24 - ijcci.info

Case
e Report
Dupllication of Inferior Vena
V
Cava
a with Pulm
monary Th
hromboem
mbolism – A
Rare
e case repo
ort
Hem
mant Gupta
a,1 Ayaz Ahmed,2 Maya Len
nde,3 Balra
am Yadav
v,4 Tanvee
er
5
6
Halg
gale, Samk
kit Mutha.
1
2
Hon. Associate Professor, 2,3
Assistant Professor, 4 Chief Reside
ent, 5,6Senio
or Resident
Grant Medical Co
ollege and Sir
S J.J. Group of Hospita
als, Mumbaii, India
E-ma
ail: drtanve
[email protected]
.
Inter
rnational Journal
J
of Clinical Ca
ases and I
Investigatio
ons. Volum
me 6 (Issu
ue
st
2), 24:30,
2
1 January
J
2015.
Absttract
Klippel Feil syndrome (SKF)) is a rare co
ongenital dissease. It is characterized mainly b
by
the in
ncorrect union or fusion of two cervical verte brae or more. This ma
alformation is
respo
onsible for limitation of
o movemen
nt of the he
ead and a ssignificant rrisk of spina
al
cord injury. Othe
er variables anomalies may be asssociated with
h increased morbidity .
We report the
e case of a child who consulted for short stature with abnorma
al
and palmed Neck
sexua
al differenc
ciation. The
e existence of short
k associate
ed
with congenital strabismu
us oriented diagnosiis and ha
as linked a
abnormalitie
es
prese
ent to Klippel Feil syndrome
Keyw
words: Dup
plication of inferior ve
ena cava, D
Deep vein thrombosis
s, pulmonarry
throm
mboembolism
Intro
oduction:
Veno
ous thrombo
oembolism (VTE), is one
o
of the leading ca
auses of ca
ardiovascula
ar
mortality. VTE typically orriginates as
s deep ven ous thromb
bosis (DVT)) in a lowe
er
1
1
extre
emity. The incidence off DVT is estimated at 1 in 1,000 in
ndividuals/ye
ear.
nferior vena
a cava (IVC
C) is a rare
e finding in radiologic studies. Th
he
Dupliication of in
w
incide
ence is abou
ut 0.2-3%. Its symptom
matic presen
ntation is ev
ven rarer. T
There are few
reporrted cases with IVC duplication and throm
mbosis of lower extremities witth
assoc
ciated pulmonary throm
mboembolism
m.2,3,4,5,6,7
erential dia
agnosis is lymphade nopathy, a
aortic aneurysm, an
nd
Its main diffe
retroperitoneal cysts.
c
The significance
e of duplica
ation of the
e IVC is tha
at it may b
be
24 associated with the recurrence of pulmonary thromboembolism if the anatomical
variation goes undiagnosed.2
Case Report:
Our patient was a 35 year old male a chronic alcoholic since 8 years, admitted with
pain in abdomen and distension of abdomen with swelling and pain in the left lower
limb extending upto the left inguinal region. He also had pitting edema of left leg and
significant size difference between circumferences of his two legs. He had no history
of DVT, clotting disorders, peripheral vascular disease, non-healing ulcerations on
the extremities, cerebrovascular accidents. All his other physical examination was
within normal limits. Almost all routine blood investigations of this patient were
within normal limits
Colour duplex sonology of the left lower limb revealed deep vein thrombosis with
complete lumen occluding thrombus of the common femoral, superficial femoral and
deep femoral veins.
In view of his pain in abdomen and distension of abdomen a CT abdomen (plain +
contrast) was done. Findings were suggestive of Duplication of the inferior vena
cava with complete lumen occluding thrombus in the left deep femoral vein
superficial femoral vein common femoral vein, external iliac, internal iliac, common
iliac and left inferior vena cava upto its confluence with the right suprarenal IVC. Also
incidentally noted was pulmonary embolism involving right main pulmonary artery
(saddle thrombus) and lobar pulmonary artery supplying the left lower lobe.
A 2D ECHO of the patient was done which was normal and then the patient was
started on heparin. The patient developed intra-abdominal hematoma after 3 days of
size 11*14 *7cm.A triple vessel angiogram and renal angiogram was done which was
normal. The patient’s heparin was stopped and he was given Fresh frozen plasma
infusion. He was kept under observation for 12 days. The patient was monitored with
repeated Ultrasonography of the abdomen which revealed decrease in the size of the
hematoma. The patient was restarted on heparin with careful monitoring for any
bleeding. After full anticoagulation with heparin for 5 days and starting warfarin
international normalized ratio (INR) range was between 2 and 3, he was discharged
from hospital. We plan to continue anticoagulation for 6 months
Discussion:
Anatomical variation of the inferior vena cava occurs in 0.4-4% of the population
(MAYO, GRAY, LOUIS et al., 1983). The most common variant is duplication of the
inferior vena cava (ARTICO, LORENZINI, MANCINI et al., 2004). Their identification
is important in the clinical realm because it may reduce misdiagnoses. The formation
of the IVC is a complex event. It is formed between the 6th weeks and 8th weeks of
embryonic development via a series of anastomoses and regressions of the primitive
trunk including: Posterior cardinal veins, subcardinal veins, and supracardinal veins.4
During the normal embryogenesis, the left subcardinal and left supracardinal veins
regress. Failure of normal regression of any of the paired venous structures leads to
abnormal persistence, duplication, or both.5 The literature strongly suggests that the
non-regression of the left supracardinal vein during embryonic development leads to
the occurrence of a second abdominal vessel, which is generally positioned to the left
25 of the aorta in adults. As shown by Natsis, Apostolidis, Noussios et al. (2010),
however, the configuration of this supernumerary vessel and thus its embryonic
origin is quite variable. These authors have proposed that duplications of the inferior
vena cava be classified as complete or incomplete. Where complete, the most likely
etiological cause is the persistence of the left supra-subcardinal anastomosis, of the
post-subcardinal anastomosis, and probably of the intersubcardinal anastomosis,
which in turn results in the persistence of the left supracardinal vein. Such a case
may also be associated with an absence of iliac anastomosis (posterior distal
intercardinal anastomosis). In cases of incomplete duplication, in which the
supernumerary vena cava to the left is smaller and sometimes irregular, the likely
cause is inadequate regression of the supracardinal vein.
Phlebography is indicated as the gold standard for diagnosing duplication of the
IVC.[5] but due to invasiveness, other authors have recommended that the
combination of CT and ultrasound are satisfactory for an adequate diagnosis. In our
case report of IVC duplication, there is a normal IVC along the right side of the
spine.2
Few studies consider double IVC to be the cause of DVT, perhaps due to retrograde
stasis.6,7 .Venous return difficulty causes stasis and thereby increases the probability
of thrombosis.4 DVT might be associated with other routine pre-existing illnesses,
such as lower limb fractures, cancer, the use of hormonal contraceptives, prior
abdominal surgeries in elderly patients, genetic predisposition to thrombosis.
Treatment choices include observation for asymptomatic duplication, anticoagulation
therapy, placing an IVC filter below the renal veins in both the main right IVC and
the duplicated left IVC segment. We started the patient on anticoagulation for 6
months
Conclusion:
This case demonstrates the importance of IVC duplication in the presence of DVT,
the challenge of diagnosis, and the various therapeutic options available for a
symptom-causing IVC duplication as some previous studies make a point of it.2,3,4,5,6
References
1. Morris TA. Natural history of venous thromboembolism. Crit Care
Clin. 2011;27:869–84. [PubMed]
2. Milani C, Constantinou M, Berz D, Butera JN, Colvin GA. Left sided inferior
vena cava duplication and venous thromboembolism: Case report and review
of literature. J Hematol Oncol. 2008;1:24–28. [PMC free article][PubMed]
3. Ng WT, Ng SS. Double inferior vena cava: A report of three cases. Singapore
Med J. 2009;50:e211–3.[PubMed]
4. Saad KR, Saad PF, Amorim CA, Armstrong D, Soares BL, Neves PC, et al.
Duplication of the inferior vena cava: Case report and a literature review of
anatomical variation. J Morphol Sci. 2012;29:60–4.
26 5. Anne N, Pallapothu R, Holmes R, Johnson MD. Inferior vena cava duplication
and deep venous thrombosis: Case report and review of literature. Ann Vasc
Surg. 2005;19:740–3. [PubMed]
6. Gayer G, Luboshitz J, Hertz M, Zissin R, Thaler M, Lubetsky A, et al.
Congenital anomalies of the inferior vena cava revealed on CT in patients with
deep vein thrombosis. AJR Am J Roentgenol. 2003;180:729–32. [PubMed]
7. Chee YL, Culligan DJ, Watson HG. Inferior vena cava malformation as a risk
factor
for
deep
venous
thrombosis
in
the
young. Br
J
Haematol. 2001;114:878–80. [PubMed]
27 Figures
Figurre 1
28 Figurre 2
29 Figurre 3
30

Download Report