- •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
538 |
24 Undescended Testes (Cryptorchidism) |
|
|
•Associated anomalies:
–Patent processus vaginalis
–Abnormal epididymis: Epididymal abnormalities are seen in 32–79 % of children with undescended testis
–Cerebral palsy
–Mental retardation
–Wilms tumor
–Abdominal wall defects (e.g. gastroschisis, omphalocele, prune belly syndrome)
–Hypospadias
•It is important to distinguish between a true undescended testis and a retractile one. In retractile testes, the scrotum is usually developed and the testes can be brought down to the bottom of the scrotum. Sometimes, the scrotum is not well developed in those with retractile testes that are outside the scrotum most of the time.
•Retractile testes are more common than truly undescended testes and do not need to be operated on.
•In those with non-palpable testes, pelvic ultrasound or magnetic resonance imaging is performed to locate the testes.
•The presence of a uterus by pelvic ultrasound suggests either persistent Müllerian duct syndrome (AMH deficiency or insensitivity) or a severely virilized genetic female with congenital adrenal hyperplasia.
•A karyotype can confirm or exclude forms of dysgenetic primary hypogonadism, such as Klinefelter syndrome or mixed gonadal dysgenesis.
•Hormone levels (especially gonadotropins and AMH) can help confirm that there are hormonally functional testes.
•An unambiguous micropenis, especially accompanied by hypoglycemia or jaundice, suggests congenital hypopituitarism.
24.8Treatment
•Benefits of orchidopexy:
–Increase the likelihood of fertility
•Infertility is associated with cryptorchidism, and the risk of infertility increases with the degree of maldescent.
•Moreover, approximately 23–86 % of maldescended testes have been associated with some form of epididymal abnormality. The severity of these epididymal abnormalities is more in those with intra-abdominal testis.
–Males with undescended testis are 40 times as likely to develop testicular cancer as males without undescended testis.
–Ten percent of testicular cancer cases involve patients with undescended testis.
•Recent studies have shown that prepubertal orchiopexy reduces this risk.
•Clearly, the ability for patients to perform testicular self-examination with the testes in the scrotum is a benefit of surgery.
•The location of the undescended testis affects the relative risk of testicular cancer. Up to 50 % of malignant testicular tumors associated with cryptorchidism involve intra-abdominal testes.
•Seminoma is the most common malignant tumor type associated with cryptorchidism.
–Correction of associated hernia: A patent processus vaginalis is found in more than 90 % of patients with undescended testis.
–Prevention of testicular torsion
–Prevention of traumatic injury to the testis against pubic bone
–Preventing of psychological effects of an empty scrotum
•The timing of orchidopexy is still not well established.
•It was shown that an undescended testes is likely to descend within the first 4–6 months, so an undescended testes that does not descend by this time is unlikely to descend and needs to be operated on.
•The treatment of undescended testes can be:
–Hormonal
–Surgical
–A combination of the two
•Hormonal treatment:
–This is based on the fact that testicular descent is hormonally mediated and so it can be induced with hormone administration.
24.8 |
Treatment |
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539 |
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– |
The use of hormonal therapy however is |
– Side effects of hCG include: |
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still controversial. |
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• |
Increased penile size |
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– |
There are those who advocate using hor- |
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• Growth of pubic hair |
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monal therapy even for palpable unde- |
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• Increase in scrotal rugae |
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scended testes. Hormonal therapy is |
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• |
Pigmentation |
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sometimes attempted and occasionally |
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• |
Erection |
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successful. |
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• |
Increased testicular size |
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– |
Hormonal therapy has been employed in |
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• Hyperactivity and aggressive behavior |
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Europe for many years as a primary ther- |
• Surgical treatment: |
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apy for cryptorchidism; the main hormones |
– The treatment of undescended testes is sur- |
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used are human chorionic gonadotropin |
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gical orchidopexy. |
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(hCG) and luteinizing hormone (LH)- |
– |
At present, surgical orchidopexy is to be |
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releasing hormone (LHRH). |
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done at approximately 1 year of age. |
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– |
The most commonly used hormone ther- |
– |
This recommendation is based on several |
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apy is human chorionic gonadotropin |
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factors: |
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(HCG). |
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• Spontaneous testicular descent is |
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– |
hCG, which is administered intramuscu- |
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unlikely after this age |
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larly is the main hormonal treatment for |
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• |
Histological abnormalities are subse- |
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undescended testes. |
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quently more likely |
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– |
There are many protocols for the use of |
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• Orchidopexy also allows early detection |
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hCG. |
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of any testicular cancer, reduced risk of |
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• One such protocol is the administration |
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trauma and torsion, and improvement in |
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of 1,500–2,500 U two times per week |
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germ cell function and ultimate progno- |
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for 4 weeks. |
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sis of fertility |
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• Others use smaller dosage depending on |
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• |
Treatment of associated hernia when |
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the age of the patient. |
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present |
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– |
<1 year old: 500 units are given |
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• Surgical orchidopexy avoids several |
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two times per week for five to ten |
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hormonal injections and their side |
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doses. |
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effects. |
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– |
1–2 year old: 1,000 units are given |
– In those with palpable undescended testes, |
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two times per week for five to ten |
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open orchiopexy (inguinal orchidopexy) is |
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doses. |
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the treatment of choice. |
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– |
>2 year old: 1,500 units are given |
– |
Others use a scrotal approach to mobilize |
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two times per week for five to ten |
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and fix palpable undescended testes. |
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doses. |
– |
This is often performed as an outpatient |
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– |
The reported success rates of hCG hor- |
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procedure. |
||
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monal therapy is variable ranging from 5 % |
– |
Several surgical approaches to the unde- |
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to as high as 50 %. |
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scended testis have been described. |
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– Others use GnRH analogs such as nafarelin |
– The approach chosen is determined by the |
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or buserelin. |
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position of the testis and the surgeon's |
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– |
Gonadotropin-releasing hormone (GnRH) |
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expertise. |
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is suggested to be more effective than hCG |
– The palpable testis can be approached via |
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in achieving testicular descent. This how- |
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one of the following approaches: |
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ever is not widely used because of conflict- |
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• |
A scrotal |
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ing results. |
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• |
A subinguinal |
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– Many surgeons do not consider the success |
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• An inguinal (Fig. 24.19) |
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rates of hormonal therapy high enough to |
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• |
A suprainguinal approach |
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|
be worth the trouble since the surgery itself |
– Sometimes exploration may reveal a small |
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|
is usually simple and uncomplicated. |
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atrophic testis with a vas and vessels. This |
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540 |
24 Undescended Testes (Cryptorchidism) |
|
|
suggests an intrauterine torsion. The contralateral testis needs to be fixed to prevent subsequent torsion (Fig. 24.20).
–Rarely, exploration for an undescended testis may reveal features of persistent Mullerian duct syndrome (Fig. 24.21).
–The nonpalpable testis can be approached via one of the following approaches:
•An inguinal
•A suprainguinal
•A laparoscopic
–In patients with intraabdominal undescended testis, laparoscopic assisted orchidopexy is performed.
–Diagnostic laparoscopy is the most reliable technique for localizing the nonpalpable testis.
–It is performed in conjunction with definitive therapy (laparoscopic orchiopexy or open orchiopexy).
–Laparoscopic findings can be helpful in determining the need for inguinal exploration, for deciding between one-stage and two-stage repair, and for assessing gonadal viability.
–Blind-ending vas and vessels confirm the diagnosis of a vanishing testis and do not warrant further therapy.
Fig. 24.19 Intraoperative photograph showing an inguinal approach to undescended testis. Note the normal looking testis
Fig. 24.20 Clinical intraoperative photograph showing a small atrophic testis. Note the intact vas and vessels
Fig. 24.21 A clinical intraoperative photograph of a patient with bilateral undescended testes. On exploration, he was found to have features of persistent Mullerian duct syndrome with uterus, and fallopian tubes
TESTIS
UTERUS
TESTIS
FALLOPIAN
TUBE
FALLOPIAN TUBE