of the literature. Outcome measures were defined as surgical or conservative intervention for the management of congenital pyriform aperture stenosis. ResultsData from 26 individual patients (7 patients from our own case series and 19 patients from previously published reports) was analysed to calculate those patients requiring surgical intervention. ConclusionsA PA width of less than 5.7mm in a neonate is 88% sensitive and specific in predicting that a patient will require surgical intervention.

Patient selection in congenital pyriform aperture stenosis repair – 14 year experience and systematic review of literature

PurposeCongenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of respiratory distress in neonates that may necessitate early surgical intervention. Restenosis and granulation are postoperative concerns that may prompt a return to the operating room. Reoperation places children at increased risk of perioperative complications and prolonged hospital stays. We are presenting a review of our institutional experience of 16 patients treated for CNPAS over a 14 year period and a systematic review with pooled data analysis to determine the effect of craniofacial and neurologic anomalies on surgical success. MethodsRetrospective chart review of all cases of CNPAS treated at our tertiary children's hospital between 1999 and 2013. Systematic review of English language literature was conducted adhering to the PRISMA statement to determine the effect of neurologic anomalies and craniofacial dysmorphism (CFD) on surgical failure for CNPAS treatment. Univariate and exact multiple logistic regression were used for analysis of an individual patient data analysis. Results10 patients had surgery and 6 were treated medically. Average pyriform apertures were 5.71±1.72mm for the surgical group and 4.83±1.26mm for the medical group (p=0.38). 31% had neurological impairments. 31% had craniofacial dysmorphisms (CFD). 2 patients developed restenosis and 1 required tracheotomy. Both of these patients had other CFDs. Literature review captured 63 surgical patients and 9 failures in 6 series of CNPAS. 4.6% of patients without CFD and 36.8% of patients with CFD required surgical revision (p=0.023, OR13.8). ConclusionWhen repairing CNPAS, co-morbidities must be considered. Impaired respiration, central neurologic deficits and extensive craniofacial anomalies may require additional surgeries or an alternative approach.

CPAS: Surgical approach with combined sublabial bone resection and inferior turbinate reduction without stents.

Congenital pyriform aperture stenosis (CPAS) is a form of nasal obstruction caused by congenital narrowing of the maxilla at the medial processes. Traditionally, surgical correction involves a sublabial approach with subperiosteal dissection, widening of the aperture by drilling, and the use of nasal stents postoperatively. Although this approach may lead to symptomatic improvement, it alone may fail to provide a patent airway secondary to unaddressed posterior narrowing. Additionally, the use of stents is problematic because they are prone to clogging and can cause internal nasal scarring and septal or alar necrosis. We present the surgical management of this condition in six patients using a novel approach that aims to correct these limitations by including both the traditional sublabial procedure and an endonasal reduction of the inferior turbinates, without the use of stents postoperatively. Retrospective chart review. Review of the medical records of six consecutive patients aged 2 weeks to 7 months, who underwent repair of CPAS via a sublabial ostectomy and endonasal inferior turbinate reduction from 2009 to 2012. All six patients were clear of airway obstruction postoperatively and at follow-up. This is an alternative approach that leads to symptomatic improvement for CPAS patients without the morbidity associated with stent use. 4.

Is There a Role for Non-Operative Management of Congenital Nasal Pyriform Aperture Stenosis (CNPAS)?

Is There a Role for Non-Operative Management of Congenital Nasal Pyriform Aperture Stenosis (CNPAS)? Objective: Describe non-operative management of neonates with congenital pyriform aperture stenosis (CNPAS) and examine clinical features of these patients. Study design: Consecutive case series with chart review. Setting: Tertiary, care referral, academic children’s hospital. Subjects and methods: Comparison of patients diagnosed with CNPAS and treated operatively or non-operatively. Results: Eleven patients were included in our study. Of eight patients treated surgically, four were female and four were male. The average diameter of the pyriform aperture of those patients was 5.55 mm. Of the non-operatively managed patients, two were female and one was male. The average diameter of the pyriform aperture was 4.83 mm. There was no significant difference in pyriform aperture dimension between the two groups, p = 0.54. All of the CNPAS patients managed non-operatively were isolated CNPAS cases, while in the surgical managed group, half of the patients had significant medical comorbidities and congenital abnormalities. Conclusion: Non-operative management may be considered in selected patients with CNPAS. Clinical severity of disease should be the primary indicator for surgical or non-operative management.

Congenital Nasal Pyriform Aperture Stenosis: Conservative Management

This case series outlines the advantages of using 0.1% intranasal dexamethasone drops (IDD) to treat nasal obstruction in patients with congenital pyriform aperture stenosis (PAS). Five consecutive cases of PAS were reviewed. Three patients were treated with IDD alone, and 2 were treated surgically and had residual postoperative nasal obstruction before IDD. Four patients in follow-up (mean, 1 year) were weaned off the steroid drops with good breathing results. One patient developed iatrogenic Cushing's disease, and another, who was initially treated with surgery, eventually required nasal vestibular stenosis repair in addition to IDD. This study is the first to review the use of IDD in treating PAS. The potential impact of midnasal stenosis is discussed, and a conservative management algorithm for patients with PAS is presented. Careful surveillance for potential side effects of IDD is recommended, especially in cases that require elevated doses.

Nasal cavity dimensions in congenital pyriform aperture stenosis

ObjectiveTo determine the dimensions of the nasal cavity in infants with congenital pyriform aperture stenosis (CPAS). Study designThe nasal cavities of seven children with CPAS were identified and were compared to the nasal cavities of 13 neonates (<30 days old) who had received CT scans for other indications. MethodsThe width of the nasal cavities was measured at the pyriform aperture, choana, and at two standardized points along the lateral nasal wall (LW-1 and LW-2) between the pyriform aperture and choana. ResultsComparison between neonates with and without CPAS demonstrates significant narrowing of the nasal cavity (not just the pyriform aperture) in infants with CPAS. Significantly smaller nasal width was noted at pyriform aperture, LW-1, and LW-2 (p<0.01, p<0.01, p=0.02). No significant narrowing was seen at the choana. ConclusionThese findings suggest that CPAS is associated with narrowing of the anterior 75% of the nasal cavity. This has implications for surgical management because simple pyriform aperture ostectomy may not be sufficient to relieve symptoms of obstruction.

Congenital nasal pyriform aperture stenosis

Congenital pyriform aperture stenosis represents an unusual cause of nasal obstruction in neonates and is caused by overgrowth of the nasal process of the maxilla. A pyriform aperture width of less than 11 mm (Fig. 1) is diagnostic [1]. Its clinical presentation is similar to that of bilateral choanal atresia and may range from mild nasal obstruction to lifethreatening asphyxia. Up to 63% of the patients have a solitary median incisor (Fig. 2), thought to represent a mild form of the holoprosencephaly spectrum [2], in which case MRI of the brain and chromosomal analysis are also indicated.

A case of solitary median maxillary central incisor (SMMCI) syndrome with bilateral pyriform aperture stenosis and choanal atresia

Solitary median maxillary central incisor syndrome is a rare disorder involving midline abnormalities. It may present with life threatening respiratory distress in the neonate secondary to nasal malformations. These include pyriform aperture stenosis and choanal atresia. We present the first reported case of simultaneous choanal atresia and pyriform aperture stenosis in a neonate with solitary median maxillary central incisor syndrome. The clinical presentation and the management of congenital pyriform aperture stenosis are discussed.

Solitary median maxillary central incisor (SMMCI) syndrome

Solitary median maxillary central incisor syndrome (SMMCI) is a complex disorder consisting of multiple, mainly midline defects of development resulting from unknown factor(s) operating in utero about the 35th–38th day(s) from conception. It is estimated to occur in 1:50,000 live births. Aetiology is uncertain. Missense mutation in the SHH gene (I111F) at 7q36 may be associated with SMMCI. The SMMCI tooth differs from the normal central incisor, in that the crown form is symmetric; it develops and erupts precisely in the midline of the maxillary dental arch in both primary and permanent dentitions. Congenital nasal malformation (choanal atresia, midnasal stenosis or congenital pyriform aperture stenosis) is positively associated with SMMCI. The presence of an SMMCI tooth can predict associated anomalies and in particular the serious anomaly holoprosencephaly. Common congenital anomalies associated with SMMCI are: severe to mild intellectual disability, congenital heart disease, cleft lip and/or palate and less frequently, microcephaly, hypopituitarism, hypotelorism, convergent strabismus, oesophageal and duodenal atresia, cervical hemivertebrae, cervical dermoid, hypothyroidism, scoliosis, absent kidney, micropenis and ambiguous genitalia. Short stature is present in half the children. Diagnosis should be made by eight months of age, but can be made at birth and even prenatally at 18–22 weeks from the routine mid-trimester ultrasound scan. Management depends upon the individual anomalies present. Choanal stenosis requires emergency surgical treatment. Short stature may require growth hormone therapy. SMMCI tooth itself is mainly an aesthetic problem, which is ideally managed by combined orthodontic, prosthodontic and oral surgical treatment; alternatively, it can be left untreated.

Congenital pyriform aperture stenosis-management and novel approach

Objectives: We present a brief review of our preoperative preparation and surgical technique used in the correction of congenital pyriform aperture stenosis. We also discuss the postoperative care and potential complications of the procedure. Methods: Surgical correction of a congenital anomaly with the aid of 3-D modelling and microinstruments. Results: The outcome of our patient is reviewed as well as considerations for improvements in the treatment of future patients. Conclusion: Congenital pyriform aperture stenosis is a rare congenital deformity. When the stenosis is severe prompt intervention is required. The use of a CT-generated 3-D model greatly enhanced our surgical planning. The use of microinstruments allowed precise surgical correction complementing the preoperative surgical plan.

Congenital nasal pyriform aperture stenosis: non-surgical management and long-term analysis

Congenital nasal pyriform aperture stenosis is a rare cause of airway obstruction in the newborn. Immediate recognition and therapy are essential for this potentially life-threatening condition. After initial management, which includes establishment of an oral airway, surgical repair of the stenotic bony inlet has been traditionally considered. We present a long-term follow-up of two patients with congenital pyriform aperture stenosis, who were managed expectantly. Pertinent embryology, clinical presentation, and general treatment strategies for these patients are also discussed.

Nasal pyriform aperture stenosis and absence of the anterior pituitary gland: Report of two cases

We describe two female infants with congenital nasal pyriform aperture stenosis and severe pituitary insufficiency. The anterior pituitary gland was undetectable with magnetic resonance imaging. Consanguinity of parents in both cases suggests autosomal recessive inheritance of this disorder. An early fetal developmental defect may explain this syndrome, which affects midline craniofacial structures. In patients with congenital pyriform aperture stenosis, magnetic resonance imaging of the brain and endocrine investigations should be performed for rapid diagnosis and treatment of the latter to avoid major neurologic complications. (J P EDIATR 1996;128:858-61)