Congenital pyriform aperture stenosis

Nasal airway obstruction is a potentially life-threatening condition in the newborn. Neonates are obligatory nasal breathers. The pyriform aperture is the narrowest, most anterior bony portion of the nasal airway, and a decrease in its cross-sectional area will significantly increase nasal airway resistance. Congenital nasal pyriform aperture stenosis (CNPAS) is a rare, unusual form of nasal obstruction. It should be considered in the differential diagnosis of any neonate or infant with signs and symptoms of upper airway compromise. It is important to differentiate this level of obstruction from the more common posterior choanal stenosis or atresia. CNPAS presents with symptoms of nasal airway obstruction, which are often characterized by episodic apnea and cyclical cyanosis.     

Stertor in the newborn due to congenital nasal pyriform aperture estenosis: case series

Stertor is a noise generated by the disturbance of the air flow passing through the nose. Its main cause -in newborns and infants- is inflammatory or infectious rhinitis. Congenital, neoplastic, traumatic or iatrogenic causes are less frequent. Congenital stenosis of the pyriform aperture is a rare etiology of nasal obstruction in the neonates. Early diagnosis and appropriate treatment are essential because of their exclusive nasal breathing. Suspicion might arise when a difficulty or even an impossibility to pass a probe of 2.8 mm (K30 tube) through anterior nares, exists. Diagnosis should be confirmed by a computed tomography of the craniofacial massif. The therapeutic behavior will depend on the severity of symptoms. We describe our experience with nine patients with this condition whose surgical correction was successful.     

The upper airway: congenital malformations

The upper airway extends from the nasal aperture to the subglottis and can be the site of multiple types of congenital malformations leading to anatomical or functional obstruction. This can cause severe respiratory distress. Newborns are obligate nasal breathers; therefore nasal obstruction can lead to airway compromise and respiratory distress. The etiologies are varied and include, choanal atresia, pyriform aperture stenosis, and rarely tumors such as glioma, encephalocele, teratoma, or dermoid. More common upper airway congenital anomalies include laryngomalacia, vocal cord paralysis, and subglottic stenosis. Laryngolmalacia is the most common congenital laryngeal anomaly. Inspiratory stridor often does not present until two weeks after birth and resolves by 18 months of age. Most cases are managed with watchful waiting. Severe cases require a surgical intervention. Bilateral vocal cord paralysis is usually idiopathic. In certain cases, paralysis may occur secondary to central nervous system abnormality including Arnold-Chiari malformation, cerebral palsy, hydrocephalus, myelomeningocele, spina bifida, or hypoxia. Severe cases may necessitate endotracheal intubation and tracheostomy. Congenital subglottic stenosis is the third most common laryngeal anomaly. It is defined as a diameter of less than 4mm of the cricoid region in a full-term infant, and less than 3mm in a premature infant. This condition is the most common laryngeal anomaly that requires tracheotomy in newborns. Laryngotracheoplasty may be required to achieve decanulation. Knowledge of the upper airway embryological development and congenital anomalies is off prime importance in assessing the newborn with respiratory distress. In most cases flexible endoscopy establishes the diagnosis. Management is tailored to each condition and its degree of severity.     

Neonatal nasal obstruction]

Because neonates are obligate nose breathers, neonatal nasal obstruction may have serious consequences. Prompt diagnosis and appropriate treatment are essential to avoid severe hypoxia. The most common cause of neonatal nasal obstruction is rhinitis. However, it is essential to first rule out anatomical causes such as choanal atresia and stenosis, pyriform aperture stenosis, traumatic deformation or tumors, as these conditions may be life-threatening and require prompt surgical treatment.     

Congenital granular-cell tumor of the gingiva

Congenital granular cell tumors of the gingiva (synonyms: congenital myoblastoma, congenital epulis) originate from the alveolar ridge in newborns. They are rare granular-cell tumors with benign histology. The main differential diagnosis is epignathus (oral teratoma). Early surgical excision is recommended due to a risk of airway obstruction and difficulty feeding. Accepted: 5 August 1997     

Neonatal nasal obstruction and a single maxillary central incisor

We present two neonates with nasal obstruction because of anterior choanal stenosis (congenital nasal pyriform aperture stenosis). An associated single maxillary central incisor was also shown on computed tomography imaging in the neonatal period. These midline anomalies are recognized minimal manifestations (microforms) of holoprosencephaly. We describe their neonatal investigation, multidisciplinary management and clinical course.     

Congenital nasal pyriform aperture stenosis associated with central diabetes insipidus

We describe a child who has central diabetes insipidus associated with congenital nasal pyriform aperture stenosis without any apparent anterior pituitary dysfunction. This association further strengthens the concept that congenital nasal pyriform aperture stenosis may be a microform of holoprosencephaly.     

Single central maxillary incisor with nasal pyriform aperture stenosis – CT diagnosis prior to tooth eruption

Two children with nasal pyriform aperture stenosis had the diagnosis of single central maxillary incisor made with CT scanning prior to tooth eruption and the clinical appreciation of this finding. The surgical and clinical implications of this diagnosis will be presented. Received: 13 July 1998 Accepted: 16 October 1998     

Vascular compression of the airway in children

Congenital heart disease (CHD) is an important clinical problem. Although survival has improved over recent decades, certain children with CHD remain difficult to treat, usually because of severe co-morbidity or uncorrectable defects. Vascular compression of the airway is one such co-morbidity, occurring in approximately 1–2% of children with CHD. It may be caused by congenital anomalies of the configuration of the great vessels, enlargement of otherwise normal structures or as a result of surgery. The anatomical patterns seen in these children may be complex, and as surgical correction is usually required to relieve the compression, the pre-operative imaging assessment should be as complete as possible. Precise diagnosis and therapy are essential because chronic airway compression in childhood carries a significant morbidity and mortality. Airway stenting is currently reserved for rare occasions when surgical correction is not possible.     

Solitary maxillary central incisor and congenital nasal pyriform aperture stenosis

Solitary maxillary central incisor (SMCI) and congenital nasal pyriform aperture stenosis (CNPAS) have been reported as an isolated morphogenic defect or associated with pituitary deficiency, holoprosencephaly, ocular coloboma, or chromosomal abnormalities. We report two cases and analyse 40 cases of SMCI and 24 cases of CNPAS, including 15 cases of combined SMCI and CNPAS, obtained from the literature. Of the patients with SMCI, 69% had short stature, 48% growth hormone deficiency or hypopituitarism, 23% pituitary absence or hypoplasia, and 17% had del (18p-) or r(18). Of the patients with CNPAS, 63% had SMCI, 75% were short, 43% had hypopituitarism or growth hormone deficiency, 36% had pituitary or CNS anomaly, and 33% had del (18p), r(18), or del (13q). Conclusions Solitary maxillary central incisor and congenital nasal pyriform aperture stenosis can be a diagnostic clue to pituitary hypofunction, CNS, ophthalmological and cytogenic anomalies. Received: 8 April 1997 and in revised from 20 June 1997 / Accepted: 6 July 1997     

Congenital dacryocystoceles controlled by nCPAP via nasal mask in a neonate

Congenital dacryocystocele is a relatively rare type of nasolacrimal duct obstruction that may induce respiratory distress during the early neonatal period. We encountered a case of bilateral congenital dacryocystoceles with intranasal cysts in a premature infant delivered at 34 weeks of gestation. The patient developed symptoms of respiratory failure immediately after birth, but no ophthalmologic symptoms. Treatment with nasal continuous positive airway pressure via a nasal mask, instead of a nasal prong, effectively relieved the symptoms. Early diagnosis and appropriate treatment are critical for infants with nasal obstruction.     

Pediatric airway surgery

Pediatric airway surgery is a challenging field in pediatric surgery. Laryngotracheal stenosis has a variety of congenital and acquired conditions that require precise assessment and tailored treatment for each individual patient. About 90% of acquired conditions are represented by subglottic stenosis (SGS) resulting as a complication of tracheal intubation. Congenital tracheal stenosis (CTS) is a rare and life-threatening malformation, usually associated with complete tracheal rings along a variable length of the trachea. Tracheomalacia (TM) is a process characterized by flaccidity of the supporting tracheal cartilage, widening of the posterior membranous wall, and reduced anterior-posterior airway caliber. The clinical presentation can vary from almost asymptomatic patients to near fatal airway obstruction. There is considerable variation in both the morphologic subtypes and the prognosis of pediatric airway. The patients are divided into three clinical groups (mild, moderate, and severe). A further division was proposed according to the presence or absence of associated anomalies. The definitive diagnosis of pediatric airway was made by means of rigid bronchoscope and computed tomography scan with three-dimensional reconstruction (3D-CT). Rigid bronchoscopy and 3D-CT confirmed the diagnosis in all the cases. Other associated anomalies include congenital heart disease, vascular anomalies, and BPFM (maldevelopment of aerodigestive tract). After definitive diagnosis of pediatric airway lesions, surgical intervention should be considered. Surgical strategy was presented on each lesion.     

Severe lower tracheal stenosis in infancy.

16 infants with severe distal tracheal stenosis (TS) seen from 1978-1988 are reviewed. Diagnosis was established by endoscopy and confirmed by radiographic methods, including magnetic resonance imaging (MRI) in three cases. Types of TS ranged from segmental stenosis to complex lesions involving the carina and bronchi. Associated vascular anomalies were found in nine patients (56%). All patients needed long-term intubation or a tracheostomy (seven cases) for stenting of the airway. Patients with congenital TS (CTS) were treated by tracheal resection (two survivors), dilatation (one survivor, two non-survivors), or observation (two non-survivors). Patients with pulmonary artery sling (PAS) were treated by division of the vascular sling (three survivors, one non-survivor), or combined vascular and tracheal surgery (one non-survivor). One patient with dilated pulmonary arteries compressing both main bronchi died in spite of oxygen therapy. Two cases of compression by an anomalous aorta were managed with dilatation (one survivor, one non-survivor). One patient with a severe stricture after a tracheal resection was successfully treated by dilatation. The overall mortality was 50%. We conclude that 1. endoscopic examination of the tracheobronchial tree and thorough evaluation of associated anomalies is mandatory in symptomatic patients; 2. if indicated surgical correction is the treatment of choice for tracheal stenosis; 3. dilatation and stenting of the stenosis are invaluable tools in the management of these infants.     

Management of congenital tracheal anomalies and laryngotracheoesophageal clefts

Congenital obstructions and anomalies of the pediatric airway are rare problems that may be associated with mild symptoms or critical stenoses that may be life threatening in the first few days of life. This review provides an overview of the embryologic development of the airway, different congenital anomalies associated with airway development, and surgical correction that may be associated with good long-term outcome.     

Respiratory distress secondary to nasopharyngeal glial heterotopia.

Nasal glial heterotopia (nasal glioma) is a rare congenital malformation of neural origin. We present a newborn baby with life-threatening respiratory distress secondary to nasopharyngeal glial heterotopia that obstructed the nasopharyngeal or nasal airway. A high degree of suspicion, early diagnosis and surgical management are essential to cure this rare and potentially life-threatening disorder.     

Nasal obstruction in neonates and infants

The main functions of the nasal airway are respiration and olfaction. The nose and sinuses condition air before reaching the lower respiratory tract by providing almost 100% humidification, warming, filtering and trapping of foreign particles. The airway epithelium contributes to the host defense system. Any alteration of this clearance system may produce significant problems, particularly in neonates, who are obligate nasal breathers until they are at least two months old. Nasal obstruction, and the inability to remove nasal secretion by nose blowing, may have serious consequences, such as respiratory distress or discomfort, altered sleep cycle, increased risk of obstructive apnoea and feeding difficulties. Most cases of nasal obstruction in neonates and infants are due to generalized nasal airway obstruction associated with neonatal rhinitis, viral upper respiratory tract infections, and possibly milk/soy allergies. Saline nasal lavage is recommended as an adjunct therapy for rhinosinusitis and allergic rhinitis, and in most cases of nasal congestion or obstruction in newborns, infants and children. In two recent experiences, was deemed to be the Narhinel method safe and effective for treatment of nasal congestion in babies with viral infections of the upper respiratory tract, or for the prevention of acute otitis media (AOM) and acute rhinosinusitis (AR) in children. Due to the efficacy, ease of use, tolerability and the lack of alternative medications in children younger than 12 years of age, nasal irrigation with physiological saline solution, followed by gentle aspiration, represent an effective method for the prevention and control of nasal congestion or obstruction in term or preterm neonates, infants and children.     

Preliminary experience with tracheal stent application in children with tracheal stenosis.

Tracheobronchial stenosis in infants and small children is a frustrating lesion for both the pediatric surgeon and the patient and his or her family. Different surgical methods have been described to treat stenoses. Recently, tracheal stents have been introduced to relieve the airway obstruction in these patients. Here, we present 6 patients (2 newborns, 3 infants, and 1 three-year-old) with tracheal or bronchial obstructions treated with age-specific Palmaz balloon-expandable tracheal stents. One patient died due to sepsis. One patient's stent was removed successfully. No other problems occurred in the other 4 patients during 4 to 12 months of follow-up.     

Severe upper airway obstruction in infectious mononucleosis: a life emergency]

BACKGROUND: Upper airway obstruction can represent a severe, life-threatening complication of infectious mononucleosis. We report a rare case of airway obstruction in a child with infectious mononucleosis associated with herpes virus infection, and we discuss management strategy that can be proposed in such cases. CASE REPORT: A 9-year-old girl was hospitalised in intensive care unit for obstructive dyspnea during infectious mononucleosis. Despite five days of corticosteroids and tracheal intubation, persistent pharyngo-tonsillar tumefaction led us to perform a surgical adenotonsillectomy. This latter treatment allowed immediate tracheal extubation and a rapid recovery. Histology showed a herpes virus infection associated with infectious mononucleosis. CONCLUSION: Maintaining airway opening in infectious mononucleosis needs sometimes to use instrumental interventions: nasal trumpet, endotracheal intubation, even tracheostomy. Early tonsilloadenoidectomy may relieve airway obstruction and allow a rapid recovery in the most severe cases. Airway obstruction in infectious mononucleosis may be aggravated by concomitant herpes virus infection that should be searched for in this situation, in order to adapt the treatment.     

Congenital airway anomalies

Congenital airway anomalies (CAA) include a variety of conditions that cause respiratory distress in neonates and infants. These malformations occur at various anatomic levels and manifest in a wide spectrum of airway symptoms, with presentation significantly influenced by the level at which obstruction occurs as well as by the severity of obstruction. The prevalence of congenital airway malformations has been estimated to range between 0.2 and 1 in 10,000 live births. The most frequent CAA are laryngomalacia, bilateral vocal cord paralysis, subglottic stenosis, laryngeal webs, subglottic hemangioma, tracheomalacia, congenital tracheal stenosis, laryngotracheal cleft, and tracheal agenesis.