- •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
Acute Scrotum |
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29.1Introduction
•The term acute scrotum refers to acute scrotal pain with or without swelling and erythema.
•Acute scrotum is considered a surgical emergency and although there are several causes for acute scrotum, prompt differentiation between testicular torsion and other causes of acute scrotum is critical.
•The possibility of testicular torsion and permanent ischemic damage to the testis must always be kept in mind if the diagnosis is delayed.
•There are several causes of acute scrotum and the etiology is age dependent.
•Testicular torsion is commonly seen in neonates and adolescents.
•Torsion of the appendix testis and acute epididymo-orchitis on the other hand are seen most commonly in prepubertal boys.
•The differential diagnosis of acute scrotum include:
–Testicular torsion (16 %)
–Torsion of a testicular appendage (46 %)
–Acute epididymo-orcitis (35 %)
–Idiopathic scrotal edema
–Schönlein-Henoch purpura
–Incarcerated inguinal hernia
–Scrotal trauma
–Splenogonadal fusion
Common Causes of Acute Scrotum
1.Testicular torsion (16 %)
2.Torsion of a testicular appendage (46 %)
3.Acute epididymo-orchitis (35 %)
4.Idiopathic scrotal edema
5.Schonlein-henoch purpura
6.Incarcerated inguinal hernia
7.Scrotal and testicular trauma
•The child with acute scrotum should be evaluated rapidly both clinically and if required radiologically and the possibility of torsion of testis must always be kept in mind.
•This is important because salvaging a testis in a child with testicular torsion is time dependent.
•In doubtful cases, it is important to do an emergency testicular exploration rather than wait and loss the testis.
29.2Torsion of Testes
29.2.1 Introduction
•Testicular torsion is the most surgical emergency of acute scrotum as delay in diagnosis is known to be associated with testicular ischemia and infarction.
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29 Acute Scrotum |
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•It results from twisting of the spermatic cord leading to venous and arterial obstruction and loss of the blood supply to the affected testis.
•Early diagnosis and treatment are vital to saving the testis and preserving future fertility.
•Testicular torsion is primarily a disease of adolescents and neonates.
•Neonates develop a special type of testicular torsion which is called intrauterine testicular torsion.
•This is different from the more common torsion seen in adolescent.
–This type of torsion occurs extravaginally while the adolescent torsion occurs intravaginally.
–This type of torsion commonly occurs in utero but there are reported cases occurring shortly after birth.
–Intrauterine torsion is known to be associated with a high incidence of testicular infarction as in the majority of reported cases, the testis is already necrotic at the time of exploration
–There are however rare reports of testicular salvage in some of these patients.
•Diagnosis of testicular torsion is clinical, and diagnostic testing should not delay treatment.
•Approximately 32 % of pediatric torsion cases resulted in the orchiectomy.
29.2.2 Classification
Testicular torsion is divided into two types (Fig. 29.1):
1.Intravaginal torsion:
•Intravaginal torsion most commonly occurs in adolescents.
•Intravaginal torsion constitutes approximately 16 % of cases in patients presenting to an emergency department with acute scrotum.
•This form of testicular torsion is most commonly seen in males younger than 30 years.
•The peak incidence occurs at age 12–18 years.
•The left testis is more frequently involved.
•Bilateral torsion account for 2 % of all torsions.
Fig. 29.1 Diagrammatic
representation of the two INTRAVAGINAL TORSION EXTRAVAGINAL TORSION types of testicular torsion
29.2 Torsion of Testes |
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2.Extravaginal torsion (Figs. 29.2 and 29.3):
•Extravaginal torsion occurs most commonly in neonates.
•It accounts for approximately 5 % of all torsions.
•The timing of occurrence of extravaginal torsion is not known exactly and it is estimated that about 70 % of the cases occur prenatally and 30 % occur postnatally.
•Extravaginal torsion is also known to occur in patients with high birth weight.
•Extravaginal torsion occurs most commonly on one side and bilateral perinatal torsion is extremely rare, but an increase in the number of case reports with bilateral intrauterine torsion has been observed.
•The occurrence of bilateral torsion can be:
–Synchronous (occurring simultaneously)
–Metachronous (occurring at different times).
Figs. 29.2 and 29.3 Intraoperative photographs of two newborns with extravaginal testicular torsion
–This is one reasons why contralateral orchidopexy is important in those with unilateral intrauterine torsion.
29.2.3 Etiology
1.Intravaginal torsion:
•The exact etiology of intravaginal torsion is not known.
•Normally, once the testes reaches the scrotum it is fixed in place by mature attachments. This ensures firm fixation of the epididymal-testicular complex posteriorly and effectively prevents twisting of the spermatic cord.
•The tunica vaginalis is normally attached to the postero-lateral aspect of the testes.
•The most accepted embryological etiology of intravaginal testicular torsion is the bell clapper deformity.
•This defect occurs in about 17 % of males and is bilateral in 40 %.
•High abnormal attachment of the tunica vaginalis to the testicle makes the spermatic cord liable to rotate within it, which can lead to intravaginal torsion.
•In intravaginal torsion, the testis can rotate freely on the spermatic cord within the tunica vaginalis.
•In males with the bell-clapper deformity, torsion can occur because of a lack of fixation, resulting in the testes being freely suspended within the tunica vaginalis (Fig. 29.4).
•The bell clapper deformity can result in the long axis of the testicle being oriented transversely rather than cephalocaudal.
•Another abnormality that can predispose to intravaginal torsion is abnormal mesentery between the testis and its blood supply.
1.Extravaginal torsion:
•This occurs because the tunica vaginalis is not yet secured to the gubernaculum and, therefore, the spermatic cord, as well as the tunica vaginalis, undergo torsion as a unit.
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29 Acute Scrotum |
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Fig. 29.4 Diagrammatic representation of the bell-clapper deformity. This predisposes to intravaginal torsion of testis
TUNICA
VAGINALIS
EPIDIDYMIS
TESTIS
•In neonates, the testes frequently has not yet fully descended into the scrotum, where it becomes attached within the tunica vaginalis. This mobility of the testicle predisposes it to extravaginal torsion.
29.2.4 Clinical Features
Intravaginal Torsion
•The usual presentation of intravaginal testicular torsion is a sudden onset of severe unilateral scrotal pain followed by inguinal and/or scrotal swelling. Gradual onset of pain is an uncommon presentation.
•The pain may decrease in severity as the necrosis of testis becomes more complete.
•Torsion of testis commonly occurs spontaneously but may occur during sports or physical activity.
•Torsion of testis can also occur in association of trauma. This is seen in 4–8 % of the cases.
•Approximately one third of patients also have gastrointestinal upset with nausea and vomiting.
•The patients rarely report voiding difficulties or painful micturition.
•In some patients, scrotal trauma or other scrotal disease (including torsion of appendix testis or epididymitis) may precede the occurrence of subsequent testicular torsion.
•The patients may describe previous episodes of recurrent acute scrotal pain that has resolved spontaneously. This history is highly suggestive of intermittent torsion and detorsion of the testis. A detailed history is important in this regard.
•Acute testicular torsion develops in 10 % of patients with intermittent torsion while they waite for surgery.
•Physical examination may reveal:
–A swollen, tender, high-riding testis
–Abnormal transverse lie of testis
–Loss of the cremasteric reflex
–Edema involving the entire scrotum
–Enlargement and edema of the testis
–Fever is uncommon
–Scrotal erythema
•The cremasteric reflex is almost always absent or diminished on the affected side in patients with testicular torsion.
•Prehn’s sign: Relief of pain with elevation of the affected testis.
•Although a negative Prehn’s sign is classically thought to be a predictor of torsion, this is unreliable for diagnosis.
•Factors predictive of testicular torsion include:
–Acute onset of testicular pain
–Duration of pain of less than 6 h
–Fever, nausea and vomiting
–History of trauma or physical activities