A Rare Case of Hydronephrotic Multicystic Dysplasia with Ipsilateral Upper Segmental Megaureter in Adults


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A Rare Case of Hydronephrotic Multicystic Dysplasia with Ipsilateral Upper Segmental Megaureter in Adults
  Case Report 34  Hong Kong J Nephrol  • April 2008 • Vol 10 • No 1 A Rare Case of Hydronephrotic Multicystic Dysplasia withIpsilateral Upper Segmental Megaureter in Adults  Naveen Acharya, Shrawan K. Singh, Arun Jindal, Ravimohan Mavuduru, Arup K. Mandal Multicystic dysplasia in adults is a rare entity. It may present as a mass, with pain, or be detected incidentallyon imaging. Here, we present a case of hydronephrotic multicystic dysplasia with ipsilateral upper segmentalmegaureter in an adult female patient. To the best of our knowledge, this is the first such case described in theEnglish literature. [  Hong Kong J Nephrol  2008;10(1):34–6] Key words:  hydronephrosis, megaureter, multicystic dysplasia, ureteropelvic junction obstruction !"#$%&'()*+,-./0123456789:-;<12%=>?@ABCD !"#$%&'()*+,-./0123*456-789:;<=>?@ABC0A! !"#$%&'()*+,-./012342567 I NTRODUCTION Dilation of the genitourinary tract is not alwayssynonymous with obstruction. Congenital malformationmay present as dilated uropathy. The patient in this casewas being treated on the lines of ureteropelvic junctionobstruction with nonfunctioning kidney due to post-obstructive atrophy. The ureteric dilatation was thoughtto be due to some vessel crossing. However,intraoperatively, we found a dilated segmental ureterwith hydronephrotic kidney. This raised the suspicionof congenital malformation. Final histopathologyconfirmed the cause of the nonfunctioning kidney tobe multicystic dysplasia with upper segmental ureter. C ASE  R EPORT A 32-year-old female patient presented with a historyof left flank pain of 3 months’ duration. The pain wasdull aching in nature. The patient denied any history of hematuria, dysuria, graveluria or fever. She had knownhypertension that was being controlled with one drug.Her general physical examination was within normallimits. Systemic examination was unremarkable.Routine hematologic and biochemical parameterswere all within normal limits. Urine routine and culturewere normal. Ultrasound of the abdomen revealed asmall (7.5 cm in dimension) and hydronephrotic leftkidney with poor corticomedullary differentiation. Theright kidney was normal. Intravenous urography (IVU)revealed a normally functioning right kidney and non-excreting left kidney. DTPA (diethylene triaminepentaacetic acid) renogram revealed a similar finding,with the left kidney contributing only 5% of total renalfunction. Subsequent cystoscopy and left retrogradepyelography (RGP) revealed a hugely dilated pelvis andupper ureter up to the sacroiliac joint. The ureter wasnormal below that level. Delayed film revealedsignificant retention of contrast in the pelvis and upperureter.The patient underwent left nephrectomy via flankapproach. Intraoperative findings were of a small sac-like left kidney with minimal parenchyma. The renalpelvis and upper ureter were hugely dilated. There wasan area of narrowing at the junction of the upper andmiddle one third. The distal ureter was normal (Figures1 and 2). Department of Urology, Postgraduate Institute of Medical Sciences and Research, Chandigarh, India. Correspondence to:  Dr. Naveen Acharya, Department of Urology, Postgraduate Institute of Medical Sciences and Research,Chandigarh 160012, India.Fax: (+91) 172-2744401; E-mail: naveenchandra_in@yahoo.com   Hong Kong J Nephrol  • April 2008 • Vol 10 • No 135  Hydronephrotic multicystic dysplasia with upper segmental megaureter The postoperative course was uneventful.Histopathologic examination revealed changes typicalof multicystic dysplasia with areas of cyst formationand primitive mesenchyme. Myohypertrophy waspresent in sections from the area of segmentalnarrowing. A final histopathologic diagnosis of unilateral hydronephrotic multicystic dysplasia withsegmental megaureter was made. The patient is doingwell after 4 years of follow-up. D ISCUSSION Multicystic dysplasia represents a severe form of nongenetic dysplasia. Typically, the kidney does nothave a reniform shape and the calyceal drainge systemis absent (pyeloinfundibular atresia). In thehydronephrotic form, there is an identifiable dilatedpelvis. The multicystic kidney is an extreme form of hydronephrosis that occurs secondary to atresia of theureter or renal pelvis, which is a frequent concomitantcondition. The fact that the left kidney is the one that ismore often affected supports this view, because this isthe kidney that is more often associated with primaryobstructive megaureter and ureteropelvic junctionobstruction. In testing this hypothesis, severalinvestigators have attempted to establish an animalmodel by ligating the ureter at various points ingestation. This approach is not effective in middle orlate gestation; early ligation of the fetal lamb ureterproduces renal dysplasia but not multicystic dysplasia[1].In our case, the diagnosis of ureteropelvic junctionobstruction was considered based on ultrasound andIVU. We went ahead with DTPA as obstruction wasour primary concern. The scan showed a smallnonfunctioning left kidney. Since the patient wassymptomatic, we decided to proceed with RGP andnephrectomy. Intraoperatively, we found primarysegmental megaureter on the left side.Primary megaureter can be ascribed to segmentaldevelopmental delay of the ureter occurring at about20 weeks of gestation, with a possible pathogeneticinvolvement of autocrine transforming growth factor-beta overexpression [2]. This may be the primary eventinducing dysplastic changes in the ipsilateral kidney. Figure 1.  Nephroureterectomy specimen showing the dilated pelvisand ureter in segmental fashion. Figure 2.  Specimen cut open.   N. Acharya, et al 36  Hong Kong J Nephrol  • April 2008 • Vol 10 • No 1 Typically, the disease presents in infants as anabdominal mass. Less severely affected patients maybe diagnosed in adulthood during evaluation of pain,hematuria or hypertension [3], as in our case. Ipsilateralureter is usually absent or atretic. The contralateralsystem is frequently abnormal as well. Commoncontralateral abnormalities include ureteropelvic junction obstruction, vesicoureteral reflux andmegacalycosis [4]. The disease can be unilateral orbilateral and even segmental cases have been reported[5]. In addition, two or more distinct congenitalmalformations of the genitourinary tract can coexist inthe same patient. Perera Soler et al described a casewith coexistence of a unilateral multicystic dysplastickidney and contralateral megacalycosis associated withipsilateral distal segmental megaureter [4]. The presentcase had two coexisting anomalies: unilateralmulticystic dysplasia with upper segmental megaureter.This type of association is very rare and may thuspresent a diagnostic and management dilemma. In thepresent case, we had the dilemma of ureteropelvic junction obstruction and salvageability, and the finaldiagnosis was obtained only after histopathologicexamination. It is essential to recognize these types of rare anomalies since they are also an embryologicenigma. R EFERENCES 1.Glassberg K. Renal dysgenesis and cystic disease of the kidney.In: Walsh PC, Retik AS, eds. Campbell’s Urology , 8 th  edition.Philadelphia: Saunders, 1998:1959–60.2.Nicotina PA, Romeo C, Arena F, Romeo G. Segmentalupregulation of transforming growth factor-beta in thepathogenesis of primary megaureter. An immunocytochemicalstudy.  Br J Urol  1997;80:946–9.3.Ramaswamy S, Bhatnagar V, Mitra DK, Gupta AK. Congenitalsegmental giant megaureter.  J Ped Surg  1995;30:123–4.4.Perera Soler R, Ruiz González AP, Molini Menchón N, GarcíaNieto VM. Unilateral multicystic dysplastic kidney andcontralateral megacalycosis. An unusual association.  An Pediatr(Barc)  2004;60:473–6. [In Spanish]5.Ambrose S, Gould R, Trulock T, Parrot T. Unilateral multicysticdysplasia in adults.  J Urol  1981;128:366–8.
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