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Horseshoe Kidney P004

Last amended 
30 June 2015
Current RMA Instruments:
Reasonable Hypothesis SOP
31 of 2015
Balance of Probabilities SOP
32 of 2015
Changes from Previous Instruments:

SOP Bulletin 179

ICD Coding:
  • ICD-9-CM Codes: 753.3
  • ICD-10-AM Codes: Q63.11

This is a congenital malformation of the kidney with the kidneys joined across the midline by a bridge of functioning or non-functioning tissue.

Is specific diagnostic evidence required to apply the SOP? - Yes.

This diagnosis is based on MRI [magnetic resonance imaging] scan, CT [computerised axial tomogram] scan or ultrasound of the abdomen. However the relevance of the finding requires a specialist opinion from an urologist or nephrologist with recourse to renal ultrasound, intravenous pyelogram and renal function tests.

Are there sub-factors that require specific information? – No.

The only factor is for clinical worsening from inability to obtain appropriate clinical management. It is difficult to ascertain a clinical worsening of this disorder. See notes below.

Additional diagnoses covered by SOP
  • Nil
Conditions excluded from SOP
  • Renal pathology from other causes.
Unconfirmed diagnosis

If, after applying the above information, you are unable to confirm the diagnosis, you should then seek medical officer advice about further investigation.

Notes on appropriate clinical management

Worsening beyond the normal progression of the disease needs to be evident before a clinical worsening factor can be considered.

No treatment is generally required for the horseshoe kidney per se. But there may be associated abnormalities that may require treatment, such as vesicoureteric reflux, uteropelvic junction obstruction, ureteral duplication, ectopic ureter, ureterocele, and a retrocaval ureter.

The majority of patients with horseshoe kidneys are asymptomatic. In these patients, the horseshoe kidney is diagnosed by coincidence (eg, routine antenatal ultrasonography). However, some patients present with pain and/or hematuria due to obstruction or infection and renal calculi. 1

One-third to one-half of patients with horseshoe kidneys will have another congenital anomaly, particularly another urological abnormality (eg, VUR [vesicoureteric reflux]) or a genital anomaly, such as bicornuate and/or septate uterus in girls, and hypospadias and undescended testis in boys.

Horseshoe kidney can be a feature of many syndromes including genetic disorders such as Turner syndrome, and Trisomy 13, 18, and 21.

Note that it seems that obstruction and infection occurring in people with horseshoe kidney are more related to the other unrelated congenital anomalies of the ureteric system rather than with the horseshoe kidney itself.