Abnormalities in renal fusion represent a subset of congenital anomalies of the kidneys and urinary tract (CAKUT). Horseshoe kidneys (HSKs) are the fusion of kidneys at their lower poles. It is the most common form of CAKUT. Symptoms are usually subtle. The diagnosis is usually made incidentally during childhood. Rarely does an HSK become symptomatic later in life. We present the case of an 88-year-old female with a history of HSK who presented to the emergency department (ED) with a three-week history of left-sided flank pain, intermittent nausea, and reduced urine output. Her inpatient workup included imaging that revealed an HSK and bilateral hydronephrosis, which was more prominent on the left. The onset of symptoms for an HSK late in life is extremely rare.

Bardet-Biedl syndrome (BBS), also known as Laurence-Moon-Bardet-Biedl syndrome, is a unique autosomal recessive genetic disorder that involves multiple organ systems with an incidence under 1/100,000 in Europe and the USA. We present a case of a 27-year-old male with BBS and a past medical history of hypertension. He was diagnosed with BBS when he was a child. His physical examination showed polydactyly in the feet. His renal ultrasound showed the left kidney with a double collecting system and measured 1.9 × 6.1 × 3.6 cm and extended from the left upper quadrant to the left lower quadrant. His CT of the abdomen showed a horseshoe-shaped kidney with right moiety. Renal abnormalities in BBS have been identified recently. BBS is also associated with various cardiac manifestations such as patent ductus arteriosus, cardiomyopathies, and valvular diseases. BBS requires multidisciplinary management and a close follow-up with a nephrologist to decrease morbidity and mortality. Genetic and molecular mapping of this disorder will aid the understanding of congenital renal ciliopathies.

Horseshoe kidney is a congenital anomaly, which consists of fusion of the lower poles of the kidneys. Cancer in a horseshoe kidney is common, possibly because of the increased risk of chronic obstruction, renal calculi, and recurrent urinary infection. We report a case of a 64-year-old male with a horseshoe kidney who presented to our hospital with gross hematuria and flank pain, which was highly suggestive of pyelonephritis. Comprehensive workup and imaging were performed and showed an extremely rare form of tumor consisting of three histological variants: squamous, glandular, and sarcomatoid. To the best of our knowledge, this is the first case reported with these three histological variants in a horseshoe kidney.

BACKGROUND: Horseshoe Kidney (HSK) is probably the most common of all renal fusion abnormalities. However the association of Abdominal aortic aneurism (AAA) and HSK is rare, and occurred in 0,12% of patient affected by AAA.
METHODS: We present a patient with the concomitant presence of AAA and HSK treated by open surgical repair with a transperitoneal approach without section of the isthmus with great outcomes after surgery.
CONCLUSIONS: The management of AAA associated with HSK presents a special challenge during vascular surgery, given the close spatial relationship and the frequent renal arterial variations that accompanies HSK.
CONCLUSIONS: Diagnosis and a well designed surgical strategy are required to avoid surgical post operative complications.