- •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
608 |
29 Acute Scrotum |
|
|
Fig. 29.12 An intraoperative photograph showing an already necrotic testis in a newborn with testicular torsion
Figs. 29.14 and 29.15 Intraoperative photographs showing viable testes in two newborns with intrauterine torsion following exploration and detorsion of the testes
Fig. 29.13 An intraoperative photograph showing already necrotic testes in a newborn with bilateral intrauterine testicular torsion
29.3Torsion of the Testicular or Epididymal Appendage
29.3.1 Introduction
•Torsion of testicular appendices is one of the most common causes of acute scrotum (Fig. 29.16).
•There are two types of testicular appendices:
–The appendix testis
–The epididymal appendix
•Torsion of testicular appendices is considered the leading cause of acute scrotum in children.
•In those with acute scrotal pain, the incidence of torsion of testicular appendage ranges from 46 % to 71 %.
•Torsion of the testicular appendices is virtually a benign condition, but must be distinguished from testicular torsion.
•The appendix testis and epididymal appendix are commonly pedunculated and because of this are predisposed to torsion.
•Torsion of either appendage (The appendix testis and epididymal appendix) produces pain similar to that experienced with testicular torsion, but the onset is usually more gradual.
29.3 Torsion of the Testicular or Epididymal Appendage |
609 |
|
|
Fig. 29.16 Diagrammatic representation of the two common testicular appendages
THE VAS AND
VESSELS
THE EPIDIDYMAL
APPENDIX
THE APPENDIX
TESTIS
29.3.2 Embryology
•The appendix testis:
–This is a Müllerian duct remnant.
–It is present in 92 % of all testes.
–It is located at the superior pole of the testis in the groove between the testis and epididymis.
–It is the most common appendage to undergo torsion.
•The epididymal appendix:
–This is a Wolffian duct remnant.
–The appendix epididymis is present in 23 % of testes.
–It is usually located on the head of the epididymis.
–It is the second common appendage to undergo torsion.
29.3.3 Clinical Features
•The majority (80 %) of torsion of the testicular or epididymal appendage occurs in boys aged 7–14 years (Mean age 10.6 years).
•The usual presentation is acute scrotal pain but the onset is more gradual. This is important in distinguishing this from testicular torsion which presents with sudden acute scrotal pain.
•The pain is more localized to the upper pole of the testis which is also tender.
Fig. 29.17 An intraoperative photograph showing torsion of the appendix testis
•The pain is usually not associated with systemic symptoms such as nausea, vomiting or urinary symptoms.
•Usually, the scrotum appears normal but sometimes there is an associated erythema and edema.
•The cremasteric reflex is usually intact.
•Occasionally, a paratesticular nodule at the superior aspect of the testicle is present. This is called the blue-dot sign which is present in only 20 % of cases (Fig. 29.17).
29.3.4 Investigations and Treatment
•Ultrasonography can be useful in distinguishing torsion of a testis and torsion of an appendix testis.
610 |
29 Acute Scrotum |
|
|
•Color Doppler ultrasonography is the imaging modality of choice for evaluation of the acute scrotum.
•This usually shows normal blood flow to the testis and sometimes an increase blood flow on the affected side due to inflammation.
•This is a selflimiting condition and most cases are treated conservatively.
•Rarely surgery is indicated:
–If it is difficult to differentiate from testicular torsion.
–If the pain is severe and cannot be controlled by analgesics.
•The management includes:
–Bed rest and scrotal elevation.
–Nonsteroidal anti-inflammatory drugs and Fig. 29.18 A clinical photograph showing a child with
analgesics.
–Torsion of a testicular appendage may be misdiagnosed as epididymitis but if the urinalysis is normal, no antibiotic therapy is required.
–The inflammation usually resolves within a week.
acute scrotum secondary to severe epididymo-orchitis with intrascrotal abscess formation
•It has been shown that 47 % of prepubertal boys and 75 % of infants with epididymitis have an underlying urogenital anomaly.
29.4Epididymitis, Orchitis, and Epididymo-orchitis
29.4.1 Introduction
•Acute epididymo-orchitis is a clinical diagnosis consisting of pain, swelling and inflammation of the epididymis, with or without inflammation of the testes.
•It is an important cause of acute scrotum in children (Fig. 29.18).
•Orchitis (infection limited to the testis) is much less common and commonly caused by mumps.
•Chronic epididymitis refers to inflammation that lasts for more than 6 months.
•Epididymitis is considered the most common cause of acute scrotum in older boys, and it is important to differentiate this from testicular torsion.
•There is an increased incidence of genitourinary abnormalities in prepubertal boys with epididymitis.
29.4.2 Etiology
•The exact etiology of acute epididymitis is unknown.
•Acute epididymitis is believed to be caused by the retrograde passage of urine from the prostatic urethra to the epididymis via the ejaculatory ducts and vas deferens.
•There are however several contributing causes for acute epididymitis including:
–Genitourinary abnormalities in infants and young boys.
–In older boys, acute epididymitis is often idiopathic.
–Epididymitis can also be secondary to systemic diseases, such as:
•Sarcoidosis.
•Kawasaki disease.
•Henoch-Schönlein purpura.
–Inflammation of the epididymis may be also reactive secondary to trauma or torsion of an appendix testis.
–Chemical irritation from sterile reflux of urine into the seminal tract.
29.4 Epididymitis, Orchitis, and Epididymo-orchitis |
611 |
|
|
–Epididymitis in children can also be druginduced (amiodarone-induced epididymitis)
•Bacterial epididymitis is caused by several organisms including:
–Coliforms,Pseudomonasspecies,Ureaplasma, Mycoplasma species, Staphylococcus, Proteus species, and Haemophilus influenzae.
–In sexually active patients, Chlamydia and/ or Neisseria gonorrhoeae may be the causative organism.
–Viral causes include paramyxovirus, Coxsackie virus, echovirus, and adenovirus.
–Granulomatous epididymitis is very rare in children and can be secondary to tuberculosis.
29.4.3 Clinical Features
•The usual presentation is with unilateral scrotal pain and swelling of relatively acute onset.
•Acute epididymitis is usually unilateral but it is bilateral in 5–10 % of the patients.
•There may be a history of a urinary tract infection.
•Tenderness on the affected side.
•The epididymis will be enlarged and tender or the whole testis and epididymis will be tender.
•There may also be erythema and/or edema of the scrotum on the affected side.
29.4.4 Investigations and Treatment
•Urinalysis with culture and sensitivity.
•Blood culture.
•Infants and children with epididymitis have a high incidence of associated urogenital abnormalities, and thus require full urological evaluation.
•Renal ultrasound, a voiding cystourethrogram and urodynamic studies are necessary investigations in prepubertal boys with acute epididymitis. This is specially so in the presence of urinary tract infection (Figs. 29.19, 29.20 and 29.21).
Clinical features |
|
Pain |
96 % |
Swelling |
100 % |
Erythema |
72 % |
Fever |
40 % |
|
|
Leucocytosis |
44 % |
A positive urinalysis |
24 % |
Lower urinary tract symptoms |
16 % |
(frquency, urgency, enuresis) |
|
|
|
Nausea and vomiting |
16 % |
•In patients with signs of urinary tract infection, treatment includes empiric antibiotic therapy until the results of a urine culture are known.
•Treatment should start with an oral or I.V broad-spectrum antibiotic depending on the presence or absence of signs of systemic infection. Treatment should be continued for 10–14 days and the antibiotics modified according to the culture result.
•Patients with underlying genito-urinary abnormalities usually require surgical intervention.
•In the absence of urinary tract infection, treatment is supportive including:
–Bed rest and scrotal elevation.
–Non-steroidal anti-inflammatory drugs and analgesics.
Differential diagnosis and management of the acute scrotum
|
Onset of |
|
Site of |
|
Cremasteric |
|
Type |
symptoms |
Age at diagnosis |
tenderness |
Urinalysis |
reflex |
Treatment |
Testicular torsion |
Acute |
Early puberty |
Diffused |
Negative |
Negative |
Surgical |
|
|
|
|
|
|
exploration |
Appendicealr |
Subacute |
Prepubertal |
Localized to |
Negative |
Positive |
Bed rest and |
torsion |
|
|
upper pole |
|
|
scrotal |
|
|
|
|
|
|
elevation |
Epididymitis |
Insidious |
Adolescence |
Epididymal |
Positive or |
Positive |
Antibiotics |
|
|
|
|
negative |
|
|
|
|
|
|
|
|
|