Endovascular treatment of intrarenal aneurysms bleeding and angiomyolipomas in a patient with tuberous sclerosis and polycystic kidney disease

Tuberous sclerosis complex (TSC) and autosomal dominant polycystic kidney disease (ADPKD) are conditions related to renal failure that can rarely occur in association as a contiguous gene syndrome. Angiomyolipomas (AMLs) are renal tumors strongly related to TSC that may rupture and cause life-threatening bleedings. We present a patient with TSC, ADPKD, and renal AMLs with persistent hematuria requiring blood transfusion. The persistent hematuria was successfully treated through endovascular embolization, a minimally invasive nephron sparing technique.

Keywords:
Tuberous Sclerosis; Polycystic Kidney Diseases; Angiomyolipoma

Authorship

Túlio Leite Correspondence to: Túlio Leite. E-mail: tuliofabiano@hotmail.com

Universidade de São Paulo, Instituto do Coração, Departamento de Radiologia, São Paulo, SP, Brasil.Universidade de São PauloBrasilSão Paulo, SP, BrasilUniversidade de São Paulo, Instituto do Coração, Departamento de Radiologia, São Paulo, SP, Brasil.

http://orcid.org/0000-0001-9427-5897

Lucas Vatanabe Pazinato

Universidade de São Paulo, Faculdade de Medicina, Departamento de Radiologia, Unidade de Radiologia Intervencionista, São Paulo, SP, Brasil.Universidade de São PauloBrasilSão Paulo, SP, BrasilUniversidade de São Paulo, Faculdade de Medicina, Departamento de Radiologia, Unidade de Radiologia Intervencionista, São Paulo, SP, Brasil.

http://orcid.org/0000-0001-5320-1648

Maria Juliana de Aquino Vidal

Hospital Santa Genoveva, Uberlândia, MG, Brasil.Hospital Santa GenovevaBrasilUberlândia, MG, BrasilHospital Santa Genoveva, Uberlândia, MG, Brasil.

http://orcid.org/0000-0003-0481-6480

Danielo de Freitas

Hospital Santa Genoveva, Departamento de Urologia, Uberlândia, MG, Brasil.Hospital Santa GenovevaBrasilUberlândia, MG, BrasilHospital Santa Genoveva, Departamento de Urologia, Uberlândia, MG, Brasil.

Joaquim Mauricio da Motta Leal Filho

http://orcid.org/0000-0001-9844-6833

Correspondence to:
Túlio Leite. E-mail: tuliofabiano@hotmail.com

Authors’ Contribution

All authors contribute to the study design, surgery, data acquisition, data analysis, drafting of manuscript, and critical revision of the manuscript.

Conflict of Interest

The authors declare that they have no conflict of interest.

SCIMAGO INSTITUTIONS RANKINGS

Hospital Santa Genoveva, Uberlândia, MG, Brasil.Hospital Santa GenovevaBrasilUberlândia, MG, BrasilHospital Santa Genoveva, Uberlândia, MG, Brasil.

Figures

Figures (2)

Thumbnail

Figure 1
a) Non-enhanced CT evidencing multiple small masses with fat attenuation in the right kidney lower pole consistent with AMLs (arrow). Hyperattenuating material filling the dilated renal pelvis and calyces due to hematuria (arrowhead). b) Maximum intensity projection arterial phase CT evidencing pseudo-aneurysms/intrarenal aneurysms in the right kidney lower pole (arrows) and bilateral cortical cysts (arrowhead). c) Coronal T2-weighted fat-suppressed magnetic resonance image shows multiple bilateral simple cysts with thin regular walls (arrows). Renal parenchyma is shown in the lower pole of the right kidney (arrowhead).

Thumbnail

Figure 2
A: Arterial phase evidenced multiple saccular dilatations (aneurysms) within the inferior interlobar branches of the right renal artery (white arrow). B: Selective arteriography of the lower pole branch with multiple saccular dilations (White arrow). C: Cone beam CT was performed to assess the remaining kidney segments. In the reconstruction, a suspicious area of active bleeding was identified in the lower pole (white arrow). D: Superselective embolization of microaneurysms with Onix-18 and control arteriography with devascularization of the lower pole branches (white arrow).

Figure 1 a) Non-enhanced CT evidencing multiple small masses with fat attenuation in the right kidney lower pole consistent with AMLs (arrow). Hyperattenuating material filling the dilated renal pelvis and calyces due to hematuria (arrowhead). b) Maximum intensity projection arterial phase CT evidencing pseudo-aneurysms/intrarenal aneurysms in the right kidney lower pole (arrows) and bilateral cortical cysts (arrowhead). c) Coronal T2-weighted fat-suppressed magnetic resonance image shows multiple bilateral simple cysts with thin regular walls (arrows). Renal parenchyma is shown in the lower pole of the right kidney (arrowhead).

Figure 2 A: Arterial phase evidenced multiple saccular dilatations (aneurysms) within the inferior interlobar branches of the right renal artery (white arrow). B: Selective arteriography of the lower pole branch with multiple saccular dilations (White arrow). C: Cone beam CT was performed to assess the remaining kidney segments. In the reconstruction, a suspicious area of active bleeding was identified in the lower pole (white arrow). D: Superselective embolization of microaneurysms with Onix-18 and control arteriography with devascularization of the lower pole branches (white arrow).

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Endovascular treatment of intrarenal aneurysms bleeding and angiomyolipomas in a patient with tuberous sclerosis and polycystic kidney disease

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[1] Correspondence to:
Túlio Leite. E-mail: tuliofabiano@hotmail.com