Congenital upper airway obstruction

Most causes of upper airway obstruction are rare in the neonatal period and during infancy. They may, however, cause major respiratory problems either initially or during the first few weeks of life. It is important to recognise these problems at an early stage so that appropriate measures to overcome airway obstruction can be initiated, thus avoiding significant hypoxia-related complications. Specific treatment includes the use of nasal stents, a nasopharyngeal airway or, in severe cases, tracheostomy. Many such infants have associated feeding problems secondary to the airway obstruction and associated swallowing difficulties, which can lead to recurrent aspiration. The anomalies seen in clinical practice are best classified anatomically. Treatment is aimed at overcoming the mechanical effects of airway obstruction and maximising nutritional input so as to promote growth of the airway and long-term recovery of normal upper airway function.     

Acquired upper airway obstruction

Acquired upper airway obstruction is a common cause of respiratory emergencies in children. Most pathologic processes that result in upper airway compromise are a consequence of infection, trauma or aspiration. Today, many of the infectious causes of upper airway obstruction have lost their threat as a result of the progress made in preventing and treating these infections. Prompt recognition and appropriate management of the child presenting with upper airway obstruction remains critical, because certain causes can progress rapidly from a mild to a potentially life-threatening disease state. A correct diagnosis can often be made by history and physical examination, but additional studies may be useful in selected cases. The child's clinical appearance is the most reliable indicator of severity, and measurable signs are of less value. If respiratory failure is imminent, airway protection and endoscopy for definitive diagnosis may have priority over any other therapeutic or diagnostic procedure.     

Incomplete duodenal obstruction in a newborn

A case of newborn with incomplete duodenal obstruction caused by superior mesenteric artery syndrome has been presented with this report. A full term, 1-day-old baby girl was referred to our hospital because of recurrent bilious vomiting since birth and upper gastrointestinal barium study revealed the incomplete obstruction at the 3^rd part of the duodenum with a vertical abrupt cutoff. The diagnosis of superior mesenteric artery syndrome was made with ultrasonography and duodenojejunostomy was carried out. Although it is extremely rare, superior mesenteric artery syndrome should also be considered as one of the rare cause of incomplete duodenal obstruction in newborn period.     

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.     

Neonatal respiratory distress secondary to an obstructing nasopharyngeal dermoid.

A case presentation of a newborn infant with intermittent, complete airway obstruction secondary to a pedunculated nasopharyngeal dermoid is presented. The differential diagnosis of obstructing nasopharyngeal lesions of the neonatal period is discussed, and an organized management strategy is proposed.     

Long Term Effects of Artificial Ventilation in Neonates

ABSTRACT. In order to evaluate long term effects of artificial ventilation, 27 children, who had been ventilated for more than five days in their neonatal period, were reinvestigated at school age. In 5 of them bronchopulmonary dysplasia had been diagnosed. Seven had more than 10 upper respiratory tract infections per year and 9 had recurrent obstructive airway disease. On pulmonary function testing ( n =23) 19% showed some airway obstruction, and in 43% bronchial hyperreactivity was found by bronchial provocation with histamine. There was a significant correlation ( p <0.05) between bronchial hyperreactivity and the duration of neonatal ventilation. The degree of hyperreactivity (PC_{20, FEVI}) also correlated with birth weight ( p <0.005) and gestational age ( p <0.02). It is concluded that prolonged neonatal ventilation might be followed by bronchial hyperreactivity, especially in the small and premature newborn.     

Long term effects of artificial ventilation in neonates

In order to evaluate long term effects of artificial ventilation, 27 children, who had been ventilated for more than five days in their neonatal period, were reinvestigated at school age. In 5 of them bronchopulmonary dysplasia had been diagnosed. Seven had more than 10 upper respiratory tract infections per year and 9 had recurrent obstructive airway disease. On pulmonary function testing (n = 23) 19% showed some airway obstruction, and in 43% bronchial hyperreactivity was found by bronchial provocation with histamine. There was a significant correlation (p less than 0.05) between bronchial hyperreactivity and the duration of neonatal ventilation. The degree of hyperreactivity (PC20, FEV1) also correlated with birth weight (p less than 0.005) and gestational age (p less than 0.02). It is concluded that prolonged neonatal ventilation might be followed by bronchial hyperreactivity, especially in the small and premature newborn.     

Four cases of congenital airway obstruction: optimising perinatal management

Congenital anomalies causing airway obstruction in the newborn are potentially fatal. However if an effective airway can be maintained the long-term prognosis is often excellent. We present four cases of airway obstruction, three of which were diagnosed antenatally. We discuss the role of antenatal imaging and review delivery options including the need for a multidisciplinary team approach. In conclusion, we recommend antenatal imaging with both ultrasound scan and magnetic resonance to inform perinatal management. Polyhydramnios, in association with suspected neonatal airway obstruction, should alert clinicians to a high-risk situation. We recognize that even in skilled hands, securing an airway can be very difficult and alternatives such as maintenance of the fetomaternal circulation or extra-corporeal life support should be considered.     

Streptococcal pharyngitis and epiglottitis in a newborn infant

We describe a newborn infant withStreptococcus sanguis septicaemia and concomitant upper airway obstruction due to epiglottitis and pharyngitis. This rare infection of the supraglottic region was treated with endotracheal intubation and antibiotics. Full recovery occurred within 4 days.     

Ulcerative esophagitis. A rare source of upper gastrointestinal bleeding in a neonate. Use of fiberoptic endoscopy for diagnosis

A case of upper gastrointestinal hemorrhage in a newborn infant due to ulcerative esophagitis is described. This is a previously undescribed cause of upper gastrointestinal neonatal bleeding. This case further illustrates the safety and efficacy of fiberoptic endoscopy in elucidating the source of upper gastrointestinal bleeding in the immediate newborn period.     

Oronasopharyngeal teratomas

Oronasopharyngeal teratomas are rare tumours that usually present in the neonatal period with airway obstruction. Management should include prompt establishment of the airway and early excision. Complete excision is recommended, which may require more than one operation, but multilating surgery should be avoided as the malignant potential of these tumours is extremely low. We describe our experience with three patients along with a literature review.     

Effects of hyperoxia on the arousal response to upper airway obstruction in lambs

Experiments were done to investigate the effects of increased inspired oxygen on the arousal response from sleep to upper airway obstruction in 10 newborn lambs. Each lamb was anesthetized and instrumented for recordings of electrocorticograms, electrooculogram, nuchal and diaphragm electromyograms, and measurements of systemic arterial blood pressure and oxygen saturation (fiberoptic catheter oximeter). A tracheotomy was performed and a fenestrated tracheostomy tube was placed in the trachea. A 5F balloon tipped catheter was inserted into the tube so that airflow could be obstructed by inflating the balloon. No sooner than 3 days after surgery, measurements were made during a control period and during an experimental period of upper airway obstruction; the inspired oxygen fraction was alternated hourly between 0.21 and 0.60. A total of 57 epochs of quiet sleep and 58 epochs of active sleep was obtained in eight lambs. Arousal was significantly delayed (p less than 0.005) during active sleep (21 +/- 6 s; mean +/- 1 SD) compared to quiet sleep (7 +/- 2 s) in room air. Increased inspired oxygen significantly delayed arousal (p less than 0.05) during active sleep (47 +/- 25 s), but had little effect on arousal in quiet sleep (10 +/- 4 s). These results provide evidence that arousal from active sleep following upper airway obstruction in lambs is primarily initiated by a decrease in arterial oxygen. However, arousal from quiet sleep following upper airway obstruction in lambs appears to be initiated by other stimuli.     

Tension chylothorax complicating acute malignant airway obstruction

Acute upper airway obstruction represents one of the most challenging emergencies in pediatric practice. In particular, a tension chylothorax complicating a malignant airway obstruction is a rare and life-threatening complication. We report a rapidly progressing tension chylothorax associated with a cervical mass in a 10-month-old male infant. To our knowledge, the extension of a cervical mass to the supraclavear region resulting in a compressive chylothorax represents an exceptional event in pediatrics. Early recognition and prompt treatment resulted to be essential to relieve the compression and to avoid end-stage hemodynamic and respiratory function derangement.     

Management of apparent life-threatening events (ALTE)

In contrast to sudden infant deaths, the incidence of apparent life-threatening events (ALTE) has remained rather constant at 0.6–2.5/1000. Identifying the underlying cause of an event is paramount, requires careful history taking and a variety of investigations, summarized in this review. If no preventable cause is found, infants should be placed on a memory monitor, which may help to identify the underlying pathophysiology from analysis of the traces potentially thus recorded. For example, a sudden increase in heart rate despite severe hypoxaemia may suggest an epileptic seizure, while certain inconsistencies between the data recorded and parental reports of the event may hint towards fabricated events. A consistent feature in recorded ALTE that remain unexplained is severe hypoxaemia, mostly without prolonged cessations in breathing movements, suggesting upper airway obstruction or intrapulmonary shunting. If no event occurs in hospital, infants should be discharged on a memory monitor. If no further events occur within 4–6 weeks of the initial event, recurrence risk is very low, and monitoring can be discontinued. A recently discovered entity are neonatal ALTE, which may be related to a potentially asphyxiating environment, e.g. during skin-to-skin contact in the prone position or breast feeding with a mother who is inexperienced in handling and assessing her newborn baby. These situations thus require parental education and careful observation of the newborn by health professionals.     

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.     

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.     

An 11-Month-Old With Vomiting,Altered Mental Status,and Hypoventilation

Vomiting is a common pediatric complaint seen frequently in both pediatric and general emergency departments. Obstructive causes of vomiting are rare outside of the newborn period compared to more benign causes such as viral gastroenteritis. Timely recognition of these rarer causes of obstruction is essential for optimal care. This case highlights a severe presentation of chronic isolated vomiting caused by obstruction. It serves as a strong reminder to emergency care providers that obstruction can occur outside of the newborn period and, if unrecognized, can result in significant morbidity.     

Vallecular cyst: an uncommon cause of stridor in newborn infants

Vallecular cyst, a rare but generally benign lesion in the larynx, may cause stridor and even life-threatening airway obstruction in early infancy. We retrospectively studied 14 cases of newborn infants with vallecular cyst. There was no gender predilection and most cases were full-term and appropriate for gestational age. The clinical presentations included stridor, chest wall retraction, feeding difficulties and failure to thrive. Laryngomalacia was the most common associated anomaly. Flexible laryngoscopy was sufficient for diagnosing the vallecular cyst and larygmalacia. Maintenance of airway patency, nutritional support, and de-roofing of the cyst were the mainstays of management. Conclusion Vallecular cyst should be included in the differential diagnosis of stridor in newborn infants. Respiratory and feeding difficulties in these patients can be dramatically improved after appropriate surgical removal of the cyst. Received: 20 February 1999 / Accepted: 25 June 1999     

Acute gastric volvulus due to congenital bands in a newborn case

Acute gastric volvulus in the newborn period is a rare condition requiring surgery. The association of gastric volvulus with congenital bands is extremely rare and unreported previously. A one-day-old male neonate was diagnosed to have acute gastric obstruction in the neonatal intensive care unit. Mesenteroaxial gastric volvulus due to dense gastrocolic bands was detected during laparotomy. Band excision and anterior gastropexy were performed. The clinical features, radiologic findings, treatment modes and possible mechanisms of gastric volvulus due to congenital bands are discussed.     

A cause of neonatal respiratory distress: cerebral tissue heterotopia in the cavum]

BACKGROUND. Brain heterotopia can be reflected in disturbances of the oropharynx during the neonatal period, such as acute upper airway obstruction. The main differential diagnosis is encephalocele, which must be recognized before undertaking any surgical procedure. CASE REPORT. A boy, aged 5 days, with a cleft palate and bifid uvula, was admitted for respiratory distress. The distress initially occurred during bottle-feeding, but later became permanent. A solid mass, seemingly implanted in the superior wall of the pharynx, was successfully removed by forceps. A later CT scan showed the absence of any defect in the cribriform plate, thus excluding diagnosis of encephalocele. Histological, examination of the mass showed glial tissue without neurons. CONCLUSION. The presence of a pharyngeal mass in a newborn signals three possibilities: teratoma, heterotopic brain tissue or encephalocele. Encephalocele must by ruled out by a preoperative CT scan with a water soluble contrast agent in order to avoid CSF leakage and meningitis. Respiratory distress should be treated by oral endotracheal intubation to allow time for CT scan investigation.