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Hypoechoic Renal Peripyramidal Rings in Primary Hyperoxaluria

Aye Erden, Suat Fitoz, Tuba Karagülle and Serdar Akyar

Ankara University Ibn-i Sina Hospital Samanpazari 06100, Ankara, Turkey

Primary hyperoxaluria is a rare autosomal recessive genetic disorder characterized by excessive synthesis and urinary excretion of oxalic acid. Nephrocalcinosis, which refers to pathologic deposition of calcium oxalate in renal parenchyma, and increased urinary excretion of oxalic acid result in progressive renal failure and nephrolithiasis, respectively [1].

Sonographic findings in nephrocalcinosis caused by hyperoxaluria include increased parenchymal echogenicity of the normal-size kidneys and loss of corticomedullary distinction. Acoustic shadowing arising from the intensely echogenic renal parenchyma may be detected [2]. Conventional radiographs and unenhanced CT scans are also valuable to visualize dense kidneys.

In a 6-year-old boy with a primary hyperoxaluria who underwent peritoneal dialysis, we observed hypoechoic rings around the renal pyramids in addition to the sonographic findings mentioned earlier (Fig. 3A). Unenhanced CT scans show these areas as peripyramidal hypodense ringlike regions (Fig. 3B).

View larger version (101K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3. —6-year-old boy with primary hyperoxaluria who underwent peritoneal dialysis. A, Sagittal sonogram of right kidney reveals marked increase in echogenicity of renal parenchyma. Note low echogenicity of peripyramidal regions and sinus structures.

View larger version (76K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3. —6-year-old boy with primary hyperoxaluria who underwent peritoneal dialysis. B, Unenhanced CT scan shows hypodense rims at corticomedullary junction and sides of pyramids in hyperdense renal parenchyma.

To our knowledge, there are few reports that include ringlike parenchymal echo changes in renal diseases [3, 4]. Päivänsalo et al. [3] described the hyperechogenic rings in the peripheries of renal medullary pyramids and proved this finding was nonspecific and poorly correlated with the severity of renal disease. However, to our knowledge no reports are available about hypoechoic peripyramidal rings. We think that these areas, which are distributed along the course of the peripyramidal vascularity (interlobar and arcuate vessels), may reflect the spared regions in a diffusely involved renal parenchyma by calcium oxalate crystals.

Further observations are required to clarify diagnostic importance of these areas during the progressive course of the disease.

References

1. Stull AM, Glass-Royal M. Primary oxalosis with renal osteodystrophy. AJR 1990;154:1334-1335[Medline]
2. Akhan O, Özmen MN, Coskun M, et al. Systemic oxalosis: pathognomonic renal and specific extra-renal findings on US and CT. Pediatr Radiol 1995;25:15-16[Medline]
3. Päivänsalo MJ, Kallioinen MJ, Merikanto JS, Jalovaara PK. Hyperechogenic "rings" in the periphery of renal medullary pyramids as a sign of renal disease. J Clin Ultrasound 1991;19:283-287[Medline]
4. Patriquin H, Robitaille P. Renal calcium deposition in children: sonographic demonstration of the Anderson-Carr progression. AJR 1986;146:1253-1256[Abstract/Free Full Text]

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This Article Figures Only Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Fitoz, S. Articles by Akyar, S. Search for Related Content PubMed PubMed Citation Articles by Fitoz, S. Articles by Akyar, S. Social Bookmarking                      What's this?