0022-5347/78/1192-0188$02.00/0 Vol. 119, February Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright © 1978 by The Williams & Wilkins Co.
CROSSED RENAL ECTOPIA FRAY F. MARSHALL
AND
MATTHEW T. FREEDMAN
From the James Buchanan Brady Urological Institute and the Department of Radiology, The Johns Hopkins Hospital and the Division of Urology, Baltimore City Hospitals, Baltimore, Maryland
ABSTRACT
Crossed renal ectopia is an unusual congenital anomaly, probably produced by abnormal development of the ureteral bud. The deformity itself produces no symptoms and the clinical presentation generally is for obstruction and infection. In our series the adult patients presented most commonly with urinary tract infections, while the pediatric patients presented most often with multiple congenital anomalies, especially of the skeletal system. Crossed renal ectopia is a congenital anomaly in which a ureter crosses the midline from a kidney on 1 side to its vesical orifice on the opposite side. An incidence has been reported of approximately 1 in 7,000 autopsies. 1 The subject Case -Pt. -Age-Sex-Race No. (yrs.)
History
symptoms unrelated to the anomaly. On physical examination 3 of the 5 adults had an abdominal mass. Only 1 patient was hypertensive. One patient (case 4) required a lower nephrectomy for pyonephrosis (fig. 1). The renal infection in 1
TABLE 1. Crossed renal ectopia in adults Physical ExLaboratory X-ray Findings amination Findings
IVP and retrograde pyelogram-rt. crossed renal ectopia IVP and retroBP 150/90, Serum urea large prosnitrogen grade pyelotate normal gram - lt. crossed fused renal ectopia IVP-calicedasis BP 110/65 Serum urea nitrogen 14, with 2 calculi in calcium 12.6 calices of lower kidney, It. crossed fused renal ectopia BP 110/60, IVP-lt. crossed Serum urea mass, puru- nitrogen 12 fused renal eclent draintopia age rt. lower quadrant Retrograde pyeloBP 205/125, mass rt. gram-lt. crossed unfused lower quadrenal ectopia rant
yrs., 210/140
BP
Normal
2-WD-77-M-W
Obstructive symptoms
Low sacral spina Transurethral re- 1 yr., asymptobifida, degenersection bladder matic ative changes neck
3-TC-64-M-B
Pneumonia, 21 years earlier had pyeloplasty on It. crossed fused ectopia
4-KB-44-F-W
Rt. flank pain and pyonephrosis
5-0C-44-F-W
Shortness of breath
The hospital records from 1956 to 1976 were reviewed. Crossed renal ectopia was diagnosed in 18 patients but some cases were documented poorly. Crossed renal ectopia was substantiated in 10 patients by exploration, retrograde pyelography or clearly identified ureters on excretory urography (IVP). These 5 adults and 5 children are described. Although not available in 2 cases the radiographic studies of the remaining patients were reviewed, especially for skeletal anomalies. In each case the presentation, physical findings, associated anomalies and radiographic findings were studied. RESULTS
The 5 adults ranged in age from 20 to 77 years (table 1). Two presented with urinary tract infections directly related to the crossed renal ectopia, while the other adults had Accepted for publication May 27, 1977.
188
Antibiotics
17
Lt. flank pain and BP 130/60, Serum urea urinary tract inmass lt. nitrogen normal fection lower quadrant
MATERIALS AND METHODS
I
Followup
Treatment
1-MK-20-F-B
has been reviewed extensively in the past. 2, 3 The complications of the anomaly, usually obstruction and infection, dictate its management rather than the anomaly itself.
f
Skeletal
Normal except for Antibiotics degenerative changes
Normal
Rt. heminephrectomy oflower kidney
Never returned
1 yr., asymptomatic
Lost to followup
Normal
patient cleared with antibiotics. Of the remaining patients the crossed renal ectopia was an incidental finding. One patient had skeletal anomalies. The crossed renal ectopia was fused in 4 cases, with the lower pole ureter traversing the midline to insert on the opposite side (fig. 2). There was 1 case of probable unfused crossed renal ectopia documented by retrograde pyelography (fig. 3). The 3 boys and 2 girls ranged in age from 2 to 17 years. They presented with either urinary tract symptoms or multiple congenital anomalies (table 2). An imperforate anus was present in 2 patients and 1 patient had vaginal agenesis. All 3 of the patients with roentgenograms available for review had multiple skeletal anomalies (figs. 4 and 5). Radiographic studies revealed the typical crossed fused renal ectopia in 4 patients. Case 6 was unusual because 3 ureters emanated from the crossed ectopia, with 2 of these crossing the midline (fig. 6). A voiding cystourethrogram in 1 patient demonstrated unilateral reflux (case 10). DISCUSSION
The differential diagnosis of crossed renal ectopia includes solitary kidney, horseshoe kidney, ipsilateral double kidney
CROSSED RENAL ECTOPIA
189
Fm. 3. Retrograde pyelograms of unfused crossed renal ectopia
Fm. 1. Pyonephrosis of lower non-visualized renal segment with deviation of ureter from upper renal segment.
Fm. 2. Typical case of crossed fused ectopia with demonstration of crossed ureter from lower pole. A, IVP. B, retrograde pyelogram. and massive displacement of the kidney. Retrograde pyelography often is required to delineate clearly the anatomy. Crossed fused ectopia with the lower ureter traversing the midline is the most common anomaly. 3 A variety of forms may include unilateral fused, sigmoid, lump, disk and Lshaped kidneys. 2 The cases presented herein were unilaterally fused, except 1 case of unfused kidney. Unfused crossed ectopia is much less common4 and crossed renal ectopia of a solitary kidney is even more rare. 5 , 6 Bilateral crossed renal ectopia has been reported in several cases. 7 There is no definite pattern of associated congenital anomalies with crossed renal ectopia, which suggests that the lesion is an acquired defect. The association with vaginal
Fm. 4. Sacral and pelvic bony anomalies with crossed ectopia in child. agenesis 8 and undescended testis 3 has been reported previously. Congenital defects are of many types but skeletal anomalies of the bony pelvis and lumbar spine do appear frequently. It has been suggested that a skeletal defect with rotational deformity of the fetus might contribute to the genesis of crossed renal ectopia. There were some significant skeletal anomalies among our patients but 4 of the 5 adults had no such abnormalities. An insult to the fetus at 4 to 5 weeks of gestation could easily be reflected in urinary and skeletal abnormalities. The association of skeletal anomalies,
190
MARSHALL AND FREEDMAN TABLE
Case -Pt. -Age- Sex-Race No. (yrs)
History
2. Crossed renal ectopia in children
Associated Anomalies
Laboratory Findings
X-Ray Findings
Followup fol-
Lost to lowup
fol-
Multiple congenital anomalies
7-BT-?-F-?
Enuresis
8-WC-17-F-W
Primary amen- Vaginal agenesis 1 yr., asymptomatic orrhea Lassitude, he11.5 yrs., maturia after asymptotrauma matic Multiple anom- Tracheoesophageal Hypoplasia lt. femur, ti- Creatinine 0.6, se- IVP and retrograde 4 mos. alies fistula, imperfor- bia, pelvis; anomalies of rum urea nitropyelogram-lt. ate anus, dextrochest wall cervical gen 21 crossed fused ectopia cardia, bilateral spine, sacrum Voiding cystourethrocryptorchidism, gram- reflux on rt. hypospadias side
10-JI-2-M-W
Scoliosis, dysplasia femo- Serum urea nitro- !VP-triple collecting ral head, separation pugen 20 system, 2 ureters enhie symphysis, hypoplater on It., 1 on rt., It. sia lt. femur, lt. belowcrossed fused ectopia knee amputation 4 lumbar vertebrae, low Retrograde lumbar and sacral scopyelogram-lt. crossed fused ectopia liosis, L4 and sacrum 1 cm. separation of lamina compatible with meningocele Not reviewed IVP- lt. crossed fused renal ectopia Not reviewed Serum urea nitro- IVP - It. crossed fused gen normal ectopia
Lost to lowup
6-JS-8-M-W
9-CM-9-M-W
Imperforate anus
Skeletal Findings
Fm. 5. Retrograde catheters in girl with crossed ectopia and sacral deformity.
particularly spinal deformities with genitourinary anomalies, is well recognized. 9, 10 In a study of 85 children with congenital scoliosis or kyphosis 3 cases of crossed renal ectopia were found. 10 Therefore, skeletal anomalies do not seem to be the cause of crossed renal ectopia. The etiology of crossed renal ectopia remains unproved but a ureteral anomaly is the most attractive hypothesis. Many bizarre cases of crossed renal ectopia have been reported, including bilateral crossed renal ectopia and 1 with multiple ureters (as in case 6). Abnormal development of the ureteral bud arising in close proximity to the metanephric blastema seems to be a more reasonable explanation than abnormal tortuous migration of the metanephros itself. In the presence of calculus disease renal pain is referred to the side of the kidney but ureteral pain is referred to the contralateral side or side of the ureteral orifice. 11 It would appear that the innervation crosses with the ureter, which supports the concept of abnormal ureteral migration. However, McDonald and McClellan did report sigmoid kidneys with pain referred to the contralateral side. This entity may be closer to a horseshoe kidney than a true crossed ectopia. Vascular impe-
Fm. 6. Crossed renal ectopia with 3 ureters (IVP)
diments also have been suggested as etiologic agents in renal fusion anomalies. Renal fusion may occur in normal embryologic development and this phenomenon does not appear secondary to vascular obstruction. 12 Urinary tract problems associated with crossed renai ectopia include calculi, obstruction and reflux. These problems can require operation. Reflux was demonstrated in 14 of 16 patients in 1 study on renal ectopia and renal fusion in children. 13 It is not surprising that if the ureter is abnormal
CROSSED REN AL ECTOPIA
proximally, it also may be abnormal distally. Only 1 voiding cystourethrogram was available in our series but it did show unilateral reflux. REFERENCES
1. Kretschmer, H. L.: Unilateral fused kidney. Surg., Gynec. &
Obst., 40: 360, 1925. 2. Abeshouse, B. S.: Crossed ectopia with fusion: review of literature and a report of 4 cases. Amer. J. Surg., 73: 658, 1947. 3. McDonald, J. H. and McClellan, D. S.: Crossed renal ectopia. Amer. J. Surg., 93: 995, 1957. 4. Arduino, L. J.: Crossed renal ectopia without fusion. J. Urol., 93: 125, 1965. 5. Marshall, V. F. and Keuhnelian, J. G.: Crossed ureteral ectopia with solitary kidney. J. Urol., 110: 176, 1973. 6. Tanenbaum, B., Silverman, N. and Weinberg, S. R.: Solitary crossed renal ectopia. Arch. Surg., 101: 616, 1970.
191
7. Beer, E. and Ferber, W. L. F.: Crossed renal ectopia (unilateral, fused or elongated kidney). With report of fourteen cases clinically diagnosed and two cases encountered at autopsy during the past eighteen years. J. Urol., 38: 541, 1937. 8. Tabrisky, J. and Bhisitkul, I.: Solitary crossed ectopic kidney with vaginal aplasia: a case report. J. Urol., 94: 33, 1965. 9. Mecklenburg, R. S. and Krueger, P. M.: Extensive genitourinary anomalies associated with Klippel-Feil syndrome. Amer. J. Dis. Child., 128: 92, 1974. 10. Vitko, R. J., Cass, A. S. and Winter, R. B.: Anomalies of the genitourinary tract associated with congenital scoliosis and congenital kyphosis. J. Urol., 108: 655, 1972. 11. Romans, D. G., Jewett, M. A. S. and Robson, C. J.: Crossed renal ectopia with colic. A clinical clue to embryogenesis. Brit. J. Urol., 48: 171, 1976. 12. Friedland, G. W. and de Vries, P.: Renal ectopia and fusion. Embryologic basis. Urology, 5: 698, 1975. 13. Kelalis, P. P., Malek, R. S. and Segura. J. W.: Observations on renal ectopia and fusion in children. J. Urol., 110: 588, 1973.
®'41/ii@
THE JOURNAL OF UROLOGY
Copyright © 1978 by The Williams & Wilkins Co.
CROSSED RENAL ECTOPIA FRAY F. MARSHALL
AND
MATTHEW T. FREEDMAN
From the James Buchanan Brady Urological Institute and the Department of Radiology, The Johns Hopkins Hospital and the Division of Urology, Baltimore City Hospitals, Baltimore, Maryland
ABSTRACT
Crossed renal ectopia is an unusual congenital anomaly, probably produced by abnormal development of the ureteral bud. The deformity itself produces no symptoms and the clinical presentation generally is for obstruction and infection. In our series the adult patients presented most commonly with urinary tract infections, while the pediatric patients presented most often with multiple congenital anomalies, especially of the skeletal system. Crossed renal ectopia is a congenital anomaly in which a ureter crosses the midline from a kidney on 1 side to its vesical orifice on the opposite side. An incidence has been reported of approximately 1 in 7,000 autopsies. 1 The subject Case -Pt. -Age-Sex-Race No. (yrs.)
History
symptoms unrelated to the anomaly. On physical examination 3 of the 5 adults had an abdominal mass. Only 1 patient was hypertensive. One patient (case 4) required a lower nephrectomy for pyonephrosis (fig. 1). The renal infection in 1
TABLE 1. Crossed renal ectopia in adults Physical ExLaboratory X-ray Findings amination Findings
IVP and retrograde pyelogram-rt. crossed renal ectopia IVP and retroBP 150/90, Serum urea large prosnitrogen grade pyelotate normal gram - lt. crossed fused renal ectopia IVP-calicedasis BP 110/65 Serum urea nitrogen 14, with 2 calculi in calcium 12.6 calices of lower kidney, It. crossed fused renal ectopia BP 110/60, IVP-lt. crossed Serum urea mass, puru- nitrogen 12 fused renal eclent draintopia age rt. lower quadrant Retrograde pyeloBP 205/125, mass rt. gram-lt. crossed unfused lower quadrenal ectopia rant
yrs., 210/140
BP
Normal
2-WD-77-M-W
Obstructive symptoms
Low sacral spina Transurethral re- 1 yr., asymptobifida, degenersection bladder matic ative changes neck
3-TC-64-M-B
Pneumonia, 21 years earlier had pyeloplasty on It. crossed fused ectopia
4-KB-44-F-W
Rt. flank pain and pyonephrosis
5-0C-44-F-W
Shortness of breath
The hospital records from 1956 to 1976 were reviewed. Crossed renal ectopia was diagnosed in 18 patients but some cases were documented poorly. Crossed renal ectopia was substantiated in 10 patients by exploration, retrograde pyelography or clearly identified ureters on excretory urography (IVP). These 5 adults and 5 children are described. Although not available in 2 cases the radiographic studies of the remaining patients were reviewed, especially for skeletal anomalies. In each case the presentation, physical findings, associated anomalies and radiographic findings were studied. RESULTS
The 5 adults ranged in age from 20 to 77 years (table 1). Two presented with urinary tract infections directly related to the crossed renal ectopia, while the other adults had Accepted for publication May 27, 1977.
188
Antibiotics
17
Lt. flank pain and BP 130/60, Serum urea urinary tract inmass lt. nitrogen normal fection lower quadrant
MATERIALS AND METHODS
I
Followup
Treatment
1-MK-20-F-B
has been reviewed extensively in the past. 2, 3 The complications of the anomaly, usually obstruction and infection, dictate its management rather than the anomaly itself.
f
Skeletal
Normal except for Antibiotics degenerative changes
Normal
Rt. heminephrectomy oflower kidney
Never returned
1 yr., asymptomatic
Lost to followup
Normal
patient cleared with antibiotics. Of the remaining patients the crossed renal ectopia was an incidental finding. One patient had skeletal anomalies. The crossed renal ectopia was fused in 4 cases, with the lower pole ureter traversing the midline to insert on the opposite side (fig. 2). There was 1 case of probable unfused crossed renal ectopia documented by retrograde pyelography (fig. 3). The 3 boys and 2 girls ranged in age from 2 to 17 years. They presented with either urinary tract symptoms or multiple congenital anomalies (table 2). An imperforate anus was present in 2 patients and 1 patient had vaginal agenesis. All 3 of the patients with roentgenograms available for review had multiple skeletal anomalies (figs. 4 and 5). Radiographic studies revealed the typical crossed fused renal ectopia in 4 patients. Case 6 was unusual because 3 ureters emanated from the crossed ectopia, with 2 of these crossing the midline (fig. 6). A voiding cystourethrogram in 1 patient demonstrated unilateral reflux (case 10). DISCUSSION
The differential diagnosis of crossed renal ectopia includes solitary kidney, horseshoe kidney, ipsilateral double kidney
CROSSED RENAL ECTOPIA
189
Fm. 3. Retrograde pyelograms of unfused crossed renal ectopia
Fm. 1. Pyonephrosis of lower non-visualized renal segment with deviation of ureter from upper renal segment.
Fm. 2. Typical case of crossed fused ectopia with demonstration of crossed ureter from lower pole. A, IVP. B, retrograde pyelogram. and massive displacement of the kidney. Retrograde pyelography often is required to delineate clearly the anatomy. Crossed fused ectopia with the lower ureter traversing the midline is the most common anomaly. 3 A variety of forms may include unilateral fused, sigmoid, lump, disk and Lshaped kidneys. 2 The cases presented herein were unilaterally fused, except 1 case of unfused kidney. Unfused crossed ectopia is much less common4 and crossed renal ectopia of a solitary kidney is even more rare. 5 , 6 Bilateral crossed renal ectopia has been reported in several cases. 7 There is no definite pattern of associated congenital anomalies with crossed renal ectopia, which suggests that the lesion is an acquired defect. The association with vaginal
Fm. 4. Sacral and pelvic bony anomalies with crossed ectopia in child. agenesis 8 and undescended testis 3 has been reported previously. Congenital defects are of many types but skeletal anomalies of the bony pelvis and lumbar spine do appear frequently. It has been suggested that a skeletal defect with rotational deformity of the fetus might contribute to the genesis of crossed renal ectopia. There were some significant skeletal anomalies among our patients but 4 of the 5 adults had no such abnormalities. An insult to the fetus at 4 to 5 weeks of gestation could easily be reflected in urinary and skeletal abnormalities. The association of skeletal anomalies,
190
MARSHALL AND FREEDMAN TABLE
Case -Pt. -Age- Sex-Race No. (yrs)
History
2. Crossed renal ectopia in children
Associated Anomalies
Laboratory Findings
X-Ray Findings
Followup fol-
Lost to lowup
fol-
Multiple congenital anomalies
7-BT-?-F-?
Enuresis
8-WC-17-F-W
Primary amen- Vaginal agenesis 1 yr., asymptomatic orrhea Lassitude, he11.5 yrs., maturia after asymptotrauma matic Multiple anom- Tracheoesophageal Hypoplasia lt. femur, ti- Creatinine 0.6, se- IVP and retrograde 4 mos. alies fistula, imperfor- bia, pelvis; anomalies of rum urea nitropyelogram-lt. ate anus, dextrochest wall cervical gen 21 crossed fused ectopia cardia, bilateral spine, sacrum Voiding cystourethrocryptorchidism, gram- reflux on rt. hypospadias side
10-JI-2-M-W
Scoliosis, dysplasia femo- Serum urea nitro- !VP-triple collecting ral head, separation pugen 20 system, 2 ureters enhie symphysis, hypoplater on It., 1 on rt., It. sia lt. femur, lt. belowcrossed fused ectopia knee amputation 4 lumbar vertebrae, low Retrograde lumbar and sacral scopyelogram-lt. crossed fused ectopia liosis, L4 and sacrum 1 cm. separation of lamina compatible with meningocele Not reviewed IVP- lt. crossed fused renal ectopia Not reviewed Serum urea nitro- IVP - It. crossed fused gen normal ectopia
Lost to lowup
6-JS-8-M-W
9-CM-9-M-W
Imperforate anus
Skeletal Findings
Fm. 5. Retrograde catheters in girl with crossed ectopia and sacral deformity.
particularly spinal deformities with genitourinary anomalies, is well recognized. 9, 10 In a study of 85 children with congenital scoliosis or kyphosis 3 cases of crossed renal ectopia were found. 10 Therefore, skeletal anomalies do not seem to be the cause of crossed renal ectopia. The etiology of crossed renal ectopia remains unproved but a ureteral anomaly is the most attractive hypothesis. Many bizarre cases of crossed renal ectopia have been reported, including bilateral crossed renal ectopia and 1 with multiple ureters (as in case 6). Abnormal development of the ureteral bud arising in close proximity to the metanephric blastema seems to be a more reasonable explanation than abnormal tortuous migration of the metanephros itself. In the presence of calculus disease renal pain is referred to the side of the kidney but ureteral pain is referred to the contralateral side or side of the ureteral orifice. 11 It would appear that the innervation crosses with the ureter, which supports the concept of abnormal ureteral migration. However, McDonald and McClellan did report sigmoid kidneys with pain referred to the contralateral side. This entity may be closer to a horseshoe kidney than a true crossed ectopia. Vascular impe-
Fm. 6. Crossed renal ectopia with 3 ureters (IVP)
diments also have been suggested as etiologic agents in renal fusion anomalies. Renal fusion may occur in normal embryologic development and this phenomenon does not appear secondary to vascular obstruction. 12 Urinary tract problems associated with crossed renai ectopia include calculi, obstruction and reflux. These problems can require operation. Reflux was demonstrated in 14 of 16 patients in 1 study on renal ectopia and renal fusion in children. 13 It is not surprising that if the ureter is abnormal
CROSSED REN AL ECTOPIA
proximally, it also may be abnormal distally. Only 1 voiding cystourethrogram was available in our series but it did show unilateral reflux. REFERENCES
1. Kretschmer, H. L.: Unilateral fused kidney. Surg., Gynec. &
Obst., 40: 360, 1925. 2. Abeshouse, B. S.: Crossed ectopia with fusion: review of literature and a report of 4 cases. Amer. J. Surg., 73: 658, 1947. 3. McDonald, J. H. and McClellan, D. S.: Crossed renal ectopia. Amer. J. Surg., 93: 995, 1957. 4. Arduino, L. J.: Crossed renal ectopia without fusion. J. Urol., 93: 125, 1965. 5. Marshall, V. F. and Keuhnelian, J. G.: Crossed ureteral ectopia with solitary kidney. J. Urol., 110: 176, 1973. 6. Tanenbaum, B., Silverman, N. and Weinberg, S. R.: Solitary crossed renal ectopia. Arch. Surg., 101: 616, 1970.
191
7. Beer, E. and Ferber, W. L. F.: Crossed renal ectopia (unilateral, fused or elongated kidney). With report of fourteen cases clinically diagnosed and two cases encountered at autopsy during the past eighteen years. J. Urol., 38: 541, 1937. 8. Tabrisky, J. and Bhisitkul, I.: Solitary crossed ectopic kidney with vaginal aplasia: a case report. J. Urol., 94: 33, 1965. 9. Mecklenburg, R. S. and Krueger, P. M.: Extensive genitourinary anomalies associated with Klippel-Feil syndrome. Amer. J. Dis. Child., 128: 92, 1974. 10. Vitko, R. J., Cass, A. S. and Winter, R. B.: Anomalies of the genitourinary tract associated with congenital scoliosis and congenital kyphosis. J. Urol., 108: 655, 1972. 11. Romans, D. G., Jewett, M. A. S. and Robson, C. J.: Crossed renal ectopia with colic. A clinical clue to embryogenesis. Brit. J. Urol., 48: 171, 1976. 12. Friedland, G. W. and de Vries, P.: Renal ectopia and fusion. Embryologic basis. Urology, 5: 698, 1975. 13. Kelalis, P. P., Malek, R. S. and Segura. J. W.: Observations on renal ectopia and fusion in children. J. Urol., 110: 588, 1973.
®'41/ii@
