Hypospadias

incidence , associated congenital anomalies , indications for repair , various procedures for hypospadias and its complications

1. DR.LEELA KRISHNA HYPOSPADIAS

2. INTRODUCTION Hypospadias is believed to result from arrested penile development, leaving a proximal urethral meatus. Hypospadias can be defined as hypoplasia of the tissues forming the ventral aspect of the penis beyond the division of the corpus spongiosum .

3. Abnorm al v e n t r al open i n g o f u r e th r al me a tus Abnorm al v e n t r al cu r v a tu r e o f the pen is Abnorm al d i s tri butio n o f f o r eskin with a do r s a l ho o d Other abnormal findings include downward glans tilt, deviation of the median penile raphe, scrotal encroachment onto the penile shaft, midline scrotal cleft, and penoscrotal transposition.

4. CLASSIFICATION

5. INCIDENCE Present in approx. 1 in 300 male newborns Typically diagnosed at new born physical examination Distal or anterior hypospadias account for 50-70 % . Female hypospadias  - a developmental anomaly in the female in which the urethra opens into the vagina. Female hypospadias is extremely rare.

6. ASSOCIATED ANAMOLIES Cryptorchidism / Inguinal Hernia Associated abnormalities include cryptorchidism (7% to 9%) and inguinal hernia and/or hydrocele (9% to 16%) The prevalence of inguinal hernia was similar in anterior, mid, and posterior hypospadias at approximately 12% Cryptorchidism predominant in boys with posterior defects.

7. ASSOCIATED ANAMOLIES A high index of suspicion for an intersex state should accompany presumed males with any degree of hypospadias and cryptorchidism Approximately 15% of individuals with hypospadias and a palpable undescended gonad will have an intersex condition.   Approximately 50% of individuals with hypospadias and a unilateral nonpalpable gonad will have an intersex condition.    A chromosomal abnormality will be present in approximately 22% of individuals with hypospadias and cryptorchidism

8. Ultrasound has been suggested as a screening procedure only in severe cases. MCUG suggested only if there is H/O recurrent UTI and in cases of True ambiguos genitalia to assess presence of Mullerian duct remnants Cystoscopy not necessary except in severe cases , where it may show Prostatic utricular enlargement ROLE OF IMAGING

9. TIMING OF REPAIR Factors to be considered Psychological implications of genital surgery in children. Age related anaesthetic and surgical risks. Ideal age of repair is 3 months- 12 months of age.

10. Advent of delicate surgical techniques, optical magnification, careful hemostasis ( Bipolar cautery ). Delayed Surgery has social implications as well.

11. Anaesthesia : General anesthesia, typically with endotracheal intubation, has been the mainstay of anesthetic technique A popular agent for adjunctive analgesia is bupivacaine (0.25%) without epinephrine administered as either a caudal or dorsal penile nerve block. Significant improvement in postoperative pain control in those patients who received a penile block both at the beginning and completion of hypospadias repair

12. Hemostasis : use of electrocautery should be limited during hypospadias repair The current of monopolar electrocautery is dispersed generally along the vessels, irreparably damage tissue microvasculature Prefer the bipolar variant and the use of fine-point neurologic forceps Injection of a vasoconstrictive agent (epinephrine diluted 1:200,000 with lidocaine ) deep to proposed glanular incision intermittent use of a tourniquet at the base of the penis during urethroplasty

13. Suture/Suture Technique Stay sutures are used whenever possible to limit tissue handling. a subcuticular technique is employed during longitudinal closure of the neourethra when performing a tubularization technique. Most important aspect of closure of the neourethra is that the edge of the epithelial surface is inverted and the raw surfaces of the subepithelial tissue are approximated

14. Dressing: Silicone foam elastomer with pantaloon spica cast postoperative immobilization after free graft hypospadias repair ( Cilento et al, 1997 ). There is little or no advantage to application of a dressing to the operated hypospadias ( Van Savage et al, 2000 ; McLorie et al, 2001 ). Method of postoperative care (dressing vs. no dressing) did not impact surgical success rate or wound healing. In general, a regimen of no dressing (antibiotic ointment only) appears to result in increased patient comfort and decreased burden for the caregiver.

15. INDICATIONS FOR REPAIR To permit emptying of the bladder standing up. Achieve sexual intercourse, and effectively inseminate. To prevent urinary tract infections (UTIs), common in hypospadiac meatus to be stenotic , or abnormally narrowed. A stenotic urethra increases the risk of frequent UTIs. To confirm the boy's sexual identity by improving the outward appearance of the penis.

16. PREOPERATIVE HORMONAL STIMULATION Repair of Hypospadiasis in child with microphallus is technically difficult. To enhance size of penis, preoperative androgen therapy suggested. Gonadotrophins or testosterone may be used. Treatment with HCG showed decrease in severity of proximal hypospadiasis and improved chordee ( Koff & Jayanthi ) Increase in penile length and distal migration of meatus observed. Testosterone more beneficial than HCG.

17. Testosterone given as either cream or intramuscular injection Intramuscular injection of testosterone enanthate , either 25 mg/dose or 2 mg/kg/dose, given for a total of two or three doses before hypospadias repair   Prepubertal exogenous testosterone administration does not appear to impair ultimate penile growth. Testosterone propionate cream 2% , 3 times daily for 3 weeks.

18. INTRAOPERATIVE ALGORITHM

19. GENERAL PRINCIPLES Regardless of the technique employed for repair of hypospadias and its associated defects Penile curvature and its correction ( orthoplasty ) Urethroplasty Meatoplasty and glanuloplasty , and finally Skin coverage

20. Orthoplasty Assessment of Penile Curvature Intraoperative assessment of penile curvature by either artificial or pharmacologic methods Needle passed through the glans to eliminate the possibility of hematoma formation beneath Buck's fascia. The degree of curvature may vary with force of injection and/or the method used to impede saline outflow. GITTES TEST

21. CONTD ….

22. ORTHOPLASTY TECHNIQUES Plicating / Heineke-Mikulicz Techniques Tunica Albuginea Plication Corporal Rotation Dermal Graft Small Intestinal Submucosa . Tunica Vaginalis Graft Total Penile Disassembly .

23. MANAGEMENT The level of the hypospadias , degree of penile curvature, and tissue availability and quality dictate repair options.    Distal hypospadias is typically amenable to advancement or tubularization techniques.    Middle hypospadias is typically amenable to tubularization or vascularized flap techniques.      Proximal defects are treated with tubularization , vascularized flap, two-stage techniques, or the incorporation of extragenital skin for repair.

24. OPERATIVE PROCEDURE DISTAL HYPOSPADIASIS Advancement Technique MAGPI technique Tubularization Technique Snodgrass Tubularized incised plate (TIP) urethroplasty Flap Techniques Mathieu perimeatal -based flap technique

25. Middle Hypospadias Procedures Onlay Techniques – Preputial flaps Onlay island flap (OIF) technique Split prepuce in-situ onlay -- modification of the OIF technique. Double- onlay preputial flap 2. Tubularization Techniques Snodgrass Tubularized incised plate (TIP) urethroplasty

26. PROXIMAL HYPOSPADIASIS 1.Onlay Techniques Onlay island flap Double- onlay preputial flap 2. Tubularization Techniques Transverse preputial island flap (TPIF)- Duckett Parameatal foreskin flap ( Koyanagi repair)

27. MAGPI Circumferential subcoronal incision-5mm proximally Longitudinal incision of transverse glanular “bridge” in urethral plate - from meatus to distal glanular groove Transverse approximation ( Heineke-Mikulicz procedure ). Ventral edge of meatus is pulled distally, and medial glans “trimming” incisions are marked. Deep suture approximation of the glans . Superficial approximation of the glans and skin.

28. MAGPI

29. Mathieu flap( Perimeatal based flap) Measure the length of the defect from the urethral meatus to the glans tip. An equal distance from the meatus is measured on the proximal penile shaft skin. A line is marked beginning at either lateral margin of the previously marked urethral plate and carried around the dorsal aspect of the penis 5 to 7 mm proximal to the corona of the glans . Glans wings are incised deeply, the penile shaft skin is degloved , and the penis is evaluated for curvature

30. Premeasured segment of penile skin proximal to the meatus is mobilized off of the urethra in a proximal to distal direction Running subcuticular sutures approximate this flap to the lateral aspects of the urethral plate Second-layer neourethral coverage is performed with dorsal dartos tissue The glans wings are then approximated without tension in two layers, and skin coverage is completed

31. MATHIEU PERIMEATAL FLAP

32. Tubularised Incised Plate Repair (TIP) Snodgrass (1994) Most commonly performed procedure Almost all cases of distal Hypospadias can be managed Most recent concept based on recognition that urethral plate is a well vascularied tissue rather than fibrous band Simple and better cosmesis and results

33. TIP Principle – based on the assumption that midline incision into the urethral plate may widen it sufficiently for urethroplasty without stricture. There are two important criteria to achieve good results: Urethral plate should not be less than 14 mm wide There should be no distal deep chordee . Critical steps- 1)midline incision of urethral plate from meatus to tip of penis 2)wide, Oval meatus 3) Neourethra cover with dartos fascia

34. Tubularised Incised Plate Repair (TIP)

35. Onlay Preputial Flap Need for a longer flap usually requires inner prepuce to be dissected from an intact dorsal hood and then straightening maneuvers, the required length of inner prepuce is harvested, tubularized, and transferred ventrally. The native urethra is secured to the corpora cavernosa and then spatulated before anastomosis to the flap to reduce incidence of stricture, anchored to the corpora cavernosa with interrupted stitches. Glans wings are sewn to the flap to create the neomeatus .

36. ONLAY FLAP

37. OIF- Onlay Island Flap

38. TPIF - Transverse preputial island flap

39. Koyanagi repair Koyanagi repair is determined from the outset of the operation based on the surgeon’s impression that VC will lead to urethral plate excision flaps are brought together ventrally, sewn into a single strip, and then tubularized proximally to distally .

40. K o y anagi r epair A The first incision is a distal circumferential incision 5 mm proximal and parallel to the corona of the glans and includes division of the urethral plate. The second encompasses the perimeter of the tissue to be tubularized as the neourethra B The proximal shaft skin is degloved .

41. C, Incision in the dorsal midline creates 2 wings that are brought around either side of the penile shaft to the ventrum D, Alternatively, the ͞neourethral͟ epithelial and subcutaneous tissue collar is left intact and passed to the ventrum via a ͞buttonhole͟ in the dartos fascia.

42. E , Do r sa l run n i n g sutu r e l i n e in p r og r ess f o r tub u lar i z a ti o n o f the neou r e th r a . F , Ventral suture is placed in similar fashion to complete tubularization of the neourethra . In the penis with a deep glanular groove glans, wings are formed and the neourethra is matured at the glans tip with simple interrupted sutures. Alternatively, with a shallow groove, the neourethra may be passed to the tip through a glans tunnel. Consider tunica vaginalis second layer coverage.

43. H, The glans is approximated in the ventral midline in several layers to cover and support the distal neourethra . I, Dorsal penile shaft skin has been incised longitudinally in the midline creating Byar's skin flaps for skin coverage. J, Skin flaps are approximated to the coronal cuff and to the midline

44. Byars Flaps 2 stage flap repair divides the dorsal preputial hood, transposes the resultant flaps ventrally, and reapproximates them in the midline from the native urethral meatus to the glans tip. Glans wings should be widely separated to allow sufficient skin to be placed for later tubularization Pedicle based on superficial dorsal artery

45. 2 s t a g e r epai r s Because the majority of hypospadias can be repaired with a one-stage procedure, the use of two-stage techniques for repair of posterior hypospadias is controversial. Possible indications – Perineal hypospadias Small penis Severe curvature Ist stage – orthoplasty , 2 nd stage – neourethra formation

46. A, proposed initial incisions for penoscrotal /scrotal hypospadias. B, Release of tethering urethral plate and ͞dropping͟ of meatus proximally. C, Curvature is assessed with artificial erection. D, Either midline incision or longitudinal incisions on either side of a deep glanular groove are placed in the glans.

47. E , Mi d li n e l ong it u d i na l i nc i s i o n o f p r epu t ial and do r sa l s ha ft sk i n. F , D ivi de d p r eput i al /do r s a l sh a f t sk in ha s bee n t r an s f er r ed t o the pen ile v e n t r u m.

48. COMPLICATIONS Bleeding and Hematoma Bleeding is the most common complication. Require simple addition of a compressive dressing due to persistent oozing. Significant postoperative bleeding may require exploration Consequences range from simple temporary cosmetic issues to wound or repair breakdown Patients with excessive bleeding , requiring reoperation should undergo evaluation for bleeding diathesis/ dyscrasias ( Horton and Horton, 1988 ).

49. Meatal Stenosis The complication of meatal stenosis is most commonly due to technical issues- fashioning of the urethral meatus with too narrow a lumen or performance of glanuloplasty too tightly. Urethral ( meatal ) dilation or meatotomy may be sufficient for the mildest forms of meatal stenosis . More complex distal urethral stricture also involving the meatus may require a more extensive flap procedure .

50. Urethrocutaneous Fistula Often reported by a parent or caregiver. Confirmed on physical examination with or without voiding, or with retrograde injection of dye such as methylene blue either alone or with glycerin ( Retik et al, 1988 ). Fistula may result from - Distal stricture or meatal stenosis - Failure to invert all epithelial edges at urethroplasty - Devitalisation of tissue - Absence of second-layer coverage. Second layer coverage of the neourethra , has been shown to significantly reduce the fistula rate

51. Infection Infection is an uncommon complication of hypospadias repair. When suspected, culture, incision and drainage, and débridement when indicated are incorporated with appropriate antibiotic therapy. Severe infection may lead to breakdown of the entire repair Urethral Diverticulum Infrequent Urethral diverticula may be associated with distal stricture or meatal stenosis

52. Balanitis Xerotica Obliterans Balanitis xerotica obliterans (BXO) is a chronic inflammatory process of unknown etiology BXO can arise spontaneously or may follow hypospadias repair or may complicate this surgery Recommended use of bladder or buccal mucosal free grafts for repair Recurrent Penile Curvature Late-onset, recurrent curvature has been described as a complication of orthoplasty alone or in conjunction with hypospadias repair Extensive fibrosis of the reconstructed urethra, corporeal disproportion, or both

53. Urethral Stricture Urethral stricture other than meatal stenosis may be a complication of proximal hypospadias repair. The proximal anastomotic site of a tubularized repair such as the TPIF appears to be particularly at risk. Successfully treated with less invasive means such as endoscopic cold knife urethrotomy More extensive stricture may warrant patch with free graft or, preferably, vascularized flap urethroplast y Two-stage mesh-graft urethroplasty using split-thickness skin ,when all other options have failed

54. Intraurethral Hair Growth Intraurethral hair growth is an uncommon complication of hypospadias repair and occurs when hair-bearing skin is incorporated into the repair L aser hair ablation for management of this complication. Repair Breakdown Repair breakdown may occur secondary to devascularization of local tissues or flaps used in urethroplasty Breakdown may also result from glanuloplasty , urethroplasty under tension, excessive use of electrocautery , unidentified vascular pedicle injury during repair, or hematoma formation require débridement of devascularized , necrotic tissue before repair.

55. Hypospadias Cripples D enotes the patient who has undergone multiple, unsuccessful hypospadias repair attempts, with significant resultant penile deformity. They require extensive repair amid scarred and devitalized tissue

56. REOPERATIVE HYPOSPADIASIS REPAIR As a general rule, reoperation for failed hypospadias repair should not be attempted less than 6 months after failure.   Provided sufficient penile tissue of appropriate quality is available, several techniques applicable to primary repair may also be used for reoperation.    Multiple previous failures of hypospadias repair in a patient may be best treated with a 2-stage technique that, at times, incorporates extragenital skin or buccal mucosa. Retrograde urethrogram and/or voiding cystourethrogram for complete urethral visualization may be necessary in complex reoperative hypospadias

57. Immediately Adjacent or Local Tissue Flap Advantages of the TIP urethroplasty include use of local, usually supple tissue with well-established vascularity for urethroplasty and skin coverage cosmetically superior result. The absence of preputial skin in reoperative cases makes TIP urethroplasty an ideal option tunica vaginalis flap as an onlay salvage procedure has fallen into disfavor because of a complication rate of 60% for both meatal stenosis and urethral stricture.

58. In more severe reoperative cases, Free graft bladder mucosa ( Baskin and Duckett , 1994) Buccal mucosa (“dry” or “wet,” onlay or tubularized ) Baskin and Duckett , 1994) combination of the above may be used ( Retik , 1996 ).

59. Split-thickness mesh skin graft as first stage, followed by tubularization as second stage , may be a last resort for the hypospadias cripple Tissue engineered constructs for urethral replacement may have a major impact on reoperative hypospadias repair in future ( Atala et al, 1999 ) Intestinal free flap urethroplasty has also been described for use in reoperative hypospadias ( Bales et al, 2002

60. DIFFERENT TISSUES USED

61. Different grafts Nove-Josserand (1897) used a split thickness skin graft on a metal probe.   Devine and Horton (1961) used preputial full thickness skin graft in single stage repair.   Bracka (1995) used full thickness skin graft in two stage repair.   Mommelaar (1947) used bladder mucosa for urethral reconstruction.   Humby (1941) first described the use of buccal mucosa for urethral reconstruction.

62. BLADDER AND BUCCAL MUCOSAL GRAFTS

63. BLADDER MUCOSA The use of bladder mucosa was first reported by Mommelaar in 1947 Bladder mucosa is harvested by distending the bladder with saline and dissecting the detrusor muscle of the underlying mucosa A rectangular donor site is marked to a size 10% greater than the size of the defect to be repaired. As part of a composite repair, tubularized skin or buccal mucosa may be added to decrease the risk of meatal complications A second layer of neourethral coverage is provided either by a dorsal subcutaneous flap or by tunica vaginalis Immobilization of the patient in the early postoperative period is crucial to graft survival

64. BUCCAL MUCOSA General anesthetic induction and nasotracheal intubation. A self-retaining retractor is then placed in the oral cavity Care taken to avoid Stensen's duct, an appropriate-sized graft is marked on the mucosa of the cheek and/or lip Graft is harvested with sharp dissection superficial to the buccinator muscle. Buccal mucosal edges at the harvest site are approximated with 5-0 chromic catgut The graft is defatted on a sterile cardboard scaffold and then tubularized in a manner similar to that for bladder muc osa

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