Hypospadias Epispadias

1. EPISPADIAS
&
HYPOSPADIAS
BY
RAJAH AMINA SULEIMAN
(RN, RM, RNE, RBPN, BNSC, PGDE, MSC)

3. REVIEW OF
ANATOMY &
PHYSIOLOGY

6. HYPOSPADIAS

7. INTRODUCTION
 Hypospadias is one of the most common, complex and diverse urologic anomalies
encountered in children.
 Hypospadias is a birth defect in boys where the opening of the urethra is not located at the
tip of the penis.
 Hypospadias is one of the most common congenital anomalies in men.
 In boys with hypospadias, the urethra forms abnormally during weeks 8–14 of pregnancy.
 The abnormal opening can form anywhere from just below the end of the penis to the
scrotum.

8. DEFINITION
 Hypospadias is an abnormality of anterior urethral and penile development, in
which the urethral opening is ectopically located on the ventral aspect of the
penis proximal to the tip of the glans penis. OR
 Hypospadias is a congenital abnormality of the penis that is characterized by an
abnormal position of the urethral opening. OR
 Hypospadias is a congenital defect causing the urethral meatus to be located at
an abnormal site, usually on the underside of the penis rather than at the tip.

9. INCIDENCE
 Hypospadias is the second most common genital birth defect in boys,
following cryptorchidism.
 With an incidence of 1 in 300 live male births worldwide.

10. ETIOLOGY
 The cause of hypospadias is a mix of monogenic and
multifactorial forms, implicating both genes and
environmental factors.

11. RISK FACTORS
 Age : Mothers who were age 35 years or older had a higher risk of having a
baby with hypospadias
 Weight: Mothers who were considered obese had a higher risk of having a baby
with hypospadias
 Fertility treatments: Women who used assisted reproductive technology to help
with pregnancy had a higher risk of having a baby with hypospadias
 Certain hormones: Women who took certain hormones just before or during
pregnancy were shown to have a higher risk of having a baby with hypospadias

12. CLASSIFICATION

13. FEMALE HYPOSPADIAS
 In contrast to male hypospadias, female hypospadias (FH) or
“hypospadia feminis” is such a rare anomaly of the lower
urogenital system that most textbooks fail even to mention it.
 Isolated Female Hypospadias, with no associated
genitourinary abnormalities, is even rarer.

14. PATHOPHYSIOLOGY
 Hypospadias is a congenital abnormality of the penis characterized by an
abnormal position of the urethral opening that occurs during the embryologic
development of the urethra, between 8 and 20 weeks' gestation.
 Males and females have identical external genital structures until 8 weeks, after
which the genital structures develop a masculine phenotype in males, primarily
under the influence of testosterone and its byproduct, dihydrotestosterone.
 Under the influence of these hormones, the genital tubercle becomes rapidly
elongated, forming the phallus, whereas the urethral folds are pulled by the
phallus to form the lateral walls of the urethral groove.

15. PATHOPHYSIOLOGY
 The urethral folds coalesce in the midline from base to tip, forming a tubularized penile
urethra and median scrotal raphe.
 The endoderm differentiates to ectoderm with subsequent canalization by apoptosis.
 The prepuce normally forms as a ridge of skin from the corona that grows
circumferentially, fusing with the glans.
 Failure of fusion of the urethral folds in hypospadias impedes this process, and a dorsal
hooded prepuce results.
 Ventral curvature of the penis, termed chordee, is often associated with hypospadias,
especially in more severe forms.
 Hypospadias occurs due to arrest of penile development, leading to hypoplasia of the
ventral tissue of the penis.

16. CLINICAL MANIFESTATIONS OF HYPOSPADIAS
There are 3 key features of hypospadias, although not all 3 are required for
the condition:
 1) Ventral opening of the urethral meatus
 2) Ventral curvature of the penis or “Chordee”
 3) Dorsal hooded foreskin
Others Include:
 Abnormal spraying of urine
 Cryptorchidism (undescended testes)

17. DIAGNOSIS
 Hypospadias is usually diagnosed during a physical examination after
the baby is born.

18. SURGICAL MANAGEMENT
 Timing: Surgical intervention for hypospadias can be performed at any
age, however, The American Academy of Pediatrics recommend
operative intervention at 6–18 months, to limit psychological stress and
subsequent behavioural problems seen in toddlers undergoing genital
surgery.
 NOTE: It is recommended that patients with hypospadias should not be
circumcised because of the superiority of the prepuce tissue covering the
urethra.

19. SURGICAL MANAGEMENT
The operative steps of hypospadias surgery involve:
 Penile Degloving
 Correction of Ventral Chordae (orthoplasty or penile straightening)
 Reconstruction of the urethra (urethroplasty)
 Providing a vascularized coverage for the urethroplasty
 Reconstruction of the glans (glansplasty)
 Meatoplasty and Glanuloplasty
 Scrotoplasty
 Cosmetic skin coverage to create a circumcised penile appearance.

20. POST-OPERATIVE COMPLICATIONS
 Urethrocutaneous fistula
 Meatal stenosis
 Urethral stenosis
 Glans dehiscence
 Urethral diverticulum or urethrocele, which can lead to infections and post-void dribbling
 Cosmetic issues: Excess residual skin, skin tags, inclusion cysts, skin bridges, suture tracts
 Hair-bearing urethra
 Recurrent or persistent penile curvature
 Spraying or misdirected urinary stream and/or irritative symptoms
 Erectile dysfunction
 Balanitis xerotica obliterans of the urethra leading to strictures

21. NURSING MANAGEMENT
PRE-OPERATIVE MANAGEMENT
 Admission
 History and physical examination
 Observations: v/signs
 Pre-operative Investigations: FBC, FBS, Hb, PCV, X-rays, HIV, Hep, Bleeding and
clotting time, Coagulation tests etc
 Nutrition: NPO at least 6 hrs
 Physical care
 Psychological care

22. NURSING MANAGEMENT
PRE-OPERATIVE MANAGEMENT
 Informed consent
 Pre-surgical medications: sedatives
 Clinical Photograph
 Counselling

23. NURSING MANAGEMENT
POST-OPERATIVE MANAGEMENT
 Preparation to receive the patient
 Reception
 Pain relief: Analgesia should be prescribed to be taken preemptively,
particularly for the first 72 hours, when the inflammatory phase is
expected to begin abating.
 Psychological care
 Clinical photograph

24. NURSING MANAGEMENT
POST-OPERATIVE MANAGEMENT
 Wound care including drains, bandaging
 Medications
 Fluid intake
 Health education
 Input and Output chart: Notify the surgeon if there is no urine drainage for 1
hour as this may indicate kinks in the system or obstruction by sediment.
 Discharge
 Follow up

25. EPISPADIAS

26. INTRODUCTION
 Epispadias is a rare congenital malformation of the male or female urogenital organs that
consists of a defect of the dorsal wall of the urethra.
 In boys with epispadias, the urethra generally opens on the top or side of the penis rather
than the tip.
 However, it is possible for the urethra to be open along the entire length of the penis. In
girls, the opening is usually between the clitoris and the labia but may be in the abdominal
area.
 Epispadias can be associated with bladder exstrophy.

27. DEFINITION
 Epispadias is a rare urogenital malformation characterized by the failure of
the urethral tube to tubularize on the dorsal aspect.
 Epispadias is an abnormality of the urethra in which the urethral opening is
ectopically located on the dorsal aspect of the penis.

29. INCIDENCE
 Epispadias occurs in
 one in 117,000 newborn boys
 one in 484,000 newborn girls.
 It is commonly seen as a component in the spectrum of bladder exstrophyepispadias-
complex (BEEC).
 Isolated epispadias constitutes less than 10 percent of the total cases of epispadias.
 Isolated male epispadias is rare.

30. ETIOLOGY
 Epispadias can be explained by defective migration of the paired primordia of the genital
tubercle that fuse on the midline to form the genital tubercle at the fifth week of
embryologic development.
 Epispadias is rarely observed in 2 members of the same family.

31. CLASSIFICATION
 Glandular
 Penile
 Complete (ie, penopubic).

32. FEMALE EPISPADIAS
 Isolated female epispadias without bladder exstrophy is an extremely rare congenital
anomaly.
 The symptoms of female epispadias are primary urinary incontinence and abnormal
anatomical features.
 In females, epispadias consists of bifid clitoris with diastases of the corpora cavernosa,
flattening of the mons, and separation of the labia.

33. CLINICAL MANIFESTATION (MALE)
 Abnormal opening from the joint between the pubic bones to the area above the tip of the
penis
 Backward flow of urine into the kidney (reflux nephropathy)
 Short, widened penis with an abnormal curvature
 Urinary tract infections
 Widened pubic bone

35. CLINICAL MANIFESTATION (FEMALE)
 Abnormal clitoris and labia
 Abnormal opening from the bladder neck to the area above the normal urethral opening
 Backward flow of urine into the kidney (reflux nephropathy)
 Widened pubic bone
 Urinary incontinence
 Urinary tract infections

36. DIAGNOSIS
 Physical Examination
 Blood test to check electrolyte levels
 Intravenous pyelogram (IVP), a special X-ray of the kidneys, bladder and ureters
 MRI and CT scans, depending on the condition
 Pelvic X-ray
 Ultrasound of the urogenital system

37. SURGICAL MANAGEMENT
 Surgical management of epispadias is challenging and requires technical expertise.
 The surgery aims to reconstruct the genitalia and urethra, providing optimal functional and
cosmetic outcomes.
 At present, surgeons across the world have adopted either of the two following approaches:
 Modified Cantwell-Ransley repair
 Complete penile disassembly technique (Mitchell and Bagli)

38. NURSING MANAGEMENT
 Assess the newborns flow of urine, exit site, and angle of urination
 Infant/child returns from surgery w/ penis wrapped in a simple dressing and a stent or
catheter for
 urinary drainage
 Fresh blood may be seen on the dressing and in the stent or catheter in the immediate
postop period, but the urine should become less bloody over the next few hours
 Encourage fluid intake to maintain adequate urinary output and patency of the stent

39. NURSING MANAGEMENT
 Accurate hourly Input & output is essential
 Notify HCP if no urine drainage for 1 hour
 Once caudal block wears off, acetaminophen or ibuprofen should be administered for pain
as ordered
 Antibiotics are usually prescribed until the urinary stent falls out or is removed
 Double diapering protects the stent

40. COMPLICATIONS
 Wound infection
 Dysuria (due to bladder spasms)
 Urethral stricture and obstruction
 Persistent chordee
 Persistent short length
 Urethral retraction and hypospadias
 Partial or complete penile loss

41. THANK YOU FOR
LISTENING
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