© Springer Science+Business Media New York 2015
Janet Lioy and Steven E. Sobol (eds.)Disorders of the Neonatal Airway10.1007/978-1-4939-1610-8_14Malformations, Deformations, and Disorders of the Neonatal Airway: A Bullet Point Review
(1)
Division of Otolaryngology, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA
(2)
Pediatric Otolaryngology, The Center for Pediatric Airway Disorders, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA
(3)
Department of Otorhinolaryngology-Head and Neck Surgery, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA
Keywords
Neonatal airway obstructionMalformationsDeformationsDisordersNoseOropharynxLarynxTracheaThe Nose
Nasal obstruction in the neonate is often symptomatic as infants are preferential nasal breathers. Immediate presentation may be cyclical cyanosis relieved by crying, airway distress, and feeding difficulties. The differential diagnosis for nasal obstruction includes: rhinitis/inflammation, arhinia, piriform aperture stenosis, nasal septal deviation, nasolacrimal duct cyst (bilateral or unilateral), nasal hemangioma, and other rare tumors, encephalocele/glioma, choanal stenosis/atresia.
Piriform aperture and nasal stenosis
Etiology
The nasal inlet is narrowed by overgrowth or medial position of the nasal process of the maxilla at the level of the piriform aperture bilaterally.
Partial obstruction of the nasal inlet, which may be associated with craniofacial or skull base anomalies.
Epidemiology
Rare
May be associated with a single central incisor or anterior pituitary abnormalities.
Pathogenesis
The narrowed aperture or nasal cavity leads to increased nasal resistance.
Clinical presentation
Difficulty is with breathing and/or feeding.
Anterior rhinoscopy identifies a narrowed nasal inlet, may be unable to pass 6 French catheter or 2.2 mm scope into the nasal aperture.
Diagnosis
Clinical evaluation is complemented by non-contrasted CT scan of the skull and sinuses inclusive of the pituitary and maxillary dentition.
Diagnosis is made when the nasal aperture is less than 11 mm on axial CT scan.
Medical management
Observation if minimally symptomatic.
Conservative management with nasal saline or steroids if moderate symptomatology.
Oral breathing appliance such as McGovern nipple or intubation for acute respiratory distress.
Surgical management
Most often required when piriform aperture width is <5 mm. Most common approach is sublabial with drill out of the piriform aperture, nasal dilation, with or without stent placement.
Multidisciplinary considerations
Endocrinology/Neurology: Association with anterior pituitary abnormalities, holoprosencephaly spectrum.
Dentistry/OMFS: Often associated with median central incisor.
Genetics: When syndrome suspected.
Choanal atresia and stenosis (bilateral and unilateral)
Etiology
Failure of recanalization of the buccal pharyngeal membrane during 4th–12th week of gestation leads to atresia (complete obstruction) or stenosis (narrowing) of the posterior choanae.
May be unilateral or bilateral.
Most common abnormality is bony/membranous obstruction; the vomer (posterior septum) is often bowed.
May be associated with skull base or midline defects, especially when presenting as part of a syndrome.
Epidemiology
1: 7,000 live births, female to males equal in the recent literature.
50 % associated with syndrome or additional anomalies.
Pathogenesis
Atresia occludes passage of air or drainage of nasal secretions. Stenosis increases airway resistance.
Clinical presentation
Neonate with bilateral atresia/stenosis will present with labored breathing, desaturations, cyclical cyanosis, and feeding difficulties.
Unilateral stenosis often presents with unilateral rhinorrhea/sinusitis, rarely with respiratory distress.
Diagnosis
Failure to pass a 5/6 french catheter.
Nasal endoscopy revealing the atretic plate.
Non-contrast fine cut CT scan of the skull base/sinuses.
Medical management
Conservative management: Oral airway or McGovern nipple and side or prone positioning may help initially. Intubation often necessary with bilateral atresia.
Surgical management
Endoscopic approach is most common. Serves to dilate and or repair the membranous and bony narrowing often with a posterior septectomy.
Bilateral atresia should be repaired once work-up is complete.
Unilateral atresia/stenosis repair can be delayed until childhood to allow for skull and patient.
Multidisciplinary considerations
Genetics: Associated syndromes: CHARGE.
The Oropharynx
The oropharyngeal airway may be obstructed by a prolapsing tongue base or a space-occupying mass. When the oropharynx is obstructed, air cannot pass fluidly from the nasal cavity or mouth into the larynx. The differential diagnosis for oropharyngeal obstruction includes: macroglossia, glossoptosis, nasopharyngeal mass extension, oropharyngeal mass, vallecular cyst, an undescended thyroid, or thyroglossal duct cyst.
Glossoptosis
Etiology
Most commonly associated with Pierre Robin Sequence: Congenital micrognathia which leads to glossoptosis (tongue base obstruction of the posterior pharynx) and airway distress of the neonate.
Most cases associated with a secondary cleft palate: U shaped, involving the soft palate.
Epidemiology
1 in 8,500–14,000 births.
Pathogenesis
Airway obstruction secondary to displacement of the tongue into the hypopharynx occluding the airway at the level of the epiglottis.
Clinical presentation
Airway distress in the neonate with apparent obstruction, which may be positional (worse on back), feeding difficulties, including airway distress or desaturation during feeding.
Diagnosis
Clinical diagnosis is complemented by a modified polysomnogram to quantify the severity of obstruction.
Awake flexible nasopharyngolaryngoscopy can aid in assessment of the tongue base position relative to the posterior pharyngeal wall.
Imaging with plain film or more commonly CT scan with 3D reconstructions of the face is obtained if considering surgical management.
Medical management
Prone or side lying positioning.
Nasal trumpet/nasopharyngeal airway.
Airway support with high flow nasal catheter or positive pressure ventilation.
Surgical management
Tongue–lip adhesion: Tongue musculature is sutured to that lower lip musculature to prevent ptosis of tongue base; the adhesion is later released.
Mandibular distraction: The mandible is advanced forward using distraction osteogenesis technique—internal and external devices.
Tracheostomy: Definitive management in refractory or complicated cases.
Multidisciplinary considerations
Plastic surgery: May consider mandibular distraction osteogenesis (MDO) for cases associated with micrognathia.
Genetics: More than 40 associated syndromes. Most common: Stickler and 22q11.2 deletion syndrome.
Vallecular cyst
Etiology
Etiology related to either a trapped minor salivary gland or a variant of a thyroglossal duct cyst present solely in the tongue base.
Epidemiology
Congenital airway cysts occur in 1.87–3.49 cases per 100,000 live births.
Vallecular cysts account for ~10.5 %.
Pathogenesis
Cyst may grow slowly or rapidly leading to a spectrum of airway symptoms. Most commonly presents within the first 2 weeks of life.
Secondary laryngomalacia may occur secondary to the Bernoulli Effect.
Clinical presentation
Most commonly presents with inspiratory stridor similar to laryngomalacia. Depending on the size may lead to complete airway obstruction with distress.
Large cysts also associated with feeding difficulties.
Diagnosis
Awake flexible fiberoptic pharyngolaryngoscopy.
Microlaryngoscopy demonstrates a mucous-filled cyst in the vallecula between the tongue base and laryngeal surface of the epiglottis.
Management
Surgical management is the mainstay of treatment.
Microlaryngoscopy and bronchoscopy with endoscopic marsupialization or excision with microlaryngeal instruments, microdebrider, or laser.
Preservation of lingual surface of the epiglottis is important to prevent vallecular scarring.
Cyst recurrence is rare.
Multidisciplinary considerations
Speech therapy: For evaluation and management of aspiration, if indicated.
The Larynx
The larynx consists of the supraglottic, glottic, and subglottic structures. Difficulties at the level of the larynx are commonly associated with stridor or noisy breathing. Some congenital anomalies present immediately with airway distress while others are asymptomatic or discovered later in infancy or childhood as feeding and growing difficulties arise.
Supraglottic Airway
Supraglottic anomalies affect the airway at the level of the epiglottis through the ventricle, which sits immediately superior to the vocal cords. Anomalies discussed below include laryngomalacia, bifid epiglottis, saccular cyst, and laryngeal cleft.
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Laryngomalacia
Etiology
Most common cause of stridor in infants, resulting from dynamic collapse of the supraglottic structures into the laryngeal inlet during inspiration.
Epidemiology
Incidence is unknown but accounts for 70–95 % of all neonatal stridor.
Incidence of synchronous airway lesions is ~15 % (more frequent in severe cases).
Pathogenesis
Collapse related to: Omega-shaped epiglottis, short aryepiglottic folds, and/or redundant supra-arytenoid tissue and cuneiform cartilages.
Clinical presentation
Fluttering inspiratory stridor most pronounced while supine, crying, sleeping, or with feeding.
Obstructive sleep apnea may be present.
Severe cases may present with failure to thrive or respiratory distress.
High concomitant incidence of gastroesophageal reflux.
Diagnosis
Awake fiberoptic laryngoscopy demonstrates an omega-shaped epiglottis, short aryepiglottic folds, and/or redundant and prolapsing soft tissue over the arytenoid cartilages.
Microlaryngoscopy and bronchoscopy may be necessary to rule out significant synchronous airway lesions.
Reflux evaluation may be indicated when feeding or swallowing symptoms are present.
Medical management
Most cases self-resolve over 12–18 months and require no medical treatment.
Reflux management should be considered in patients with feeding or respiratory concerns.
Surgical management
Consider microlaryngoscopy and bronchoscopy in recalcitrant cases to identify secondary airway lesions.
Supraglottoplasty is performed with microlaryngeal instruments or laser for children with failure to thrive, cyanotic spells, severe OSA, or recurrent respiratory admissions.
Multidisciplinary considerations
Pulmonary: May consider PSG to evaluate for central and obstructive sleep apnea.
Speech therapy: Preoperative and postoperative aspiration risk.
Gastroenterology: If significant reflux is present and recalcitrant to therapy.
Bifid epiglottis
Etiology
Clefted epiglottis involving at least 2/3 of the height of the epiglottis. The embryology is not clear though the epiglottis is derived from the hypobranchial eminence with likely involvement of the 4th branchial pouch.
Teratogen theory as hand, hypothalamus, and oral cavity development occurs during the same time period and may have associated anomalies.
Epidemiology
Usually does not present as an isolated anomaly, incidence not well reported.
Associated with Pallister–Hall syndrome.
Pathogenesis
Midline cleft within the epiglottis rendering it incompetent.
Clinical presentation
Inspiratory stridor, worse with feeding.
Choking or gagging with feeds if aspiration.
Diagnosis
Awake flexible laryngoscopy.
Consider rigid laryngoscopy and bronchoscopy to assess for additional anomalies.
Modified barium swallow to assess epiglottic competency and aspiration risk.
Management
Medical and genetic work-up for associated conditions including: Pallister–Hall, polydactyly, congenital hypothyroidism, and hypothalamic dysfunction.
Surgical management is not well described.
Multidisciplinary considerations
Genetics: Commonly associated with anomalies of the hands/feet (most commonly syndactyly), oral cavity, and hypothalamus/pituitary axis.
Endocrine: Hypothalamus and pituitary axis abnormalities.
Speech therapy: If aspiration or feeding issues.
Saccular cyst
Etiology
Cystic blockage at the opening of the saccule of the laryngeal ventricle, which extends between the false and true vocal folds.
Originate from an obstruction of the excretory duct of laryngeal epithelial mucous glands.
Epidemiology
Congenital airway cysts occur in 1.87–3.49 cases per 100,000 live births.
Saccular cysts account for ~25 %.
Pathogenesis
Cystic accumulation of fluid within the laryngeal saccule. May partially or complete block the laryngeal inlet.
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