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A 51-Year-Old Woman With Hypoacusia and Increased Respiratory Effort in the Supine Position and OSA FREE TO VIEW

Marcos Fernández-Barriales, MD; Virginia Vázquez Marcos, MD; Ainhoa Álvarez, MD; Beatriz Odriozola, MD; Juan Alonso, MD; Carlos Egea Santaolalla, MD
Author and Funding Information

aOral and Maxillofacial Surgery Department and Sleep Disorders Department, Araba University Hospital, Vitoria-Gasteiz, Spain

bOral and Maxillofacial Surgery Department, Araba University Hospital, Vitoria-Gasteiz, Spain

cSleep Disorders Department, Araba University Hospital, Vitoria-Gasteiz, Spain

dOtorhinolaringology Department, Araba University Hospital, Vitoria-Gasteiz, Spain

CORRESPONDENCE TO: Marcos Fernández-Barriales, MD, Oral and Maxillofacial Surgery Department and Sleep Disorders Department, Araba University Hospital, C/Olaguíbel, 29 (01004 Vitoria-Gasteiz), Spain


Copyright 2016, American College of Chest Physicians. All Rights Reserved.


Chest. 2016;150(2):e59-e64. doi:10.1016/j.chest.2016.02.686
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Published online

A 51-year-old woman with a personal history of vitiligo, normal thyroid hormone studies, a simple hysterectomy for multiple uterine myomas at age 35 years, and childhood adenotonsillectomy was seen for progressive hearing loss. She reported mild asthenia, cold intolerance, mild dysphagia with frequent choking while eating and drinking, and a progressive increase in inspiratory effort, especially in the supine position. Her partner described a progressively worsening history of snoring and witnessed apneic episodes, mostly in the supine position. Mild to moderate daytime sleepiness was also present.

Figures in this Article

Intraoral examination revealed a Mallampatti score of IV without tonsil hypertrophy. There were no palpable neck masses, and thyroid palpation was unspecific. Her neck circumference was 38 cm. A slightly nasal voice was perceived. Fiber-optic laryngoscopy revealed a highly vascular symmetrical hypertrophy at the base of the tongue and a vallecular collapse of the epiglottis (Fig 1, Video 1). Her BMI was 26.89 kg/m2. The remainder of the physical examination was normal.

Figure 1
Figure Jump LinkFigure 1 Flexible fiber-optic laryngoscopy.Grahic Jump Location

Thyroid hormone test results showed a low level of thyroid stimulating hormone (TSH) (0.03 International Unit/mL) and high levels of free T3 (3.78 pg/mL). A sleep study with respiratory polygraphy (Embletta Gold) confirmed severe obstructive sleep apnea (OSA) with an apnea hypopnea index (AHI) of 45½ events/h, an AHI in the supine position of 89.7 events/h, and the percentage time during which arterial oxygen saturation was lower than 90% was 0.2 and the lowest oxyhemoglobin saturation during sleep was 87%. The CT and thyroid scintigraphy findings are shown in Figures 2 and 3.

Figure 2
Figure Jump LinkFigure 2 CT scan with endovenous contrast medium.Grahic Jump Location
Figure 3
Figure Jump LinkFigure 3 Thyroid scintigraphy with 99mtechnetium pertechnetate. There is an irregular increase in the uptake localized at the base of the tongue and an absence of orthotopic thyroid tissue.Grahic Jump Location

What is the diagnosis?

What is the most likely cause of her dyspnea in the supine position along with severe OSA?

Diagnosis: Hyperfunctioning multinodular goiter within a lingual thyroid.

A CT scan confirmed the existence of a heterogeneous solid mass at the base of the tongue with nodules and calcifications and increased uptake (Fig 2). Thyroid scintigraphy with 99mtechnetium pertechnetate showed an irregular increase in the uptake localized at the base of the tongue and an absence of orthotopic thyroid tissue (Fig 3). MRI confirmed the size and location of the mass (Fig 4).

Figure 4
Figure Jump LinkFigure 4 Sagittal magnetic resonance image (T2 sequence). Mass at base of tongue completely obliterates posterior airway space.Grahic Jump Location

Thyroid gland ectopia is an infrequent condition that results from an abnormal migration of the thyroid anlage from the floor of the primitive foregut to its final pretracheal position. Approximately 90% of ectopic thyroid tissue is found as lingual thyroid at the base of the tongue. Prevalence estimates range from 1/100,000 to 1/300,000, although some authors have found ectopic lingual tissue in up to 10% of necropsies in the general population. Hypothyroidism occurs in two-thirds of patients with thyroid ectopia, and subclinical hypothyroidism that becomes clinically manifest during periods of physiological stress will develop in a larger percentage of patients. Hyperthyroidism is less frequent.

High-resolution ultrasonographic imaging and 99mtechnetium pertechnetate scintigraphy are the most common diagnostic tools, whereas CT imaging and MRI are usually reserved for accurate location and preoperative planning. Although it is usually asymptomatic, lingual thyroid can lead to dysphagia, dysphonia with stomatolalia, bleeding, dyspnea, and as reported recently, OSA. The latter can be explained by a hypopharyngeal space-occupying lesion that compromises upper airway patency, especially in the supine position, because of its collapse toward the posterior pharyngeal wall. Other oropharyngeal and parapharyngeal masses reported to cause OSA by compressing the upper airway are lipoma, non-Hodgkin lymphoma, parotid tumor, plasmacytoma, paraganglioma, hemangioma, laryngeal cyst, lingual cyst, lingual tonsil hypertrophy, neurilemmoma, neurofibroma, and retention cysts. Indeed, any anatomic alteration or obstruction of the upper airway from the nasal ali to the trachea may cause OSA. Anatomic alterations of the pharynx that cause obstruction or malfunction of the eustachian tube's ventilation of the middle ear are known to cause recurrent middle ear infections and hypoacusia.

Lingual thyroid treatment depends on mass size and location, symptoms, surgical and anesthetic risk, and thyroid functional status. Current therapeutic alternatives can be seen in literature review reports in Table 1.

Table Graphic Jump Location
Table 1 Review of Reported Cases of Lingual Thyroid Causing Obstructive Sleep Apnea

AHI = apnea hypopnea index; ESS = Epworth Sleepiness Scale; RDI = respiratory disturbance index.

Observation and thyroid tissue preservation is an option for small lingual thyroid and asymptomatic patients. Close follow-up must be established to assess any increase in size or symptoms, and any suspected malignant transformation should be followed by prompt histopathologic examination of specimens.

Suppressive thyroid hormone therapy with levothyroxine can be tried in patients with mild or absent symptoms who present with an elevated TSH level. A slow reduction in the size of the lingual thyroid can be expected, but surgery might eventually be required to achieve resolution of OSA. Antithyroid and beta-blocker drugs are usually combined with radioiodine therapy or surgery to manage hyperfunctioning thyroid tissue; however, there are no previous reports of hyperfunctioning lingual thyroid causing OSA.

Surgical removal is the first-line therapy for bleeding, suspected malignancy, recurrent pathologic conditions, or upper airway obstruction. A transoral approach should be considered for small masses, when feasible, to avoid the risk of complications (ie, lingual nerve injury, fistula formation, deep cervical infection, and an unaesthetic cervical scar). Cervical approaches are better suited to control bleeding and upper airway patency in bulky masses. Combined transoral and transcervical approaches produce the best exposure and control of vascular and neural cervical structures in the bulkiest and most posterior-inferior masses.

Partial radiofrequency ablation has been proposed as an alternative to conventional surgical treatment, with claims that it minimizes the risks of vascular injury, hemorrhage, sequelae from the surgical approach (including temporary tracheostomy), and the need for thyroid hormone replacement therapy. A similar point of view is held by other authors defending carbon dioxide laser partial ablation. Brisk hemorrhage at the base of the tongue or suffocating edema can occur in less invasive techniques as well as in conventional surgery, thus suggesting the performance of a preventive tracheostomy to avoid potential respiratory distress and aspiration. Radiofrequency ablation provides no histologic sample to rule out malignancy, the frequency of carcinoma arising in an lingual thyroid being estimated at one in 100 cases. No long-term data on recurrence are available, and a partial surgical resection of either lingual or orthotopic thyroid has been known to relapse.

Radioiodine therapy is a second-line therapy usually reserved for older patients or those unfit for surgery. It is absolutely contraindicated in pregnant women and is not recommended in young patients or those with otherwise normally functioning thyroid tissue. However, its ability to reduce the size of the mass, and therefore manage OSA, is arguable, and high doses of radioiodine are usually required because hypothyroid glands are more common.

Standard OSA therapies such as CPAP or mandibular advancement devices should be considered according to current treatment guidelines, depending on the severity and patient-specific circumstances, whenever OSA is present (or persists after treatment). Nevertheless, if such therapies are delivered without surgical resection or medical assessment of the lingual thyroid, the possibility of growth, bleeding, and malignant transformation should always be considered; physically obstructed upper airways might also limit treatment response and adherence.

Clinical Course

Thiamazole antithyroid treatment normalized thyroid hormone test results (TSH, 1.17 International Units/mL; free T3, 2.39 pg/mL), but the obstructive symptoms remained, thus prompting surgical resection of the hypopharyngeal mass. The lingual thyroid was excised through a lingual pull-through technique and midline glossotomy, preserving the bilateral lingual arteries and nerves. The musculature of the floor of the mouth was detached from its insertion over the lingual surface of the mandible and subsequently resuspended with a Mitek hook (Mitek-DePuy Synthes/Johnson & Johnson) at the genial tubercles (Fig 5). The patient was discharged 2 weeks later after the tracheostomy was closed and the nasogastric feeding tube was removed.

Figure 5
Figure Jump LinkFigure 5 Surgical intervention. A, Submental cervicotomy; B, subplatysmal dissection—exposure of supra- and infrahyoid musculature; C, lingual pull-through technique with gingival papillae and detachment of geniohyoid/mylohyoid musculature; D, midline glossotomy until visualization of mass at base of tongue, preserving bilateral lingual arteries and nerves; E, musculature in floor of mouth resuspended to the genial tubercles by a Mitek hook.Grahic Jump Location

The obstructive symptoms improved significantly. A controlled sleep study with respiratory polygraphy was performed 1 year after surgery and demonstrated a reduction in AHI to 31.1 events/h, AHI in the supine position of 50.9 events/hour, AHI in a nonsupine position of 30.9 events/h. In addition, arterial oxygen saturation lower than 90% was 0.2, and the lowest oxyhemoglobin saturation during sleep was 88%. Therefore, and despite surgical resection of the mass as well as clinical improvement in daytime and nighttime symptoms, confirmed fiber-optic laryngoscopic improvement in upper airway patency (Video 2), and a BMI decrease to 24.02 kg/m2, severe OSA persisted. The patient is currently undergoing further evaluation for starting treatment with CPAP.

Because of the scarcity of reported cases of lingual thyroid with adequate pretreatment and posttreatment OSA assessment, recommendations must be taken with care.

  • 1.

    Unusual symptoms or clinical findings in a patient with OSA should alert the physician to begin a workup looking for a specific area of anatomic obstruction that may alter management. Imaging techniques and flexible fiber-optic nasofibroscopy can be used to evaluate upper airway pathologic features that might be misdiagnosed by simple direct intraoral or nasal exploration.

  • 2.

    Surgical treatment is preferred for bulkier masses or those that do not respond to or are not candidates for conservative thyroid hormone suppressive therapy.

  • 3.

    The benefits of partial ablation of lingual thyroid with respect to reduction in surgical sequelae should be appropriately weighed against incomplete oncological assessment and the possibility of relapse.

  • 4.

    Postoperative sleep studies should be performed systematically after surgical treatment to confirm OSA resolution, and appropriate additional treatment should be delivered if necessary.

Financial/nonfinancial disclosures: None declared.

Other contributions:CHEST worked with the authors to ensure that the Journal policies on patient consent to report information were met.

Additional information: The Videos can be found in the Multimedia section of the online article.


Figures

Figure Jump LinkFigure 1 Flexible fiber-optic laryngoscopy.Grahic Jump Location
Figure Jump LinkFigure 2 CT scan with endovenous contrast medium.Grahic Jump Location
Figure Jump LinkFigure 3 Thyroid scintigraphy with 99mtechnetium pertechnetate. There is an irregular increase in the uptake localized at the base of the tongue and an absence of orthotopic thyroid tissue.Grahic Jump Location
Figure Jump LinkFigure 4 Sagittal magnetic resonance image (T2 sequence). Mass at base of tongue completely obliterates posterior airway space.Grahic Jump Location
Figure Jump LinkFigure 5 Surgical intervention. A, Submental cervicotomy; B, subplatysmal dissection—exposure of supra- and infrahyoid musculature; C, lingual pull-through technique with gingival papillae and detachment of geniohyoid/mylohyoid musculature; D, midline glossotomy until visualization of mass at base of tongue, preserving bilateral lingual arteries and nerves; E, musculature in floor of mouth resuspended to the genial tubercles by a Mitek hook.Grahic Jump Location

Tables

Table Graphic Jump Location
Table 1 Review of Reported Cases of Lingual Thyroid Causing Obstructive Sleep Apnea

AHI = apnea hypopnea index; ESS = Epworth Sleepiness Scale; RDI = respiratory disturbance index.

References

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