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Asian Cardiovasc Thorac Ann 2003;11:165-166
© 2003 Asia Publishing EXchange Ltd


CASE STUDY

Giant Substernal Goiter with Chylothorax

Bassam K Darwish, MD, Sami S Kabbani, MD

Damascus University Medical School, Damascus, Syria

For reprint information contact: Sami Kabbani, MD Tel: 963 11 2129437 Fax: 963 11 2129437 email: dam-uncv{at}net.sy PO Box 2837, Damascus, Syria.


    ABSTRACT
 TOP
 ABSTRACT
 INTRODUCTION
 CASE REPORT
 DISCUSSION
 REFERENCES
 
A 60-year-old woman presented with a huge goiter extending from the lower jaw to the diaphragm. Right pleurocentesis produced chylous fluid. A cervicothoracic incision was used to totally excise the substernal goiter, with near-total excision of the cervical goiter, and repair of the lymphatic leakage.


    INTRODUCTION
 TOP
 ABSTRACT
 INTRODUCTION
 CASE REPORT
 DISCUSSION
 REFERENCES
 
Substernal or intrathoracic goiter accounts for 7% of mediastinal masses, and represents 2%–15% of all goiters designated for surgery.1–4 The most important clinical manifestations result from mediastinal compression.


    CASE REPORT
 TOP
 ABSTRACT
 INTRODUCTION
 CASE REPORT
 DISCUSSION
 REFERENCES
 
A 60-year-old diabetic female presented with dyspnea, a choking sensation, and progressive right chest pain of 4-months’ duration. She had undergone subtotal thyroidectomy 20 years earlier; the pathology report was unavailable. She was on antidiabetic and antihypertensive medication. Physical examination revealed a large nodular goiter in the neck extending to the thoracic inlet, dilated cervical veins with prominent collateral circulation on the chest wall, and dullness in the base of the right hemithorax. Laboratory findings showed mild hypochromic anemia (hemoglobin 9.2 g•dL-1), an elevated erythrocyte sedimentation rate (60 mL in 1st hour), and mildly elevated blood glucose (137 mg•dL-1). Pulmonary function tests indicated moderate pulmonary restrictive disease with acceptable blood gases. Chest radiography demonstrated widening of the mediastinum, especially on the right side, with right pleural effusion. Fine-needle aspiration of the cervical goiter yielded epithelial conjunctive tissue containing follicular cells. Right-sided pleurocentesis revealed chylothorax (lymphocytes forming 93% of cells, triglycerides 336 mg•dL-1). Pleural biopsy disclosed chronic nonspecific inflammation with no granulomatous or atypical cells. Computed tomography and magnetic resonance imaging confirmed a huge mass extending from the lower jaw to the diaphragm on the right side of the heart (Figures 1AGo and 1BGo). The mass filled the anterior mediastinum, pushing the great vessels to the left and narrowing the trachea. There was suspicion of invasion of the superior vena cava (SVC), trachea, and ascending aorta, with diffusely calcified areas, and some right pleural effusion. Vena caval angiography revealed a blockage in the axial vein at the thoracic inlet, collateral veins in the chest wall, and no SVC shadow. An iodine nucleotide scan showed no uptake inside the chest. Through a T-incision (cervicothoracic approach) and complete median sternotomy, a huge goiter was seen to extend from the lower jaw to the diaphragm on the right side of the heart, with SVC compression, complete obstruction of the innominate vein, compression of the trachea, and no evidence of tissue invasion. Total resection of the chest goiter and near-total resection of the cervical goiter were performed. During dissection, the SVC was injured at its junction with the innominate vein; it was successfully repaired with 4/0 polypropylene suture. On opening the right pleural space, chylothorax was encountered, with a very thick pleura and "trapped lung". Aspiration, pleural decortication, and repair of 2 sites of lymphatic leakage in the mediastinum were undertaken. Pathology indicated a benign multinodular goiter. Postoperatively, there was transient hoarseness lasting 3 weeks and hypoparathyroidism for 2 weeks, which improved with calcium treatment given intravenously for 5 days and then orally for 10 days.




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Figure 1. Magnetic resonance imaging in (A) axial and (B) sagittal views, confirming a huge mass extending from the lower jaw to the diaphragm on the right side of the heart, pushing the mediastinum to the left, and narrowing the trachea.

 

    DISCUSSION
 TOP
 ABSTRACT
 INTRODUCTION
 CASE REPORT
 DISCUSSION
 REFERENCES
 
This substernal goiter was of exceptional size. No previous reports of such a large goiter or of chylous effusion associated with retrosternal goiter could be found. Pleural effusion is unusual in substernal goiter.5 Chronic compression of the SVC and right chest lymphatics was possibly responsible for the pleural chylous effusion and thickening. Contrary to other reports, hoarseness and dysphagia were not features of the clinical presentation in this case.4 Tracheal deviation is usually found.2,6 Computed tomography and magnetic resonance imaging confirmed substernal goiter, but the suspicion of invasion was incorrect. Angiography could not determine whether the SVC obstruction was due to compression or invasion. At surgery, there was a good dissection plane with no invasion of the mediastinal structures. Surgery was indicated in this patient despite age, diabetes, hypertension, and restricted lung function, in order to alleviate intrathoracic compression and avoid potential intrathyroid hemorrhage or a missed malignancy which occurs in 4%–17% of cases.2–4,6 While the cervical approach can be used to excise most substernal goiters, it was obviously not adequate in this case.1–8 Operative complications were few and transient: the SVC injury was immediately repaired; recurrent laryngeal nerve paralysis, reported in substantial numbers of operations for substernal goiter, lasted only 3 weeks; and hypoparathyroidism, common with extensive resections, resolved after 2 weeks.3,7,8 Tracheomalacia, frequent in substernal goiter excisions, was not observed. The pathological finding of benign multinodular goiter concurs with that of most substernal goiters. The fact that malignancy was found in 17% of excised intrathoracic goiters highlights the need to operate on such patients.2


    REFERENCES
 TOP
 ABSTRACT
 INTRODUCTION
 CASE REPORT
 DISCUSSION
 REFERENCES
 

  1. Newman E, Shaha AR. Substernal goiter. J Surg Oncol 1995;60:207–12.[Medline]

  2. Netterville JL, Coleman SC, Smith JC, Swith MM, Day TA, Burkey BB. Management of substernal goiter. Laryngoscope 1998;108:1611–7.[Medline]

  3. Rodriguez JM, Hernandez Q, Pinero A, Ortiz S, Soria T, Ramirez P, et al. Substernal goiter: clinical experience of 72 cases. Ann Otol Rhinol Laryngol 1999;108:501–4.[Medline]

  4. Ozdemir A, Hasbahceci M, Hamaloglu E, Ozenc A. Surgical treatment of substernal goiter. Int Surg 2000;85:194–7.[Medline]

  5. Gallelli A, Pelaia G, Calderazzo M, Roccia F, Vero G, Gallelli G, et al. Transudative right pleural effusion due to compression of the brachiocephalic vein caused by an intrathoracic goiter. Monaldi Arch Chest Dis 2000;55:210–1.[Medline]

  6. Shai SF, Chen CY, Hsu CP, Hsia JY, Yang SS, Chuang CY, et al. Surgical management of substernal goiter. J Formosa Med Assoc 2000;99:827–32.[Medline]

  7. Torre G, Borgonovo G, Amato A, Arezzo A, Ansaldo G, De Negri A, et al. Surgical management of substernal goiter: analysis of 237 patients. Am Surg 1995;61:826–31.[Medline]

  8. Makeieff M, Marlier F, Khudjadze M, Garrel R, Crampette L, Guerrier B. Substernal goiter. Report of 212 cases [French]. Ann Chir 2000;125:18–25.[Medline]





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