URETERAL ANOMALIES

Pediatric GU Review

UCSD Pediatric UrologyGeorge Chiang MD

Sara Marietti MD

Outlined from The Kelalis-King-Belman Textbook of Clinical Pediatric Urology 2007

(not for reproduction, distribution, or sale without consent)

Sphincteric

Sphincterostenotic

Cecoureterocele

Intravesicle vs Ectopic

URETEROCELES Demographics

• 4:1 female to male ratio

• occur almost exclusively in Caucasians

URETEROCELES Diagnosis

• Before the advent of prenatal U/S, most diagnosed clinically

• most common presentation of UTI or urosepsis

• palpable abdominal mass• ureterocele may prolapse out of

urethra

URETEROCELES Diagnosis

• With advent of prenatal U/S, most now dx with this modality.

• Still diagnosed clinically with UTI/urosepsis (Retik and Peters 1992)

• Earlier detection of ureteroceles associated with duplex systems led to a treatment dilemmas.

URETEROCELES Treatment

• Treatment should be individualized• Goals of treatment should be:

– preservation of renal function– elimination of infection, obstruction and

reflux– maintenance of urinary continence

• Surgical morbidity should be minimized.

URETEROCELES Treatment

• Much controversy surrounds the optimal treatment of ureteroceles

• Little is known about the natural history of asymptomatic ureteroceles diagnosed prenatally.

• Shankar and Rickwood (2001) suggest that patients with favorable characteristics can be followed successfully on prophylaxis alone.

URETEROCELESNon-operative managment

• Of 52 children with duplex system ureteroceles, 38 treated with surgery

• 14 with favorable characteristics have been followed successfully on Abx alone

• upper pole less than 10% renal fct• unobstructed lower pole• lower pole VUR not greater than grade 3• unobstructed bladder outflow

URETEROCELES Non-operative management

• Median follow up 8 years• more studies are needed to examine the

natural history of these asymptomatic, prenatally diagnosed patients

• suggests that natural history in select patients is benign

Shankar KR, Vishwanath N, Rickwood AMK, J Urol 165, 1226-8, Apr 2001

Ureteroceles : Endoscopic Managment

• When treatment is required, the recent trend has been towards less invasive procedures.

• Endoscopic incision has been advocated by many.

• Historically, it fell out of favor because of massive reflux of the involved system after incision

Ureteroceles : Endoscopic Managment

• Then advocated only with urgent need for decompression with urosepsis

• However, with newer technology, regained momentum as a treatment modality

• Theoretically, obstruction is relieved and the roof of the incised ureterocele should collapse, preventing reflux in a flap-valve manner.

Endoscopic managment

• However, oftentimes, newly created reflux does occur.

• It also appears that endoscopic treatment has very different results between intravesical and extravesical ureteroceles.

Intravesical Ureterocele

Endoscopic Management : Intravesical ureterocele

• Endoscopic incision of an intravesical ureter appears to be efficacious

• Blyth et al (1993) found that 93% of intravesical ureteroceles decompressed with endoscopic incision

• also, upper pole function was preserved in 96%, only 18% had new reflux, and only 7% had need for secondary procedures

Extravesical Ureterocele

Endoscopic management : Extravesical Ureterocele

• Initial management of extravesical ureterocele with incision remains controversial

• some advocate its use for various reasons• Cooper et al (2000) state that although a

high percentage required a second procedure (64%), a decompressed ureterocele facilitates reimplantation

Extravesical ureterocele

• Also at least a third of their patients receive definitive treatment

• decompression reduces risk of pyelonephritis

• decompression may salvage renal function

• decompression allows for a delay in definitive surgery if required

Extravesical ureterocele

• Also, should patients require additional surgery, they will generally only require a single incision, open bladder level procedure

Cooper CS, Passerini-Glazel G, Hutcheson JC, Iafrate M, Camuffo C, Milani C, Snyder HM. JUrol 164, 1097-1100, Sept 2000

Extravesical Ureterocele

• Others do not recommend endoscopic incision as a suitable initial treatment

• Shekarriz et al (1999) state that reoperation rate after incision is approximately 70%

• in their series, 100% required further surgery

Upper Tract Approach

• proponents believe that optimal treatment begins with the upper tract approach

• minimal contribution of upper pole unit

• upper pole nephrectomy and partial ureterectomy

• ureteropyelostomy

Upper Tract Approach

• theoretically, ureterocele should decompress

• subsequently, with trigone returned to more normal morphologic configuration, lower pole reflux should resolve

• also, theoretically able to avoid a second surgical procedure

Upper Tract Approach

• avoiding potentially morbid and difficult bladder level procedure at a young age attractive

• if second procedure required, buys time until child is older

Upper Tract Approach

• Mandell et al (1980) advocated this approach

• 18 patients were treated ; only 3 required a second surgical procedure

• however, if pt has high grade reflux into ipsilateral lower pole ureter, pt should undergo complete reconstruction

Mandell J, Colodny A, Lebowitz RL, J.Urol 1980;123:921

Upper Tract Approach

• Data suggests that this approach quite effective in select cases e.g those without high grade reflux

• if reflux is present, different procedure may be warranted

Complete Reconstruction• Hendren and Mitchell (1979) recommended

complete repair except in acutely ill child with urosepsis

• complete repair includes upper pole nephrectomy, ureterocele excision, and ureteral reimplantation

• others have continued to recommend this approach Hendren WH, Mitchell ME. Surgical correction of ureterocele. J.Urol. 1979:121:590

Complete Reconstruction

• Overall, they report a 100% success rate in 18 children averaging 1 year of age

• others report a success rate ranging from 86% -88%

• Decter et al (2001) also advocate complete reconstruction at an early age

• they report an 81% success rate and feel total reconstruction provides reliable and safe correction, even in infants

Complete Reconstruction

• Proponents cite high success rate and safety in even young children

• Opponents loath to subject infants to potentially morbid procedure ; would rather “buy time” or cure with alternative, less morbid approach

Megaureter

• Classification

King LR: Megaloureter: Definition, diagnosis and management. J Urol 1980, 123, 222.

Megaureter

• Non-refluxing non-obstructed megaureter– Fetal urine production is 4-6x greater before than after

delivery=high outflow– Increased compliance of fetal ureter– Circular muscular pattern of fetal ureterdouble muscular

layer of full term infant

A Megaureter with normal muscle cells. B, Megaureter containing separated muscle cells and ruptured nexus. C, Megaureter containing excessive collagen and ground substance with dying muscle cells.

Megaureter

• Demographics– 185 neonates with obstructive uropathy VUJ

obstruction in 23%– 4 x more common in boys– Left ureter is 1.6-4.5x greater than right

• Treatment:– Symptoms– Renal Function– Drainage

Megaureter

• Surgical Options– Conservative observation

• Depends on degree of hydronephrosis (Grades 1 to 3 12.9, 23.9 and 34.6 months)

• Ureteral diameter • Laterality• Gender

McLellan DL, Retik A., Peters C., Bauer S., Atala A., Mandell J. Rate and predictors of

spontaneous resolution of prenatally diagnosed primary nonrefluxing megaureter. J Urol. 2002 Nov;168(5):2177-80

Megaureter

• Surgical Options– Reconstruction vs. Removal– Depends on initial function, bilaterality,

reflux, drainage curve and symptoms

Megaureter

• Surgical Options– Intravesical – Extravesical – Laparoscopic

Megaureter

• 38 males and 15 females with 65 megaureters (POM 31 and Refluxing 34)

• Mean patient age 4.9 years with followup 3.5 years– 36 intravesical units, 29 extravesical units

Defoor W et al. Results of tapered ureteral reimplantation for primary megaureter: Extravesical vs. intravesical approach. J Urol. 172, pp 1640-1643 Oct 2004

Megaureter

• Patients with voiding dysfunction or pre-operative reflux may benefit from an intravesical approach

Defoor W et al. Results of tapered ureteral reimplantation for primary megaureter: Extravesical vs. intravesical approach. J Urol. 172, pp 1640-1643 Oct 2004

Megaureter

• Surgical Options– Folding vs. Plication vs. Tapering

Ehrlich RM: The ureteral folding technique for megaureter surgery. J Urol 134:668, 1985

94.6% success 74 megaureters

Stent for 4-5 days

Megaureter

• Surgical Options– Folding vs. Plication vs. Tapering

Starr A: Ureteral plication. A new concept in ureteral tailoring for megaureter. Invest Urol 17:153, 1979

Reduces lumen 50-60%

Severely tortuous ureter

Megaureter

• Surgical Options– Folding vs. Plication vs. Tapering

Hendren WH: Complications of megaureter repair in children. J Urol 113:238, 1975

Lower versus total ureteral reconstruction

41% re-operation

UPJ

• Restricted flow of urine from renal pelvis to ureter– M>F (2:1 in newborns)– L>R– 10-40% bilateral presentation

• Asynchronous or synchronous• Infants < 6 months• May be heritable

• Etiology-Intrinsic– Aperistaltic segment

• Most common, alteration of collagen and composition between muscle cells

– Persistent fetal convolutions• Common in upper ureter after 4th months, differential

ureteral growth

– Valvular mucosal folds– Upper ureteral polyp

• 0.5% of all UPJ

• Etiology-Extrinsic– 15-52% – Majority in adults– More commonly artery

Clinical Presentations

• Infants– Asymptomatic, FTT, antenatal screening

• Children– UTI (30%), pain, N/V, hematuria after

trauma, HTN

• Both: stones, sepsis, abdominal mass

Diagnostics

• U/S-->AP diameter may correlate with significant obstruction

• Cannot diagnose obstruction

• Sequential studies may be meaningful

Diagnostics

• Diuretic Renography– T 1/2 >20 min is classically obstructed– 10-20 min indeterminate– Variability

• State of hydration/urine output• Functional status of kidney• Renal responsiveness to diuretic• Collecting system capacity/compliance• Bladder fullness• Operator dependency (area of interest and diuretic

timing)

Diagnostics

• IVP (poor handling by infants)• MRU-Renal transit time but expensive

Associated GU Anomalies

• Contralateral UPJ 10-40%• Renal dysplasia• Unilateral renal agenesis 5%• Duplex system• Horseshoe kidney• Ectopic kidney• Congenital syndromes (21% VATER)• VUR 13-42% (secondary UPJ 10%)

When to Intervene

• Symptomatic (UTIs, stones, pain)• Significant hydronephrosis

– Grade 1-2 U/S in 3 months (<3% risk obstruction)

– Grade 3-4 Mag 3

• Tx: Open/Lap/RA pyeloplasty, endopyelotomy

Postnatal Eval

• Ultrasound at 2-4 weeks (unless bilateral/male)

• VCUG at 4-6 weeks

• Mag-3 if no reflux and Grade 3-4 hydro

• Labs-Cr If bilateral hydro

Ectopic Ureter

Ectopic Ureter

• In females, insertion can be distal to external sphincter– 1/3 in bladder but below trigone (refluxing)– 1/3 open at the level of the bladder neck or slightly

more distal in the upper urethra (obstructed)– 1/3 open in area of vagina vestibule or proximal

vagina (incontinence)

• In males– 1/2 in the posterior urethra above sphincter– 1/3 in seminal vesiclae– Other locations: bladder neck, epididymis, vas

Epidemiology

• 1 in 1900

• Male to female 1:5-6

• Ectopic duplication is associated with females and single systems with males

• Bilateral 10%

Associated Anomalies

• Duplication (80%); contralateral system is duplicated 80%

• Ipsilateral renal hypoplasia or dysplasia

• Obstruction or VUR

• Abnormalities of the bladder neck usually associated with bilateral ureteral ectopia

Symptoms

• Males: flank pain, UTI, urinary retention (elevation of bladder neck by ectopic system), epididymitis, prostatitis, infected seminal vesicle cyst

• Females: incontinence, UTI, hematuria, prenatal hydro

Diagnosis

• Physical exam with girls

• US-->dilated ectopic ureter

• VCUG-->refluc into ectopic system

• IVP: may or may not visualize; or absence of upper pole calyx

• CT/MRI

• Cysto/vaginoscopy

Treatment

• Poor renal function-->Nephro-U

• Good renal function-->reimplantation

• Upper to lower pole U-U

QUESTIONS

In a boy with a large ureterocele in a duplex system, the most compelling reason for combining bladder reconstruction with partial nephrectomy is:

a) Ipsilateral Grade 3 Reflux

b) Contralateral Grade 3 Reflux

c) Contralateral hydronephrosis

d) Bladder outlet obstruction

e) Sepsis

A 14 year old girl has primary amenorrhea. She is in the 25th percentile for height and has a webbed neck. Karyotype is 45 XO. The most likely GU abnormaility is:

a) Renal agenesis

b) Horseshoe kidney

c) VUR

d) UPJ obstruction

e) Vaginal agenesis

A 4 month-old girl has APN. She remains febrile after 4 days of IV antibiotics. An ultrasound and VCUG are shown. The next step is:

a) Bladder biopsy

b) Partial nephrectomy

c) Endoscopic incision

d) Ureteroneocystostomy

e) vesicostomy

An 8 month old child is hospitalized for pyelonephritis has an oral temp of 39C. The child weighs 8 kg. The appropriate acetaminophen dosage to manage the fever is:

a) 40 mg

b) 80 mg

c) 120 mg

d) 160 mg

e) 200 mg

A 9 year old boy is in an MVA. He has right flank pain and has voided spontaneously. Physical exam reveals a right flank contusion. Chest X ray reveals a fracture of the 12th rib. The next step is:

a) Ultrasound

b) CT scan

c) Observation

d) IVP

e) Renal scan

A seven year old girl has a UTI. A longitudinal renal ultrasound is shown in the exhibit. The diagnosis is:

a) UPJ obstruction

b) Duplication with upper pole hydro

c) Multicystic renal dysplasia

d) Acute lobar nephronia

e) Polycystic renal disease

In a duplicated urinary system, obstructive hydronephrosis in the lower pole system is usually due to:

a) Orthotopic ureterocele

b) Ectopic ureter

c) UPJ obstruction

d) Ectopic ureterocele

e) UVJ obstruction

A premature infant requiring long term IV alimentation and antibiotics develops a left flank mass and hematuria. U/S reveals an echogenic mass within a hydronephrotic kidney. Renal scan shows decreased blood flow and function in the left kidney. Blood and urine are positive for Candida albicans. In addition to systemic antifungal therapy, the next step is:

a) Nephrectomy

b) Cutaneous loop ureterostomy

c) PCN and antifungal irrigation

d) Ureteral stent

e) Ureteral stent and ESWL

A 2 year old girl has a febrile UTI with fever spikes continuing despite 3 days of IV antibiotics. The next step is:

a) VCUG

b) MAG-3

c) DMSA

d) Renal ultrasound

e) Abdominal CT scan

A 9 year old boy has gross, painless hematuria. Urinalysis is normal except for numerous red cells without casts. Urine culture and sonogram are negative. A percutaneous renal biopsy shows segmental glomerulonephritis with immunoglobulin deposition in the mesangial areas. The diagnosis is:

a) Alport’s syndrome

b) Henoch-Schonlein purpura

c) Membranous glomerulonephritis

d) IgA nephropathy

e) Hemolytic uremic syndrome

An 8 year old 25 kg boy with APN has vomitting and diarrhea for 3 days. Serum electrolytes are: Na 150, K 3.0, Cl 117, HC03 25. The most appropriate IV therapy is:

a) 0.25 NS with 40 mEq/l of KCl at 100 cc/hr

b) D5W with 40 mEg/l of KCl at 200 cc/hr

c) NS with 40 mEg/L of KCL at 100 cc/hr

d) NS with 40 mEq/l of KCl at 75 cc/hr

e) D5W with 10 mEq/l of KCl at 200 cc/hr

A 6 year old uncircumcised boy has an acute onset of gross hematuria, dysuria, and urinary frequency. There is no fever or flank pain. The urine is sterile and ultrasound shows normal kidneys with diffuse thickening of the bladder wall. The most appropriate management is:

a) Observation

b) Circumcision

c) Acyclovir

d) Trimethoprim-sulfa

e) Bladder biopsy

A 4 year old boy undergoes a left dismembered pyeloplasty with placement of an extraperitoneal drain. By POD#3, there is minimal output from the drain. The urethral catheter is removed. The next day, output from the drain is 400 cc. The next step is:

a) Advance drain slowly

b) Replace urethral catheter

c) PCN

d) Internal ureteral stent

e) Open repair of anastomotic leak

Ectopic thoracic kidney is most commonly associated with:

a) Anomalous vasculature

b) Contralateral UPJ obstruction

c) VUR

d) No intrinsic pathology

e) Duplex drainage system

A 3 year old child with a duplicated collecting system has reflux to the upper renal segment upon voiding. The most appropriate statement is:

a) This type of anomaly is commonly bilateral

b) The ureter to the upper segment is probably ectopic

c) The ureter to the lower segment is probably ectopic

d) The ureter to the lower segment is probably obstructed

e) Reflux to the upper system occurs frequently with complete ureteral duplication

An infant girl has severe upper pole hydronephrosis with associated hydroureter. The renal parenchyma is thinned on ultrasound and the VCUG is normal. Urine can be seen dripping from an ectopic orifice adjacent to the urethral meatus. In this child, the finding most predictive of poor function of the upper pole renal segment is:

a) Parenchymal thickness

b) Degree of hydroureter

c) Degree of hydronephrosis

d) Age

e) Location of the orifice