- •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
342 |
13 Bladder Exstrophy-Epispadias Complex |
|
|
is incomplete separation of the urinary tract, genital tract and hindgut.
•The exact embryological defect is not known and several theories have been proposed to explain this.
•Marshall and Muecke theory is based on an abnormal lower overdevelopment of the cloacal membrane, which prevents the medial migration of the mesenchymal tissue which impairs the proper development of the abdominal wall.
•Ambrose and O’Brian postulated that an abnormal development of the genital hillocks with fusion in the midline below rather than above the cloacal membrane result in the exstrophy defect.
•Another hypothesis describes an abnormal caudal insertion of the body stalk with failure of the interposition of the mesenchymal tissue in the midline. As a consequence of this failure, translocation of the cloaca into the depths of the abdominal cavity does not occur. A cloacal membrane that remains in a superficial infraumbilical position represents an unstable embryonic state with a strong tendency to disintegrate.
13.3Epispadias
13.3.1 Introduction
•Epispadias, bladder extrophy and cloacal extrophy are major embryological defects that are known to be associated with long term morbidity. With the recent advances in surgical techniques including bladder closure, pelvic osteotomies, and traction and immobilization the majority of these patients can achieve full continence, adequate sexual function, and improvement in quality of life.
•In males, the external urethral opening (external meatus) is normally located at the tip of the penis.
•In females, the external urethral opening is normally located between the clitoris and the vagina.
•Epispadias is the least severe form of Bladder Exstrophy-Epispadias Complex and it is characterized by a dorsally open urethral
meatus with mild pubic diastasis and a closed anterior abdominal wall and bladder.
•Epispadias is a very rare congenital malformation of the male or female urethra, in which the urethra opens dorsally.
•In females with epispadias, there is a fissure in the upper wall of the urethra and out of the body through an opening above the clitoris or it can present as a double clitoris.
•In males with epispadias, the urethra opens on the superior surface of the penis. The extent of this opening is variable. It can involve only the glans or extend to involve the whole shaft of penis.
•This differentiates it from hypospadias which is a congenital defect, in which the urethra opens on the ventral surface of the penis.
•Epispadias is very rare, with an estimated incidence of approximately 1 in 100,000 live male births.
•It is extremely rare in females.
•Complete epispadias is a rare congenital malformation.
–It occurs in 1 in every 117,000 male births
–It occurs in only 1 of every 484,000 female births
•Epispadias rarely occur as an isolated defect.
•It is commonly seen as part of the exstrophyepispadias complex.
•Diastasis of the pubic bone and external displacement of the hips is seen in severe cases of epispadias.
•In males, epispadias is characterized by (Figs. 13.7, 13.8, 13.9, and 13.10):
–The urethra opens on the dorsum of the penis. This can affect only the glans or extend to affect the whole urethra.
–The normal urethra is replaced by a broad mucosal strip lying on the dorsum of the corpora cavernosa.
–A short phallus.
–A penis that is typically broad.
–Dorsal chordee (marked upward curvature of the penis).
•Patients with isolated epispadias have a low incidence of associated abnormalities but those with the more severe form of exstrophyepispadias complex are at a slightly increased risk for associated malformations
13.3 Epispadias |
343 |
|
|
Figs. 13.7, 13.8, and 13.9 Clinical photographs showing epispadias. The first is a glanular type of epispadias while the lower two show complete epispadias involving
the whole urethra. Note the urethral opening on the dorsum of the penis
– These patients are also at increased risk for retrograde ejaculation when they reach adolescence because of failure of bladder neck to close completely.
|
|
13.3.2 Etiology |
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|
|
• The penis is formed by the corpus spongiosum |
|
|
|
surrounding the urethra and by two corpora |
|
Fig. 13.10 A clinical photograph showing epispadias. |
cavernosa. |
||
• These are composed of erectile tissue sur- |
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Note the short phallus which is also broad. Note also the |
|||
upward chordee of the penis and the urethral opening on |
rounded by the tunica albuginea (Buck fascia) |
||
the dorsum of the penis |
and the dartos fascia more superficially. |
||
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|
||
|
|
• Normally, the male urethra runs through the |
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|
|
penile shaft, ventrally to the corpora cavernosa, |
|
• These include: |
and meets with the meatus at the tip of the |
||
– |
Hydroureters |
glans. |
|
– |
Hydronephrosis |
• The exact etiology of eispadias is not |
|
– |
Vesicoureteral reflux. |
known. |
|
344 |
13 Bladder Exstrophy-Epispadias Complex |
|
|
•Epispadias and exstrophy of the bladder are considered varying degrees of the same disorder.
•Epispadias results from defective migration of the paired primordia of the genital tubercle.
•These fuse on the midline to form the genital tubercle at the fifth week of embryologic development.
13.3.3 Classification
•The extent of epispadias is variable.
•It can present as a small dimple on the tip of the glans penis just above the normal urethral opening (Figs. 13.11 and 13.12).
•If the urethra and bladder are involved, the epispadias is severe and this is part of the spectrum of malformations called the exstrophy-epispadias complex (Fig. 13.13).
•Commonly, the classification of epispadias is based on the location of the urethral meatus as follows:
–Glandular epispadias (Figs. 13.11, 13.12, and 13.14):
•The extent of epispadias is limited to the glans of the penis.
•This is the rarest type.
–Penile epispadias (Figs. 13.15 and 13.16):
•The epispadias opening extends along the whole shaft of the penis.
–Penopubic epispadias (complete):
•The epispadias opening extends to the pubic bone.
–Part of the exstrophy-epispadias complex (Figs. 13.17 and 13.18).
•Patients with glanular and about one-third of those penile epispadias usually have good
BLADDER
EXTROPHY
COMPLETE
EPISPADIAS
Fig. 13.13 A clinical photograph showing complete epispadias as part of the extrophy-epispadias complex. Note the dorsal chordee
Figs. 13.11 and 13.12 Clinical photographs showing glandular epispadias. Note the urethral opening on the dorsum of the penis. It appear as a dimple on the dorsum of the glans
Fig. 13.14 A clinical photograph showing glandular epispadias. Note the urethra opening on the dorsum of the penis and limited to the glans of the penis
13.3 Epispadias |
345 |
|
|
prognosis with normal urinary capacity and no urine incontinence.
•In most cases of penopubic epispadias, and approximately two-thirds of those with penile epispadias have urine incontinence.
•These patients and those with exstrophyepispadias complex will require reconstructive bladder neck surgery to achieve urine continence.
•Currently, about 80 % of patients with male epispadias patients are continent postoperatively.
•The remaining patients who are still incontinent may require later bladder neck reconstruction.
•It is also estimated that about 87–100 % of isolated female epispadias are continent postoperatively.
13.3.4 Treatment
Figs. 13.15 and 13.16 Clinical photographs showing penile epispadias. Note the dorsal chordee and the urethral opening on the dorsum of the penis and extending along the shaft of the penis
•The goals of treatment of epispadias are:
–To lengthen and straighten the penis by correcting dorsal bend and chordee.
–To create a functionally and cosmetically acceptable penis with an external urethral meatus at the tip of the penis.
–To establish urinary continence and preserve fertility in those with the more severe forms.
•Patients with epispadias are born with a very small or severely underdeveloped penis. These patients are usually treated with long acting testosterone preoperatively to enlarge the size of the penis (Figs. 13.19, 13.20, and 13.21).
Figs. 13.17 and 13.18 Clinical photographs showing epispadias as part of the bladder extrophy. Note the scars after closure of the urinary bladder
346 |
13 Bladder Exstrophy-Epispadias Complex |
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|
Figs. 13.19, 13.20, and 13.21 Clinical photographs showing epispadias before testosterone, after testosterone and at the time of surgery. Note the increase in the size of the penis following testosterone injections
•They are given one or a maximum of three doses at 3–4 weeks intervals (2 mg/kg/dose). Others use topical testosterone.
•The surgical treatment of epispadias differs according to the complexity of the malformation.
•A staged approach has often been used for the management of the exstrophy-epispadias complex.
•Currently, epispadias are treated with single stage procedures.
•There are three surgical techniques used to correct epispadias:
–The Cantwell-Ransley procedure.
–The Young procedure.
–The complete disassembly procedure.
•In this technique the two corpora cavernosa and a single corpus spongiosum are totally separated and the three components are reassembled and the urethra constructed in such a way that the urethra is in its normal position.
13.3 Epispadias |
347 |
|
|
– A large number of children with the |
– Glanular and urethral reconstruction: |
exstrophy-epispadias complex will subse- |
• The urethral meatus is correctly posi- |
quently require bladder neck repair to |
tioned using a reversed meatal advance- |
achieve continence. |
ment and glanuloplasty technique. |
|
– Closure of penile skin |
|
• The modern modified Cantwell-Ransley |
13.3.5 Surgical Repair of Male |
repair is the most commonly used technique to |
Epispadias (Figs. 13.22, 13.23, |
repair epispadias. |
13.24, 13.25, 13.26, 13.27, |
• Mitchell and Bagli have described a further |
13.28, 13.29, 13.30, 13.31, |
modification of Cantwell-Ransley repair |
13.32, 13.33, 13.34, 13.35, |
“complete penile disassembly technique”. |
and 13.36) |
– The urethral plate and each corporeal body |
|
along with its hemiglans are dissected |
• Intramuscular or topical testosterone is given |
completely free from each other. |
preoperatively to increase the size of the |
– The urethra is then tubularized and placed |
penis. |
into an anatomic, ventral position. |
• The surgical repair of epispadias include: |
– This method of epispadias repair is often |
– Correction of dorsal chordee: |
performed at the time of primary bladder |
• This is achieved by mobilizing the ure- |
closure, the combination of which is called |
thral plate from the underlying corpora |
“complete primary repair of bladder |
from the level of glans down to the pros- |
exstrophy”. |
tatic urethra. |
– This technique may be complicated by |
• The corporal bodies are anastomosed at |
ischemia as a result damage to the blood |
the dorsal medial aspect over the tubu- |
supply during dissection. |
larized urethra. |
– The lateral dissection during this procedure |
• Persistent chordee is corrected by |
can result in neurovascular bundle injury |
cavernostomy. |
and consequent erectile dysfunction. |
Figs. 13.22 and 13.23 Clinical photographs showing epispadias being repaired. This was part of the exstrophyepispadias complex that was treated in two stages. Note
the broad penis. The length of the penis increased as a result of testosterone injections