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Postpartum Retroperitoneal Fibrosis With IVC Obstruction: A Case Report
Roy Clark Basiimwa1, Samuel Bugeza1, Rita Nassanga1
1Department of Radiology, School of Medicine, Makerere University, Kampala, Uganda, mak.ac.ug.
Abstract:
Retroperitoneal fibrosis (RPF) is a rare fibroinflammatory condition producing inflammatory masses in the retroperitoneum that can obstruct retroperitoneal structures, including vessels and ureters. RPF typically presents in middle-aged men and may be associated with IgG-4-related disease (IgG-4 RD). This report describes a 34-year-old woman who presented 6 months postpartum with left leg swelling and lower abdominal pain. Contrast-enhanced CT revealed a homogenous retroperitoneal soft-tissue mass encasing the aorta and iliac arteries, with inferior vena cava (IVC) obstruction and collateral venous return through extensive pelvic varices. MRI findings included hypointensity on T2-weighted imaging and restricted diffusion. Management with thrombolytics and steroids resulted in recanalization of the IVC, though persistent hydronephrosis required nephrostomy and further urological intervention. The presentation of RPF in a young woman is unusual, and we theorize that immune system reactivation following the relative immunosuppression during pregnancy may have precipitated the condition. The case illustrates one of several immunologic factors associated with RPF.
Insights
Retroperitoneal fibrosis (RPF) is a rare condition. This case highlights an unusual presentation in a young woman postpartum, suggesting immune system reactivation may trigger RPF.
Area of Science:
- Medicine
- Immunology
- Radiology
Background:
- Retroperitoneal fibrosis (RPF) is a rare fibroinflammatory condition.
- It typically affects middle-aged men and can be linked to IgG-4-related disease (IgG-4 RD).
- RPF can obstruct vital retroperitoneal structures like vessels and ureters.
Purpose of the Study:
- To report an unusual case of RPF in a young woman.
- To explore potential immunologic triggers for RPF.
- To discuss diagnostic findings and management strategies.
Main Methods:
- Case report of a 34-year-old woman presenting postpartum.
- Contrast-enhanced CT and MRI were used for diagnosis.
- Treatment involved thrombolytics, steroids, nephrostomy, and urological intervention.
Main Results:
- A homogenous retroperitoneal mass encasing major arteries and obstructing the IVC was identified.
- MRI showed characteristic findings of hypointensity on T2-weighted imaging and restricted diffusion.
- Treatment led to IVC recanalization but persistent hydronephrosis required further intervention.
Conclusions:
- RPF presentation in young women is atypical.
- Postpartum immune system reactivation is theorized as a potential trigger for RPF.
- This case underscores the diverse immunologic factors associated with RPF.