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.

Poland's syndrome.

Poland's syndrome is characterized by hypoplasia or absence of the breast or nipple, hypoplasia of subcutaneous tissue, absence of the costosternal portion of the pectoralis major muscle, absence of the pectoralis minor muscle, and absence of costal cartilages or ribs 2, 3, and 4 or 3, 4, and 5. Clinical manifestations of Poland's syndrome are extremely variable and rarely are all the features recognized in one individual. Depending on the physician's specialty and the referral pattern, a variable incidence of the anomalous defects is recognized. Syndactyly or bony abnormalities of the forearm are seldom treated by thoracic surgeons.

The history of surgery for thoracic outlet syndrome.

The history of surgery for thoracic outlet syndrome (TOS) is examined, ranging from the earliest recorded reference of TOS in the anatomic recognition of cervical ribs by Galen and Vesalius, to the current research on the diagnosis and treatment of TOS. The author discusses various cases that helped advance the use of surgery in treating TOS, tracing the years of progress that led to the present day understanding of the disease.

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.

Alcohol use following liver transplantation for alcoholic cirrhosis.

Due to the significant increase in the number of patients with alcoholic liver cirrhosis being referred for liver transplantation, studies to determine recidivism rates and influential factors affecting those rates have become increasingly crucial. Between 12/85 and 12/91, 67 patients diagnosed with alcohol related end-stage liver disease underwent orthotopic liver transplantation at Baylor University Medical Center. A 3-8 year follow-up study was conducted wherein surviving patients were contacted by phone to evaluate subsequent alcohol consumption following transplantation (with the exception of two patients whose primary physicians were contacted). Of the 67 patients transplanted, 18 had expired, 7 were alive but unavailable, and 1 had been lost to follow-up. Of the remaining 41 patients interviewed, 21 had remained abstinent, while the other 20 had returned to some form of drinking. Of patients with less than 6 months of pretransplant abstinence, only 30% remained abstinent, while the other 70% had resumed drinking. Regarding patients with at least 6 months of pretransplant abstinence, 58% had remained abstinent, while the other 42% had resumed drinking. In both groups, nearly 1/3 of those who had admitted to posttransplant drinking reported themselves as again abstinent and recommitted to sobriety when interviewed. In conclusion, 49% of patients interviewed had resumed some type of drinking following transplantation-- however, this appears not to have affected compliance or survival potential. Only 2 (4.8%) of the 41 patients interviewed had returned to excessive drinking. Thus, our findings support the use of orthotopic liver transplantation for patients with alcohol related end-stage liver disease.

The transaxillary approach for treatment of thoracic outlet syndromes.

The diagnosis and management of thoracic outlet syndromes is based on the surgical management of more than 3,000 patients, 800 of which have had recurrent thoracic outlet syndromes. Accurate diagnosis for peripheral nerve compression is based on measurement of the ulnar and median nerve conduction velocities across the thoracic outlet. For sympathetic maintained pain syndrome or causalgia, a stellate ganglion block is helpful. Arteriography and venography are critical to show vascular compression. Conservative management is successful in most cases (70%) initially. For arterial reconstruction, the supraclavicular-infraclavicular approach is recommended. For the Paget-Schroetter syndrome (effort thrombosis of the axillary subclavian vein), prompt thrombolysis followed by transaxillary first rib resection is mandatory. No long-term anticoagulants are necessary. For hyperhidrosis, causalgia, sympathetic maintained pain syndrome or reflex sympathetic dystrophy, transaxillary dorsal sympathectomy with first rib resection or thoracoscopy is the preferred management when conservative therapy fails. For recurrent thoracic outlet syndrome and sympathetic maintained pain syndrome, a high thoracoplasty posterior approach is preferable with neurolysis of the nerve roots and brachial plexus as well as a dorsal sympathectomy. The technique of transaxillary first rib resection with or without dorsal sympathectomy is presented. The use of the thoracoscope expedites the procedure and improves the teaching capability.

Solo or partnership.

Reassessment of the managed care environment and restructuring of our practice will be accomplished by transitional short-term actions while we institute our long-range plan. In the short term we must develop appropriate relationships with hospitals and medical groups that interrelate to our specialty, and develop a "love our enemy" philosophy. We must establish operating organizational units that will be competitive. Reassessment of our own practice, our unique skills, our inadequacies, our competition, and our performance through outcome studies is essential to retake positions weakened by loss of contract inclusion. Long-term strategy must develop insurance coverage in conjunction with our patients in such a way that the middleman is eliminated and the profit currently extracted by managed care is reinvested in patient care, research, and education. Whoever controls payment determines the quality of medicine and how it is delivered.

Dorsal sympathectomy and management of thoracic outlet syndrome with VATS.

Dorsal sympathectomy and the management of the thoracic outlet syndrome have been considerably improved with the use of video assistance because it affords both magnification and an improved light system. Two techniques of video assistance were employed in the group of patients described here. One involved the sympathectomy done through three ports using standard video-assisted thoracic surgical methods. The second technique involved a transaxillary incision with removal of the first rib using video-assistance magnification and light, operating either directly or secondarily while visualizing the image on the television set. (The vast majority of cases have been performed using this latter technique.) Major indications for performing dorsal sympathectomy include (1) hyperhidrosis, (2) Raynaud's phenomenon, (3) Raynaud's disease, (4) causalgia, (5) reflex sympathetic dystrophy, and (6) vascular insufficiency of the upper extremity. Except for hyperhidrosis, all of the other indications require the usual diagnostic techniques, including cervical sympathetic blockade to assess whether the symptoms are relieved by temporary blockade of the sympathetic ganglia. In 326 patients, sympathectomy, performed either alone or in conjunction with first-rib removal for relief of the thoracic outlet syndrome, has been successful. In only 6 patients has sympathetic activity recurred in less than 6 months. Initially all of them were treated conservatively. Three of the 6 required a repeat sympathectomy. Postsympathectomy neuralgia occurred in only 2 of more than 326 patients. Both cases were managed successfully in a conservative fashion. Among the patients in whom a Horner's syndrome was not deliberately induced, the syndrome developed in 2. In both, the syndrome resolved spontaneously within several months.

New approaches to Pancoast and chest wall tumors.

Superior pulmonary sulcus carcinoma, or Pancoast's tumor, was first identified in 1932 by H. K. Pancoast, who described a small tumor at the apex of the lung producing a characteristic pain pattern and rapid, universal mortality. Despite early indications that this type of carcinoma was amenable to neither radiation nor surgery, the current treatment approach calls for irradiation therapy (3,000 rad over 2 to 3 weeks) followed by surgical resection of the chest wall, lower brachial plexus, and en bloc resection of the lung. In selected patients with negative mediastinal nodes, this approach has been associated with a 34% 5-year and a 29% 10-year survival.

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.

Superior pulmonary sulcus carcinoma.

Carcinomas in the superior pulmonary sulcus produce "Pancoast's syndrome," involving pain in the shoulder and the ulnar distribution of the arm and hand, as well as Horner's syndrome. Careful evaluation and appropriate staging select the patients for preoperative irradiation (3000 rads) over a two- or three-week period. After a two- to four-week interlude, surgical resection of the chest wall, lower brachial plexus, and lung en bloc produces a five-year survival rate of over 30 per cent. Contraindications to this therapeutic approach include an excessive extension of the tumor into the neck or vertebrae, substantial mediastinal node metastases, and peripheral tumor dissemination.