Nasolacrimal Duct Cysts

Nasolacrimal duct cysts can occur either unilaterally or bilaterally. Children present with varying degrees of nasal obstruction. Often, there is associated epiphora. Intranasal examination shows a smooth, mucosa-covered mass under the inferior turbinate. The diagnosis is confirmed with CT scanning (Figure 76-1). Surgery is usually required to eliminate the nasal obstruction. Often, simply marsupializing the cyst results in complete resolution. This can be accomplished with a cup forceps or CO₂ laser.

Choanal Atresia

Neonates are preferential nasal breathers for the first 4 to 6 weeks of life. The entire length of the neonate’s tongue is in close proximity to the hard and soft palate, which creates a vacuum and resultant respiratory distress when nasal obstruction is present. Bilateral choanal atresia is the most common cause of complete nasal obstruction in the neonate, occurring in approximately 1 in 7000 live births.¹³ Associated anomalies occur in 20% to 50% of infants with choanal atresia.³³ The CHARGE syndrome includes coloboma or other ophthalmic anomalies, heart defect, atresia choanae, restriction of growth and development, genital hypoplasia, and ear anomalies with hearing loss.⁹ Mutations in the CHD7 gene (member of the chromodomain helicase DNA-binding protein family) located in chromosome 8q12 have been detected in more than two thirds of patients with CHARGE syndrome.^19,22 Data suggest overlap between CHARGE syndrome and 22q11.2 deletion syndrome in immunodeficiency states and hypocalcemia.²⁰ Children with CHARGE syndrome require intensive medical management as well as numerous surgical interventions. They also need multidisciplinary follow-up. A complete evaluation to rule out associated anomalies is, therefore, mandatory in all infants with bilateral choanal atresia.

Although bilateral obstruction always produces symptoms in the neonatal period, the degree of distress and cyanosis varies from severe asphyxia to cyanosis only with sucking. Typically, the infant has a history of distress when resting that is relieved with agitation and crying.

In a suspected case of choanal atresia, an attempt should be made to pass a 6-French catheter into the nasopharynx. Failure of the catheter to pass suggests choanal atresia. Computed tomography scan is required to differentiate between stenosis and atresia and to determine whether the atretic plate is bony or membranous.

Treatment of choanal atresia depends on the severity of the obstruction and the clinical presentation of the infant. Unilateral atresia rarely requires surgical intervention during infancy and is usually corrected before the child begins school (4-5 years of age). Bilateral atresia is usually repaired within the first few days of life. Historically, this was performed transpalatally, but today it is most commonly performed endoscopically through a transnasal route. Stenting of the repair has been associated with a higher rate of restenosis. As a result, single-stage repair is preferable.⁴⁰ Postoperatively, these patients do quite well, although repeated dilations may be necessary during the first year of life to maintain choanal patency. In CHARGE patients, tracheostomy may be preferable to immediate repair, depending on the severity of the associated anomalies.¹

Intranasal Tumors

In addition to anomalous nasal development, nasal obstruction with airway distress can result from intranasal tumors such as dermoids, gliomas, encephaloceles, or teratomas. Any infant with an intranasal mass should be fully evaluated, including magnetic resonance imaging (MRI) to assess for skull base involvement and intracranial extension before intervention is undertaken. Biopsy of an unsuspected nasal encephalocele can lead to cerebrospinal fluid leak, meningitis, and death.

Gliomas and encephaloceles are rare lesions of neurogenic origin containing glial tissue. Gliomas are benign but locally aggressive tumors that are usually noticeable at birth or during early infancy. Approximately 15% of gliomas have a fibrous stalk with connection to the subarachnoid space.¹⁶ Failure to recognize the fibrous stalk can lead to incomplete resection and tumor recurrence. Encephaloceles maintain their intracranial communication, with herniated brain tissue, dura, and cerebrospinal fluid constituting the tumor. Early surgical resection is generally recommended to alleviate the risk of meningitis that accompanies these tumors. In addition, progressive growth of the lesion can result in marked nasal deformity.

Teratomas are composed of multiple heterotopic tissues that are foreign to the site from which they arise. The etiology of these tumors is unknown, although they are believed to arise from rests of pluripotential cells sequestered during embryogenesis. The occurrence of nasopharyngeal teratomas is unusual, but when present, they may be associated with significant airway distress.5,7

Generally, four classifications of teratomas are described. Dermoids are the most common subtype and are composed of epidermal and mesodermal elements. Teratoid tumors are composed of all three germ layers but are incompletely organized, whereas true teratomas are composed of all three germ layers with recognizable early organ differentiation. Epignathi are highly differentiated tumors and are rarely compatible with life.

Treatment of the nasopharyngeal teratoma involves airway stabilization and complete surgical resection. Prognosis is generally excellent with complete excision. Mortality in infants with teratomas is generally the result of airway obstruction.

Mucosal Obstruction

Generalized mucosal hypertrophy or edema can result in significant anterior nasal congestion and symptomatic obstruction in the neonate until oral breathing becomes reflexive. Treatment of these patients is generally conservative and includes humidification, saline drops, and judicious suctioning. Excessive attempts at suctioning can result in increased edema and exacerbation of the infant’s symptoms. Gastroesophageal reflux may reach the level of the nasopharynx and can exacerbate nasal obstruction as well. Appropriate treatment of reflux may improve the nasal airway. Intranasal steroid drops might be helpful during periods of increased congestion such as that associated with viral rhinitis. Prolonged use of intranasal decongestants could lead to rhinitis medicamentosa (paradoxical mucosal swelling) and should be avoided.

Continuous Positive Airway Pressure Trauma

Care must be taken with the use of continuous positive airway pressure (CPAP) nasal prongs and masks in the neonate because nasal trauma can occur. Injuries such as excoriation of the nasal septum, necrosis of the columella, and lacerations of the nasal ala have been reported.³⁴ If injury is imminent and discontinuation of nasal CPAP is not possible, then changing to a different style of delivery (e.g., nasal mask) may be useful.

Lymphatic Malformations

Lesions of the floor of the mouth or base of the tongue that cause posterior tongue displacement also can be associated with secondary airway obstruction. Lymphatic malformations are known to infiltrate the soft tissue of the floor of the mouth and cause significant upper airway obstruction. Lymphatic abnormalities appear as persistent clusters of thin-walled vesicles, usually filled with clear, colorless fluid. Tissues affected by lymphatic anomalies are notorious for the speed at which infection can spread through them.³⁹ At the first signs of inflammation, aggressive antimicrobial therapy is mandatory. Such infections may be life threatening, especially if inflammation leads to increased airway obstruction.

Because of the infiltrative nature of these lesions in the oral cavity, extensive lymphatic anomalies are often not amenable to surgical excision. Serial resection is ineffective in most instances and could in fact exacerbate the degree of oropharyngeal obstruction. Spontaneous resolution is uncommon. For macrocystic cervicofacial lymphatic malformations, the immunostimulant OK-432 (Picibanil) has been shown to be effective.³⁷ Tracheostomy is the treatment of choice for patients with a large oral cavity and oropharyngeal lymphatic malformations and associated airway obstruction.

Tongue Cysts

Cysts of the base of the tongue are a rare but serious cause of airway obstruction in the newborn infant (Figure 76-3). An acute airway crisis could appear shortly after birth or several months later. A 43% mortality rate has been reported in the literature, with most deaths attributed to delayed diagnosis and acute airway obstruction. In most patients, these cysts represent thyroglossal duct remnants that arise from the foramen cecum.²¹

Dermoid cysts have been reported in the literature as tongue lesions associated with airway obstruction in the young infant. In contrast with the infant with a thyroglossal duct cyst at the base of the tongue, oral dermoids have a more insidious onset of symptoms, with presentation after 6 months of age.¹¹ Airway obstruction is in part caused by the mass effect in the hypopharynx and inferoposterior displacement of the epiglottis, which causes supraglottic obstruction.

Surgical excision is the treatment of choice in these patients. Marsupialization may be an option for the large cyst that is not amenable to complete resection. Significant tongue swelling can develop postoperatively, and temporary intubation may be necessary to ensure a protected upper airway.