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Superior vena caval syndrome is usually due to malignant disease, however, benign cases do occur. Two patients with superior vena caval syndrome due to substernal goitre are reported, and the use of phleboscintigraphy and/or vena caval phlebography are advocated. A review of potentially helpful diagnostic procedures is given.
OBJECTIVE: The aim of this study was to compare the subjective tracheoesophageal pressure symptoms associated with substernal goitres with objective cross-sectional radiographic measurements. DESIGN: Patients with substernal goitre were questioned regarding tracheoesophageal compression symptoms and these data were analysed for correlation with radiographic and intra-operative findings. RESULTS: Twenty-six patients were included. Patients with airway symptoms including dyspnoea, stridor and coughing were more likely to have significant tracheal narrowing on pre-operative computed tomography (CT) imaging. There was also a significant correlation between the perceived severity of lump sensation in the throat and the presence of tracheal and oesophageal displacement and retrotracheal extension of the thyroid mass. CONCLUSION: Pre-operative CT measurements of tracheal and oesophageal displacement, retrotracheal extension and the degree of tracheal compression correlate well with the presence and severity of tracheoesophageal pressure symptoms. None of the radiographic findings that are exclusive to substernal goitre (percentage of substernal mass, goitre size at the level of the thoracic inlet, ratio of goitre size to the vertebral body at the thoracic inlet and retrosternal isthmus size) had any correlation with clinical symptoms in this study.
A patient with a superior mediastinal mass on an admission chest radiograph was initially evaluated by an 131I thyroid scan which failed to demonstrate a substernal thyroid. However, the tomographic 67Ga scan clearly showed an abnormal uptake in the area corresponding to the mass lesion on radiographic examination. Subsequent resection and biopsy of the substernal mass revealed a poorly differentiated follicular carcinoma with foci of anaplastic carcinoma. The differential diagnosis of the anterior mediastinal mass and the usefullness of the tomographic gallium scan are briefly discussed.
OBJECTIVES: To examine the presentation, workup, and surgical complications of substernal goitre. DESIGN: Retrospective study. SETTING: Regional hospital, Hong Kong. PATIENTS: Twenty-four mostly elderly patients (mean age, 60.1 years) who underwent thyroidectomy for substernal goitres between 2000 and 2003 (substernal goitres were defined as those having either a caudal mass transgressing the fourth thoracic vertebra or having more than 50% of their overall mass residing within the thorax). MAIN OUTCOME MEASURES: Symptoms, histopathological diagnoses, morbidities, and complications. RESULTS: Dyspnoea was the most common symptom (n=8, 33%). Three (12.5%) patients presented with acute airway obstruction; however, 13 (54.2%) were asymptomatic apart from the presence of cervical masses. Computed tomographic scans were performed on all but two patients. Malignancy was present in 12.6% of patients, or 16.8% if occult papillary carcinoma is included. Partial or full sternotomies were performed in two (8.3%) patients. Complications included recurrent laryngeal nerve injury (n=1, 2.7% of nerves at risk), transient hypoparathyroidism (n=2, 13.3% of patients at risk), haematoma (n=1, 2.7%), pneumonia (n=1, 2.7%), and wound infection (n=1, 2.7%). There was no operative mortality or permanent hypoparathyroidism. The complication rate was significantly lower in the asymptomatic patients (P=0.033 by Fisher's exact test); clinicopathological parameters were otherwise statistically comparable between the two groups. CONCLUSIONS: There is rarely any mortality in thyroidectomy for substernal goitre, and the morbidity is also very low, especially in asymptomatic patients. In the absence of contra-indications, substernal goitre should be treated with early surgery rather than having it run the risk of acute airway distress or cancer.
Substernal goitre is a clinical condition in which the masin bulk of the enlarged gland is firmly located in the chest. The incidence of this pathology ranges in literature from 1.7% and 30%. This study examines 230 cases of substernal goitre out of a total 5.362 operations performed from 1965 to 2000, for thyroid gland pathologies (4.36%). According to their experience the Authors propose a classification based on the anatomical location of the goitre: right, left, anterior and posterior goitre are therefore identified. The surgical procedures performed include 136 subtotal thyroidectomies (59.1%), 59 emithyroidectomies (25.7%) and 23 total thyroidectomies (10%). In 12 cases the operation was confined to removal of the mediastinal mass (5.2%). The cervical approach was the only surgical access route used in all the patients, regardless of the different anatomical variants. Appreciable venous stasis, due to the mediastinal mechanical obstruction exerted by the goitre, was always evident at the operation. In order to limit the risk of bleeding during operation, careful hemostasis of the major vascular pedicles must be performed. Any attempt to legate the smallest vessel, should be avoided since it is a difficult, useless and time-consuming procedure. Minor bleeds promptly stop as soon as the pathological mass is removed. Ligation of the vascular pedicles can be easily achieved; in this way, the goitre is freed from its anatomical connections and the surgeon can safely manage the substernal portion of the mass. The mortality reported in this study was 0.43% (one patient died 30 days postoperatively due to respiratory complications), whereas the morbidity rate was 2.6%.
All MR findings in the study of mediastinal masses are discussed in this report, with the aim of evaluating the reliability of MR imaging in this pathology. The results obtained in the assessment of the extension of these masses are very useful and MR imaging appears to be the most informative of all techniques. Masses originating from the thymus, cystic lesions, substernal goitre, and esophageal tumors were considered. MRI results were compared to those obtained by CT scan which is considered of proven reliability for the evaluation of mediastinal masses.
Surgical treatment was applied to 18 patients for intrathoracic struma. Dyspnoea, dysphagia, recurrent palsy, and dilatated cervical veins with facial flushing were indications for surgery. Surgical access routes depended on localisation of the intrathoracic struma and its connection to the thyroid gland. Goitre located in the anterior mediastinum (substernal) can be extirpated, using the cervical approach (Kocher). Sternotomy was found to be necessary only in cases with tracheal resection. Goitres located in the posterior mediastinum were removed by means of right or left thoracotomy. Postoperative "collapse" of the posterior tracheal wall in patients with extreme dislocation of the trachea was successfully avoided by means of intratracheal intubation for 24 hours.
Four men with clinical features of superior vena caval syndrome due to large substernal non-toxic multinodular goitre have undergone radionuclide superior vena caval studies. In all cases the dynamic scintigraphs showed a characteristic thyroidal configuration attributed to the definition of stretched thyroidal veins and their tributaries. Radiocontrast studies of two patients demonstrated the characteristic thyroid configuration. A radionuclide superior venacavogram taken post-thyroid lobectomy in one case showed the features had reverted to normal.
A substernal goitre is of clinical significance because its growth between the sternum anteriorly and vertebral bodies posteriorly leads to impingement on the surrounding structures and compressive symptoms. The incidence of substernal goitre is documented to vary between .02 and .5%. It accounts for 3-12% of mediastinal masses and is the most common superior mediastinal mass. This condition is important because it presents a diagnostic dilemma as its size and compressive symptoms mimic malignant disease, and an operative dilemma for the approach to its management. We present one of the largest reported retrosternal goitre cases in the literature.
This report describes the surgical experience gained from 32 patients with substernal goitre, operated on during January 1995 to December 1997. The material corresponds to 15.7% of the total thyroidectomies performed. The diagnosis was clinical. In spite of 65.6% of the patients being asymptomatic, breathing problems, dysphagia and hyperthyroidism were observed. The patients underwent tests of thyroid function, simple chest radiograph and computerized tomography of mediastinum. All patients underwent surgical treatment through a transverse cervical incision. Two patients (6.25%) needed median sternotomy. Vocal cord palsy (3.12%), transient hypocalcemia (6.25%) and one death due to cardiac causes (3.12%) were the complications that occurred.
A case of varices of the entire esophagus in a patient who had undergone resection of a substernal thyroid gland 29 years previously is reported. Dilated esophageal veins, varices, may serve as collateral channels between the portal and systemic systems. When the superior vena cava is obstructed, blood from the upper extremities and head is returned to the heart via downhill varices. Obstruction of the superior vena cava proximal to the azygos vein results in varices of the upper esophagus. Obstruction distal to the asygos vein, as in this patient, results in varices of the entire esophagus. Portacaval shunt has no place in the management of these patients. Downhill varices should be considered in any patient with superior vena caval obstruction from any cause.
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A 28-year-old trumpet player underwent multiple treatments with radioactive iodine for Graves disease associated with an unusually large goiter. Following his second treatment, the patient developed acute neck pain and swelling. Radiographic studies and a laryngoscopy demonstrated bilateral symptomatic external laryngoceles, a very rare entity, not previously known to be associated with radioiodine treatment or Graves disease. The patient's profession placed him at risk for the development of a laryngocele, but the temporal relationship to goiter regression following radioiodine therapy suggests that this occurred as a result of this treatment. The patient's disease was managed nonsurgically, and he has subsequently done well. This represents the first known association of symptomatic laryngocele with radioiodine treatment for Graves disease.
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HYPOTHESIS: Complications associated with thyroidectomy for intrathoracic goiters have been underestimated because of the lack of a precise definition of high-risk patients. DESIGN: Retrospective multicenter multinational review of medical records and radiographic images of patients who underwent thyroidectomy for intrathoracic goiters reaching the carina tracheae. Demographic, clinical, operative, anatomical, and pathological data were recorded. RESULTS: There were 35 patients (mean +/- SE age, 63 +/- 11 years) included in the study. In 4 patients, the goiter was asymptomatic; 10 patients had dysphagia, 24 patients had dyspnea, and 3 patients had superior vena cava syndrome. A median sternotomy was required in 12 patients and a right-sided thoracotomy in 1 patient. The mean +/- SE operative time was 145 +/- 72 minutes (range, 50-360 minutes). Transient hypoparathyroidism developed in 13 patients. Four patients experienced transient hoarseness, and 1 patient had permanent vocal cord paralysis. There were no significant differences between the proportion of patients who underwent or did not undergo sternotomy or thoracotomy regarding vocal cord dysfunction (2 [15%] of 13 patients vs 3 [13%] of 22 patients) or hypoparathyroidism (5 [38%] of 13 vs 6 [28%] of 22 patients). The mean postoperative hospital stay was 10 days (range, 2-84 days). Four patients required reoperation. Two patients died. Nine of 14 patients with thyroid glands weighing at least 260 g required sternotomy vs 3 of 14 patients with thyroid glands weighing less than 260 g (P = .02). Overall, 18 [52%] of 35 patients were discharged without any complication. CONCLUSION: Intrathoracic goiters reaching the carina tracheae carry a high unreported risk of sternotomy, postoperative complications, reoperation, and death.
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