- •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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19 Posterior Urethral Valve |
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Further Reading
1. Ansari MS, Gulia A, Srivastava A, Kapoor R. Risk factors for progression to end-stage renal disease in children with posterior urethral valves. J Pediatr Urol. 2010;6(3):261–4.
2. Bhadoo D, Bajpai M, Panda SS. Posterior urethral valve: prognostic factors and renal outcome. J Indian Assoc Pediatr Surg. 2014;19(3):133–7.
3. Capitanucci ML, Marciano A, Zaccara A, La Sala E, Mosiello G, De Gennaro M. Long-term bladder function followup in boys with posterior urethral valves: comparison of noninvasive vs invasive urodynamic studies. J Urol. 2012;188:953–7.
4. Casey JT, Hagerty JA, Maizels M, et al. Early administration of oxybutynin improves bladder function and clinical outcome in newborns with posterior urethral valves. J Urol. 2012;188:1516–20.
5. Clark TJ, Martin WL, Divakaran TG, Whittle MJ, et al. Prenatal bladder drainage in the management of fetal lower urinary tract obstruction: a systematic review and meta-analysis. Obstet Gynecol. 2003; 102(2):367–82.
6. Cuckow PM, Dineen MD, Risdon RA, Ransley PG, et al. Longterm renal function in posterior urethral valves, unilateral reflux and renal dysplasia syndrome. J Urol. 1997;158(3 pt 2):1004–7.
7. De Gennaro M, Mosiello G, Capitanucci ML, Silveri M, et al. Early detection of bladder dysfunction following posterior urethral valves ablation. Eur J Pediatr Surg. 1996;6:163–5.
8. DeFoor W, Clark C, Jackson E, Reddy P, et al. Risk factors for end stage renal disease in children with posterior urethral valves. J Urol. 2008;180: 1705–8.
9.DeFoor W, Tackett L, Minevich E, McEnery P, Kitchens D, Reeves D, et al. Successful renal transplantation in children with posterior urethral valves. J Urol. 2003;170(6 Pt 1):2402–4.
10. Dewan PA, Zappala SM, Ransley PG, Duffy PG. Endoscopic reappraisal of the morphology of congenital obstruction of the posterior urethra. J Urol. 1992;70:439–44.
11. Ghanem MA, Wolffenbuttel KP, de Vylder A, Nijman RJM. Long-term bladder dysfunction and renal function in boys with posterior urethral valves based on urodynamic findings. J Urol. 2004;171:2409–12.
12. Godbole P, Wade A, Mushtaq I, Wilcox D. Vesicostomy vs. Primary valve ablation of posterior urethral valves: always a difference in outcome? J Pediatr Urol. 2007;3:273–5.
13.Heikkila J, Holmberg C, Kyllonen L, Rintala R, et al. Long term risk of end stage renal disease in patients with posterior urethral valves. J Urol. 2011;186:2392–6.
14. Holmdahl G, Sillen U, Hanson E, Hermansson G, et al. Bladder dysfunction in boys with posterior urethral valves before and after puberty. J Urol. 1996; 155:694–8.
15. Hoover DL, Duckett JJ. Posterior urethral valves, unilateral reflux and renal dysplasia: a syndrome. J Urol. 1982;128(5):994–7.
16. Kitagawa H, Pringle KC, Koike J, Zuccullo J, et al. Vesicoamniotic shunt for complete urinary tract obstruction is partially effective. J Pediatr Surg. 2006;41(2):394–402.
17. Kousidis G, Thomas DFM, Morgan H, Haider N, et al. The long-term outcome of prenatally detected posterior urethral valves: 10 to 23 year follow-up study. BJU Int. 2008;102:1020–4.
18. Lopez Pereira P, Espinosa L, Martinez Urrutina MJ, Lobato R, et al. Posterior urethral valves: prognostic factors. BJU Int. 2003;91:687–90.
19. Nakamura S, Kawai S, Kubo T, Kihara T, Mori K, Nakai H. Transurethral incision of congenital obstructive lesions in the posterior urethra in boys and its effect on urinary incontinence and urodynamic study. BJU Int. 2011;107:1304–11.
20.Nanda M, Bawa M, Narasimhan KL. Minivesicostomy in the management of PUV after valve ablation. J Pediatr Urol. 2012;8:51–4.
21.Narasimhan KL, Mahajan JK, Kaur B, Mittal BR, Bhattacharya A. The vesicoureteral reflux dysplasia syndrome in patients with posterior urethral valves.
J Urol. 2005;174(4 Pt 1):1433–5.
22. Riley P, Marks SD, Desai D, Mushtaq I, et al. Challenges facing renal transplantation in pediatric patients with lower urinary tract dysfunction. Transplantation. 2010;89(11):1299–307.
23.Sarhan O, Zaccaria I, Macher M, Muller F, et al. Longterm outcome of prenatally detected posterior urethral valves: a single centre study of 65 cases managed by primary valve ablation. J Urol. 2008;179(1):307–12.
24.Soliman SM. Primary ablation of posterior urethral valves in low birth weight neonates by a visually guided Fogarty embolectomy catheter. J Urol.
2009;181(5):2284–9. discussion 2289–90.
25. Tikkinen KA, Heikkilä J, Rintala RJ, Tammela TL, Taskinen S. Lower urinary tract symptoms in adults treated for posterior urethral valves in childhood: matched cohort study. J Urol. 2011;186(2):660–6.
26.Young HH, Fronz WA, Baldwin JC. Congenital obstruction of the posterior urthera. J Urol. 1919;3:289.
Utricular Cyst (Prostatic Utricular |
20 |
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20.1Introduction
•The prostatic utricle is a small, epitheliumlined diverticulum of the prostatic urethra.
•Utricle is derived from the Latin word “pouch,” which forms a cul-de-sac. The prostatic utricle (pouch of the prostate) is a small diverticulum (6 mm long) in the prostatic urethra (Fig. 20.1).
•It is located in the verumontanum (seminal colliculus) between the two openings of the ejaculatory ducts and extends backward and slightly upward for a very short distance within the medial lobe of the prostate.
•It is also known as the vagina masculina or vesicula prostatica.
•It is a normal anatomic variant representing the remnant of the fused caudal ends of the Müllerian ducts. This origin is occasionally disputed.
•It is considered to represent the male homologue of the female uterus and vagina.
•In 1905, Robert William Taylor stated the function of the prostatic utricle thusly: “In coitus it so contracts that it draws upon the openings of the ejaculatory ducts, and thus renders them so patulous that the semen readily passes through.”
•Utricle cysts always arise from the level of the verumontanum and are always in the midline.
•The cyst typically lies between the bladder and the rectum and, thus, is palpable on per rectal exam in 50 % of the cases.
•They are variable in size but are usually small in size (commonly <10 mm). They can however grow to reach a large size.
•Morphologically, prostatic utricle cyst appears as a small, single, smooth, unilocular cyst of variable size.
•The cyst lining can be:
–Cuboidal
–Columnar
–Squamous or transitional type
URINARY |
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UTRICULAR CYST |
BLADDER |
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Fig. 20.1 A micturating cystogram showing a large utricular cyst
© Springer International Publishing Switzerland 2017 |
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A.H. Al-Salem, An Illustrated Guide to Pediatric Urology, DOI 10.1007/978-3-319-44182-5_20 |
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20 Utricular Cyst (Prostatic Utricular Cyst) |
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Figs. 20.2 and 20.3 Clinical photographs showing severe hypospadias. This is known to be associated with utricular cyst. The increasing severity of the hypospadias correlates with increasing size of the utricle
Fig. 20.4 A clinical photograph showing Prune belly syndrome which is known to be associated with utricular cyst
•Prostatic utricle cyst is usually seen during the first to second decades of life, with a mean age range of 26 years.
•There is an association between utricular cysts and:
–Renal agenesis/dysgenesis (10–25 %)
–Hypospadias (25 %) (Figs. 20.2 and 20.3)
–Prune-belly syndrome (Fig. 20.4)
–Anorectal agenesis
–Down’s syndrome
•Hypospadias is considered the most commonly associated malformation with the prostatic utricle (14–47 %), and the increasing severity of the hypospadias correlates with increasing size of the utricle (Figs. 20.2 and 20.3).
20.3 Classification of Utricular Cysts |
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•Normally, the prostatic utricle distends with urine during voiding and then passively drains.
•Poor emptying leads to urine retention and stasis and this leads to complications including:
–Recurrent urinary tract infection
–Hematuria
–Urethral discharge
–Recurrent epididymitis
–Voiding dysfunction
–Urine retention
–Post voiding urine drippling
–Stone formation within the utricle cyst
–Malignant transformation (e.g. clear cell carcinoma, or squamous cell carcinoma) with a reported prevalence as high as 3 %.
•Utricular cyst is rare but along with its rarity, it presents a challenge in its diagnosis and proper management. Awareness of this is important.
•Persistence or untreated prostatic cyst could be a cause of infertility.
•The differential diagnoses include mullerian duct cysts, bladder diverticulum, cystic teratoma, seminal vesicle cyst, epididymal cyst and Wolffian duct cyst.
20.2Embryology
•Embryologically, in the male fetus the Mullerian ducts regress under the influence of anti-Mullerian hormone (Mullerian inhibiting substance).
•This is a glycoprotein secreted by the Sertoli cells of the fetal testes at eight gestational weeks.
•Persistence of the Mullerian ducts as result of failure of synthesis or action of Mullerian inhibitory substance results in persistent Mullerian duct syndrome.
•This is characterized by the presence of uterine tissue and fallopian tubes in a phenotypic and genotypic male.
•Utricular cysts are thought to result from incomplete regression of the Mullerian ducts or incomplete androgen-mediated closure of
the urogenital sinus caused by an error in the production or sensitivity to local testosterone or anti-Mullerian hormone.
•These cysts are differentiated anatomically from Mullerian duct cysts.
–Utricular cysts:
•Always in the midline
•They present during the first to second decade of life
•They communicate with the urethra
•They have a tubular or vesicular shape
•The majority are seen in younger patients
•They have an association with unilateral/bilateral renal agenesis, and hypospadias
•They can be visualized with a micturating cystourethrogram or a retrograde urethrogram.
–Mullerian duct cysts:
•Usually seen above the prostate
•They are seen in the older age group ranging from 2 to 75 years
•Mullerian duct cysts generally do not communicate with the urethra
•They cannot be visualized with a micturating cystourethrogram or a retrograde urethrogram.
•It is proposed that these cysts originate due to failure of fusion of Mullerian duct resulting from deficient Mullerian inhibitory factor.
•They are associated with intersex conditions
•They are also seen in those with normal external genitalia
•Based on this, it has been postulated that the Mullerian duct cysts are the remnants of the paramesonephric ducts rather than the Mullerian ducts.
20.3Classification of Utricular Cysts
•Ikoma et al. in 1985 classified utricular cysts into four types depending on the size of the cyst and the site of communication with the urethra.