IVCA in a Boy with Multilocular Renal Cyst as a Risk Factor for Deep Vein Thrombosis

Szmigielska, Agnieszka; Skrzypczyk, Piotr; Szyszka, Michał; Bukowska, Magdalena; Wojtas, Malwina; Jakimów-Kostrzewa, Aleksandra

doi:10.34763/jmotherandchild.20242801.d-24-00045

Abstract

Introduction

The triad of symptoms: renal defects, congenital inferior vena cava agenesis (IVCA) and deep vein thrombosis of the lower limbs make up the KILT syndrome (kidney and IVC abnormalities with leg thrombosis).

Case report

A 17-year-old boy complained of periodic abdominal pain. Abdominal ultrasonography revealed a multilocular cyst in the right kidney. Physical examination showed no abnormalities, and his blood pressure was 120/80mmHg. Abdominal ultrasonography showed a cyst measuring 36×30×25mm in the right kidney hilum. Computed tomography did not show the hepatic and suprarenal sections of the inferior vena cava. Numerous varicose-dilated collateral vessels, including renal venous vessels, were found in the right kidney hilum. The collateral vessels in the tomography matched the described in the ultrasound renal cyst. MRI confirmed IVCA with no other additional vascular abnormalities. Due to the risk of deep vein thrombosis of the lower limbs, non-pharmacological antithrombotic prophylaxis was recommended.

Conclusions

Early detection of inferior vena cava agenesis allows for the reduction of the risk of dangerous thrombotic complications.

References

Mensel B, Kühn JP, Kracht F, et al. Prevalence of renal cysts and association with risk factors in a general population: an MRI-based study. Abdom Radiol (NY). 2018;43(11):3068–74.
Search in Google Scholar Back to article
Lauener S, Bütikofer A, Eigenheer S, et al. Thrombophlebitis hiding under a KILT - case report on 40 years long-term follow-up of neonatal renal vein thrombosis. BMC Pediatr. 2019;19(1):183.
Search in Google Scholar Back to article
Pasqui E, de Donato G, Camarri S, et al. Front Surg. 2022 Mar 22;9:832336.
Search in Google Scholar Back to article
Ruggeri M, Tosetto A, Castaman G, et al. Congenital absence of the inferior vena cava: a rare risk factor for idiopathic deep-vein thrombosis. Lancet. 2001;357(9254):441.
Search in Google Scholar Back to article
Van Veen J, Hampton KK, Makris M. Kilt syndrome? Br J Haematol. 2002;118(4):1199–200.
Search in Google Scholar Back to article
Gayer G, Zissin R, Styrauss S, et al. IVC anomalies and right renal aplasia detected on CT: a possible link? Abdom Imaging. 2003;28(3):395–9.
Search in Google Scholar Back to article
Bass JE, Redwine MD, Kramer LA, et al. Absence of the infrarenal inferior vena cava with preservation of the suprarenal segment as revealed by CT and MR venography. AJR Am J Roentgenol. 1999;172(6):1610–2.
Search in Google Scholar Back to article
Pomeranz CB, Cullen DL, Bellah RD. Deep venous thrombosis in a child with inferior vena cava and renal anomalies: KILT syndrome. Pediatr Radiol. 2018;48(10):1521–5.
Search in Google Scholar Back to article
Bami S, Vazquez Y, Chorny V, et al. Deep venous thrombosis of the leg, associated with agenesis of the infrarenal inferior vena cava and hypoplastic left kidney (KILT syndrome) in a 14-year-old child. Case Rep Pediatr. 2015;2015:864047.
Search in Google Scholar Back to article
Kalicki B, Sadecka M, Wawrzyniak A, et al. Absence of inferior vena cava in 14-year old boy associated with deep venous thrombosis and positive Mycoplasma pneumoniae serum antibodies--a case report. BMC Pediatr. 2015;15:40.
Search in Google Scholar Back to article
Lauener S, Butikofer A, Eigheer S, et al. Thrombophlebitis hiding under a KILT – case report on 40 years long-term follow-up of neonatal renal vein thrombosis. BMC Pediatr. 2019; 19:183.
Search in Google Scholar Back to article
British Thoracic Society Standards of care Committee Pulmonary Embolism Guideline Development Group. British Thoracic Society guidelines for the management of suspected acute pulmonary embolism. Thorax. 2003;58:470–84.
Search in Google Scholar Back to article
Paddock M, Robson N. The curious case of the disappearing IVC: a case report and review of the aetiology of inferior vena cava agenesis. J Radiol Case Rep. 2014;8(4):38–47.
Search in Google Scholar Back to article
Massicotte P, Julian JA, Gent M, et al. REVIVE Study Group. An open-label randomized controlled trial of low molecular weight heparin compared to heparin and coumadin for the treatment of venous thromboembolic events in children: the REVIVE trial. Thromb Res. 2003;109(2–3):85–92.
Search in Google Scholar Back to article

IVCA in a Boy with Multilocular Renal Cyst as a Risk Factor for Deep Vein Thrombosis

Abstract

Introduction

Case report

Conclusions

Paradigm

My account