Elsevier

Thrombosis Research

Volume 135, Issue 4, April 2015, Pages 648-651
Thrombosis Research

Regular Article
Congenital abnormalities of the inferior vena cava presenting clinically in adolescent males

https://doi.org/10.1016/j.thromres.2015.01.032Get rights and content

Highlights

  • Five adolescents males with DVTs and congenital IVC abnormalities are described

  • IVC abnormality is an under-recognized risk factor for DVT in adolescents

  • Detailed vascular imaging should be obtained in select cases of spontaneous DVT

  • Developmental stage of adolescence is relevant to management

Abstract

Introduction

Congenital anatomic abnormality of the inferior vena cava (IVC) is an important risk factor for the development of spontaneous proximal lower extremity deep vein thrombosis (DVT) in young adults. The incidence of DVT associated with congenital IVC anomalies in paediatric populations has not been described, and the implications of IVC anomalies for treatment and outcomes of DVT are unknown.

Methods

This study reports a series of five adolescent males with spontaneous lower extremity DVTs and underlying congenital IVC abnormalities. Cases were identified by searching the institutional database of patients treated with anticoagulation for venous thromboembolism at a tertiary children's hospital.

Results

The demographics, clinical presentations, imaging findings, treatment courses, and outcomes are described. All cases occurred in males, and accounted for approximately twenty percent of adolescent males presenting with DVT.

Conclusions

IVC abnormality is likely an under-recognized risk factor for DVT in this age group, and detailed vascular imaging should be pursued in adolescents with spontaneous proximal lower extremity DVT when initial ultrasonography does not delineate the proximal clot extent. Management requires individual risk-benefit assessment in the context of providing developmentally appropriate care. Further research is required to establish long-term outcomes and determine optimal treatment strategies.

Introduction

Congenital anatomic abnormality of the inferior vena cava (IVC) is an important risk factor for the development of spontaneous venous thrombosis in young adults. A number of congenital IVC anomalies have been described [1], and the prevalence of IVC anomalies in the general population has been estimated to be between 0.07 and 8.7% [6]. These abnormalities are often asymptomatic, and identified incidentally in patients undergoing imaging investigations for other reasons [8]. It is likely that many, if not most, cases of congenital IVC abnormalities remain undiagnosed and of little or no consequence to affected individuals. However, there is a subset of patients with congenital IVC anomalies who present with clinically significant deep vein thrombosis (DVT). Because the mainstay of diagnosis of DVT is ultrasound with venous Doppler, which does not readily identify IVC anomalies, these anomalies may be under-diagnosed in patients presenting with spontaneous DVTs.

Previous estimates of the prevalence of IVC anomalies among young adult patients with DVTs have been in the range of 5% in patients under 30 years of age, [3], [7]. Although there are reported cases occurring in adolescent patients [3], [4], [7], [13], the incidence of DVT associated with congenital IVC anomalies has not been described in paediatric populations. Implications of IVC anomalies for treatment and long-term outcomes of DVT are also unknown.

We report a series of five adolescents who presented with spontaneous DVTs of the proximal lower extremities and have been found to have underlying congenital abnormalities of their IVCs. We describe the demographics, clinical presentations, imaging findings, treatment courses, and outcomes of these patients. To our knowledge, this is the first report of IVC anomalies presenting with venous thrombosis in the paediatric age group that have been managed at a tertiary children’s hospital.

Section snippets

Methods

We searched our institutional database of patients who have been treated with anticoagulation for venous thromboembolism between 1999 and 2012 within the department of Clinical Haematology at the Royal Children’s Hospital, a tertiary children’s hospital in Melbourne, Australia. The database includes all patients requiring anticoagulation for more than 3 months. Therefore, there is the potential to have missed patients with lower limb venous thromboembolism if they were anticoagulated for a

Cases

A total of five patients with DVTs who were diagnosed with underlying congenital anatomic IVC abnormalities were identified. An additional two children had IVC anomalies that were acquired as a result of other medical conditions (one secondary to central line placement during neonatal gastroschisis repair, one secondary to multiple vascular catheterizations for dialysis). These patients were not included in this case series. There was a total of 49 adolescent patients (ages 12 – 18 years); 25

Discussion

Congenital anomalies of the IVC are known to be a risk factor for the development of venous thrombosis in young adults, however the prevalence of these anomalies among paediatric patients with spontaneous DVT has not been studied. We report 5 consecutive cases which presented during adolescence. The cases only occurred in males, and accounted for approximately twenty percent of all adolescent males presenting with DVT at our institution. Comprehensive imaging and consideration of congential IVC

Conflict of interest statement

The authors have no conflicts of interest to report.

Acknowledgements

The authors would like to acknowledge the assistance of staff in Health Information Services at the Royal Children’s Hospital for assisting with the recovery of the records identified for review.

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