Neoplasms of the Larynx, Trachea, and Bronchi

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Chapter 382 Neoplasms of the Larynx, Trachea, and Bronchi

382.1 Vocal Nodules

Lauren D. Holinger

Vocal nodules are the most common cause of chronic hoarseness in children. They are not true neoplasms. Chronic vocal abuse or misuse produces nodules at the junction of the anterior and middle thirds of the phonating edge of the vocal cords. These symmetric, bilateral swellings interfere with voice production and cause children to strain the voice. Vocal nodules can occur in infants and are exacerbated by laryngopharyngeal reflux.

Voice therapy may be effective in the cooperative child, but for most toddlers and young children, behavioral therapy is necessary. Nodules usually resolve by early teenage years as the child matures and vocal abuse is moderated. Surgical excision is rarely indicated but may be necessary if the child is unable to communicate adequately, becomes aphonic, or requires tension and straining to make any utterance whatsoever.

Laryngopharyngeal reflux commonly exacerbates vocal abuse, adding to swelling of the vocal cords. When vocal abuse is the main factor, the voice is worse in the evenings; with laryngopharyngeal reflux, hoarseness is worse in the morning. An antireflux regimen is indicated (Chapter 315.1).

382.2 Recurrent Respiratory Papillomatosis

Lauren D. Holinger

Papillomas are the most common respiratory tract neoplasms in children, occurring in 4.3/100,000. They are simply warts—benign tumors—caused by the human papillomavirus (HPV) (Chapter 258); the same pathology is found in condylomata acuminata (vaginal warts). HPV types 6 and 11 are most commonly associated with laryngeal disease. Fifty percent of recurrent respiratory papillomatosis (RRP) cases occur in children <5 yr, but the diagnosis may be made at any age; 67% of children with RRP are born to mothers who had condylomata during pregnancy or parturition. The risk for transmission is ∼1/500 vaginal births in mothers with active condylomata. Neonates have been reported to have RRP, suggesting intrauterine transmission of HPV.

Clinical Manifestations

These benign squamous lesions can produce chronic hoarseness in the infant. Most occur in the larynx, specifically on the vocal cords, but in 31% these lesions occur in other areas of the respiratory tract: nose, pharynx (especially the uvula and posterior soft palate), trachea, bronchi, and lungs. As growth of the lesions on the vocal cords progresses, hoarseness increases and communication becomes difficult. Respiratory distress develops. Surgical excision is a quality of life issue that warrants removal to improve the voice. Intervention becomes a medical necessity when airway obstruction progresses (Fig. 382-1). Symptoms often occur first during sleep with symptoms typical of obstructive sleep apnea. Progressive respiratory distress during sleep, exertion, daily activities, and finally at rest indicates the need for surgical intervention.

Figure 382-1 Laryngoscopic view of respiratory papillomas causing near complete obstruction at glottic level.

(From Derkay CS, Wiatrak B: Recurrent respiratory papillomatosis: a review, Laryngoscope 118:1236–1245, 2008.)

Treatment

The treatment of RRP is endoscopic surgical removal. Most surgeons in North America prefer the microdébrider. Laryngopharyngeal reflux can require treatment when reflux laryngitis is a factor. Supplemental medical management including injection of cidofovir may benefit some patients.

Bibliography

Derkay CS, Wiatrak B. Recurrent respiratory papillomatosis: a review. Laryngoscope. 2008;118:1236-1245.

Hawks M, Campisi P, Zafar R, et al. Time course of juvenile onset recurrent respiratory papillomatosis caused by human papillomavirus. Pediatr Infect Dis J. 2008;27:149-154.

382.3 Congenital Subglottic Hemangioma

Lauren D. Holinger

Clinical Manifestations

Typically, congenital subglottic hemangiomas are symptomatic within the 1st 2 mo of life, almost all occurring before 6 mo of age. Stridor is biphasic but usually more prominent during inspiration. The infant may be hoarse, have a barking cough, and present with croup. Fifty percent of congenital subglottic hemangiomas are associated with facial lesions. Radiographs classically delineate an asymmetric subglottic narrowing. The diagnosis is made by direct laryngoscopy.

Treatment

Medical management includes systemic steroids. Prednisone 2-4 mg/kg/day is given orally for 4-6 wk, less if the lesions stabilize sooner. The dosage is then tapered. If there is no response, the drug is discontinued. Propranolol 2-3 mg/kg/day has also been effective.

Although tracheostomy establishes a safe airway, every effort should be made to find an alternative. Corticosteroids can also be injected directly into the lesion. Endoscopic excision with the CO₂ laser is effective. Combining several modalities increases the possibility of avoiding tracheotomy. External surgical excision is suitable for some lesions.

Bibliography

Leaute-Labreze C, Dumas de la Roque E, Hubiche T, et al. Propranolol for severe hemangiomas of infancy. N Engl J Med. 2008;358(24):2649-2651.

382.4 Vascular Anomalies

Lauren D. Holinger

Vascular malformations are not true neoplastic lesions. They have a normal rate of endothelial turnover and various channel abnormalities. They are categorized by their predominant type (capillary, venous, arterial, lymphatic, or a combination thereof). Slow-flow malformations have capillary, lymphatic, or venous components. These incorrectly were called capillary hemangiomas, cystic hygromas or lymphangiomas, and cavernous hemangiomas, respectively.

Lymphatic malformations (cystic hygromas) rarely occur in the larynx. When they do, they are invariably an extension of disease from elsewhere in the head and neck. Airway obstruction can necessitate tracheostomy. The lesion can be debulked with the CO₂ laser.

382.5 Other Laryngeal Neoplasms

Lauren D. Holinger

Neurofibromatosis rarely involves the larynx. When children are affected, limited local resection is undertaken to maintain an airway and optimize the voice. Complete surgical extirpation is virtually impossible without debilitating resection of vital laryngeal structures. Most surgeons select the option of less-aggressive symptomatic surgery because of the poorly circumscribed and infiltrative nature of these fibromas. Rhabdomyosarcoma and other malignant tumors of the larynx are rare. Symptoms of hoarseness and progressive airway obstruction prompt initial evaluation by flexible laryngoscopy in the office.

382.6 Tracheal Neoplasms

Lauren D. Holinger

Tracheal tumors include malignant and benign neoplasms. The two most common benign tumors are inflammatory pseudotumor and hamartoma. The inflammatory pseudotumor is probably a reaction to a previous bronchial infection or traumatic insult. Growth is slow and the tumor may be locally invasive. Hamartomas are tumors of primary tissue elements that are abnormal in proportion and arrangement.

Tracheal neoplasms manifest with stridor, wheezing, cough, or pneumonia and are rarely diagnosed until 75% of the lumen has been obstructed (Fig 382-2). Symptoms mimic asthma and are often misdiagnosed as such. Chest radiographs or airway films can identify the obstruction. Pulmonary function studies demonstrate an abnormal flow-volume loop. A mild response to bronchodilator therapy may be misleading. Rational treatment is based upon the histopathology.

Figure 382-2 A CT scan of the trachea with a circumscribed intraluminal tracheal mass (arrow) in the tracheal wall.

(From Venizelos I, Papathomas T, Anagnostou E, et al: Pediatric inflammatory myofibroblastic tumor of the trachea: a case report and review of the literature, Pediatr Pulmonol 43:831–835, 2008.)