1. Genital Anomalies H. Salimi M.D. Hasheminejad Kidney Center
Iran Medical University of Science

2. Normal Anatomy

3. Phimosis

4. Paraphimosis

6. Complication of Circumcision
Bleeding
Edema
Infection
Meatal stenosis
Adhesions
Penis loss

7. Meatus stenosis

8. Hypospadias Ventral mallocation of meatus Ventral chordee
No prepuce at ventral side

10. Embryology
At the ninth week of gestation, the external genitalia begin to differentiate between the two sexes.
In the male fetus, genetic programming results in androgenic stimulation that induces posterior fusion of the genital folds and growth of the genital tubercle into a phallic structure . The penile urethra forms as a result of remolding and fusion of the medial edges of the endodermal urethral folds .
By 12 weeks gestation, the coronal sulcus separates the glans from the shaft of the penis.
By 16 to 18 weeks, urethral formation is complete, and the foreskin covers the glans.

13. Pathogenesis
Disruption of the androgenic stimulation that is required for the development of the normal male external genitalia is the proposed mechanism resulting in hypospadias
Genetic factor
Exposure to estrogenic compounds (eg, maternal progestin or diethylstilbestrol) during pregnancy
In most cases of hypospadias, however, the cause is unknown. Both genetic and environmental factors are associated with hypospadias, suggesting that the etiology is multifactorial [These include the following:
Advanced maternal age
Pre-existing maternal diabetes mellitus
Gestational age before 37 weeks
History of paternal hypospadias
Exposure to smoking and pesticides

14. Epidemiology
 Hypospadias is one of the most common congenital anomalies with an incidence that varies from 0.3 to 0.7 percent in live male births.
The risk of hypospadias appears to be increasing based upon population surveys throughout the world.

15. Classifaication
mild – Urethral opening within the glans or subcorona : 50%
moderate– Urethral opening on the penile shaft : 30%
severe)– Urethral opening within the scrotum or perineum: 20%

16. Evaluation
Initial evaluation — Once hypospadias has been identified, the initial evaluation includes:
a focused history
examination of the genitalia, and
identification of other congenital anomalies

17. Evalation for DSD
Cryptorchidism and hypospadias 
Proximal hypospadias without cryptorchidism
Pelvic ultrasound to evaluate internal genitalia
Karyotype
Serum electrolytes as a screen for salt-wasting forms of congenital adrenal hyperplasia (CAH), one of the causes of DSD.

19. Surgery
Timing 
 The American Academy of Pediatrics recommends that genital surgery be performed before 18 months of age based upon the premise that gender identity is not defined until 18 months of age
General consensus within the pediatric urology community is to perform surgery at six months of age in full term healthy infants

21. Epispadias
Isolated

22. Associated with
Bladder Extrophy

23. Cryptorchidism

24. Introduction
Cryptorchidism is the most common genital problem encountered in pediatrics
Untreated cryptorchidism clearly has deleterious effects on the testis over time
89% of untreated males with bilateral cryptorchidism develop azospermia
Lifetime risk of neoplasia 2-3%
4 fold higher than average risk
Despite more than 100 years of research, many aspects of cryptorchidism are not well defined and remain controversial

25. Definitions
Cryptorchid: testis neither resides nor can be manipulated into the scrotum
Ectopic: aberrant course
Retractile: can be manipulated into scrotum where it remains without tension

26. Nonpalpable testes occur in approximately 20-30% of those who have cryptorchidism
Abdominal=10%; inguinal=68%; prescrotal= 24%; ectopic= 11.5%;
bilateral, 30%; and unilateral testis, 70%.

27. Epidemiology & Risk Factors
Frequency 3.4 % in term boys , By 1 yo, incidence 0.8%.
Risk factors:
IUGR, prematurity
Incidence in premies 30%
First-or second-born
Perinatal asphyxia
C-section
Toxemia of pregnancy
Congenital subluxation of hip
Seasonal (especially winter)

28. Radiology
Radiologic studies to localize the testis are currently of very little value.
To date, examination by a pediatric urologist has proven to be more valuable than ultrasound, CT scan, or MRA findings.
Ultrasound of abdomen and pelvis along with genitography should be used when the diagnosis of intersex is considered.

30. Laparoscopy if the testis is not palpable

32. Treatment Hormonal (HCG, GnRH) Surgery
Timing: 6 month in normal term boy

33. Hernia , Hydrocele