Something’s wrong here
Symptoms spell danger… but what danger?
Vol.20, No.01, March 2012

HR was a 67-year-old married man who had been a patient of mine for over 35 years. He came to the office complaining of gradual swelling in his neck. His physical exam was negative, and there were no other signs or symptoms. His alcohol intake was minimal, and he’d stopped smoking in 1998; he did cardio for about 25 minutes three times a week and was retired.

He’d had a heart attack 15 years ago, with such minimal symptoms that I nearly missed it. I remembered calling him at his job in the bowling alley, telling him to go to the hospital because he’d had a myocardial infarct.

There was nothing to palpate in his neck. Wondering if the swelling might be allergic in nature, I arranged for referral to an allergist, but there was a wait.

HR returned for a second visit a month later, as instructed, and told me he had to buy shirts with a bigger collar. Moreover, his right arm was swelling and indeed when I examined it, there was pitting edema. He also showed me some bluish spots on his anterior chest, suggesting these were a reaction to electrocardiogram pads. But on closer exam they looked like swollen veins.

Obviously, something serious was going on, but I couldn’t figure out what. I proposed sending him to emergency department, but he wasn’t keen on the idea. So we agreed he’d sit in the waiting room while I saw a few other patients, meanwhile trying to figure out what to do with him! I couldn’t come up with any more diagnostic tests except an ultrasound of his neck and a chest x-ray, so I gave him requisitions for these, and he left.

Good call

A few patients later, I had time to phone the medicine internist on call. He diagnosed this patient over the telephone, and told me I might as well send him to the ER, as he’d be ending up there, sooner or later! Both diagnosis and prognosis were to prove absolutely correct.

I tried to phone HR that night, a Friday night, but only got his answering machine. I called Saturday morning and Sunday with the same result. Finally I got his wife, and she told me they’d taken him to Emergency after hearing my Friday night message. She mentioned that she was sorry they hadn’t gotten my call as they went out for dinner Friday night, and had other activities all weekend… so he wasn’t really that sick.

He was dead within 2 months. Why?

ANSWER
Superior vena cava syndrome

Superior vena cava syndrome is obstruction of the main vein draining the upper body. It can be due to many causes; the internist I talked to suggested a likely one would be Pancoast tumour in the right upper lobe of lung. In fact, this patient had metastases in the paratracheal region, and his chest x-ray showed a small pleural effusion. The definitive test was a CT scan chest, and then, MRI neck. The patient showed evidence of adrenal lesion, which was initially thought to be a metastasis (it turned out to be a benign adenoma). Bronchoscopy yielded a diagnosis of adenocarcinoma. He continued to have signs of high-grade SVC obstruction, e.g. substantial edema of face, neck and arms. The oncologist thought there was no prospect of success with chemotherapy given the cancer cell type. HR underwent radiotherapy and was given steroids to shrink the carinal masses and to reduce edema. He was discharged home for a short time, but had to return due to confusion; subsequent MRI showed brain infarcts plus possible metastases. He developed a fever and succumbed to S. aureus bacteremia.

The specialists suspected that HR was immunosuppressed by cancer and the steroid therapy. He may have been hypoxic chiefly because of the load of his tumour and inflammatory effect of his radiation therapy.

A useful indicator of SVC syndrome is Pemberton’s sign: if the patient raises his arms, for instance to put cans in a cupboard, this puts pressure on the vein under the clavicle, and the patient faints! So if you get this history, or one of a swollen neck/face that becomes better as the day progresses, this is very suggestive of SVC syndrome.

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