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 Table of Contents  
Year : 2021  |  Volume : 1  |  Issue : 1  |  Page : 36-39

Inferior vena cava agenesis-associated thrombosis in a neonate

1 Department of Neonatology, Surya Children Medicare Pvt. Ltd., Mumbai, Maharashtra, India
2 Department of Radiology, Surya Children Medicare Pvt. Ltd., Mumbai, Maharashtra, India

Date of Submission19-Sep-2020
Date of Decision29-Sep-2020
Date of Acceptance03-Nov-2020
Date of Web Publication27-Feb-2021

Correspondence Address:
Dr. Javed Ahmed
Department of Neonatology, Surya Children Medicare Pvt. Ltd., Mumbai, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ipcares.ipcares_4_21

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Background: Inferior vena cava (IVC) malformations are rare developmental anomalies and are usually asymptomatic. Agenesis of IVC is an uncommon form of IVC malformation, and symptomatic neonatal presentation is unusual. We describe an unusual case of symptomatic venous thrombosis in the neonatal period after central venous cannulation, which was later found to have agenesis of IVC. Clinical Description: An extreme preterm (28 weeks) baby developed deep vein thrombosis of the femoral and external iliac vein after femoral venous catheterization and required anticoagulation therapy. Later, during the hospital stay, he was noted to have developed dilated abdominal veins over the flanks and paraspinal region, with the direction of flow below upward 2 months after the resolution of the thrombus. Management and Outcome: Ultrasonography (USG) color Doppler ruled out portal hypertension or persistence of IVC obstruction. Computed tomography (CT) venography resolved it to be the case of agenesis of the infrarenal portion of IVC. No consensus guidelines exist for neonatal management and it is usually conservative. Conclusion: IVC developmental anomalies including agenesis of IVC are very rare and are usually an asymptomatic condition, but can cause symptoms due to venous congestion in the lower limb or pressure from enlarged collaterals and rarely associated with deep venous thrombosis usually seen in young adults. Neonatal presentation is extremely rare. Diagnosis is by USG color Doppler and CT venography. Neonatal management guidelines are lacking and the risk of anticoagulation must be cautiously weighed against the risk of hemorrhage.

Keywords: Agenesis of inferior vena cava, deep vein thrombosis, giant abdominal wall varicosities, inferior vena cava malformations

How to cite this article:
Ahmed J, Balasubramanian H, Ansari V, Kabra N. Inferior vena cava agenesis-associated thrombosis in a neonate. Indian Pediatr Case Rep 2021;1:36-9

How to cite this URL:
Ahmed J, Balasubramanian H, Ansari V, Kabra N. Inferior vena cava agenesis-associated thrombosis in a neonate. Indian Pediatr Case Rep [serial online] 2021 [cited 2023 Feb 3];1:36-9. Available from: http://www.ipcares.org/text.asp?2021/1/1/36/310233

Dilated tortuous abdominal veins are a rare finding in a neonate and evoke the fear of either caput medusa (seen in portal hypertension) or inferior vena cava (IVC) obstruction among physicians. We describe an unusual case of giant dilated abdominal wall and paravertebral varicosities in a newborn with a previous history of deep vein thrombosis (DVT). IVC agenesis (AIVC) is a very rare developmental malformation, and only a handful of cases have been reported in adult literature.[1] This case report describes the development of neonatal DVT after central vein catheterization which was followed by the development of abdominal wall varicosity in the neonatal period. We also review the literature of AIVC, various clinical presentation, and therapeutic options for its management. This case report is prepared following the CARE guidelines for the case report.

  Clinical Description Top

A 28-week-old preterm baby (birth weight 1.08 kg) was transferred at 6 weeks of age for total parenteral nutrition (TPN) dependency after ileostomy for necrotizing enterocolitis (NEC). This neonate was born to a primigravida mother, of an uncomplicated antenatal course and was delivered vaginally. He required surfactant and mechanical ventilation for severe respiratory distress syndrome initially, followed by a gradual introduction of feeds. The umbilical venous line, secured at admission, was electively removed within the 1st week and was later managed with a peripheral vascular catheter as per the previous unit policy. He was started on enteral feeding (expressed breast milk and formula-mixed feeding) and reached half enteral feeding when he developed NEC on day 15 of life following which feeding was stopped. Later, he also required ileostomy for stage 2 NEC. The right femoral central venous catheterization was performed for dedicated venous access for TPN after surgery. However, the neonate developed venous congestion of the foot the very next day. Ultrasonography (USG) color Doppler confirmed it to be DVT with near-total thrombotic occlusion of the right external iliac, common femoral, and proximal superficial femoral veins. There was no family history of thrombophilia or DVT. The central line was promptly removed and he was started on low-molecular-weight heparin. His venous congestion improved gradually over the next week. Heparin was given for a total of 6 weeks. However, as he could not be established on full enteral nutrition due to high ileostomy output and was not thriving (weight 1.3 kg at 8 weeks postmenstrual age, he was referred to our neonatal intensive care unit for further care.

At admission, he was noted to have dilated veins over both flanks with the flow of blood below upward toward the superior vena cava [Figure 1]. A USG color Doppler, at our institute, showed complete resolution of the previous thrombus and normal flow in femoral, external iliac vein, and intrahepatic IVC; however, infrarenal IVC could not be visualized due to bowel gases. Fearing hidden persistent thrombus, we searched the portal vein and remaining visualized IVC which showed normal caliber and venous flow. Heparin was stopped and he was continued on TPN through a Peripherally inserted central catheter (PICC line) from the upper limb with the gradual building of enteral nutrition. During the course in the hospital, his flank veins became more prominent and tortuous, due to limited subcutaneous fat and poor nutritional status, with visible and frightening looking paraspinal collaterals. The direction of flow of blood tested clinically by Harvey's Sign was always from below upward toward the heart.
Figure 1: Giant dilated tortuous veins over the abdomen

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Management and Outcomes

As dilated veins have the direction of flow from below upward toward the heart, clinically we thought of persistent or recurrent thrombotic occlusion of the IVC. However, repeated USG color Doppler showed a complete resolution of the previous thrombus. A computed tomography (CT) venography was performed and showed nonvisualization of bilateral common iliac and infrarenal IVC. Bilateral external iliac vein continued as dilated para-vertebral veins, azygos/hemiazygos vein, consistent with the diagnosis of agenesis of infrarenal IVC (AIVC) [Figure 2]. A retrospective review of maternal antenatal scan did not show any concern for neonatal thrombosis or any other associated abnormality. Further workup for thrombophilia in the child was negative and parents were counseled about the general principle of prevention of venous thrombosis. No prophylactic anticoagulation was started for lack of its safety and paucity of efficacy in the neonatal literature. Dilated veins became less prominent after his nutrition improved and there was no reoccurrence of venous thrombosis at follow-up till 2 years of age.
Figure 2: Computed tomography scan showing the continuation of external iliac vein as paravertebral vein and azygous vein and absent common iliac and infrarenal vena cava

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  Discussion Top

IVC developmental anomalies are uncommon, present in 0.3%–2% of the general population, and are usually asymptomatic.[2] Agenesis of the suprarenal segment is called IVC interruption with azygous and hemiazygos continuation (incidence 0.6%),[2] while agenesis of the infrarenal portion is called AIVC which has an extremely rare incidence 0.0005%–1%.[1],[3] While interrupted IVC is considered to be a developmental anomaly, AIVC could also be due to intrauterine thrombosis, followed by absorption of the portion of the IVC. In AIVC, lower limb blood is shunted to the heart through collaterals involving the deep channels (retroperitoneal, para, and interspinal collaterals), portal (internal iliac to hemorrhoidal plexus), and superficial channels (abdominal wall).[2] These venous collaterals get enlarged and tortuous when there is any thrombotic obstruction of the veins, to shunt the lower limb returning blood to the heart from these alternative collaterals. These were visibly seen as giant abdominal wall [Figure 1] and paravertebral varicosities in our patient.

AIVC is usually an asymptomatic condition and by itself not a prothrombotic state. However, chronic venous stasis caused by inadequate paravertebral collaterals can lead to chronic venous hypertension and stasis in the lower limb leading to nonhealing venous ulcers, thrombosis, and embolism.[3],[4],[5] It is present in 5% of unprovoked thrombosis in young adults.[1] Other presenting complaints described in adult and pediatric literature include association with leg swelling, pain, the varicose vein of the lower extremity, abdominal pain, DVT, symptoms due to enlarged collateral compression on adjacent neural structures, and rarely hematochezia.[4] IVC abnormalities are rarely diagnosed in the newborn period and reported presentations in neonates includes an unusual course and tip position of the Peripherally inserted central catheter (PICC) on X-ray due to IVC interruption,[6] association with fetal ascites,[7] and dilated venous plexus over lower abdominal wall due to AIVC.[8] [Table 1] summarizes the commonly described presentations of neonatal and pediatric cases of IVC malformations.
Table 1: Neonatal and pediatric inferior vena cava malformations reported cases

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Symptomatic DVT in the neonatal period is a very rare presentation. Central venous catheter (especially malpositioned umbilical venous line which is a risk factor for umbilical vein thrombosis) and prolonged catheter duration >7 days is the most important reason for deep venous thrombosis in neonates. Other common neonatal risk factors such as sepsis, congenital heart disease, polycythemia, perinatal asphyxia, and maternal risk factors such as diabetes, infection, thrombophilia, and antiphospholipid syndrome, need to be evaluated for deep venous thrombosis in the neonates. Our patient did not have any of the above risk factors for lower limb DVT.

USG color Doppler is a simple and easy way to diagnose this condition but requires a high index of suspicions, CT venography is sometimes required for confirmation, and is the gold standard.[2] Interrupted IVC with azygous can be diagnosed antenatally with “Double vessel” sign on the antenatal scan.[2] As AIVC-associated thrombosis is a rare condition, no standard guidelines are available for its management.[1] The therapy aims are to prevent the progression and recurrence of thrombosis. Heparin is used widely for the prevention of progression of thrombosis. Surgical treatment is also described and may be indicated in associated acute DVT, bleeding from a venous aneurysm, or nonhealing venous ulcer, and consist of prosthetic surgical reconstruction of IVC.[4] Long-term anticoagulation prophylaxis is employed when there are other associated risk factors of thrombosis or embolism such as old age, smoking, pulmonary hypertension, recurrent thrombosis, and medication which increase the risk of thrombosis like contraceptive pills.[1],[4] Avoidance of other risk factors that may increase the risk of DVT like prolonged immobilization, certain medicines like contraceptive pills, and elastic stockings are all helpful in the prevention of recurrence of DVT as suggested by pediatric literature.[1],[4] This episode of DVT in our patient was after central venous cannulation and the chances of its reoccurrence, in absence of other associated risk factors were considered low. We, therefore, did not initiate the neonate on long-term anticoagulation therapy as the risk of potential hemorrhagic complication in developing neonate was considered much worse than the chances of reoccurrence of the DVT and its consequences. General principles of DVT prevention such as hydration, prompt infection control, and avoidance of vigorous exercise were discussed and explained to the parents.

Although extremely rare, IVC developmental anomalies should be considered in the differential diagnosis of lower limb DVT (especially if it is recurrent). Patient education about preventive measures should be instated to decrease the chances of reoccurrence of thrombosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal parents have given his consent for images and other clinical information to be reported in the journal. The parents understand that name and initial will not be published and due efforts will be made to conceal patient identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.


  References Top

Lambert M, Marboeuf P, Midulla M, et al. Inferior vena cava agenesis and deep vein thrombosis: 10 patients and review of the literature. Vasc Med 2010;15:451-9.  Back to cited text no. 1
Malaki M, Willis AP, Jones RG. Congenital anomalies of the inferior vena cava. Clin Radiol 2012;67:165-71.  Back to cited text no. 2
Gil RJ, Pérez AM, Arias JB, et al. Agenesis of the inferior vena cava associated with lower extremities and pelvic venous thrombosis. J Vasc Surg 2006;44:1114-6.  Back to cited text no. 3
Sagban TA, Grotemeyer D, Balzer KM, et al. Surgical treatment for agenesis of the vena cava: A single-centre experience in 15 cases. Eur J Vasc Endovasc Surg 2010;40:241-5.  Back to cited text no. 4
Gupta A, Kumar S, Kothari SS. Congenital absence of infrarenal inferior vena cava and deep veins of the lower limbs: A case report. J Med Case Rep 2016;10:218.  Back to cited text no. 5
Al Mandhari H, Rolnitsky A. Interrupted inferior vena cava with azygos continuation in an extreme preterm infant: Unusual lower-limb peripherally inserted central catheter line tip position. J Clin Neonatol 2019;8:122.  Back to cited text no. 6
Mercado PL, Liberto DH, Udaquiola J, et al. Fetal ascites as clinical presentation of inferior vena cava agenesis. Arch Argent Pediatr 2018;116:e621-25.  Back to cited text no. 7
López-Núñez M, Tuneu-Valls A, Jaka-Moreno A, et al. Cutaneous aspects of congenital absence of the inferior vena cava in a newborn. Pediatr Dermatol 2013;30:e18-9.  Back to cited text no. 8
Raposo Rodríguez L, Recio Rodríguez M, Álvarez Moreno E, et al. Agenesia de la vena cava inferior infrarrenal con trombosis de la vena renal fetal: A propósito de un caso. Radiologia 2012;54:457-61.  Back to cited text no. 9
Ojha V, Pandey NN, Jagia P. Hemiazygos continuation of isolated left-sided inferior vena cava into persistent left superior vena cava: Rare association of left isomerism. BMJ Case Rep. 2019 Apr 20;12:e230350.  Back to cited text no. 10


  [Figure 1], [Figure 2]

  [Table 1]


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