Brief clinical reports Deep venous thrombosis complicating a congenital absence of the inferior vena cava Nikhil L. Shah, BS, Charles J. Shanley, MD, Martin R. Prince, MD, PhD, and Thomas W. Wakefield, MD, Ann Arbor, Mich.
From the Section of Vascular Surgery, Depmement of Surgery, and the Department of Radiology, University of Michigan Medical Center, Ann Arbor, Mich.
CONGENITALANOMALIESo f the i n f e r i o r v e n a cava (IVC), such as absence o r atresia, a l t h o u g h well d o c u m e n t e d , are u n c o m m o n a n d result f r o m a b e r r a n t d e v e l o p m e n t d u r i n g embryogenesis. A b s e n c e or atresia o f the 1VC usually is discovered incidentally. Patients are typically asymptomatic o f the c o n d i t i o n itself b u t s y m p t o m a t i c o f associated c o n d i t i o n s s u c h as c o n g e n i t a l h e a r t defects, polysplenia, a n d inversion o f bowel viscera. 1, 2 M a n y previously r e p o r t e d cases were d i a g n o s e d with c o n t r a s t e n h a n c e d c o m p u t e d t o m o g r a p h y (CT) a n d p h l e b o g r a phy. H o w e v e r , with t h e a d v e n t o f m o d e r n i m a g i n g t e c h n i q u e s such as m a g n e t i c r e s o n a n c e i m a g i n g (MRI), these cases can be d i a g n o s e d w i t h o u t invasive p r o c e dures, i o n i z i n g radiation, o r the use o f potentially toxic contrast m e d i u m . Previous reports have d e s c r i b e d this a n o m a l y typically as " c o n g e n i t a l a b s e n c e " or " i n f r a h e patic i n t e r r u p t i o n " o f t h e WC, r e f e r r i n g to the suprar e n a l p o r t i o n o f the IVC with azygous o r h e m i a z y g o u s c o n t i n u a t i o n . We d e s c r i b e a p a t i e n t with r e c u r r e n t episodes o f d e e p v e n o u s t h r o m b o s i s (DVT) a n d c h r o n i c v e n o u s insufficiency c a u s e d by c o n g e n i t a l i n f r a r e n a l absence o f the IVC.
CASE R E P O R T A 30-year-old man presented to our institution for evaluation of chronic lower extremity venous insufficiency and leg ulceration. His medical history was significant for three episodes of DVT involving the left lower extremity during the preceding 8 years and an episode of DVT of the right leg. Previous phlebographic studies (superior and inferior vena cavography, contrast CT scans of the abdomen and pelvis, left lower extremity phlebography) suggested possible absence of the Accepted for publication March 26, 1996. Reprint requests: Thomas W. Wakefield, MD, University of Michigan Medical Center, 1500 E. Medical Center Dr., 2210 THCC, Ann Arbor, MI 48109-0329. Surgery 1996;120:891-6. Copyright 9 1996 by Mosby-Year Book, Inc. 0039-6060/96/$5.00 + 0 11/57/73860
IVC with azygous continuation and the presence of extensive venous collateral vessels. The results of blood coagulation studies, including levels of anfiphospholipid antibodies, protein C and S, and antithrombin III, were normal. The results of physical examination were essentially within normal limits except for signs of chronic venous insufficiency of the lower extremities bilaterally (more severe on the left) and a 2 cm superficial left medial malleolar ulcer. In addition, prominent, dilated, tortuous superficial vessels on the lower abdominal wall and pelvis were noted. The patient was taking coumadin on a long-term basis for treatment of his episodes of DVT, but the coumadin level was subtherapeutic at the time of presentation. An MR[ scan of the chest and abdomen showed a large, dilated, azygous vein with slow flow that emptied into a normal appearing superior vena cava. Just below the level of the diaphragm, a tiny infiahepatic IVC was seen with slow flow and a markedly enlarged azygous vein (Fig. 1, A and B). Numerous retroperitoneal collateral veins below the level of the renal veins were noted. The infrarenal IVC and left iliac vein were not visualized; only T1 bright fat was noted in the expected location of the WC (Fig. 1 C through F). The profunda femoral vein was not detected on the left side, and we noted evidence of previous thrombosis with scarring and recanalization inside the left common femoral and superficial femoral veins (Fig. 2, A). Reduced flow and caliber in the main deep venous system were detected down to the left knee (Fig. 2, B). Large subcutaneous collateral vessels were noted over the medial aspect of the left thigh. The right iliac vein was present and continued into the right femoral system with no evidence of scarring. Additional findings included normal flow in the hepatic and portal veins. The renal veins were small, and a circumaortic left renal vein was visualized. No congenital defects of the heart or abdominal viscera were seen. Treatment consisted of stocking support, leg elevation, exercise, and a venous pump. The patient continues to take coumadin for his multiple episodes of DVT, with intensification of his regimen to keep the international normalized ratio (INR) between 2 and 3.
DISCUSSION T h e e m b r y o g e n e s i s o f the v e n o u s system occurs b e t w e e n the sixth a n d e i g h t h gestational weeks. T h e SURGERY
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Fig. 1. MRI scans including axial T1 weighted spin echo images (A, C, E) and axial two-dimensional time of flight images of abdomen show absence of infrarenal IVC and multiple collateral vessels. Just below level of diaphragm (A, B) there is tiny infrahepatic IVC (arrow) and markedly enlarged azygous vein (AV). Aorta (AO) is normal. At level of renal hila (C, D) and below kidneys (E, F), only T1 bright fat is seen at expected location of 1VC (arrows). Numerous paralumbar, retorperitoneal, and subcutaneous collateral vessels (CV) are present.
venous system develops from three pairs of primitive venous channels. 2, ~ The posterior cardinal veins appear first, coming from the dorsal aspect of the embryo. This system almost completely regresses to form only the iliac bifurcation and does not contribute to any portion of the adult IVC. The subcardinal veins develop next,
and the cranial portion of the right subcardinal vein undergoes anastamosis with the right hepatic vein (resulting from a coalition of hepatic sinusoids) forming the prerenal (or suprarenal) segment of the IVC. Failure of this anastamosis to occur results in the classically reported absent 1VC or infrahepatic interruption. Just
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Fig. 2. Axial two-dimensional time of flight images just below inguinal ligament (A) and in mid-thigh (B) show evidence of chronic deep venous thrombotic changes involving the left common femoral and superficial femoral veins (arrows). These veins are reduced in caliber compared with normal veins on right side. Femoral arteries (i~)t) and greater saphenous veins (SV) are normal bilaterally. A large subcutaneous collateral vessel (CV) is present in left groin. caudal to this section, the right and left subcardinal systems anastamose to form the left renal vein, after which point the left system regresses. The final pair of venous channels, the supracardinal veins, develop dorsal to the subcardinal veins and medial to the posterior cardinal veins. An anastamosis between the supracardinal veins and the subcardinal veins forms the renal segment of the IVC. The caudal aspect of the right supracardinal system becomes the postrenal (or infrarenal) segment of the 1VC, and the cranial aspect develops into the azygous vein. Again, the left system regresses, yielding a unilateral right-sided system composing the adult 1VC. 3 In our patient we hypothesized that failure of development of the caudal aspect of the right supracardinal vein most likely resulted in his congenital abnormality. The p h e n o m e n o n of absence of the IVC has been described in a variety of ways such as absence, agenesis, anomalous, and interruption of a particular segment ([infra] hepatic, prerenal, renal, or infrarenal) of the IVC. We reviewed 80 patients reported in the Englishlanguage literature from 1961 to the present (not
including the current patient) who had various types of 1VC absence. 553 In 1961, Anderson et al. 1 reviewed 41 cases of infrahepatic absence of tile IVC associated primarily with congenital cyanotic heart disease (Table). An association with congenital anomalies of the abdominal viscera such as polysplenia, asplenia, and sims inversus has been established. 2-5 We chose to group all congenital absences that occurred above the renal veins as suprarenal because considerable ambiguity exists in the literature concenaing distinguishing between infrahepatic and prerenal absences. Approximately 90% of previously reported cases involved absence of the suprarenal portion of the IVC, whereas a renal or infrarenal absence was reported in only five cases (6%). Furthermore, only the cases involving suprarenal absence were reported to be associated with congenital heart or abdominal defects. Unlike Anderson et al., we found that a greater number of adult patients in the current review were diagnosed with IVC interruption and absence. In 25% of cases the patients were asymptomatic, and a mediastinal
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T a b l e . Review of patients previously r e p o r t e d with absence or interruption of IVC
1VC anomaly* Suprarenal absence with continuation + Complete absence with continuation Renal absence with continuation Infrarenal absence with continuationw Totalw
Mean age (yr)
Symptomatic of venous anomaly (%)
Associated congenital heart or abdominal defects (%)
Patients reported (male.female)
*Does not specify type of continuation responsible for venous return in association with each anomaly. ~Refers to symptoms occurring directly as result of absence and not any other associated congenital defects or conditions. ~tRef~rs to both infi~ahepatic and prerenal absence of the IVC. w patient in current report.
mass or hilar density on a routine chest x-ray film suggested the diagnosis. Additional radiographic testing revealed a dilated vein, typically the azygous or hemiazygous, which a c c o m p a n i e d the tVC abnormality. In addition, continuations of the perivertebral venous plexus, l u m b a r vein, hepatic vein, a n d portal vein have been reported.7, 9,19, 4548 In five patients a complete absence involved all four segments of the IVC. O n review, however, only three of these patients showed a true complete congenital absence. 4547 Moreover, three o f these five patients, as well as two patients r e p o r t e d with infrarenal absence, displayed pelvic a n d a b d o m i n a l varicosities, which also were present in o u r patient. Only a small percentage of patients p r e s e n t e d with symptoms resulting from their venous anomaly. Only one r e p o r t e d case has involved surgical reconstruction to treat the anomalous IVC. 5a Typically, however, when surgery has b e e n performed, it has b e e n used to treat an associated congenital defect, usually cardiac in nature, that was life threatening to the patient. T h r e e previous reports used M R / i n addition to o t h e r radiographic procedures, a n d in almost every case m o r e than one imaging modality was used to make a definitive diagnosis. In our patient we used MRI to d o c u m e n t a n d diagnose the venous anomaly. T h e advantages of MRI in this setting include the ability n o t only to visualize the venous circulation without the use o f potentially toxic contrast agents b u t also to visualize other associated soft tissue or congenital anomalies. In addition, abnormalities within the veins, such as the postthrombotic scarring n o t e d in our patient, may be readily identified. We do not believe the infi-arenal absence was acq u i r e d o r caused by a t h r o m b o t i c event. In this case the pattern of venous collateral vessels and the total absence o f IVC signal up to the level o f the renal veins suggest congenital absence of the infrarenal IVC. We also f o u n d evidence of phlebitis involving the left lower extremity
a n d the left iliofemoral system. O u r patient d i d n o t have any associated congenital heart or a b d o m i n a l defects, o t h e r than a circumaortic left renal vein, which is the most fl'equent major venous anomaly occurring in 1.5% to 8.7% of individuals. 52 Unlike most o t h e r r e p o r t e d cases oflVC interruption in which patients were asymptomatic o f the venous defect, our patient was symptomatic, experiencing four episodes of DVT a n d chronic lower extremity venous insufficiency bilaterally for m o r e than 8 years, as well as ulceration o f his left lower extremity for 5 years. Moreover, three of the four episodes of DVT occurred solely on the left side. It is likely that the episodes o f thrombosis e x p e r i e n c e d by the patient were exacerbated by venous stasis caused by his absent 1VC and compression of the left iliac vein by the right iliac artery. We believe it was the d e v e l o p m e n t of scarring in the left iliac a n d femoral systems subsequent to the three episodes o f DVT that led to the loss o f his crucial venous compensatory mechanisms, which ultimately led to the sequelae of chronic venous insufficiency. Surgical therapy, reconstructive or otherwise, could n o t be offered because o f the lack of the d e e p venous system in his left lower extremity and, consequently, the lack of adequate inflow for bypass. Reconstruction o f the IVC for this anomaly recently has been r e p o r t e d y a n d the long-term benefit o f such intervention r e m a i n s to be seen. We i m p l e m e n t e d conservative, nonsurgical therapy consisting o f a venous p u m p in addition to stocking support, leg elevation, exercise for the left lower extremity, a n d continuation of c o u m a d i n on a long-term basis. With this p r o g r a m the patient has exp e r i e n c e d some relief o f his chronic venous insufficiency a n d stabilization o f his venous stasis ulcer. In future follow-up visits MRI will continue to serve as a reliable and safe means for reevaluation of the venous anomaly o f this patient.
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In summary, we report a case of a symptomatic patient with congenitaI absence of his IVC below the level of the renal veins with extensive superficial venous collaterali z a t i o n , r e c u r r e n t D V T o f h i s left l o w e r e x t r e m i t y , a n d c h r o n i c v e n o u s i n s u f f i c i e n c y w i t h stasis c h a n g e s a n d venous ulceration.
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