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UNUSUAL PRESENTATION OF
SUPERIOR VENA CAVA SYNDROME
Resident(s): Lauren Evans, MD
Attending(s): Steve Ruiz, MD
Program/Dept(s): Scott & White Memorial Hospital, Temple, TX
CHIEF COMPLAINT & HPI
 26 year old African American female with PMH significant for SLE
and ESRD who was transferred from outside facility for
management of acute hypoxic respiratory failure associated with
subepiglottic narrowing.
 On arrival, she was emergently nasally intubated by ENT due to
impending airway compromise from what appeared to be acute
bacterial epiglottitis.
HPI
▪She was also noted to have diffuse edema of the face, lips, gingiva, and neck which was
then presumed due to angioedema. The patient was treated with solumedrol,
plasmaphersis x 7 days, 2 doses of a C1 inhibitor, and Ecallantide® (Kalbitor), a Kallikrein
inhibitor. The patient failed to respond to antibiotic and steroid therapy.
▪The patient also experienced severe epistaxis which required transfusion of 7 units of
pRBCs. An epistaxis embolization procedure also failed to achieve hemostasis.
▪When symptoms of angioedema failed to respond adequately to these measures,
rheumatology and hematology/oncology were consulted for further investigation.
▪It had been previously noted during an attempted tunneled right IJ PICC placement, that
the patient’s SVC and other named veins within the thorax were occluded with collateral
formation.
RELEVENT HISTORY
▪Past Medical History
▪Systemic Lupus Erythematosus and End Stage Renal Disease secondary to lupus nephritis
▪Past Surgical History
▪Left upper extremity dialysis fistula-chronically thrombosed and not in use
▪Right subclavian dialysis catheter
▪Family & Social History
▪Recent sick contact (young child with presumed viral URI)
▪Review of Systems
▪Shortness of breath, cough, subjective fevers
▪Medications
▪Plaquenil and prednisone
▪Allergies
▪Iodinated Contrast
DIAGNOSTIC WORKUP
▪Physical Exam on admission
▪Temp: 98.1, BMI 21.7
▪Inspiratory and expiratory stridor with bilateral wheezing
▪Extensive soft tissue swelling of the head, face, neck, and upper chest
▪Laboratory Data
▪WBC 4.7, Hemoglobin 8.6 g/dL, Hematocrit 27.6%, C3 level of 39 (low), and C4 level of 8
(low), double stranded DNA antibodies were positive
DIAGNOSTIC WORKUP
▪Non-Invasive Imaging
▪Hospital Day 1: Soft Tissue Neck Radiograph
performed at outside facility shows marked
narrowing of the trachea at the glottis
(arrow). Lateral image hints at the diffuse
soft tissue swelling.
DIAGNOSTIC WORKUP
▪Hospital Day 9: CT Soft Tissue Neck for neck swelling shows diffuse, extensive edema and
swelling of all imaged subcutaneous soft tissues of the head, face, neck, and upper chest.
Edema extends into the deep soft tissues of the neck.
DIAGNOSTIC WORKUP
▪During attempted placement of a tunneled right
IJ PICC, it was noted that the SVC and all named
veins in the chest were occluded. Opacification
of collateral veins was demonstrated.
▪During epistaxis
embolization, it was
noted that the right IJ
was patent to the level
of brachiocephalic with
minimal residual lumen
(arrow).
DIAGNOSIS
▪Superior Vena Cava Syndrome
▪As mentioned in the HPI, this was initially diagnosed as bacterial epiglottitis and
then diagnosed as angioedema. All treatments failed to achieve significant
response.
INTERVENTION
▪Venogram and Treatment of SVC Syndrome
▪The patient was referred for recannalization
treatment. Catheter venogram of the right IJ
demonstrated chronic occlusion extending
from the lower neck to the clavicle with
opacification of numerous venous
collaterals in the lower neck and chest wall.
▪A fibrin sheath surrounding the right
subclavian hemodialysis catheter left a small
residual patent lumen in the SVC. From this
approach, multiple attempts were made to
reconnect the occluded portion of the right
IJ to the residual patent lumen of the right
brachiocephalic via a sheath and snare
placed in the right subclavian.
▪Ultimately, using sharp recannalization of a
22 gauge 20cm needle, the chronically
occluded portions of the right IJ and
brachiocephalic veins were snared and
bridged to the right subclavian.
▪Balloon angioplasty of the right IJ,
brachiocephalic, and SVC was performed.
INTERVENTION (CONT.)
▪Following balloon angioplasty, a
20mm Gianturco tracheobronchial
Z stent was placed in the SVC.
▪Simultaneous placement of 12 mm
Wallstents in the right IJ and
brachiocephalic/ subclavian veins
was performed. Stents were
brought to profile by balloon
angioplasty. The central aspect of
both stents were within the
Gianturco SVC stent.
▪The right IJ stent successfully
extended across the previously
occluded segment.
▪Subsequent venography
demonstrated wide patency of all 3
stents with no further opacification
of the previously noted collaterals.
A right subclavian hemodialysis
catheter was replaced.
QUESTION SLIDE
1) Which of the following is NOT a reason to perform therapeutic intervention on
someone with SVC syndrome?
A: Acute respiratory failure
B: Mental status changes
C: Symptomatic improvement after antineoplastic therapy
D: Persistent head, neck, and upper extremity soft tissue swelling
CORRECT!
1) Which of the following is NOT a reason to perform therapeutic intervention on someone
with SVC syndrome?
A: Acute respiratory failure. This is a reason for urgent intervention. Venous hypertension is
causing soft tissue edema which may be life-threatening.
B: Mental status changes. This is another reason for urgent intervention. Venous
hypertension is causing cerebral edema which may be life-threatening.
C: Symptomatic improvement after antineoplastic therapy. Current antineoplastic therapy
can be continued if the patient’s symptoms have significantly improved or resolved.
D: Persistent head, neck, and upper extremity soft tissue swelling. Persistent symptoms
despite maximal medical therapy is an indication for intervention and improves the patient’s
quality of life.
CONTINUE WITH CASE
SORRY, THAT’S INCORRECT.
1) Which of the following is NOT a reason to perform therapeutic intervention on someone
with SVC syndrome?
A: Acute respiratory failure. This is a reason for urgent intervention. Venous hypertension is
causing soft tissue edema which may be life-threatening.
B: Mental status changes. This is another reason for urgent intervention. Venous
hypertension is causing cerebral edema which may be life-threatening.
C: Symptomatic improvement after antineoplastic therapy. Current antineoplastic therapy
can be continued if the patient’s symptoms have significantly improved or resolved.
D: Persistent head, neck, and upper extremity soft tissue swelling. Persistent symptoms
despite maximal medical therapy is an indication for intervention and improves the patient’s
quality of life.
CONTINUE WITH CASE
CLINICAL FOLLOW UP
▪Because of the patient’s
contrast allergy and resolution
of symptomatology, no direct
imaging follow up has been
performed. The patient remains
anticoagulated with Coumadin.
▪Pre- and post-procedural chest
radiographs demonstrate much
improved soft tissue swelling.
Pre-procedure
1 month
Post-procedure
SUMMARY & TEACHING POINTS
▪SVC syndrome can have a confusing presentation with many overlapping symptoms with other diseases1-4.
▪The patient’s clinical presentation was probably caused by acute on chronic venous obstruction secondary to
hypercoagulability from active lupus (positive ds-DNA antibodies) and the long standing presence of a dialysis
catheter5, 6.
▪Diagnosis was confounded by the recent sick contact in the setting of acute hypoxic respiratory failure.
Diagnosis was further delayed because her clinical presentation was then presumed to represent complement
mediated angioedema secondary to active lupus7.
▪The unsuccessful treatment of her epistaxis by embolization also likely reflected the ongoing venous
hypertension6, 8.
▪Today, SVC syndrome is usually (~70%) seen in the setting of an obstructing neoplasm, though benign causes
(~30%) are occasionally noted8-10.
▪Before antibiotics were widely available, this ratio was essentially reversed. Many cases were caused by
syphilitic aortic aneurysms or an infectious mediastinitis.
▪SVC syndrome was initially described by William Hunter in 1757. Hunter was an Scottish physician and
anatomist who described a case of a 39 yo man with SVC syndrome secondary to a syphilitic aortic aneurysm11.
▪More recent cases seem to suggest that an increasing incidence of SVC syndrome is caused by indwelling
catheters1, 5, 6, 8.
REFERENCES
1.
Chen JC, Bongard F, Klein SR. A contemporary perspective on superior vena cava syndrome. Am J Surg. Aug 1990;160(2):207-11.
2.
Lanciego C, Pangua C, Chacon JI, et al. Endovascular stenting as the first step in the overall management of malignant superior vena cava syndrome.
AJR Am J Roentgenol. Aug 2009;193(2):549-58.
3.
Abner A. Approach to the patient who presents with superior vena cava obstruction. Chest. Apr 1993;103(4 Suppl):394S-397S.
4.
Baker GL, Barnes HJ. Superior vena cava syndrome: etiology, diagnosis, and treatment. Am J Crit Care. Jul 1992;1(1):54-64.
5.
Madan AK, Allmon JC, Harding M, et al. Dialysis access-induced superior vena cava syndrome. Am Surg. Oct 2002;68(10):904-
6.
Warwicker, P and Gopaluni, S. Superior vena cava obstruction presenting with epistaxis, haemoptysis and gastro-intestinal haemorrhage in two
men receiving haemodialysis with central venous catheters: two case reports. Journal of Medical Case Reports. May 2009, 3:6180,
7.
Megerian, C. A., Arnold, J. E. and Berger, M., Angioedema: 5 Years' experience, with a review of the disorder's presentation and treatment. The
Laryngoscope 1992, 102: 256–260.
8.
Cheng, S. Superior vena cava syndrome: a contemporary review of a historic disease. Cardiol Rev. 2009 Jan-Feb;17(1):16-23.
9.
Queen JR, Berlin J. Superior vena cava syndrome. J Emerg Med. Aug 2001;21(2):189-91.
10.
Wan JF, Bezjak A. Superior vena cava syndrome. Emerg Med Clin North Am. May 2009;27(2):243-55.
11.
Hunter W. History of aneurysm of the aorta with some remarks on aneurysm in general. Med Obser Inq. 1757;1:323–357.