A Case For 2-stage Repair of Perineoscrotal Hypospadias With Severe Chordee

A Case For 2-stage Repair of Perineoscrotal Hypospadias With Severe Chordee

0022-5347/02/1684-1727/0 THE JOURNAL OF UROLOGY® Copyright © 2002 by AMERICAN UROLOGICAL ASSOCIATION, INC.® Vol. 168, 1727–1729, October 2002 Printed...

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0022-5347/02/1684-1727/0 THE JOURNAL OF UROLOGY® Copyright © 2002 by AMERICAN UROLOGICAL ASSOCIATION, INC.®

Vol. 168, 1727–1729, October 2002 Printed in U.S.A.

DOI: 10.1097/01.ju.0000027276.83141.8b

A CASE FOR 2-STAGE REPAIR OF PERINEOSCROTAL HYPOSPADIAS WITH SEVERE CHORDEE MEYER D. GERSHBAUM, JEFFREY A. STOCK

AND

MONEER K. HANNA

From the Departments of Urology, Schneider Children’s Hospital, Long Island Jewish Medical Center, New Hyde Park, New York, and the Children’s Hospital of New Jersey, St. Barnabas Health Care System, Livingston, New Jersey

ABSTRACT

Purpose: In the majority of children hypospadias can be corrected in a single stage procedure. However, there is a subgroup of patients with perineoscrotal meatus and severe chordee who frequently exhibit some degree of penile scrotal transposition. It is this subgroup of patients in whom a critical review of the long-term results is examined. Materials and Methods: Between 1980 and 1995, 1,934 children underwent repair of hypospadias and chordee. Of these patients 51 had perineoscrotal hypospadias with severe chordee and 40 underwent single stage repair (23), which included either full thickness skin graft urethroplasty (6), a proximal Thiersch procedure with distal free preputial skin graft (7) or an island tubularized flap (10). The chordee were corrected by either Nesbit or tunica albuginea plication. A 2-stage repair was performed in the remaining 11 children. Stage 1 consisted of chordee repair by either dermal (5) or tunica vaginalis (6) grafting of the ventral tunica albuginea surface, while stage 2 urethroplasty was performed 6 months later. These 2 groups were compared in regard to function status and cosmetic results. Results: Of the patients 34 (61%) were available for a greater than 5-year followup. An excellent outcome (terminal meatal voiding, near normal appearance, no complications) was obtained in only 5 (21%) patients of the single stage repair group, while satisfactory (subterminal meatus, irregularities in meatal, glandular or penile skin) or complicated results were obtained in 4 (17%) and 14 (61%), respectively. The overall complication rate was 61% (14 of 23 patients), including fistula, urethral diverticulum, distal breakdown or stricture formation. Recurrent chordee was noted in 5 (22%) children. Of the 2-stage group excellent results were obtained in 7 (63%), patients, while satisfactory and complicated results was seen in 2 (18%) and 2 (18%), respectively. Overall complication rate was 18%, and included fistula and diverticular formation. However, no recurrence of the initial chordee was noted. Conclusions: Our long-term followup suggests that ventral grafting of the corporal wall in patients with severe chordee as a staged procedure is superior to the dorsal plication or Nesbit procedure. The overall functional and cosmetic results are excellent with the 2-stage compared to the single stage repair. KEY WORDS: hypospadias, urogenital surgical procedures; penis

Hypospadias, a congenital defect, is defined as an abnormal urethral opening anywhere along the penile shaft and down onto the perineum. Throughout the 125-year history of hypospadias repair the goal of the pediatric urologist has been to develop a universally applicable single stage technique that offers good functional and cosmetic results with a low complication rate. However, given the broad spectrum of abnormalities seen with this condition, that task seems difficult. Although in the majority of children born hypospadias can be corrected with a single operation, there exists a subgroup that may require a staged surgical approach. These children are born with a perineoscrotal meatus and associated severe chordee. In addition, they often exhibit some degree of penile scrotal transposition and a small phallus. In this study we describe our long-term followup of this select group of children treated with a single or staged surgical repair. MATERIAL AND METHODS

From 1980 to 1995, 1,934 children underwent hypospadias and chordee repair by 2 of the authors (M. K. H., J. A. S.). Of these patients 51 had perineoscrotal hypospadias associated with severe chordee. A single stage repair was done in 40

children (group 1), which included a free full-thickness skin urethroplasty, a proximal Thiersch skin tubulerization with distal free preputial skin graft or an island tubularized flap. The associated chordee was corrected by Nesbit or tunica albuginea plication which was confirmed by intraoperative artificial erection. Group 2 consisted of 11 children who underwent a 2-stage repair. Stage 1 included induction of an artificial erection to evaluate degree of curvature, penile degloving with correction of chordee by release of skin attachment, excision of the ureteral plate and either dermal or tunica vaginalis grafting of the ventral tunica albuginea surface. The patients were rehospitalized 6 months to 1 year after the first operation for stage 2 urethroplasty. The duration allowed for adequate vascularity and tissue compliance to return, therefore decreasing the risk of contraction or impaired healing. The urethroplasty was performed using the standard Thiersch-Duplay technique. A Duplay tube 12 mm. in diameter was formed and tapered distally. Several layers of subcutaneous tissue were placed over the neourethra along with a tension-free closure over the repair. Urinary diversion was usually accomplished with a suprapubic catheter for 10 to 12 days. Transposition of the penis was performed at that time if present. Adjuvant testosterone therapy (50 mg. intramus-

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CASE FOR 2-STAGE HYPOSPADIAS REPAIR

cular injection 3 to 4 weeks before repair) was used in the setting of small penile length. The 2 groups were then compared in regard to long-term functional status, cosmetic results and complications. We excluded patients with short-term followup because urethral fistula or diverticular formation may take years to form and may not be diagnosed until the patient is toilet trained. In addition, stricture formation, particularly with full-thickness free skin graft, may also occur many years later. RESULTS

Routine evaluation of greater then 5 to 15 years was available for 34 (61%) of the 51 children with perineoscrotal hypospadias. Of the 40 group 1 patients 23 (58%) were available for followup, while all 11 (100%) group 2 children were available. The cosmetic results, although difficult to quantify, were divided into excellent, satisfactory or poor. Of group 1 only 5 (21%) patients had excellent results (terminal meatus with no chordee and near normal appearance), while satisfactory (subterminal meatus or irregularities in the glandular or penile skin) and poor (persistent hypospadias or chordee and/or complications) results were seen in 4 (17%) and 14 (61%), respectively. Excellent results were seen in 7 (63%) group 2 patients, while satisfactory and poor results were obtained in 2 (18%) and 2 (18%), respectively. The functional outcome was determined based on the presence or absence of terminal voiding, spraying or misdirection of the urinary stream as noticed by parents or physician. Uroflowmetery was not used routinely in followup evaluation of these children. Of group 1 patients 5 (21%) demonstrated normal terminal voiding while 18 (79%) did not. Of group 2 children 7 (63%) were able to void with a normal stream and 4 (37%) had difficulty with terminal voiding and spraying. Complications included urethral fistula or diverticular formation, recurrent chordee, distal urethral breakdown and stricture formation (see table). The overall complication rate in group 1 was 14 (61%), while group 2 had 2 (18%) complications, which included 1 urethral fistula and 1 diverticulum. However, chordee did not recur in group 2 on long-term followup. DISCUSSION

The goal of the pediatric urologist is reconstruction of the penile shaft with a terminal meatus that allows for a forward directed stream and normal coitus. The most challenging of these defects is perineoscrotal (proximal) hypospadias associated with severe chordee. In the early 1950s the standard approach to the defect was a 2-stage surgical repair using multiple techniques.1–3 However, as time progressed the tide changed and surgeons such as Devine and Horton4 and Broadbent et al5 popularized the single stage approach. This approach had many potential benefits over its predecessor, such as using unscarred skin for primary repair, decrease in cost, anesthetic risk, anxiety, and convenience to the patient, parent and surgeon. As in other cases of surgical correction, a unified approach to all patients is not always feasible. Of all hypospadias cases 20% are in the category of a proximal defect, and can be corrected in a 1-stage fashion.6 However, in a subset of paComplications of 1-stage repair Complications

No. Pts. (%)

Fistula Chordee Urethral diverticulum Distal breakdown Anastomotic stricture

9 (42) 5 (24) 3 (14) 3 (14) 1 (5)

tients with perineoscrotal hypospadias and associated severe chordee a single stage repair may not yield acceptable final results. Koyanangi,7 Glassberg8 and Emir9 et al reported complication rates of 20% to 50%. In our review the children who underwent a 2-stage repair had superior cosmetic and functional results with fewer complications, whereas in the single stage group only 21% of children demonstrated terminal meatal voiding with no complications (an excellent result). At our 15-year followup 63% of group 2 children had excellent outcomes and similar findings were seen in regard to the complication rate. In group 1 the overall complication rate was 61% versus 12% in group 2, particularly there was no recurrent chordee in any group 2 patients. In most cases chordee is due to skin and dartos fascia abnormalities that are corrected by simple degloving of the penile shaft and lysis of adhesive bands. However, in patients with perineoscrotal hypospadias the chordee is almost always secondary to corporal disproportion and the penis is small. Our experience suggests that ventral grafting of the corporal wall in patients with severe chordee is superior to the dorsal or Nesbit plication. Ventral grafting, as opposed to plication, maintains penile length, improves cosmesis and decreases risk of chordee recurrence. Although ventral dermal grafting can be combined with urethroplasty in a single stage, we believe that surgical morbidity is increased and have not adopted this approach. CONCLUSIONS

In the majority of children hypospadias can be surgically corrected with a 1-stage technique. When the option exists it is the preferred method of treatment as long as the goal of terminal voiding can be achieved. However, there exists a subgroup of patients with perineoscrotal hypospadias and severe chordee who will best benefit from a staged procedure consisting of initial ventral grafting followed by a separate urethroplasty. The overall functional and cosmetic results are superior with 2-stage repair group compared to single stage repair. This finding is also true in regard to the overall morbidity as seen in 1-stage hypospadias repair. The 2-stage technique will deliver the best overall results when used in patients with perineoscrotal hypospadias and severe chordee. REFERENCES

1. Brown, D.: A comparison of Duplay and Denis Browne techniques for hypospadias operation. Surgery, 34: 787, 1953 2. Bayers, L. T.: Techniques for consistently satisfactory repair of hypospadias surgery. Gynec Obst, 100: 184, 1955 3. Duckett, J. W.: Hypospadias. In: Campbell’s Urology, 7th ed. Edited by P. C. Walsh, A. B. Retik, E. D. Vaughan, Jr. and A. J. Wein. Philadelphia: W. B. Saunders Co., chapt. 68, pp. 2093– 2116, 1998 4. Devine, C. J. and Horton, C. E.: One stage hypospadias repair. Acta Chir Plast, 26: 196, 1961 5. Broadbent, R., Woolf, R. and Tosku, E. A.: Hypospadias, one stage repair. Plast Reconst Surg, 26: 164, 1951 6. Retik, A. B., Bauer, S. B., Mandell, J., Peters, C. A., Colodny, A. and Atala, A.: Management of severe hypospadias with a 2-stage repair. J Urol, 152: 749, 1994 7. Koyanangi, T., Matsuno, T., Nonomura, K. and Sakakibara, N.: Complete repair of severe penoscrotal hypospadias in 1 stage: experience with urethral mobilization, wing flap-flipping urethroplasty and “glanulomeatoplasty.” J Urol, 130: 1150, 1983 8. Glassberg, K., Hansbrough, F. and Horowitz, M.: The Koyanangi-Nonomura 1-stage bucket repair of severe hypospadias with and without penoscrotal transposition. J Urol, 160: 1104, 1998 9. Emir, H., Jayanthi, V. R., Nitahara, K., Danismend, N. and Koff, S. A.: Modification of the Koyanangi technique for the single stage repair of proximal hypospadias. J Urol, 164: 973, 2000

CASE FOR 2-STAGE HYPOSPADIAS REPAIR

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DISCUSSION

Dr. Larry Baskin. When do you think that a 2-stage repair is necessary? Dr. Moneer Hanna. In the patient with severe hypospadias and chordee, when you do a Nesbit or a tunica albuginen plication, you shorten the penis, whereas if you put a free graft tunica or dermal graft, I think you lengthen the penis. So even after you give them testosterone, it would be better to do it in 2 stages. Now, whether you make the urethra out of a tube or a free graft, I think the tube obviously as a vascularized flap would be a much better option if you have the courage to put a tube flap on top of a free graft. But I personally would do it in 2 stages once I graft the corporal bodies. Another group in whom I would do a 2 stage is, I hate to use the term, the “hypospadias cripple” who has been operated on 5, 6 or 7 times, and the plate is completely gone. I do not think even buccal mucosa is good in these patients. You are better off to do it in 2 stages. Dr. Antoine Khoury. I agree with Doctor Hanna that in patients with severe chordee a plication is not an adequate solution for them because as the penis grows, if the cause of the chordee is disproportionate between the ventral and dorsal growth of the penis, that process is going to continue. We have not altered the under surface of that corpus. But what I would like to propose is vaginalis flap rather than a free graft of tunica vaginalis or dermal graft. Then you can lay a urethral tube or onlay on top of that in a single stage rather than having to do 2 stages.