- •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
Further Reading |
617 |
|
|
ACUTE SCROTUM
HISTORY AND PHYSICAL
EXAMINATION
SHORT DURATION OF |
LONG DURATION OF |
|
SYMPTOMS AND HIGHLY |
SYMPTOMS AND NOT |
|
SUGGESTIVE OF |
HIGHLY SUGGESTIVE OF |
|
TESTICULAR TORSION |
TESTICULAR TORSION |
|
EMERGENCY |
URINE ANALYSIS AND COLOR |
|
DOPPLER ULTRASONOGRAPHY |
||
SURGICAL |
||
|
||
EXPLORATION |
|
|
|
INCREASED OR NORMAL |
|
DECREASED OR ABSENT |
BLOOD FLOW AND |
|
BLOOD FLOW OR |
POSITIVE URINE |
EQUIVOCAL RESULT |
|
|
INCREASED OR NORMAL BLOOD |
TREAT AS |
|
EPIDIDMO-ORCHITIS |
||
FLOW AND NEGATIVE URINE |
||
|
NON-OPERATIVE MANAGEMENT
OR OBSERVATION
Further Reading
1.Boettcher M, Bergholz R, Krebs TF, Wenke K, Aronson DC. Clinical predictors of testicular torsion in children. Urology. 2012;79(3):670–4.
2. Dajusta DG, Granberg CF, Villanueva C, Baker LA. Contemporary review of testicular torsion: new concepts, emerging technologies and potential therapeutics. J Pediatr Urol. 2013;9(6 Pt A):723–30.
3. Karmazyn B, Steinberg R, Kornreich L. Clinical and sonographic criteria of acute scrotum in children: a
retrospective study of 172 boys. Pediatr Radiol. 2005;35(3):302–10.
4.Kass EJ, Lundak B. The acute scrotum. Pediatr Clin North Am. 1997;44:1251.
5.Schalamon J, Ainoedhofer H, Schleef J, et al. Management of acute scrotum in children – the impact of Doppler ultrasound. J Pediatr Surg. 2006;41:1377.
6. Yang Jr C, Song B, Liu X, Wei GH, Lin T, He DW. Acute scrotum in children: an 18-year retrospective study. Pediatr Emerg Care. 2011;27(4): 270–4.
Hydrocolpos, Vaginal Agenesis |
30 |
and Atresia |
30.1Introduction
•Hydrocolpos is the distension of the vagina caused by accumulation of fluid due to congenital vaginal obstruction.
•The obstruction is often caused by an imperforate hymen or less commonly a transverse vaginal septum.
•The fluid consists of cervical and endometrial mucus.
•In rare instances the hydrocolpos is secondary to urine accumulated through a vesicovaginal fistula proximal to the obstruction or due to the presence of urogenital sinus with collection of urine.
•The term hydrometrocolpos is when hydrocolpos is associated with fluid accumulation in the uterus.
•Vaginal atresia is a congenital abnormality of the female genital system.
•It represent a spectrum of malformations ranging from total vaginal agenesis to vaginal atresia.
opening which completely obstructs the vagina (Fig. 30.1).
•It is caused by a failure of the hymen to perforate during fetal development.
•It is most often diagnosed in adolescent girls when menstrual blood accumulates in the vagina (hematocolpos) and sometimes also in the uterus.
•In adolescent females:
–The most common symptoms of an imperforate hymen are cyclic pelvic pain and amenorrhea.
–The cyclic pain is secondary to hematocolpos.
–Other symptoms include urinary retention, constipation, back pain, nausea, and diarrhea.
30.2Imperforate Hymen
•Imperforate hymen is the most common and most distal form of vaginal outflow obstruction.
•An imperforate hymen is a congenital malformation characterized by a hymen without an
Fig. 30.1 A clinical photograph showing a newborn with imperforate hymen. Note the bulging hymenal membrane
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30 Hydrocolpos, Vaginal Agenesis and Atresia |
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Figs. 30.2 and 30.3 Clinical photographs showing a newborn with imperforate hymen. Note the whitish bulging hymenal membrane
•The hymen originates from the embryonic vagina buds from the urogenital sinus.
•As a consequence, the hymen is a composite of vaginal epithelium and epithelium of the urogenital sinus interposed by mesoderm.
•Once the hymen becomes perforated or forms a central canal, it establishes a communication between the upper vaginal tract and the vestibule of the vagina.
•Normally, there are anatomic variations of the patent hymen.
–The most common being an annular or circumferential hymen in which the hymen completely surrounds the vaginal orifice and has a central opening.
–Other appearances of the hymen include crescentic, fimbriated, septate, cribriform, and microperforate forms.
•It is proposed that an imperforate hymen is formed during fetal development when the sinovaginal bulbs fail to canalize with the rest of the vagina. This result is a solid membrane interposed between the proximal uterovaginal tract and the introitus.
•The exact cause of imperforate hymen is not known.
•Imperforate hymen may result from failure of apoptosis due to a genetically transmitted signal, or it may be related to an inappropriate hormonal milieu.
•Familial inheritance in successive generations has been described
•Imperforate hymen is the most frequent cause of vaginal outflow obstruction, occurring in 0.1 % of infant girls.
•This vaginal outlet obstruction results in the entrapment of vaginal and uterine secretions under the influence of maternal estrogens and this becomes evident when the distensible membrane bulges between the labia.
•In adolescence, the retained secretions consist of menstrual products, and the resulting mass effect in the vagina and uterus are referred to as hematocolpos and hematometrocolpos, respectively.
•Pyocolpos (infection of a hydrocolpos) may result from an infection that is ascending through microperforations in the hymen membrane.
•Clinical presentations of imperforate hymen in newborns include:
– An incidental finding on routine physical examination of a newborn.
– The neonate with imperforate hymen typically presents with a bulging membrane between the labia.
– The membrane may be white or yellow-grey because it is distended from trapped mucoid material secreted as a result of stimulation by maternal estrogen (Figs. 30.2 and 30.3).
– In severe cases, the distention extends proximally into the uterus (hydrometrocolpos).
– A lower abdominal midline mass may be evident on physical examination because
30.4 Classification |
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Fig. 30.4 A clinical photograph showing labial adhesions (fused labia) not to be confused with imperforate hymen
the shallow pelvis of a neonate allows the uterus to be palpated above the pubis symphysis.
–Hydrocolpos can lead to urinary tract infections or bladder obstruction and acute urinary retention.
•Asymptomatic imperforate hymen should not be confused with labial adhesions (fused labia) (Fig. 30.4)
•The diagnosis is made clinically and can be confirmed by ultrasonography.
•The treatment is hymenotomy in those with evidence of obstruction or urinary symptoms.
•Others advocate observation throughout childhood, with a planned hymenotomy after the onset of puberty.
•If a patient is diagnosed with an asymptomatic imperforate hymen in infancy or childhood
beyond the neonatal period, the optimal time for surgical repair is after the onset of puberty and prior to menarche.
30.3Vaginal Atresia
•This is a birth defect where the vagina is blocked off to varying degrees.
•It is often associated with syndromes such as:
–Bardet-Biedl syndrome
–Fraser syndrome
–Mayer-Rokitansky-Küstner-Hauser (MRKH) syndrome
•Mayer-Rokitansky-Küstner-Hauser (MRKH) syndrome: This syndrome is characterized by the followings:
–Absent uterus
–A deformed or absent vagina
–Normal ovaries
–Normal external genitalia.
•Vaginal atresia is estimated to occur in 1 in 5,000–10,000 live female births.
•Vaginal atresia is a congenital developmental defect resulting in uterovaginal outflow obstruction which can present either:
–In the neonatal period with hydrocolpos as a result of accumulation of secretions from the normal cervical glands under the influence of maternal hormones. Extension of fluid accumulation to involve the uterus will result in hydrometrocolpos.
–Hydrocolpos can be complicated by infection leading to pyocolpos.
–In adolescence with hematocolpos as a result of obstruction to the normal menstrual flow or with primary amenorrhea. Vaginal atresia is reported to be the second most common cause of primary amenorrhea.
30.4Classification
•Vaginal atresia is classified anatomically into three types:
– Vaginal agenesis