International Urology and Nephrolooy 24 (5), pp. 491---493 (1992)
Late Development of Pelviureteric Junction Obstruction (PUJO) in a Girl with Previously Normal Pyelogram: A Case Report F. ARAGONA,C. CAMUFFO,O. PASSERINI-GLAZEL Institute of Urology, University of Padua, Italy (Accepted December 23, 1991) A case of ureteropelvic junction obstruction in a girl with previously normal pyelogram is presented. The aetiology of this development is unclear but this report emphasizes the need for a close follow-up of patients with persistent flank pain even if the initial pyelograms are normal Pelviuretic junction obstruction (PUJO) is commonly believed to be a congenital anomaly. When discovered in adults, PUJO is thought to be a late clinical manifestation of a congenital pathology. Herein a case of PUJO in a girl, who previously had normal intravenous pyelogram (IVP), is presented.
Case report A 12-year-old girl was seen in February 1990 for severe left flank pain and arterial hypertension. IVP revealed a non-functioning left kidney (Fig. la). Five years previously an IVP, obtained to evaluate left plank pain, was normal (Fig. lb); there was no history of recent trauma and the patient was free of other medical problems. Renal sonography demonstrated a markedly dilated left pelvicalyceal system and an 8F pig-tail nephrostomy tube was placed. Nephrostography was compatible with PUJO (Fig. lc). Peripheral venous renin was also elevated. In order to treat hypertension and because the left kidney did not recover after relief o f obstruction, a nephrectomy was done in April 1990. Convalescence was uneventful and the patient is now normotensive.
Discussion The aetiology of PUJO is thought to be congenital. The obstruction may result from an imbalance in the growth of the lumbar region compared to ureteral growth [1 ] or it is attributed to ureteral valves at PUJ level [2] or to a congenital stricture represented by an inelastic collar of collagen [3]. Foote et al. [4] postu3
VSP, Utrecht Akad~miai lfiad6, Budapest
492
A r a g o n a et al. : Pelviureteric obstruction
Fig. 1. (a) IVP shows non-functioning left kidney; (b) 15-minute IVP 5 years before hospitalization is normal; (c) nephrostography shows markedly dilated renal pelvis and calyces owing to PUJO
lated that the cause is a congenital deficiency in smooth muscle development. Allen [5] attributed PUJO to an incomplete ureteral development, resulting from compression by contiguous fetal vessels that may disappear, leaving a stricture as the only evidence of its former existence. A very attractive theory is that proposed by Ruano-Gil et al. [6], who suggested that PUJO results from problems in recanalization of the embryonic ureter between the fifth and sixth weeks of fetal development. However, the pathogenesis of PUJO is always thought to be congenital in origin. This does not mean that patients with PUJO should have pyelographic changes all their lives. Indeed, an inelastic collar could significantly and irreversibly decompensate a renal pelvis only late in life. In patients with intermittent hydronephrosis [7] the obstruction is related to hyperdiuresis or to positional changes. These patients demonstrate hydronephrosis during an attack of colicky pain while these same patients have normal pyelograms between attacks. However, they have box-shaped pelvis which may suggest transient impairment in urine transport at the UPJ. Our patient was studied close to a symptomatic episode, she did not show the classical pyelectasis on the initial IVP and the renal pelvis appeared to be not dilated and funnel shaped. There must be some heretofore unexplained factor that can cause decompensation at the PUJ late in life. Scanty reports of late PUJO are found in the literature: Friedland et al. [8 ] documented the development of PUJO during a 3-year period in a 74-year-old woman. Histological specimens were compatible with an ischaemic stricture similar to that seen in ischaemic bowel disease but our patient had no evidence of vascular disease. International Urology and Nephrology 24, 1992
Aragona et al. : Pelviureteric obstruction
493
A l t h o u g h a congenital predisposition o f the development o f P U J O is generally present, this m a y n o t be the rule. Moreover, o u r case emphasizes the need for periodic reevaluation of patients with persistent flank p a i n even if the initial pyelograms are n o r m a l .
References 1. Jewett, H. J. : Stenosis of the ureteropelvic juncture: congenital and acquired, d. Urol., 44, 247 (1940). 2. Lich, R., Barnes, M. L. : A clinicopathologic study of ureteropelvic obstructions. J. UroL, 77, 382 (1957). 3. Hanna, M. K., Jeffs, R. D.,. Sturgess, J. M., Barkin, M.: Ureteral structure and ultrastructure. Part II. Congenital ureteropelvicjunctionobstruction and primary obstructive megaureter. J. UroL, 116, 725 (1976). 4. Foote, J. W., Blennerhasset, J. B., Wiglesworth, F. W., McKinnon, K. J. : Observations on the ureteropelvic junction. J. Urol., 104, 252 (1970). 5. Allen, T. D.: Congenital ureteral stricture. J. UroL, 104, 196 (1970). 6. Ruano-Gil, D., Coca-Payeras, A., Tejedo-Mateu, A. : Obstruction and normal recanalization of the ureter in the human embryo. Its relation to congenital ureterie obstruction. Eur. UroL, 1, 287 (1975). 7. Nesbit, R. M. : Diagnosis of intermittent hydronephrosis: Importance of pyelography during episodes of pain. J. UroL, 75, 767 (1956). 8. Friedland, G. W,, Droller, M. J., Stamey, T. A. : Obstruction of ischemic ureteropelvic junction. Urology, 4, 439 (1974).
3*
International Urology and Nephrology 24, 1992
Late Development of Pelviureteric Junction Obstruction (PUJO) in a Girl with Previously Normal Pyelogram: A Case Report F. ARAGONA,C. CAMUFFO,O. PASSERINI-GLAZEL Institute of Urology, University of Padua, Italy (Accepted December 23, 1991) A case of ureteropelvic junction obstruction in a girl with previously normal pyelogram is presented. The aetiology of this development is unclear but this report emphasizes the need for a close follow-up of patients with persistent flank pain even if the initial pyelograms are normal Pelviuretic junction obstruction (PUJO) is commonly believed to be a congenital anomaly. When discovered in adults, PUJO is thought to be a late clinical manifestation of a congenital pathology. Herein a case of PUJO in a girl, who previously had normal intravenous pyelogram (IVP), is presented.
Case report A 12-year-old girl was seen in February 1990 for severe left flank pain and arterial hypertension. IVP revealed a non-functioning left kidney (Fig. la). Five years previously an IVP, obtained to evaluate left plank pain, was normal (Fig. lb); there was no history of recent trauma and the patient was free of other medical problems. Renal sonography demonstrated a markedly dilated left pelvicalyceal system and an 8F pig-tail nephrostomy tube was placed. Nephrostography was compatible with PUJO (Fig. lc). Peripheral venous renin was also elevated. In order to treat hypertension and because the left kidney did not recover after relief o f obstruction, a nephrectomy was done in April 1990. Convalescence was uneventful and the patient is now normotensive.
Discussion The aetiology of PUJO is thought to be congenital. The obstruction may result from an imbalance in the growth of the lumbar region compared to ureteral growth [1 ] or it is attributed to ureteral valves at PUJ level [2] or to a congenital stricture represented by an inelastic collar of collagen [3]. Foote et al. [4] postu3
VSP, Utrecht Akad~miai lfiad6, Budapest
492
A r a g o n a et al. : Pelviureteric obstruction
Fig. 1. (a) IVP shows non-functioning left kidney; (b) 15-minute IVP 5 years before hospitalization is normal; (c) nephrostography shows markedly dilated renal pelvis and calyces owing to PUJO
lated that the cause is a congenital deficiency in smooth muscle development. Allen [5] attributed PUJO to an incomplete ureteral development, resulting from compression by contiguous fetal vessels that may disappear, leaving a stricture as the only evidence of its former existence. A very attractive theory is that proposed by Ruano-Gil et al. [6], who suggested that PUJO results from problems in recanalization of the embryonic ureter between the fifth and sixth weeks of fetal development. However, the pathogenesis of PUJO is always thought to be congenital in origin. This does not mean that patients with PUJO should have pyelographic changes all their lives. Indeed, an inelastic collar could significantly and irreversibly decompensate a renal pelvis only late in life. In patients with intermittent hydronephrosis [7] the obstruction is related to hyperdiuresis or to positional changes. These patients demonstrate hydronephrosis during an attack of colicky pain while these same patients have normal pyelograms between attacks. However, they have box-shaped pelvis which may suggest transient impairment in urine transport at the UPJ. Our patient was studied close to a symptomatic episode, she did not show the classical pyelectasis on the initial IVP and the renal pelvis appeared to be not dilated and funnel shaped. There must be some heretofore unexplained factor that can cause decompensation at the PUJ late in life. Scanty reports of late PUJO are found in the literature: Friedland et al. [8 ] documented the development of PUJO during a 3-year period in a 74-year-old woman. Histological specimens were compatible with an ischaemic stricture similar to that seen in ischaemic bowel disease but our patient had no evidence of vascular disease. International Urology and Nephrology 24, 1992
Aragona et al. : Pelviureteric obstruction
493
A l t h o u g h a congenital predisposition o f the development o f P U J O is generally present, this m a y n o t be the rule. Moreover, o u r case emphasizes the need for periodic reevaluation of patients with persistent flank p a i n even if the initial pyelograms are n o r m a l .
References 1. Jewett, H. J. : Stenosis of the ureteropelvic juncture: congenital and acquired, d. Urol., 44, 247 (1940). 2. Lich, R., Barnes, M. L. : A clinicopathologic study of ureteropelvic obstructions. J. UroL, 77, 382 (1957). 3. Hanna, M. K., Jeffs, R. D.,. Sturgess, J. M., Barkin, M.: Ureteral structure and ultrastructure. Part II. Congenital ureteropelvicjunctionobstruction and primary obstructive megaureter. J. UroL, 116, 725 (1976). 4. Foote, J. W., Blennerhasset, J. B., Wiglesworth, F. W., McKinnon, K. J. : Observations on the ureteropelvic junction. J. Urol., 104, 252 (1970). 5. Allen, T. D.: Congenital ureteral stricture. J. UroL, 104, 196 (1970). 6. Ruano-Gil, D., Coca-Payeras, A., Tejedo-Mateu, A. : Obstruction and normal recanalization of the ureter in the human embryo. Its relation to congenital ureterie obstruction. Eur. UroL, 1, 287 (1975). 7. Nesbit, R. M. : Diagnosis of intermittent hydronephrosis: Importance of pyelography during episodes of pain. J. UroL, 75, 767 (1956). 8. Friedland, G. W,, Droller, M. J., Stamey, T. A. : Obstruction of ischemic ureteropelvic junction. Urology, 4, 439 (1974).
3*
International Urology and Nephrology 24, 1992
