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Mediastinum is the central portion of the chest and extends from the thoracic inlet above - the diaphragm below. To assist in the localization of mediastinal abnormalities, the mediastinum traditionally divided into : - superior, - anterior, - middle, and - posterior compartments.
Mediastinum is the central portion of the chest and extends from the thoracic inlet above - the diaphragm below. To assist in the localization of mediastinal abnormalities, the mediastinum traditionally divided into : - superior, - anterior, - middle, and - posterior compartments.
Mediastinum is the central portion of the chest and extends from the thoracic inlet above - the diaphragm below. To assist in the localization of mediastinal abnormalities, the mediastinum traditionally divided into : - superior, - anterior, - middle, and - posterior compartments.
2 hours SURGICAL DISEASES OF MEDIASTINUM 2 hours 2 C 3 - Surgical Diseases Of Mediastinum The mediastinum is the central portion of the chest and extends from: the thoracic inlet above the diaphragm below. It is bounded anteriorly by the sternum, laterally by the mediastinal pleura, posteriorly by the vertebral bodies. To assist in the localization of mediastinal abnormalities, the mediastinum is traditionally divided into : superior, anterior, middle, and posterior compartments. SURGICAL DISEASES OF MEDIASTINUM 3 If on a lateral chest radiograph, a line is drawn from the lower end of the manubrium to the lower edge of the body of the fourth thoracic vertebral body, the superior mediastinum is the area above that line. The pericardial sac and its contents divide the inferior mediastinum into its anterior, middle, and posterior compartments.
4 T4 The contents of the mediastinum and its compartments are :
Superior Mediastinum
Thymus gland Aortic arch and great vessels Upper trachea Upper esophagus
Esophagus Descending aorta Nerves (sympathetic, parasympathetic, and intercostal) SURGICAL DISEASES OF MEDIASTINUM 5 CT has permitted visualization of mediastinal structures that in the past were obscured on the standard posteroanterior chest radiograph by the sternum, spine, and cardiac silhouette
SURGICAL DISEASES OF MEDIASTINUM 6 (A) Scan at level of the aortic arch and mid trachea. (B) Scan at level of carina. (C) Scan at the level of the left atrium. ACUTE MEDIASTINITIS Acute infection of the mediastinum, regardless of the cause, is associated with great morbidity and mortality. Although acute mediastinitis can follow: penetrating wounds of the chest complicated with esophageal perforation - the most common cause of acute suppurative mediastinitis. tracheobronchial tree,wounds cardiac operations in which the mediastinum has been opened
NONINSTRUMENTAL Barogenic trauma Postemetic (Boerhaave syndrome) Blunt chest or abdominal trauma Other (eg, labor, convulsions, defecation) Penetrating neck, chest, or abdominal trauma Operative trauma Esophageal reconstruction (anastomotic disruption) Vagotomy, pulmonary resection, hiatal hernia repair, esophagomyotomy Corrosive injuries (acid or alkali ingestion) Erosion by adjacent infection Swallowed foreign body ESOPHAGEAL PERFORATION Pathophysiology Regardless of the specific cause, the resulting mediastinitis and his severe consequences demand prompt recognition and treatment of the esophageal disruption. Esophageal and gastric contents are sucked into the mediastinum by respiratory movements and negative intrathoracic pressure. Salivary enzymes, gastric acid, bile, and food enter the mediastinum, the presence of oral bacteria in these fluids initiates a fulminant infection and an inflammatory response progresses. This mediastinal burn produces massive fluid accumulation, which can displace the trachea, heart, or lungs The entire process is aggravated if there is preexisting esophageal disease Clinical Features Patients with esophageal perforation characteristically present with: cervical or thoracic pain, difficulty swallowing, respiratory distress, fever. Pain features depends with esophageal perforation location Cervical or upper thoracic esophagus generally cause cervical or high retrosternal pain Middle or distal esophagus produce anterior thoracic, posterior thoracic, interscapular, or epigastric pain. Upper thoracic esophageal perforations may produce signs of right pleural effusion Distal esophageal perforation is associated with left pleural effusion. SURGICAL DISEASES OF MEDIASTINUM
8 ESOPHAGEAL PERFORATION ACUTE MEDIASTINITIS Diagnosis Pain or fever after esophageal instrumentation or operation is indicative of an esophageal perforation and is an indication for an immediate contrast esophagogram with hidro soluble contrast substance.
A chest roentgenogram may help to confirm the diagnosis by demonstrating air in the soft tissues of the neck or mediastinum(pneumomediastinum) or a hydrothorax or pneumothorax. A contrast-enhanced CT scan may lead to the diagnosis The morbidity and mortality rates associated with esophageal perforation are directly related to the time interval between diagnosis of the injury and its repair or drainage SURGICAL DISEASES OF MEDIASTINUM
9 Barium esophagogram demonstrates a perforation (arrow) in the middle third of the thoracic esophagus. ESOPHAGEAL PERFORATION ACUTE MEDIASTINITIS Management(Principles of Surgical Treatment) The initial treatment of an acute esophageal perforation focuses on: decreasing bacterial and chemical contamination of the mediastinum restoring intravascular volume losses.
Oral intake is withheld, the patient is instructed not to swallow saliva. A disposable oral dental suction is often helpful for evacuating oral secretions. Broad-spectrum intravenous antibiotics with activity against oral flora are administered using a combination of a cephalosporin (cefazolin or cefamandole), 1 g/4 h, and an aminoglycoside (gentamicin or tobramycin), 1 to 1.5 mg/kg/8 h. Nasogastric tube decompression of the stomach is instituted to minimize possible gastroesophageal reflux and further soiling of the mediastinum. Therapy of esophageal perforation is influenced by: The location of the tear The size of the tear The cause of the tear, The length of delay in diagnosis, The extent of mediastinal and pleural contamination The presence of intrinsic esophageal disease. The treatment of an acute esophageal perforation must be individualized. SURGICAL DISEASES OF MEDIASTINUM
10 ESOPHAGEAL PERFORATION ACUTE MEDIASTINITIS Nonoperative Therapy Although most esophageal perforations require operative intervention, BUT selected patients may be managed nonoperatively with: Cessation of oral intake, Administration of antibiotics, Intravenous hydration until the disruption heals or the small contained cavity begins to decrease in size. Criteria for nonoperative therapy of an esophageal perforation include the following: A local, contained disruption without evidence of pleural contamination (hydrothorax or pneumothorax), A walled-off extravasation in which contrast material drains back into the esophagus, Minimal or no symptoms, Minimal or no evidence of systemic infection (fever or leukocytosis). The usual clinical settings in which such perforations are encountered are: cervical esophageal tears caused by esophagoscopy; intramural dissections that have occurred during dilation of a stricture or pneumatic dilation for achalasia; asymptomatic esophageal anastomotic disruption discovered on a routine postoperative contrast study. SURGICAL DISEASES OF MEDIASTINUM
11 ESOPHAGEAL PERFORATION ACUTE MEDIASTINITIS When treating such perforations conservatively, oral hygiene should be optimized to minimize further contamination by oral bacteria A nasogastric tube is seldom helpful. Nutrition may be maintained by a nasogastric feeding tube, gastrostomy, or jejunostomy or by intravenous hyperalimentation until oral intake can be resumed, usually 1 to 3 weeks after the injury. Nonoperative therapy is best suited for patients presenting more than 24 hours after the injury with no systemic evidence of sepsis and clearly demonstrable, contained, internally drained leaks on barium esophagogram. Infants with iatrogenic perforation can often be successfully managed without operation. Perforations complicating pneumatic dilation for achalasia occur in 4% to 6% of patients, and most are small and well-managed medically with antibiotics and intravenous hyperalimentation. For the remainder of patients with perforations, operative therapy is generally indicated. Operative Therapy Of Esophageal Perforations Cervical and Upper Thoracic Esophageal Perforations lead to: Progressive contamination of the mediastinum as infection descends dependently along the fascial planes from the neck. Unless adequate drainage is accomplished, death from mediastinitis follows. Most cervical and upper thoracic perforations may be adequately drained through a cervical approach, placing drains in the retroesophageal space. An incision is made parallel to the anterior border of the sternocleidomastoid muscle, which is retracted laterally along with the carotid sheath and its contents. The trachea, thyroid gland, and strap muscles are retracted medially. It may be necessary to divide the omohyoid muscle, middle thyroid vein, and occasionally the inferior thyroid artery to reach the prevertebral fascia. Once this is identified, blunt finger dissection into the prevertebral space gives access to the abscess cavity, and appropriate drains are placed and brought out through the skin incision. When a cervical esophageal perforation extends into either pleural cavity or the lower mediastinum, the cervical approach is inadequate, and transthoracic drainage is required. SURGICAL DISEASES OF MEDIASTINUM
12 ESOPHAGEAL PERFORATION ACUTE MEDIASTINITIS Operative Therapy Of Esophageal Perforations(suite)
SURGICAL DISEASES OF MEDIASTINUM
13 Approach for drainage of a cervical esophageal perforation. ESOPHAGEAL PERFORATION ACUTE MEDIASTINITIS Operative Therapy Of Esophageal Perforations(suite) Thoracoesophageal Perforations Normal esophagus The earlier an esophageal perforation is recognized and treated, the better is the chance for successful primary repair. Most agree that such perforations that are not associated with intrinsic esophageal disease are best treated with primary repair of the tear combined with wide mediastinal drainage. A change in philosophy has occurred regarding the application of primary repair to perforations occurring in an normal esophagus regardless of the duration of the injury. Perforations of the lower third of the esophagus are approached through a left thoracotomy in the sixth or seventh interspace, while more proximal thoracic esophageal tears are approached through a right thoracotomy. Mediastinal drainage is achieved by opening the mediastinal pleura from the level of the tear to the thoracic inlet superiorly and the diaphragm inferiorly, irrigating the mediastinum, and placing a large-bore chest tube that allows transpleural drainage. Perforations of the intraabdominal esophagus unassociated with pleural contamination are approached through the abdomen. Esophagus With Intrinsic Disease Perforations associated with distal obstruction from intrinsic esophageal disease constitute a problem because breakdown of an attempted repair is common in the presence of distal obstruction. The associated obstruction must be relieved at the same time of repair and drainage.
SURGICAL DISEASES OF MEDIASTINUM
14 ESOPHAGEAL PERFORATION ACUTE MEDIASTINITIS Operative Therapy Of Esophageal Perforations(suite) Patients with intrinsic esophageal disease that cannot be treated effectively by more conservative means are best treated by esophageal resection. Immediate esophageal substitution with colon(retrosternal) or stomach (in the posterior mediastinum) in the native esophageal bed. SURGICAL DISEASES OF MEDIASTINUM 15 Final position of the mobilized stomach in the posterior mediastinum after transhiatal esophagectomy and cervical esophagogastric anastomosis. ESOPHAGEAL PERFORATION ACUTE MEDIASTINITIS Operative Therapy Of Esophageal Perforations(suite) In situations in which immediate esophageal reconstruction is not possible, the stomach is divided from the esophagus, the cardia is oversewn, The intrathoracic esophagus is then mobilized through the diaphragmatic hiatus and a cervical incision, delivering the entire thoracic esophagus through the neck wound and placing it on the anterior chest wall. The mediastinum can be copiously irrigated through the cervical incision and the diaphragmatic hiatus at the time of esophagectomy A feeding jejunostomy is used for enteral alimentation until reconstruction is performed several weeks later.
SURGICAL DISEASES OF MEDIASTINUM 16 Irrigation of the posterior mediastinum after transhiatal esophagectomy for irreparable esophageal disruption. ESOPHAGEAL PERFORATION ACUTE MEDIASTINITIS Late Esophageal Perforation The longer the time interval between the occurrence of the perforation and operative treatment, the more inflamed are the tissues adjacent to the tear and, at least theoretically, the greater is the risk of failure of primary suture repair. Patients with late-recognized esophageal perforations have been treated in a variety of ways, with wide drainage alone, drainage and closure, drainage over a T-tube, esophageal resection, exclusion and diversion, and even nonoperative management.
SURGICAL DISEASES OF MEDIASTINUM 17 ESOPHAGEAL PERFORATION ACUTE MEDIASTINITIS Infection after cardiac surgery Infection after a median sternotomy for cardiac surgery is a serious complication, especially if the patient has prosthetic aortic graft material at the base of the wound. Mediastinitis in this setting is typically heralded by sternal instability, drainage from the wound, and fever. Various approaches have been used to treat postoperative sternal wound infections: simple dbridement and sternal reapproximation to staged reconstruction and the use of muscle flap rotation into the sternal wound edges. We favor thorough sternal and mediastinal dbridement and one-stage reconstruction with a rotated muscle flap or omentum to fill the retrosternal space. Regardless of the technique used, it is important to obliterate the retrosternal space to prevent reaccumulation of infection. Adequate dbridement of all exposed cartilage from the sternum and ribs is critical because cartilage is avascular, does not heal, and promotes formation of a chronic draining wound sinus. If the sternum is clearly devascularized, it is necessary to remove completely all necrotic bone and cartilage and to then fill the anterior mediastinal space with a muscle flap, which also aids with chest wall stability. SURGICAL DISEASES OF MEDIASTINUM 18 INFECTION AFTER CARDIAC SURGERY ACUTE MEDIASTINITIS Descending necrotizing mediastinitis(DNM) is a lethal form of AM in which infection arising from the oropharynx spreads to the mediastinum. DNM typically develops as a complication of oropharyngeal infection (eg, odontogenic, peritonsillar, or retropharyngeal abscesses, Ludwig angina, or infection after a pharyngeal perforation). Although transcervical drainage is generally adequate treatment for AM resulting from a cervical esophageal perforation, this approach does not provide adequate drainage in the patient with descending necrotizing mediastinitis, and the resulting mortality rate for this condition approaches 40%. Although the standard chest radiograph may demonstrate typical findings of mediastinitis, the CT scan is the most valuable tool in this condition for evaluating the presence of a gas-forming infection within the mediastinum and for following the adequacy of surgical drainage. These patients are ill with: fever, pleuritic chest pain, dysphagia, and varying degrees of airway obstruction resulting from dissection of large amounts of air and acute inflammation within the mediastinal fascial planes. They require a tracheostomy to ensure an adequate airway during treatment of the acute mediastinitis: If only the superior mediastinum is involved and the infection remains above the level of the fourth thoracic vertebra, standard transcervical mediastinal drainage may be adequate but this is rarely the case. SURGICAL DISEASES OF MEDIASTINUM 19 Descending Necrotizing Mediastinitis Most of these patients have extensive mediastinitis, which requires a combination of : Transcervical and subxiphoid or transthoracic drainage. Broad-spectrum aerobic and anaerobic antibiotic coverage should be instituted immediately when the diagnosis is considered, and culture- specific antibiotics should be used as the culture reports return.
The highly lethal nature of this fulminant infection cannot be overemphasized, and patients may experience exsanguination from erosion of the great vessels of the neck and mediastinum, aspiration, cranial nerve paralysis, brain abscesses, and necrotizing fasciitis. Recognition of the fact that one is dealing with more than localized upper mediastinal infection of the type commonly associated with acute esophageal perforations is critical. Aggressive drainage of the mediastinum using the cervical, subxiphoid, or transthoracic route is virtually the only means of salvaging these patients.
SURGICAL DISEASES OF MEDIASTINUM 20 Descending Necrotizing Mediastinitis Chronic Granulomatous Mediastinitis (CGM) CGM frequently involve paratracheal (right more than left) and subcarinal mediastinal lymph nodes. Tuberculosis was in the past, the most common cause of mediastinal granulomatous infection; Histoplasmosis is now the leading etiologic agent. The granulomatous reaction within the mediastinal lymph node may incite an intense surrounding inflammatory response that produces mediastinal fibrosis, which can cause compression of the superior vena cava (SVC), esophagus, trachea, or airways. Resection of large, acutely inflamed mediastinal lymph nodes involved with histoplasmosis has been recommended to minimize the late sequelae of this disease. In cases of vena cava obstruction due to mediastinal fibrosis, vascular reconstruction using either a spiral vein graft or a Gore-Tex graft may be required if symptoms are severe. The necrosis of the adjacent esophageal and tracheobronchial walls with the development of a bronchoesophageal fistulais another potential complication of mediastinal granulomatous disease. This complication requires a transthoracic approach, identification and division of the fistulous opening, closure of both the tracheal and esophageal openings, and interposition of a flap of viable adjacent pleura or mediastinal fat to prevent reformation of the fistula. SURGICAL DISEASES OF MEDIASTINUM 21 Chronic Granulomatous Mediastinitis MEDIASTINAL TUMORS AND CYSTS Mediastinal tumors can arise: primarily, secondary metastases to mediastinal lymph nodes and direct invasion of the mediastinum by tracheobronchial, esophageal, or other malignancies. The differential diagnosis of a mediastinal mass includes: Neoplasms, Congenital cysts, which are not neoplastic. The most reported series of mediastinal tumors include both congenital and neoplastic processes: Neurogenic tumors are most common and account for 21% of mediastinal masses Thymomas (20%), Cysts (20%), and Lymphomas (12%). SURGICAL DISEASES OF MEDIASTINUM 22 The locations of tumors in the mediastinal compartments are as follows:
SURGICAL DISEASES OF MEDIASTINUM 23 MEDIASTINAL TUMORS AND CYSTS Superior Mediastinum Thymoma Lymphoma Thyroid adenoma Parathyroid adenoma Anterior Mediastinum Thymoma Teratoma Carcinoma Lymphangioma Hemangioma Lipoma Posterior Mediastinum Neurogenic tumor Enteric cyst Middle Mediastinum Pericardial cyst Bronchogenic cyst Lymphoma MASAOKA STAGING SYSTEM FOR THYMOMA
SURGICAL DISEASES OF MEDIASTINUM 24 Stage Definition I Macroscopically, completely encapsulated; microscopically, no capsular invasion IIA Macroscopic invasion in surrounding fatty tissues or mediastinal pleura IIB Microscopic invasion into the capsule III Macroscopic invasion into a neighboring organ, such as pericardium, great vessels, or lung IVA Pleural or pericardial dissemination IV B Hematogenous or lymphogenous metastases GENERALITIES In nearly two thirds of adult patients, mediastinal masses cause the following symptoms: chest pain, cough, Dyspnea Half of symptomatic mediastinal tumors are malignant, 95% of asymptomatic mediastinal masses discovered fortunately are benign. Children have a higher incidence of malignancy of mediastinal tumors than adults. Patients with mediastinal masses and evidence of involvement of adjacent structures are more likely to have malignant disease. For example: Hoarseness is indicative of recurrent laryngeal nerve invasion, Horner syndrome signifies invasion of the stellate ganglion, SVC syndrome or tracheal compression suggests mediastinal infiltration by tumor, Back pain may be indicative of chest wall invasion with intercostal nerve involvement IMAGING STUDIES A variety of radiographic studies are used in assessing a mediastinal mass: The standard posteroanterior and lateral chest radiograph assists in localization of the mass. Fluoroscopy, barium esophagogram, and laminography have also been of value. These latter studies have all been replaced by CT SURGICAL DISEASES OF MEDIASTINUM 25 MEDIASTINAL TUMORS AND CYSTS IMAGING STUDIES (suite) magnetic resonance imaging, (MRI) These modern imaging studies provide the most information about: the exact location of the mediastinal mass, its vascularity, relation to adjacent mediastinal structures, and consistency (ie, cystic, solid, or fat). Aortography is needed to differentiate a mediastinal tumor from an aneurysm. BIOPSIES Fine-needle aspiration or core-needle biopsy of mediastinal masses under CT scan or fluoroscopic guidance may provide enough tissue for cytologic or pathologic diagnosis, A transbronchial needle aspiration of subcarinal or paratracheal lymphadenopathy using the flexible fiberoptic bronchoscope and fluoroscopic guidance, Mediastinoscopy, This procedure provides access to paratracheal and subcarinal lymph nodes for the purpose of biopsy but is inappropriate for the assessment of an anterior mediastinal mass. requires general anesthesia, involves passage of a rigid endoscope through a low cervical incision along the anterior trachea into the mid-mediastinum. Because the mediastinoscope follows the course of the trachea into the mid-mediastinum, it cannot be angled forward sufficiently to reach the anterior mediastinum. In patients thought to have an unresectable anterior mediastinal or anterior hilar tumor (eg, lymphoma or metastatic carcinoma), a limited anterior second or third interspace parasternal (Chamberlain) approach can be used. Diagnosis of the paratracheal, superior mediastinal and hilar regions can also be obtained through a transaxillary third interspace minithoracotomy, displacing the apex of the lung downward. SURGICAL DISEASES OF MEDIASTINUM
26 MEDIASTINAL TUMORS AND CYSTS Thoracoscope-directed biopsy is appropriate in most patients who require diagnosis before complete excisionm, when the other modalities fail, For most newly diagnosed mediastinal masses, unless there is strong evidence to suggest unresectability, excisional biopsy is the standard approach.
SURGICAL DISEASES OF MEDIASTINUM
27 MEDIASTINAL TUMORS AND CYSTS PULMONARY ANGIOGRAM Acute shortness of breath. The right pulmonary artery is cut off by an extrinsic mediastinal mass. POSTEROANTERIOR (A) AND LATERAL (B) CHEST ROENTGENOGRAMS a mediastinal neurofibroma that appears as a typically rounded posterior mediastinal paravertebral mass. NEUROGENIC TUMORS Neurogenic tumors, are the most common mediastinal tumors, typically occur in a paravertebral location in the posterior mediastinum, where they arise from the intercostal nerves or sympathetic nerve trunks. Their classic appearance on a standard posteroanterior and lateral chest radiograph is that of a rounded paravertebral mass. The spectrum of neurogenic tumors includes: neurilenoma, neurofibroma, neurosarcoma, ganglioneuroma, neuroblastoma, paraganglioma, and pheochromocytoma. In adults, most neurogenic tumors are benign; in children, they tend to be malignant. Because of their neural crest origin some neurogenic tumors have hormonal activity. Elevated vasoactive intestinal polypeptide levels have been reported with ganglioneuromas and neurofibromas, whereas elevated urinary vanillylmandelic acid levels occur with ganglioneuromas. Neurogenic mediastinal tumors may produce hypertension, flushing, diaphoresis, diarrhea, and abdominal distention, just as is the case with pheochromocytomas. Mediastinal neurofibromas arise from the nerve sheaths and fibers and occur in patients with von Recklinghausen disease. SURGICAL DISEASES OF MEDIASTINUM
28 MEDIASTINAL TUMORS AND CYSTS Ganglioneuromas arise from the sympathetic chain, contain ganglion cells, and are the most common neurogenic tumor in children. The biologic behavior of these tumors varies considerably. The ganglioneuroblastoma, the most aggressive, is associated with an 85% 5-year survival rate if it is completely excised. Neuroblastomas which are highly aggressive malignant tumors that require multimodality therapy combining resection, radiation, and chemotherapy. Neuroblastomas in children may be associated with a neurologic syndrome that includes cerebellar ataxia, opsoclonus, and polymyoclonia; these neurologic changes often regress when the tumor is resected. The cellular DNA content of the tumor has been used as a predictor of response to chemotherapy and as a prognostic indicator in children with neuroblastomas. A major preoperative concern in the patient with a neurogenic tumor is whether there is an intraspinous extension of the tumor through the intervertebral foramen. A tumor with both an intraspinous and intrathoracic component is termed a dumbbell neurogenic tumor(10%) and has the potential for intraoperative disaster if it is not recognized and planned for preoperatively. This should be suspected in any patient with a posterior mediastinal tumor who presents with either radicular pain, vertebral body pedicle erosion, or enlargement of an intervertebral foramen on spinal radiographs or CT scan. In such situations, either a myelogram or magnetic resonance imaging scan of the spine is indicated to determine if there is an intraspinous component of the tumor.
SURGICAL DISEASES OF MEDIASTINUM
29 MEDIASTINAL TUMORS AND CYSTS
SURGICAL DISEASES OF MEDIASTINUM 30 MEDIASTINAL TUMORS AND CYSTS MR image demonstrating intraspinous and intrathoracic components of a dumbbell neurogenic tumor. CT tomography shows the mass to be cystic (cursor), and the right pulmonary artery draped over it. Patients with an asymptomatic posterior mediastinal tumor that is suspected on the basis of chest radiograph and CT scan to be a neurogenic tumor require only a routine preoperative assessment before thoracotomy. Those in whom an intraspinous component of the tumor has been demonstrated require a combined neurosurgicalthoracosurgical approach. If such a dumbbell tumor is inadvertently amputated during resection of the intrathoracic component of the tumor, allowing the remainder of the tumor to retract into the spinal canal, subsequent intraspinous bleeding may result in paraplegia or death. Therefore, when dealing with a dumbbell neurogenic tumor, the intraspinous component should be resected first and then the intrathoracic component. This is achieved in one operation by extending the posterior laminectomy neurosurgical incision into a posterior thoracotomy in the appropriate interspace. SURGICAL DISEASES OF MEDIASTINUM 31 MEDIASTINAL TUMORS AND CYSTS Pheochromocytoma Pheochromocytomas are hormonally active tumors of the sympathetic nervous system. They are termed chromaffin tumors because of their affinity for chromic salts on staining. Chromaffin tissue is of neural crest origin and occurs in the adrenal medulla, in the sympathetic ganglia, in the paraganglia along the sympathetic chain and the organ of Zuckerkandl, in small nests scattered along the aorta, in walls of blood vessels, and in the heart, prostate, and ovary. Any of these aberrant collections of chromaffin tissue can give rise to pheochromocytomas. 90 % of pheochromocytomas occur in the adrenal gland, where they produce excessive catecholamines, predominantly norepinephrine. Ten percent of pheochromocytomas occur in an extraadrenal location, and fewer than 2% of all pheochromocytomas occur in the chest. using the relatively newly developed radiopharmaceutical 131I-metaiodobenzylguanidine (131-MIBG), which has permitted scintigraphic localization of pheochromocytomas. Using the 131-MIBG scan in combination with contrast-enhanced CT, a growing number of cardiac pheochromocytomas are reported. Intrathoracic pheochromocytomas present both diagnostic and therapeutic challenges. Unlike pheochromocytomas of the abdomen or posterior chest, these tumors do not shell out from adjacent tissue. They frequently require resection of involved myocardium or coronary vessels with pericardial patching or coronary artery bypass grafting, and therefore cardiopulmonary bypass must be available for their removal.
SURGICAL DISEASES OF MEDIASTINUM
32 MEDIASTINAL TUMORS AND CYSTS Paragangliomas Paragangliomas (chemodectomas) of the parasympathetic nervous system do not contain chromaffin and, unlike pheochromocytomas, usually do not produce hormones. These tumors typically occur in carotid body, glomus jugulare, aorticopulmonary glomus, vagal body, and ciliary glomus chemoreceptor tissues. These relatively rare tumors most often are localized to the posterior mediastinum, as is the case with most thoracic neurogenic tumors. In this latter location, where they arise from the paravertebral sympathetic ganglia, they are readily resectable, as are most posterior mediastinal neurogenic tumors.
Teratoma Teratomas are composed of cells that arise from more than one embryonic germ cell layer. Totipotential cells of the ovary, testis, and embryonic rests that can differentiate into any of the three primary germ cells layers give rise to teratomas. Mediastinal teratomas typically occur as dermoid cysts in the anterior mediastinum, frequently growing to large size and containing hair and teeth. Most teratomas are benign, and the 10% to 20% that are malignant are frequently associated with elevated -fetoprotein and carcinoembryonic antigens. When a malignant teratoma is suspected preoperatively, serum tumor marker levels should be obtained and a needle biopsy performed to establish a tissue diagnosis. SURGICAL DISEASES OF MEDIASTINUM 33 MEDIASTINAL TUMORS AND CYSTS Multidrug chemotherapy, based primarily on cisplatin, is then instituted, and when the tumor markers fall to a normal range, wide resection of the tumor is carried out. Although most teratomas are benign and can be resected through a median sternotomy, their large size and the surrounding inflammatory response that they induce may complicate the resection.
Thymoma Thymic tumors occur more frequently in adults than in children. Rarely, infants with marked thymic enlargement due to hyperplasia require emergent thymectomy to relieve their cardiorespiratory embarrassment. This approach is now thought to be preferable to radiotherapy, which was used in the past, but which is associated with a definite increased risk of malignancy in the field of radiation. The relation between the thymus gland and myasthenia gravis has been appreciated for many years. Myasthenia gravis is generally regarded as an immunologic disorder in which serum antibodies form against acetylcholine receptors in the muscle. The thymus gland has been postulated to be the source of the acetylcholine receptorlike antigen. Some 10% to 20% of patients with myasthenia gravis have thymomas, and 60% have thymic hyperplasia. Nearly 75% of patients with thymomas develop myasthenia gravis within 10 years. Thymoma has been associated with other conditions besides myasthenia gravis, specifically, red blood cell aplasia, Cushing syndrome, hypogammaglobulinemia, and collagen vascular disease.
SURGICAL DISEASES OF MEDIASTINUM 34 MEDIASTINAL TUMORS AND CYSTS Thymectomy is generally most beneficial in young women with myasthenia gravis who have no thymomas and a short duration of their disease. Most neurologists still use anticholinesterase drugs (pyridostigmine and neostigmine), immunosuppressants (azathioprine, antilymphocyte serum, antithymocyte serum), and occasionally steroids as the primary treatment for myasthenia gravis. Any patient who has a thymoma should undergo a thymectomy both to establish the diagnosis of the mediastinal mass and to prevent potential spread of the tumor. One quarter of thymomas are malignant. Traditional teaching has held that the diagnosis of malignant thymoma is extremely difficult on the basis of histologic criteria, the most important determinant of malignancy being the surgeons assessment of invasion by the tumor of adjacent tissues such as pleura, blood vessels, pericardium, or lung. The Masaoka staging system for thymoma combines both operative findings and histologic evaluation to guide therapy A 10-year survival rate of 87% has been reported for encapsulated thymoma, 62% for locally invasive, and 40% if there is pleural seeding. With the addition of chemotherapy and radiotherapy for patients with stage II or greater disease, survival was markedly improved by the addition of neoadjuvant radiation and chemotherapy. SURGICAL DISEASES OF MEDIASTINUM 35 MEDIASTINAL TUMORS AND CYSTS Miscellaneous Tumors Mediastinal lymph node involvement by lymphoma may present as an anterior mediastinal mass on chest radiograph. Because the accurate diagnosis of lymphoma by cytology of tissue obtained from fine-needle aspiration is notoriously difficult unless there is associated adenopathy at other sites that are more amenable to biopsy, biopsy of mediastinal lymph nodes may be required to obtain adequate diagnostic specimens. Either mediastinoscopy (for paratracheal or subcarinal adenopathy), or an anterior mediastinotomy through a parasternal second or third interspace incision (Chamberlain) approach is used. Radical resection or debulking of mediastinal lymphoma is inappropriate therapy because these tumors are more responsive to combined radiation and chemotherapy. Large goiters of the thyroid gland may grow retrosternally into the superior mediastinum. Although these substernal goiters present as an anterior superior mediastinal mass on chest radiograph, their resection rarely requires a sternal split, and they can virtually all be resected through a standard transcervical approach. Because tracheomalacia may result from prolonged pressure on the trachea by the enlarged thyroid, prolonged ventilatory assistance may be required postoperatively. Ectopic mediastinal thyroid tissue may present as a thyroid adenoma. These tumors characteristically present as asymptomatic mediastinal masses. Ten percent of parathyroid adenomas are located within the anterosuperior mediastinum and may require a variety of studies for diagnosis and localization (eg, venous angiography with sequential parathyroid hormone assays and CT, thallium, and technetium scanning). Less common mesenchymal tumors of the mediastinum include fibrosarcomas and liposarcomas, fibrous histiocytomas, leiomyosarcomas, and mesotheliomas. Mediastinal tumors of the vascular and lymphatic systems (hemangiomas, hemangiopericytomas, and lymphangiomas) are extremely rare and usually occur in the anterior mediastinum. SURGICAL DISEASES OF MEDIASTINUM
36 MEDIASTINAL TUMORS AND CYSTS Mediastinal Cysts Mediastinal cysts are classified as: bronchogenic, enteric (duplication), pericardial. Bronchogenic cysts originate from the primordial respiratory tissues of the ventral foregut and are generally found in proximity to the trachea, main-stem bronchi, or posterior carina. They have a ciliated respiratory epithelial lining. Although usually asymptomatic in adults and presenting as a smooth mediastinal mass near the carina, these cysts can attain large size and cause compression of adjacent structures. Enteric (duplication or enterogenous) cysts arise from the dorsal foregut from which the alimentary tract evolves and are therefore located in proximity to the esophagus in the posterior mediastinum, occasionally being found intramurally within the wall of the esophagus Functioning gastric epithelium that lines some of these cysts may result in ulceration and bleeding. Technetium scanning has been used to localize gastric mucosa within the mediastinum. Enterogenous cysts are often associated with vertebral body abnormalities and spinal cord attachments.
SURGICAL DISEASES OF MEDIASTINUM 37 MEDIASTINAL TUMORS AND CYSTS Mediastinal Cysts (suite) Pericardial cysts Almost 75% of them occur in the right cardiophrenic angle, either in continuity with the pericardial space or separately as a pericardial developmental abnormality. They rarely cause symptoms and are most often detected on a chest radiograph obtained for other reasons. In the past, resection of these masses was recommended to establish a tissue diagnosis; the CT scan now usually can identify the cystic nature of the lesion, making the need for resection less compelling. Percutaneous CT-guided needle aspiration of pericardial cysts has been recommended, and the fluid obtained is evaluated cytologically.
SURGICAL DISEASES OF MEDIASTINUM 38 MEDIASTINAL TUMORS AND CYSTS Barium esophagogram, posteroanterior (A) and lateral (B) views, showing an intramural esophageal duplication cyst. Mediastinal emphysema (pneumomediastinum) Entry of air into the mediastinum may occur from the tracheobronchial tree, the neck, or the abdomen. Both penetrating wounds of the mediastinum as well as blunt chest trauma may be responsible for mediastinal emphysema. Compression injuries of the thorax may cause a marked rise in intrathoracic pressure, rupture of peripheral alveoli, and the initiation of dissection of air in the interstitial planes of the lung toward the hilum and then into the mediastinum. A forceful sneeze or bout of asthma can produce mediastinal emphysema in the same way (spontaneous mediastinal emphysema). Clinical presentation mediastinal emphysema may have a dramatic clinical presentation but is usually not life-threatening. Patients often complain of : retrosternal discomfort subcutaneous crepitus at the base of the neck air continues to dissect upward from the mediastinum into the subcutaneous tissue planes, cervical, fascial, thoracic, truncal, scrotal, and extremity swelling and crepitus can develop. air within the periorbital tissues may cause sufficient swelling to prevent the patient from opening the eyelids. a precordial crunch is heard typically during systole (Hamman sign) on auscultation over the anterior chest.
SURGICAL DISEASES OF MEDIASTINUM
39 Mediastinal emphysema (pneumomediastinum) Mediastinal emphysema (pneumomediastinum) (suite) Air is seen in the mediastinal tissue planes, along the pericardium, and in the soft tissues of the neck, chest, and upper abdomen on chest radiographs. Rarely in adults, tension pneumomediastinum may interfere with venous return to the heart and result in cardiovascular collapse. Most often, spontaneous mediastinal emphysema is self-limiting and requires little treatment other than sedation and supplemental oxygen. If an associated pneumothorax is identified, a tube thoracostomy should be performed. If the patient is distressed by the inability to open the eyes, 5-mm decompressing incisions made in the skin folds of the eyelids or neck using local anesthesia permit the subcutaneous air to be milked out by gentle pressure, allowing the patient to open the eyes.
SURGICAL DISEASES OF MEDIASTINUM 40 Mediastinal emphysema (pneumomediastinum) Superior vena cava syndrome is due to obstruction of the SVC and presents clinically : facial and upper extremity edema, distention of the veins of the head, neck, arms, and upper thorax, dusky rubor of these areas suggesting cyanosis. Patients complain of : periorbital swelling, a full feeling in their head, and a roaring in the ears aggravated by lying supine or bending. Differential diagnosis congestive heart failure, cirrhosis, constrictive pericarditis may be included in, these conditions are excluded by the absence of swelling in the lower half of the body. 75% of patients with SVC syndrome have malignant disease within the mediastinum compressing the SVC, most often bronchogenic carcinoma involving the right upper lobe. SVC obstruction can also result from lymphoma and metastatic carcinoma within the mediastinum. 25% of patients with SVC syndrome have a benign cause, such as mediastinal granulomatous disease, idiopathic mediastinal fibrosis, goiter, or bronchogenic cysts. Despite the rather characteristic clinical presentation that leaves little doubt as to the diagnosis, many physicians still feel compelled to obtain a venogram. SURGICAL DISEASES OF MEDIASTINUM
41 Superior vena cava syndrome Not only does this study add little information to that provided by the physical examination, but it may also be associated with considerable morbidity if injected contrast material extravasates into the subcutaneous tissues of the arm when an injection is performed in the presence of marked venous hypertension. The venogram for evaluation of SVC obstruction has been replaced with the use of MR imaging and angiography, which delineate mediastinal structures as well as vascular impingement from extrinsic compression. In most patients with SVC syndrome due to malignant disease, cure is not possible because of the extent of mediastinal invasion by the tumor. Nevertheless, a tissue diagnosis is important because it may alter therapy. Therefore, sputa should be collected for cytologic evaluation. A central lung mass identified on CT scan may be diagnosed with bronchoscopy and biopsy or transthoracic fine-needle aspiration biopsy. Mediastinoscopy has also been used in patients with SVC syndrome, although there are two concerns with the use of this procedure in this setting. First, mediastinal venous hypertension may be responsible for bleeding complications. Second, SVC obstruction may produce submucosal edema of the tracheobronchial tree that can precipitate acute airway obstruction when aggravated by endotracheal intubation that is required for general anesthesia in these patients. Both radiotherapy and chemotherapy are often effective in providing rapid and acute relief of SVC obstruction due to malignant disease. Because of the poor prognosis of patients with SVC obstruction due to malignant disease, surgical therapy is seldom indicated. Most patients with SVC obstruction due to benign disease gradually develop chest wall and mediastinal venous collaterals, so that their symptoms improve with time. In rare cases, autogenous or prosthetic vein graft replacement or bypass is used to relieve the SVC obstruction. SURGICAL DISEASES OF MEDIASTINUM 42 Superior vena cava syndrome