Kidney Week

Abstract: TH-PO491

Melorheostosis and Renal Artery Stenosis in Solitary Kidney: A Pediatric Case

Session Information

• Pediatric Nephrology - I
  November 02, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Pediatric Nephrology

• 1900 Pediatric Nephrology

Authors

• Bigatti, Carolina, Istituto Giannina Gaslini, Genova, Liguria, Italy

Carolina Bigatti,

• Mortari, Gabriele, Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia, Brescia, Lombardia, Italy

Gabriele Mortari,

• Angeletti, Andrea, Istituto Giannina Gaslini, Genova, Liguria, Italy

Andrea Angeletti,

• Lugani, Francesca, Istituto Giannina Gaslini, Genova, Liguria, Italy

Francesca Lugani,

• Cheli, Martino, Istituto Giannina Gaslini, Genova, Liguria, Italy

Martino Cheli,

• Caridi, Gianluca, Istituto Giannina Gaslini, Genova, Liguria, Italy

Gianluca Caridi,

• Verrina, Enrico E., Istituto Giannina Gaslini, Genova, Liguria, Italy

Enrico E. Verrina,

• Ghiggeri, Gian Marco, Istituto Giannina Gaslini, Genova, Liguria, Italy

Gian Marco Ghiggeri,

• Magnasco, Alberto, Istituto Giannina Gaslini, Genova, Liguria, Italy

Alberto Magnasco,

• Lancieri, Maddalena, Istituto Giannina Gaslini, Genova, Liguria, Italy

Maddalena Lancieri,

• Lionetti, Barbara, Istituto Giannina Gaslini, Genova, Liguria, Italy

Barbara Lionetti,

• La Porta, Edoardo, Istituto Giannina Gaslini, Genova, Liguria, Italy

Edoardo La Porta,

Introduction

Melorheostosis is a rare bone dysplasia characterized by irregular hyperostosis of bones, diagnosed by typical radiological signs. Additionally, soft tissue changes have been described. We present a patient with melorheostosis and severe hypertension due to angioplasty-resistant renal artery stenosis (RAS) of the solitary kidney.

Case Description

A 7-years-old girl presented with severe hypertension. She had a history of stroke, absence of left kidney and scoliosis.
In 2022, due to severe RAS she underwent percutaneous cutting balloon angioplasty with improvement on blood pressure control. Few days later the occurrence of subacute thrombosis was treated by drug eluting stent. MRI imaging of the spine revealed evidence of bony aspects compatible with melorheostosis.
She was considered not suitable for vascular surgery and proposed to our Institution for nephrectomy or renal transplant.
In our Institute she was treated with 9 antihypertensive drugs without reaching pressure control. Kidney ultrasound demonstrated in-stent fracture with severe restenosis. Two additional percutaneous angioplasties were attempted with no significant improvement. Then we introduced ACE inhibitor (ACEi) titrated to benefit on blood pressure control without compromising kidney function. Her blood pressure is now acceptable.

Discussion

Melorheostosis is associated with different malformation of soft tissue: we present the first case of melorheostosis with RAS in solitary kidney patient.
RAS causes activation of RAAs to increase renal blood flow, leading to high blood pressure. The use of ACEi in patients with RAS is controversial: it acts directly on the causative mechanism of hypertension, but it leads to a reduction in the kidney blood flow, with the risk of developing acute kidney damage. Given the poor response to several angioplasties, we started with Ramipril at very low dose progressively increased until 0.11 mg/kg/die. She is treated now with 5 antihypertensive drugs, with better pressure control.