American Academy of Pediatrics Task Force on Circumcision.

TABLE OF CONTENTS page

1. Phimosis 5

1.1 Background 5

1.2 Diagnosis 5

1.3 Treatment 5

1.4 References 5

2. Cryptorchidism 7

2.1 Background 7

2.2 Diagnosis 7

2.3 Treatment 7

2.4 References 8

3. Hypospadias 11

3.1 Background 11

3.2 Diagnosis 11

3.3 Treatment 11
3.3.1 Complications 12

3.4 References 13

4. Incontinence 15

4.1 Background 15

4.2 Classification 15

 

4.2.1 Enuresis 15

4.2.2 Urinary incontinence 15

 

4.3 Diagnosis 16

4.4 Treatment 16

 

4.4.1 Nocturnal enuresis 16

4.4.2 Diurnal enuresis 16

4.4.3 Urinary incontinence 16
4.4 References 17

5. Dilatation of the upper urinary tract 19

5.1 Background 19

5.2 Diagnosis 19

 

5.2.1 Ultrasound examination 19

5.2.2 Voiding cystourethrography 19

5.2.3 Diuresis renography 19

5.2.4 Static renal scintigraphy 19

5.2.5 Intravenous urogram 19

5.2.6 Whitaker's test 19

5.3 Treatment 20

5.3.1 UPJ-stenosis 20

5.3.2 Megaureter 20

5.3.3 Ureterocele 20

5.3.4 Retrocaval ureter 21

5.3.5 Bilateral hydronephrosis 21

5.4 References 22

6. Urinary tract infection (UTI) 24

6.1 Classification 24

6.2 Diagnosis 24

6.3 Treatment 24

 

6.3.1 Asymptomatic bacteriuria 24

6.3.2 Acute UTI without pyelonephritis 25

6.3.3 Pyelonephritis 25

6.3.4 Complicated UTI 25

6.3.5 Antibiotic prophylaxis 25

6.4 References 25

7. Reflux 27

7.1 Background 27
7.1.1 Secondary reflux 27

7.2 Classification 27

7.3 Diagnosis 27
7.3.1 Secondary reflux 28

7.4 Treatment 29

 

7.4.1 Conservative therapy 29

7.4.2 Surgical therapy 29

7.4.3 Endoscopic therapy 29

7.4.4 Open surgery 29

7.4.5 Follow-up 30

7.5 References 30

8. Paediatric nephrolithiasis 33

8.1 Background 33

8.2 Diagnosis 33

8.3 Treatment 33

 

8.3.1 Conservative treatment 34

8.3.2 Metaphylaxis of paediatric nephrolithiasis 34

8.4 References 34

9. Obstructive pathology of renal duplications:

ureterocele and ectopic ureter 37

9.1 Background 37

9.1.1 Ureterocele 37

9.1.2 Ectopic ureter 37

9.2 Classification 37

9.2.1 Ectopic ureterocele 37

9.2.2 Orthotopic ureterocele 37

9.2.3 Caecoureterocele 37

9.3 Diagnosis 38

9.3.1 Ureterocele 38

9.3.2 Ectopic ureter 38

9.4 Treatment 38

9.4.1 Ureterocele 38

9.4.2 Ectopic ureter 39

9.5 References 39

10. Intersex (abnormal sexual differentiation) 41

10.1 Background 41

10.2 Classification 42

 

10.2.1 Female pseudohermaphrodite 43

10.2.2 Male pseudohermaphrodite 43

10.2.3 True hermaphrodite 43

10.2.4 Mixed gonadal dysgenesis 43

10.2.5 Pure gonadal dysgenesis 43

10.3 Diagnosis 43

10.3.1 The neonatal emergency 43

10.3.2 Late diagnosis and management 44

10.4 Treatment 45

10.4.1 Genitoplasty 45

10.4.2 Indications for the removal of gonads 45

10.5 References 45

11. Urodynamics 47

11.1 Standardization of investigations 47

11.1.1 Residual urine 47

11.1.2 Uroflowmetry 47

11.1.3 Cystometry 47

11.1.4 Video-urodynamic evaluation 47

11.1.5 Urethral pressure profile (sphincterometry) 48

11.1.6 Electromyography of the external sphincter 48
11.2 References 48

12. Abbreviations used in the text 50

PHIMOSIS

BACKGROUND

At the end of the first year of life, retraction of the foreskin behind the glandular sulcus is possible in only about 50% of boys; this rises to approximately 89% by the age of three. The incidence of phimosis is 8% in 6- to 7-year-olds and just 1 % in males aged 16 to 18 years.

Paraphimosis must be regarded as an emergency situation: retraction of a too narrow prepuce behind the glans penis into the glandular sulcus may interfere with perfusion of the preputial surface distally from the constrictive ring and possibly of the glans penis with the risk of consecutive necrosis.

DIAGNOSIS

If the prepuce is not or only partly retractable, or shows a constrictive ring on drawing back over the glans penis, a disproportion between the width of the foreskin and the diameter of the glans penis has to be assumed. In addition to the constricted foreskin, there may be adhesions between the inner surface of the pre­puce and the glandular epithelium and/or a frenulum breve. A frenulum breve leads to a ventral deviation of the glans once the foreskin is retracted.

The diagnosis of paraphimosis is made by physical examination.

TREATMENT

Treatment of phimosis in children is dependent on parents' preferences and can be plastic or radical circumcision after completion of the second year of life. In case of complicating findings, such as recurrent urinary tract infections (UTIs) or ballooning of the foreskin during micturition, circumcision should be performed without delay regardless of the patient's age. Plastic circumcision has the objective of achieving a widening of the foreskin circumference with full retractability, while the foreskin is preserved cosmetically. In the same session, adhesions are released and an associated frenulum breve is corrected by frenulotomy with ligation of the frenular artery. Routine neonatal circumcision to prevent penile carcinoma is not indicated. Contra­indications of the operation are acute local infection and congenital anomalies of the penis, particularly hypospadias, as the foreskin may be required for plastic correction.

As a conservative treatment option, a corticoid ointment (0.05-0.1 %) can be administered twice a day over a period of 20-30 days. This treatment is not indicated in babies and children still wearing diapers, but may be considered around the third year of life.

Treatment of paraphimosis consists of manual compression of the oedomatous tissue with the sub­sequent attempt to retract the tightened foreskin over the glans penis. If this manoeuvre fails, a dorsal incision of the constrictive ring is required. Depending on the local findings, a circumcision is carried out immediately or can be performed in a second session.

REFERENCES

Cansever G.

Psychological effects of circumcision. Brit J Med Psychol 1965; 38: 321-331.

Goepel M, P Rathert.

Leitlinie zur Phimose. Urologe A, 1998; 37: 664-665.

Maxwell LG, Vaster M.

Analgesia for neonatal circumcision: no more studies, just do it [editorial comment]. Arch Pediatr Adolesc Med 1999; 153: 444-445.

Preston EN.

Whither the foreskin? JAMA 1970; 213: 1853-1858.

Schoen EJ.

The status of circumcision of newborns. New Engl J Med 1990; 322: 1308-1312.

Schoen EJ.

Advantages and disadvantages of neonatal circumcision [letter]. JAMA 1997; 278: 201.

Van Howe RS.

Cost-effective treatment of phimosis. Pediatrics 1998; 102: E43.

Wallerstein E.

Circumcision. The uniquely American medical enigma. Urol Clin North Am 1985; 12: 123-132.

Williams N, Kapila L.

Complications of circumcision. Br J Surg 1993; 80: 1231-1236.

Wiswell ТЕ, Geschke DW.

Risks from circumcision during the first month of life compared with those for uncircumcised boys. Pediatrics 1989; 83: 1011-1015.

21. Wiswell ТЕ, Tencer HL, Welch CA, Chamberlain JL

Circumcision in children beyond the neonatal period. Pediatrics 1993; 92: 791-793.

CRYPTORCHIDISM

BACKGROUND

The incidence of maldescensus testis after the first year of life is 1.8-2%. A distinction is made between abdominal, inguinal or prescrotal testis retention and epifascial, femoral or penodorsal testis ectopy. Sliding and pendulous (retractile) testes are particular variations of cryptorchidism. Sliding testis with a too short spermatic cord relocates into its non-physiological position when pulled into the scrotum and then released. Pendulous (retractile) testis with hypertrophic cremaster muscle fibres is associated with an intermittent retraction of the usually orthotopic testis.

DIAGNOSIS

Maldescensus testis is diagnosed by clinical examination and sonography. Sonography and magnetic resonance imaging (MRI) may help in localizing the impalpable testis; the accuracy of the latter is 90% for intra-abdominal testis. Once abdominal retention is suspected, laparoscopy has been established as a diagnostic and therapeutic procedure. In this procedure, the testis can be localized in its abdominal position and placed scrotally using the technique appropriate to the anatomical conditions. A human chorionic gonadotrophin (HCG) stimulation test, as evidence of testosterone-producing testis tissue, should precede operative exploration for bilaterally impalpable testes.

TREATMENT

The objective of treatment is to achieve an orthotopic scrotal position of the testis, before the child's second birthday, in order to prevent irreversible damage of spermatogenesis in the affected testis. Hormone therapy (optional) is applied for testis retention only. It is ineffective for ectopy, but can be helpful for preparation of local tissue: HCG as an intramuscular injection (9000-30,000 III in different protocols) or luteinizing hormone releasing hormone (LHRH) as a nasal spray (400 ug, three times daily). Both methods are effective in about 20-30% of cases. Follow-up is important because the benefits may fail after a period of time.

Surgical orchidofuniculolysis and orchidopexy are first-line treatment options. Pendulous (retractile) testes are not indicated for surgical repair. Absolute indications for a primary surgical approach are testis retention after failed hormone therapy or after previous inguinal surgery, testis ectopy and all maldescended testes with associated pathology (hernia and/or open processus vaginalis). Inguinal access of the spermatic cord is gained after opening the inguinal canal. Associated pathological conditions (open processus vaginalis, inguinal hernia) are dealt with in the same session. After the spermatic chord and testis have been freed of con­nective tissue and cremaster fibres have been resected, the testis is relocated tension free by pexis in the scrotum. If no testis or spermatic funicle tissue can be found during exploration of the inguinal canal, opening of the peritoneum and intraperitoneal orchidofuniculolysis is performed. If the spermatic funicle is too short, the Fowler-Stephens technique (ligation and dissection of the spermatic vessels) can be applied. Pre-conditions are intact deferent duct and epididymis vessels; these are tested by a temporary clamping of the testicular artery. In rare cases, auto-transplantation by microsurgical anastomosis of the testis vessels with the epigastric vasculature can be considered.

Table 1: Management of cryptorchidism

CRYPTORCHIDISM

Physical examination sonography

Detectable

Unilateral undetectable

Bilateral undetectable

 

MRI (optional)

HCG stimulation

 

v Therapy

Laparoscopy

Intersex?

REFERENCES

Alaish SM, Stylianos S.

Diagnostic laparoscoy. Curr Opin Pediatr 1998; 10: 323-327

Balaji КС, Diamond DA.

Laparoscopic diagnosis and management of transverse testicular ectopia. Urology 1995; 46: 879-880.

Caldamone AA, Amaral JF.

Laparoscopic stage 2 Fowler-Stephens orchiopexy. J Urol 1994; 152: 1253-1256.

8. Canavese F, Cortes MG, Magro P, Lonati L, Teruzzi E, de Sanctis C, Lala R.
Cryptorchidism: medical and surgical treatment in the 1st year of life. Pediatr Surg Int 1998; 14: 2-5.

Cortes D.

Cryptorchidism — aspects of pathogenesis, histology and treatment. Scand J Urol Nephrol Suppl 1998; 196: 1-54.

Davenport M.

ABC of general paediatric surgery. Inguinal hernia, hydrocele, and the undescended testis. BMJ 1996; 312: 564-567.

12. Davenport M, Brain C, Vandenberg C, Zappala S, Duffy P, Ransley PG, Grant D.
The use of the hCG stimulation test in the endocrine evaluation of cryptorchidism.
BrJ Urol 1995; 76: 790-794.

DocimoSG.

The results of surgical therapy for cryptorchidism: a literature review and analysis. J Urol 1995; 154: 1148-1152.

DuckettJW.

Pediatric laparoscopy: prudence, please [editorial]. J Urol 1994; 151: 742-743.

Esposito C, Garipoli V.

The value of 2-step laparoscopic Fowler-Stephens orchiopexy for intra-abdominal testes [see comments]. J Urol 1997; 158: 1952-1954.

17. Fahlenkamp D, Winfield HN, Schonberger B, Mueller W, Loening SA.
Role of laparoscopic surgery in pediatric urology. Eur Urol 1997; 32: 75-84.

Fedder J, Boesen M.

Effect of a combined GnRH/hCG therapy in boys with undescended testicles: evaluated in relation to testicular localization within the first week after birth. Arch Androl 1998; 40: 181-186.

Gill B, Kogan S.

Cryptorchidism. Current concepts. PediatrClin North Am 1997; 44: 1211-1227.

Gordon N.

Undescended testes: screening and early operation. Br J Clin Pract 1995; 49: 318-320.

22. Hadziselimovic F.

Cryptorchidism: the disease and its management. Acta Urol Belg 1995; 63: 83-88.

Hadziselimovic F, Herzog B.

Treatment with a luteinizing hormone-releasing hormone analogue after successful orchiopexy markedly improves the chance of fertility later in life. J Urol 1997; 158: 1193-1195.

Hamm B.

Sonography of the testis and epididymis. Andrologia 1994; 26: 193-210.

Kiely EA.

Scientific basis of testicular descent and management implications for cryptorchidism. Br J Clin Pract 1994; 48: 37-41.

King LR.

Orchiopexy for impalpable testis: high spermatic vessel division is a safe maneuver. J Urol 1998; 160: 2457-2460.

Maddern GJ, Sutherland PD.

Laparoscopic exploration for a presumed intra-abdominal testicle. Endosc Surg Allied Technol 1994; 2: 293.

30. McAleer IM, Packer MG, Kaplan GW, Scherz HC, Krous HF, Billman GF.
Fertility index analysis in cryptorchidism. J Urol 1995; 153: 1255-1258.

Puri P, Nixon HH.

Bilateral retractile testes subsequent effects on fertility. J Pediatr Surg 1977; 12: 563.

Ritchey ML, Bloom D.

Summary of the urology section. American Academy of Pediatrics. Pediatrics 1995; 96: 138-143.

Rubben H, Goepel M.

Leitlinie zum Kryptorchismus. Urologe A 1998; 37: 666-667.

HYPOSPADIAS

BACKGROUND

Depending on the localization of the external urethral orifice, hypospadias can be subdivided into distal (75%; glandular, coronary, subcoronary), intermediate (13%) and proximal (12%; penoscrotal, scrotal, perineal) forms. Differentiation between functionally necessary and aesthetically feasible operative procedures is important for therapeutic decision-making. As all surgical procedures carry the risk of complications, thorough pre-operative counselling of the parents is crucial.

DIAGNOSIS

Apart from the description of the local findings (position, shape and width of the orifice, size of the penis, information on the curvature of the penis on erection and inflammation), the diagnostic evaluation includes assessment of associated anomalies:

• Open processus vaginalis (in 9% of cases)

• Testis in a high position (in 5% of mild forms of hypospadias; in 31 % of posterior hypospadias)

• Anomalies of the upper urinary tract (3%)

Severe hypospadias with unilaterally or bilaterally impalpable testis and scrotal transposition require a complete genetic work up. In case of ambiguous genitalia, retrograde genitography should be performed soon after birth to exclude an adrenogenital syndrome (AGS).

A thorough physical examination, urinalysis and usually sonography are performed routinely in all forms of hypospadias. Excretory urogram or voiding cystourethrography (VCUG) are required only when the findings in the investigations mentioned above are inconclusive. Urine trickling and ballooning of the urethra requires exclusion of meatal stenosis by careful inspection.

TREATMENT

Surgical intervention is recommended for intermediate and more severe forms of hypospadias, and for distal forms with associated pathology (penile curvature, meatal stenosis and phimosis). In simple distal hypospadias, cosmetic correction should be performed only after a thorough discussion of the psychological aspects and clarification of the lack of a functional indication.

The therapeutic objective is to correct the penile curvature, to form a neo-urethra and to bring the neo-meatus to the tip of the glans penis, if possible. The use of magnifying spectacles and special suture materials, knowledge of a variety of plastic surgical techniques (use of rotational skin flaps, as well as free tissue transfer), the handling of dermatomes, wound care and post-operative treatment are essential for a satisfactory outcome.

Pre-operative treatment with the local application of testosterone propionate over a period of 4 weeks can be helpful. For distal forms of hypospadias a range of techniques are available (e.g. Mathieu, MAGPI, King, Duplay, Snodgrass, Onlay). Along with the 'skin' chorda, the connective tissue of the genuine chorda and the distal corpus spongiosum, running longitudinally under the glans on both sides of the urethral channel, are usually responsible for the curvature. If there is a residual curvature after chordectomy, and if the remaining skin channel of the open urethra is thin and of poor circulation, incision or excision of the urethral plate may be required. In corporeal dysproportion, orthoplasty (modification of Nesbit dorsal corporeal plication) must be added. Orthoplasty (Nesbit, modified Nesbit, Schroder-Essed) and closure may be considered in a two-stage procedure.

The Onlay technique with preservation of the urethral plate and avoidance of circumferential anastomosis is the method of choice, with low complication rates for moderate and severe hypospadias. Prerequisite is an intact and well-vascularized urethral plate, or a satisfactory result after the first session with a straight penis and a well-covered shaft. If the urethral plate is not completely preserved (after excision or division), a tube-onlay flap or an inlay-onlay flap are used. The two-stage procedure may be an option in severe hypospadias. If preputial or penile skin is not available, buccal mucosa, bladder mucosa and free skin grafts can be used.

Only fine absorbable suture materials should be used (6/0-7/0). For blood coagulation, bipolar instruments are required along with swabs soaked in 1:10,000 epinephrine solution. Glans preparation may be facilitated by infiltration with a 1:100,000 epinephrine solution. Tourniquets should not be used for longer than 20 minutes.

After preparation of the dorsal neurovascular bundle, modified Nesbit sutures (monofilic non-resorbable suture material 4/0-5/0, e.g. Goretex, Prolene) are placed with the knots folded in. Urine is drained via a transurethral or suprapubic catheter. In case of a suprapubic catheter, the neo-urethra should be stented. For urethral stenting and drainage, an 810 F catheter with multiple side holes is used ending in the bulbar urethra (not into the bladder). Circular dressings with slight compression, as well as an antibiotic administration have become established procedures.

Complications

Whereas meatal narrowing after splint removal can be corrected by careful stretching and the Dittel device, operative revision is needed in cases of a scarred meatus as slitting and stretching are ineffective in the long term. In urethral stricture, open surgery should be performed after one attempt at internal urethrotomy. With fistulae, revision should not be planned before a lapse of 6 months. Urethral stricture should be excluded intra-operatively as a cause of the fistula. To prevent the recurrent fistula formation, a dartos flap or a free tunica vaginalis patch may be used. Attention should be paid to adequate subsequent cover with mobilized Scarpa's fasciae.

Correction of a minor residual curvature, sometimes reported by the parents, should be discouraged, as it has no functional relevance. It can be easily corrected after puberty, if significant.

Proximal

 

Chordee

No Chordee

 

Urethral plate cut

Urethral plate preserved

 

MAGPI, Mathieu, King, Duplay, Snodgrass etc.

Tube-onlay

Inlay-onlay,

Two-stage procedure

REFERENCES

Ahmed S, Gough DC.

Buccal mucosal graft for secondary hypospadias repair and urethral replacement. Br J Urol 1997; 80:328-330.

Asopa HS.

Newer concepts in the management of hypospadias and its complications. Ann R Coll Surg Engl 1998; 80: 161-168.

Baskin LS, Duckett JW.

Dorsal tunica albuginea plication for hypospadias curvature. J Urol 1994; 151: 1668-1671.

Borer JG, Retik AB.

Current trends in hypospadias repair. Urol Clin North Am, 1999; 26: 15-37.

Bracka A.

Hypospadias repair: the two-stage alternative. Br J Urol 1995; 76 Suppl 3: 31-41.

Burbige К A.

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Distal hypospadias repair without stents: is it better? J Urol 1994; 151: 1059-1060.

12. Caldamone AA, Edstrom LE, Koyle MA, Rabinowitz R, Hulbert WC.
Buccal mucosal grafts for urethral reconstruction. Urology 1998; 51: 15-19.

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Hipospadias proximal. Arch Esp Urol 1998; 51: 551-559.

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Hypospadie 2000. Akt Urol 2000; 31:147-148.

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INCONTINENCE

BACKGROUND

As a result of maturation, children usually become continent during the day-time by the second year and at night by the fourth year. In cases of persistent wetting, a distinction has to be made between enuresis and urinary incontinence. Particular attention should be paid to:

• Anatomic defects of sphincteric muscle and bladder (e.g. complete epispadias, bladder exstrophy,
urogenital sinus, ectopic ureter)

• Impaired innervation of bladder and sphincteric muscle (e.g. myelomeningocele)

• Functional disturbances of the bladder and sphincteric muscle.

CLASSIFICATION

Enuresis

This is defined as a normal void occurring at an inappropriate or socially unacceptable time or place. Children with nocturnal enuresis void in bed while asleep and are generally not aroused by the wetting. The condition is mono-symptomatic and has a clear familial tendency.

Primary nocturnal enuresis

Involuntary bed wetting from the time of birth without an uninterrupted period of at least 6 months.

Secondary (onset) nocturnal enuresis

Involuntary bed wetting after a dry period of at least 6 months.

Nocturnal polyuria enuresis

This describes nocturnal enuresis in children who have urine production in excess of their functional bladder

capacity, as shown by voiding charts.

Diurnal enuresis

Wetting in attention-deficit disorders; voiding is complete; bladder and urethral function are normal.

Urinary incontinence

This is defined as the involuntary loss of urine, objectively demonstrable and constituting a social or hygienic problem. It denotes a symptom (patient's recognition of involuntary urine loss), a sign (the objective demonstration of urine loss) and a condition (the urodynamic demonstration of urine loss). Day-time, night-time or both day- and night-time incontinence may be encountered. Urinary incontinence is classified according to aetiology:

Incontinence associated with anatomic abnormalities of the urinary tract

Ectopic ureter, ureterocele, prune-belly syndrome, bladder exstrophy, epispadias, posterior urethral valves and

cloacal abnormalities.

Incontinence associated with neurogenic disorder

Spinal dysraphism, caudal regression condition and other central nervous system (CNS) disorders.

Functional incontinence in non-neuropathic bladder sphincter dysfunction (urge syndrome and dysfunctional

voiding)

Often associated with recurrent UTIs and occur predominantly in girls.

DIAGNOSIS

The basic diagnostic evaluation includes:

• History (including family and social history)

• Physical, urological and gross neurological examination (urinalysis and culture, specific gravity)

• Sonography (residual urine, bladder wall thickness, upper tracts)

• Frequency-volume chart (after treatment of infection)

If the basic evaluation does not reveal any pathology, an enuresis (uncomplicated, mono-symptomatic) can be assumed and no further examinations are required.

Further evaluation is required if any pathology is revealed in the basic evaluation and includes:

• Uroflow (if pathological, repeated uroflow and uroflow-electromyography (EMG) study)

• VCUG in case of thickened bladder wall and/or residual urine, in case of pathological, but co-ordinated
micturition

• (Video) urodynamics on suspicion of functional voiding disturbances

• Intravenous urogram (optional if sonography is normal)

• Examination under anaesthesia (urethrocystoscopy, urethral calibration, mainly for UTIs)

• Extended neurological, radiological and psychiatric examinations (including MRI of the spinal cord)

• Radionuclide renal study to assess renal function

TREATMENT

4.4.1 Nocturnal enuresis (mono-symptomatic)

Therapy is started when the condition becomes distressing and the child becomes motivated to be dry, usually after the age of 5-6 years. Behavioural therapy including motivation, counselling about regular voiding and drinking habits, classical conditioning with an alarm-clock, and appropriate handling of constipation, is the first option. Complete dryness in 70% and considerable improvement in 12% of cases can be achieved by treatment with 10-40 mg DDAVP (Desmopressin) nasal spray over a maximum period of 6 months. However, relapse occurs in almost all cases after discontinuation of the medication. Oxybutynin can be helpful in some cases with wetting at the beginning of the night (uninhibited contractions of the bladder).

Urinary incontinence

If recurrent UTIs are present, long-term antibiotic therapy should be started, initially for 6 months. Urinary incontinence is treated according to the aetiology and to urodynamic findings.

Incontinence associated with anatomical abnormalities of the urinary tract Treatment is within the framework of the operative repair of the abnormality.

Incontinence associated with neurogenic disorder

The cornerstone of treatment in young children is oriented towards the protection of renal function and efficient evacuation of the bladder. When the child becomes motivated to be dry (usually after the age of 5 years), the persistent urinary incontinence is addressed. Clean intermittent catheterization to ensure an efficient bladder emptying is mostly applied in patients with detrusor-sphincter dyssynergia. Medical therapy is applied according to the urodynamic findings:

• Detrusor hyperreflexia: oxybutinin, propiverin, tolterodine

• Detrusor sphincter dyssynergia: alpha-blockers, polysynaptic inhibitor (baclofenum)

Surgical therapy can be conducted in the case of renal function deterioration, in persistent high filling detrusor pressure and/or urinary incontinence. Options include bladder augmentation, treatment of sphincter weakness and formation of a catheterizable channel.

Functional incontinence in non-neuropathic bladder-sphincter dysfunction

Urge syndrome: Bladder rehabilitation (counselling about regular voiding and drinking habits, about the

technique of voiding); pharmacotherapy (oxybutynin, propiverin, tolterodine); intravesical stimulation and

transcutaneous neuromodulation (optional).

Dysfunctional voiding (detrusor sphincter dysco-ordination): Bladder rehabilitation (counselling about regular voiding and drinking habits, about the technique of voiding, biofeedback, clean intermittent catheterization if residual urine is significant) and pharmacotherapy (alpha blockers, polysynaptic inhibitor-baclofenum).

Lazy bladder syndrome: Counselling about regular voiding; clean intermittent catheterization; treatment of constipation and intravesical stimulation.

Hinman syndrome: According to urodynamic examination; counselling about regular voiding, usually clean intermittent catheterization if emptying not complete.

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Pediatr Surg Int 1998; 13: 281-284.

BACKGROUND

Hydronephrosis is detectable within the uterus by ultrasound from the 16th week of pregnancy. The commonest causes are ureteropelvic junction (UPJ)-stenosis, megaureters, urethral valve syndrome, vesicorenal reflux and multicystic renal dysplasia.

DIAGNOSIS

Ultrasound examination

Ectasia (anterior-posterior diameter of the renal pelvis, caliceal ectasia), kidney size, thickness of the parenchyma, cortical echo-pattern, width of the ureter, bladder wall thickness and residual urine are assessed during ultrasound examination. With a diameter of the renal pelvis greater than 15 mm, obstruction of the upper urinary tract is likely and correction may be considered. The first ultrasound examination of prenatally diagnosed ectasia of the renal pelvis should be carried out within the first 2 days of life, after 3-5 days and after 3 weeks. A normal ultrasound during the first days of life can be secondary to the oliguria of the newborn.

Diuresis renography

Because of its low radiation exposure, Tc99m-MAG3 is the radionuclide of choice in diuresis renography. The examination is carried out after standardized hydration with a transurethral catheter in place. Renal arterial perfusion, intrarenal cortical transit and excretion of the tracer into the collecting system are measured. If excretion is impaired, it takes longer for half the maximum activity of the radio-isotope to reach the renal pelvis (T1/2) after application of furosemide. With rapid absorption of the tracer and prompt washing out effect on diuresis (T1/2 < 10 min), obstruction is unlikely. Impaired or deteriorating split renal function in newborns or young infants with upper tract dilatation may be the best indicator of significant obstruction.

Static renal scintigraphy

Renal scintigraphy with di-mercaptosuccinic acid (DMSA) is an ideal method for assessment of renal mor­phology, acute infectious changes, renal scars and functional impairment, for example in multicystic renal dysplasia and reflux nephropathy. This investigation should not usually be used within the first 2 months of life.

Intravenous urogram (IVU)

The IVU is an optional examination method and may be performed pre-operatively and in case of inconclusive findings on sonography. The indication for an IVU in the first year of life is problematical.

Whitaker's test

Whitaker's test is carried out as an optional antegrade pressure flow study if diagnosis of obstruction is obscure. The measurement involves continuous perfusion of the renal pelvis via a percutaneous puncture or, if necessary, nephrostomy. Shortcomings of Whitaker's test are the unphysiologically high perfusion rate, lack of normal ranges in children, dependence on the examiner and the invasiveness of the procedure.

TREATMENT

UPJ-stenosis

Symptomatic UPJ-stenosis requires surgical correction, whereas asymptomatic unilateral UPJ-stenosis can be subjected to surveillance depending on the grade of obstruction and split renal function. If function does not improve within 3-6 months in patients with a renal function of less than 10% on the affected side, nephrectomy should be considered, especially when symptoms occur (e.g. UTI, hypertension, flank pain). Patients with a function of less than 40% should undergo pyeloplasty. If the partial function is greater than 40%, operative intervention is indicated only if new symptoms occur or function drops more than 10% with time. Sonography should be performed every 4 weeks. Renal function scintigraphy must be repeated within 1 year. Children with a pre-operative partial function below 45% show enhanced growth and an improved overall renal function post-operatively.

Megaureter

According to the official international classification, megaureters are subdivided into primary and secondary, obstructive and/or refluxive and non-refluxive, non-obstructive. Treatment of the refluxive megaureter is dealt with in the section on vesicoureteral reflux (VUR). The diagnostic evaluation is the same as in unilateral hydronephrosis. The degree of obstruction and the split renal function is determined by renal scintigraphy and IVU.

In view of equivalent results of surveillance, surgical intervention based only on the excretory urogram is now rare. With spontaneous remission rates of up to 85% in primary obstructive megaureters, high drainage by means of Sober's or ring-ureterocutaneostomy is no longer justified.

Operative ureterocystoneostomy according to Cohen, Politano-Leadbetter or the Psoas-Hitch-technique can be considered as an operative measure. Indications for surgical treatment of megaureter are recurrent infections under prophylactic antibiotic medication, deterioration of split renal function, reflux persisting for more than 1 year under prophylaxis and significant obstruction.

Ureterocele

Orthotopic ureterocele is a rare finding in children, exclusively observed in females and mostly associated with a single kidney system. It seldom requires surgical intervention, mostly due to complications (e.g. lithiasis). Diagnostic evaluation follows the scheme 'Unilateral hydronephrosis'. VCUG is mandatory prior to a planned operation. An excretory urogram and cystourethroscopy provide additional information on specific questions. Ectopic ureterocele is dealt with in the section 'Obstructive pathology of renal duplication' (see chapter 9).

Retrocaval ureter

A retrocaval ureter is not an anomaly of the ureter but of the vena cava. Diagnostic evaluation follows the scheme 'Unilateral hydronephrosis' (including excretory urogram). In this rare condition, the ureter is divided, its course corrected and end-to-end anastomosis is performed.

Bilateral hydronephrosis

Bilateral hydronephrosis and megaureter are very rare in girls and require an individual approach, which cannot be dealt with by an algorithm. In boys, the most frequent cause is infravesical obstruction by urethral valves. The obstruction may result in the formation of a trabecular bladder with secondary megaureters and hydronephrosis. After sonography, a VCUG should be carried out without delay. If no pathological findings are discovered, further evaluation follows the 'Unilateral hydronephrosis' scheme. In cases of reflux, diagnostic evaluation follows the 'Reflux' scheme (see chapter 7). In cases of infravesical obstruction with urinary retention, a suprapubic catheter should be inserted immediately and an antegrade VCUG carried out later. Sonography and serum creatine controls should be monitored daily. If sonographic findings improve and serum creatinine falls below 0.6 mg/dL, endoscopic valve resection should be planned between the first and sixth month of life (depending on the baby's size and weight). Endoscopy should take place when the traumatization of the urethra can be minimized. In case of early endoscopic treatment, a stent can be placed in the urethra (6-8 ch) pre-operatively. If there is no improvement of sonographic findings and the serum creatinine does not fall below 0.6 mg/dL, supravesical diversion is necessary and delayed reconstructuion 6 to 9 months later should be planned.

Immediate supravesical diversion is indicated (rarely) in a septic patient with a gross bilateral dolichomegaureter and renal impairment, or in a gross bilateral dolichomegaureter and renal insufficiency that does not respond or continues to deteriorate. The endoscopic cold valve ablation (transurethral or suprapubic) is then performed according to the development of patient conditions and the possibility of spontaneous voiding. A VCUG and a radiological imaging of the diverted upper urinary tract should be carried out pre-operatively.

Recovery No recovery

Reconstruction Nephrectomy

Reconstruction Observation

Reconstruction

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CLASSIFICATION

Asymptomatic bacterium

Significant bacteriuria can be documented in consecutive urine samples without any symptoms.

Cystitis

The infection is limited to the bladder; irritative symptoms are present, but no systemic symptoms or fever.

Acute pyelonephritis

Febrile infection of the renal parenchyma.

Complicated UTI

Due to a urine transport disturbance, malformation or a relevant bladder voiding disturbance.

DIAGNOSIS

Indications for chemical and microbiological urine examination include fever of unknown origin, unclear growth impairment in infants, unclear abdominal complaints or flank pain, frequency, dysuria, smelly urine and gross haematuria. In infants and small children, urine is usually collected in a bag attached to the external genitalia. A positive urine culture requires confirmation by suprapubic puncture (withdrawal of urine by transurethral catheterization is optional). Once the child can void on demand, mid-stream urine is used in boys and transurethral catheterization in girls.

The urine examination consists of paper-strip tests, microscopy and microbiology. Only the examination of bladder puncture urine achieves a sensitivity of > 95%. In cases of a positive urine culture, complete blood count, differential blood count, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are determined together with clinical examination.

Sonography is performed to assess kidney volume, parenchymal echogenicity, thickness and scarring, anomalies (e.g. duplex system), hydronephrosis, dilatation of the ureter and calculi. Bladder wall thickness, bladder configuration, dilatation of the ureter (VCUG, to rule out reflux) and residual urine are assessed with a full bladder. In case of pyelocaliectasis, an IVU should be considered. A VCUG should be carried out after successful antibiotic therapy.

TREATMENT

Asymptomatic bacteriuria

No treatment is required in children with a normal urinary tract; functional voiding disturbances should be excluded.

Pyelonephritis

Intravenous antibiotic therapy with a broad-spectrum penicillin or cephalosporin is started immediately. In case of non-sensitive bacteria, a reserve antibiotic agent is chosen. In newborns, treatment should last for 14-21 days. The therapy is then continued orally for 7-14 days. In impaired renal function, the dosage is adjusted according to the serum creatinine level. Therapy can be stopped when urine culture is sterile and all signs of inflammation have disappeared. DMSA scintigraphy may be performed 3 months after the end of treatment. In obstructive pyelonephritis, urinary drainage should be considered as an emergency.

Complicated UTI

Efficient management of complicated UTIs requires appropriate treatment of the underlying condition (e.g. impaired urinary drainage).

Antibiotic prophylaxis

Nitrofurantoin and trimethoprim are currently considered as first-line treatment; alternatives are amoxycillin or cephalosporins. Long-term antibiotic prophylaxis is required particularly in children with VRR. Other indications for antibiotic prophylaxis are recurrent cystitis with or without voiding disturbances, medullary sponge kidneys, kidney duplication with reflux to the lower pole, ectopic ureters, dilatation of the upper urinary tract, ureterocele and neurogenic bladder dysfunction (e.g. MMC or tethered chord syndrome).

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BACKGROUND

VUR or VRR are defined as non-physiological back-flow of urine from the urinary bladder into the ureter or the renal pelvis. The worst consequence of both primary and secondary VRR is the development of progressive renal failure secondary to recurrent pyelonephritic episodes and subsequent loss of renal parenchyma.

Ten to 15% of patients with reflux suffer from a renin-dependent hypertension as a sequela of focal ischaemia due to parenchymal scarring. The probability of hypertension is highly correlated with the extent and number of parenchymal scars. Reflux is found in 0.5-1 % of normal children without UTIs. While in newborns the incidence of reflux is the same in both sexes, in later life, girls are approximately four times more frequently affected than boys.

In children with recurrent UTI, the incidence of VUR is significantly higher (about 14-29% of kindergarten and school girls and about 30% of boys of the corresponding age).

The main symptoms of VUR are recurrent and occasionally febrile UTIs. Radiological investigations verify uni- or bilateral reflux in 30-50% of cases. A VRR may also make itself apparent in later stages by its consequences, such as hypertension, renal insufficiency or delayed renal growth. Undetected reflux may lead to reflux nephropathy.

Secondary reflux

Predecessor of a secondary reflux is not an inborn primary disturbance of the terminal ureter, but rather an anatomically or functionally obstructive or inflammatory illness of the bladder, or direct damage to a previously intact orifice. It seems that many refluxes, especially of lower grade l-lll, are secondary refluxes and may disappear with the maturation of the bladder function.

CLASSIFICATION

The International Reflux Study Committee introduced a uniform system for the description of reflux, based on the earlier classification by Heikel and Parkkulainen, in 1985. Furthermore, a standardized performance of a voiding cystourethrogram was described to allow comparative studies.

DIAGNOSIS

All patients awaiting clarification of a reflux should undergo a basic diagnostic work up, comprising laboratory tests (kidney function), urine status and urine cultures, together with a detailed medical history and physical examination. Direct evidence of a reflux or its exclusion, obtained by VCUG or sonography supplements the initial diagnosis. An optional IVU is performed before surgery and in any case with an inconclusive sonography. During the examination, a catheter should be in place.

Without exposure to radiation, sonography can provide detailed information on the size of the kidney,

any possible duplicate formations, the shape and size of the collecting system, and also permits assessment of the renal parenchyma. Detection of reflux itself is only possible by using special contrast media and in case of gross reflux.

If VCUG or sonography is negative, but clinical evidence of a suspected reflux persists, the examination should be repeated after an interval; the grade of the reflux is not constant under different conditions of examination.

If residual urine is apparent after micturition, without presence of an infravesical obstruction, a urodynamic examination should be performed in order to exclude bladder/sphincter dysfunction and thus secondary reflux.

Evidence of or exclusion of a reflux can also be provided by nuclear medicinal methods. Due to the much lower exposure to radiation, a radionuclide cystography to verify or to exclude reflux can also be used, especially for follow-up examinations.

Endoscopic examination is helpful in case of planned operative treatment and can provide evidence of a pathological configuration of the ureteral orifices and position, as well as the exclusion of infravesical obstruction. The examination is carried out under anaesthesia and preparation made for subsequent reflux correction. For any reflux therapy to be performed endoscopically, the configuration of the ureteral orifices is of greater importance than the reflux grading; in case of a golf-hole orifice, the prospects of success are markedly lower.

Surgical treatment

Medical treatment

IVU = intravenous urogram; DMSA = di-mercaptosuccinic acid; VCUG = voiding cystourethrography.

Secondary reflux

Diagnosis and treatment of the underlying disease are, of course, primary aspects in secondary acquired reflux. If reflux persists after successful treatment of the underlying condition, therapy of reflux is oriented with respect to clinical conditions. In order to diagnose a secondary reflux, it has to be ensured that VCUG was carried out in a non-inflammatory interval and, if necessary, repeated after clearing up the infection. Further diagnosis then proceeds according to the same rules as primary reflux.

7.4 TREATMENT

The objective of treatment is the avoidance of late complications, such as reflux nephropathy. Therapeutic options consist of conservative medical treatment and endoscopic or open surgical procedures. The choice is influenced by the age of the patient, the grade of reflux, the position or configuration of the ureteral orifices and the clinical course.

Surgery

(not before the age of 6 months)

Surgical therapy

Surgery should not be performed before the age of 6 months.

Endoscopic therapy

Currently, experience with endoscopic treatment as an operative option is limited.

Open surgery

Various techniques for reflux correction have been described (e.g. Lich-Gregoir, Politano-Leadbetter, Cohen, Psoas-Hitch), the principle being to lengthen the intramural part of the ureter by submucosal embedding of the ureter. A high success rate of over 95%, with only a small rate of complications, is shared by all surgical procedures.

As a rule, prior to extravesical procedures an endoscopy should be performed, whereas the ureteral orifices can be directly visualized with intravesical operations. Other important technical details are an absolutely tension free ureteral anastomosis, as well as meticulous preservation of the blood supply of the distal ureter. In addition, a sufficient length and width of the tunnel is mandatory.

In case of a bilateral reflux the Lich-Gregoir as well as the Psoas-Hitch procedure should be performed in two stages to prevent bladder dysfunction.

Follow-up

After surgical reflux correction, patients require peri-operative antibiotic therapy, this being continued as prophylaxis for 6 weeks post-operatively. Optionally, a VCUG is carried out 3 months post-operatively in order to prove successful reflux correction. Obstruction of the upper urinary tract is ruled out by sonography on patients' discharge and this should be repeated after 4-6 weeks if any sign of obstruction is present. In all other cases, a routine ultrasound is carried out 3 months post-operatively.

All patients with parenchymal damage at the time of reflux correction should receive a control scintigraphy 12 months post-surgery. The follow-up examination should include blood pressure controls for early detection of renal hypertension.

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