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CCongenital genital defects
ZoReKh
C
• embryos of both sexes develop identically for approximately 6 weeks’ gestation, known
as the indifferent stage.
• At the...
• The tunica albuginea fascia envelops the corporal bodies tightly but is perforated by
an intercavernosal membranous sept...
• Cryptorchidism - absence of one or both testes from the scrotum. This includes undescended testes.
• Hypospadias - an ab...
• Exstrophy of the bladder is an uncommon condition that occurs in approximately 1 of every 30
000 live births, of which b...
Scar removal may help
in gain lenth of peinus
,some patient with no
umblicus which will need
reconstruction
(A)Bladder dys...
The reconstruction of a penis with
a pedicled ALT flap used as a
tube-within-a-tube technique (only
possible in thin ‘boys...
• The buried penis deformity is present in both the pediatric and adult populations.
• A buried penis is defined as a peni...
(A) Typical buried penis in
an infant. (B) Ventral
incision of the skin with
maximal preservation of skin
at the start of ...
• Chordee is a condition in which the head of the peius curves downward or upward, at the junction of
the head and shaft o...
• Hypospadias is an abnormality of anterior urethral and penile development in which the urethral opening is
ectopically l...
• The goals of surgical treatment of hypospadias are as follows:
1. Widening of the meatus
2. Correction of the curvature
...
Preservation of urethral plate
• Distal hypospadias
1. MAGPI (Meatal Advancement and Glanuloplasty) Duckett
2. TIP (Tubula...
TIP (Tubularized incised plate
urethraplasty)
• Complications:
1. Urethral-cutaneous fistula (<10%).
2. Meatal stenosis spraying of urine.
3. Urethral stricture.
4. Ure...
THANK YOU
male genitalia congenital diseases
male genitalia congenital diseases
male genitalia congenital diseases
male genitalia congenital diseases
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male genitalia congenital diseases

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basic knowledge of male genitalia diseases for plastic surgeon

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male genitalia congenital diseases

  1. 1. CCongenital genital defects ZoReKh C
  2. 2. • embryos of both sexes develop identically for approximately 6 weeks’ gestation, known as the indifferent stage. • At the 6th week, the urorectal septum begins to grow downward and inward from the sides into the cloacal cavity, thereby separating the cloaca into the bladder and rectum. • differentiated stage begins at the 7th week of intrauterine life many genes are responsible for gonadal defrintiation.
  3. 3. • The tunica albuginea fascia envelops the corporal bodies tightly but is perforated by an intercavernosal membranous septum that allows blood flow between the corpora cavernosa. • Buck fascia is a strong laminar structure that tightly surrounds and binds the corpora cavernosa together and, in the case of the corpus spongiosum, envelops these tissues into a single-functioning entity. The urethra and its overlying corpus spongiosum are also protected proximally by surrounding muscles and by their location within the intercorporal groove distally. • The penile glans itself is a vascular spongiosum containing unique sensory endings that are erogenous and tactile. The glans epithelium is a unique uroepithelium that contains sensory cells, particularly around the corona.
  4. 4. • Cryptorchidism - absence of one or both testes from the scrotum. This includes undescended testes. • Hypospadias - an abnormally placed urinary meatus (opening). • Chordee - a condition in which the head of the penis curves downward (that is, in a ventral direction) or upward, at the junction of the head and shaft of the penis. • Micropenis - an unusually small penis. Sometimes defined as being 2.5 times the standard deviation smaller than the mean size. • Aphallia - the phallus (penis or clitoris) is congenitally absent. 75 cases are known worldwide as of 2005. • Diphallia, penile duplication (PD), Diphallic Terata, or Diphallasparatus, is a medical condition in which a male infant is born with two penises. • Bladder exstrophy exstrophy-epispadias complex - a spectrum of anomalies of the lower abdominal wall, bladder, anterior bony pelvis, and external genitalia. Most common male genitala defects
  5. 5. • Exstrophy of the bladder is an uncommon condition that occurs in approximately 1 of every 30 000 live births, of which boys predominate in 3 : 1 • The defining features of epispadias and exstrophy are an open and protruding bladder, an open urethra, and a foreshortened epispadiac penis. • anomalies may extend to involve the musculoskeletal structures and the gastrointestinal tract. • Classic exstrophy –defined by bladder exstrophy, epispadias (epispadias alone occurs in 30% of cases), diastasis recti, absence of fusion of the pubic symphysis, and deformed pubic escutcheon. • The pubic rami are widely separated, and the inferior pubic rami are consequently laterally rotated. This defect produces a widened and foreshortened urethra and bladder neck. It also produces an incompletely formed penis that remains rudimentary. • The crural bodies are attached to the splayed pubic tubercles, producing a penis that is short, wide, and with dorsal chordee. • As they pass through their post-adolescent period, many of these young men will benefit from further lengthening procedures or even complete penile reconstruction. In some patients correction of unaesthetic scars and further release of insufficiently released corpora can help to gain length. Exstrophy and epispadias
  6. 6. Scar removal may help in gain lenth of peinus ,some patient with no umblicus which will need reconstruction (A)Bladder dystrophy with very small gentalia (B)Free Radial forarm flap was done (C)Glans of the redimantry peines was left for sensation
  7. 7. The reconstruction of a penis with a pedicled ALT flap used as a tube-within-a-tube technique (only possible in thin ‘boys without a penis’ and after defatting). No real urethra was reconstructed here since the patient had a urostomy. (A) Preoperative view. (B) After flap dissection. (c) The flap is tunneled underneath the rectus femoris muscle. (D) Suturing and nerve connection (ilioinguinal nerve to lateral femoral cutaneous nerve).
  8. 8. • The buried penis deformity is present in both the pediatric and adult populations. • A buried penis is defined as a penis that is of normal size for age but hidden within the peripenile fat and subcutaneous tissues. • buried penis must be differentiated from a micropenis in this group of obyse patient. • In adults, the problem is almost always associated with obesity and the development of pubic, scrotal, and peripubic ptosis, Liposuction and lipectomy are part of the treatment. • However in children the fat resection is abandoned. With pubertal development the prepubic fat deposit often decreases in size. The focus is on the release of the penis from the fibrotic dartos tissue Many techniques are described but the most important steps include keeping all available skin from the start of the procedure, to resect all dartos tissue and to recover the released corpora with the skin. Buried penis
  9. 9. (A) Typical buried penis in an infant. (B) Ventral incision of the skin with maximal preservation of skin at the start of the procedure. (c) After complete resection of the fibrotic dartos tissue the penis is released from its buried position and the skin is extendable. (D) Coverage of the released corpora with the extended skin creating a penis with normal length.
  10. 10. • Chordee is a condition in which the head of the peius curves downward or upward, at the junction of the head and shaft of the penis. • The curvature is usually most obvious during erection • Chordee may be caused by disorder of sex development or as a complication of circumcision though some medical professionals do not consider it to be true chordee because the corporal bodies are normally formed. • The curvature of a chordee can involve (1)tethering of the skin with urethra and corpora of normal size,(2)curvature induced by fibrosis and contracture of the fascial tissue (Buck's fascia or dartos) ,(3)fibrotic urethra that tethers the penis downward • the preferred method of surgical treatment is a z-plasty,The preferred time for surgery is between the ages of 6 and 18 months . Chordee
  11. 11. • 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 • The urethral opening may be located as far down as in the scrotum or perineum it’s likely to have associated ventral shortening and curvature (chordee) with more proximal urethral defects. • Hypospadias is a congenital defect that is thought to occur embryologically during urethral development, between 8 and 20 weeks' gestation. • Hypospadias occurs in approximately 1 in every 250 male births in the US incidence doubled from 1970 to 1993. suggestions that this doubling actually reflects increased reporting of minor grades of hypospadias, increases in severe hypospadias have also been noted. • The more proximally ectopic the position of the urethral meatus, the more likely the urinary stream is to be deflected downward, which may necessitate urination in a seated position.chordee can exacerbate this abnormality. Fertility may be affected. The abnormal deflection of ejaculate may preclude effective insemination, and significant chordee can preclude vaginal insertion of the penis or can be associated with inherently painful erections. • Minor cases of hypospadias, in which the meatus is located up toward the tip of the glans, may not require surgical repair and may simply be managed with observation. Hypospadias
  12. 12. • The goals of surgical treatment of hypospadias are as follows: 1. Widening of the meatus 2. Correction of the curvature 3. Reconstruction of the missing portion of the urethra 4. Restoration of the normal aspect of the external genitalia • Hypospadias repair was performed in children older than 3 years because of the larger size of the phallus and a technically easier procedure; however, genital surgery at this age (genital awareness occurs at about age 18 months) can be associated with significant psychological morbidity, including abnormal behavior, guilt, and gender identity confusion. • Currently, most physicians attempt to repair hypospadias when the child is aged 4-18 months. • A meatotomy is required if the size of the external urethral meatus is inferior to that considered normal according to the age of the patient. • The distal urethra missing in glanular hypospadias, usually without recurvatum, is well reconstructed with local flaps based on the meatus (eg, Santanelli procedure, Flip Flap, MAGPI [meatal advancement and glanuloplasty]), including preputium plasty at the same sitting.
  13. 13. Preservation of urethral plate • Distal hypospadias 1. MAGPI (Meatal Advancement and Glanuloplasty) Duckett 2. TIP (Tubularized incised plate urethraplasty) Snodgrass 3. Mathieu • Middle Hypospadias: 1. Onlay island flap • Proximal hypospadias: 1. Onlay island flap 2. Buccal mucosa graft Two-stage procedure
  14. 14. TIP (Tubularized incised plate urethraplasty)
  15. 15. • Complications: 1. Urethral-cutaneous fistula (<10%). 2. Meatal stenosis spraying of urine. 3. Urethral stricture. 4. Urethral diverticulum. 5. Hair in urethra: UTI and stone formation. 6. Dissatisfaction with penile size.
  16. 16. THANK YOU

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