Sarcoidosis: still a mystery?

Sarcoidosis is a systemic granulomatous disease of unknown etiology. It has diverse clinical manifestations, most frequently including pulmonary disorders. It is associated with immunological abnormalities, the intricacies of which have yet to be clearly delineated. In the immunologically susceptible individual, genetic, environmental, nutritional, and socioeconomic factors may play a governing role in its development. Sarcoidosis is a diagnosis of exclusion established by clinical manifestations, radiologic findings, and histologic evidence of noncaseating epithelioid-cell granulomas in >1 organ. We will discuss parameters that are helpful in making this diagnosis.

Paget-Schroetter syndrome: what is the best management?

BACKGROUND: The evaluation of 312 extremities in 294 patients with Paget-Schroetter syndrome (effort thrombosis of the axillary-subclavian vein) over 30 years provides the basis for optimal management determination. METHODS AND RESULTS: Group I (35 extremities) was initially treated with anticoagulants only. Twenty-one developed recurrent symptoms after returning to work, requiring transaxillary resection of the first rib. Thrombectomy was necessary in eight. Group II (36 extremities) was treated with thrombolytic agents initially, with 20 requiring subsequent rib resection after returning to work. Thrombectomy was necessary only in four. Of the most recent 241 extremities (group III), excellent results accrued using thrombolysis plus prompt first rib resection for those evaluated during the first month after occlusion (199). The results were only fair for those if seen later than 1 month (42). CONCLUSIONS: An early diagnosis (less than 1 month), expeditious thrombolytic therapy, and prompt first rib resection are critical for the best results.

Neurovascular compression in the thoracic outlet: changing management over 50 years.

SUMMARY BACKGROUND DATA: During the past five decades, significant improvements have been made in the diagnosis and treatment of thoracic outlet syndrome (TOS) secondary to sports activities, breast implants, or median sternotomy. METHODS, RESULTS, AND CONCLUSIONS: Of more than 15,000 patients evaluated for TOS, 3914 underwent primary neurovascular decompression procedures and 1221 underwent second surgical procedures for recurrent symptoms. Of 2210 consecutive patients, 250 had symptoms of upper plexus compression only (median nerve), 1508 had symptoms of lower plexus compression only (ulnar nerve), and 452 patients had symptoms of both. Ulnar and median nerve conduction velocities confirmed the clinical diagnosis. Transaxillary first rib removal alone for neurovascular decompression relieved both upper and lower plexus symptoms (without a combined transaxillary and supraclavicular approach). There are two reasons for this: most upper compression mechanisms attach to the first rib, and the median nerve is also supplied by C8 and T1 as well as C5, C6, and C7 nerve roots. Axillary subclavian artery aneurysm or occlusion was treated successfully in 240 patients. Dorsal sympathectomy was performed concomitantly in 71 patients for occlusion or embolectomy. It was combined with first rib resection in 1974 patients for sympathetic maintained pain syndrome and causalgia that did not improve with conservative therapy. Of 264 patients with effort thrombosis (Paget-Schroetter syndrome), 211 were treated by urokinase thrombolysis and prompt first rib resection with excellent long-term results. Recurrent TOS symptoms required a second procedure using the posterior approach in 1221 patients with brachial plexus neurolysis and dorsal sympathectomy. The use of hyaluronic acid significantly reduced recurrent scarring.

Upper plexus thoracic outlet syndrome: optimal therapy.

BACKGROUND: Previously, transaxillary first rib resection alone was not considered adequate therapy for "upper plexus" (median nerve) thoracic outlet syndrome. It was thought that the "combined" approach with upper plexus dissection through a supraclavicular incision in addition to the transaxillary approach was necessary. However, with better understanding of anatomy--that the median nerve receives fibers from C8 and T1 as well as the upper plexus and that muscles that compress the upper plexus attach to the first rib--it is now recognized that first rib removal alone will relieve upper plexus compression. METHODS: Assessment of 2,210 operations for thoracic outlet syndrome revealed 250 patients (11%) had symptoms and nerve conduction velocity slowing of the median nerve only (upper plexus), whereas 452 (20%) patients had both median and ulnar nerve compression (upper and lower), and 1,508 patients exhibited compression symptoms and nerve conduction velocity slowing of the ulnar nerve alone (lower plexus). RESULTS: Transaxillary first rib resection relieved symptoms of median nerve (upper plexus) compression as well as it did for ulnar nerve (lower plexus) compression. Treatment outcome comparisons of patients with median and ulnar compression show no significant differences. CONCLUSIONS: These data refute the need for supraclavicular or combined supraclavicular and transaxillary approaches to treat patients with upper plexus (median) thoracic outlet syndrome compression as previously recommended. The transaxillary approach alone is satisfactory.

Improved management of the Paget-Schroetter syndrome secondary to thoracic outlet compression.

For years, patients with "effort" thrombosis were treated by anticoagulants and conservative exercise; and if recurrent symptoms developed when they returned to work, they were considered for first-rib resection. Recently, use of thrombolytic agents with early surgical decompression of the neurovascular compression has markedly reduced morbidity, such as postphlebitic syndrome and the necessity for thrombectomy. A review of 67 patients seen over 25 years showed that 34 were initially treated with heparin sodium and then Coumadin (crystalline warfarin sodium). Recurrent symptoms developed in 21 after they returned to work and necessitated transaxillary first-rib resection to relieve symptoms. Eight also underwent thrombectomy. Recently, 33 patients were initially treated with thrombolytic agents and heparin, followed promptly by early first-rib resection. The evaluation and efficacy of this therapy have been established by frequent and repetitive venograms and careful follow-up of patients. Most of the patients showed improvement with thrombolytic agents. Remaining stenoses that suggested intravascular thrombosis were usually secondary to external compression of the vein by the clavicle, costoclavicular ligament, rib, or scalenus anterior muscle. Venous thrombectomy was necessary in only 4 patients in whom the clot was not controlled by thrombolytic therapy and operative release of compression. There were no deaths in the series.

Sclerosing mediastinitis: improved management with histoplasmosis titer and ketoconazole.

Recognition that many patients with benign sclerosing mediastinitis have smoldering disease responsible for failure of surgical procedures or for development of collateral circulation in patients with superior vena caval obstruction has markedly improved management of these difficult patients. Histoplasmosis complement fixation titers have been used to detect unsuspected subacute disease and to follow the therapeutic adjunctive management with ketoconazole, an oral antifungal agent. Twenty-two patients with benign sclerosing mediastinitis demonstrated a variety of symptoms relating to the area of compression: superior vena cava, 13; esophagus, 3; pulmonary artery and pericardium, 3; and trachea, 3. Histoplasmosis was documented in 12 patients. Operation is used initially for diagnosis, to rule out carcinoma, and to treat the complications: superior vena caval reconstruction, 6; tracheal decompression, 2; right middle lobectomy, 1; esophageal decompression, 2; division of tracheoesophageal fistula, 1; and release of pericardial effusion and cardiac tamponade, 1. Postcardiotomy syndrome occurred in 1 patient and wound infection in another. No deaths resulted. In 6 cases of histoplasmosis, symptoms recurred in 100% of patients and were successfully managed with ketoconazole treatment, and then clinical progress was monitored with serial histoplasmosis complement fixation studies. One patient had four superior vena caval reconstructions at an outside hospital, each 1 year apart, with symptoms recurring each time. With ketoconazole therapy alone, she has been asymptomatic for more than 2 years. Vigorous search for a fungal cause may even obviate the necessity for surgical intervention. If an operation is necessary, preoperative and postoperative use of ketoconazole has assured success.

Operative transluminal balloon angioplasty. Adjunct to coronary bypass for extended myocardial revascularization of more than 3000 lesions in 1000 patients.

Operative transluminal coronary artery balloon angioplasty has been used for over 3000 lesions in 1000 patients since 1980. Initially it was only used for distal stenoses not accessible to coronary bypass grafting in 200 patients. Recatheterization of patients who had intraoperative transluminal balloon angioplasty of the proximal left anterior descending, right, and circumflex coronary arteries 3 years previously revealed excellent patency of both the bypass grafts and the dilated native coronary arteries. This observation supports the thesis that with properly constructed bypass anastomoses competitive flow does not significantly mandate graft thrombosis. Subsequently, intraoperative balloon angioplasty has been performed for both proximal and distal stenoses in 800 patients to improve native coronary artery perfusion and maximize revascularization. Follow-up from 1 to 7 years revealed perioperative myocardial infarction in 21 patients (2.1%) and death in 19 patients (1.9%). Recatheterization from 1 to 7 years after the operation in 51 patients (41 with symptoms) revealed that patency was almost as prevalent in arteries subjected to angioplasty (82%; 137/167) as in bypass grafts (84%; 102/122). Intraoperative balloon angioplasty appears to improve coronary artery perfusion without detrimental competitive flow when used with bypass grafts.

The failed operation for thoracic outlet syndrome: the difficulty of diagnosis and management.

In 225 patients requiring reoperation for recurrent thoracic outlet syndrome, "pseudorecurrences" were noted in 43 patients not relieved of symptoms after the initial operation. Such recurrences were associated with technical errors at the initial operation including resection of the second rib instead of the first, resection of the first with a cervical rib left in place, or resection of a cervical rib with an abnormal first rib left. True recurrences occurred in 182 patients, 154 of whom had a substantial piece of rib remaining from the initial procedure. Indications for reoperation included persistent pain, ulnar nerve conduction velocity of 60 m/sec or less (normal, 72 to 82 m/sec), and failure of appropriate physical therapy. Reoperation involved neurolysis of the brachial plexus, decompression of the vessels, and dorsal sympathectomy performed through a posterior thoracoplasty incision. One hundred seventy-seven patients (79%) had improvement, 32 (14%) had moderate improvement, and 16 (7%) were either considered failures or had recurrent scarring.

Median sternotomy as a standard approach for pulmonary resection.

Pulmonary resection was performed electively through a median sternotomy in 174 patients. The first 61 of these patients were compared with an equal number having pulmonary resection through a lateral thoracotomy. Both groups were similar in regard to sex, age, pathological condition, and type of resection. The patients undergoing median sternotomy had a shorter operative time and less postoperative pain, and were discharged from the hospital sooner than the patients having resection through a lateral thoracotomy. The cardinal technical essentials to expeditious pulmonary resection through a median sternotomy involve unilateral pulmonary ventilation with a double-lumen endotracheal tube, arterial pressure and gas monitoring, and proper lung packing and retraction. Two patients died, 1 of infection and 1 of bleeding; neither death was related to the incision. Certain pulmonary procedures are better performed through a lateral thoracotomy, and these include resection of a superior sulcus carcinoma, pulmonary resection with posterior chest wall extension, and left lower lobe resection in patients who demonstrate obesity, cardiomegaly, or an elevated diaphragm.

Poland's syndrome: improved surgical management.

Single-stage reconstruction of the chest wall combined with simultaneous augmentation mammoplasty and transfer of an island pedicle myocutaneous flap of latissimus dorsi muscle are major improvements over previous multiple-stage procedures that provide less satisfactory cosmetic results in management of patients with Poland's syndrome. Utilization of the single-stage technique in 2 patients demonstrated its efficacy as proven by excellent cosmetic results. In 1 patient with absent second, third, and fourth costal cartilages and ribs, Marlex mesh covered with a synthetic dura mater graft was employed to stabilize the chest wall. Simultaneously, an island pedicle myocutaneous flap of latissimus dorsi with its neurovascular bundle preserved was transferred to cover the prosthesis. The other patient had a coexistent pectus carinatum defect, which was repaired by resection of the costal cartilages and osteotomy of the sternum without use of Marlex. The breast implant was covered concomitantly with the myocutaneous flap of latissimus dorsi. No morbidity or mortality occurred. The cosmetic and functional results are superior to those obtained with standard techniques.

Bypass grafting and aneurysmorrhaphy for aortic arch aneurysms.

The technique of permanent aortic arch bypass grafting combined with simultaneous aneurysmorrhaphy, excision or exclusion of the aneurysm without the use of systemic heparinization, cardiopulmonary bypass, or external bypass shunting has been used successfully in treating 5 patients with aneurysms of the aortic arch. There were no complicating cerebral vascular accidents, bleeding diatheses, or signs of distal embolization. There were 2 deaths; 1 patient died early (10 days postoperatively) of myocardial infarction, and the other died over one year later of unrelated causes. Follow-up has extended to an interval of seven years. The early death subsequent to myocardial infarction in 1 patient prompted the routine use of preoperative carotid and coronary angiography for assessment of these systems. Severe occlusive disease in these vessels requires a staged or simultaneous reconstruction prior to management of the aortic aneurysm.

"Collis-Belsey" fundoplication for uncomplicated hiatal hernia and gastroesophageal reflux.

The combined Collis gastroplasty-Belsey Mark IV fundoplication was used in 86 patients with uncomplicated hiatal hernia followed for up to 8 years. Marked relief of symptoms was obtained, with no initial morbidity and mortality. Recurrence of hernia occurred in 1 patient. Minimal gastroesophageal reflux was observed in a few patients. Manometric and pH studies performed after operation showed a competent valve without notable esophageal reflux. The Collis gastroplasty creates a lesser curvature gastric tube that lengthens the so-called functional esophagus and eliminates tension at the suture line of the Belsey Mark IV fundoplication.

Management of malignant esophagorespiratory fistula.

Esophagorespiratory communication developed in 46 patients among 570 with esophageal cancer. Therapy was basically palliative and aimed at mechanical interruption of the fistula, restoration of esophageal continuity, and avoidance of external tubes and appliances. Supportive therapy, gastrostomy, tracheostomy, and esophageal exclusion and diversion procedures resulted in little prolongation of life and poor palliation of the patient. Permanent endoesophageal intubation with tubes of the Celestin variety resulted in best palliation with minimal operative risk for most terminal patients. Colon bypass and occasional resection can accomplish the same goal and possibly provide long-term survival in good-risk, young patients with small tumors.

Primary pulmonary hemangiopericytoma.

Hemangiopericytoma is a potentially malignant tumor of vascular origin. It usually is a solitary lesion and may arise in the chest or in extrathoracic areas. It is usually encapsulated. Microscopic features vary with the degree of differentiation of the individual tumor. The more differentiated variety exhibits abundant capillaries with open lumina surrounded by ovoid tumor cells; in the less differentiated tumors, the capillaries lack lumina and the tumor cells are spindle shaped. The prognostic significance of the microscopic pattern is controversial. However, prominent mitotic activity, necrosis, hemorrhage, and increased cellularity are ominous signs and are usually noticed in tumors that later exhibit malignant behavior. The larger the lesion, the more likely that it will be symptomatic. Treatment of choice is ample surgical resection.

Management of concomitant occlusive disease of the carotid and coronary arteries.

With the advent of direct coronary artery revascularization, the high mortality rate from cardiac disease associated with carotid endarterectomy can be favorably altered by simultaneous or staged revascularization for combined lesions. The choice for combined or sequential procedures is determined by the severity of the disease both clinically and anatomically in each system. Review of 32 patients with both severe coronary and carotid occlusive disease established that selective surgical intervention has been successful, with no deaths and only minimal morbidity. Simultaneous revascularization was carried out in 8 patients with preinfarctional angina, significant left main lesion or triple vessel disease producing a functional lesion of the left main coronary artery, and tight carotid lesion. Staged operations were performed in the remaining 24 patients. Priority of staging was determined by the extent of disease in each system.

Coronary artery bypass occlusion second to postcardiotomy syndrome.

Postcardiotomy syndrome following coronary vein bypass procedures may cause graft occlusion as a result of the associated myxedematous hyperemic inflammation that later forms dense obliterative fibrosis. Variability of symptoms and onset make early recognition of postcardiotomy syndrome difficult. However, the presence of the classic triad of fever, chest pain, and pleuroericardial reactions along with leukocytosis and elevated sedimentation rate should suggest the diagnosis. Early recognition and prompt institution of steroid therapy offer relief of symptoms and regression of inflammation and probably reduce the incidence of graft occlusion. From early experience with 14 patients who developed postcardiotomy syndrome and received only symptomatic treatment, 12 developed graft occlusion, whereas in 31 subsequent patients with this syndrome who were treated with steroids, only 5 demonstrated graft occlusion.

Giant fibrovascular polyp of the esophagus.

A case of giant fibrovascular polyp of the esophagus with a review of the literature is presented. The lesion is benign and pedunculated and may attain giant proportions. Symptoms are related to esophageal obstruction. Death by asphyxia can occur. Small lesions can be removed endoscopically with a snare. Larger lesions should be excised using a formal surgical approach.

Pulmonary giant cell carcinoma.

Pulmonary giant cell carcinoma is one of the most highly malignant neoplasms of the lung. Eighteen patients with this tumor are presented. Histologically the neoplasm is composed of a preponderance of multinucleated giant cells, round cells, and spindle cells. Some of the most differentiated tumors show adenocarcinomatous foci and ultrastructural features of secretory epithelium favoring its classification as a variant of pulmonary adenocarcinoma. The giant cells of this neoplasm can be differentiated from those encountered in undifferentiated large cell carcinoma and epidermoid carcinoma by the abundant cytoplasm, the presence of more nuclei and nucleoli, and the significant degree of phagocytosis. The clinical picture and roentgenographic findings present no pathognomonic features.