- •Preface
- •Acknowledgments
- •Contents
- •1.1 Introduction
- •1.2 Normal Embryology
- •1.3 Abnormalities of the Kidney
- •1.3.1 Renal Agenesis
- •1.3.2 Renal Hypoplasia
- •1.3.3 Supernumerary Kidneys
- •1.3.5 Polycystic Kidney Disease
- •1.3.6 Simple (Solitary) Renal Cyst
- •1.3.7 Renal Fusion and Renal Ectopia
- •1.3.8 Horseshoe Kidney
- •1.3.9 Crossed Fused Renal Ectopia
- •1.4 Abnormalities of the Ureter
- •1.5 Abnormalities of the Bladder
- •1.6 Abnormalities of the Penis and Urethra in Males
- •1.7 Abnormalities of Female External Genitalia
- •Further Reading
- •2.1 Introduction
- •2.2 Pathophysiology
- •2.3 Etiology of Hydronephrosis
- •2.5 Clinical Features
- •2.6 Investigations and Diagnosis
- •2.7 Treatment
- •2.8 Antenatal Hydronephrosis
- •Further Reading
- •3.1 Introduction
- •3.2 Embryology
- •3.3 Pathophysiology
- •3.4 Etiology of PUJ Obstruction
- •3.5 Clinical Features
- •3.6 Diagnosis and Investigations
- •3.7 Management of Newborns with PUJ Obstruction
- •3.8 Treatment
- •3.9 Post-operative Complications and Follow-Up
- •Further Reading
- •4: Renal Tumors in Children
- •4.1 Introduction
- •4.2 Wilms’ Tumor
- •4.2.1 Introduction
- •4.2.2 Etiology
- •4.2.3 Histopathology
- •4.2.4 Nephroblastomatosis
- •4.2.5 Clinical Features
- •4.2.6 Risk Factors for Wilms’ Tumor
- •4.2.7 Staging of Wilms Tumor
- •4.2.8 Investigations
- •4.2.9 Prognosis and Complications of Wilms Tumor
- •4.2.10 Surgical Considerations
- •4.2.11 Surgical Complications
- •4.2.12 Prognosis and Outcome
- •4.2.13 Extrarenal Wilms’ Tumors
- •4.3 Mesoblastic Nephroma
- •4.3.1 Introduction
- •4.3.3 Epidemiology
- •4.3.5 Clinical Features
- •4.3.6 Investigations
- •4.3.7 Treatment and Prognosis
- •4.4 Clear Cell Sarcoma of the Kidney (CCSK)
- •4.4.1 Introduction
- •4.4.2 Pathophysiology
- •4.4.3 Clinical Features
- •4.4.4 Investigations
- •4.4.5 Histopathology
- •4.4.6 Treatment
- •4.4.7 Prognosis
- •4.5 Malignant Rhabdoid Tumor of the Kidney
- •4.5.1 Introduction
- •4.5.2 Etiology and Pathophysiology
- •4.5.3 Histologic Findings
- •4.5.4 Clinical Features
- •4.5.5 Investigations and Diagnosis
- •4.5.6 Treatment and Outcome
- •4.5.7 Mortality/Morbidity
- •4.6 Renal Cell Carcinoma in Children
- •4.6.1 Introduction
- •4.6.2 Histopathology
- •4.6.4 Staging
- •4.6.5 Clinical Features
- •4.6.6 Investigations
- •4.6.7 Management
- •4.6.8 Prognosis
- •4.7 Angiomyolipoma of the Kidney
- •4.7.1 Introduction
- •4.7.2 Histopathology
- •4.7.4 Clinical Features
- •4.7.5 Investigations
- •4.7.6 Treatment and Prognosis
- •4.8 Renal Lymphoma
- •4.8.1 Introduction
- •4.8.2 Etiology and Pathogenesis
- •4.8.3 Diagnosis
- •4.8.4 Clinical Features
- •4.8.5 Treatment and Prognosis
- •4.9 Ossifying Renal Tumor of Infancy
- •4.10 Metanephric Adenoma
- •4.10.1 Introduction
- •4.10.2 Histopathology
- •4.10.3 Diagnosis
- •4.10.4 Clinical Features
- •4.10.5 Treatment
- •4.11 Multilocular Cystic Renal Tumor
- •Further Reading
- •Wilms’ Tumor
- •Mesoblastic Nephroma
- •Renal Cell Carcinoma in Children
- •Angiomyolipoma of the Kidney
- •Renal Lymphoma
- •Ossifying Renal Tumor of Infancy
- •Metanephric Adenoma
- •Multilocular Cystic Renal Tumor
- •5.1 Introduction
- •5.2 Embryology
- •5.4 Histologic Findings
- •5.7 Associated Anomalies
- •5.8 Clinical Features
- •5.9 Investigations
- •5.10 Treatment
- •Further Reading
- •6: Congenital Ureteral Anomalies
- •6.1 Etiology
- •6.2 Clinical Features
- •6.3 Investigations and Diagnosis
- •6.4 Duplex (Duplicated) System
- •6.4.1 Introduction
- •6.4.3 Clinical Features
- •6.4.4 Investigations
- •6.4.5 Treatment and Prognosis
- •6.5 Ectopic Ureter
- •6.5.1 Introduction
- •6.5.3 Clinical Features
- •6.5.4 Diagnosis
- •6.5.5 Surgical Treatment
- •6.6 Ureterocele
- •6.6.1 Introduction
- •6.6.3 Clinical Features
- •6.6.4 Investigations and Diagnosis
- •6.6.5 Treatment
- •6.6.5.1 Surgical Interventions
- •6.8 Mega Ureter
- •Further Reading
- •7: Congenital Megaureter
- •7.1 Introduction
- •7.3 Etiology and Pathophysiology
- •7.4 Clinical Presentation
- •7.5 Investigations and Diagnosis
- •7.6 Treatment and Prognosis
- •7.7 Complications
- •Further Reading
- •8.1 Introduction
- •8.2 Pathophysiology
- •8.4 Etiology of VUR
- •8.5 Clinical Features
- •8.6 Investigations
- •8.7 Management
- •8.7.1 Medical Treatment of VUR
- •8.7.2 Antibiotics Used for Prophylaxis
- •8.7.3 Anticholinergics
- •8.7.4 Surveillance
- •8.8 Surgical Therapy of VUR
- •8.8.1 Indications for Surgical Interventions
- •8.8.2 Indications for Surgical Interventions Based on Age at Diagnosis and the Presence or Absence of Renal Lesions
- •8.8.3 Endoscopic Injection
- •8.8.4 Surgical Management
- •8.9 Mortality/Morbidity
- •Further Reading
- •9: Pediatric Urolithiasis
- •9.1 Introduction
- •9.2 Etiology
- •9.4 Clinical Features
- •9.5 Investigations
- •9.6 Complications of Urolithiasis
- •9.7 Management
- •Further Reading
- •10.1 Introduction
- •10.2 Embryology of Persistent Müllerian Duct Syndrome
- •10.3 Etiology and Inheritance of PMDS
- •10.5 Clinical Features
- •10.6 Treatment
- •10.7 Prognosis
- •Further Reading
- •11.1 Introduction
- •11.2 Physiology and Bladder Function
- •11.2.1 Micturition
- •11.3 Pathophysiological Changes of NBSD
- •11.4 Etiology and Clinical Features
- •11.5 Investigations and Diagnosis
- •11.7 Management
- •11.8 Clean Intermittent Catheterization
- •11.9 Anticholinergics
- •11.10 Botulinum Toxin Type A
- •11.11 Tricyclic Antidepressant Drugs
- •11.12 Surgical Management
- •Further Reading
- •12.1 Introduction
- •12.2 Etiology
- •12.3 Pathophysiology
- •12.4 Clinical Features
- •12.5 Investigations and Diagnosis
- •12.6 Management
- •Further Reading
- •13.1 Introduction
- •13.2 Embryology
- •13.3 Epispadias
- •13.3.1 Introduction
- •13.3.2 Etiology
- •13.3.4 Treatment
- •13.3.6 Female Epispadias
- •13.3.7 Surgical Repair of Female Epispadias
- •13.3.8 Prognosis
- •13.4 Bladder Exstrophy
- •13.4.1 Introduction
- •13.4.2 Associated Anomalies
- •13.4.3 Principles of Surgical Management of Bladder Exstrophy
- •13.4.4 Evaluation and Management
- •13.5 Cloacal Exstrophy
- •13.5.1 Introduction
- •13.5.2 Skeletal Changes in Cloacal Exstrophy
- •13.5.3 Etiology and Pathogenesis
- •13.5.4 Prenatal Diagnosis
- •13.5.5 Associated Anomalies
- •13.5.8 Surgical Reconstruction
- •13.5.9 Management of Urinary Incontinence
- •13.5.10 Prognosis
- •13.5.11 Complications
- •Further Reading
- •14.1 Introduction
- •14.2 Etiology
- •14.3 Clinical Features
- •14.4 Associated Anomalies
- •14.5 Diagnosis
- •14.6 Treatment and Prognosis
- •Further Reading
- •15: Cloacal Anomalies
- •15.1 Introduction
- •15.2 Associated Anomalies
- •15.4 Clinical Features
- •15.5 Investigations
- •Further Reading
- •16: Urachal Remnants
- •16.1 Introduction
- •16.2 Embryology
- •16.4 Clinical Features
- •16.5 Tumors and Urachal Remnants
- •16.6 Management
- •Further Reading
- •17: Inguinal Hernias and Hydroceles
- •17.1 Introduction
- •17.2 Inguinal Hernia
- •17.2.1 Incidence
- •17.2.2 Etiology
- •17.2.3 Clinical Features
- •17.2.4 Variants of Hernia
- •17.2.6 Treatment
- •17.2.7 Complications of Inguinal Herniotomy
- •17.3 Hydrocele
- •17.3.1 Embryology
- •17.3.3 Treatment
- •Further Reading
- •18: Cloacal Exstrophy
- •18.1 Introduction
- •18.2 Etiology and Pathogenesis
- •18.3 Associated Anomalies
- •18.4 Clinical Features and Management
- •Further Reading
- •19: Posterior Urethral Valve
- •19.1 Introduction
- •19.2 Embryology
- •19.3 Pathophysiology
- •19.5 Clinical Features
- •19.6 Investigations and Diagnosis
- •19.7 Management
- •19.8 Medications Used in Patients with PUV
- •19.10 Long-Term Outcomes
- •19.10.3 Bladder Dysfunction
- •19.10.4 Renal Transplantation
- •19.10.5 Fertility
- •Further Reading
- •20.1 Introduction
- •20.2 Embryology
- •20.4 Clinical Features
- •20.5 Investigations
- •20.6 Treatment
- •20.7 The Müllerian Duct Cyst
- •Further Reading
- •21: Hypospadias
- •21.1 Introduction
- •21.2 Effects of Hypospadias
- •21.3 Embryology
- •21.4 Etiology of Hypospadias
- •21.5 Associated Anomalies
- •21.7 Clinical Features of Hypospadias
- •21.8 Treatment
- •21.9 Urinary Diversion
- •21.10 Postoperative Complications
- •Further Reading
- •22: Male Circumcision
- •22.1 Introduction
- •22.2 Anatomy and Pathophysiology
- •22.3 History of Circumcision
- •22.4 Pain Management
- •22.5 Indications for Circumcision
- •22.6 Contraindications to Circumcision
- •22.7 Surgical Procedure
- •22.8 Complications of Circumcision
- •Further Reading
- •23: Priapism in Children
- •23.1 Introduction
- •23.2 Pathophysiology
- •23.3 Etiology
- •23.5 Clinical Features
- •23.6 Investigations
- •23.7 Management
- •23.8 Prognosis
- •23.9 Priapism and Sickle Cell Disease
- •23.9.1 Introduction
- •23.9.2 Epidemiology
- •23.9.4 Pathophysiology
- •23.9.5 Clinical Features
- •23.9.6 Treatment
- •23.9.7 Prevention of Stuttering Priapism
- •23.9.8 Complications of Priapism and Prognosis
- •Further Reading
- •24.1 Introduction
- •24.2 Embryology and Normal Testicular Development and Descent
- •24.4 Causes of Undescended Testes and Risk Factors
- •24.5 Histopathology
- •24.7 Clinical Features and Diagnosis
- •24.8 Treatment
- •24.8.1 Success of Surgical Treatment
- •24.9 Complications of Orchidopexy
- •24.10 Infertility and Undescended Testes
- •24.11 Undescended Testes and the Risk of Cancer
- •Further Reading
- •25: Varicocele
- •25.1 Introduction
- •25.2 Etiology
- •25.3 Pathophysiology
- •25.4 Grading of Varicoceles
- •25.5 Clinical Features
- •25.6 Diagnosis
- •25.7 Treatment
- •25.8 Postoperative Complications
- •25.9 Prognosis
- •Further Reading
- •26.1 Introduction
- •26.2 Etiology and Risk Factors
- •26.3 Diagnosis
- •26.4 Intermittent Testicular Torsion
- •26.6 Effects of Testicular Torsion
- •26.7 Clinical Features
- •26.8 Treatment
- •26.9.1 Introduction
- •26.9.2 Etiology of Extravaginal Torsion
- •26.9.3 Clinical Features
- •26.9.4 Treatment
- •26.10 Torsion of the Testicular or Epididymal Appendage
- •26.10.1 Introduction
- •26.10.2 Embryology
- •26.10.3 Clinical Features
- •26.10.4 Investigations and Treatment
- •Further Reading
- •27: Testicular Tumors in Children
- •27.1 Introduction
- •27.4 Etiology of Testicular Tumors
- •27.5 Clinical Features
- •27.6 Staging
- •27.6.1 Regional Lymph Node Staging
- •27.7 Investigations
- •27.8 Treatment
- •27.9 Yolk Sac Tumor
- •27.10 Teratoma
- •27.11 Mixed Germ Cell Tumor
- •27.12 Stromal Tumors
- •27.13 Simple Testicular Cyst
- •27.14 Epidermoid Cysts
- •27.15 Testicular Microlithiasis (TM)
- •27.16 Gonadoblastoma
- •27.17 Cystic Dysplasia of the Testes
- •27.18 Leukemia and Lymphoma
- •27.19 Paratesticular Rhabdomyosarcoma
- •27.20 Prognosis and Outcome
- •Further Reading
- •28: Splenogonadal Fusion
- •28.1 Introduction
- •28.2 Etiology
- •28.4 Associated Anomalies
- •28.5 Clinical Features
- •28.6 Investigations
- •28.7 Treatment
- •Further Reading
- •29: Acute Scrotum
- •29.1 Introduction
- •29.2 Torsion of Testes
- •29.2.1 Introduction
- •29.2.3 Etiology
- •29.2.4 Clinical Features
- •29.2.5 Effects of Torsion of Testes
- •29.2.6 Investigations
- •29.2.7 Treatment
- •29.3 Torsion of the Testicular or Epididymal Appendage
- •29.3.1 Introduction
- •29.3.2 Embryology
- •29.3.3 Clinical Features
- •29.3.4 Investigations and Treatment
- •29.4.1 Introduction
- •29.4.2 Etiology
- •29.4.3 Clinical Features
- •29.4.4 Investigations and Treatment
- •29.5 Idiopathic Scrotal Edema
- •29.6 Testicular Trauma
- •29.7 Other Causes of Acute Scrotum
- •29.8 Splenogonadal Fusion
- •Further Reading
- •30.1 Introduction
- •30.2 Imperforate Hymen
- •30.3 Vaginal Atresia
- •30.5 Associated Anomalies
- •30.6 Embryology
- •30.7 Clinical Features
- •30.8 Investigations
- •30.9 Management
- •Further Reading
- •31: Disorders of Sexual Development
- •31.1 Introduction
- •31.2 Embryology
- •31.3 Sexual and Gonadal Differentiation
- •31.5 Evaluation of a Newborn with DSD
- •31.6 Diagnosis and Investigations
- •31.7 Management of Patients with DSD
- •31.8 Surgical Corrections of DSD
- •31.9 Congenital Adrenal Hyperplasia (CAH)
- •31.10 Androgen Insensitivity Syndrome (Testicular Feminization Syndrome)
- •31.13 Gonadal Dysgenesis
- •31.15 Ovotestis Disorders of Sexual Development
- •31.16 Other Rare Disorders of Sexual Development
- •Further Reading
- •Index
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8 Vesicoureteral Reflux (VUR) in Children |
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Fig. 8.39 A micturating cystourethrogram showing grade V VUR. Note also the enlarged urinary bladder
8.6Investigations
•VUR not only increases the frequency of UTI’s, but also the risk of damage to upper urinary structures.
•Vesicoureteral reflux can result in substantial morbidity, both from acute infection and from the sequelae of reflux nephropathy.
•Early diagnosis and treatment of VUR is crucial to avoid permanent renal cortical scarring and subsequent renal insufficiency.
•CBC
•ESR and C-reactive protein
•BUN, serum electrolytes and creatinine
•The diagnosis of UTI depends on urine analysis and urine culture.
•The 2011 American Academy of Pediatrics (AAP) guidelines specify that both a urinalysis showing pyuria and a culture growing more than 50,000 CFU/mL should be the basis for a diagnosis of UTI.
•To diagnose UTI, it is important to obtain a non-contaminated urine specimen.
–A clean-catch midstream urine specimen is the most acceptable method especially in children who are toilet trained.
–Growth of more than 100,000 CFU/mL is a significant finding on a midstream-voided specimen.
–Suprapubic urine aspiration is used in infants and children.
–Any growth in suprapubic urine sample should be considered significant.
–Urethral catheterization is an alternative way to obtain a clean urine sample but it is more invasive.
–Growth of more than 1,000 colony-form- ing units (CFU)/mL is considered significant in a urine sample obtained by urethral catheterization.
–The least reliable method is the most common method of obtaining a urine specimen in babies. A urine sample is obtained from a urine collection bag. This method is not reliable and should be discouraged.
–This method is more useful if there is no growth in the urine sample as false-negative results are unlikely.
–As many as 10 % of urine specimens obtained by this method grow more than 50,000 CFU/mL with no correlation to actual presence of infection.
•Imaging studies are the basis of diagnosis and management of VUR.
•The standard imaging tests include renal and bladder ultrasonography and voiding cystourethrography (VCUG) (Figs. 8.40 and 8.41).
•Indications for imaging studies are as follows:
–After the first UTI in all children younger than 5 years
–Children of any age with febrile UTI
–Boys of any age with UTI
–Children with prenatally diagnosed hydronephrosis should be evaluated postnatally.
–Ultrasonography should be performed after the first 3 days of life.
–Ultrasonography performed during the first 3 days of life may have a high rate of falsenegative results because of relative dehydration during the neonatal period.
•The following radiological investigations can be performed to evaluate for VUR:
•Ultrasonography
•VCUG or radionuclide cystography (RNC)
8.6 Investigations |
255 |
|
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Figs. 8.40 and 8.41 Micturating cystourethrograms showing severe VUR
• Some advocate that children with a history of |
– |
Ultrasonography is not useful to diagnose |
|
|
febrile UTI undergo a dimercaptosuccinic |
|
or rule out VUR. |
|
acid (DMSA) renal scan, to assess for evi- |
– A normal ultrasonography does not exclude |
|
|
dence of kidney involvement, kidney scarring, |
|
vesicoureteral reflux. |
|
or both; if DMSA scan findings are positive, |
– |
Ultrasonography is useful to detect upper |
|
VCUG is recommended. |
|
urinary tract obstruction, to detect dilated |
• Others advocate performing RNC as the ini- |
|
ureters and renal scarring. |
|
|
tial screening test in girls and then to perform |
– |
All children with a history of febrile UTI |
|
standard VCUG when VUR is observed. |
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should undergo kidney and bladder |
• |
Other clinicians use VCUG for the initial |
|
ultrasonography. |
|
diagnosis and use RNC for follow-up |
– Abdominal ultrasound is usually normal in |
|
|
studies. |
|
those with low to moderate VUR. |
• |
The 2011 American Academy of Pediatrics |
– |
An abdominal ultrasound might suggest |
|
(AAP) guidelines for management of UTI in chil- |
|
the presence of VUR if ureteral dilatation is |
|
dren aged 2–24 months recommend that VCUG |
|
present. |
|
not be performed after an initial febrile UTI. |
• Voiding cystourethrography (VCUG): |
|
• |
Abdominal ultrasound: |
– This is the method of choice for diagnosing |
|
|
– The 2011 AAP guidelines recommend that |
|
and grading VUR. |
|
ultrasonography alone should be the initial |
– |
VCUG provides precise anatomic detail |
|
screening test for children after UTI. |
|
and allows grading of the reflux. |
|
– The Society for Pediatric Urology contin- |
– The International Classification System for |
|
|
ues to recommend that both ultrasonogra- |
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VUR is as follows: |
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phy and cystography be performed. |
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• Grade I: Reflux into nondilated ureter |
256 |
8 Vesicoureteral Reflux (VUR) in Children |
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•Grade II: Reflux into renal pelvis and calyces without dilation
•Grade III: Reflux with mild to moderate dilation and minimal blunting of fornices
•Grade IV: Reflux with moderate ureteral tortuosity and dilation of pelvis and calyces
•Grade V: Reflux with gross dilation of ureter, pelvis, and calyces, loss of papillary impressions, and ureteral tortuosity
–VCUG should not be performed in the presence of UTI and should be delayed till the child has fully recovered from the UTI. This should be confirmed with a fresh urine analysis and culture.
–Performance of VCUG during an episode of acute cystitis can result in overestimation of the grade of reflux because of paralysis and laxity of the ureteral musculature by bacterial endotoxin. Conversely, some children demonstrate reflux only during an episode of cystitis.
–VCUG outlines the urethra in males with posterior urethral valves.
–VCUG provides information in both boys and girls about bladder capacity and emptying and may reveal signs of outlet obstruction, such as bladder trabeculae or diverticula.
–Standard VCUG is recommended as the initial study in boys.
•Radionuclide cystography:
–This is performed by instillation of technetium-99m pertechnetate into the bladder and observation with a gamma camera.
–This is a highly sensitive test to diagnose VUR.
–The disadvantage of this investigation is the poor anatomical detail and failure to precisely classify the degree of VUR.
–Grade I reflux is poorly detected by this study, because the distal ureters are commonly obscured by the bladder
–Grading of VUR by nuclear cystography is limited to mild, moderate, and severe grades.
–Many clinicians use VCUG for the initial diagnosis and use RNC for follow-up studies.
•Dimercaptosuccinic acid (DMSA) renal scan:
–Although the traditional approach in children with UTI has been evaluation for vesicoureteral reflux with VCUG or radionuclide cystography (RNC), some authorities are now advocating a “topdown” approach for children with UTI.
–In this approach, a child with a history of febrile UTI undergoes a dimercaptosuccinic acid (DMSA) renal scan to assess for evidence of kidney involvement, kidney scarring, or both.
–Negative DMSA scan findings suggest that clinically significant vesicoureteral reflux is unlikely, obviating the need for VCUG.
–However, if DMSA scan findings are positive, VCUG is recommended.
–Reflux grade was significantly associated with the prevalence of renal scarring.
–VCUG is recommended after the first acute episode of infection is confirmed using dimercapto-succinic acid scintigraphy.
–Areas of acute inflammation or scarring do not take up the radiopharmaceutical and are revealed as cold spots on imaging.
–DMSA is used to identify and monitor progression of renal scarring.
–Patients who are medically treated and develop new or progressive scarring are often considered candidates for surgical correction of vesicoureteral reflux.
–DMSA can also be used as a diagnostic tool during suspected episodes of acute pyelonephritis but this role is not well defined.
•Urodynamic studies (Cystometrography):
–These reveal functional abnormalities of the lower urinary tract.
–These studies are however difficult to perform in infants and small children.
–Urodynamic studies are important in patients with secondary VUR, such as patients with spina bifida, detrusor instability or boys whose VCUG is suggestive of residual posterior urethral valves.
–Antireflux surgery is much less successful in patients with secondary reflux, and because of this it is important to identifying