Ureteropelvic Junction Obstruction as a Cause for Intermittent Abdominal Pain in Children

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Pediatr NephroL 1984;5:205-208

17. Grunfeld B, Gimenez M, Liapchuc 5, Mendilaharzu J, Gian antonio C. Systemic hypertension and plasma renin activity in children with the hemolytic-uremic syndrome. Int J Pe diatr NephmL 1982;3:211—214

18. Clozel M, Fischli W, Guilly C. Specific binding of endothelin on human vascular smooth muscle cells in culture. J Clin Invest. 1989;83:1758—1761

19. Vierhapper H, Wagner 0, Nowotny P, Waldhausl W. Effect

of endothelin-1 in man. Circulation. 1990;81:1415—1418 20. Kohno M, Yasunari K, Murakawa K, et aL Plasma immu

noreactive endothelin in essential hypertension. Am J Med. 1990;88:614—618

21. de Nticci G, Thomas R, D'Orleans-Juste P, et al. Pressor effects of circulating endothelin are limited by its removal in the pulmonarycirculationand by the release of prosta cydlin and endothelium-derived relaxing factor. Proc Nati Acad Sci USA. 1988;85:9797-9800

problems with constipation, diarrhea, or encopresis. The

results of physical examination and routine laboratory

studies carried out by the pediatrician, including urinal ysis, were all reported as being normal.

When seen by the Gastrointestinal Service, no addi

tional pertinent history was noted. There were no abnor

mal fmdings on physical examination. Routine urinalysis, complete blood cell count, and sedimentation rate were obtained again, and the results were found to be normal. The child was sent for a screening abdominal sonogram prior to any further testing. Left moderately severe hy dronephrosis was reported (Fig 1). The ureter was not identified below the dilated renal pelvis. On receiving the report of the sonogram, the Gastrointestinal Service con tacted the Urology service. A diuretic renal scan was obtained which demonstrated slightly diminished uptake of isotope on the left side on the initial images with marked impairment in drainage on delayed images (Fig 2). The computer-generated excretion curve was normal on the right but flat for the left side (Fig 3). The results of a voiding cystourethrogram were normal. The diagno sin of probable ureteropelvic junction obstruction was made and surgical correction advised. At exploration an accessory renal vessel was found to cross the upper ureter, compressing it (Fig 4). The ureter was transected at this site, brought over the vessel, and a spatulated uretero pyelostomy performed. Fo!!ow-up included a renal scan performed 2 months postoperatively demonstrating ex cellent early function and drainage without the necessity for diuretic stimulation.

DISCUSSION

Ureteropelvic junction (UPJ) obstruction is the result ofa relative narrowing ofthe proximal ureter, most commonly at the site of its junction with the renal pelvis. However, the term is applied loosely, and it refers also to such instances as cited in this case where the obstruction actually may involve only the most proximal ureter. Other causes include

intrinsic narrowing of the upper ureter and a “¿high

riding―insertion of the ureter into the renal pelvis. With increasing output urine excretion surpasses the ability of the narrowed ureteral segment to convey this urine and the pelvis and collecting system (calices and infundibula) become progres sively stretched. Ultimately the filling pelvis further

distorts the abnormal anatomy kinking the ureter

1066 PEDIATRICS Vol. 88 No. 5 November 1991

UreteropelvicJunction

Obstructionas a Causefor

IntermittentAbdominalPainin

Children

Dietl's crisis: “¿suddensevere attack of nephralgia or gastric pain, chills, fever, nausea and vomiting, and general collapse; said to be due to partial turn ing of the kidney on its pedicle.―

—¿Dorlan4'sIllustrated Medical Dictionary. 25th Edition

Two or three times each year children are seen by the Urology Service at Children's Hospital, Washington, DC, who have a history of intermit tent, severe midabdominal pain associated with vomiting due to intermittent ureteropelvic junction obstruction. These symptoms may have been on going for years. The following case history serves as a typical example.

CASE REPORT

A 6-year-old boy was referred to the Urology Service from the Gastrointestinal Service where he had been sent by his primary physician. He had a 1-year history of episodic abdominal pain occurring one to two times per month initially, but becoming more frequent during the past few months. This pain was associated with vomiting

unless the patient was given rectal acetaminophen. Each episode occurred in the evening and often lasted through

the night. The pain was described as severe, causing the child to cry out. He would lie with his legs drawn up to his abdomen during the episode. Vomiting often relieved the pain for about 1 hour, and by morning he would be well. He was otherwise healthy with no prior history of urinary infection or hematuria. There was no history of

Received for publication Jan 10, 1991; accepted Feb 11, 1991. Reprint requests to (A.B. B.) Department ofUrology, Children's Hospital, 111 Michigan Avenue, NW, Washington, DC 20010.

PEDIATRICS (ISSN 0031 4005). Copyright ©1991 by the

American Academyof Pediatrics.

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Fig 1. Sonogram of left kidney demonstrating full renal pelvis (arrows).

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Fig 2. Left, early scan reveals delayed uptake on left. Right, on delayed image the right

kidney has drained (B = bladder); however, left system is full (arrows outline renal pelvis).

and shutting off its lumen. Dieti's crisis is the result of acute dilatation of the collecting system due to virtual complete obstruction at the UPJ. Distention ofthe hollow viscus (collecting system) and stretch ing of the renal capsule are the explanation for the acute pain. Although adults may describe flank pain in this situation, children generally experience only periumbilical discomfort. The absence of symptoms suggesting urinary tract involvement may contrib ute to a delay in diagnosis. Vomiting leads to de

hydration with resorption of urine from the collect ing system by renal veins and lymphatics resulting in resolution and relief of symptoms.

With the ubiquitous use of antenatal sonography the recognition of hydronephrosis commonly is made prior to birth. However, historically these patients come to attention at all ages, but are recognized most commonly when evaluated for un nary tract infection or incidentally at the time of an intravenous urogram for other reasons. They

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than those of urinary origin should first be ex cluded. Sonography, particularly if carried out dun ing the acute episode, has the capability of exclud ing a urinary problem in a simple noninvasive man ner.

Although screening with sonography may iden

tify the offending system, the study may be negative

if carried out when the patient is not having symp toms. Because the pain may last for several hours one can readily study the patient when sympto matic. If that is not possible the sonogram should follow the intake of large amounts of oral fluids. Shanon et a!' recently addressed the question of sonographic evaluation of children with abdominal pain. They correctly point out that the results of most of these studies will be normal, but it is unclear if any were obtained during the painful episode.

The diagnosis of UPJ obstruction should be con firmed with a dynamic study. Classically an excre tory urogram (intravenous pyebogram) has been used for this purpose. Although it may demonstrate a dilated collecting system with nonvisualization of the ureter, highly suggestive of UPJ obstruction, the intravenous pyebogram offers little information regarding renal physiology. It gives no measurable

data pertaining to either function or drainage. Even

with diuretic stimulation the interpretation re mains subjective. A retrograde pyebogram, the in jection of contrast up the ureter done endoscopi cabby, offers no functional information. Addi tionally, in children urobogic endoscopy always requires an anesthetic with its inherent risks and tremendous hospital costs.

The advent of diuretic augmented isotope renal scans virtually has replaced all other diagnostic

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Fig3. Computer-generatedexcretion curves. Lower curve defines normal excretion from right kidney with a half-time for excretion of about 6 minutes. Upper curve is at a higher level and flat, the result of retained isotope in renal pelvis. Time from diuretic injection plotted on x-axis. Isotope activity on y-axis.

rarely have such severe hydronephnosis to cause an abdominal mass.

Periodically, however, patients still have a classic

history of acute abdominal or flank pain lasting for

several hours and often associated with and relieved by vomiting. Frequently, these patients have symp toms after entering college and shortly following their first binge of heavy beer drinking. Rarely do victims recognize the relationship between fluid intake and onset of symptoms. A fortunate few will have associated hematunia, the result of acute stretching of the renal collecting system, leading to early diagnosis. However, this finding is rarely sought and is often not present. Some patients suffer for years prior to a correct diagnosis being made.

Evaluation of the child with intermittent abdom inal pain accompanied by vomiting should at some point include sonography. However, causes other

Fig4. Intraoperativephotographof vesselcrossingureter(V) with dilatedpelvis(P).

1068 PEDIATRICS Vol. 88 No. 5 November1991

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studies to document urinary obstruction defini tively. Intravenously injected technetium-99m di ethylene triamine-pentaacetic acid is picked up se bectively by the kidneys based on perfusion, is cleared rapidly by the gbomeruli, and excreted.2 Poor drainage of isotope from the collecting system is indicative of dilatation. After complete fibbingof the dilated collecting system with isotope as demon strated on the oscilloscope screen, 1 mg/kg of fu rosemide is injected intravenously (the child has been well-hydrated by mouth and by intravenous fluids prior to this). If the system is dilated but nonobstructed there is rapid washout of the isotope. Delayed drainage delineates a degree of obstruction such as was noted in the example given (Figs 2 and 3).

Open surgical treatment of UPJ obstruction is highly effective with an extremely bowcomplication rate, even in babies.3 However, success is being

reported using endoscopic instrumentation passed

percutaneously with incision and stenting of the narrowed segment as a means of definitive treat ment.4 Initial success is not as yet as high as

achieved with open surgery, and the applicability of this modality to very small children remains in question.

REFERENCES

A. BARRYBELMAN,MD, MS

Departments of Urology and

Pediatrics

Children's Hospital and George Washington University School of Medicine

Washington, DC

1. Shanon A, Martin DJ, Feldman W. Ultrasonographic stud. ies in the management of recurrent abdominal pain. Pedi atrics 1990;86:35—38

2. Majd M. Nuclear medicine. In: Kelaiis PP, King LR, Belman AB, eds. Clinical Pediatric Urology. 2nd ed. Philadelphia, PA: WB Saunders; 1985:150-154

3. Bejjani B, Belman AB. Ureteropelvic junction obstruction in newborns and infants. J Urol. 1982;128:770-779 4. Van Cangh PJ, Jorion JL, Wese FX, et al. Endoureteropye

lostomy: percutaneous treatment of ureteropelvic junction obstruction. J Urol. 1989;141:1317—1322

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Figure

Fig 1.Sonogram of left kidney demonstrating

Fig 1.Sonogram

of left kidney demonstrating p.2
Fig 2.Left, early scan reveals delayed uptake on left. Right, on delayed image the rightkidney has drained (B = bladder); however, left system is full (arrows outline renal pelvis).

Fig 2.Left,

early scan reveals delayed uptake on left. Right, on delayed image the rightkidney has drained (B = bladder); however, left system is full (arrows outline renal pelvis). p.2