Absent inferior venacava and anti phospholipid antibody syndrome: Compounding risk factor for deep vein thrombosis and recurrent pregnancy loss

Aruna Nigam; Nidhi Gupta; Abhinav Jain; Arima Nigam

doi:10.4103/INJMS.INJMS_67_19

CASE REPORT

Year : 2019 | Volume : 10 | Issue : 4 | Page : 219-221

Absent inferior venacava and anti phospholipid antibody syndrome: Compounding risk factor for deep vein thrombosis and recurrent pregnancy loss

Aruna Nigam¹, Nidhi Gupta¹, Abhinav Jain², Arima Nigam³
¹ Department of Obstetrics and Gynaecology, Hamdard Institute of Medical Sciences and Research, Jamia Hamdard, New Delhi, India
² Department of Radiology, Hamdard Institute of Medical Sciences and Research, Jamia Hamdard, New Delhi, India
³ Department of Cardiology, G B Pant Hospital, New Delhi, India

Date of Submission	23-Jun-2019
Date of Decision	04-Oct-2019
Date of Acceptance	04-Oct-2019
Date of Web Publication	18-Nov-2019

Correspondence Address:
Arima Nigam
Department of Obstetrics and Gynaecology, Hamdard Institute of Medical Sciences and Research, Jamia Hamdard, New Delhi - 110 001
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/INJMS.INJMS_67_19

Abstract

The spectrum of congenital anomalies of the inferior vena cava (IVC) has been well described in the literature, but the absence of IVC is a rare occurrence. Most of these patients remain asymptomatic, and the age of presentation depends on the development of deep-venous thrombosis. A young female presented to us with irregular bleeding per vaginum and recurrent pregnancy loss. A thorough general examination of the female patient revealed dilated veins over the abdomen and lower limbs. Investigations of recurrent pregnancy loss led to the diagnosis of antiphospholipid antibody syndrome and computed tomography venography to find out the cause of dilated veins lead to the diagnosis of absent IVC. Both these factors, i.e., absent IVC and the presence of autoimmune antibodies, have compounded the risk factor of thrombosis and management challenging which is discussed.

Keywords: Antiphospholipid antibody, inferior vena cava, recurrent pregnancy loss

How to cite this article:
Nigam A, Gupta N, Jain A, Nigam A. Absent inferior venacava and anti phospholipid antibody syndrome: Compounding risk factor for deep vein thrombosis and recurrent pregnancy loss. Indian J Med Spec 2019;10:219-21

How to cite this URL:
Nigam A, Gupta N, Jain A, Nigam A. Absent inferior venacava and anti phospholipid antibody syndrome: Compounding risk factor for deep vein thrombosis and recurrent pregnancy loss. Indian J Med Spec [serial online] 2019 [cited 2023 Mar 31];10:219-21. Available from: http://www.ijms.in/text.asp?2019/10/4/219/271226

Introduction

The prevalence of congenital inferior vena cava (IVC) anomalies in the general population is estimated to be 0.5%–3%, but the congenital absence of IVC is extremely rare condition with a prevalence rate of 0.0005%–0.001%.^[1],[2] In most studies, the most common anomaly of IVC is hypoplasia of prerenal and renal segments, followed by hypoplasia of postrenal segment and IVC duplicity.^[3] The first case of congenitally absent intrahepatic as well as infrahepatic course of IVC and bilateral common iliac vein is reported who presented with recurrent pregnancy loss and irregular bleeding per vaginum.

Case Report

A 27-year-old nulliparous woman presented with a complaint of recurrent pregnancy loss. She had five abortions, of which four were spontaneous first-trimester abortions. The last abortion was the second-trimester-induced abortion in view of absent liquor and sluggish fetal movements. She also gives a history of frequent menstrual cycles along with intermenstrual spotting. Her general physical examination revealed normal blood pressure and pulse rate. She had dilated superficial veins [Figure 1] over the abdomen (with flow from below upward) and the lower limbs with bilateral pedal edema. There was no calf tenderness. Her gynecological examination was unremarkable.

Figure 1: Dilated superficial veins over the abdomen (arrows)

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Her gynecological ultrasound was normal. On blood investigations, her hemoglobin was 12.1 g/dl with raised hematocrit (41.4%) and fasting blood sugar was 75 mg/dl. Her thyroid profile (thyroid-stimulating hormone: 3.1 micro IU/L, normal range: 0.55–4.78 micro IU/L) was normal. Her coagulation profile was deranged (prothrombin time: 13.4 s [normal: 11–14 s] and activated partial thromboplastin time: 74.3 s [normal: 25–35 s] with international normalized ratio: 1.05 [normal: <1.3]). On investigating for thrombophilia profile, protein C (109 U/dl [normal: 65–135 U/dl]), protein S (39 U/dl), anti-thrombin (106 U/dl), anticardiolipin antibodies (immunoglobulin G [IgG]: 5.9 GPL/ml, IgM 6.8 MPL/ml, normal <12 MPL/ml), and antiphospholipid antibodies (APLA) (3.3 U/ml normal <12 U/ml) were normal. Her lupus anticoagulant (LAC), anti-beta2 glycoprotein (IgM: 96.2 MPL/ml and IgG: 4.6 GPL/ML) antibodies, and antinuclear antibodies ([ANA] positive in 1: 40 dilution) were done which came out to be positive. In view of bilateral pedal edema and dilated veins over the abdomen, her computed tomography (CT) venography was done which revealed the absent intrahepatic as well as infrahepatic course of IVC with the presence of only suprahepatic segment and dilated collateral channels in the retroperitoneum, paravertebral regions, abdominal wall, chest wall, and axilla draining into brachiocephalic veins [Figure 2] and [Figure 3]. Bilateral common iliac veins were also absent with superficial dilated channels in the groin and visualized parts of the lower limb. Azygos vein was prominent.

Figure 2: Contrast-enhanced axial computed tomography scan in the venous phase reveals the absence of the inferior vena cava. Collateral flow from the lower extremities reaches the azygos vein through paravertebral collateral veins. Aorta (long white arrow), absent inferior vena cava (small white arrow), and subcutaneous collaterals (arrowhead)

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Figure 3: Contrast-enhanced coronal reformats in the venous phase reveals the absence of the inferior vena cava. Multiple collaterals (arrow)

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The diagnosis of congenital IVC agenesis/hypoplasia with antiphospholoipid antibody syndrome was made. The patient was started on anticoagulants along with cyclical progesterone therapy (for menstrual complaint). The patient has responded well to the treatment and is under follow-up.

Discussion

The spectrum of congenital anomalies of the IVC has been well described. Most of these patients remain asymptomatic, and the age of presentation depends on the development of deep-venous thrombosis as the presence of IVC anomalies itself increases the risk of deep-vein thrombosis.^[4] The presence of autoimmune antibodies has compounded the risk factor of thrombosis and made this case unique. Interestingly, this patient presented with recurrent pregnancy loss and the presence of dilated veins and pedal edema pointed toward associated abnormalities for which ultrasound Doppler and CT were done.

Normally, the IVC is formed by the union of the common iliac veins anterior to the fifth lumbar vertebral body, a little to its right. It ascends cephalad to receive blood from the renal and hepatic veins and then crosses the diaphragm to empty into the right atrium. The azygos vein normally receives blood from the right ascending lumbar and lower right intercostal veins and lies right of the spine and empty into the superior vena cava. The hemiazygos vein ascends to the left of the spinal column and receives blood from the left lumbar, the left renal and fourth through the seventh intercostal veins. In lower thorax, both azygos and hemiazygos veins course parallel to the descending aorta. At the level of the 8^th thoracic vertebral body, the hemiazygos vein crosses the midline behind the aorta to drain into the azygos vein.

The absence of IVC predisposes the patient for thrombosis. As it is evident that the absence of venous system in the lower part of the body leads to the development of the collaterals through the retroperitoneum and abdominal wall. However, this collateral venous system is not enough for efficient blood return and there occurs relative venous stasis in the lower limb, leading to the development of varicose veins and pedal edema. It is postulated that this generally occurs in the event of vigorous physical exercise. In our case, the presence of APLA had compounded the risk for thrombosis and lead to the complicated management in this case.

CT has been recommended as the method of choice for the diagnosis of anomalies of IVC anatomy.^[5] There is controversy as to whether an absent IVC is a true embryonic anomaly or whether it is the result of perinatal IVC thrombosis causing regression and disappearance of the once present IVC.^[6] If it is a developmental defect, the nature of defect will depend on the timing of embryonic insult as IVC develops from four segments and each segment is derived from different embryonic vein.^[7]

The present case of total absence of IVC is the first case reported in the English literature. The presence of LAC, anti-beta2 glycoprotein, and ANA along with absent IVC compounded the risk factors for recurrent pregnancy loss and management difficult. This case also emphasizes the importance of complete general physical examination in all the cases as the presence of pedal edema and dilated veins in the lower limbs and abdomen pointed toward the additional factor in the workup of recurrent pregnancy loss. Although the treatment for recurrent pregnancy loss and deep-vein thrombosis will be same as anticoagulation therapy is needed in both the cases, the anticoagulation is needed for whole life. Besides there is a high index of thrombosis recurrence because of inadequate venous return which needs regular monitoring. Surgical intervention is rarely indicated in these cases. Anticoagulation coupled with lifestyle changes and compression stockings is usually the therapy of choice for these patients, but the data about the optimal timing of anticoagulation are still lacking.

Conclusion

Complete general physical examination should be done in all cases of recurrent pregnancy losses. CT is the method of choice for the diagnosis of anomalies of IVC. Life style changes and anticoagulation therapy are needed for whole life in these patients.

Declaration of patient consent

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

Financial support and sponsorship

None.

Conflicts of interest

There are no conflicts of interest.

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