Misdiagnosed rare subglottic lesions with bronchial asthma as the initial symptom

Unlabelled image American Journal of Emergency Medicine 31 (2013) 1295.e1-1295.e4

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American Journal of Emergency Medicine

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Case Report

Misdiagnosed rare subglottic lesions with Bronchial asthma as the initial symptom?

Abstract

Early-stage subglottic lesions are easily misdiagnosed as bronchial asthma. We report on 4 cases of rare subglottic lesions. Our aim is to provide insights for the accurate diagnosis of subglottic lesions.

Four cases of subglottic lesions with bronchial asthma as the initial symptom were studied retrospectively. The lesions were a mixed tumor, a mucoepidermoid carcinoma, laryngeal amyloidosis, and relapsing chondritis.

All of the patients had received standard but ineffective antiasthma treatment when their tumors were in the early stage. Each patient underwent a tracheotomy because of a laryngeal obstruction. Each patient was treated properly and in a timely manner after the primary disease had been diagnosed, and the tracheal cannula was removed; however, 1 patient survived with a tracheal cannula in place.

We must be aware of the risk of ineffective treatment because subglottic lesions may be misdiagnosed as bronchial asthma. The accurate diagnosis and timely treatment of the primary disease are critical to a good prognosis.

Bronchial asthma is a chronic disease of the airways that is characterized by variable airflow obstruction, airway hyperrespon- siveness, and airway inflammation [1]. However, some endotra- cheal diseases are often misdiagnosed as bronchial asthma, including the presence of a foreign body [2] and benign or malignant tumors [3-5]. Laryngeal inflammation can be misdiag- nosed as or masked by bronchial asthma, as in the case of vocal cord dysfunction [6]. Laryngeal tumors can also be misdiagnosed as bronchial asthma and thereby mismanaged. Children with laryn- geal papillomas can receive treatment for bronchial asthma [7]. We are able to detect endotracheal tumors, but not laryngeal tumors, in a timely fashion, especially early-stage subglottic tumors that are observable by bronchoscopy. A patient with a laryngeal chondroma presented with recurring episodes of asthma. He was admitted in a Severe asthmatic state that could not be resolved with the usual bronchodilatatory therapy. He died before an emergency tracheot- omy could be performed, and the tumor was confirmed by autopsy [8]. A 16-year-old adolescent girl with subglottic adenoid cystic carcinoma was reported to have been misdiagnosed as having asthma [9]. She presented with long-term exacerbating dyspnea. The patient was successfully treated by surgery and postoperative radiotherapy. The etiology and pathogenesis of subglottic lesions need to be better elucidated. Because bronchial asthma is the initial symptom of some rare subglottic lesions, the diagnosis and treatment of the primary disease are usually delayed. We report on 4 patients with various subglottic lesions who were misdiag-

? Funding source: Science and Technology Bureau of Suzhou (No:SYS201228).

nosed with bronchial asthma. Each patient underwent time- consuming and ineffective treatment before receiving a tracheot- omy to relieve shortness of breath.

We conducted a retrospective analysis of 4 patients who were admitted to our hospital with rare subglottic lesions that were misdiagnosed as bronchial asthma. All 4 cases of subglottic lesions with bronchial asthma as the initial symptom were reviewed. Their medical records were examined to obtain information on the patients’ age, sex, location of the lesions, misdiagnosis, tracheot- omy, duration of misdiagnosis, histopathologic results, treatment, and outcome. One patient was cured, and the other 3 patients were followed up for 1 to 3 years. Details of 4 cases of rare subglottic lesions are shown in the Table. This study was approved by the Institutional Review Board of the First Affiliated Hospital of Soochow University.

All of the patients exhibited the symptoms of bronchial asthma when they First visited a doctor. They received antiasthma treatment from a doctor in the respiratory department. However, the intermit- tent asthma was not mitigated, and all of the patients eventually experienced laryngeal obstruction and required a tracheotomy to relieve their dyspnea. We discovered that they had subglottic or (and) glottic lesions by performing video fibrolaryngoscopy and computed tomography (CT); the lesions were ultimately diagnosed by histopa- thology. The 2 patients underwent tumor resection, whereas the other 2 patients received immunotherapy. We recommended postoperative radiotherapy for the 1 patient who had a malignant tumor.

Histopathology of their biopsy samples confirmed that each of thepatients had a rare subglottic lesion. The lesions were a mixed tumor, a mucoepidermoid carcinoma, laryngeal amyloidosis, and relapsing chondritis. The diagnosis of bronchial asthma was eventually excluded for these 4 patients. All of the patients survived in good health without dyspnea. The tracheal cannulas of 3 of the patients were removed

after their primary diseases were controlled.

A 52-year-old man complained of a cough and asthma for 8 years. His symptoms exacerbated, and he began to complain of dyspnea 3 days before coming to the hospital. He was diagnosed with bronchial asthma before he came to our department. His symptoms were not resolved after receiving the standard treatment for asthma. He did not have an allergic disorder or a family history of bronchial asthma. His pulmonary function tests revealed an obstructive pattern. When admitted to the emergency ward, he presented with hypoventilation and dyspnea. While oxygen was being delivered, the patient experienced worsening dyspnea and a sharp decline in the oxygen partial pressure, followed by cardiac arrest. Inserting a small tracheal cannula and cardiopulmonary resuscitation restored the patient’s vital signs, including his breathing and heartbeat. The anesthetist provided the crucial information that a large subglottic blockage had been encountered during the endotracheal intubation. We performed a tracheotomy on the next day, when the patient’s vital signs had

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Table

Details of 4 cases of rare subglottic lesions

Patient no	Sex	Age	Location of lesions	Misdiagnosis with bronchial asthma	Tracheotomy	Duration of misdiagnosis (y)	Histology	Treatment	Prognosis
1	M	52	Subglottic	Yes	Yes	8	Mixed tumor	Surgery	Cured
2	F	48	Subglottic	Yes	Yes	1	Mucoepidermoid	Surgery + radiotherapy	Under follow -up for 2 y^a
3	F	56	Glottic and	Yes	Yes	2	carcinoma Amyloidosis	Laser surgery + immunotherapy	Under follow -up for 1 y^a
4	M	45	subglottic Glottic and	Yes	Yes	3	Relapsing chondritis	Laser surgery + immunotherapy	Under follow -up for 3 y^a
			subglottic

Abbreviations: M, male; F, female.

^a The follow-up is ongoing as of the date of publication.

returned to normal. The patient regained consciousness 2 days later and breathed free of the ventilator 1 week later. We then performed a cervicothoratic CT on this patient and found that his subglottic region was nearly filled with a neoplasm (Fig. 1A). Video fibrolaryngoscopy revealed a subglottic tumor covered by a pale smooth mucous membrane. It was confirmed to be a mixed tumor by a histopathologic examination of a biopsy obtained using direct laryngoscopy (Fig. 1B). We performed a subglottic tumor resection through the Cricothyroid membrane. There was a neoplasm located on the posterior wall of the subglottic trachea. We removed the tumor without damaging the lamina of the cricoid cartilage. The patient recovered completely. We therefore unplugged the tracheal cannula after 48 hours of steady breathing. No evidence of recurrence was observed by video fibrolaryngoscopy 2 years after the surgery.

A 48-year-old woman who had dyspnea for 1 year came to our department. She had visited the respiratory department and been

diagnosed with asthma. However, her symptoms were not relieved but instead deteriorated. Because we suspected that she had a life- threatening laryngeal obstruction, we performed a tracheotomy. We performed a video fibrolaryngoscopy after she was breathing normally. The fibrolaryngoscopy image indicated the presence of a tumor with a pale rugged surface in the right subglottic region. Cervicothoratic CT confirmed the presence of a subglottic mass (Fig. 2A). We performed a direct laryngoscopy and biopsy under general anesthesia. The tumor was pathologically confirmed to be a well-differentiated mucoepidermoid carcinoma (Fig. 2B). The patient refused to allow a total laryngotomy because she insisted on retaining her vocal function. Therefore, we resected the tumor through the cricothyroid membrane and performed a selective dissection on the right side of the neck. After the operation, the patient received adjuvant radiotherapy. No evidence of a relapse of the tumor was observed 2 years later.

Fig. 1. Laryngeal CT scan revealed severe stenosis and soft tissue in the subglottic area. A, The white arrowhead indicates the neoplasm. B, Histopathology revealed a mixed tumor (hematoxylin and eosin staining) (magnification x200).

Fig. 2. Laryngeal CT scan revealed a neoplasm in the subglottic area. A, The white arrowhead indicates the neoplasm. B, Histopathology revealed a well-differentiated mucoepidermoid carcinoma (hematoxylin and eosin staining) (magnificationx200).

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A 56-year-old woman complained of intermittent asthma for 2 years. She was diagnosed with bronchial asthma and was treated accordingly. She underwent a long-term antispasmodic and antiasth- matic treatment that failed to relieve her breathing problems. She complained of chronic hoarseness for 3 months. Her physical condition worsened progressively until she could climb less than 1 flight of stairs. A respiratory doctor performed a bronchoscopy. However, there was an accident during the operation. The patient experienced apnea for 1 minute but retained a steady heartbeat. Tracheal cannula and cardiopulmonary resuscitation restored her normal breathing. The patient was admitted to our department and received a tracheotomy. To determine whether the apnea had been caused by a laryngeal obstruction, we performed a video fibrolar- yngoscopy and confirmed the presence of extensive subglottic lesions (Fig. 3A). The histopathologic examination revealed that the lesions were the result of laryngeal amyloidosis with chronic inflammation (Fig. 3B). The patient received immunotherapy after we resected the tumor using a CO₂ laser. The patient was surviving with a tracheal cannula in place 1 year later.

A 45-year-old man complained of bronchial asthma for 3 years. He was treated with antiasthma drugs after visiting the respiratory department. His symptoms began to exacerbate approximately 3 months before he was admitted to our department. After the patient had been sent to the emergency department for shortness of breath,

Fig. 3. A, Video fibrolaryngoscopy revealed extensive lesions in the glottic and subglottic areas. B, Histopathology revealed an amyloidosis with chronic inflammation (hematoxylin and eosin staining) (magnification x200).

a tracheotomy was performed to resolve his breathing symptom. A laryngeal endoscopic examination revealed a broad soft tissue hyperplasia in the glottic and subglottic regions. A laryngeal CT scan verified the presence of a subglottic neoplasm (Fig. 4A). A biopsy was performed, which demonstrated that the neoplasm was an inflammatory reaction. We performed CO₂ laser surgery on this patient. The patient returned to the hospital for reexamination 1 month after the surgery. An endoscopic examination showed hyperplasia and stenosis in the subglottic region. A biopsy was performed, and the pathologic diagnosis was still inflammation. The patient received antibacterial drugs and lived with only a tracheal cannula in place. Two weeks later, the patient presented with a saddle nose but without auricular chondritis (Fig. 4B). Laboratory tests revealed a high erythrocyte sedimentation rate value. The test for antinuclear antibodies was positive, and the staff of the department of rheumatology then doubted the patient with relapsing chondritis. Steroid pulse therapy significantly relieved his symptoms. He then received routine immunosuppressant therapy, including oral prednisone and cyclophosphamide or methotrexate. The patient could breathe normally after the tracheal cannula was removed 1 year later.

Bronchial asthma is the most common disease observed in respiratory departments. With industrialization, increasingly large numbers of allergic factors are contributing to asthma. Many diseases with similar symptoms can be misdiagnosed as bronchial asthma.

Fig. 4. Laryngeal CT scan revealed soft tissue surrounding the subglottic area. A, The white arrowhead indicates the neoplasm. The profile photograph of the patient reveals that he has a saddle nose. B, The white arrowhead indicates the saddle nose.

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When patients have atypical symptoms and signs and a poor response to standard asthma therapy, we must be aware of the possibility of a different diagnosis [10].

An airway obstructing lesion should be considered if the symptoms of asthma persist throughout continuous antiasthmatic therapy [11]. Endotracheal tumors and laryngeal diseases, specifi- cally subglottic lesions, can be easily misdiagnosed as bronchial asthma. We reported on 4 patients with rare subglottic lesions, none of whom had a family history of asthma or allergy or a history of childhood asthma.

The symptoms of an early-stage subglottic tumor are not unique. The characteristic symptoms are dyspnea, prolonged cough, wheez- ing, and stridor, which are similar to the symptoms of bronchial asthma. The tumor is rarely observable and can easily be ignored until it evolves into an advanced state [12]. Subglottic tumors can be misdiagnosed as asthma [13]. The 4 cases that we reported were slow- growing, rare diseases that were wrongly diagnosed as asthma over a long period. The dyspnea that appeared eventually was provoked by a subglottic ventilation disorder that impeded the delivery of air to the alveoli. All of the patients received a tracheotomy to relieve the dyspnea. Therefore, the precise diagnosis of subglottic lesions is extremely challenging.

It is difficult for a doctor to examine the subglottic region because of its anatomy. However, we can obtain a precise diagnosis much more quickly if we use endoscopy or imaging in the early stages. Laryngoscopy may be helpful in detecting subglottic lesions with obvious signs, but it is not helpful in detecting early-stage lesions that are hidden from the reach of the light, especially submucosal lesions. Enhanced imaging studies of some lesions, such as malignant tumors that display remarkable potentialization, may be helpful. The diagnosis of a subglottic carcinoma should be considered, even if the pulmonary function test, chest CT, and fiberoptic bronchoscopy show no evident abnormality, as in the case that we reported of a patient with a mucoepidermoid carcinoma. However, we also need to consider that a small percentage of patients will refuse to have a physical examination because of the increasing cost of medical care, especially those patients in Developing countries.

Mixed tumor (pleomorphic adenoma) and mucoepidermoid carcinoma are the most common tumors of the salivary glands, but they rarely occur in the larynx. A mixed tumor can occur in any part of the larynx with an intact mucosal lining [14]. A subglottic mixed tumor is a benign disease that develops slowly [15]. A well-differentiated mucoepidermoid carcinoma, although it is a type of malignant tumor, is difficult to detect because it grows slowly and seldom metastasizes. Laryngeal amyloidosis is an uncommon disease, accounting for 1% of the benign lesions of the larynx. It is also easily mistaken for asthma, especially when the tracheobronchial tree is affected [16].

Relapsing polychondritis is a multisystem disease that is characterized by the recurrent inflammation of cartilaginous and noncartilaginous structures, such as connective tissue. It is an immune system disease with a low incidence and unclear patho- genesis. The elastic cartilage of the ears and nose is most often damaged, followed by the hyaline cartilage of the peripheral joints, the vertebral fibrocartilage, and the tracheobronchial cartilage. The respiratory tract is thought to be involved in as many as 50% of the documented cases [17]. Relapsing polychondritis patients are often first diagnosed in respiratory or immunology departments. The symptoms of case 4 were similar to those of relapsing polychondritis, with laryngitis and unaffected ears and nose. We could not give the correct diagnosis, although he had had asthma for a long time. We did not make the correct diagnosis until he had a laryngeal obstruction. If we had considered immunological tests earlier, the misdiagnosis might have been avoided.

Respiratory doctors are usually unable to diagnose a laryngeal disease during an attack of intermittent asthma. An early-stage subglottic tumor may have little effect on pulmonary function, so the

pulmonary ventilatory function may appear normal. If the lesion is small and is not shifted by the patient’s respiration, the lesion thus does not affect the pulmonary ventilatory function. The lesion usually becomes symptomatic when it occludes more than 75% of the lumen [18]. The symptoms of a slow-growing tumor in the subglottic region are closely related to the size of the mass. Therefore, the disease is often diagnosed at a late stage, and the treatment fails.

The final diagnosis is based on the imaging and histopathologic results. Surgical excision of the tumor, as in cases 1and 2, is the best option for treatment. The prognosis is good if the surgical excision is complete. Imaging is significantly helpful in reaching a diagnosis, and a histopathologic examination can provide a definitive diagnosis. However, laboratory tests are required for the diagnosis of case 4.

Patients with early-stage subglottic lesions may be misdiagnosed as bronchial asthma. An incorrect diagnosis leads to Improper treatment and may even lead to Life-threatening complications, such as laryngeal obstruction. The patients considered in this study ultimately had to receive a tracheotomy to save their lives. The accurate diagnosis and timely treatment of the primary disease affecting a patient are critical for a good prognosis.

Ya-Feng Yu, MD Hong-Yang Ling, MD Gen-Sheng Xiao, MD

Peng Sun, MD Man-Yi Li, MD Wen-Ying Wu, MD

Department of Otolaryngology the First Affiliated Hospital of Soochow University, Suzhou, China

E-mail address: [email protected]

http://dx.doi.org/10.1016/j.ajem.2013.04.023

References

AJEM

Misdiagnosed rare subglottic lesions with bronchial asthma as the initial symptom

Aleva RM, Wouters B, Kraan J, et al. Late onset asthma? Eur Respir J 1991;4(2): 239-41.

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