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Hypospadias

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Hypospadias

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Hypospadias

  1. 1. HYPOSPADIAS Dr.K.Priyatham
  2. 2. DEFINITION • Hypospadias is defined as hypoplasia of the tissues forming the ventral aspect of the penis beyond the division of the corpus spongiosum. • Hypospadias is believed to result from arrested penile development, leaving a proximal urethral meatus.
  3. 3. • Incidence- 1/250 male newborns • Association of 3 anomalies Abnormal ventral opening of urethral meatus Abnormal ventral curvature of the penis Abnormal distribution of foreskin with a dorsal hood
  4. 4. DIAGNOSIS • Hypospadias is diagnosed by physical examination, first suspected by the ventrally deficient prepuce and confirmed by the proximal meatus.
  5. 5. • Abnormal ventral findings potentially include 1. Downward glans tilt, 2. Deviation of the median penile raphe, 3. Ventral Curvature, 4. Scrotal encroachment onto the penile shaft, 5. Midline scrotal cleft, and 6. penoscrotal transposition.
  6. 6. NORMAL PENILE ANATOMY 2 Corpora Cavernosa, Corpus Spongiosum Enclosed In A Fascial Sheath- T.Albuginea Bucks Fascia, Thick Fibrous Envelope
  7. 7. Embryology of Penile Development • The external genital anlage is initially indifferent and develops the female phenotype unless exposed to androgens during the critical gestational time period of 8 to 12 weeks. • The urethral plate develops as an extension of endoderm from the cloaca along the ventral midline of the genital tubercle.
  8. 8. EMBRYOLOGY
  9. 9. • Proliferating mesenchyme to either side creates urethral folds and establishes the urethral groove. • Fusion of the urethral folds begins proximally and continues distally at least to the glans. • Two theories are proposed for glanular urethra development: ectodermal ingrowth cannulating the glans to the urethral plate versus urethral plate tubularization to the tip of the glans.
  10. 10. ETIOLOGY  Genetic Factors • Familial aggregation is found in 4% to 10% of hypospadias cases, including first-, second-, and third- degree relatives.  Gene Mutations • Murine studies indicating androgen receptor activity regulates Fgf8, Fgf10, and Fgfr2 involved in urethral development have led to screening for defects in these candidate genes in patients with hypospadias.
  11. 11. ETIOLOGY  In most cases, the cause of this congenital defect is not fully understood.  Treatment with hormones such as progesterone during pregnancy may increase the risk of hypospadias.  Certain hormonal fluctuations, such as failure of the fetal testes to produce enough testosterone or the failure of the body to respond to testosterone, increase the risk of hypospadias and other genetic problems.
  12. 12. ASSOCIATED ANOMALIES • Cryptorchidism • Prostatic utricle • Renal agenesis
  13. 13. CLASSIFICATION
  14. 14. Indications for operation Functional indications: 1. Proximally located meatus 2. Ventrally deflected urinary stream 3. Meatal stenosis 4. Curved penis.
  15. 15. The cosmetic indications, which are strongly linked patient’s future psychology, are: 1. Abnormally located meatus 2. Cleft glans 3. Rotated penis with abnormal cutaneous raphe 4. Preputial hood 5. Penoscrotal transposition 6. Split scrotum.
  16. 16. Pre operative Consideration  Timing of surgery: • Performed between 6 and 12 months. • Healing seems to occur more quickly and with fewer scars, and young infants overcome the stress of surgery more easily. • This age seems to insulate most children form the psychologic, physiologic, and anaesthetic trauma associated with hypospadias surgery.
  17. 17. Preoperative Hormonal Stimulation: HCG 250-500 U sc twice a week for 3 weeks. Increase in penile size and length Decrease in hypospadias and chordee severity Increased vascularity and thickness of corpus spongiosum Allowance of more simple repairs IM testosterone enanthate – 2mg/kg/dose given for a total of 2 or 3 doses before hypospadias repair Testosterone propionate cream – 2% three times daily for 3 weeks
  18. 18. GENERAL PRINCIPLES OF HYPOSPADIAS REPAIR ORTHOPLASTY URETHROPLASTY MEATOPLASTY GLANULOPLASTY SKIN COVERAGE
  19. 19. Serafeddin (15TH century) was a surgeon from central Antolia during the Ottoman period. • In one of his books he describes the fine scalpel “mibza” used for the treatment of meatal stenosis in hypospadias.
  20. 20. ORTHOPLASTY • Correction of ventral curvature • Ventral tissues—including shaft skin, dartos, corpus spongiosum, urethral plate, and overlying tunics of the corpora cavernosa may be shortened relative to the dorsal surface. • VC occurred in 11% of primary distal cases, 30% midshaft, and 81% proximal hypospadias.
  21. 21. • Preoperative assessment cannot accurately predict either the extent of curvature or the means required for straightening. • Intraoperative assessment of penile curvature by either artificial or pharmacologic methods is a critical step in hypospadias repair. • Performed after degloving of penile shaft skin.
  22. 22. • Artificial erection induced by saline injection remains the most commonly used means to assess presence and severity of VC. • Pharmacologic erection allows for a more accurate and continued assessment of penile curvature before, during, and after its correction. • Intracorporal injection of the arterial vasodilator prostaglandin E1.
  23. 23. • Curvature up to 30 degrees can be corrected by Midline Dorsal Plication into the tunica albuginea of the corpora cavernosa directly opposite the area of greatest bending.
  24. 24.  Nesbit technique : • Excision of diamond shaped wedge/s at the point of maximum curvature and closing the tunica transversely with absorbable sutures.
  25. 25. • Ventral corporeal lengthening : • Ventral corporotomy with grafting. • Multiple corporotomy without grafting.
  26. 26. • Dermal Graft – Devine and Horton.
  27. 27. URETHROPLASTY  Distal hypospadias : • TIP repair • Others like MAGPI, Mathieu flip-flap, and Urethral advancement.  Midshaft hypospadias : • TIP repair • Onlay preputial flap
  28. 28. Proximal hypospadias : • TIP repair • Onlay preputial flap • Single stage urethroplasty with preputial flap or the Koyanagi flap. • Two stage repair with Byars flaps or grafts.
  29. 29. Distal Hypospadias  Tubularized incised plate (TIP) repair: • Circumscribing incision is made approximately 2 mm below meatus • Ventral V incision • Penis degloved • Midline incision of the urethral plate • Urethral plate tubularization begins distally approximately 3 mm from the end of the plate, ensuring an oval, not rounded, meatus.
  30. 30. • Dartos flap is dissected from the dorsal prepuce and shaft skin, buttonholed, and transposed ventrally to cover the neourethra. • Glansplasty begins distally, and a 7-0 polyglactin suture.
  31. 31. Midshaft Hypospadias  TIP repair  Onlay preputial flap : • Thin skin proximal to the urethral meatus is incised to the midline convergence of corpus spongiosum wings. • Then inner prepuce is harvested on its vascular pedicle from either the dorsal hood or dartos flap. • The flap should be gently stretched to fit the urethral plate without redundancy.
  32. 32. Proximal Hypospadias • The greatest controversy in primary hypospadias surgery concerns decision making for proximal cases. • Options depend on whether the urethral plate is available for urethroplasty after associated VC is straightened. • If so, then either TIP repair or an onlay preputial flap can be used. • When the urethral plate is transected a one-stage urethroplasty can be accomplished by tubularized preputial flaps or the Koyanagi flap or a two-stage repair done with Byars flaps or preputial grafts.
  33. 33.  Proximal tubularized incised plate repair : • Circumscribing incision preserves urethral plate in patient desiring circumcision. • After degloving, glans wings are separated from the urethral plate. • Corpus spongiosum is dissected from the cavernosal bodies • Midline urethral plate incision. • Spongioplasty over the neourethra.
  34. 34.  Tunica vaginalis flap :
  35. 35.  Koyanagi flap • Proposed lines of incisions to create flap • The flap can be divided into two wings as shown or maintained in one piece with a central buttonhole to transpose it ventrally.
  36. 36. • The urethral plate in the center of the flap is dissected from the corpora to near the meatus, and the glanular portion of the plate is excised as glans wings are made.
  37. 37. • Inner flap margins are reapproximated, and excess flap skin is excised. • The outer margins are closed to complete tubularization
  38. 38.  Byars flap • After degloving and release of ventral dartos, persisting ventral curvature greater than 30 degrees led to excision of the urethral plate. • The dorsal preputial hood is incised in the midline and the two flaps transposed ventrally on either side of the penis. • The prepuce is advanced into the glans; alternatively, the urethral plate can be maintained within the glans.
  39. 39. • Flap edges are approximated in the midline. • Six months later a U- shaped incision is made approximately 10 mm wide.
  40. 40. • The resultant strip is tubularized in two layers.
  41. 41. COMPLICATIONS • Bleeding/hematoma, • Meatal stenosis, • Urethrocutaneous fistula, • Urethral stricture, • Urethral diverticulum, • Wound infection, • Impaired healing, and • Breakdown of the repair
  42. 42. EPISPADIASIS
  43. 43. • Epispadias is a congenital malformation in which the opening of the urethra is on the dorsum of the penis. • 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 belly area.
  44. 44. Incidence • Epispadias occurs in 1 in 117,000 newborn boys and 1 in 484,000 newborn girls.
  45. 45. Causes • Unknown • Related to improper development of the pubic bone • Failures of abdominal and pelvic fusion in the first months of embryogenesis • Epispadias can be associated with bladder exstrophy, an uncommon birth defect in which the bladder is inside out, and sticks through the abdominal wall • Also occur with other defects
  46. 46. Classification Classification of epispadias is based on the location of the meatus the penis. It can be positioned: • On the glans (glanular) • Along the shaft of the penis (penile) • Near the pubic bone (penopubic).
  47. 47. • The position of the meatus is important because it predicts the degree to which the bladder can store urine (continence). The closer the meatus is to the base of the penis, the more likely the bladder will not hold urine
  48. 48. Symptoms In males: • 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
  49. 49. In females: • Abnormal clitoris and labia • Abnormal opening where the 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 infection
  50. 50. Diagnostic measures • Prenatal diagnosis - rare • 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
  51. 51. Surgical technique in males: • The modified Cantwell technique It involves partial disassembly of the penis and placement of the urethra in a more normal position.
  52. 52. Treatment The primary goals of treatment of epispadias are to: • maximize penile length and function by correcting dorsal bend and chordee; and • create functionality and cosmetically acceptable external genitalia with as few surgical procedures as possible. • If the bladder and bladder neck are also involved, surgical treatment is required to establish urinary continence and preserve fertility.
  53. 53. • The second technique is the Mitchell technique. »It involves complete disassembly of the penis into its three separate components. »Following disassembly, the three components are reassembled such that the urethra is in the most functional and normal position and dorsal chordee is corrected.
  54. 54. Surgical technique in females • The urethra and vagina may be short and near the front of the body and the clitoris is in two parts. • If diagnosed at birth, the two parts of the clitoris can be brought together and the urethra can be placed into the normal position. • If repaired early enough, lack of urinary control (incontinence) may not be a problem.
  55. 55. • If the diagnosis is missed or if early repair is not performed, then incontinence can be surgically corrected at the time of diagnosis. • If the vaginal opening is narrow in older girls or younger women, reconstruction can be performed after puberty.
  56. 56. Take home message • Hypospadias – ectopic ventral opening of the meatus • Associations- chordee and hernia • Dorsal hood of foreskin, glanular groove with ventral incomplete prepuce • Circumcision contraindicated • Surgery- 4 to 18 months
  57. 57. THANK YOU

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