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Case
Report -
A 25-year-old Female with Neck Pain
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James
E. Conti, MD
The
patient is a 25-year-old Caucasian female with a past medical
history of extensive sarcoidosis for many years, for which
she is currently treated with Methotrexate 10mg weekly and
Prednisone 1mg daily, Protein C deficiency with documented
DVTs and two episodes of pulmonary emboli, currently being
treated with Coumadin 5mg daily, and status post SA node
ablation in 1999 for tachycardia with DDD pacemaker insertion.
The patient presented to her primary care doctor, stating
that over the past three months she has been experiencing
worsening discomfort at the base of her neck that is associated
with bending over and lying down. Symptoms are also present
when the patient is laughing. She also recently noticed
that the veins in her neck are protruding and the earlier
discomfort has progressed to a feeling of pain at the base
of her neck. It has come to the point were she is sleeping
in the upright position because the discomfort has become
so noticeable. Patient also admits having discomfort in
both arms and hands, she states they have become swollen
over the past few months and they appear very “puffy”
to her. Patient denies any difficulty swallowing, or loss
of range of motion in her neck. She also denies any pain
in her upper extremities as well as loss of range of motion.
The patient denies shortness of breath or chest pain, recent
weight loss, or paroxysmal nocturnal dyspnea; orthopnea
is pain-related.
Past
Medical History / Past Surgical History
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Sarcoidosis with documented lung involvement
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Recent CT scan with contrast demonstrated significant
adenopathy, and pulmonary changes
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Status post node biopsies for diagnosis of Sarcoidosis
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Protein C deficiency with documented episodes of DVTs
and 2 episodes of pulmonary emboli
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Inappropriate sinus Tachycardia
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Status post SA node ablation 5 years ago with DDD pacemaker
insertion
Social
History / Family History
The
patient is single, lives at home with her parents, and denies
any smoking or alcohol consumption. Mother has Factor V
Leiden deficiency.
Hospital
Course
Due
to the progression of her symptoms, she presented to the
emergency room. Her examination is as follows.
Blood
pressure was 122/60 mmHg, heart rate was 76 beats per minute,
respiratory rate was 16. Temperature was normal and afebrile.
In general, the patient appeared her age, lying down comfortably,
talkative, in no acute distress.
HEENT: PERL, no erythema of throat, no lymphadenopathy.
NECK:
No initial signs of JVD, but when applying pressure over
liver, JVD was present with slow resolution of distention;
no lymphadenopathy.
CHEST/HEART:
S1,S2 were appreciated with no S3, regular rate and rhythm,
no murmurs.
ABDOMEN:
Normal bowel sounds, soft, non-tender, non-distended, no
masses.
RECTAL:
Hemoccult negative.
EXTEMITIES:
Upper extremities appear swollen, no pitting edema of upper
or lower extremities, no gross varicosities, no cyanosis,
no clubbing.
SKIN
: No rashes or lesions.
NEURO:
Alert and oriented, no obvious focal deficits.
Labs
No
electrolyte abnormalities were observed.
| WBC Count |
7.8 thousand/cmm |
| Hemoglobin |
14.0 gm/dL |
| Hematocrit |
41.4 % |
| Platelet Count |
223 thousand/uL |
| Prothrombin Time |
14.9 seconds (elevated) |
| INR |
1.5 |
| PTT |
51.7 seconds (elevated) |
A
CT scan showed significant pretrachea, precarinal, and AP
window adenopathy, and pulmonary changes with multiple nodular
densities present in the periphery of both lung fields.
No extensive adenopathy in the region of the superior vena
cava (SVC) right atrial junction. Venogram was also performed
from the left arm, which showed focal occlusion of the SVC
at the right atrial junction; no venographic evidence of
other thrombus in the area; no intraluminal filling defects
consistent with the clot. EKG showed sinus rhythm at 80
beats per minute with normal PR, QRS, and QT interval.
The
patient was then admitted to the CCU and IV thrombolytics
(TPA) were administered for 12 hours at 2mg per hour. Eptifibatide
75mg/100ml and Heparin 25,000u/500ml were also administered.
The
next morning, balloon angioplasty was successfully performed,
decreasing the SVC occlusion from 100% to 20%. The patient
was then continued on aspirin and clopidogrel. She also
remained on heparin therapy until a therapeutic INR of 2.5
to 3.5 was achieved using with oral anticoagulation with
warfarin 5mg daily. In the days following the catherization
procedure the patient began to notice a decrease in the
swelling of her upper extremities as well as the discomfort
in her neck. She was discharged on hospital day 6, with
continued use of plavix, warfarin, as well as previously
prescribed medications; she was instructed to follow up
with her cardiologist in 4 weeks.
See
SVC before balloon angioplasty. (Microsoft Windows Media
player required)
See
SVC after balloon angioplasty. (Microsoft Windows Media
Player required)
DISCUSSION
Obstruction
of blood flow in the superior vena cava results in symptoms
and signs of Superior Vena Cava (SVC) syndrome. Obstruction
can either be caused by a blockage within the vessel itself,
such as thrombosis, or from external compression of the
SVC by pathologic processes involving the right lung, lymph
nodes, and other mediastinal structures. In this patient’s
case, she had three different possible causes for SVC obstruction.
Either from manifestation of sarcoidosis, in which a granuloma
or lyphadenopathy in the region of the SVC could cause external
compression; Protein C deficiency leading to direct thrombosis
of SVC; or the indwelling central venous pacemaker lead.
When an obstruction of flow through the SVC develops, venous
collaterals begin to form. The rate of the obstruction will
determine the body’s ability to adapt in forming these
collaterals. In conditions in which the rate of obstruction
occurs more rapidly such as malignant disease, patients
may develop symptoms of SVC syndrome in weeks to months
as compared to fibrosing mediastinitis due to infection
which may take years to develop symptoms. The most commonly
reported symptom is dyspnea. Patients also frequently complain
of facial swelling or head fullness, a symptom that may
be exacerbated by bending over or lying down; cough, arm
edema, and cyanosis. The most common finding on physical
exam is venous distention in the neck and chest wall, and
facial edema.
Lung
cancer is the most common malignant cause of SVC syndrome,
followed by lymphoma; together they represent 94% of cases
of SVC syndrome. Other malignancies that metastasize to
the mediastinum can also be responsible. In both cases approximately
2-4% of patients with either lung cancer or lymphoma will
develop SVC syndrome, through external compression of the
SVC. Nonmalignant disorders account for the remaining 6-15%
of SVC syndrome. Of which fibrosing mediastinitis most commonly
due to Histoplasmosis infection comprises 50% of these cases;
other infectious causes include tuberculosis, actinomycosis,
aspergillosis, blastomycosis, and Bancroftian filariasis.
Other nonmalignant causes include nocardiosis, sclerosing
cholangitis, sarcoidosis, and post-radiation fibrosis. Thrombosis
accounts for a significant percentage of the nonmalignant
causes of SVC syndrome and is actually the fastest growing
population of new cases. This is mainly due to the increasing
demand of pacemaker insertions, in which indwelling catheters
or lines cause turbulent flow leading to thrombus formation.
Finally, patients who have hypercoagulable conditions such
as Protein C or S deficiency, Factor V Leiden deficiency
and many others are at higher risk for thrombus formation.
Diagnosis
most commonly can be made by chest x-ray, due to the fact
that the majority of cases are caused by external compression
from a solid tumor or lymph node. Other radiographic studies
used are CT scan and venography. Venography is considered
the gold standard for diagnosis of SVC syndrome. It is helpful
for showing the location and the degree of blockage; although
it has limitations on helping with identifying the primary
cause of the obstruction unless it is purely an intravascular
process. MRI can be used for patients with contrast allergies.
Treatment
is directed toward the underlying disease causing SVC syndrome.
Radiation and/or chemotherapy are effective in patients
with cancer. Malignancies that have high rates of response
to chemotherapy, will usually show rapid regression of SVC
syndrome. Thrombolytics play a role in patients who are
eligible and endovascular therapy such as intraluminal stenting
and balloon angioplasty can be used for patients with either
refractory disease or who need immediate symptomatic improvement.
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