Horseshoe Kidney Clinical Presentation

Updated: Feb 25, 2020
  • Author: Nicholas N Tadros, MD, MCR; Chief Editor: Bradley Fields Schwartz, DO, FACS  more...
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Nearly one third of patients with a horseshoe kidney remain asymptomatic, and the horseshoe kidney is an incidental finding during radiological examination. Physical examination may reveal a midline lower-abdominal mass.

Symptoms, when present, are usually due to obstruction, stones, or infection. In children, urinary tract infection is the most common presenting symptom. Clinical signs of disease are similar to those that would be found in a normal kidney. However, symptoms may be vague. Instead of flank pain, abdominal pain, and gastrointestinal symptoms such as nausea, abdominal distension and fullness may predominate. The Rovsing sign, consisting of abdominal pain, nausea, and vomiting with hyperextension of the spine, is rare.

The horseshoe kidney may be at higher risk from blunt abdominal trauma because it is unprotected by the rib cage and may be compressed or fractured across the lumbar vertebral column by an abdominal blow. This can occur during a car crash when the victim is restrained by a seat belt— the kidney is compressed between the belt and the spine.

Associated genitourinary anomalies in horseshoe kidney are common and occur in as many as two thirds of patients. Vesicoureteral reflux is present in approximately half the patients. Ureteral duplication is present in 10%. Hypospadias or undescended testis is present in 4% of males. Bicornuate uterus or septate vagina is present in 7% of females.

Other anomalies may be present in other organ systems as well. In autopsy series, these abnormalities are more prevalent in children because some of the congenital anomalies associated with horseshoe kidney are incompatible with long-term survival. Therefore, the incidence of other anomalies is greater in those who die at birth or early infancy than in those who reach adulthood. These coexisting abnormalities in the cardiovascular, gastrointestinal, and skeletal systems occur in up to 85% of patients. These include ventriculoseptal defects, hemivertebrae with scoliosis, myelomeningocele, and colobomata of the iris. Horseshoe kidney is associated with known genetic syndromes, including Turner syndrome, oral-cranial-digital syndrome, and trisomy 18 (Edwards syndrome).

Autosomal-dominant polycystic kidney disease has also been found in the horseshoe kidney, with 20 such cases reported in the literature.