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Obstructive lingual thyroid causing sleep apnea: a case report and review of the literature
Sleep Medicine 5 (2004) 605–607 www.elsevier.com/locate/sleep
Case report
Obstructive lingual thyroid causing sleep apnea: a case report and review of the literature Terrance W. Barnesa,*, Kerry D. Olsenb, Timothy I. Morgenthalera,c a
Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine, Desk East 18, Mayo Clinic, 200 1st St SW, Rochester, MN 55905, USA b Department of Otorhinolaryngology, Mayo Clinic College of Medicine, Rochester, MN c Mayo Clinic Sleep Disorders Center, Mayo Clinic College of Medicine, Rochester, MN Received 19 March 2004; received in revised form 18 June 2004; accepted 18 June 2004
Abstract Lingual thyroid has been reported to cause obstructive sleep apnea (OSA) only once in the literature. We present a case of a 49-year-old nonobese female with a 2-year history of progressive snoring, apneas, snort arousals, and daytime somnolence associated with the sensation of an ‘enlarging tongue’. She was found to have severe OSA caused by a large lingual thyroid. While positive airway pressure therapy was not successful, surgical resection of the thyroid was curative. OSA caused by lingual thyroid and other oropharyngeal/parapharyngeal tumors are discussed. A careful oropharyngeal examination is important in evaluating patients with complaints of OSA. q 2004 Elsevier B.V. All rights reserved. Keywords: Obstructive sleep apnea; Lingual thyroid; Tongue neoplasms; Oropharyngeal tumors; Pharyngeal tumors; Thyroid abnormalities
1. Introduction Obstructive sleep apnea syndrome (OSA) results in part from anatomic obstruction of the airway during sleep. Thyroid abnormalities, including the presence of ectopic thyroid tissue, represent a rare cause of this disorder. We present a patient with OSA caused by a lingual thyroid and we discuss several reports of OSA caused by other oropharyngeal and parapharyngeal tumors. 2. Case report A 49-year-old female was referred to our Sleep Disorders Center for three years of progressive, non-positional snoring, snort arousals, and apneas. Seven months earlier, she noted a sensation of tongue enlargement. She denied dysphagia, stridor, or orthopnea while awake, but she preferred sleeping in a reclining chair to avoid the sensation of her tongue ‘compressing the back of her throat’. The patient noted progressive daytime somnolence; her * Corresponding author. Tel.: C507-284-2957; fax: C507-266-4372. E-mail address: [email protected] (T.W. Barnes). 1389-9457/$ - see front matter q 2004 Elsevier B.V. All rights reserved. doi:10.1016/j.sleep.2004.06.005
Epworth Sleepiness Scale (ESS) was 15. Her weight remained at 140 pounds (63.5 kg, BMIZ23.3). On examination, the patient’s voice was slowed, slurred, and without audible stridor. Her tongue base appeared slightly enlarged and a firm, midline mass could be palpated in the posterior tongue base. No thyroid tissue was palpated in her anterior neck. On flexible laryngoscopy, a large, white, vascular mass was noted on the tongue base, nearly obstructing the posterior oropharynx. Beyond the mass, the larynx was normal. A thyroid uptake scan demonstrated iodine uptake in the lingual mass, with no evidence of thyroid tissue elsewhere. The patient was diagnosed with a lingual thyroid and referred to our institution for further evaluation and treatment recommendations. A magnetic resonance image (MRI) of the head and neck with and without gadolinium demonstrated only a 3.5 cm! 3.8 cm!4 cm heterogeneously enhancing mass arising from the tongue base (Fig. 1). The patient’s thyroid hormone levels were within normal range. Polysomnography demonstrated an apnea-hypopnea index of 79 per hour and, on oximetry, 62 desaturations greater than three percent per hour. Nasal continuous positive airway pressure (CPAP) at 8 cm of water pressure relieved obstructive events.
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T.W. Barnes et al. / Sleep Medicine 5 (2004) 605–607
Fig. 1. Horizontal (A and B) and coronal (C) MRI images of the head and neck demonstrating a 3.5 cm!3.8 cm!4 cm mass (solid arrows) arising from the tongue base and nearly completely obstruction the airway (dashed arrows). The mass demonstrates heterogenous enhancement following gadolinium administration (panel B).
The patient was started on nasal CPAP to treat her severe OSA. Supplemental thyroxine was prescribed in an attempt to suppress endogenous thyroid stimulating hormone production and thereby shrink the mass. Despite a month of suppressive therapy, the patient noted no change in the size of her tongue and she felt that her sleep and breathing had worsened despite CPAP. The patient was admitted to the hospital and underwent the transoral removal of the 7 cm!5 cm!1.5 cm mass and an awake tracheotomy. Pathology demonstrated only benign thyroid parenchyma, confirming the presumed diagnosis. Her tracheostomy was removed prior to discharge home. Eight weeks following the procedure, snoring and apneas had disappeared and her daytime somnolence was gone (ESSZ8). An overnight pulse oximetry performed on room air was normal. 3. Discussion Ectopic thyroid tissue develops because of the incomplete or failure of descent of thyroid tissue during embryogenesis. The tissue can be located at any point along the normal path of descent (thyroglossal duct) from the foramen cecum of the tongue to the isthmus of the thyroid, with the tongue base being the most common location. Ectopic thyroid tissue may mimic a thyroglossal duct cyst, but these entities can be differentiated based upon radioactive thyroid uptake scan. The prevalence of this abnormality is not well known, but is estimated to occur in one in 200,000 individuals or one in 6000 patients with thyroid disease [1]. Lingual thyroid is noted more frequently in women and the ectopic thyroid often represents the only functioning thyroid tissue. Hypothyroidism is frequently encountered in patients with lingual thyroid. Hyperthyroidism and thyroid malignancies have also been reported [2]. Symptoms may not develop unless the gland enlarges due to gland hypertrophy or malignancy. Many patients remain asymptomatic, although symptoms may include dysphagia, dysphonia, hemoptysis, and respiratory difficulties. The diagnosis is generally
suspected when physical examination demonstrates a midline tongue mass with the appearance of thyroid tissue and little or no palpable thyroid tissue in the anterior neck. If symptoms of hypothyroidism are noted, this should increase suspicion. Radioactive iodine uptake scan should demonstrate uptake in the mass, confirming the presence of thyroid tissue. Treatment options for symptomatic lingual thyroid may include hormone suppression with supplemental thyroxine, radioactive iodine ablation, and surgery. Lingual thyroid has been a reported cause of airway obstruction in children [3]. In adults, OSA due to a lingual thyroid has been reported only once in the literature, the patient being a pregnant, hypothyroid female [4]. She had documented obstructive and central sleep apnea that responded well to treatment with supplemental L-thyroxine, but she did not receive treatment with CPAP. There have been only a limited number of reports of oropharyngeal abnormalities that have resulted in OSA [5–10] (Table 1). Most reported cases have been male (17/20), with an age of 52.5G16.9 (meanGSD). Table 1 Previously reported parapharyngeal and oropharyngeal tumors associated with obstructive sleep apnea syndrome Tumor type
Number reported
Reference(s)
Lipoma Non-Hodgkin’s lymphoma Parotid tumor Plasmacytoma Paraganglioma vagus nerve Hemangioma Laryngeal cyst Lingual cyst Lingual tonsil hypertrophy Lingual thyroid Neurilemmoma Neurofibroma Retention cyst
5 2 2 2 1 1 1 1 1 1 1 1 1
[6,8,10] [6,7] [6] [5] [6] [6] [9] [9] [9] [4] [6] [6] [6]
T.W. Barnes et al. / Sleep Medicine 5 (2004) 605–607
The average duration of OSA symptoms reported was 2.9 years (range 2 months–10 years), with lipomas tending to present later and much larger. Most tumors were visible by direct intra-oral exam, and were subsequently investigated with CT, with MRI more commonly used in later reports. Clinicians should evaluate for OSA in patients with upper airway masses and abnormalities in an effort to identify the condition and to assess its response to CPAP. This information would also help guide the need for further treatment (medical or surgical). Often, CPAP provided only transient control of symptoms, but was not tolerated or effective over time. Speculatively, this is because tumors cause a more fixed anatomical obstruction than the dynamic neuromuscular control associated obstruction found in traditional OSA. It is not surprising; therefore that surgical therapy was in most cases successful and that following surgery, most patients did not require CPAP [6,9]. A careful evaluation for oropharyngeal masses should be performed in all patients with suspected OSA, especially for patients with the recent onset of symptoms (snoring, apnea, etc.) that are not associated with weight gain or those who do not tolerate CPAP. While direct intra-oral inspection reveals most abnormalities, otorhinological consultation and MRI are indicated when a parapharyngeal abnormality is suspected.
607
References [1] Di Benedetto V. Ectopic thyroid gland in the submandibular region simulating a thyroglossal duct cyst: a case report. J Pediatr Surg 1997; 32(12):1745–6. [2] Seoane JM, Cameselle-Teijeiro J, Romero MA. Poorly differentiated oxyphilic (Hurthle cell) carcinoma arising in lingual thyroid: a case report and review of the literature. Endocr Pathol 2002;13(4):353–60. [3] Chanin LR, Greenberg LM. Pediatric upper airway obstruction due to ectopic thyroid: classification and case reports. Laryngoscope 1988; 98(4):422–7. [4] Taibah K, Ahmed M, Baessa E, et al. An unusual cause of obstructive sleep apnoea presenting during pregnancy. J Laryngol Otol 1998; 112(12):1189–91. [5] Byrd Jr. RP, Roy TM, Bentz W, et al. Plasmacytoma as a cause of obstructive sleep apnea. Chest 1996;109(6):1657–9. [6] Desuter G, Castelein S, de Toeuf C, et al. Parapharyngeal causes of sleep apnea syndrome: two case reports and review of the literature. Acta Otorhinolaryngol Belg 2002;56(2):189–94. [7] Gomez-Merino E, Arriero JM, Chiner E, et al. Obstructive sleep apnea syndrome as first manifestation of pharyngeal non-Hodgkin’s lymphoma. Respiration 2003;70(1):107–9. [8] Hockstein NG, Anderson TA, Moonis G, et al. Retropharyngeal lipoma causing obstructive sleep apnea: case report including fiveyear follow-up. Laryngoscope 2002;112(9):1603–5. [9] Olsen KD, Suh KW, Staats BA. Surgically correctable causes of sleep apnea syndrome. Otolaryngol Head Neck Surg 1981;89(5):726–31. [10] Pellanda A, Zagury S, Pasche P. Parapharyngeal lipoma causing obstructive sleep apnea syndrome. Otolaryngol Head Neck Surg 2003; 128(2):301–2.
Case report
Obstructive lingual thyroid causing sleep apnea: a case report and review of the literature Terrance W. Barnesa,*, Kerry D. Olsenb, Timothy I. Morgenthalera,c a
Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine, Desk East 18, Mayo Clinic, 200 1st St SW, Rochester, MN 55905, USA b Department of Otorhinolaryngology, Mayo Clinic College of Medicine, Rochester, MN c Mayo Clinic Sleep Disorders Center, Mayo Clinic College of Medicine, Rochester, MN Received 19 March 2004; received in revised form 18 June 2004; accepted 18 June 2004
Abstract Lingual thyroid has been reported to cause obstructive sleep apnea (OSA) only once in the literature. We present a case of a 49-year-old nonobese female with a 2-year history of progressive snoring, apneas, snort arousals, and daytime somnolence associated with the sensation of an ‘enlarging tongue’. She was found to have severe OSA caused by a large lingual thyroid. While positive airway pressure therapy was not successful, surgical resection of the thyroid was curative. OSA caused by lingual thyroid and other oropharyngeal/parapharyngeal tumors are discussed. A careful oropharyngeal examination is important in evaluating patients with complaints of OSA. q 2004 Elsevier B.V. All rights reserved. Keywords: Obstructive sleep apnea; Lingual thyroid; Tongue neoplasms; Oropharyngeal tumors; Pharyngeal tumors; Thyroid abnormalities
1. Introduction Obstructive sleep apnea syndrome (OSA) results in part from anatomic obstruction of the airway during sleep. Thyroid abnormalities, including the presence of ectopic thyroid tissue, represent a rare cause of this disorder. We present a patient with OSA caused by a lingual thyroid and we discuss several reports of OSA caused by other oropharyngeal and parapharyngeal tumors. 2. Case report A 49-year-old female was referred to our Sleep Disorders Center for three years of progressive, non-positional snoring, snort arousals, and apneas. Seven months earlier, she noted a sensation of tongue enlargement. She denied dysphagia, stridor, or orthopnea while awake, but she preferred sleeping in a reclining chair to avoid the sensation of her tongue ‘compressing the back of her throat’. The patient noted progressive daytime somnolence; her * Corresponding author. Tel.: C507-284-2957; fax: C507-266-4372. E-mail address: [email protected] (T.W. Barnes). 1389-9457/$ - see front matter q 2004 Elsevier B.V. All rights reserved. doi:10.1016/j.sleep.2004.06.005
Epworth Sleepiness Scale (ESS) was 15. Her weight remained at 140 pounds (63.5 kg, BMIZ23.3). On examination, the patient’s voice was slowed, slurred, and without audible stridor. Her tongue base appeared slightly enlarged and a firm, midline mass could be palpated in the posterior tongue base. No thyroid tissue was palpated in her anterior neck. On flexible laryngoscopy, a large, white, vascular mass was noted on the tongue base, nearly obstructing the posterior oropharynx. Beyond the mass, the larynx was normal. A thyroid uptake scan demonstrated iodine uptake in the lingual mass, with no evidence of thyroid tissue elsewhere. The patient was diagnosed with a lingual thyroid and referred to our institution for further evaluation and treatment recommendations. A magnetic resonance image (MRI) of the head and neck with and without gadolinium demonstrated only a 3.5 cm! 3.8 cm!4 cm heterogeneously enhancing mass arising from the tongue base (Fig. 1). The patient’s thyroid hormone levels were within normal range. Polysomnography demonstrated an apnea-hypopnea index of 79 per hour and, on oximetry, 62 desaturations greater than three percent per hour. Nasal continuous positive airway pressure (CPAP) at 8 cm of water pressure relieved obstructive events.
606
T.W. Barnes et al. / Sleep Medicine 5 (2004) 605–607
Fig. 1. Horizontal (A and B) and coronal (C) MRI images of the head and neck demonstrating a 3.5 cm!3.8 cm!4 cm mass (solid arrows) arising from the tongue base and nearly completely obstruction the airway (dashed arrows). The mass demonstrates heterogenous enhancement following gadolinium administration (panel B).
The patient was started on nasal CPAP to treat her severe OSA. Supplemental thyroxine was prescribed in an attempt to suppress endogenous thyroid stimulating hormone production and thereby shrink the mass. Despite a month of suppressive therapy, the patient noted no change in the size of her tongue and she felt that her sleep and breathing had worsened despite CPAP. The patient was admitted to the hospital and underwent the transoral removal of the 7 cm!5 cm!1.5 cm mass and an awake tracheotomy. Pathology demonstrated only benign thyroid parenchyma, confirming the presumed diagnosis. Her tracheostomy was removed prior to discharge home. Eight weeks following the procedure, snoring and apneas had disappeared and her daytime somnolence was gone (ESSZ8). An overnight pulse oximetry performed on room air was normal. 3. Discussion Ectopic thyroid tissue develops because of the incomplete or failure of descent of thyroid tissue during embryogenesis. The tissue can be located at any point along the normal path of descent (thyroglossal duct) from the foramen cecum of the tongue to the isthmus of the thyroid, with the tongue base being the most common location. Ectopic thyroid tissue may mimic a thyroglossal duct cyst, but these entities can be differentiated based upon radioactive thyroid uptake scan. The prevalence of this abnormality is not well known, but is estimated to occur in one in 200,000 individuals or one in 6000 patients with thyroid disease [1]. Lingual thyroid is noted more frequently in women and the ectopic thyroid often represents the only functioning thyroid tissue. Hypothyroidism is frequently encountered in patients with lingual thyroid. Hyperthyroidism and thyroid malignancies have also been reported [2]. Symptoms may not develop unless the gland enlarges due to gland hypertrophy or malignancy. Many patients remain asymptomatic, although symptoms may include dysphagia, dysphonia, hemoptysis, and respiratory difficulties. The diagnosis is generally
suspected when physical examination demonstrates a midline tongue mass with the appearance of thyroid tissue and little or no palpable thyroid tissue in the anterior neck. If symptoms of hypothyroidism are noted, this should increase suspicion. Radioactive iodine uptake scan should demonstrate uptake in the mass, confirming the presence of thyroid tissue. Treatment options for symptomatic lingual thyroid may include hormone suppression with supplemental thyroxine, radioactive iodine ablation, and surgery. Lingual thyroid has been a reported cause of airway obstruction in children [3]. In adults, OSA due to a lingual thyroid has been reported only once in the literature, the patient being a pregnant, hypothyroid female [4]. She had documented obstructive and central sleep apnea that responded well to treatment with supplemental L-thyroxine, but she did not receive treatment with CPAP. There have been only a limited number of reports of oropharyngeal abnormalities that have resulted in OSA [5–10] (Table 1). Most reported cases have been male (17/20), with an age of 52.5G16.9 (meanGSD). Table 1 Previously reported parapharyngeal and oropharyngeal tumors associated with obstructive sleep apnea syndrome Tumor type
Number reported
Reference(s)
Lipoma Non-Hodgkin’s lymphoma Parotid tumor Plasmacytoma Paraganglioma vagus nerve Hemangioma Laryngeal cyst Lingual cyst Lingual tonsil hypertrophy Lingual thyroid Neurilemmoma Neurofibroma Retention cyst
5 2 2 2 1 1 1 1 1 1 1 1 1
[6,8,10] [6,7] [6] [5] [6] [6] [9] [9] [9] [4] [6] [6] [6]
T.W. Barnes et al. / Sleep Medicine 5 (2004) 605–607
The average duration of OSA symptoms reported was 2.9 years (range 2 months–10 years), with lipomas tending to present later and much larger. Most tumors were visible by direct intra-oral exam, and were subsequently investigated with CT, with MRI more commonly used in later reports. Clinicians should evaluate for OSA in patients with upper airway masses and abnormalities in an effort to identify the condition and to assess its response to CPAP. This information would also help guide the need for further treatment (medical or surgical). Often, CPAP provided only transient control of symptoms, but was not tolerated or effective over time. Speculatively, this is because tumors cause a more fixed anatomical obstruction than the dynamic neuromuscular control associated obstruction found in traditional OSA. It is not surprising; therefore that surgical therapy was in most cases successful and that following surgery, most patients did not require CPAP [6,9]. A careful evaluation for oropharyngeal masses should be performed in all patients with suspected OSA, especially for patients with the recent onset of symptoms (snoring, apnea, etc.) that are not associated with weight gain or those who do not tolerate CPAP. While direct intra-oral inspection reveals most abnormalities, otorhinological consultation and MRI are indicated when a parapharyngeal abnormality is suspected.
607
References [1] Di Benedetto V. Ectopic thyroid gland in the submandibular region simulating a thyroglossal duct cyst: a case report. J Pediatr Surg 1997; 32(12):1745–6. [2] Seoane JM, Cameselle-Teijeiro J, Romero MA. Poorly differentiated oxyphilic (Hurthle cell) carcinoma arising in lingual thyroid: a case report and review of the literature. Endocr Pathol 2002;13(4):353–60. [3] Chanin LR, Greenberg LM. Pediatric upper airway obstruction due to ectopic thyroid: classification and case reports. Laryngoscope 1988; 98(4):422–7. [4] Taibah K, Ahmed M, Baessa E, et al. An unusual cause of obstructive sleep apnoea presenting during pregnancy. J Laryngol Otol 1998; 112(12):1189–91. [5] Byrd Jr. RP, Roy TM, Bentz W, et al. Plasmacytoma as a cause of obstructive sleep apnea. Chest 1996;109(6):1657–9. [6] Desuter G, Castelein S, de Toeuf C, et al. Parapharyngeal causes of sleep apnea syndrome: two case reports and review of the literature. Acta Otorhinolaryngol Belg 2002;56(2):189–94. [7] Gomez-Merino E, Arriero JM, Chiner E, et al. Obstructive sleep apnea syndrome as first manifestation of pharyngeal non-Hodgkin’s lymphoma. Respiration 2003;70(1):107–9. [8] Hockstein NG, Anderson TA, Moonis G, et al. Retropharyngeal lipoma causing obstructive sleep apnea: case report including fiveyear follow-up. Laryngoscope 2002;112(9):1603–5. [9] Olsen KD, Suh KW, Staats BA. Surgically correctable causes of sleep apnea syndrome. Otolaryngol Head Neck Surg 1981;89(5):726–31. [10] Pellanda A, Zagury S, Pasche P. Parapharyngeal lipoma causing obstructive sleep apnea syndrome. Otolaryngol Head Neck Surg 2003; 128(2):301–2.