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Year : 2019  |  Volume : 8  |  Issue : 2  |  Page : 122-124

Interrupted inferior vena cava with azygos continuation in an extreme preterm infant: Unusual lower-limb peripherally inserted central catheter line tip position

1 Neonatal Unit, Child Health Department, Sultan Qaboos University Hospital, Al Khoudh, Muscat, Oman
2 Sunnybrook Health Sciences Centre NICU, University of Toronto, Toronto, Canada

Date of Web Publication25-Apr-2019

Correspondence Address:
Dr. Hilal Al Mandhari
Neonatal Unit, Sultan Qaboos University Hospital, P.O. Box 38, Al Khoudh 123, Muscat
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcn.JCN_92_18

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We report a case of interrupted inferior vena cava (IVC) with azygos continuation in an extremely preterm infant born at 23 weeks of gestation. This anomaly was detected after the insertion of lower-limb peripherally inserted central catheter. The tip of the catheter on lateral chest X-ray was in an unusual position as the catheter continued to run anterior to the spinal vertebrae and posterior to the umbilical arterial catheter within the aorta on two separate insertion attempts. Ultrasound Doppler and echocardiogram confirmed the diagnosis of interrupted IVC with azygos continuation. Neonatal practitioners need to be aware of this anomaly to avoid unnecessary interventions.

Keywords: Interrupted inferior vena cava with azygos continuation, peripherally inserted central catheter, preterm

How to cite this article:
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;8:122-4

How to cite this URL:
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 [serial online] 2019 [cited 2021 Aug 2];8:122-4. Available from: https://www.jcnonweb.com/text.asp?2019/8/2/122/257142

  Introduction Top

Interrupted inferior vena cava (IVC) with azygos/hemizygous continuation to superior vena cava (SVC) is a rare anatomical variant.[1] It has been previously described in association with congenital heart disease; nevertheless, isolated cases may go unrecognized and may be incidentally diagnosed, especially in young patients (adolescents and children), while it may be symptomatic in adult population with symptoms of venous insufficiency and deep venous thrombosis.[2] The aim of this case report is to raise the awareness of neonatal practitioners of this anatomical variant, due to its potential implication of peripherally inserted central catheter (PICC) placement, inserted through lower-limb approach. Here, we report a case of interrupted IVC in an extreme preterm, detected after PICC inserted twice through saphenous vein on either leg. Lateral chest and abdominal X-ray showed the PICC tip in an unusual position posterior to an arterial umbilical catheter.

  Case Report Top

Our patient is an extreme preterm infant, born at 23 weeks and 6 days of gestation to a 29-year-old mother. Prenatal ultrasounds 3 weeks prior to delivery revealed normal fetal anatomy. The baby was born at a tertiary care perinatal center due to preterm labor. The birth weight was 698 g. He was admitted to the neonatal intensive care unit. Umbilical arterial catheter (UAC) and venous catheter were inserted after birth. PICC line was inserted on day 6 of life for prolonged intravenous therapy through the right saphenous vein. Insertion was uneventful, with good blood return and free flush. X-ray for line position showed tip of the PICC line on the anterolateral view at T9; however, on the lateral view, the PICC line tip continued to run behind the UAC just anterior to the vertebrae [Figure 1]. Due to suspicion about the position of PICC line tip and concern of arterial insertion, the PICC line was removed. A new PICC line was inserted through the left saphenous vein. X-ray post insertion showed similar findings of the course of PICC line compared to the previous X-ray [Figure 1]. An ultrasound Doppler was done which showed an interrupted IVC with azygos continuation, with PICC tip in the azygos vein. Echocardiogram confirmed the same findings. The PICC line, thus, was kept in situ for usage and remained functioning for 2 weeks.
Figure 1: Chest and abdominal X-ray of left saphenous peripherally inserted central catheter line. (a) Anteroposterior view with tip of the peripherally inserted central catheter to the right of umbilical arterial catheter at T9. (b) Lateral view showing the peripherally inserted central catheter line tip lying posterior to the umbilical arterial catheter. White arrow – Peripherally inserted central catheter tip; Black arrow – expected location of peripherally inserted central catheter tip within the inferior vena cava

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  Discussion Top

Interrupted IVC with azygos continuation is a rare congenital anomaly, with a reported prevalence of < 0.3% in otherwise normal patients. The prevalence is reported to be more in patients with congenital heart disease, with a prevalence of 0.6%–2%.[2] It is caused by developmental anomaly resulting in termination of the IVC below the hepatic vein. The venous blood flow, thus, is carried out by dilated azygos and hemiazygos veins, which eventually empty into the SVC. The hepatic veins drain directly into the right atrium.[1]

Interrupted IVC with azygos continuation has been reported in association with cardiac malformations, for example, sick sinus syndrome,[3] congenital heart defects and heterotaxy syndrome,[4],[5] persistent left SVC, left single coronary aorta,[6] and supraventricular tachycardia.[7]

Most cases of interrupted IVC with azygos continuation are detected incidentally, usually on computed tomography where the condition may simulate paraspinal lymphadenopathies.[8],[9] However, cases of antenatal diagnosis of this anomaly had been reported.[10],[11] Antenatal ultrasound in such cases typically shows undetectable IVC between the kidneys and liver, and a venous vessel is observed parallel to the aorta between the kidney and right atrium. The azygos vein is typically dilated and results in the presence of “double-vessel” sign behind the heart in a transverse image.[5]

Interrupted IVC with azygos continuation can have significant clinical implications. In our patient, PICC line was inserted twice due to unusual course of the catheter and concern of arterial insertion. Venous access to the right side of the heart using femoral venous access might be a challenge in cases of interrupted IVC with azygos continuation.[12]

PICC insertion is a very common procedure in preterm infants admitted to neonatal intensive care units for prematurity care. The saphenous vein is used often due to its large size and visibility in a small extreme and very low-birthweight infant. In our case, the tip of the PICC was within the azygos vein in both PICC line insertion attempts. The initial PICC was removed due to concern of the arterial placement. Hence, the infant was unnecessarily exposed to a second PICC insertion, due to undiagnosed interrupted IVC. In retrospect, ultrasound and echocardiogram could have been done after the first PICC insertion when the X-ray showed an unusual tip position. Neonatal practitioners need to be aware of this rare anomaly, to avoid exposing those infants to unnecessary interventions.

Take-home message

Although rare, interrupted IVC with azygos continuation may be encountered in preterm infants. Neonatologists need to be aware of this variant, to expect lower-limb PICC line to have a different course and to avoid unnecessary removal potentially.

Declaration of patient consent

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

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Conflicts of interest

There are no conflicts of interest.

  References Top

Onbaş O, Kantarci M, Koplay M, Olgun H, Alper F, Aydinli B, et al. Congenital anomalies of the aorta and vena cava: 16-detector-row CT imaging findings. Diagn Interv Radiol 2008;14:163-71.  Back to cited text no. 1
Bass JE, Redwine MD, Kramer LA, Huynh PT, Harris JH Jr. Spectrum of congenital anomalies of the inferior vena cava: Cross-sectional imaging findings. Radiographics 2000;20:639-52.  Back to cited text no. 2
Vijayvergiya R, Bhat MN, Kumar RM, Vivekanand SG, Grover A. Azygos continuation of interrupted inferior vena cava in association with sick sinus syndrome. Heart 2005;91:e26.  Back to cited text no. 3
Sunilkumar MN, Parvathy VK. Heterotaxy syndrome in a preterm baby-a rare case report with review of literature. Int J Clin Case Rep 2015;5:1-5.  Back to cited text no. 4
Sheley RC, Nyberg DA, Kapur R. Azygous continuation of the interrupted inferior vena cava: A clue to prenatal diagnosis of the cardiosplenic syndromes. J Ultrasound Med 1995;14:381-7.  Back to cited text no. 5
Kim YJ, Kwon SH, Ahn SE, Kim SJ, Shin JS, Oh JH. Interrupted inferior vena cava with hemiazygos continuation in an adult with a persistent left superior vena cava and left single coronary artery: A case report. J Korean Soc Radiol 2016;74:394-8.  Back to cited text no. 6
Gonzalez JE, Nguyen DT. Supraventricular tachycardia in a patient with an interrupted inferior vena cava. Card Electrophysiol Clin 2016;8:45-50.  Back to cited text no. 7
Spinnato P, Bazzocchi A, Albisinni U. How the absence of infrarenal inferior vena cava can mimic lymphoma. Pediatr Neonatol 2018. pii: S1875-9572(17)30695-2.  Back to cited text no. 8
Milner LB, Marchan R. Complete absence of the inferior vena cava presenting as a paraspinous mass. Thorax 1980;35:798-800.  Back to cited text no. 9
Giang do TC, Rajeesh G, Vaidyanathan B. Prenatal diagnosis of isolated interrupted inferior vena cava with azygos continuation to superior vena cava. Ann Pediatr Cardiol 2014;7:49-51.  Back to cited text no. 10
Celentano C, Malinger G, Rotmensch S, Gerboni S, Wolman Y, Glezerman M, et al. Prenatal diagnosis of interrupted inferior vena cava as an isolated finding: A benign vascular malformation. Ultrasound Obstet Gynecol 1999;14:215-8.  Back to cited text no. 11
Kler TS, Bhatia A, Saxena A, Sardana R, Arora V. Catheter ablation of left free wall accessory pathway in a patient with inferior vena cava interruption. Indian Heart J 2002;54:705-7.  Back to cited text no. 12


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