- Research article
- Open Access
Emergency total thyroidectomy due to non traumatic disease. Experience of a surgical unit and literature review
© Testini et al; licensee BioMed Central Ltd. 2012
Received: 2 January 2012
Accepted: 11 April 2012
Published: 11 April 2012
Acute respiratory failure due to thyroid compression or invasion of the tracheal lumen is a surgical emergency requiring urgent management. The aim of this paper is to describe a series of six patients treated successfully in the emergency setting with total thyroidectomy due to ingravescent dyspnoea and asphyxia, as well as review related data reported in literature.
During 2005-2010, of 919 patients treated by total thyroidectomy at our Academic Hospital, 6 (0.7%; 4 females and 2 men, mean age: 68.7 years, range 42-81 years) were treated in emergency. All the emergency operations were performed for life-threatening respiratory distress. The clinical picture at admission, clinical features, type of surgery, outcomes and complications are described. Mean duration of surgery was 146 minutes (range: 53-260).
In 3/6 (50%) a manubriotomy was necessary due to the extension of the mass into the upper mediastinum. In all cases total thyroidectomy was performed. In one case (16.7%) a parathyroid gland transplantation and in another one (16.7%) a tracheotomy was necessary due to a condition of tracheomalacia. Mean post-operative hospital stay was 6.5 days (range: 2-10 days). Histology revealed malignancy in 4/6 cases (66.7%), showing 3 primitive, and 1 secondary tumors. Morbidity consisted of 1 transient recurrent laryngeal palsy, 3 transient postoperative hypoparathyroidism, and 4 pleural effusions, treated by medical therapy in 3 and by drains in one. There was no mortality.
On the basis of our experience and of literature review, we strongly advocate elective surgery for patients with thyroid disease at the first signs of tracheal compression. When an acute airway distress appears, an emergency life-threatening total thyroidectomy is recommended in a high-volume centre.
Acute respiratory failure due to thyroid compression or invasion of the tracheal lumen is a surgical emergency requiring urgent management.
Laryngo-tracheal compression may be caused by giant or cervico-mediastinal goiter, acute intra-thyroidal hemorrhage, anaplastic carcinoma, lymphoma, and metastases from breast, lung, gastro-enteric and renal cancer [6–12]. Bilateral recurrent laryngeal nerve infiltration by anaplastic cancer, lymphoma, metastasis can also result in vocal cord palsy with worsening dyspnoea .
Hemorrhage in cysts and adenoma of thyroid gland is a common asymptomatic event ;
On the contrary, massive hemorrhage, severe enough to result in acute airway distress is exceptional and more frequently secondary to neck trauma rather than a spontaneous complication of thyroid disease [14–16].
The aim of this paper is to describe a series of six patients treated successfully in the emergency setting with total thyroidectomy because of ingravescent dyspnoea and asphyxia, as well as review related data reported in literature.
During 2005-2010, of 919 patients treated by total thyroidectomy at our Academic Hospital, 6 (0.7%; 4 females and 2 men, mean age: 68.7 years, range 42-81 years) were treated in emergency. All the emergency operations were performed for life-threatening respiratory distress, and by the same surgeon (M.T.) with high level of thyroid surgical skill. The clinical picture at admission, clinical features, type of surgery, outcomes and complications are described below. Mean duration of surgery was 146 minutes (range: 53-260).
In 3/6 (50%) a manubriotomy was necessary due to the extension of the mass into the upper mediastinum. In all cases total thyroidectomy was performed by 3× loupe magnification  to aid dissection of parathyroid glands, and recurrent laryngeal nerves. In one case (16.7%) a parathyroid gland transplantation  and in another one (16.7%) a tracheotomy was necessary due to a condition of tracheomalacia. Mean post-operative hospital stay was 6.5 days (range: 2-10 days). Histology revealed malignancy in 4/6 cases (66.7%), showing 3 primitive, and 1 secondary tumors. Morbidity consisted of 1 transient recurrent laryngeal palsy, 3 transient postoperative hypoparathyroidism, and in 4 pleural effusions, treated by medical therapy in 3 cases and by drains in one. There was no mortality.
In spite of Hedenus reporting successful thyroidectomies in six patients for goiters, which he described as "suffocating"  in 1821, nowadays airway obstruction due to goiter is exceptionally reported in literature [2–5, 7, 9, 14] due to improved diagnostic methods and earlier treatment.
Although this dramatic occurrence seems to be more frequent in developing countries due to ignorance and lack of ready access to affordable medical services, in western countries the phenomenon of giant goiters is very uncommon though not completely absent [21, 22]. A truly severe life-treating airway obstruction is, therefore, currently an extremely rare event [2, 21, 23, 24], also because the tracheal lumen may be progressively compressed without causing symptoms up to 75% .
The causes of severe respiratory distress related to non traumatic thyroid disease show four different etiopathogeneses: rapidly progressive pressure on the tracheal lumen by spontaneous intrathyroideal hemorrhage, invasion of the tracheal lumen by primitive or secondary tumors, severe compression from benign or malignant masses and bilateral vocal cords palsy resulting from infiltration of recurrent nerves from thyroid malignancy.
Among the causes, spontaneous hemorrhage is often but not always  related to benign condition and is paradoxically the most insidious because it suddenly and unexpectedly appears in its full strength, sometimes in patients without previous history of thyroid disease; consequently diagnosis may be delayed. Indeed, literature [26–28] reports mortality related to this event of up to 27.8% . The most likely explanation for hemorrhage in goiters is thought to be venous bleeding . The adenomatous goiters are usually more fragile than normal thyroid because of the increased vascular flow and the lack of a true capsule; these aspects easily explain the great propensity for injury by blunt trauma , or iatrogenic bleeding resulting from fine-needle aspiration biopsy [30, 31].
In the spontaneous thyroid hemorrhage, however, the mechanism is unclear. Johnson  and Terry  proposed that the inciting event for the hemorrhage was increased venous pressure resulting from the Valsalva maneuver. Therefore, most spontaneous cases are found to have an associated external event, such as various forms of light housework, coughing, straining at defecation, crying, which are, however, seemingly insignificant . However, on appearance of a stable spontaneous hematoma of the thyroid gland without airway compromise, we believe that a conservative treatment could be safely performed; however, the increasing size of neck swelling or acute worsening of respiratory distress must be absolute, although rare indications point to early intubation and emergency surgery , as in the case reported in this series.
Anaplastic thyroid cancer is a rare tumor, ranging from 1-3% of all thyroid neoplasms, but is characterized by a very aggressive loco-regional disease, with mortality often related to respiratory failure from infiltration of the tracheal lumen . Indeed, the main indication for surgery is just palliative decompression and debulking to prevent invasion of larynx, trachea, nerves and vessels of the neck, in the presence of a median survival of 4-5 months from the time of diagnosis .
On the other hand, well-differentiated thyroid carcinoma may, on occasion, cause airway obstruction . The usual treatment of carcinoma invading the trachea is by "shaving" the tumor off the trachea, expecting to control residual neoplasm by postoperative radioactive iodine or external irradiations therapies [37, 38]. However, the prognosis for well-differentiated carcinomas worsens when the neoplasm invades the trachea; indeed, the cause of death in nearly half of the fatal cases of papillary carcinomas is caused by obstruction of the trachea [37, 39]. Moreover, the survival rate of patients treated by incomplete resection of the affected trachea is much worse than patients treated by complete resection [40, 41]. For these reasons, with progress in tracheal surgical techniques, resection of portions of the trachea with primary anastomosis en bloc with thyroid is nowadays the treatment of choice [40–43]. Four cases (66.7%) in this reported series were well-differentiated carcinoma. In case 1, 2, and 6 (Hürthle cell, follicular, and medullary carcinomas, respectively), the airway obstruction was determined by the compression but not by the infiltration of trachea from the thyroid mass, and a comfortable cleavage plain between trachea and thyroid was evident at operation during dissection. For this reason a trachea resection was deemed unnecessary and the long-term disease-free follow up provides proof of the correctness of the surgical decision. In case 4 (thyroid metastasis from renal cancer), however, despite the invasion of the trachea, the staging of a metastatic disease contraindicated resection. Indeed, the patient died 7 months after the operation, due to the disease progress, but without local recurrence.
When the respiratory distress is caused by benign thyroid disease, usually the compression ab estrinseco of the trachea is determined by a giant cervical or cervicomediastinal goiters. However, there being enough room to accommodate the gland, acute respiratory failure secondary to tracheal compression by goiter is extremely rare , affecting 0.6% of reported cases . However, when the extension of the goiter is retroclavicular, it can cause airway obstruction that may progress to arrest respiration [2, 45, 46]. Nevertheless, in the presence of benign thyroid disease, chronic obstructive airways disease, substernal extension, and long-standing goiter are considered as risk factors for developing acute, life-threatening airway compromission .
It is clear that the appearance of an acute airway obstruction requires urgent management to ensure an adequate ventilation and oxygenation.
The first step in the management of this emergency is represented by the anesthesia. An awake fiberoptic intubation using a small endotracheal tube followed by induction of general anesthesia, as always performed in this reported series, seem to be the gold standard in the approach to this emergency. Indeed, a standard sequence of induction and intubation could be considered at risk of aspiration in an unfasted patient, and besides this, the possibility of unsuccessful intubation due to the compression by the goiter is very high. On the other hand, an inhalation induction followed by laringoscopy and orotracheal or blind nasal intubations, may be considered dangerous because of complete airway obstruction following loss of consciousness [47, 48]. When assisted intubation cannot be achieved, local or regional anesthesia are described too .
The second step is the choice of surgical treatment to be performed. Indeed, surgery - emergency or early - is always indicated for severe airway obstruction caused by thyroid mass . An emergency tracheostomy is hindered by the presence of the thyroid mass which prevents access to the trachea, obliterating all landmarks . An isthmectomy to allow a tracheostomy, appears to be an incomplete treatment, referring to a second surgical procedure for removing the entire thyroid. Moreover, in the presence of diagnosis of proven or suspected malignancy, it would cause a further delay in cancer treatment and exposes the patient to the risk of tumor dissemination. However, even in the presence of a benign goiter, re-surgery would mean higher morbidity [49, 50]. Finally, once an endotracheal intubation has been performed, tracheotomy is questionable. Since a total thyroidectomy is capable of resolving airway obstruction, tracheostomy would result in unnecessary discomfort for the patient, furthermore exposing then to the need of a second operation to close the stomy. In our experience tracheostomy was necessary in only one case (16.7%) due to the evidence of a marked tracheomalacia.
Then, total, near-total or sub-total thyroidectomy represents the treatment of choice of acute airway obstruction resulting from compression of thyroid mass.
On the basis of our experience and that in literature, in the presence of warning signs such as a developing mass in patients with history of thyroid disease, increasing and/or intermittent dyspnoea, stridor, sudden onset of neck swelling, we stress the importance of immediate hospitalization to perform a controlled approach to the progressively acute disease, avoiding treatment in emergency. But, when this event occurs, like in our reported series, the approach to this emergency operation should be performed in highly specialized high-volume centers combining multidisciplinary anesthesiological and surgical strategies. Indeed, when total thyroidectomy is performed for cervicomediastinal goiters, there is a higher risk of postoperative hypoparathyroidism, recurrent laryngeal nerve palsy and hemorrhage, as reported in literature [8, 51–57] and in our experience too,  which sometimes requires sternal split, as in 50% of this series. However, in our experience, the use of loupe magnification and parathyroid autotransplantation during thyroid surgery showed a significant improvement of results, with faster and safer identification of the nerve, and decreasing permanent and transient hypoparathyroidism [17, 18]. Some authors suggest the use of the recurrent nerve monitor, especially in the presence of a large retrosternal goiter [59, 60].
Moreover, when the upper mediastinum is occupied by a goiter, the endocrine surgeon is not usually familiar with the course of the RNLs and their anatomical variability in this district, and the cardiothoracic surgeon is not familiar with endocrinosurgical challenges. Therefore, the emergency extracervical approach could require multidisciplinary collaboration .
In conclusion, on the basis of our experience and of the literature review, we strongly advocate elective surgery for patients with thyroid disease at the first signs of tracheal compression. When an acute airway distress appears, an emergency life-threatening total thyroidectomy is recommended in a high-volume centre.
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