Massive and Substernal Goiter


Prior to the 20th century, thyroid surgery was associated with a high operative mortality rate. The meticulous surgical technique advanced by Kocher in the late 1890s transformed thyroidectomy into a safe operative procedure associated with a 1% mortality rate. Although generally safe and effective, surgery for massive and substernal goiter remains subject to potentially significant surgical complications.

Approximately 5% of the population in the United States is estimated to have benign enlargement of nodules within the thyroid gland, known as a goiter. Iodine deficiency has been associated with the development of goiter, but this cause of goiter is now uncommon in the developed world. The vast majority of goiters are not associated with abnormal thyroid hormone function, and most are asymptomatic. However, massive enlargement can occur in which the gland extends around the pharynx or extends below the level of the thoracic inlet into the chest. In 1939 Crile described substernal goiters as those extending to the aortic arch. Recently In 2008, Huins et al. described, a three-tiered system derived from a large meta-analysis which classified substernal goiters in relation to the aortic arch, pericardium, and atrium.

Massive and substernal goiters are often technically demanding procedures, due to the gland’s vascularity and its proximity to the larynx, trachea, and the recurrent laryngeal nerves controlling vocal cord movement. In addition, the exposure needed to identify and preserve important structures is hampered when the goiter extends below the level of the thoracic inlet into the enclosed space of the chest. Nevertheless, even large goiters can usually be delivered out of the chest through an incision low in the neck using technical maneuvers. However, extension inferior to the aortic arch more often necessitates additional exposure.

Substernal goiters may come to clinical attention as a visible mass or as a result of goiter-related symptoms: a compressive pressure–type sensation, shortness of breath on lying down, and problems swallowing. Indeed, substernal goiters are more often symptomatic than massive goiters limited to the neck. Substernal goiters may not be visible on inspection because most of the gland is in the mediastinum ( Figs. 81.1 to 81.3 ). Patients develop symptoms due to cumulative growth within the confines of the thoracic inlet and chest cavity, compressing or displacing the trachea, esophagus, and mediastinal blood vessels. Enlargement is typically slow and progressive over many years. Sudden, rapid enlargement of a long-standing goiter suggests the development of cancer but could also be due to abrupt bleeding into the gland.

Fig. 81.1

A large substernal goiter is not necessarily associated with an obvious abnormality in the neck visible by inspection alone.

Fig. 81.2

The cervical incision must be large enough to accommodate delivery of the gland. A continuous laryngeal nerve monitor stimulates (yellow wire) the ipsilateral vagus nerve, monitoring the functional integrity of the recurrent laryngeal nerve during the procedure.

Fig. 81.3

The massive substernal component was delivered prior to definitive identification of the recurrent laryngeal nerve. In the absence of amplitude or latency changes, safe delivery without traction injury was accomplished.

Surgery for goiter is the definitive treatment for massive or substernal goiter but is associated with a number of potential complications. The surgery is highly effective in ameliorating goiter-related symptoms, but diligent surgical execution is needed to optimize favorable outcomes. Strategies to limit intraoperative bleeding, enhance exposure, and facilitate atraumatic surgical dissection will diminish intraoperative complications.

Key Operative Learning Points

  • 1.

    Assess the level of patient-reported symptoms, prior to surgery. Consider using a thyroid-specific tool, such as the short version of the thyroid-related patient-reported outcome (ThyPRO) tool prior to surgery; this tool may also be administered several months after surgery. Rapid enlargement associated with pain and dysphagia suggests the development of an aggressive malignancy.

  • 2.

    Preoperative evaluation should include serum thyroid-stimulating hormone (TSH) and imaging. Both an ultrasound and a computer tomography (CT) scan should be obtained. The CT scan with intravenous contrast will determine the substernal extent of the goiter and its relationship with large blood vessels and identify posterior mediastinal extension associated with displacement of the recurrent laryngeal nerve. The appearance may also suggest the presence of an infiltrative malignancy that could alter the treatment plan.

  • 3.

    Continuous laryngeal nerve monitoring that tests the functional integrity of the recurrent laryngeal nerve by means of continuous intraoperative vagal nerve stimulation is particularly useful in the management of large substernal goiters. During these cases the recurrent laryngeal nerve may not be readily identified because it is hidden behind a large obstructing goiter and subject to traction injury. Evidence for the use of continuous laryngeal nerve monitoring is mounting, but this technology does not yet represent standard of care.

  • 4.

    Instrumentation used for minimally invasive thyroid surgery is useful in substernal goiter surgery, which usually requires dissection in narrow spaces with limited exposure; having the proper instrumentation may obviate the need for manubrium or sternal osteotomies, which may be required in revision cases or those involving substernal thyroid cancer ( Fig. 81.4 ).

    Fig. 81.4

    Specialized equipment can be helpful. A custom, long McCabe is superior to the classic McCabe dissector, a Schnidt, or a right-angle clamp in reaching into deep narrow spaces. A suction dissector may be used like a Kittner sponge but is less abrasive.

Preoperative Period


  • 1.

    History of present illness

    • a.

      History of anterior neck mass

      • 1)

        Duration of mass, interval enlargement

    • b.

      Patient symptoms

      • 1)

        Direct compression of the trachea or esophagus by a substernal goiter can cause dysphagia to solids, positional dyspnea, and dysphonia. Positional dyspnea and orthopnea are frequently associated with substernal goiter with tracheal compression on cross-sectional imaging.

    • c.

      History of snoring, daytime somnolence, sleep apnea

    • d.

      Factors predisposing to thyroid cancer, such as history of neck radiation, or family history of thyroid cancer

  • 2.

    Past medical history

    • a.

      A complete past medical and surgical history must be obtained from the patient. Prior imaging and biopsies should be reviewed. A history of hypertension and prior neck surgery should be noted.

Physical Examination

  • 1.

    Respiratory distress? Stridor? Hoarseness?

  • 2.

    Venous congestion of neck/facial venous collaterals

  • 3.


    • a.

      Size and location of the goiter

      • 1)

        Consistency: Is the mass firm or hard?

      • 2)

        Is it tethered to the overlying skin; is the skin indurated?

      • 3)

        Does it extend to the clavicle?

      • 4)

        Does it move with swallowing? Is the inferior extent palpable with swallowing?

      • 5)

        Is the trachea palpable, and is it deviated?

      • 6)

        World health organization (WHO) classification ( Table 81.1 ): Patients with goiters visible on casual inspection may have few goiter-related symptoms. Substernal goiters associated with patient symptoms may not necessarily be appreciated on clinical examination alone (see Fig. 81.1 ).

        TABLE 81.1

        World Health Organization Classification of Goiter

        Grade 0 Impalpable/invisible
        Grade 1a Palpable but invisible even in full extension
        Grade 1b Palpable in neutral position/visible in extension
        Grade 2 Visible no palpation required to make diagnosis
        Grade 3 Visible at a distance

        WHO , World Health Organization.

    • b.

      Palpable cervical lymphadenopathy and neck levels involved

  • 4.

    Larynx: Examination of the larynx should also assess the pharynx for compression, as well as vocal cord mobility.

  • 5.

    Pemberton maneuver: The maneuver is performed by having the patient raise both arms against the face for several minutes to see if the patient develops reversible facial flushing or cyanosis, sometimes associated with shortness of breath or stridor. A positive Pemberton sign indicates superior vena cava syndrome causing diminished venous outflow from and venous congestion of the head and neck due to goiter-related compression of the vessels in the mediastinum. Patients may also have enlargement of venous collaterals in the chest and neck.


Apr 3, 2019 | Posted by in OTOLARYNGOLOGY | Comments Off on Massive and Substernal Goiter
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