Superior vena cava syndrome: a case report
M. Nečas, V.Vašků
Dept. of Dermatovenereology, Masaryk University Faculty of Medicine, and St. Anne’s Faculty
Hospital in Brno
CZECH REPUBLIC

Personal history:
uPatient: V. S. , born in 1943
uPH: CHOPD
uMedication: negative
uAllergies: negative
usmokes 20 cigarettes daily

Course of the disease
usent to the emergency department  of  DVC
u    by his GP because of  4 days lasting swelling
 of the face as susp. allergic angioedema,
  u nothing unusual done, did not take any medication, alimentary history without anything
suspicious, admitted to hospital

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Differential diagnosis
uAcute angioedema (Quincke) allergic/nonallergic
uAllergic contact dermatitis/toxic contact dermatitis
uCardial swelling
uNephrogenic swelling
uLymphoedema
uAbscess,  cellulitis
uTrichinosis (trichinellosis), Trypanosomiasis
uHeliotropic swelling - dermatomyositis
uSuperior vena cava syndrome
uCushing syndrome
uThyroid disease - myxoedema
u
u

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Further investigation
uSR 34/62   IgE 359 IU/ml
uPSA 6,15 ug/l
u
uChest X-ray: pulmonary parenchyma without any  clear infiltration or deposits.
Sizeable extension of the mediastinum to the right-hand side by a mass measuring 10 x 4 cm causing
a deviation of trachea to the left.
u    The tip of the right lung with pleural thickening. Diaphragm normal. Heart shadow unextended




Further investigation and course:
uPneumology Consilium
u Syndroma venae cavae superioris with acute course, TU of the mediastinum susp.
Patient transferred to pulmonary clinic  u   at FUHB, bronchoscopy with histology:
Small cell carcinoma of the upper lobe of the right lung

Treatment
uI. Course od CHT:
       4 cycles cbdc (carboplatine,
       cyclophosphamide) according to AUC 5  u           and ifosfamide
    + Concomitant RT of the chest (30 Gy)
recurrence of the sy VCS due to the progression of the underlying disease
II. Course of  CHT:
      cisplatine, doxorubicine
 due to significant toxicity untimely terminated
 symptomatic treatment

Further course
u significant swelling, shortness of breath,
     cough disturbing sleep
  admitted to hospital at pulmonary clinic
     symptomatic treatment with bronchodilators, antiedematous medication, analgesics, antibiotics
due to elevated CRP u
exitus letalis: in bed rest  with symptoms of terminal bronchopneumonia
uDEATH within 10 month of the dg of SVC sy

Superior vena cava syndrome
u
ufirst described by W. Hunter in the year 1757
caused by poor blood flow through SVC
into the right atrium of the heart caused by:
u               - Extravascular pressure
                (tumor, enlarged lymph. nodes)
u              - Intraluminal problem
u                                (thrombosis, tumor)

Superior vena cava syndrome
uCauses:  70% maligant
u
u bronchogenic carcinoma (cca 70%)
u lymphomas (mostly NHL)  (cca 10%)
u metastases into mediastinal LN (cca 10%)
u     (kidney tu, testical, ovarial tu, breast ca)
u primary mediastinal tu (thymoma 2%)
uother
u
u

Superior vena cava syndrome
uCauses:  30% benign
u
u benign tumors (thymoma, teratoma  …)
u retrosternal struma
u aortal aneurysm, pericarditis
u thrombosis (catheter in SVC, electrode of PM)
u septic thrombosis, thromboflebitis
u postiradiation fibrosis,  fibrosing mediastinitis
u TB, syphilis, sarcoidosis

Clinical presentation
u
uSwelling of the head and neck (and arms)
uCyanosis, plethora
uDilated subcutaneous veins
uCough, dyspnea, stridor, hoarseness
uHeadache, vertigo, confusion
uNasal congestion, epistaxis
uSyncope
u
u
u

               Diagnostics
u chest X-ray
u         when susp. bronchogenic ca
u            - bronchoscopy
u              - transparietal punction under CT control
u              - mediastinoscopy
u            - videothoracoscopy, thoracotomy
uCT or angio CT
uNMR, PET
uinvasive contrast venography
utransoesophageal sonography

                  Treatment
ucausative treatment: depends on etiology of SVCS
u        - surgery
u        - RT
u        - chemotherapy
u        - thrombolysis
uapplication of a stent into SVC
usymptomatic treatment:
u         - elevation of the head, oxygenotherapy
u         - corticosteroids: reduction of the swelling aroud
u                                             tumor
u         - diuretics
u

Conclusion
u   SVC syndrome should be considered in
every case of swelling of the head and neck without any apparent cause , especially when associated
with dilatation of veins of the neck and collateral venous circulation or  with other symptoms
Dermatologist should provide diagnosis and treatment in a specialized center

u
u
u      Thank you for your attention