Articles

Hypospadias Classification and Repair: The Riddle of the Sphinx

By: and Antonella Giannantonilowast

Published online: 01 December 2011

Abstract Full Text Full Text PDF (80 KB)

Refers to article:

Trends in Hypospadias Surgery: Results of a Worldwide Survey

Alexander Springer, Wilfried Krois and Ernst Horcher

Accepted 11 August 2011

December 2011 (Vol. 60, Issue 6, pages 1184 - 1189)

Hypospadias is the most common congenital anomaly of the penis, affecting 0.4–8.2 of 1000 live male babies [1]. The term hypospadias stems from two Greek words: hypo, which means “below,” and spadon, which means “hole.” The anomaly is characterized by a urethral meatus ectopically located proximal to the normal place on the ventral side of the penis. Different anatomic presentations can be observed. The position of the urethral meatus can be classified as anterior or distal (glandular, coronal, or subcoronal; 60–65% of cases), middle (midpenile; 20–30% of cases), or posterior or proximal (posterior penile, penoscrotal, scrotal, or perineal; 10–15% of cases) [2]. The subcoronal position is the most common. Proximal cases are considered severe and can be associated with chordee [2]. The term chordee derives from the Greek word chorda, which means “string” or “rope” and indicates the ventral curvature of the penis.

Clinical symptoms vary and depend on the severity of the disease. In mild hypospadias with a urethral meatus located on the glans, a normal urinary flow can be maintained. In cases with a stenotic meatus, a weak urinary flow can be observed. Children with proximal hypospadias with penile curvature might not be able to void while standing. Actually, we do not know precisely what degree of penile curvature in children will inhibit sexual intercourse in adulthood or what the long-term psychosexual outcome will be in these patients.

Many questions still arise on several aspects of the disorder, such as how to objectively define the severity of the disease, what is the real benefit of preoperative hormonal stimulation, and particularly, what is the best surgical correction to adopt according to type and severity of hypospadias [3]. Generally speaking, the assessment of severity is based on meatal position, quality of the urethra and urethral plate, and presence or absence of penile curvature. As suggested by Snodgrass et al, patients with hypospadias present with a continuum of abnormalities ranging from simple glanular forms to perineal presentations with different degrees of penile curvature, and it is not clear in what situation the adjective severe should be applied [3]. The assessment of severity is obviously influenced by subjective judgment, which can differ among surgeons. Is it possible to create a more objective severity classification taking into account meatal position, quality of urethra and urethral plate, and presence or absence of penile curvature? Or as some maintain, can the assessment of hypospadias severity be adequately performed only in the operating room [3]? Indeed, the solution is of great importance, as the choice of surgical repair is based on it.

While the goals of hypospadias surgery have been clearly stated—create a functional penis adequate for sexual intercourse, produce a correct urethral reconstruction to allow the patient to stand to urinate, and offer satisfactory cosmetic results [4]—the choice of urethroplasty is still a matter of debate. Many types of urethroplasty can be performed with the goal of adequately correcting the congenital disorder (Duplay/tubularized incised plate [TIP], Mathieu, onlay, Koff, Bracka, Duckett, and Koyanagi/Hayashi) [3] and [5]. In addition, Nesbit, tunica albuginea plication, ventral plasty, and many other techniques have been developed to correct the chordee [3] and [5]. To date, also in systematic reviews of hypospadias corrections, no urethroplasty technique appears to be definitively superior [4] and [6]. What we know is that in most cases, hypospadias can be repaired in a single-stage operation that includes meatal advancement and glanuloplasty (MAGPI), glans approximation procedure, and tubularization following incision of the urethral plate (TIP) [5]. A two-stage operation may be required for more severe forms of penoscrotal or perineal hypospadias [5].

But what is the actual evidence from the literature on the preferred method of urethroplasty by surgeons to correct primary hypospadias? In a previously reported North American survey, Cook et al. found that 90% and 83% of pediatric urologists used a TIP procedure to repair distal and midshaft hypospadias without penile curvature, respectively, and only 6% preferred an onlay island flap [7]. In that study, the preferred surgical procedure to correct proximal hypospadias without penile curvature was, again, one-stage repair (TIP repair and onlay island flap repair were each preferred by 43% of participants). When considering the preferred surgical urethroplasty to correct proximal hypospadias with severe penile curvature, 40% of urologists in that study preferred the Duckett repair, 11% preferred the onlay island flap, and 3% preferred the TIP repair [7].

What emerges from the interesting study of Springer and coauthors is consistently different [8]. The authors tried to identify actual international trends in the management of hypospadias by inviting pediatric urologists, pediatric surgeons, urologists, and plastic surgeons worldwide to participate in an anonymous multiple-choice online questionnaire. Completed questionnaires were obtained from 377 participants from 68 countries. From the results, it appeared that in distal hypospadias (subcoronal to midshaft), TIP repair was the preferred method of 52.9–71.0% of participants. MAGPI was the preferred method to correct glandular hypospadias. The two-stage repair was the preferred method to correct proximal hypospadias for 43.3–76.6% of participants, which is in contrast to the results of the North American survey in which one-stage procedure was the method of choice [7]. These data are also of particular interest considering the recently published excellent long-term results of island flaps or tubes in the correction of proximal hypospadias, which should convince surgeons to use these techniques in hypospadias repair [9].

Once again, we have to admit that publications do not represent what happens in everyday clinical practice, and there may be additional factors influencing the choice of surgical technique in hypospadias correction. As suggested by Springer et al, “personal taste, training, experience and personal success” lead surgeons every day to choose a proper surgical technique for hypospadias repair, and surprisingly, this choice is not influenced by the personal number of surgical repairs per year [8].

We all well know that to establish the best treatment for a pathologic condition requires evaluation of the reported results. To date, the reports on outcome of hypospadias repair are sparse, and results of prospective studies with long follow-up are still lacking. In addition, functional and cosmetic outcomes are generally assessed in a subjective manner, and a standardized evaluation of these outcomes is urgently needed.

Use of the same language is necessary, particularly in the surgical field. We need to better define moderate and severe in regard to penile curvature and high quality in regard to urethral plate. Perhaps having more reliable results and using a more comprehensive classification could help solve the Riddle of the Sphinx in hypospadias classification and correction.

Conflicts of interest

The author has nothing to disclose.

References

  • [1] L.S. Baskin, M.B. Ebbers. Hypospadias: anatomy, etiology, and technique. J Pediatr Surg. 2006;41:463-472 Crossref.
  • [2] A.R.C. Leung, W.L.M. Robson. Hypospadias: an update. Asian J Androl. 2007;9:16-22 Crossref.
  • [3] W. Snodgrass, A. Macedo, P. Hoebeke, P.D.E. Mouriquand. Hypospadias dilemmas: a round table. J Pediatr Urol. 2011;7:145-157 Crossref.
  • [4] M. Castagnetti, A. El-Ghoneimi. Surgical management of primary severe hypospadias in children: 20-year review. J Urol. 2010;184:1469-1474
  • [5] Y. Hayashi, Y. Kojima. Current concepts in hypospadias surgery. Int J Urology. 2008;15:651-663
  • [6] W. Snodgrass. Hypospadia reporting—how good is the literature?. J Urol. 2010;184:1255-1256 Crossref.
  • [7] A. Cook, A.E. Khoury, C. Neville, W.A. Farhat, J.L. Pippi Salle. A multicenter evaluation of technical preferences for primary hypospadias repair. J Urol. 2005;174:2354-2357 Crossref.
  • [8] A. Springer, W. Krois, E. Horcher. Trends in hypospadias surgery: results of a worldwide survey. Eur Urol. 2011;60:1184-1189 Abstract, Full-text, PDF, Crossref.
  • [9] W. Snodgrass, N. Bush. Tubularized incised plate proximal hypospadias repair: continued evolution and extended application. J Pediatr Urol. 2011;7:2-9 Crossref.

Footnotes

Department of Urology and Andrology, University of Perugia, Opsedale Santa Maria della Misericordia, Perugia, Italy

lowast Department of Urology and Andrology, University of Perugia, Opsedale Santa Maria della Misericordia, Perugia, Italy. Tel. +39 075 5784416; Fax: +39 075 5784416.