Bilateral malrotated kidneys with left partial duplication and right pelviureteric junction stenosis with staghorn calculus

Case contributed by Muhammad Shoyab
Diagnosis certain


Dull pain in right flank and iliac fossa pain for several years.

Patient Data

Age: 80 years
Gender: Male

The right kidney is grossly hydronephrotic, with severe thinning of its cortex, and minimal or no excretion of contrast for up to 12 hours.

The right renal pelvis is located at L4 level, having a large staghorn calculus impacted inside. It is directed inferomedially instead of posteromedially. Multiple smaller calculi are floating in the right renal calyces.

Multiple aberrant vessels arising from abdominal aorta enter the upper and lower poles of right kidney.

The left kidney is duplicated, with two hila having discrete vessels arising from the aorta and inferior vena cava. Its upper moiety hilum is directed anterolaterally, and lower moiety hilum is directed laterally. Its pelves unite at the level of the pelviureteric junction, forming a single ureter, which is normal in course, caliber and configuration.

Case Discussion

This is a case that demonstrates multiple congenital abnormalities in the same patient, along with their long-term sequelae.

It is likely that the PUJ obstruction is secondary to the malrotation and/or the crossing vessels, and the staghorn calculus is a consequence of the PUJ obstruction. In retrospect, such sequelae may have remained unforeseen even if the malrotation had been diagnosed earlier.

Similar to this patient, many other patients of abnormal renal configuration remain undiagnosed or unevaluated, due to the vagueness and fluctuating nature of their symptoms, 1 accompanied by intermittent phases of no or non-specific imaging findings 1. In many cases, undetected renal abnormalities have culminated in dire consequences 2 like nephrectomy, renal pelvis rupture 3, etc.

Hence, it has been advised to apply a high index of suspicion, 4 and to defer or review imaging during phases of acute symptoms 1

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