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Horseshoe kidney occurs in about one in 500 children. It occurs during fetal development as the kidneys move into their normal position in the flank area (area around the side, just above the waist). With horseshoe kidney, however, as the kidneys of the fetus rise from the pelvic area, they fuse together at the lower end or base. By fusing, they form a "U" shape, which gives it the name "horseshoe."
One-third of individuals with horseshoe kidney will have at least one other anomaly or complication involving the cardiovascular system, the central nervous system, or the genitourinary system, such as the following:
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- kidney stones - crystals and proteins that form stones in the kidney that may lead to a urinary tract obstruction.
- hydronephrosis - enlargement of the kidneys that usually results from a urinary tract obstruction.
- Wilm's tumor - an embryonic tumor of the kidneys that usually occurs during early childhood.
- renal cancer, or polycystic kidney disease
- hydrocephaly and/or spina bifida
- various cardiovascular, gastrointestinal findings (i.e., anorectal malformations, malrotated bowel), or skeletal problems (i.e., cleft lip/palate, clubfoot, polydactyly)
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Horseshoe kidney can occur alone or in combination with other disorders. The following are the most common disorders seen with horseshoe kidney:
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- Turner syndrome - Turner syndrome is a genetic disorder seen in girls that causes them to be shorter than others and to not mature sexually as they grow into adulthood. The severity of these problems varies among affected individuals. Other health problems may also be present involving the heart or renal system (kidneys, etc.). Turner syndrome is caused by the presence of a single X sex chromosome, rather than a pair. Sixty percent of girls with Turner syndrome have horseshoe kidneys.
- Trisomy 18 - Trisomy 18 is caused by the presence of three #18 chromosomes, rather than a pair. Trisomy 18 is a serious, usually fatal, chromosome abnormality involving defects in nearly all organ systems, including horseshoe kidney in 20 percent of children affected.
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About one-third of children will have no symptoms. The following are the most common symptoms of horseshoe kidney. However, each individual may experience symptoms differently. Symptoms may include:
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- urinary tract infection - urinary tract infections are usually uncommon in children under five years and unlikely in boys at any age.
- kidney stones - if the stones remain in the kidney, your child may have no symptoms. If the stones pass through the urinary tract, the following symptoms may be present:
- agonizing flank (around the side, just above the waist) pain
- hydronephrosis - hydronephrosis occurs when there is a urinary tract obstruction and the kidney(s) become enlarged and potentially damaged. Symptoms of hydronephrosis may include the following:
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The symptoms of horseshoe kidney may resemble other conditions or medical problems. Always consult your child's physician for a diagnosis.
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In a child without symptoms, diagnosis or treatment may not be necessary. If your child is having any of the mentioned complications, your child's physician may order one or more of the following diagnostic tests:
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- renal ultrasound - a diagnostic imaging technique that uses high-frequency sound waves and a computer to create images of blood vessels, tissues, and organs. Ultrasounds are used to view internal organs as they function, and to assess blood flow through various vessels.
- voiding cystourethrogram (VCUG) - a specific x-ray that examines the urinary tract. A catheter (hollow tube) is placed in the urethra (tube that drains urine from the bladder to the outside of the body) and the bladder is filled with a liquid dye. X-ray images will be taken as the bladder fills and empties. The images will show if there is any reverse flow of urine into the ureters and kidneys.
- intravenous pyelogram (IVP) - a diagnostic imaging technique which uses an x-ray to view the structures of the urinary tract. An intravenous contrast of dye is given so that the structures can be seen on film. An IVP also reveals the rate and path of urine flow through the urinary tract.
- blood tests (to determine how well the kidneys may be functioning)
- urine tests (including a culture)
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In a child without symptoms, treatment may not be necessary. If your child has any of the mentioned complications, he/she may require supportive treatment, which means his/her symptoms will be treated, but there is no cure for the condition.
Specific treatment for horseshoe kidney will be determined by your child's physician based on:
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- your child's age, overall health, and medical history
- the extent of the disease
- your child's tolerance for specific medications, procedures, or therapies
- expectations for the course of the disease
- your opinion or preference
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Your child may be referred to an urologist (a physician who specializes in disorders and care of the urinary tract and the male genital tract) and/or a nephrologist (a physician who specializes in disorders or diseases of the kidney) for evaluation.
Treatment may include:
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- antibiotics (to treat an underlying infection)
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- antibiotics (to treat an underlying infection)
- surgical intervention (for symptomatic kidney stones)
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If your child has hydronephrosis, multiple treatment options may be discussed including non-surgical or surgical options.
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Horseshoe Kidney
