- •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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26 Testicular Torsion and Torsion of the Testicular or Epididymal Appendage |
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Fig. 26.1 Diagrammatic representation of the bell-clapper deformity. This allows the testicle to rotate freely on the spermatic cord within the tunica vaginalis and predisposes to intravaginal torsion of testis
TUNICA
VAGINALIS
EPIDIDYMIS
TESTIS
•This calls for early diagnosis and emergency correction of torsion to minimize the risk of testicular infarction.
•The diagnosis of testicular torsion is clinical and if doubt an emergency Doppler ultrasound can be done.
•Testicular torsion commonly develops during puberty but can also be seen in newborns as a result of intrauterine torsion or soon after birth.
•The exact incidence of testicular torsion is not known but it has been estimated to occur in about 1 in 4,000 to 1 in 25,000 males per year before 25 years of age.
•Testicular torsion is most frequent among adolescents with about 65 % of cases presenting between 12 and 18 years of age.
•It has been estimated that in 95 % of men with testicular torsion the testis can be saved if treated within six hours of the onset of pain.
•Other causes of acute scrotal pain that should be ruled out include:
–Orchitis
–Epididymitis, epididymo-orchitis
–Torsion of the testicular or epididymal appendages
–Trauma-related causes of acute scrotum
–Acute hydrocele
–Testicular tumor
–Idiopathic scrotal edema
26.2Etiology and Risk Factors
•The exact etiology of testicular torsion is not known
•The “bell-clapper deformity”
–This is the commonest cause of testicular torsion
–It accounts for 90 % of the cases
–It is a congenital malformation of the processus vaginalis
–In this condition, rather than the testes attaching posteriorly to the inner lining of the scrotum by the mesorchium, the mesorchium terminates early and the testis is free floating in the tunica vaginalis.
–This condition is bilateral and calls for fixation of the other testis when one testis is affected. This is to prevent subsequent torsion of the other side.
•A large mesentery between the epididymis and the testis can also predispose itself to torsion, although this is rare.
•Contraction of the spermatic muscles shortens the spermatic cord and may initiate testicular torsion.
•Other etiologic factors involved in intravaginal testicular torsion include:
–Undescended testicle
–Sexual arousal or activity
–Physical exercise
26.3 Diagnosis |
555 |
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–An active cremasteric reflex
–Cold weather.
•A larger testicle either due to normal variation or a tumor increases the risk of torsion.
26.3Diagnosis
•The diagnosis of testicular torsion is clinical based on the history and presenting signs and symptoms.
•With a convincing history and physical examination, no time should be wasted on investigations and immediate surgical exploration should be done.
•Doppler ultrasound should be done only in low suspicion cases to rule out torsion and differentiate this from epididymo-orchitis. Doppler ultrasonography can be used to demonstrate arterial blood flow to the testis while providing information about other testicular
pathology. It is about 90 % accurate in diag- Figs. 26.2 and 26.3 A Doppler ultrasound showing
nosing testicular torsion.
–In testicular torsion, there is no blood flow or the blood flow is markedly decreased.
–In epididymo-orchitis, there is normal or slightly increased blood flow.
–The sensitivity of color Doppler in detecting acute testicular torsion in children is 90–100 %, with specificity being 100 %.
–Other studies have suggested that color Doppler ultrasonography was only 86 % sensitive, 100 % specific, and 97 % accurate in the diagnosis of testicular torsion (Figs. 26.2 and 26.3).
–Doppler ultrasonography has (Figs. 26.4 and 26.5):
•94 % sensitivity.
•96 % specificity.
•95.5 % accuracy.
•89.4 % positive predictive value.
•98 % negative predictive value.
•In doubtful cases, an isotope scan (techne- tium-99 m pertechnetate) can be done. Radionuclide scans have a sensitivity of
bilateral torsion of testes
90–100 % accuracy in detecting testicular blood flow.
–This is the most accurate investigation but it is not readily available
–Add to this the time it requires to organize and do this investigation particularly in cases of torsion where urgency is required
–In testicular torsion, there is no uptake of the uptake is markedly reduced
–In epididymo-orchitis, there is normal uptake and uniformly symmetric activity.
–A urine analysis and culture can be done to roll out infection.
–The complete blood count can be normal. However, the WBC count is elevated in as many as 60 % of patients who have testicular torsion.
•Surgical exploration should not be delayed for the sake of performing imaging studies.
556 |
26 Testicular Torsion and Torsion of the Testicular or Epididymal Appendage |
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Figs. 26.4 and 26.5
A Doppler ultrasound showing epididymitis. Note the enlarged left epididymis and the good blood flow to the testis
26.4Intermittent Testicular Torsion
•This is a less serious variant of testicular torsion.
•It is a chronic condition characterized by the symptoms of testicular torsion but followed by eventual spontaneous detortion and resolution of pain.
•The attack may be associated with nausea or vomiting
•These patients are however at significant risk of developing complete torsion
•It is important recognize this condition and physicians treating these patients should be aware of this.
•The treatment is elective bilateral orchiopexy.
•This is curative and 97 % of patients who undergo bilateral orchidopexy experience complete relief from their symptoms.
26.5Classification of Testicular Torsion
•Testicular torsion is classified is classified into two types depending on onset:
–Acute testicular torsion
–Intermittent testicular torsion
•Testicular torsion is also classified anatomically into two types:
–Intravaginal torsion
•This is the commonest type
•Commonly develops during puberty
•The torsion occurs within the tunica vaginalis
•Intravaginal torsion comprises approximately 16 % of patients with torsion presenting in emergency departments with acute scrotum.
•Thepeak incidence occurs in adolescents aged 13 years.
•The left testis is more frequently involved.
•Bilateral cases account for 2 % of all testicular torsions.
–Extravaginal torsion (Figs. 26.6, 26.7, 26.8, 26.9, and 26.10)
•This much less common
•Extravaginal torsion comprises approximately 5 % of all testicular torsions.
•This type occurs exclusively in newborns
•The condition is most often a prenatal (in utero testicular torsion) event.
•It is associated with high birth weight.
•Up to 20 % of cases are synchronous, and 3 % are asynchronous bilateral.
26.6 Effects of Testicular Torsion |
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•This type of torsion occurs outside of the tunica vaginalis, when the testis and gubernaculum can rotate freely.
•The torsion usually occurs intrauterine and rarely soon after birth
•It is usually unilateral but can also occur bilaterally.
•The newborns with this type of torsion usually present immediately after birth with scrotal swelling, and dark discoloration of the scrotum
•Clinically, the affected testis is usually firm and painless
•The scrotal skin characteristically fixes to the necrotic gonad.
•The affected testis is usually necrotic
•The treatment is orchidectomy and contralateral orchidopexy to obviate the risk of torsion on the other side
Fig. 26.6 A clinical photograph showing a newborn with intrauterine torsion of testis. Note that the patient is healthy and of good weight
26.6Effects of Testicular Torsion
•Torsion of the testes causes venous occlusion and engorgement as well as arterial obstruction and ischemia and subsequent infarction of the testis. The extent of this depends on two factors:
–The degree of torsion:
•Torsion of testis occurs as the testis and the cord rotate between 90° and 180°, compromising blood flow to and from the testis.
Figs. 26.7, 26.8, and 26.9 Clinical and intraoperative photographs showing intrauterine torsion of testes. Note the discoloration of the affected scrotum which is slightly elevated. Note also the frankly necrotic testis