Hypospadias and various repair options.pptx

A concise power point presentation on hypospadias and its various repair options.

1. Hypospadias Dr. Syed Mohammad Nabeel Aamir PGY-2 Surgical Resident Department of Urology Abbasi Shaheed Hospital

2. Hypospadias A congenital hypoplasia and arrest of penile development, leaving a proximal meatus Urethral opening is situated on the ventral side of the penile shaft in one of several positions Caused by an abnormal or incomplete closure of the urethral folds Most common congenital anomaly of the penis. Estimated to be around 20.9 cases per 100,000 live male births, which translates to roughly 1 in 500, as per the CDC

3. Hypospadias Triad Association of 3 anomalies: Abnormal ventral opening of urethral meatus Abnormal ventral curvature of the penis Abnormal distribution of the foreskin with a dorsal hood

4. Embryology The Genital tubercle is initially indifferent and develops the female phenotype unless exposed to androgens during the critical period of 8-12 weeks The Urethral plate develops as an extension of endoderm along the ventral midline of the genital tubercle Proliferating mesenchyme on either side creates urethral folds and establishes the urethral groove Fusion of these urethral folds begins proximally and continues distally till the glans

5. Female Hypospadias (hypospadias feminis ) Characterized by ectopic dorsal location of the external urethral opening, which is a rare congenital malformation Failure of urethral-fold fusion and distal urethral migration It is defined as female urethral opening on the anterior vaginal wall (proximal to the hymenal ring) anywhere between the introitus and the vaginal fornix Rarely diagnosed because physicians are not well-informed about female hypospadias T hought to be due to an abnormal development of the urogenital sinus or to a lack of differentiation of Wolff ian tissue. Etiology though still remains unknown.

6. Associated Anomalies Genital: Undescended testes Hypoplastic testes Chordee Pre-penile scrotum Bifid Scrotum Enlarged Prostatic Utricle Urinary tract: Renal Hypoplasia Horseshoe Kidney Hydronephrosis Extra-urogenital: PPV (patent processus vaginalis) Cleft Palate Congenital Heart Defects Musculoskeletal Abnormalities Anorectal Malformations DSD

7. Syndromes Smith-Lemli-Opitz: DHCR7 gene mutation. Mental retardation, facial dysmorphism, microcephaly and syndactyly WAGR: WT1 gene deletion. Wilm’s tumor, Aniridia, Genital anomalies, mental Retardation Hand-foot-genital: HOXA13 gene mutation. Bilateral thumb and great toe hypoplasia with hypospadias Opitz G: Midline 1 gene mutation or 22q11 deletion. Hypertelorism, tracheoesophageal defects, cleft lip/palate, mental retardation and hypospadias Wolf-Hirschhorn: 4p Ch deletion. Mental retardation, seizures, abnormal facies and midline defects 13q deletion: Mental retardation, facial dysmorphia, imperforate anus and hypospadias

8. Classification

9. American Urological Association (A.U.A.) Classification The American Urological Association (AUA) classifies hypospadias  based on the degree of urethral defect The urethral defect ratio (UDR) is a measurement used to classify the severity of hypospadias. It's calculated by dividing the distance of the urethral defect by the stretched length of the penis Urethral defect ratio : The AUA classifies hypospadias into three classes based on the urethral defect ratio: 0.5 0.5-0.99 1.0

10. Non-Operative Management This approach is only suitable for very mild forms of hypospadias where the urethral opening is positioned close to the tip of the glans.  Regular monitoring: Even with non-operative management, regular checkups with a urologist are crucial to monitor for any potential complications or changes in the condition.  Psychological considerations: Even in mild cases, discuss potential psychological impacts with the parents and child, especially regarding body image concerns.

11. Non-Operative Management Factors considered for non-operative management: Location of the urethral opening: The primary factor is how close the urethral opening is to the tip of the glans.  Severity of curvature: If there is minimal penile curvature associated with the hypospadias, non-operative management might be considered.  Patient's age and development: Younger children may be monitored more closely as the penis continues to develop. 

12. Complications of Untreated Hypospadias Non-Operative management may lead to persistent urinary stream abnormalities, sexual dysfunction and subfertility Urination difficulties Sexual difficulties Lower self-esteem and psychological issues Worse sexual health Worse prostatic symptoms Ventral penile curvature

13. Pre-Operative Evaluation Karyotyping: Helps categorize Hypospadias as syndromic when the defect is not isolated A hormonal evaluation may be needed if a disorder of sexual development (DSD) is suspected Ultrasonography: A renal ultrasound may be performed if other features are present, such as dysmorphic features  Radiologic Studies: Retrograde urethrography may be performed to investigate proximal hypospadias or a CT Scan if there is suspicion of associated renal anomalies Circumcision is not performed in cases where a hypospadias repair is needed as the foreskin can be utilized during surgery

14. Indications for Operation Functional Indications: Proximally located meatus Ventrally deflected Urinary Stream Meatal Stenosis Curved Penis Cosmetic Indications: Abnormally located meatus Cleft Glans Chordee Preputial hood Penoscrotal Transposition Split Scrotum

15. Pre-Operative Hormonal Stimulation Goal: To increase the size of the glans, penis and improve the vascularity of the penile tissues, potentially leading to better surgical conditions for hypospadias repair, especially in cases with severe hypospadias or micropenis , by enhancing the available tissue for reconstruction during the hypospadias repair procedure Hormones used: Most commonly, testosterone or dihydrotestosterone (DHT) are used, either via injection or topical application Controversy: While some studies suggest improved surgical outcomes with preoperative hormonal stimulation, there is no clear consensus on its routine use due to concerns about potential side effects, lack of standardized treatment protocols, and inconsistent results across studies

16. Pre-Operative Hormonal Stimulation Intramuscular testosterone enanthate 2 mg/kg/dose given 5 and 2 weeks preoperatively 2% Testosterone propionate cream TDS for 3 weeks HCG 250-500 IU SC twice a week for 3 weeks. Increases penile size and length Decrease in chordee and hypospadias severity Increased vascularity and thickness of corpus spongiosum Allowance of more simple repairs

17. Timing of Surgery Ideally performed between 6 and 12 months Same-day surgery can be performed after 50 gestational weeks in otherwise healthy boys Elective repair may be performed at 3 months of age or older for distal hypospadias and selected proximal cases with apparently normal-sized phallus Infants with proximal hypospadias and a small-appearing glans are reassessed at 3 months and then administered hormonal stimulation, before surgery at approximately 6 months of age 18 months till 3 years of age is described as a difficult period for hospitalization; repair postponement to age greater than 3 years is recommended

18. General Principles of Hypospadias Repair Orthoplasty Urethroplasty Meatoplasty Glanuloplasty Skin Coverage

19. Orthoplasty Correction of Ventral curvature Ventral tissues may be shortened relative to the dorsal surface Ventral curvature occurs in 11% of distal, 30% midshaft and 81% proximal cases of hypospadias Preoperative assessment cannot accurately predict neither the extent of the curvature nor 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

20. Orthoplasty Artificial erection induced by saline injection remains the most commonly used means to assess presence and severity of VC; less risk of hemorrhage Pharmacologic erection allows for a more accurate (closely mimics physiology) and continued assessment of penile curvature before, during and after its correction however, it is more prone to hemorrhage Intracorporeal injection of the arterial vasodilator Prostaglandin E1

21. Orthoplasty Curvature of 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

22. Orthoplasty Nesbit Technique: Excision of a diamond shaped wedge/s at the point of maximum curvature and closing the Tunica Albuginea transversely with absorbable sutures

23. Orthoplasty Ventral Corporeal Lengthening: Ventral Corporotomy with grafting Multiple Corporotomies without grafting

24. Urethroplasty Distal Hypospadias TIP repair Others like MAGPI, Mathieu flip-flap and Urethral advancement Midshaft Hypospadias TIP repair Onlay preputial flap 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

25. Tubularized Incised Plate (TIP) repair: Circumscribing incision is made approximately 2 mm below meatus (when circumcision is desired) Ventral V incision (when foreskin reconstruction is planned) Penis degloved (or only ventral surface exposed during foreskin preservation) Visible junction of glans wings to urethral plate is marked and infiltrated with 1:100,000 epinephrine Midline incision of the urethral plate, extending from within the meatus to the end of the plate, without entering the distal glans Urethral plate tubularization begins distally approximately 3 mm from the end of the plate, ensuring an oval, not rounded, meatus Dartos flap is dissected from the dorsal prepuce and shaft skin, buttonholed and transposed ventrally to cover the neourethra Glansplasty begins distally using a 7-0 polyglactin suture to create the meatus at the desired location

26. Meatal Advancement and Glanuloplasty (MAGPI) Liberating the ventral skin : The surgeon frees up the skin on the ventral side of the penis.  Making a triangular incision: The surgeon makes a triangular incision from the point where the new meatus will be.  Suturing the meatus: The surgeon sutures the center of the hypospadiac meatus to the top of the triangle.  Suturing the preputial frenulum : The surgeon sutures an inverted "V" shape to simulate the preputial frenulum.  Making a longitudinal incision: The surgeon makes a long incision from the inside of the dorsal edge of the meatus to the distal glans groove.  Approximating the tissue: The surgeon uses two layers of tissue approximation to support the advanced ventral urethral wall. 

27. Onlay preputial flap Thin skin proximal to the urethral meatus is incised to the midline convergence of corpus spongiosum wings 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 Flap is sewn to the urethral plate

28. Proximal Hypospadias The greatest controversy 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 TIP repair or onlay preputial flap can be used When the urethral plate is transected: One stage urethroplasty can be accomplished by tubularized preputial flap or the Koyanagi flap Two stage repair done with Byars flaps or preputial grafts

29. Proximal TIP 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 given Spongioplasty over the neourethra

30. Koyanagi Flap The flap can be divided into two wings or in one piece with a central buttonhole to transpose it ventrally 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 Inner flaps margins are reapproximated and excess is excised Outer margins are closed to complete tubularization

31. Byars Flap After degloving and release of ventral dartos, persisting ventral curvature of more than 30 degrees, leads to excision of the urethral plate Dorsal preputial hood is incised in the midline and two flaps transposed ventrally on either side of the penis The prepuce is advanced into the glans Flap edges are approximated in the midline 6 months later, a U-shaped incision is made, approximately 10 mm wide The resultant strip is tubularized in two layers

32. Skin Closures Circumcision vs Foreskin Reconstruction: The abnormal prepuce can be removed for circumcision or reconstructed ventrally as per preference If circumcision is desired, a circumferential skin incision is given initially, creating lateral darts to approximate the mucosal collar ventrally When foreskin reconstruction is desired, the initial skin incision extends in a V from the corners of the dorsal prepuce ventrally to below the meatus, without a circumferential incision

33. Inner Preputial Collar Ventral preputial deficiency found in most hypospadias patients includes its inner mucosal surface, which normally provides coverage between the corona and shaft skin Instead, the shaft skin occupies the space from the margins of the open glans wings to the urethral plate and meatus This skin must be removed and ventral midline closure should be done using inner prepuce transposed from the dorsal hood

34. Scrotoplasty Scrotal encroachment onto the ventral penile shaft can occur associated with any extent of hypospadias Techniques employed are: Major scrotoplasty Minor scrotoplasty

35. Major Scrotoplasty After completion of urethroplasty, skin incisions are made along the abnormal extensions of scrotum on either side of the penis These flaps are rotated down and to the midline Skin closure is achieved using subepithelial sutures

36. Minor Scrotoplasty Ventral incisions at the penoscrotal junction allow ventral rotation of shaft skin and scrotum Incision continues to approximately the 3 to 4 and 8 to 9 ‘o’ clock positions with subsequent ventral approximation of the shaft skin

37. Assessing Surgical Outcomes The goal of repair is to improve function and appearance as near to normal as possible Success requires more than straightening the curvature and extending the urethra to the glans Post-operative follow-up for distal to midshaft repairs at 6 weeks and 6 months Proximal repair has to have additional annual follow-ups until toilet training Periodic assessment should continue till puberty but is highly unlikely

38. Assessing Surgical Outcomes Neourethral Calibration: Passage of a sound or bougie can be done to determine if a small appearing neomeatus is stenotic and to exclude neourethral stricture Given the low rates of meatal stenosis and stricture, instrumentation could be reserved for those with obstructive voiding symptoms Uroflowmetry: Non-invasive means to assess neourerthal function

39. Surgical Complications Bleeding/Hematoma Wound Infection Impaired healing/Dehiscence Breakdown of the repair Meatal Stenosis Urethral Stricture Urethral Diverticulum Urethro-cutaneous Fistula

40. Thank you!