Challenging and Rare Cases in Urology Narmada Prasad Gupta
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Congenital Anomalies of Kidney and UreterChapter 1

1.1 ANTENATAL PELVIURETERIC JUNCTION OBSTRUCTION
 
INTRODUCTION
With routine use of prenatal ultrasonography, hydronephrosis is the most commonly diagnosed condition antenatally. The complete natural history of this condition is still not known. There has been much concern for the unilateral antenatally diagnosed pelviureteric junction (ADPUJ) obstruction due to the studies which note the vulnerability of developing kidneys. If the function of the affected kidney is well preserved, then conservative treatment with regular follow-up is recommended, otherwise surgery should be done.
 
CLINICAL PRESENTATION
A 28-weeks pregnant lady had prenatal ultrasound which revealed dilatation of the pelvicalyceal system of the right kidney (Fig. 1.1.1).
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Fig. 1.1.1: Prenatal ultrasound showing hydronephrosis in the right kidney
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COMMENTS
Prenatal ultrasound has become routine during pregnancy. A large number of children are diagnosed with prenatal hydronephrosis. It is important to differentiate between pelviureteric junction (PUJ) obstruction and posterior urethral valves. After birth, these children need further evaluation to decide for conservative treatment or surgery.
 
BIBLIOGRAPHY
  1. Beck AD. The effect of intra-uterine urinary obstruction upon the development of the fetal kidney. J Urol. 1971;105:784–9.
  1. Cartwright PC, Duckett JW, Keating MA, et al. Managing apparent ureteropelvic junction obstruction in the newborn. J Urol. 1992;148:1224–8.
  1. Chevalier RL, Gomez RA, Jones CE. Developmental determinants of recovery after relief of partial ureteral obstruction. Kidney Int. 1988;33:775–81.
  1. Gupta DK, Bajpai M, Chandrasekharan V, et al. Antenatally detected pelviureteric junction obstruction: safety of conservative management by our protocol. Indian J Urol. 2000;17:24–7.
  1. King LR, Hatcher PA. Natural history of fetal and neonatal hydronephrosis. Urology. 1990;35:433–8.
  1. Kitagawa H, Pringle KC, Stone P, et al. Postnatal follow-up of hydronephrosis detected by prenatal ultrasound: the natural history. Fetal Diagn Ther. 1998;13:19–25.
  1. Subramaniam R, Kouriefs C, Dickson AP. Antenatally detected pelvi-ureteric junction obstruction: concerns about conservative management. BJU Int. 1999;84(3):335–8.3
 
1.2 BILATERAL PELVIURETERIC JUNCTION OBSTRUCTION
 
INTRODUCTION
Bilateral ureteropelvic junction (UPJ) obstruction is rare. Traditionally, surgical management is by staged pyeloplasty to minimize the morbidity associated with performing procedures concurrently. With the advent of minimal invasive approaches concurrent surgical management can more readily be performed, laparoscopically or robotically.
 
CLINICAL CASES
 
Case 1
A 42-year-old male presented with right flank pain. Intravenous urogram (Fig. 1.2.1) revealed bilateral UPJ obstruction. Diuretic renogram confirmed the obstruction. Patient underwent bilateral pyeloplasty.
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Fig. 1.2.1: Case 1—Intravenous urogram showing bilateral PUJ obstruction
 
Case 2
A 39-year-old male presented with right flank pain. Intravenous urogram (Fig. 1.2.2) revealed right UPJ obstruction with hydronephrosis and left dilatation of pelvis with normal calyx. Dynamic renal scan confirmed obstruction on the right side but pattern III on left side. Right pyeloplasty was done and followed by left pyeloplasty.4
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Fig. 1.2.2: Case 2—Intravenous urogram
 
Case 3
A 45-year-old female presented with dull abdominal pain. Intravenous urogram (Fig. 1.2.3) revealed bilateral pelviureteric junction (PUJ) obstruction. Bilateral pyeloplasty was done in the one sitting.
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Fig. 1.2.3: Case 3—Intravenous urogram
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COMMENTS
Bilateral PUJ obstruction can occur in about 10% of cases of PUJ obstruction, can be diagnosed synchronously, or later in life. Concurrent bilateral pyeloplasty can be done safely.
 
BIBLIOGRAPHY
  1. Freilich DA, Nguyen HT, Borer J, et al. Concurrent management of bilateral ureteropelvic junction obstruction in children using robotic-assisted laparoscopic surgery. Int Braz J Urol. 2008;34(2):198–204; discussion 204–5.
  1. Koff SA, Mutabagani KH. Anomalies of the kidney. In: Gillenwater JY, Grayhack JT, Howards SS, Mitchell ME (Eds). Adult and Pediatric Urology, 4th edition. Lippincott Williams and Wilkins;  Philadelphia:  2002.p.2129.6
 
1.3 CIRCUMCAVAL URETER
 
INTRODUCTION
It is commonly referred to as circumcaval or retrocaval ureter. More appropriate term may be preureteral vena cava. It is due to a congenital abnormality in development of the vena cava where the right ureter courses posterior to the inferior vena cava and partially encircles it. Although the lesion is congenital, patients usually present in the third to fourth decade of life.
 
EMBRYOLOGY
It results from persistence of the subcardinal venous system that anomalously forms the inferior vena cava (IVC) (Fig. 1.3.1).
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Fig. 1.3.1: Embryology of circumcaval ureter
 
INCIDENCE
The incidence of circumcaval ureter is 1 in 1100. It commonly presents in the third to fourth decade of life, being more common in females and mostly on the right side.7
 
CLINICAL PRESENTATION
Abdominal and flank pain, recurrent urinary tract infection, and hypertension.
 
Types of Circumcaval Ureter
 
Type I
Ureter with an S-shaped, fish hook, or Shepherd's crook appearance, more hydronephrosis and more symptomatic (Fig. 1.3.2A).
 
Type II
A less angulated “sickle-shaped” ureteral deformity is classified as type II, less hydronephrosis and less symptomatic (Fig. 1.3.2B).
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Figs 1.3.2A and B: Types of circumcaval ureter
 
IMAGING
Intravenous pyelogram (IVP) or contrast enhanced computed tomography (CECT) with urogram (Figs 1.3.3 to 1.3.8).
 
TREATMENT
The classical treatment for retrocaval ureter consists of separating the ureter, re-anastomosing its stumps and replacing the ureter in its usual position while maintaining its patency. Pyeloureterostomy or ureteroureterostomy can be done and the approach may be open laparoscopic transabdominal/ retroperitoneoscopic/robotic (Fig. 1.3.9).8
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Fig. 1.3.3: Intravenous pyelogram (IVP) showing hydroureteronephrosis (HDUN) in upper ureter
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Fig. 1.3.4: Contrast enhanced computed tomography (CECT) showing reverse J picture
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Fig. 1.3.5: Contrast enhanced computed tomography (CECT) showing reverse J picture
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Fig. 1.3.6: Computed tomography (CT) Urogram showing reverse J picture
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Fig. 1.3.7: Non Contrast CT showing hydroureteronephrosis (HDUN) with thickening around vena cava
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Fig. 1.3.8: Contrast enhanced computed tomography (CECT) showing hydroureteronephrosis (HDUN) with enhanced mass lesion around vena cava
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Fig. 1.3.9: Intraoperative picture showing caval division and cirmumcaval ureter
 
BIBLIOGRAPHY
  1. Gupta NP, Hemal AK, Singh I. Retroperitoneoscopic ureterolysis and reconstruction of retrocaval ureter. J Endourol. 2001;15(3):291–3.
  1. Kenawi MM, Williams DI. Circumcaval ureter: a report of four cases in children with a review of the literature and a new classification. Br J Urol. 1976;48(3):183–92.
  1. Salonia A, Maccagnano C, Lesma A, et al. Diagnosis and treatment of the circumcaval ureter. European Urology supplements. 2006;5:449–62.11
 
1.4 CROSSED RENAL FUSED ECTOPIA
 
INTRODUCTION
Crossed fused ectopia of the kidneys is the most common fusion abnormality of the urinary tract after horseshoe kidney. In this abnormality, both kidneys are present on the same side of the body (in most cases fused, with two separate ureters arising from the two kidneys). The crossed kidney has an aberrant vascular supply. The ureter arising from the crossed over kidney travels back to the opposite side and enters the bladder. Although crossed renal ectopia is usually clinically silent, they are sometimes responsible for infection and urinary stones and may be associated with a high incidence of ureteropelvic junction obstruction, vesicoureteral reflux and renal multicystic dysplasia.
 
CLINICAL PRESENTATION
A 26-year-old male presented with complaints of hematuria off and on for 6 months duration, and pain in right lower abdomen and periumbilical region for 4 months duration.
No association with bowel or bladder symptoms, no history of blood in stool or altered bowel habits and no history of weight loss.
 
GENERAL EXAMINATION
Pulse: 80/min, Blood pressure: 124/86 mm Hg, Per abdominal examination: Soft, non-tender, no lump palpable.
 
INVESTIGATIONS
  • CBC and RFT: Normal, Urine R/M-normal—WBC: 6‒10/hpf, RBC 3+, Urine C/S: Sterile, 24 hrs Urine—albumin: 71 mg%, creatinine: 31 mg/dL, calcium: 2 mg/dL, po4: 7 mg%
  • Kidney, Ureter, Bladder (KUB) X-ray (Fig. 1.4.1): 2 × 2.5 cm stone seen in lower abdomen
  • IVP (Fig.1.4.2): Left kidney—placed on right side with a stone in the left renal pelvis, Pelvicalyceal system (PCS) dilated on left side. Rotation of left kidney noted. Urinary bladder normal
  • CECT (Figs 1.4.3 and 1.4.4): 2.5 × 2.2 cm stone in extrarenal pelvis of crossed fused ectopic left kidney. The left kidney at L3–5 location in right paraspinal location and fused with lower pole of right kidney. Left ureter crossing midline towards the left side with drainage at the normal left vesicoureteric junction (VUJ).
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Fig.1.4.1: Plain X-ray of the kidney, ureter, bladder (KUB) region showing radio-opaque shadow in right lumbar vision
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Fig.1.4.2: Intravenous pyelogram showing left crossed renal ectopia with calculus
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Fig. 1.4.3: Noncontrast computed tomography (NCCT) of the abdomen
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Fig. 1.4.4: Contrast enhanced computed tomography (CECT) of the abdomen
 
TREATMENT
Open pyelolithotomy was done.
 
COMMENTS
Crossed fused ectopia of kidney associated with stones can be managed by open surgery, and recently by percutaneous nephrolithotomy (PCNL) or laparoscopic-assisted PCNL.
 
BIBLIOGRAPHY
  1. Abeshouse BS, Bhisitkul I. Crossed renal ectopia with or without fusion. Urol Int. 1959;9:63–91.
  1. Bernstein I. Developmental abnormalities of renal parenchyma, renal hypoplasia and dysplasia. Pathobiol Annu. 1968;3:213–5.
  1. Hertz M, Rubinstein ZJ, Shahin M, et al. Crossed renal ectopia: clinical and radiological findings in 22 cases. Clin Radiol. 1977;28:339–44.
  1. Mohammad M. Association of multicystic dysplasia and crossed non fused renal ectopia: a case report. SJKDT. 1997;8(21):148–51.
  1. Mouriquand P. Renal fusions and ectopia. In: O'Neill JA, Rowe MI, Grosfeld JL, Fonkalsrud EW, Coran AG (Eds), 5th edition. Pediatric Surgery. Mosby:  St Louis;  1998.pp. 1583–8.
  1. Patel TV, Singh AK. Crossed fused ectopia of the kidneys. Kidney Int. 2008;73(5):662.14
 
1.5 RETROILIAC URETER WITH CONTRALATERAL TRANSVERSE MALROTATION OF KIDNEY
 
INTRODUCTION
Retroiliac ureter is a well recognized, although extremely rare, congenital anomaly. It can be associated with other congenital anomalies.
 
CLINICAL PRESENTATION
An 18-year-old male presented with the complaints of nonradiating central abdominal pain, intermittent moderate fever and diarrhea for 3 months duration. He also had nausea, anorexia and weight loss. He noticed a lump in the lower abdomen. Abdominal examination revealed a 10 × 6 cm sausage shaped, smooth, firm, non-tender, midline, intra-abdominal lump in the hypogastrium and extending into both iliac region. There were no associated congenital anomalies.
 
INVESTIGATIONS
Hemoglobin (Hb): 6.7 gm, Blood urea: 72 mg/dL, Serum creatinine: 2.6 mg/ dL. Creatinine clearance was 20 mL/ minute. Urine examination revealed pus cells and urine culture was positive for Escherichia coli. Plain X-ray KUB region revealed a radiopaque stone opposite L4 vertebra on left side. Intravenous urogram revealed a left hydronephrotic kidney opposite L4-S1 with nonvisualized right kidney. Ultrasound revealed a transversely oriented left kidney with moderate hydronephrosis that crossed the midline. The right kidney was in normal position with hydroureteronephrosis. A dynamic renal scan confirmed a non-functioning right kidney with transverse poorly functioning left kidney. A voiding cystourethrogram revealed normal capacity bladder with grade IV reflux on right side. Cystopanendoscop revealed normal left and gaping right ureteric orifice. A left retrograde ureteropyelogram revealed ureteropelvic junction obstruction with transverse kidney.
 
TREATMENT
Abdominal exploration revealed left kidney was malrotated transversely with a dilated pelvis cephalad to the kidney. The calculus was extracted through vertical pyelotomy and nephrostomy was done. A nephrostogram (Fig. 1.5.1) done on 14th day revealed no drainage. Re-exploration was done. Right kidney was found hydronephrotic with dilated ureter coursed posterior to the iliac artery. Right nephrectomy was done and ureter was divided 3 cm above the iliac vessels. The distal half of the ureter freed and brought anterior to the iliac artery and crossed ureterocalyceal anastomosis was done with the left inferior calyx after excision of a disc of the parenchyma. A stent and nephrostomy was kept. Postoperative nephrostogram (Fig. 1.5.2) revealed a good drainage with patent anastomosis.15
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Fig. 1.5.1: Retrograde pyelogram (RGP)
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Fig. 1.5.2: Postoperative nephrostogram
 
FOLLOW-UP
After 3 months, postoperative cystogram (Fig. 1.5.3) revealed right refluxing ureter. Right ureteral advancement procedure was done for the vesicoureteral (VU) reflux. His renal function improved and was normal for a 5-year follow-up.
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Fig. 1.5.3: Postoperative cystogram
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COMMENTS
A retroiliac ureter is very rare and preoperative diagnosis is difficult. By crossed ureteral re-implantation single functioning kidney can we salvaged by use of contralateral ureter of nonfunctioning kidney.
 
BIBLIOGRAPHY
  1. Dee E: Anomalous relationship between ureter and external iliac artery. Urol. 1940;44:207–15.
  1. Gupta NP, Gill IBS, Bhatia V. Retroiliac Ureter with contralateral Transverse malrotation of kidney treated by crossed ureterocaliceal anastomosis. Urol. 1987;138(1):1181.17
 
1.6 DOUBLE MOIETY WITH PELVIURETERIC JUNCTION OBSTRUCTION LOWER MOIETY
 
INTRODUCTION
Ureteropelvic junction (UPJ) obstruction may also occur in the upper or lower, usually lower half of a duplicated collecting system.
 
CLINICAL CASES
 
Case 1
A 26-year-old female presented with vague pain in abdomen. Ultrasound (Fig.1.6.1) revealed double moiety with pelviureteric junction (PUJ) obstruction inferior moiety. Intravenous urogram (Fig.1.6.2) and CECT (Fig.1.6.3) showing double moiety with PUJ obstruction inferior moiety. Treatment was done by right pyeloplasty of the inferior moiety.
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Fig. 1.6.1: Case 1—Ultrasound showing double moiety with PUJ obstruction inferior moiety
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Fig. 1.6.2: Case 1—Intravenous pyelogram (IVP) showing double moiety with PUJ obstruction inferior moiety
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Fig. 1.6.3: Case 1—Contrast enhanced computed tomography (CECT) showing double moiety with PUJ obstruction inferior moiety
 
Case 2
A 32-year-old female presented with pain in upper abdomen. Intravenous pyelogram (IVP) (Fig. 1.6.4) revealed double moiety with PUJ obstruction of inferior moiety. Right pyeloplasty of the inferior moiety was done.19
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Fig. 1.6.4: Case 2—Intravenous pyelogram (IVP) showing double moiety with PUJ obstruction inferior moiety
 
Case 3
A 32-year-old female presented with right flank pain. Contrast enhanced computed tomography (Fig. 1.6.5) revealed double moiety with obstruction of inferior moiety. Dynamic renal scan revealed very poorly functioning inferior moiety. Right lower pole nephrectomy was done.
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Fig. 1.6.5: Case 3—Contrast enhanced computed tomography (CECT) showing double moiety with PUJ obstruction inferior moiety
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COMMENTS
Careful preoperative evaluation of patients with ureteropelvic junction obstruction will usually identify segmental obstruction in a duplicated system. Treatment should be individualized based on site of obstruction and degree of function remaining in the affected segment.
 
BIBLIOGRAPHY
  1. Amar AD. Congenital hydronephrosis of lower segment in duplex kidney. Urology. 1976;7:480–5.
  1. Joseph DB, Bauer SB, Colodny AH, et al. Lower pole ureteropelvic obstruction and incomplete renal duplication. J Urol. 1989;141(4):896–9.21
 
1.7 ECTOPIC PELVIC KIDNEY
 
INTRODUCTION
An ectopic kidney is a birth defect in which a kidney is located below, above, or on the opposite side of its usual position. About one in 900 people has an ectopic kidney.
Abnormal positions are described as pelvic, iliac, abdominal, thoracic and crossed, with pelvic location being the most common. An ectopic kidney may not cause any symptom and may function normally, even though it is not in its usual position. No treatment for an ectopic kidney is needed if urinary function is normal and no blockage of the urinary tract is present. Common complications of ectopic kidney are infection, stone formation and obstruction.
 
CLINICAL PRESENTATION
A 32-year-old male presented with lower abdominal pain of 6 months duration. On examination, there were no positive findings.
 
INVESTIGATIONS
Plain X-ray KUB region (Fig. 1.7.1) revealed no radiopaque shadow. Intravenous urogram (Figs 1.7.2A and B) revealed left ectopic kidney with hydronephrosis, with normal functioning right kidney.
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Fig. 1.7.1: Plain X-ray KUB region
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Figs 1.7.2A and B: Intravenous urogram
 
TREATMENT
Left calicovesicostomy was done. Patient did well after surgery.
 
COMMENTS
Pelvic ectopic kidney with hydronephrosis is an extremely rare condition. Due to proximity of the kidney with the bladder, pelvio/calicovesicostomy can be done with good outcome.
 
BIBLIOGRAPHY
  1. Bauer SB. Anomalies of the upper urinary tract. In: Wein A (Ed). Campbell-Walsh Urology, 9th edition. Saunders Elsevier;  Philadelphia:  2007.pp. 3269–3304.
  1. Colley N, Hooker JG. Prenatal diagnosis of pelvic kidney. Prenat Diagn. 1989;9(5):361–3.
  1. Hill LM, Peterson CS. Antenatal diagnosis of fetal pelvic kidneys. J Ultrasound Med. 1987;6(7):393–6.
  1. King KL, Kofinas AD, Simon NV, et al. Prenatal diagnosis of fetal pelvic kidney: a case report. J Reprod Med. 1993;38(3):225–6.
  1. Moore KL. The Developing Human: Clinically Oriented Embryology, 3rd edition. WB Saunders;  Philadelphia:  1982.pp. 255–71.
  1. Walsh P, Gittes R, Perlmutter A, et al. Campbell's Urology, Second Volume, Fifth edition. WB Saunders;  Philadelphia:  1986.pp. 1674–5.23
 
1.8 ECTOPIC URETER
 
INTRODUCTION
An ectopic ureter is one which is not opening at the normal location at the angle of the trigone. Eighty percent of ectopic ureter occurs with ureteral duplication; however, single system ectopic ureter can also occur. Ectopic ureter is more common in females. Ectopic ureter can be unilateral or bilateral. Ectopic ureter may open into the urinary tract or genital tract following an ectopic pathway. Opening of ectopic ureter from the vesical neck distally can cause symptoms of obstruction; reflux and in females, incontinence. It can be associated with other congenital anomalies.
 
CASE 1
 
Clinical Presentation
A 14-year-old female presented with incontinence of urine since birth. She was voiding normally along with one to two pads leakage of urine.
 
Investigations
Intravenous urogram (Fig. 1.8.1) revealed right double collecting system with dilated superior moiety. Antegrade pyelogram (Figs 1.8.2A and B) through superior moiety was done which revealed dilated superior moiety and ureter with ectopic opening of ureter.
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Fig. 1.8.1: Case 1—Intravenous urogram
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Figs 1.8.2A and B: Case 1—Antegrade pyelogram
 
Treatment
Superior moiety nephroureterectomy was done. As a result, her incontinence stopped.
 
CASE 2
 
Clinical Presentation
A 38-year-old female presented with incontinence of urine few drops along with normal voiding.
 
Investigations
Intravenous urogram (Fig. 1.8.3) revealed right double moiety with poorly functioning dilated superior moiety with ectopic opening.
 
Treatment
Right superior moiety nephroureterectomy was done which stopped her incontinence.25
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Fig. 1.8.3: Case 2—Intravenous urogram
 
CASE 3
 
Clinical Presentation
A 15-year-old female presented with history of paradoxical incontinence since birth. She had ectopic anus for which cut back operation was done at birth. Local examination showed patulous urethral orifice with two separate vaginal introitus leading to separate 5 cm deep vagina. There was one cervix and one uterus on each side (uterus didelphus).
 
Investigations
Intravenous urogram showed normal upper tract. Voiding cystourethrogram (Fig. 1.8.4) showed grade III left vesicoureteral reflux with a wide open bladder neck and contrast in both vagina. Cystopanendoscopy revealed an absent trigone, both ureteric orifices at the bladder neck at 3 and 9 O'clock position. The length of the urethra was 3 cm.
 
Treatment
Cohen's bilateral transtrigonal ureteroneocystostomy with Young-Dees-Leadbetter bladder neck reconstruction and Marshall-Marchetti-Krantz urethral fixation was done. Postoperative recovery was uneventful and continence was achieved.26
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Fig.1.8.4: Case 3—Voiding cystourethrogram
 
COMMENTS
Ectopic ureter usually occurs with double collecting system but can also occur in a single system and can be unilateral or bilateral. If symptomatic, needs surgical treatment.
 
BIBLIOGRAPHY
  1. Borer JG, Bauer SB, Peters CA, et al. A single-system ectopic ureter draining an ectopic dysplastic kidney: delayed diagnosis in the young female with continuous urinary incontinence. Br J Urol. 1998;81(3):474–8.
  1. Brannan W, Henry HH 2nd. Ureteral ectopia: report of 39 cases. J Urol. 1973;109(2):192–5.
  1. Khan M, Gupta NP. Bilateral single system ureteral ectopia. The Medicine & Surgery. 1988;28:43–4.27
 
1.9 EQUIVOCAL PELVIURETERIC JUNCTION OBSTRUCTION
 
INTRODUCTION
Intravenous urogram is the most common investigation for evaluation of pelviureteric junction (PUJ) obstruction. Classic urographic features of equivocal PUJ obstruction are dilatation of renal pelvis, minimum or no calyceal dilation and minimum filling of ureter. Diuresis renogram will differentiate whether functional obstruction is present or not. In the absence of obstruction, these patients can be kept under observation and unnecessary surgery can be avoided.
 
CLINICAL PRESENTATION
A 35-year-old male presented with right flank pain off and on for a duration of 6 months. Examination was normal.
 
INVESTIGATIONS
Intravenous urogram (Fig. 1.9.1) revealed right dilatation of the renal pelvis, with minimum dilatation of the calyces and the ureter was partially filled. Diuresis renogram revealed Type 3A pattern.
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Fig. 1.9.1: Intravenous pyelogram (IVP) showing features of equivocal PUJ obstruction—dilated pelvis, cupping of calyx maintained and partial filling of ureter
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TREATMENT
He was kept under follow-up. He was asymtomatic there was no further deterioration of renal function.
 
COMMENTS
It is important to identify cases of hydronephrosis without functional obstruction. They can be followed-up periodically, and if asymptomatic and without any complications, then surgery can be avoided.
 
BIBLIOGRAPHY
  1. O'Reilly PH, Testa HJ, Charlten EE. Diuresis renography in equivocal urinary tract obstruction. Br J Urol. 1978;50(2):76–80.
  1. Singh SS, Gupta NP, Padhy AK, et al. Diuresis renography in the evaluation of equivocal PUJ obstruction. Ind J Urol. 1985;2:16–8.29
 
1.10 EXTRARENAL PELVIS AND CALYCES
 
INTRODUCTION
Extrarenal calyces (wherein the calyces and renal pelvis lie outside the renal parenchyma) is one of the rare anomaly of the collecting system. It may develop due to a disparity resulting from slow development of the metanephric tissue or to a relatively rapid development of the ureteric bud. This anomaly may be associated with other anomalies of the urogenital system. Extrarenal pelvis and calyces usually do not produce symptoms, but failure to drain normally may lead to stasis, infection and calculi formation. Surgery is reserved for those patients in whom infection or obstruction is present.
 
CLINICAL CASES
 
Case 1
A 28-year-old male presented with vague pain in the left flank. Intravenous urogram (Fig. 1.10.1) revealed left malrotated ectopic kidney with extrarenal pelvis. Dynamic renal scan revealed no obstruction. He was kept under observation.
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Fig. 1.10.1: Case 1—Intravenous urogram
 
Case 2
A 35-year-old female presented with left flank pain. Intravenous urogram (Fig. 1.10.2) revealed bilateral extrarenal pelvis with left PUJ obstruction. Dynamic renal scan revealed obstruction on the left side. She underwent left pyeloplasty.30
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Fig. 1.10.2: Case 2—Intravenous urogram
 
Case 3
A 32-year-old male presented with vague pain in the abdomen. Intravenous urogram revealed a right double collecting system with an extrarenal pelvis of the inferior moiety (Fig. 1.10.3). Dynamic renal scan revealed no obstruction. He was kept under observation.
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Fig. 1.10.3: Case 3—Right double collecting system with extrarenal pelvis of inferior moiety
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COMMENTS
Extrarenal pelvis and calyces can be incidental findings on imaging. If asymptomatic, can be observed, otherwise; surgery is required.
 
BIBLIOGRAPHY
  1. Nataraju G, Nandeesh BN, Gayathri MN. Extrarenal calyces: a rare anomaly of the renal collecting system. Indian J Pathol Microbiol. 2009;52(3):368–9.
  1. Rao S, Nikolai O, Avinash J, et al. Bilateral extrarenal calyces, renal pelvis and associated variations of renal vessels: a rare variation of the renal collecting system. Int J Anat Var (IJAV). 2012;5:62–4.32
 
1.11 INCOMPLETE DUPLICATION OF URETER
 
INTRODUCTION
Ureteral duplication is the most common ureteral anomaly. It can be complete duplication, where second ureter opens in the bladder, urethra or ectopic position. In incomplete duplication, there is bifid ureter. In many cases, it can be an accidental finding causing no functional disturbances, otherwise it can be associated with upper tract stasis, obstruction and reflux. There is a higher incidence of duplicated ureter in cases with urinary infection. It is more common in females.
 
CLINICAL PRESENTATION
A 35-year-old female presented with vague pain in the abdomen.
 
INVESTIGATIONS
Intravenous urogram (Fig.1.11.1) revealed a left incomplete duplication of ureter. Urine examination was normal.
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Fig. 1.11.1: Intravenous urogram
 
TREATMENT
She was counseled and was advised for observation.33
 
COMMENTS
Duplication of ureter can be detected on imaging. Asymptomatic cases without obstruction can be kept on follow-up.
 
BIBLIOGRAPHY
  1. Colosimo C. Double and bifid ureters. Urologia. 1938;5:239–67.34
 
1.12 MEGAPELVIS
 
INTRODUCTION
Megapelvis means a big renal pelvis. It is a condition in which there is congenital dilation of the renal pelvis due to smooth muscle aplasia. This causes hydronephrosis also. Renal pelvis is the area of kidney where urine is collected before passing through ureter. When urine gets backed up, the renal pelvis gets dilated. It can occur in adults as well as infants.
 
CLINICAL PRESENTATION
A 20-year-old female presented with bilateral dull flank pain for 6 months duration. No urinary symptoms, no operative intervention in the past and no physical findings on examination.
 
INVESTIGATIONS
Urine R/M: Normal, Urine C/S: Sterile, Renal function test: Normal. Ultrasound (Fig. 1.12.1): showing dilatation of pelvis of both kidneys. Plain X-ray (Fig. 1.12.2): No radiopaque shadow. IVP (Fig. 1.12.3): showing dilatation of both pelvis and calyces but cupping is maintained. Dynamic Renal Scan (Fig. 1.12.4): showing normal function of both kidneys with normal drainage. MRI T1 images (Fig. 1.12.5), MRI T2 images (Fig.1.12.6) and MRI T2 coronal images (Fig.1.12.7) showing dilated pelvis and calyces with no obstruction.
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Fig. 1.12.1: Ultrasound
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Fig. 1.12.2: Plain X-ray
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Fig. 1.12.3: Intravenous pyelogram
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Fig. 1.12.4: Dynamic renal scan
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Fig. 1.12.5: MRI T1 images
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Fig. 1.12.6: MRI T2 images
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Fig. 1.12.7: MRI T2 coronal images
 
MANAGEMENT
The patient was counseled for conservative treatment and follow-up.
 
COMMENTS
Megapelvis is a very rare condition and less reference about it is available in the literature. If there is no obstruction in the dynamic renal scan (DRS), patients can be kept under follow-up.38
1.13 PRIMARY MEGAURETER
 
INTRODUCTION
Megaureter is a ureter that exceeds the upper limits of normal size. Any ureter greater than 7 mm in diameter is defined as a megaureter based on measurements in fetuses greater than 30 weeks gestation and children younger than 12 years.
Primary megaureter results from a functional or anatomical abnormality involving the ureterovesical junction. Megaureter can be obstructive or nonobstructive and can be refluxing or nonrefluxing.
 
TREATMENT
Treatment is decided on the basis of obstruction and associated complications like stones, infection, etc.
 
CLINICAL PRESENTATION
Nine cases are presented with variable symptoms and their imaging results (Figs 1.13.1 to 1.13.9).
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Fig. 1.13.1: Case 1—Intravenous pyelogram: rat tail appearance of megaureter
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Fig.1.13.2: Case 2—Intravenous pyelogram: left megaureter
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Fig. 1.13.3: Case 3—Intravenous pyelogram: right megaureter
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Fig. 1.13.4: Case 4—Intravenous pyelogram: right megaureter
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Fig. 1.13.5: Case 5—Intravenous pyelogram: bilateral megaureter
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Fig. 1.13.6: Case 6—Micturating cystourethrogram (MCU): Bilateral refluxing megaureter
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Fig. 1.13.7: Case 7—Intravenous pyelogram: single ectopic kidney with megaureter
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Fig. 1.13.8: Case 8—Intravenous pyelogram: single pelvic ectopic kidney with megaureter
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Fig. 1.13.9: Case 9—Ultrasound showing left megaureter
 
COMMENTS
Most cases of primary megaureter resolve spontaneously or improve without loss of function or development of symptoms. Careful observation allows surgery to be delayed beyond the neonatal period in most patients. Longterm follow-up is recommended because symptoms can develop years later. Washout pattern and age at presentation are statistically significant predictors of spontaneous resolution.
 
BIBLIOGRAPHY
  1. Baskin LS, Zderic SA, Snyder HM, et al. Primary dilated megaureter: long-term followup. J Urol. 1994;152(2 pt 2):618–21.
  1. Calisti A, Oriolo L, Perrotta ML, et al. The fate of prenatally diagnosed primary nonrefluxing megaureter: do we have reliable predictors for spontaneous resolution? Urology. 2008;72(2):309–12.
  1. Cussen LJ. Dimensions of the normal ureter in infancy and childhood. Invest Urol. 1967;5(2):164–78.
  1. Dorairajan LN, Hemal AK, Gupta NP, et al. Primary obstructed megaureter in adults: need for an aggressive management strategy. Int Urol Nephrol. 1999;31(5):633–41.
  1. Gimpel C, Masioniene L, Djakovic N, et al. Complications and long-term outcome of primary obstructive megaureter in childhood. Pediatr Nephrol. 2010;25(9):1679–86.
  1. Hemal AK, Ansari MS, Doddamani D, et al. Symptomatic and complicated adult and adolescent primary obstructive megaureter-indications for surgery: analysis, outcome, and follow-up. Urology. 2003;61(4):703–7.42
  1. Keating MA, Escala J, Snyder HM, et al. Changing concepts in management of primary obstructive megaureter. J Urol. 1989;142(2 pt 2):636–40.
  1. Liu HY, Dhillon HK, Yeung CK, et al. Clinical outcome and management of prenatally diagnosed primary megaureters. J Urol. 1994;152(2 pt 2):614–7.
  1. McLellan DL, Retik AB, Bauer SB, et al. Rate and predictors of spontaneous resolution of prenatally diagnosed primary nonrefluxing megaureter. J Urol. 2002;168(5):2177–80.
  1. Peters CA, Mandell J, Lebowitz RL, et al. Congenital obstructed megaureters in early infancy: diagnosis and treatment. J Urol. 1989;142(2 pt 2):641–5.
  1. Shukla AR, Cooper J, Patel RP, et al. Prenatally detected primary megaureter: a role for extended followup. J Urol. 2005;173(4):1353–6.43
 
1.14 PARAPELVIC CYST
 
INTRODUCTION
Parapelvic cysts of the kidneys are simple renal cysts, which are adjacent to the renal pelvis or the renal sinus. They are usually derived from lymphatic vessels or from renal parenchyma. Normally, there are no complaints. Parapelvic cysts can compress the pyelocalyceal system or ureter, cause flank pain or lead to other symptoms of urinary obstruction. Renal ultrasound should be sufficient for the diagnosis; in doubtful situations an intravenous pyelography, computed tomography (CT) of abdomen or renal scintigraphy can rule out urinary obstruction.
 
CLINICAL PRESENTATION
A 30-year-old female presented with left hypochondrium and flank discomfort, post-meal fullness. Her history suggested no urinary symptoms, normal bowel functions and no operative intervention in the past.
 
EXAMINATION
No palpable mass or organomegaly, no lymphadenopathy and no pedal edema.
 
INVESTIGATION
Hemogram, RFT, LFT: Normal. Urine R/M: Normal; Urine culture: Sterile, UGI endoscopy: Normal. Barium meal and follow through: Normal; USG: Normal right kidney, 5 × 6 cm parapelvic cyst in left kidney. CECT revealed parapelvic cyst (Fig.1.14.1).
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Fig. 1.14.1: Parapelvic cyst revealed by contrast enhanced computed tomography (CECT)
 
TREATMENT
Retroperitoneoscopic cyst deroofing was done.44
 
COMMENTS
Ultrasonography and CT scan are the main diagnostic methods. Enhanced CT is extremely helpful in differential diagnosis of hydronephrosis. Surgical management is suitable for big cysts, lumbar pain, hematuria, hypertension and other complications.
 
BIBLIOGRAPHY
  1. Israel GM, Bosniak MA. An update of the Bosniak renal cyst classification system. Urology. 2005;66(3):484–8.
  1. Li ZL, Kong CZ, Wang Y, et al. Parapelvic cyst of kidney. Zhonghua Wai Ke Za Zhi. 2003;41(3):208–10.45
 
1.15 PELVIURETERIC JUNCTION OBSTRUCTION WITH ECTOPIC KIDNEY
 
INTRODUCTION
Pelvic ectopic kidney can be associated with congenital PUJ obstruction with hydronephrosis. Due to close proximation with the bladder, the surgical treatment options are calycovesical or pyelovesical anastomosis which provide excellent drainage.
 
CLINICAL PRESENTATION
A 22-year-old male presented with periumbilical pain for 2 years, no operative intervention and no bowel/bladder symptoms.
 
INVESTIGATIONS
Urine routine and culture, and renal function were normal. Plain X-ray KUB region (Fig. 1.15.1): No radiopaque calculus; Intravenous urogram (Fig. 1.15.2) showed normal right kidney and ectopic left kidney. Intravenous urogram with delayed films (Fig. 1.15.3) show left hydronephrosis.
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Fig. 1.15.1: Plain X-ray KUB region
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Fig. 1.15.2: Intravenous urogram
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Fig. 1.15.3: Intravenous urogram with delayed films
 
TREATMENT
Pyelovesicostomy was performed.
 
COMMENTS
Pyelovesicostomy is a good surgical option for patients with PUJ obstruction with hydronephrosis in ectopic pelvic kidney.
 
BIBLIOGRAPHY
  1. Hsieh MY, Ku MS, Tsao TF, et al. Rare case of atrophic ectopic kidney with giant hydronephrosis in a 7-year-old girl. Urology. 2013;81(3):655–8.47
 
1.16 PERSISTENT UMBILICAL ARTERY CAUSING URETERAL OBSTRUCTION
 
INTRODUCTION
Hyams, in 1929, first described vascular obstruction of the distal ureter and illustrated the anomalous routes of the umbilical arteries. Since then only few cases have been described in the literature. The diagnosis is usually made at laparotomy, frequently following previous failed attempts at endourological management.
 
CLINICAL PRESENTATION
A 32-year-old female presented with the history of recurrent left flank pain of 1 year duration. She had no urinary complaints, was married for 3 years and was investigated for infertility.
 
INVESTIGATIONS
Intravenous urogram (Fig. 1.16.1) revealed moderate hydroureteronephrosis on the left side, with ureter seen 1 cm distal to sacroiliac joint. Magnetic resonance (MR) urogram (Fig. 1.16.2) revealed left hydroureteronephrosis. Left retrograde pyelogram (Fig. 1.16.3) and drainage film (Fig. 1.16.4) revealed hydroureteronephrosis with sudden cut of lower ureter and was hook shaped. Ureteric catheter could not be negotiated beyond 8 cm.
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Fig.1.16.1: Intravenous urogram
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Fig. 1.16.2: Magnetic resonance urogram
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Fig. 1.16.3: Retrograde pyelogram
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Fig. 1.16.4: Retrograde pyelogram drainage film
 
TREATMENT
On exploration, a 4-mm vessel was seen crossing over the ureter at the site of narrowing of the ureter. The ureter was divided and ureteral reimplantation was done.
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Fig. 1.16.5: Postoperative intravenous urogram
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FOLLOW-UP
Postoperative intravenous urogram (Fig. 1.16.5) after 3 months showed nonobstructive drainage on left side.
 
COMMENTS
Persistent umbilical artery causing ureteral obstruction is a very rare condition and should be kept as a differential diagnosis in lower ureteric obstruction.
 
BIBLIOGRAPHY
  1. Gupta NP, Kumar M, Karan SC, et al. Lower ureteral obstruction due to a persistent umbilical artery. Urol Int. 1999;63(4):249–51.
  1. Hyams J. Aberrant blood vessels as a factor in lower ureteric obstruction. Surg Gynecol Obstet. 1929;48:474–9.50
 
1.17 PELVIURETERIC JUNCTION OBSTRUCTION WITH CALCULI
 
INTRODUCTION
Pelviureteric junction obstruction can be associated with renal calculi. The etiology for calculus is due to stasis of urine and can be associated with metabolic abnormalities. These stones are usually round or facetted and can be multiple. Management is removal of calculi along with obstruction by endopyelotomy or more commonly performed pyeloplasty.
 
CLINICAL CASES
 
Case 1
A 45-year-old female presented with left flank pain. Plain X-ray KUB region (Fig. 1.17.1) revealed multiple radiopaque round shadows in the left renal area. Intravenous pyelogram (Fig. 1.17.2) revealed left pelviureteric junction obstruction with hydronephrosis with calculi. Left Anderson-Hynes pyeloplasty with removal of calculi was done. Figure 1.17.3 shows multiple calculi with excised pelvis. She recovered well.
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Fig. 1.17.1: Case 1—Plain X-ray KUB region
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Fig. 1.17.2: Case 1—Intravenous pyelogram
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Fig. 1.17.3: Case 1—Picture of calculi with pelvis
 
Case 2
A 28-year-old male presented with left flank pain. Plain X-ray KUB region (Fig. 1.17.4) revealed three radiopaque round shadows in the left renal area. Intravenous pyelogram (Fig. 1.17.5) revealed left pelviureteric junction obstruction with hydronephrosis with calculi. Left robotic Anderson-Hynes pyeloplasty with removal of calculi was done. The patient recovered well.52
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Fig. 1.17.4: Case 2—Plain X-ray KUB region
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Fig. 1.17.5: Case 2—Intravenous pyelogram
 
COMMENTS
Concurrent stone extraction and pelviureteric junction obstruction (PUJO) repair can be successful. Operative time is longer than in patients with isolated PUJO repair, but this is to be expected as there is an additional procedure.
 
BIBLIOGRAPHY
  1. Atug F, Castle EP, Burgess SV, et al. Concomitant management of renal calculi and pelvi-ureteric junction obstruction with robotic laparoscopic surgery. BJU Int. 2005;96(9):1365–8.53
 
1.18 PELVIURETERIC JUNCTION OBSTRUCTION WITH MALROTATED KIDNEY
 
INTRODUCTION
Pelviureteric junction (PUJ) obstruction associated with malrotated kidney is very rare. In such cases, dismembered pyeloplasty poses technical difficulties.
 
CLINICAL CASES
 
Case 1
A 28-year-old male presented with vague abdominal pain. Plain X-ray of KUB region revealed no radiopaque shadows. Intravenous urogram (Fig. 1.18.1) revealed bilateral malrotated kidneys with hydronephrosis. Dynamic renal scan revealed bilateral partially obstructed pattern. Bilateral dismembered open pyeloplasty was done.
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Fig. 1.18.1: Case 1—Intravenous urogram
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Case 2
A 35-year-male presented with left flank pain. Plain X-ray of KUB region revealed no radiopaque shadows. Intravenous urogram (Fig. 1.18.2) revealed left malrotated kidney with hydronephrosis with right ectopic pelvic kidney.
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Fig. 1.18.2: Case 2—Intravenous urogram
 
COMMENTS
PUJ obstruction with malrotated kidney is very rare and only few cases are reported. The surgical outcome of pyeloplasty may not be good.
 
BIBLIOGRAPHY
  1. Jayakumar S, Antao BA, Ninan GK. Pyeloplasty for pelvi-ureteric junction obstruction in malrotated kidneys in children. Eur J Pediatr Surg. 2012;22(4):279–82.55
 
1.19 PELVIURETERIC JUNCTION OBSTRUCTION WITH MEGAPELVIS
 
INTRODUCTION
Patients with extrarenal pelvis with pelviureteric junction obstruction can have a large renal pelvis. The obstruction can be anywhere in the ureter and above that pelvis can be dilated.
 
CLINICAL CASES
 
Case 1
A 24-year-old male presented with right flank pain. Plain X-ray KUB region revealed no radiopaque shadow. Intravenous urogram (Fig. 1.19.1) revealed right hydronephrosis with long dilated pelvis with obstruction at the L4 level. Right dismembered pyeloplasty with ureterization of pelvis was done.
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Fig. 1.19.1: Case 1—Intravenous urogram
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Case 2
A 28-year-old male presented with right flank pain. Plain X-ray KUB region revealed no radiopaque shadow. Intravenous urogram (Fig. 1.19.2) revealed right hydronephrosis with long hugely dilated pelvis with obstruction at the L4 level. Right dismembered pyeloplasty with ureterization of pelvis was done.
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Fig. 1.19.2: Case 2—Intravenous urogram
 
COMMENTS
Pelviureteric junction obstruction with large megapelvis is a rare congenital anomaly and can be managed surgically by excision of large pelvis.
 
BIBLIOGRAPHY
  1. Gupta NP, Chahal R, Seth A. “Ureterisation of Megapelvis” in a solitary kidney with a low lying pelviureteric junction obstruction-a point of technique. Ind J Urol. 1996;13(1):49–50.57
 
1.20 PELVIURETERIC JUNCTION OBSTRUCTION WITH SINGLE KIDNEY
 
INTRODUCTION
Pelviureteric junction (PUJ) obstruction is associated with other anomalies. Unilateral agenesis of the kidney can be associated in almost 5% of children with PUJ obstruction.
 
CLINICAL PRESENTATION
A 35-year-old male presented with left flank pain. Abdominal examination was normal. Blood and urine examination was normal. Intravenous urogram (Fig. 1.20.1) revealed single kidney with PUJ obstruction. Cystoscopy revealed right hemitrigone with absence of ureteric orifice. Right retrograde pyelography (RGP) confirmed PUJ obstruction. Left open pyeloplasty was done.
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Fig. 1.20.1: Intravenous urogram
 
COMMENTS
Congenital absence of kidney can be associated with pelviureteric junction obstruction and management is same as in the presence of two kidneys and prognosis is good.
 
BIBLIOGRAPHY
  1. Johnston JH, Evans JP, Glassberg KI, et al. Pelvic hydronephrosis in children: a review of 219 personal cases. J Urol. 1977;117(1):97–101.58
 
1.21 PELVIC ECTOPIC KIDNEY
 
INTRODUCTION
When a fully mature kidney is not in its normal location, it is known as renal ectopia. Pelvic kidney is the most common type of renal ectopia. The ectopic pelvis kidney is opposite the sacrum and below the aortic bifurcation. The contralateral kidney is usually normal. This can be associated with other congenital anomalies, the common ones are genital anomalies. Imaging by intravenous pyelogram, ultrasound and CT scan can diagnose ectopic kidney. The kidney function can be normal. These abnormally positioned kidneys are often clinically asymptomatic. However, they are prone to urinary infection, stone formation and trauma. It can be associated with obstruction and needs surgical correction.
 
CLINICAL PRESENTATION
A 32-year-old male presented with vague abdominal pain.
 
INVESTIGATIONS
Intravenous urogram revealed normal right kidney with ectopic pelvic kidney with mild hydronephrosis (Fig. 1.21.1). Dynamic renal scan revealed normal function and drainage of the ectopic kidney.
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Fig. 1.21.1: Intravenous urogram
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TREATMENT
He was advised conservative treatment.
 
COMMENTS
Pelvic ectopic kidney can be an incidental finding on imaging. If symptomatic, surgery is recommended.
 
BIBLIOGRAPHY
  1. Dretler SP, Olsson CA, Pfister RC. The anatomic, radiologic and clinical characteristics of the pelvic kidney, an analysis of 86 cases. J Urol. 1971;105(5);623–7.60
 
1.22 URETEROCELE
 
INTRODUCTION
A ureterocele is cystic dilatation of the intravesical submucosal ureter. The size may vary from a small bulge of 1–2 cm to a size which can fill complete bladder. This swollen area can block urine flow. Ureterocele occurs in about one in 500 to one in 4,000 people. It is more common in Caucasians. Ureterocele is equally common in left- and right-side ureters.
Most common symptoms are abdominal pain, back pain, possibly only on one side, blood in the urine, burning pain while urinating (dysuria), flank pain, lump (mass) in the abdomen that can be felt, ureterocele tissue falls down (prolapse) through the female urethra and into the vagina, urinary incontinence, urinary tract infection.
During the last 20 years, the surgical approach to ureterocele has evolved from major open surgery to minimally invasive endoscopic puncture. The author believes that the endoscopic approach decreases the need for open surgical procedures.
 
CLINICAL CASES
 
Case 1
A 36-year-old female presented with left flank pain. Plain X-ray (Fig. 1.22.1) revealed a round radiopaque shadow in the pelvis. Intravenous urogram (Fig. 1.22.2) revealed left ureterocele with a calculus with mild hydroureteronephrosis.
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Fig. 1.22.1: Case 1—Plain X-ray pelvis
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Ultrasound (Fig. 1.22.3) revealed left ureterocele with a calculus. Cystopanendoscopy revealed left ureterocele. A smiling incision was made by holmium laser and stone was also fragmented with the laser.
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Fig.1.22.2: Case 1—Intravenous urogram showing ureterocele with calculus
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Fig. 1.22.3: Case 1—Ultrasound showing left ureterocele with calculus
 
Case 2
Another case with similar presentation. Intravenous urogram (Fig. 1.22.4) revealed a large left ureterocele (Cobra head appearance) with hydroureteronephrosis (HDUN). Endoscopic incision was done.62
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Fig. 1.22.4: Case 2—Intravenous urogram showing cobra head appearance of ureterocele
 
Case 3
Another case with left flank pain. Intravenous urogram revealed bilateral double collecting system with left lower moiety ureterocele (Fig. 1.22.5). Common sheath ureteric reimplantation was done.
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Fig. 1.22.5: Case 3—Intravenous urogram showing left ureterocele
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Case 4
A 56-year-old presented with vague abdominal pain. CECT abdomen revealed a mass lesion near left ureteric orifice (Fig. 1.22.6). Cystoscopy revealed left ureterocele (Fig. 1.22.7) which was ballooning with efflux of urine. Endoscopic incision was made.
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Fig. 1.22.6: Case 4—CECT showing a mass lesion at the base of bladder
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Fig. 1.22.7: Case 4—Cystoscopic view showing ureterocele
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Case 5
A 6-year-old male child presented with a single episode of periumbilical, moderate, non-colicky, nonradiating pain associated with vomiting; 6 months ago. He had no associated bowel, bladder symptoms. No similar episodes in past. No history of urinary tract infection (UTI), hematuria/lithuria/poor stream/operative intervention. Urine and blood investigations were normal. Ultrasound (Figs 1.22.8A and B) revealed right double moiety with dilatation of upper moiety ureter with ureterocele. Intravenous urogram (Figs 1.22.9A and B) revealed the same findings. Dynamic renal scan (Figs 1.22.10A and B) revealed poor function in the right upper moiety. CECT scan (Figs 1.22.11A to C) reconfirmed the findings of ultrasound. Excision of ureterocele with common sheath ureteroneocystostomy of both ureters was done. Patient recovered well. Postoperative intravenous urogram (Fig. 1.22.12) revealed improved renal function in right superior moiety with normal ureters.
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Figs 1.22.8A and B: Case 5—Ultrasound
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Figs 1.22.9A and B: Case 5—Intravenous urogram
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Figs 1.22.10A and B: Case 5—Dynamic renal scan
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Figs 1.22.11A to C: Case 5—Contrast enhanced computed tomography (CECT) scan
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Fig. 1.22.12: Case 5—Intravenous urogram
 
COMMENTS
Ureterocele can be an incidental finding on imaging and can occur in single ureter or in the upper moiety of a duplicated ureter. If asymptomatic, does not require any treatment. If associated with ureteric obstruction, mostly can be managed endoscopically or by ureteric reimplantation.
 
BIBLIOGRAPHY
  1. Guay-Woodford LM. Hereditary nephropathies and developmental abnormalities of the urinary tract. In: Goldman L, Schafer AI (Eds). Cecil Medicine, 24th edition. Saunders Elsevier;  Philadelphia,  Pa: 2011: chap 130.
  1. Hagg MJ, Mourachov PV, Snyder HM, et al. The modern endoscopic approach to ureterocele. J Urol. 2000;163(3):940–3.
  1. Peters CA, Schlussel RN, Mendelsohn C. Ectopic ureter, ureterocele, and ureteral anomalies. In: Wein AJ (Ed). Campbell-Walsh Urology, 10th edition. Saunders Elsevier;  Philadelphia,  Pa: 2011: chap 116.69
 
1.23 VESICOURETERAL REFLUX
 
INTRODUCTION
Vesicoureteral reflux (VUR) is the retrograde flow of urine from the bladder into the ureter and towards the kidney due to abnormal functioning of vesicoureteric junction. This junction usually acts like a one-way valve, allowing urine flow from the ureter into the bladder and closing during micturition, preventing back flow. Most review articles suggest a frequency of around 1% in healthy children. It can be primary or secondary to high vesical pressure and obstructive uropathy. It is commonly associated with recurrent urinary tract infection. It is diagnosed by cystography and radionuclide scanning. Reflux can be graded from I to V. For grade I and II, conservative treatment is recommended whereas higher grade can be managed by endoscopic injection or by antireflux surgery.
 
CLINICAL PRESENTATION
A 15-year-old female presented with history of recurrent urinary tract infection (UTI) and frequency of urination. Her creatinine was 3.2 mg/ dL. Ultrasound revealed bilateral hydroureteronephrosis. Micturating cystourethrogram (MCU) revealed small capacity bladder with bilateral grade V bilateral vesicoureteral reflux (Fig. 1.23.1). Initially, she was kept on continuous drainage for improvement of renal function.
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Fig.1.23.1: Micturating cystourethrogram showing bilateral vesicoureteral reflux
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COMMENTS
Vesicoureteral reflux is a common cause for recurrent UTI and can be primary or secondary. Sometimes it can compromise the renal function. It should be managed appropriately to prevent renal damage by medically or surgically accroding the grade of vesicoureteral reflux.
 
BIBLIOGRAPHY
  1. Baker DK, Gomez RA. Embryonic development of the ureter and bladder: acquisition of smooth muscle. J Urol. 1998;160:545–50.
  1. Eccles MR, Jacobs GH. The genetics of primary vesico-ureteric reflux. Ann Acad Med Singapore. 2000;29:337–45.
  1. Lama G, Russo M, De Rosa E, et al. Primary vesicoureteric reflux and renal damage in the first year of life. Pediatr Nephrol. 2000;15:205–10.
  1. Mak RH, Kuo HJ. Primary ureteral reflux: emerging insights from molecular and genetic studies. Curr Opin Pediatr. 2003;15:181–5.
  1. Stephens F, Lenaghan D. Anatomical basis and dynamics of vesicoureteric reflux. J Urol. 1962;87:669–80.
  1. Tanagho EA, Guthrie TH, Lyon RP. The intravesical ureter in primary reflux. J Urol. 1969;101:824–32.
  1. Vermillion CD, Heale WF. Position and configuration of the ureteral orifice and its relationship to renal scarring in adults. J Urol. 1973;109:579–84.