Dermatology Online Journal

Cutaneous findings leading to a diagnosis of superior vena cava syndrome: A case report and review of the literature
Mondhipa Ratnarathorn MD¹, Errol Craig MD PhD²
Dermatology Online Journal 17 (6): 4

1. University of California at Davis, Department of Dermatology, Sacramento, California
2. Associate Physician, Kaiser Permanente Walnut Creek California and Volunteer Clinical Faculty, UC Davis, Department of Dermatology, Sacramento, California

---

Abstract

Superior vena cava syndrome, which occurs in approximately 15,000 persons in the United States annually, consists of a collection of symptoms and signs resulting from the obstruction of the superior vena cava (SVC). An early and prominent symptom of this condition is a constellation of superficial, dilated, vertically oriented and tortuous cutaneous venules or veins above the ribcage margins, often presenting as a blanchable violaceous eruption. Herein, we report a case of superior vena cava syndrome diagnosed in our dermatology clinic.

---

Introduction

Superior vena cava syndrome, which occurs in approximately 15,000 persons in the United States annually [1], consists of a collection of symptoms and signs resulting from the obstruction of the superior vena cava. Originally described in 1757 by William Hunter in a patient afflicted with a saccular aneurysm of the ascending aorta secondary to syphilis [2], this condition is characterized by compromised blood flow in the vena cava because of extrinsic compression or intraluminal occlusion. This leads to elevated venous pressure and increased blood flow through collateral vessels.

Common presenting complaints of patients with superior vena cava syndrome include nausea, vomiting, fever, chills, headache, dyspnea, neck swelling, decreased appetite, and fatigue. An early and prominent symptom of this condition is a constellation of superficial, dilated, vertically oriented and tortuous cutaneous venules or veins above the ribcage margins. Further examination of the patient often reveals suffusion, cyanosis, and edema of the head, tongue, oral and nasal mucosa, neck, chest, and upper abdomen owing to venous congestion in the upper body. Proptosis, periorbital swelling, conjunctival suffusion and elevated intraocular pressure are common ophthalmic findings in superior vena cava syndrome [3].

Physical exam usually establishes the presence of superior vena cava syndrome. The condition is usually associated with external compression from intrathoracic masses, most commonly lung cancers. Additional known masses of the middle or right anterior mediastinum causing superior vena cava syndrome include enlarged paratracheal lymph nodes, lymphoma, leiomyosarcomas, carcinoids, germ cell tumors, fibrosing mediastinitis, intrathoracic goiter, thymoma, and aortic aneurysm. Cases of other less common etiologies: pulmonary amoebiasis, antiphospholipid syndrome, chronic lymphocytic leukemia, thoracic actinomycosis, invasive epidermoid carcinoma, cutaneous polyarteritis, and merkel cell carcinoma [4-10] have also been reported. Iatrogenic thrombosis associated with central venous catheters is the most frequent cause of intraluminal occlusion of the superior vena cava. Primary or metastatic cancer presenting as an obstructive mass within the SVC is rare [11].

Timely diagnosis of superior vena cava syndrome and treatment of the underlying disease are critical, for increased cervical venous pressure can compromise the larynx and pharynx. This occurrence can subsequently cause cough, hoarseness, dyspnea, stridor, dysphagia, and life-threatening respiratory distress requiring intubation. Furthermore, severe cerebral edema producing headaches and confusion can result in a comatose state. In the outpatient setting, recognizing the early cutaneous presentation of superior vena cava syndrome requires a high index of suspicion.

Case report

Figure 1	Figure 2

Figure 3

A 77-year-old male presented with a one week history of “rash” on his chest, and several weeks of swelling under the eyes. He also complained of mild shortness of breath. On exam he displayed prominent non-pitting infraorbital edema (Figure 1), as well as jagged, blanching, violaceous plaques on the upper chest and abdomen (Figure 2), which were interpreted as dilated cutaneous vessels. He had no other notable physical findings. He denied current or prior alcohol use, but admitting to an extensive smoking history. The combination of the subacute infraorbital swelling and vascular venous congestion suggested superior vena cava syndrome. A chest X-ray was performed which revealed a large right hilar mass (Figure 3). A CT scan confirmed the presence of a 7 cm by 11 cm right upper lobe mass with moderate to severe compression of the superior vena cava. The patient subsequently underwent a bronchoscopic biopsy that revealed malignant carcinoma, with a suspicion for small cell carcinoma. Immunohistochemistry was attempted to further investigate this latter possibility. However, because of the scantness of tissue a definite diagnosis could not be reached and the patient declined additional efforts to characterize the tumor as well as conventional chemotherapy and radiation.

Over the next two months he developed shortness of breath, lost 15 pounds, and was placed in hospice care. He was treated with home oxygen and decadron, which helped reduce his facial swelling. After two additional months he developed bilateral forearm swelling and an ultrasound revealed bilateral subclavian and right jugular thrombosis. He soon developed hemoptysis and dyspnea on exertion, but was steadfast in declining chemotherapy or radiation. Barely 4 months after his initial diagnosis in the dermatology department, he died in his sleep.

Conclusion

Our patient presented to the dermatology clinic for a rash characterized by irregularly demarcated purpuric plaques, randomly distributed on his torso. His history of dyspnea and extensive smoking, along with the physical exam findings of superficially dilated cutaneous venules and infraorbital edema, strongly suggested a diagnosis of superior vena cava syndrome. Because malignancy affecting the thorax and mediastinum is the most common cause of SVC syndrome, our patient was promptly and carefully evaluated for this condition. Survival in patients with SVC syndrome depends upon its etiology. Life expectancy is unchanged in benign cases. However, if SVC syndrome is secondary to a cancer, patient survival is correlated with the course of the underlying disease. Clinical observation demonstrates that approximately 10 percent of patients with a bronchogenic carcinoma treated with irradiation live at least 30 months, whereas those untreated survive only about 30 days.

References

1. Wilson LD, Detterbeck FC, Yahalom J. Clinical practice. Superior vena cava syndrome with malignant causes. N Engl J Med. 2007 May 3;356(18):1862-9. [PubMed]

2. Wudel LJ Jr, Nesbitt JC. Superior vena cava syndrome. Curr Treat Options Oncol. 2001 Feb;2(1):77-91. [PubMed]

3. Saeed AI, Schwartz AP, Limsukon A. Superior vena cava syndrome (SVC syndrome): a rare cause of conjunctival suffusion. Mt Sinai J Med. 2006 Dec;73(8):1082-5. [PubMed]

4. Lichtenstein A, Kondo AT, Visvesvara GS, Fernandez A, Paiva EF, Mauad T, Dolhnikoff M, Martins MA. Pulmonary amoebiasis presenting as superior vena cava syndrome. Thorax. 2005 Apr;60(4):350-2. [PubMed]

5. Ozkan M, Eser B, Er O, Altuntas F, Coskun R, Topsakal R, Mavili E, Kala M, Altinbas M. Antiphospholipid syndrome associated with malignant mesothelioma presenting with superior vena cava thrombosis: a case report. Clin Appl Thromb Hemost. 2004 Oct;10(4):393-6. [PubMed]

6. Liu DT, Fan DS, Chan WM, Choi PC, Gao L, Lam DS. Challenging problems in malignancy: case 3. Chronic lymphocytic leukemia with ocular complications and superior vena cava obstruction. J Clin Oncol. 2004 Sep 15;22(18):3830-2. [PubMed]

7. Kotoulas C, Foroulis C, Letsas K, Papamichalis G, Lioulias A. Thoracic actinomycosis as a cause of superior vena cava syndrome: report of a case. Surg Today. 2004;34(5):447-9. [PubMed]

8. Halkic N, Henchoz L, Gintzburger D, Nordback P, Ksontini R, Boumghar M. Superior vena cava syndrome caused by invasive epidermoid carcinoma. J Cardiovasc Surg (Torino). 2000 Jun;41(3):499-501. [PubMed]

9. Lightman HI, Valderrama E, Ilowite NT. Cutaneous polyarteritis nodosa and thromboses of the superior and inferior venae cavae. J Rheumatol. 1988 Jan;15(1):113-6. [PubMed]

10. Superior vena cava obstruction from Merkel cell carcinoma. Arch Dermatol. 1987 Nov;123(11):1433. [PubMed]

11. Chan SL, Arifi AA, Chan AB, Chan AT, Ma BB. An unusual cause of superior vena cava obstruction. Thorax. 2006 Feb;61(2):182. [PubMed]

© 2011 Dermatology Online Journal