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Noncrossed renal ectopia with fusion associated with single ectopic ureterocele
NONCROSSED ASSOCIATED
RENAL ECTOPIA
WITH FUSION
WITH SINGLE ECTOPIC
URETEROCELE
KYUN PAK, M.D. TAIRA KONISHI,
M.D.
TADAO TOMOYOSHI,
M.D.
From the Department of Urology, Shiga University of Medical Science, Seta, Otsu, Japan
ABSTRACTWe report a case of noncrossed renal ectopia with fusion associated with a single ectopic ureterocele. This association is rare, and it is extremely rare that an ectopic ureterocele is not a part of the duplicated urinary collecting system. Some problems about classification of renal fusion and ectopia are presented, and a new classification is introduced.
Renal fusion except for horseshoe kidney is an uncommon anomaly, and renal fusion associated with ureterocele is a rare condition. This association is sporadically and statistically reported mainly in horseshoe kidney.‘m4 Ureteroceles are classified as either orthotopic or ectopit. An orthotopic ureterocele is usually small, rarely obstructive, seen more frequently in adults, and associated with a single upper urinary tract. An ectopic ureterocele is usually obstructive and seen in infants and children and almost as part of a duplicated urinary collecting system. We report a case of noncrossed renal ectopia with fusion associated with a single ectopit ureterocele seen in a thirty-nine-year-old man. Case Report A thirty-nine-year-old man was seen in February, 1983, because of gross hematuria three days prior to his first visit to our hospital, He had noticed. neither difficulty in urination nor pain in the back. Past history was unremarkable except for left upper lobectomy due to pulmonary tuberculosis at age twenty-two. Physical examination revealed a palpable nontender
246
mass just below the umbilicus. The results of routine laboratory studies including complete blood cell count, liver function test, serum electrolytes, serum creatinine, and blood urea nitrogen were within normal limits, and urinalysis showed trace proteinuria and microscopic hematuria. Urine culture yielded no bacterial growth. An excretory urogram showed bizarre pyelogram on the right side, in which the kidney was located medially and lower than the left one, the ureter was dilated, and an oval filling defect was seen in the cystogram (Fig. 1A). A renal scintigram was done using technetium dimercaptosuccinic acid which showed L-shaped fused kidney (Fig. 2). Computed tomography scans showed fusion of kidneys and a round mass in the bladder that was enhanced and delineated with a discrete membranous structure (Fig. 3). No reflux was observed on a cystogram. Cystoscopy showed a large flabby mass in the right lateral half covered with normal mucosa. This mass extended distally to the bladder neck at the six o’clock position. The left ureteral orifice was normally located, but the right one could not be identified.
UROLOGY
/
SEPTEMBER
1988
i
VOLUME
XxX11,
NUMBER
3
FIC:URIC1. (A) Excretory urogram: bizarre pyelocaliteal system on right side; dilated and elliptical right ureter and oval filling defect in bladder. (B) Retrograde urogram: bizarre pyelocaliceal system, narrow ureteropelvic junction, and ureter that does not run across midline.
FIGUHE 2.
Renal scintigram:
L-shaped fused kid-
ney.
At operation, a large ureterocele was identified in the right trigone and lateral wall. The distal portion of the ureterocele stretched over the bladder neck, where there was a slit-like recessus through which a 6-F catheter was introduced easily into ureterocele. After unroofing of the ureterocele, the intramural ureter was closed. The markedly dilated right ureter was
UROLOGY
FIGURE 3. Computed tomography scans: fusion of kidneys (A) and round mass in bladder that was enhanced and delineated with discrete membranous structure (B).
severed near the ureterovesical junction, and after tapering the distal portion it was reimplanted through 3-cm long submucosal tunnel. The patient’s postoperative course was uneventful, and retrograde urograms taken
/ SEPTEMBER1988 i VOLUMEXXXII.NUMBER3
247
FIGURES.
Classification of renal fusion and ectopia.
through an indwelling ureteral splint catheter revealed bizarre pyelocaliceal system, narrow ureteropelvic junction, and ureter that did not run across the midline (Fig. 1B). The patient has been carefully followed up, and fifteen months after operation there are no findings which show developments of stasis in the right urinary tract. Comment Classifications of renal fusion and ectopia are somewhat complicated and overlapped. According to Wilmer,5 who considered the position of ureters important to differentiate fused kidney, renal fusion is classified into four types: (1) horseshoe kidney; (2) L-shaped kidney that is a transitional form between horseshoe kidney and unilateral fused kidney (when neither ureter crosses the midline of the vertebral column, the L-shaped kidney is merely a variation of the horseshoe kidney); (3) unilateral fused kidney (the ureter of the ectopic kidney crosses the midline); and (4) miscellaneous. McDonald and McClellan6 classified crossed renal ectopia into four types: (1) crossed renal ectopia with fusion; (2) crossed renal ectopia without fusion; (3) solitary crossed renal ectopia; and (4) bilaterally crossed renal ectopia. They also classified crossed renal ectopia with fusion into six forms: unilateral fused kidney with ectopic kidney inferior; S-shaped kidney; lump kidney; L-shaped kidney; disk kidney; and unilateral fused kidney with ectopic kidney superior. In our case, the ureter does not cross the midline, therefore, if we describe this case as an L-shaped kidney, it will be confused with
248
crossed renal ectopia with fusion. Furthermore, it is less likely that our case is merely a variation of the horseshoe kidney. To avoid overlapping in classification of renal fusion and ectopia, Namiki and Shimoe7 proposed a new classification according to presence of fusion, presence of symmetry, and run of ureters (Fig. 4). This classification covers almost all types of renal fusion and ectopia without overlapping, and our case is described as noncrossed renal ectopia with fusion. Characteristic forms are described within a parenthesis, such as (Sshaped kidney) or (lump kidney), so this case is also described as noncrossed renal ectopia with fusion (L-shaped kidney). Our case is also unique in that a ureterocele is ectopic and associated with a single upper urinary tract unit. Ureteroceles are subdivided usually into either simple or ectopic, but this distinction often proves vague and confusing, since it is often based solely on the position or solely on the presence of associated ureteral duplication. ‘ml0Giant ureteroceles are sometimes misdiagnosed as ectopic ureteroceles simply because they extend to or beyond the internal meatus or partly prolapse through it.” In our case, the ureterocele was exactly ectopic because its orifice was located in the bladder neck and the ureter was mildly dilated. According to Uson’P classification of ureterocele, this case is a type A-IV ureterocele that is extremely rare and associated with a single upper urinary tract unit which appears mildly obstructed and dilated.
UROLOGY
Seta, Otsu, Japan (DR. PAK)
y/ SEPTEMBER1988 / VOLUMEXXXII,NUMBER3
References 1. Malck RS, and Utz DC: .Crossed, fused, renal ectopia with an cctopic ureterocele, J Urol 104: 665 (1970). 2. Boatman DI,, Kolln CP, and Flocks RH: Congenital anomalies associated with horseshoe kidney, J Ural 107: 205 (1972). 3. Segura JW, Kelalis PP. and Burke EC: Horseshoe kidney in children, J Urol 108: 333 (1972). 4. Novak ME, Baum NH, and Gonzales ET Jr: Horseshoe kidney with multicy;tic dysplasia associated with ureterocele, Urolok? 10: 456 (1971). fused kidney. A report of five cases 3. Wilmer HA: Unilateral and a review of the literature, J Urol 40: 551 (1938). 6. McDonald JH, and McClellan DS: Crossed renal ectopia, Am J Surg 93: 995 (1957).
UROLOGY
/ SEPTEMBER
1988
/
VOLUME
7. Namiki M, and Shimoe S: Asymmetric fused kidney: a report of two cases and a discussion on its classification, Acta Urol Jap 24: 1061 (1978). 8. Ericsson NO: Ectopic ureterocele in infants and children: a clinical study, Acta Chir Stand (suppl) 197: 1 (1954). 9. Malek RS, Kelalis PP, Burke EC, and Stickler GB: Simple and ectopic ureterocele in infancy and childhood, Surg Gynecol Obstet 134: 611 (1972). 10. Johnston JH, and Johnson LM: Experiences with ectopic ureteroceles, Br J Urol 41: 61 (1969). 11. Tanagho EA: Anatomy and management of ureteroceles, J Urol 107: 729 (1972). 12. Uson AC: Overview of surgical management of ureterocele, in Whitehead ED (Ed): Current Operative Urology, New York, Harper and Row, 1975, p 839.
XXXII, NUMBER
3
249
RENAL ECTOPIA
WITH FUSION
WITH SINGLE ECTOPIC
URETEROCELE
KYUN PAK, M.D. TAIRA KONISHI,
M.D.
TADAO TOMOYOSHI,
M.D.
From the Department of Urology, Shiga University of Medical Science, Seta, Otsu, Japan
ABSTRACTWe report a case of noncrossed renal ectopia with fusion associated with a single ectopic ureterocele. This association is rare, and it is extremely rare that an ectopic ureterocele is not a part of the duplicated urinary collecting system. Some problems about classification of renal fusion and ectopia are presented, and a new classification is introduced.
Renal fusion except for horseshoe kidney is an uncommon anomaly, and renal fusion associated with ureterocele is a rare condition. This association is sporadically and statistically reported mainly in horseshoe kidney.‘m4 Ureteroceles are classified as either orthotopic or ectopit. An orthotopic ureterocele is usually small, rarely obstructive, seen more frequently in adults, and associated with a single upper urinary tract. An ectopic ureterocele is usually obstructive and seen in infants and children and almost as part of a duplicated urinary collecting system. We report a case of noncrossed renal ectopia with fusion associated with a single ectopit ureterocele seen in a thirty-nine-year-old man. Case Report A thirty-nine-year-old man was seen in February, 1983, because of gross hematuria three days prior to his first visit to our hospital, He had noticed. neither difficulty in urination nor pain in the back. Past history was unremarkable except for left upper lobectomy due to pulmonary tuberculosis at age twenty-two. Physical examination revealed a palpable nontender
246
mass just below the umbilicus. The results of routine laboratory studies including complete blood cell count, liver function test, serum electrolytes, serum creatinine, and blood urea nitrogen were within normal limits, and urinalysis showed trace proteinuria and microscopic hematuria. Urine culture yielded no bacterial growth. An excretory urogram showed bizarre pyelogram on the right side, in which the kidney was located medially and lower than the left one, the ureter was dilated, and an oval filling defect was seen in the cystogram (Fig. 1A). A renal scintigram was done using technetium dimercaptosuccinic acid which showed L-shaped fused kidney (Fig. 2). Computed tomography scans showed fusion of kidneys and a round mass in the bladder that was enhanced and delineated with a discrete membranous structure (Fig. 3). No reflux was observed on a cystogram. Cystoscopy showed a large flabby mass in the right lateral half covered with normal mucosa. This mass extended distally to the bladder neck at the six o’clock position. The left ureteral orifice was normally located, but the right one could not be identified.
UROLOGY
/
SEPTEMBER
1988
i
VOLUME
XxX11,
NUMBER
3
FIC:URIC1. (A) Excretory urogram: bizarre pyelocaliteal system on right side; dilated and elliptical right ureter and oval filling defect in bladder. (B) Retrograde urogram: bizarre pyelocaliceal system, narrow ureteropelvic junction, and ureter that does not run across midline.
FIGUHE 2.
Renal scintigram:
L-shaped fused kid-
ney.
At operation, a large ureterocele was identified in the right trigone and lateral wall. The distal portion of the ureterocele stretched over the bladder neck, where there was a slit-like recessus through which a 6-F catheter was introduced easily into ureterocele. After unroofing of the ureterocele, the intramural ureter was closed. The markedly dilated right ureter was
UROLOGY
FIGURE 3. Computed tomography scans: fusion of kidneys (A) and round mass in bladder that was enhanced and delineated with discrete membranous structure (B).
severed near the ureterovesical junction, and after tapering the distal portion it was reimplanted through 3-cm long submucosal tunnel. The patient’s postoperative course was uneventful, and retrograde urograms taken
/ SEPTEMBER1988 i VOLUMEXXXII.NUMBER3
247
FIGURES.
Classification of renal fusion and ectopia.
through an indwelling ureteral splint catheter revealed bizarre pyelocaliceal system, narrow ureteropelvic junction, and ureter that did not run across the midline (Fig. 1B). The patient has been carefully followed up, and fifteen months after operation there are no findings which show developments of stasis in the right urinary tract. Comment Classifications of renal fusion and ectopia are somewhat complicated and overlapped. According to Wilmer,5 who considered the position of ureters important to differentiate fused kidney, renal fusion is classified into four types: (1) horseshoe kidney; (2) L-shaped kidney that is a transitional form between horseshoe kidney and unilateral fused kidney (when neither ureter crosses the midline of the vertebral column, the L-shaped kidney is merely a variation of the horseshoe kidney); (3) unilateral fused kidney (the ureter of the ectopic kidney crosses the midline); and (4) miscellaneous. McDonald and McClellan6 classified crossed renal ectopia into four types: (1) crossed renal ectopia with fusion; (2) crossed renal ectopia without fusion; (3) solitary crossed renal ectopia; and (4) bilaterally crossed renal ectopia. They also classified crossed renal ectopia with fusion into six forms: unilateral fused kidney with ectopic kidney inferior; S-shaped kidney; lump kidney; L-shaped kidney; disk kidney; and unilateral fused kidney with ectopic kidney superior. In our case, the ureter does not cross the midline, therefore, if we describe this case as an L-shaped kidney, it will be confused with
248
crossed renal ectopia with fusion. Furthermore, it is less likely that our case is merely a variation of the horseshoe kidney. To avoid overlapping in classification of renal fusion and ectopia, Namiki and Shimoe7 proposed a new classification according to presence of fusion, presence of symmetry, and run of ureters (Fig. 4). This classification covers almost all types of renal fusion and ectopia without overlapping, and our case is described as noncrossed renal ectopia with fusion. Characteristic forms are described within a parenthesis, such as (Sshaped kidney) or (lump kidney), so this case is also described as noncrossed renal ectopia with fusion (L-shaped kidney). Our case is also unique in that a ureterocele is ectopic and associated with a single upper urinary tract unit. Ureteroceles are subdivided usually into either simple or ectopic, but this distinction often proves vague and confusing, since it is often based solely on the position or solely on the presence of associated ureteral duplication. ‘ml0Giant ureteroceles are sometimes misdiagnosed as ectopic ureteroceles simply because they extend to or beyond the internal meatus or partly prolapse through it.” In our case, the ureterocele was exactly ectopic because its orifice was located in the bladder neck and the ureter was mildly dilated. According to Uson’P classification of ureterocele, this case is a type A-IV ureterocele that is extremely rare and associated with a single upper urinary tract unit which appears mildly obstructed and dilated.
UROLOGY
Seta, Otsu, Japan (DR. PAK)
y/ SEPTEMBER1988 / VOLUMEXXXII,NUMBER3
References 1. Malck RS, and Utz DC: .Crossed, fused, renal ectopia with an cctopic ureterocele, J Urol 104: 665 (1970). 2. Boatman DI,, Kolln CP, and Flocks RH: Congenital anomalies associated with horseshoe kidney, J Ural 107: 205 (1972). 3. Segura JW, Kelalis PP. and Burke EC: Horseshoe kidney in children, J Urol 108: 333 (1972). 4. Novak ME, Baum NH, and Gonzales ET Jr: Horseshoe kidney with multicy;tic dysplasia associated with ureterocele, Urolok? 10: 456 (1971). fused kidney. A report of five cases 3. Wilmer HA: Unilateral and a review of the literature, J Urol 40: 551 (1938). 6. McDonald JH, and McClellan DS: Crossed renal ectopia, Am J Surg 93: 995 (1957).
UROLOGY
/ SEPTEMBER
1988
/
VOLUME
7. Namiki M, and Shimoe S: Asymmetric fused kidney: a report of two cases and a discussion on its classification, Acta Urol Jap 24: 1061 (1978). 8. Ericsson NO: Ectopic ureterocele in infants and children: a clinical study, Acta Chir Stand (suppl) 197: 1 (1954). 9. Malek RS, Kelalis PP, Burke EC, and Stickler GB: Simple and ectopic ureterocele in infancy and childhood, Surg Gynecol Obstet 134: 611 (1972). 10. Johnston JH, and Johnson LM: Experiences with ectopic ureteroceles, Br J Urol 41: 61 (1969). 11. Tanagho EA: Anatomy and management of ureteroceles, J Urol 107: 729 (1972). 12. Uson AC: Overview of surgical management of ureterocele, in Whitehead ED (Ed): Current Operative Urology, New York, Harper and Row, 1975, p 839.
XXXII, NUMBER
3
249