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Horseshoe Kidney

Author: Abid Irshad, MD, Assistant Professor, Department of Radiology, Medical University of South Carolina
Coauthor(s): Susan Ackerman, MD, Director of Ultrasound, Associate Professor, Department of Radiology,
Medical University of South Carolina; James G Ravenel, MD, Associate Professor of Radiology, Vice Chair for
Education, Chief of Thoracic Imaging, Department of Radiology, Medical University of South Carolina
Contributor Information and Disclosures
Updated: Mar 1, 2010

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Introduction

Background
Congenital anomalies of the kidneys include a group of so-called fusion anomalies,
in which both kidneys are fused together in early embryonic life. Fusion anomalies of
the kidneys can generally be placed into 2 categories: (1) horseshoe kidney and its
variants and (2) crossed fused ectopia. Horseshoe kidney is probably the most
common fusion anomaly.

The term horseshoe kidney refers to the appearance of the fused kidney, which
results from fusion at one pole (see some examples in the images below). In more
than 90% of cases, fusion occurs along the lower pole. Technically, the term
horseshoe kidney is reserved for cases in which most of each kidney lies on one
side of the spine. It includes symmetric horseshoe kidney (midline fusion) or
asymmetric horseshoe kidney (L-shaped kidney). In the latter, the fused part, or
isthmus, lies slightly lateral to the midline (lateral fusion). Horseshoe kidney is
generally differentiated from crossed fused ectopia, in which both fused kidneys lie
on one side of the spine, and the ureter of the crossed kidney crosses the midline to
enter the bladder.
Plain radiograph of the abdomen shows calcific opacities in the region of left lower
renal pole. Note the reversed axis of the kidneys, which suggests horseshoe kidney.

Intravenous urogram (IVU) demonstrates horseshoe kidney. Note the malrotated


collecting systems on both sides. The lower pole calyx of the right kidney lies medial
to the ureter.

Axial computed tomography (CT) scan obtained through the abdomen after the
intravenous administration of contrast material. Fused kidneys are revealed, with a
parenchymal isthmus at the lower poles. Note the malrotated collecting system of
the left kidney, facing anterolaterally.

Recent studies
Glodny et al examined the radiologic findings of horseshoe kidneys and crossed
fused ectopias in 209 patients to assess the frequency and clinical significance of
associated anomalies and diseases. CT scanning was the most reliable imaging
modality for both horseshoe kidneys and crossed fusion ectopias, but individual
cases with complex anatomic configurations required special examination strategies.
Crossed fused ectopias differed anatomically from horseshoe kidneys in having a
lower position, greater axial rotation, smaller pelvic width, more caudal origin, and
fewer vessels. Children had higher rates of malformations than adults.1

In a retrospective analysis, Zumsteg et al reported on laparoscopic


heminephrectomy for benign anomalies, as compared with traditional open surgical
removal. Of 11 patients who underwent heminephrectomy, 8 had duplicated
collecting systems, 2 had horseshoe kidneys, and 1 had a cross-fused renal ectopic
kidney. There were no intraoperative complications, and all presenting symptoms
resolved with preservation of renal function. There were, however, 3 major and 2
minor postoperative complications, such as 50% loss of remaining ipsilateral renal
parenchyma, postoperative neuralgia and anejaculation, urinoma, and partial wound
separation.2

Pathophysiology
The development of the normal kidney depends on the union of ureteric buds from
the mesonephric ducts with the nephrogenic cords in the embryo. The union is
believed to occur around the fourth gestational week, normally occurring at the level
of the first or second sacral vertebral segment. Subsequent straightening of the hind
end of the embryo, along with differential growth of the developing pelvic structures,
leads to the ascent of both kidneys to their normal dorsolumbar regions between the
fourth and ninth weeks of gestation.

The abnormal fusion probably occurs at the 5- to 12-mm embryonic stage, when the
kidneys are in the true pelvis and the renal capsule has not yet matured. One
embryologic explanation regarding midline fusion is that, at that stage, abnormal
variation in growth, ventral flexion of the hind end of the embryo, or other variations
in the growth of pelvic structures may bring the metanephric blastemas (developing
kidneys) abnormally close together for a longer period; this proximity can lead to
fusion.

The explanation for lateral fusion is that, during early embryonic life, lateral flexion of
the lumbosacral spine may push one of the developing kidneys toward the midline.
This positioning can lead to asymmetric fusion. In later embryonic life, the ascent of
the fused kidney is hindered by the inferior mesenteric artery, with the isthmus of the
horseshoe kidney becoming trapped under it. Consequently, the horseshoe kidney
always lies at a position that is lower than normal. However, whether the cause is
abnormal fusion, abnormal migration of the posterior nephrogenic areas, or another
teratogenic factor is not yet clear.

Frequency
United States
Horseshoe kidney is the most common renal fusion anomaly, the incidence being
about 1 case per 400 persons worldwide. As reported, it can occur in a single
member of a set of identical twins and in both members. At present, no clear
evidence of a hereditary trait exists.

International

The incidence of horseshoe kidney is about 1 case per 400 persons worldwide.

Mortality/Morbidity
Complications of horseshoe kidney include the following:

• Ureteropelvic junction (UPJ) obstruction is a common complication, possibly


because of the high insertion of the ureter.
• Recurrent infections occur because of urine stasis and associated
vesicoureteric reflux.
• Recurrent stone formation related to UPJ obstruction or infection may occur.
• An increased risk of trauma to the isthmus exists because of its position
anterior to the spine.
• Horseshoe kidney may pose problems for surgeons during abdominal surgery
for other abdominal problems.
• Evidence indicates that an increased incidence of certain renal tumors is
associated with horseshoe kidney.

Horseshoe kidney may occur as an isolated anomaly or in association with other


congenital anomalies. The morbidity and mortality rates largely depend on whether it
is associated with other anomalies.

• Horseshoe kidney with other congenital anomalies


o About one third of cases of horseshoe kidney are associated with
other congenital anomalies, which include anomalies of the urogenital,
gastrointestinal, neurologic, and skeletal systems, as well as some
chromosomal abnormalities.
o Some of these anomalies are not compatible with life, whereas others
have a minor degree of morbidity.
o Depending on the severity of the associated anomalies, outcomes
vary, in more severe cases, from stillbirth to early neonatal death.
o When patients have minor associated anomalies, they may live
relatively healthy lives.
o In patients with other anomalies, the horseshoe kidney itself is usually
not the major cause of morbidity or mortality.
• Horseshoe kidney as an isolated entity
o About one third of patients with horseshoe kidney remain
asymptomatic.
o Horseshoe kidney is often an incidental finding at autopsy.
o The cause of morbidity in symptomatic patients is most commonly
related to recurrent urinary tract infection (UTI) and stones, which are
frequent.
o No significant decrease in the survival rate has been established in
association with this isolated anomaly.3

Sex
Renal fusion anomalies occur predominantly in males. The male-to-female ratio is
approximately 2:1 for horseshoe kidney and 6:1 for crossed fused ectopia.

Age
Clinically, this congenital anomaly is diagnosed in individuals of all ages;
horseshoe kidney is found prenatally, as well as in the elderly. However, because of
its association with other congenital anomalies, horseshoe kidney is more commonly
diagnosed in children.

Anatomy
In more than 90% of cases, fusion in horseshoe kidney occurs along the lower pole.
This region of fusion, called the isthmus, is usually composed of renal parenchymal
tissue. However, in many instances, it may consist of fibrous tissue. The isthmus can
be wide or narrow, depending on the degree of fusion. The isthmus usually lies
anterior to the aorta and inferior vena cava (IVC), and it is posterior to the inferior
mesenteric artery. In rare cases, however, the isthmus passes between, or even
posterior to, the aorta and IVC. The ureters usually pass anterior to the isthmus, and
they may have a high insertion point in the renal pelvis. The renal pelves are usually
malrotated and lie anteriorly or laterally.
In the midline fusion anomaly, the kidneys are symmetric, with each of the lower
poles of the kidneys converging toward the midline. In the lateral fusion anomaly,
one kidney is more vertical, while the other kidney is more horizontal; the isthmus
lies slightly toward one side. In rare cases, the upper poles fuse, reversing the
horseshoe appearance. In another rare event, the upper and lower poles will fuse,
producing a ringlike mass, a condition termed disc kidney, doughnut kidney, or
pancake kidney.

Blood vessel variations occur in horseshoe kidney. In about 30% of cases, blood is
supplied to each kidney by 1 renal artery. In other instances, 1 or both kidneys are
supplied by 2 or 3 renal arteries. The blood supply to the isthmus also varies. It may
come from the renal artery, or it may arise directly from the aorta, above or below the
isthmus. Occasionally, the blood supply arises from the common iliac, the external
iliac, or the inferior mesenteric arteries.

Presentation
Clinically, horseshoe kidney can be divided into 2 groups: horseshoe kidney with
associated anomalies and isolated horseshoe kidney with no associated anomalies.

Horseshoe kidney with associated anomalies

About one third of the patients with horseshoe kidney have associated anomalies.
These include multisystem abnormalities, such as urogenital anomalies (eg, UPJ
obstruction, vesicoureteric reflux, ureteral duplication, hypospadias, undescended
testis, ectopic ureter, retrocaval ureter, bicornuate and/or septate uterus).

Gastrointestinal (GI) abnormalities include anorectal malformations, such as


imperforate anus, malrotation, and Meckel diverticulum. Central nervous system
(CNS) anomalies, such as neural tube defects, may be seen. Skeletal anomalies
include rib defects, clubfoot, or congenital hip dislocation. Cardiovascular
abnormalities, such as a ventricular septal defect (VSD), occur in some patients.

Horseshoe kidney has also been found in association with some chromosomal
abnormalities, such as Turner syndrome and trisomy 18.

The clinical course largely depends on the nature of the anomalies, because
horseshoe kidney itself is relatively asymptomatic.

Isolated horseshoe kidney

In the pediatric clinical setting, about 90% of patients are asymptomatic, and the
most common presentation is UTI.

When symptoms are present, they are usually related to hydronephrosis, infection,
stone formation, or hematuria. The most common symptom is vague abdominal pain,
which may radiate to the back. Occasionally, nausea and vomiting may be reported.
Also, the so-called Rovsing sign (nausea, vomiting, and abdominal pain with
hyperextension of the spine) may be positive in some patients.

A small percentage of patients may have a palpable lump in the abdomen.

Horseshoe kidney has been reported to be associated with increased risk for renal
neoplasms, such as Wilms tumors, renal carcinoids, and transitional cell carcinoma.

Preferred Examination
Intravenous urography (IVU), computed tomography (CT) scanning, magnetic
resonance imaging (MRI), and scintigraphy depict horseshoe kidney with a high
degree of accuracy. For the purpose of diagnosis, IVU is usually the first-line
investigation, followed by CT scanning or scintigraphy in cases with doubtful
findings. Ultrasonography also is helpful, but it may have some technical limitations
(which are discussed in the next section).

Although MRI accurately reveals the anatomy associated with horseshoe kidney, it is
not generally used for diagnosis because of its high cost. MR angiography provides
additional information about the vascular anatomy. A voiding cystourethrogram is
usually required to evaluate associated vesicoureteric reflux. A diuretic renal
scintigram is helpful in differentiating obstructed and nonobstructed dilated collecting
systems. Angiography is usually reserved for presurgical planning to fully evaluate
the arterial supply pattern.

CT angiography scanning with 3-dimensional reconstruction also may reveal the


vascular anatomy and collecting system for presurgical planning.
Limitations of Techniques
Most of the time, IVU cannot be used to differentiate between a fibrous isthmus and
a parenchymal isthmus. Also, in many cases, the diagnosis of a horseshoe kidney is
difficult to make on the basis of only IVU findings. In these instances, CT scanning or
scintigraphy may be helpful.

Ultrasonography sometimes has technical limitations, especially in patients with a


large body habitus, in whom visualization of the isthmus may be difficult. Also,
horseshoe kidney may be missed on routine abdominal scans unless particular
attention is paid to ruling out this condition.

Differential Diagnoses

Other Problems to Be Considered


On images, the main differential diagnosis of horseshoe kidney includes another
fusion anomaly, crossed fused ectopia. However, in this latter condition, the fused
kidneys lie on the same side of the spine, and the ureter of the crossed kidney
crosses the midline to enter the bladder. However, this differentiation may not always
be possible.4

On IVUs, a malrotated or an ectopic kidney may sometimes be confused with


horseshoe kidney.

Gibbous deformity of the spine may alter the renal axis, which may then resemble
horseshoe kidney.

More on Horseshoe Kidney

Overview: Horseshoe Kidney


Imaging: Horseshoe Kidney
Follow-up: Horseshoe Kidney
Multimedia: Horseshoe Kidney
References
Further Reading
Next Page »

Horseshoe Kidney: Imaging
Author: Abid Irshad, MD, Assistant Professor, Department of Radiology, Medical University of South Carolina
Coauthor(s): Susan Ackerman, MD, Director of Ultrasound, Associate Professor, Department of Radiology,
Medical University of South Carolina; James G Ravenel, MD, Associate Professor of Radiology, Vice Chair for
Education, Chief of Thoracic Imaging, Department of Radiology, Medical University of South Carolina
Contributor Information and Disclosures
Updated: Mar 1, 2010

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Radiography

Findings
Plain radiographs may show low-lying renal outlines with an altered renal axis.
Usually, the kidneys follow the axis of the psoas muscles, with the lower poles lying
at a more lateral position than the upper poles. In horseshoe kidney, this axis is
reversed, with the lower poles lying closer to the spine. With plain radiographs alone,
however, the degree of confidence in this finding is usually low.

IVU usually reveals the classic findings associated with horseshoe kidney. Findings
on the initial tomogram may be deceptive because of the exclusion of the anteriorly
lying isthmus. Renal axis abnormalities are confirmed, as seen on the plain
radiographs. In midline fusion, the kidneys are symmetric, with the lower pole
calyces lying closer to or actually overlying the spine. The lower calyces are usually
medially rotated, and they may actually lie medial to the ureters. Some degree of
malrotation of the kidneys is usually present. A renal pelvis is often extrarenal and
large.

The degree of malrotation has been associated with the degree of fusion. If the
isthmus is narrow, the kidneys are usually less malrotated, with the pelvis lying
anteromedially in its near-normal position. In cases of a wide isthmus, the renal
pelves lie anteriorly or laterally. Associated UPJ obstruction may be present because
of the higher ureteric insertion point, which leads to delayed pelvic emptying. Ureters
may have the so-called flower-vase appearance, in which the upper ureters diverge
laterally over the isthmus and then converge inferiorly.

The L-shaped kidney's lateral fusion anomaly can also be readily appreciated on
IVUs. In this anomaly, one kidney has a relatively vertical position, while the other
kidney is relatively horizontal.

Degree of Confidence
The degree of confidence associated with IVU for the diagnosis of horseshoe kidney
is high in most cases. Sometimes, ascertaining the diagnosis on the basis of IVU
alone is difficult, and further workup — using CT scanning, MRI, or scintigraphy —
may be necessary. IVU is also a good modality for discovering associated findings,
such as the presence of stones, scarring, and duplex collecting systems.

False Positives/Negatives
Sometimes, malrotated kidneys can be mistaken for horseshoe kidneys. IVU does
not aid in the differentiation of a parenchymal isthmus from a fibrous isthmus.

Computed Tomography

Findings
Contrast-enhanced CT scanning has a high degree of accuracy in defining the
structural abnormalities of horseshoe kidney, including the degree and site of fusion,
the degree of malrotation, associated renal parenchymal changes (eg, scarring,
cystic disease), and collecting system abnormalities (eg, duplex system,
hydronephrosis). It can also be used to differentiate a parenchymal isthmus from a
fibrous isthmus and to show the relation of the isthmus to surrounding structures.

Although routine CT scanning may show the variant arterial supply, this is better
defined with CT angiography scanning with 3-dimensional reconstruction and volume
rendering. In cases of neoplasm associated with horseshoe kidney, the use of 3-
dimensional multisection helical CT scanning also has been advocated, because it
further clarifies the structural details.

Degree of Confidence
The degree of confidence associated with the use of CT scanning for diagnosing
horseshoe kidney is high. However, associated stone disease may be missed with
contrast-enhanced CT scanning alone, and nonenhanced CT scanning,
ultrasonography, or plain radiography may be required.

Magnetic Resonance Imaging

Findings
MRI has an advantage in depicting structural details because of its ability to permit
multiplanar imaging, but it is more costly than other examinations. However, an
added advantage may be obtained by using MR angiography to delineate the
vascular anatomy. MRI is probably the best modality to use in evaluating the extent
of renal tumors associated with horseshoe kidney.

Degree of Confidence
The degree of confidence connected with the use of MRI for the diagnosis of
horseshoe kidney, as well as for the purpose of defining associated structural
findings, is high. However, associated small stones may be missed on MRIs.
Ultrasonography

Findings
Ultrasonography can be useful for diagnosing horseshoe kidney. To establish the
diagnosis, the most important ultrasonographic findings are the presence of the
isthmus and its continuity with the lower poles. Other features, such as malrotation
and an altered renal axis, may be difficult to assess with ultrasonography. In cases in
which the isthmus is composed of only a thin, fibrous band, this midline soft tissue
may not be seen.

Various findings, such as a curved configuration of the lower poles, elongation of the
lower poles, and poorly defined lower poles, suggest the presence of horseshoe
kidney. Other associated findings, such as stones, hydronephrosis, and cortical
scarring, are reliably depicted on sonograms. Ultrasonography has also been useful
in the diagnosis of horseshoe kidney in utero.

Degree of Confidence
The degree of confidence depends on the visualization of the isthmus and the proof
of its continuity with the lower poles. In many patients, especially those with a large
body habitus, overlying bowel gas makes the acquisition of adequate scans difficult
for technical reasons. In cases in which the continuity of the poles with the isthmus
cannot be clearly demonstrated, the degree of confidence is low.

False Positives/Negatives
Occasionally, a midline soft-tissue mass over the spine (eg, a lymphomatous mass)
may be mistaken for an isthmus, especially if it extends laterally to the kidneys. If a
horseshoe kidney has a thin, fibrous isthmus, ultrasonography may produce a false-
negative result.

Nuclear Imaging

Findings
Scintigraphy best demonstrates the fusion if the isthmus consists of functioning
parenchymal tissue, because this imaging modality depends not only on the
structure of the tissue but also on the function of the tissue. Technetium-99m (99m Tc)
– labeled dimercaptosuccinic acid (DMSA) can be used to define the fused
segments, as well as the altered axis of both kidneys.

Many reports of horseshoe kidney exist. This condition is incidentally diagnosed on


bone scans,99m Tc-labeled red blood cell studies, or other nuclear medicine studies
obtained for reasons other than the evaluation of horseshoe kidney. The use of
mercaptoacetyltriglycine (MAG-3) with diuresis is helpful in differentiating
nonobstructed parts from obstructed parts of the collecting systems.

Degree of Confidence
Horseshoe kidney can be confidently diagnosed with scintigraphy, which reveals the
functioning parenchymal isthmus.
False Positives/Negatives
Occasionally, the hot isthmus of a horseshoe kidney can be mistaken for spinal
metastatic disease.

Angiography

Findings
Angiography is not normally performed to diagnose horseshoe kidney, but it is
performed to evaluate the vascular anatomy and its variations in a presurgical
setting. Angiograms may show 1, 2, or 3 renal arteries in either of the fused kidneys
and can reveal large variations in the blood supply of the isthmus, as discussed in
Anatomy. In cases of associated renal tumors, angiography is used to evaluate
tumor vascularity. Angiography is occasionally performed to check renal artery
stenosis in hypertensive patients who have horseshoe kidney.

More on Horseshoe Kidney

Overview: Horseshoe Kidney


Imaging: Horseshoe Kidney
Follow-up: Horseshoe Kidney
Multimedia: Horseshoe Kidney
References
Further Reading
« Previous Page

Horseshoe Kidney: Follow­up
Author: Abid Irshad, MD, Assistant Professor, Department of Radiology, Medical University of South Carolina
Coauthor(s): Susan Ackerman, MD, Director of Ultrasound, Associate Professor, Department of Radiology,
Medical University of South Carolina; James G Ravenel, MD, Associate Professor of Radiology, Vice Chair for
Education, Chief of Thoracic Imaging, Department of Radiology, Medical University of South Carolina
Contributor Information and Disclosures
Updated: Mar 1, 2010

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Four experts review metastatic GIST case
Connect with the GIST Exchange to view a new program on
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Intervention

Image-guided percutaneous nephrostomy can be performed to relieve


hydronephrosis that is associated with horseshoe kidney. Percutaneous stone
removal also may be achieved with image guidance.5,6,7,8,9

Horseshoe Kidney: Multimedia
Author: Abid Irshad, MD, Assistant Professor, Department of Radiology, Medical University of South Carolina
Coauthor(s): Susan Ackerman, MD, Director of Ultrasound, Associate Professor, Department of Radiology,
Medical University of South Carolina; James G Ravenel, MD, Associate Professor of Radiology, Vice Chair for
Education, Chief of Thoracic Imaging, Department of Radiology, Medical University of South Carolina
Contributor Information and Disclosures
Updated: Mar 1, 2010

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Metastatic GIST: New Case Study Program
Dr. Joaquina Baranda and three colleagues present the case of a 53-
year-old female with unresectable GIST to help you gain insight into
diagnosing and treating gastrointestinal stromal tumors.
Click here
Multimedia
Media file 1: Plain radiograph of the abdomen shows calcific
opacities in the region of left lower renal pole. Note the reversed axis
of the kidneys, which suggests horseshoe kidney.

(Enlarge Image)

Media file 2: Intravenous urogram (IVU) shows an altered renal


axis with medially directed lower renal poles, which suggests
horseshoe kidney. Also note the dilated collecting system of
the left kidney, resulting from a ureteropelvic junction
obstruction; this is a frequently associated finding.

(Enlarge Image)

Media file 3: Intravenous urogram (IVU) demonstrates


horseshoe kidney. Note the malrotated collecting systems on
both sides. The lower pole calyx of the right kidney lies medial
to the ureter.

(Enlarge Image)
Media file 4: Axial computed tomography (CT) scan obtained
through the abdomen after the intravenous administration of
contrast material. Fused kidneys are revealed, with a
parenchymal isthmus at the lower poles. Note the malrotated
collecting system of the left kidney, facing anterolaterally.

(Enlarge Image)

Media file 5: Computed tomography (CT) scan of the abdomen


obtained after the intravenous administration of contrast
material. The isthmus of a horseshoe kidney, consisting of
parenchymal tissue, is clearly demonstrated. Note the cortical
continuity of the fused kidneys.

(Enlarge Image)

Media file 6: Axial contrast-enhanced T1-weighted magnetic


resonance image shows the isthmus of a horseshoe kidney; it
consists of parenchymal tissue and lies anterior to the spine.

(Enlarge Image)
Media file 7: Transverse ultrasonogram of the abdomen
showing a soft-tissue hypoechoic mass (isthmus) that is
anterior to the spine and aorta and that unites the lower renal
poles.

(Enlarge Image)

Media file 8: Transverse slightly oblique ultrasonogram of the


right kidney, showing the lower pole of the right kidney; the
pole crosses over the spine, anterior to the aorta and inferior
vena cava.

(Enlarge Image)

Media file 9: Posterior technetium-99m methylene diphosphonate


nuclear medicine bone scan shows incidental findings that
suggest horseshoe kidney.

(Enlarge Image)

Media file 10: Angiogram shows incidental findings of a


horseshoe kidney. The lower poles are connected by a fibrous
isthmus.
(Enlarge Image)

Media file 11: Transverse sonogram of the abdomen


demonstrates a soft-tissue isthmus anterior to the spine.

(Enlarge Image)

Media file 12: Intravenous urogram (IVU) of a male patient displays


findings consistent with the presence of horseshoe kidney.

(Enlarge Image)

Media file 13: Ultrasonogram of a pediatric patient displays a


hypoechoic soft-tissue mass anterior to the spine. The finding
is consistent with the presence of horseshoe kidney.

(Enlarge Image)

More on Horseshoe Kidney

Overview: Horseshoe Kidney


Imaging: Horseshoe Kidney
Follow-up: Horseshoe Kidney
Multimedia: Horseshoe Kidney
References
Further Reading
« Previous Page
NEW CHAPTER

Horseshoe Kidney
Author: Robert C Allen, Jr, MD, Consulting Staff, Alaska Urological Associates
Contributor Information and Disclosures
Updated: Dec 9, 2008

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Introduction

The horseshoe kidney is the most common type of renal fusion anomaly. It consists
of two distinct functioning kidneys on each side of the midline, connected at the
lower poles by an isthmus of functioning renal parenchyma or fibrous tissue that
crosses the midline of the body.

Frequency
Horseshoe kidney occurs in 1 per 400-800 live births. The true incidence probably
lies somewhere between these two extremes. Horseshoe kidney is twice as common
in males as in females. No genetic determinant is known, although it has been
reported in identical twins and in siblings within the same family.

Etiology
Two theories regarding the embryogenesis of the horseshoe kidney have been
proposed. The classic teaching of mechanical fusion holds that the horseshoe kidney
is formed during organogenesis, when the inferior poles of these early kidneys touch,
fusing in the lower midline. The theory of mechanical fusion is valid for horseshoe
kidneys with a fibrous isthmus. Alternatively, more recent studies postulate that the
abnormal fusion of tissue associated with the parenchymatous isthmus of some
horseshoe kidneys is the result of a teratogenic event involving the abnormal
migration of posterior nephrogenic cells, which then coalesce to form the isthmus.
This teratogenic event may also be responsible for the increased incidence of related
congenital anomalies and of certain neoplasias, such as Wilms tumor and carcinoid
tumor associated with the isthmus of the horseshoe kidney.

Pathophysiology
By itself, the horseshoe kidney does not produce symptoms. However, by virtue of
its embryogenesis and anatomy, it is predisposed to a higher incidence of disease
than the normal kidney. The variable blood supply, presence of the isthmus, high
insertion point, and abnormal course of the ureters all contribute to these problems.
Because of these embryogenic and anatomical factors, the rates of hydronephrosis,
stone formation, infection, and certain cancers are higher, resulting in a diseased
horseshoe kidney (see image below).

Excretory urogram shows a horseshoe kidney with left hydronephrosis.

The most common associated finding in horseshoe kidney is ureteropelvic junction


(UPJ) obstruction, which occurs in up to 35% of patients. It causes most problems.
Obstruction is due to the high insertion of the ureter into the renal pelvis. The
crossing of the ureter over the isthmus may also contribute to obstruction.
Nonobstructive dilatation must be distinguished from obstructive dilatation using
diuresis radioisotope renal scans.

The prevalence of stones in the horseshoe kidney ranges from 20-60%. Stone
disease is thought to be due to the associated hydronephrosis or UPJ obstruction
that causes urinary stasis, which hinders stone passage. Metabolic factors, as in the
normal population, have also been suggested as contributing to stone formation in
these patients. The orientation of the calyces also impairs drainage, resulting in
stasis. These kidneys appear dilated or abnormal on most imaging studies, although
the radionuclide scans are generally accepted as being diagnostic.
Urinary stasis and stone disease also predispose the horseshoe kidney to infection,
which occurs in 27-41% of patients. Ascending infection from vesicoureteral reflux is
another cause of infection in the horseshoe kidney.

Certain cancers are more common in the horseshoe kidney. This is thought to be
due to teratogenic factors present at birth and the susceptibility of the diseased
horseshoe kidney to certain cancers. Renal cell carcinoma is the most common renal
cancer in horseshoe kidney, accounting for 45% of tumors. The incidence of renal
cell cancer in the horseshoe kidney is no different from that of the normal kidney.

Transitional cell cancer and sarcoma account for 20% and 7% of tumors,
respectively. The relative risk of transitional cell carcinoma in the horseshoe kidney
is increased 3- to 4-fold. This is thought to be due to chronic obstruction, stones,
and/or infection in the affected kidneys.

The incidence of both Wilms and carcinoid tumors is also higher in the horseshoe
kidney. Examination of these tumors may provide an insight into the development
and embryogenesis of the horseshoe kidney and the predilection of these two
tumors to form in the horseshoe kidney.

Wilms tumor accounts for 28% of malignant lesions. The relative risk of Wilms tumor
is increased 2-fold. Half of these arise from the isthmus.

Renal carcinoids are rare, with only 32 reported cases. Of the 32 cases, 5 of these
renal carcinoids arose in a horseshoe kidney. The relative risk of a carcinoid tumor in
a patient with a horseshoe kidney is 62 times that found in the normal population. Of
the 5 reported carcinoid tumors reported in patients with horseshoe kidneys, 3 have
originated in or have involved the isthmus. The location of these tumors in the
isthmus may be explained by the embryogenesis involving abnormal migration of
posterior nephrogenic cells, leading to the formation of the isthmus. This is a
teratogenic event, which may explain this increased incidence of tumor within the
isthmus. This theory may also explain the greater incidence of Wilms tumor in the
isthmus. When compared with carcinoid tumor arising in a normal kidney, those that
arise in a horseshoe kidney follow a more benign course.
Presentation
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 predisposed
to 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 occur in other organ systems. In autopsy series, these


abnormalities are more prevalent in children because 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 trisomies 18 and E.

Autosomal-dominant polycystic kidney disease has also been found in the


horseshoe kidney, with 20 such cases reported in the literature.

Relevant Anatomy

Horseshoe kidneys may be found at any location along the path of normal renal
ascent from the pelvis to the mid abdomen. The kidneys may be lower than normal
because the isthmus is tethered by the inferior mesenteric artery during renal ascent.
The isthmus usually lies anterior to the great vessels, at the level of the third to fifth
lumbar vertebra. Rarely, it is posterior to these vessels or runs between them.

The vascular supply is variable and originates from the aorta, the iliac arteries, and
the inferior mesenteric artery. Bilateral single renal hilar arteries occur in 30% of
cases, and various combinations of single and multiple renal hilar and isthmus
vessels are seen in 70% of cases. The isthmus of the kidney may not have a
separate blood supply or, in 65% of cases, is supplied by a single vessel from the
aorta. The blood supply to the isthmus may arise from the common iliac or inferior
mesenteric arteries.

The collecting system has a characteristic appearance on intravenous urography


because of an incomplete inward rotation of the renal pelvis, which faces anterior.
The axis of the collecting system is deviated inward at the lower poles because of
the lower pole's connection with the isthmus. The ureter may have a high insertion
point into the renal pelvis and may cross anteriorly over the isthmus as it descends
to the bladder. Rarely does the collecting system cross the isthmus to the
contralateral kidney.

Contraindications
In the presence of UPJ obstruction, symphysiotomy (division of the isthmus) was
once recommended routinely after pyeloplasty to improve drainage. However, this
procedure is associated with an increased risk of hemorrhage, fistula, and renal
infarction. Also, because of their abnormal vasculature, the kidneys return to their
original position after division of the isthmus. Because of this, symphysiotomy is
rarely, if ever, indicated in conjunction with pyeloplasty.

The presence of obstruction or hydronephrosis precludes treatment of kidney stones


using extracorporeal shockwave lithotripsy (ESWL).

More on Horseshoe Kidney

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References
Horseshoe Kidney: Workup
Author: Robert C Allen, Jr, MD, Consulting Staff, Alaska Urological Associates
Contributor Information and Disclosures
Updated: Dec 9, 2008

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Workup

Laboratory Studies

• Once a horseshoe kidney is diagnosed or suspected, further laboratory and


imaging evaluation should be performed to assess the status of the kidneys
and to look for treatable causes of renal pathology.
• Urinalysis with urine culture should be performed. Abnormalities of the urine
sediment should be evaluated as clinically indicated. Infection should be
treated.
• Serum chemistry with creatinine is recommended to determine baseline renal
function.

Imaging Studies

• Intravenous pyelography (IVP) protocol CT scanning (CT scanning of the


abdomen and pelvis, with and without intravenous contrast) is the best initial
radiologic study to determine anatomy and relative renal function. See CT
scan below.
o

This CT scan demonstrates the isthmus of a horseshoe kidney. Note


the uptake of contrast in the isthmus.

• Abdominal and pelvic CT scanning or renal ultrasonography is helpful to


screen for the presence of stones, masses, or hydronephrosis.

Other Tests

• Further studies are performed as indicated and tailored to the clinical


situation. These include dedicated CT scanning of the kidneys with and
without intravenous contrast, diuresis renal scanning to assess renal function
and drainage, and voiding cystourethrography to rule out concomitant
vesicoureteral reflux. The frequency of vesicoureteral reflux in association
with horseshoe kidney warrants voiding cystourethrography in all pediatric
patients.

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Horseshoe Kidney: Treatment
Author: Robert C Allen, Jr, MD, Consulting Staff, Alaska Urological Associates
Contributor Information and Disclosures
Updated: Dec 9, 2008

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Treatment

Medical Therapy
The horseshoe kidney is susceptible to medical renal disease. These diseases, if
present, are treated as indicated. A metabolic evaluation should be performed
because metabolic causes for kidney stone disease are no less common in the
patient with horseshoe kidney than in the general population with kidney stone
disease. Any identified metabolic abnormality should be treated. Metabolic
evaluation includes a 24-hour stone risk assessment and serum studies, including
calcium, uric acid, and phosphorous.

Surgical Therapy
Surgical treatment is based on the disease process and standard surgical
indications. The anomalous vascular supply to the kidney should be kept at the
forefront of the surgeon's mind when planning the surgical approach. Generally, the
midline abdominal incision provides access to both sides of the horseshoe kidney
and vessels.

Ureteropelvic junction obstruction

UPJ obstruction is usually treated with open pyeloureteroplasty or


ureterocalicostomy. With the advent of and surgeon familiarization with laparoscopic
techniques, the preferred approach has become laparoscopic dismembered
pyeloplasty. More recently, robotic dismembered pyeloplasty using the da Vinci
surgical system has been successfully performed.1,2 Endopyelotomy is an option but
yields success rates that are inferior to those of open or laparoscopic techniques.3

In bilateral UPJ obstruction, the midline transperitoneal incision provides access to


both sides of the horseshoe kidney and the vessels. Symphysiotomy (division of the
isthmus) is controversial and was recommended routinely after pyeloplasty to
improve drainage. However, it is associated with an increased risk of hemorrhage,
fistula, and renal infarction. In addition, after division of the isthmus, the kidneys,
because of their abnormal vasculature, return to their original position. Therefore,
symphysiotomy is rarely, if ever, indicated in conjunction with pyeloplasty.

Kidney stones

Kidney stones can be treated with ESWL, endoscopy, or open surgery. If pyeloplasty
is to be performed, stones can be removed concomitantly with a flexible
nephroscope and stone basket.4 Treatment decisions and indications are similar to
those for a normal kidney. The presence of untreated obstruction or hydronephrosis
precludes ESWL. Concomitant percutaneous nephrostolithotomy with
endopyelotomy has been used successfully in the treatment of stones with
obstruction. Alternatively, successful retrograde therapy of the UPJ with an
"Accusize" cutting-balloon dilator followed by ESWL has also been performed.

Renal tumors

Guided by the angiographic findings, interruption of the blood supply of the tissue to
be resected is the first step. This prevents significant hemorrhage during dissection
and tissue removal. In cancer surgery, the isthmus usually needs to be divided to
gain access to the tumor and surrounding lymph nodes.

Abdominal aneurysmectomy

Problems may arise when operating on the great vessels, eg, with abdominal aortic
aneurysm repair. Horseshoe kidney complicates aortic aneurysm surgery in 1 out of
200 cases. The most important aspect of abdominal aneurysm repair in association
with horseshoe kidney is appropriate surgical management of the common renal
artery anomalies. Survival in these patients is predicated on preservation of renal
function. Renal artery continuity can be established via branch grafts or
reimplantation into the aortic graft. The approach can be midline-abdominal or
retroperitoneal through a low-left thoracoabdominal incision. Successful endoluminal
transfemoral repair via stent placement has also been described.

Renal transplantation

Horseshoe kidneys can be used for transplantation. They can be transplanted into a
single recipient en bloc or can be divided and transplanted into two individuals.
Dividing the isthmus can increase the risk of urinary fistula.

The donor’s medical history must be obtained to preclude complications such as


hydronephrosis, renal calculi, and urinary tract infection. The decision to transplant a
horseshoe kidney en bloc depends on renal isthmus morphology and vascular
anatomy, as well as the medical and functional status of the kidney.

Preoperative Details
Because of the anomalous and variable vascular supply to the horseshoe kidney,
authorities recommend preoperative arteriography to delineate the vascular anatomy
in addition to the routine diagnostic procedures. Accessory and aberrant arteries to
the parenchyma and the tumor are the rule.

Intraoperative Details
Because of the anomalous vasculature, the blood supply to the kidney must be
identified and preserved.

Follow-up
For excellent patient education resources, visit eMedicine's Kidneys and Urinary
System Center. Also, see eMedicine's patient education article Kidney Stones.

More on Horseshoe Kidney

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Horseshoe Kidney: Follow­up
Author: Robert C Allen, Jr, MD, Consulting Staff, Alaska Urological Associates
Contributor Information and Disclosures
Updated: Dec 9, 2008

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Outcome and Prognosis

The horseshoe kidney does not complicate pregnancy or delivery. Importantly, note
that the presence of the horseshoe kidney alone does not affect survival. As
mentioned above, the horseshoe kidney does have a higher propensity to become
diseased. Therefore, survival depends on the disease process that the affected
horseshoe kidney may harbor or develop.

Future and Controversies

Despite the increased incidence of Wilms tumor in children with horseshoe kidney
and carcinoid tumor in adults with horseshoe kidney, no recommendations regarding
periodic surveillance to detect occult malignancies have been made.

Performing periodic renal ultrasonography in children with horseshoe kidney seems


prudent for early detection of Wilms tumor. Periodic renal ultrasonography in the
adult may also be prudent, especially in patients with hydronephrosis. All patients
with horseshoe kidneys and stones should undergo 24-hour urine tests for kidney
stone prophylaxis. Otherwise, surveillance should be performed as indicated based
on the clinical situation.

More on Horseshoe Kidney

Overview: Horseshoe Kidney


Workup: Horseshoe Kidney
Treatment: Horseshoe Kidney
Follow-up: Horseshoe Kidney
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References
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