MAUI, HAWAII — All that appears to be croup is not, said Dr. Seth M. Pransky.
The conditions mimicking croup range from anatomical problems to gastroesophageal reflux disease (GERD) to an infection contracted at birth, said Dr. Pransky, director of pediatric otolaryngology at Rady Children's Hospital, San Diego. They include the following:
▸ Subglottic hemangioma. These patients “present with 'croup' at about 6 weeks of age, but they have no fever, they have a good cry, and up until that point they've been eating pretty well,” he said at a meeting sponsored by the University Childrens Medical Group and the American Academy of Pediatrics. “But they come in with some respiratory difficulty.”
These patients have a submucosal mass, located beneath the vocal cords, most likely in the left posterolateral wall, he explained. Females are more likely to have it than are males, by a ratio of 3.5:1.
Management options include the use of steroids, GERD therapy, and—in life-threatening cases only—vincristine, he said. Surgery is usually necessary and often is done endoscopically. Open surgical excision also can be performed. Rarely, tracheostomy is necessary.
▸ Postintubation glottic and subglottic lesions. These may be caused by either long or quick intubations, he said at the meeting, which was also sponsored by California Chapter 2 of the AAP.
In the subglottis, mucous glands abraded by intubation become obstructed and secrete heavily, creating a thick-walled cyst, especially when the patient has a cold; this causes airway obstruction.
“These patients present with stridor, typically when they have a cold,” he said. The situation usually resolves with the abatement of the cold. A history will reveal the prior intubation that caused the situation, and there are surgical options for treatment.
▸ Congenital airway abnormalities. These include glottic webs, which tend to be thick and to extend to the subglottis. They include the 22q deletion syndrome, which is associated with a variety of abnormalities, including speech abnormalities. It's a form of laryngeal atresia, an abnormality in the pharynx. There are surgical options for treatment.
▸ Laryngeal clefts. These are associated with recurrent cough and aspiration problems, he explained. “Clefts [classified as Veau I or II] can be treated endoscopically. Clefts [classified as Veau III] go into the trachea itself and require open surgical intervention.”
▸ Recurrent respiratory papillomatosis (RRP). The patient may have a history of “asthma” or recurrent croup, but the correct diagnosis is usually identified at about age 3 years, although this infection has been found in neonates. Ask about maternal vaginal condylomata, Dr. Pransky recommended. In preadolescent patients, especially girls, think about the possibility of sexual abuse, because this disease is caused by the human papillomavirus (HPV). If the child was infected by the mother during delivery, keep in mind that the infection may clear from women within 2 years, and therefore may be absent from the mother by the time the physician finds the infection in the child.
The goals of treatment are to maintain the airway and voice and to prevent the disease from spreading to the lungs, where it can convert to a malignancy. Tracheostomy is avoided because the site of incision is where the virus is likely to go.
A drug that may be helpful in patients with RRP from HPV infection is cidofovir, which is approved for treatment of cytomegalovirus retinitis in HIV patients. Several studies have found “lasting remission in pediatric patients about 50% of the time,” he said. “Lasting remission is 5 years without disease,” he added.
But the most important measure to take in preventing RRP is vaccination for HPV, he emphasized.
▸ Gastroesophageal reflux disease (GERD). The occurrence of nocturnal cough is the most important piece of clinical information suggesting GERD. Ear-nose-throat manifestations of and associations with GERD include rhinitis of infancy, recurrent otitis, chronic sinusitis, stridor, hoarseness, chronic cough (especially nocturnal), halitosis, laryngospasm/apnea, severe laryngomalacia, recurrent croup, subglottic stenosis, recurrent bronchitis, and asthma.
▸ Eosinophilic esophagitis (EE). The symptoms of this disorder are similar to GERD but often do not respond to GERD therapy. Adolescents who do not have a history of a tracheoesophageal fistula but who gag on food or have swallowing problems may have EE, he said, “unless proven otherwise.”
“It's very hard to treat; right now, the only treatment is swallowed steroids,” he noted. He predicted that there would be a patented product available for EE treatment within a year.
▸ Foreign body. A history may reveal this problem, along with x-rays and the presence of a transient wheeze. The use of a double-headed stethoscope allows the physician to listen to both sides of the lungs at the same time and to check for subtle differences suggestive of a foreign body. “The critical issue for tracheal or airway foreign bodies is history, history, history,” said Dr. Pransky who disclosed that he is on the speakers' bureau for Merck & Co.; he also conducts research with ArthroCare Corp. and Medtronic Inc.