COWDEN's disease, known also as multiple hamartoma syndrome, is a rare, predominantly inherited condition characterized by various ecto-, meso-, and endodermal benign and malignant tumors that may affect the skin; oropharyngeal, laryngeal, and gastrointestinal mucosa; thyroid; breast; and other organs. 1,2We report a case of airway obstruction during induction of general anesthesia caused by the presence of extended multiple papillomas on the lingual tonsils, epiglottis, and the surrounding structure, for which a diagnosis of Cowden's disease was made postoperatively.
A 55-yr-old woman was scheduled for elective mastectomy for cancer of the right breast. At the age of 12 yr, the patient had undergone bilateral tonsillectomy. Three years later, she underwent surgery for chronic sinusitis. At age 27 yr, she underwent partial thyroidectomy for benign adenomatous changes in the right lobe of the thyroid gland. The patient denied having had any complications from previous anesthesia. Preoperative plain radiograph and computed tomography images showed a giant adenoma in the left lobe of the thyroid gland that did not show any signs of compressing the trachea. The patient had neither history of decreased activity nor any obvious breathing difficulty.
Anesthesia was induced with intravenous propofol (1.2 mg/kg) and vecuronium (0.1 mg/kg). While performing tracheal intubation, visualization of the hypopharynx by direct laryngoscopy showed a mass of tumors occupying the airway. The epiglottis and the surrounding structures could not be identified because of the presence of numerous papilloma-like lesions. Two blind oral intubations were attempted without success. Bleeding from the papillomas was easily induced during this procedure. Thereafter, complete airway obstruction developed. A fiberoptic intubation attempt was also unsuccessful because of the nature of the papillomas and bloody secretions that obscured the visual field. The patient's oxyhemoglobin saturation had decreased to < 40%, and urgent tracheostomy was performed. A 6.0-mm ID endotracheal tube was inserted via the tracheostomy incision, resulting in an increase in oxyhemoglobin saturation to 100% with an inspired oxygen fraction of 1.0. The patient was delivered to the intensive care unit and treated under mild hypothermia (34°C) for 3 days to prevent brain damage from hypoxia. This resulted in full recovery of consciousness without any neurologic complications. Fiberoptic laryngoscopy performed in the intensive care unit showed extensive confluent papillomatous lesions on the root of the tongue and hypopharynx (fig. 1). However, the laryngoscope was not able to be advanced near the larynx because the hypopharynx was occupied by the extended papillomas. Laryngoscopy performed 5 days later showed that the papillomas on the lingual tonsils extended posteriorly beyond the tip of the epiglottis and filled the valleculae (fig. 2). Barium fluoroscopic examination also showed multiple papillomas on the hypopharynx and the esophagus (fig. 3). Eight days later, right mastectomy, left thyroidectomy, and resection of the papillomatous lesions on the lingual tonsils were performed. The pathology report showed that the lesions were hamartoma. Postoperative endoscopic examination showed a large number of polyps in the esophagus, hyperplastic polyposis and tubular adenomas in the stomach, and a hamartoma in the sigmoid colon. In addition, the patient's daughter and son also presented with some papillomas on the root of the tongue. On the basis of these results, the patient was diagnosed as having Cowden's disease.
This is the first reported case of critical airway obstruction that developed during induction of anesthesia, was caused by oropharyngeal and laryngeal papillomas, and was later proved to be a rare condition of Cowden's disease.
Cowden's disease is a rare inherited genodermatosis, the major features of which are multiple hamartomatous papules and nodules on the skin and oropharyngeal, laryngeal, and gastrointestinal mucosa, together with thyroid and breast anomalies and polyposis of the gastrointestinal tract. 1,2Since the first report of this disease more than 35 yr ago, 1< 200 cases have been documented. In > 80% of reported cases, papular or verrucous oral lesions on the lips, tongue, gingiva, edentulous alveolus, buccal mucosae, palate, or tonsillar fossae have been found. 3–5However, there has been no report of critical airway obstruction caused by pharyngeal and laryngeal lesions occurring during induction of anesthesia as a result of Cowden's disease.
In the present case, Cowden's disease was not diagnosed preoperatively, although the patient had a thyroid tumor and breast cancer. Ovassapian 6described three cases of difficult intubations caused by a mass at the base of the tongue. He concluded that such cases cannot be identified by routine external physical examination of the airway and that anesthesiologists will continue to be surprised by unexpected difficult airways and difficult intubations. In fact, the lingual tonsils, being located on the posterior one third of the tongue in the hypopharynx, would not usually be visible by simple visual inspection of the oropharynx without the use of a mirror or endoscope. However, in the present case, we did a less-than-thorough airway examination preoperatively. If we did as thorough an evaluation of the preoperative intra-oral examination and on the computed tomography scans as was possible, it would be possible to predict the patient's oropharyngeal condition.
A difficult airway is particularly challenging for anesthesiologists. In the present case, we performed a fiberscopic trial in an attempt to achieve intubation within a limited period. However, our decision was inappropriate because we had known about the lack of space in the hypopharynx in which to advance the fiberscope and the bloody secretions that obscured the visual field. Laryngeal mask airway, Combitube (Kendall, Argyle, NY), or transtracheal jet ventilation might be of value to try to establish ventilation, but we do not know if the laryngeal mask airway would work in this particular case. We decided to perform tracheostomy when the patient's oxyhemoglobin saturation had become critical. However, we should have proceeded more rapidly to a tracheostomy once we encountered trouble.
In summary, multiple papillomas on the lingual tonsils, epiglottis, and the surrounding structure that occupied the hypopharynx caused difficulty in airway management and prevented tracheal intubation during induction of anesthesia. This is the first reported case of difficulty with airway management in a patient with the Cowden's disease.