Unfavourable results in hypospadias.

Hypospadias urethroplasty is considered difficult as the complications and unfavourable results are not uncommon. At the turn of the century, due to a better understanding of applied anatomy of hypospadias, new techniques were developed which significantly brought down the complication rate. However unfavourable results are still disturbing. An algorithm for selection of surgery has been presented. Forty three secondary surgeries were performed over 3 years for correction of unfavourable results. The urethrocutaneous fistula was the most common (21%) followed by meatal stenosis (14%) and narrow neourethra (14%). Common unfavourable results have been discussed. On the basis of experience with a large number of hypospadias urethroplasty 'tips to avoid or minimise unfavourable results' have been presented. However, one should assess the final outcome of urethroplasty using hypospadias objective scoring evaluation.

INTRODUCTION

Hypospadias is one of the most common congenital anomalies encountered in surgical practice affecting 1:300 live births. However, hypospadias urethroplasty is not a popular surgery amongst the reconstructive surgeons as it should be. This Hypospadias does not draw the attention of the general public because it affects a hidden organ. Further, the affected individuals and parents of the affected children have reservations about seeking advice regarding problems related to sex organs, almost akin to sexually transmitted diseases. This trend is observed more commonly in conservative, poorly literate and low socio-economic population of India.

The social aspect of inability to pass urine in male urinals and the risk of inability to perform sex augments the problems of hypospadias individuals. Further there is always a risk of labelling such patients as a hermaphrodite or a eunuch in the Indian conservative society.

Hypospadias reconstruction does not attract many surgeons because of the high risk of complications of various kinds. Many surgeons give up hypospadias urethroplasty after encountering high rate of unfavourable results and dissatisfaction of patients and parents. It has a very long learning curve too. The complications and unfavourable results of this surgery are so high that urethroplasty and complications become synonymous for some reconstructive surgeons.[1]

SURGICAL GOALS OF HYPOSPADIAS RECONSTRUCTION

Culp and McRoberts emphasised “It is the inalienable right of every boy to be a pointer instead of a sitter by the time he starts school and to write his name legibly in the snow.”[2]

The present day objectives of hypospadias reconstruction are:[3]

Complete removal of chordee to achieve a straight penis in erection

Reconstruction of absent urethra with hairless tissue with obstruction free uniform optimum calibre tube

Placement of new vertical slit urethral meatus of adequate calibre over the tip of the glans

Symmetric and normal looking penis and conical glans

Ability to void urine normally

Aesthetically normal looking penoscrotal complex (male external genitalia)

Ability to perform normal intercourse

A psychologically normal person with a positive body image.

CHOICE OF URETHROPLASTY PROCEDURES

A large number of urethroplasty procedures are available because a majority of the surgeons are not happy with the existing techniques. However, the scenario has changed in the last couple of decades. Multi-stage procedures have been largely replaced with single stage procedures in uncomplicated hypospadias. Duckett[3] in 1998 reported more than 200 original techniques of hypospadias urethroplasties published in literature till then. Today, it must be more than 300. Further there are hundreds of minor and major modifications of these named techniques. This indicates how unsatisfied the hypospadias surgeons are with the results of this reconstructive surgery. In 1997 Duckett et al. proposed an algorithm for primary repair of hypospadias.[4] This is almost obsolete in today's practice. A simple algorithm, which is followed by us is given in Figure 1. This algorithm is based on the one proposed by Sozubir and Snodgrass in 2003.[5]

Figure 1

Algorithm for selection of surgical procedures for various types of hypospadias

In brief, following procedures are used in the author's practice:

Meatal advancement and glanuloplasty

Tubularised incised urethral plate (TIP) urethroplasty

TIP with full thickness graft (FTG) - Preputial or buccal mucosa graft

Two stage Bracka's urethroplasty using preputial or buccal mucosa graft

Tubularised transverse preputial island flap urethroplasty (Asopa's technique)

Preputial onlay patch urethroplasty

Mathieu's flip flap procedure.

EARLY COMPLICATIONS OF HYPOSPADIAS SURGERY

All attempts should be made to avoid the early complications of hypospadias surgery. A large number of them do occur in clinical practice [Table 1]. These complications of urethroplasty are general problems and discussion of these is beyond the scope of this review.

Table 1

Early complications of hypospadias urethroplasty

UNFAVOURABLE RESULTS OF HYPOSPADIAS SURGERY

The results of hypospadias corrective surgery are frequently unfavourable [Table 2] with reported complication rate as high as 50% or above.[36] In a series of consecutive 108 hypospadias surgeries performed over a 3 year period by us, 65 were primary and 43 were secondary surgeries, for correction of unfavourable results. The commonest secondary surgeries were for: Closure of urethrocutaneous fistula 77% (33 out of 43), meatal stenosis 21% (9/43), narrow neo urethra 14% (6/43) and receding urethra 14% (6/43). The detail causes of urethrocutaneous fistulae are not available. This is only to highlight the fact that unfavourable results are very common in hypospadias urethroplasty.

Table 2

Common unfavourable results in hypospadias urethroplasty

Urethrocutaneous fistula

Urethrocutaneous fistula following hypospadias reconstruction is the most feared but anticipated complication. In spite of performing hundreds of urethroplasties, every surgeon explains about the possibility of this complication to the parents. The effectiveness of any urethroplasty technique is judged by the fistula rate. Recent reviews have reported less than 5% fistula rate. Post-surgery fistula could occur because of one or more factors [Table 3].

Table 3

Factors favouring development of urethrocutaneous fistula

In recent times, there has been a better understanding of the occurrence of these complications. Hence the incidence of such secondary problems is likely to reduce significantly. Urethrocutaneous fistula occurs anywhere through the reconstructed urethra [Figure 2]. This occurs because of other concomitant unfavourable conditions. However, in our series of secondary surgeries the common fistula sites were junctional (54.5% of all fistulae) and around corona (30% of all fistulae). The junctional area is prone to fistula formation because of the changing tissue type from normal to neo urethra making it a delicate area. The coronal fistula occurs due to distal narrowing of the urethra in the glanular region. This relative narrowing and lack of extensibility of glanular urethra causes back pressure, which causes breakdown at the coronal region. Management of fistula is summarised in Figure 3. While performing fistula repair, one should calibrate the distal urethra from proximal to distal to ensure the presence of adequate size of the urethra and to exclude any distal obstruction. Any doubtful segment should be laid open and managed appropriately. Urethra should be reconstructed with synthetic absorbable sutures. Water proofing should be provided with a vascular tissue. Skin cover should be provided in such a way that suture should not overlap the urethral suture line. If needed, a tube drain is placed and gentle dressing is applied. A perurethra stent is placed to drain urine for 7-9 days.

Figure 2

Multiple uretherocutaneous fistulae

Figure 3

Management of hypospadias urethrocutaneous fistula: A kaleidoscope

In case of tissue deficiency in the urethra, a staged procedure using preputial or buccal mucosa FTG is used.

The commonest cause of recurrence of fistula is failure to recognise the basic problems causing fistula [Table 3]. Appropriate corrective measures should be taken to manage the cause and there after fistula should be closed [Figure 3].

Meatal stenosis

This is relatively common but avoidable unfavourable result. This occurs due to an attempt to reconstruct the urethra too distally and create circular opening. This may also have occurred following suture dehiscence at the meatus causing secondary contraction and stenosis. Glanular flaps should be sutured so as to not cause compression over the underlying urethra and one should always attempt at raw area free reconstruction. These steps will prevent meatal stenosis.

The management is surgical. Dorsal meatotomy with or without ‘V’ flap is the surgery of choice.[7] If stenosis is too long due to suture dehiscence and scarring, the glanular urethra needs to be reopened and FTG may be required to reconstruct an adequate glanular urethra.

Urethral strictures

Like meatal stenosis, urethral stricture occurs when the basic surgical principles are ignored. Urethral junction is the commonest site of stricture, but it can occur wherever there is a shortage of tissue or a circular scar contraction. This can be prevented by avoiding a circular anastomosis and by avoiding a raw area. Many surgeons prefer to resurface the TIP raw area with FTG named as Snodgraft technique[8] to ensure good primary healing. However, Snodgrass emphasises that the TIP raw area epithelialises spontaneously and does not cause narrowing of neourethra.[9]

There is no place for internal urethrotomy or endoscopic release in these patients.[3] It is best managed by reopening of the stricture urethra, excision of scar, resurfacing with FTG and thereafter reconstruction of urethra.

Megalourethra and urethral diverticulum

When extra tissue is available, for example, in Byer's technique or any other preputial flap techniques, there is always a risk of using too wide a skin for urethral reconstruction resulting in megalourethra or urethrocele or a diffuse diverticulum [Figure 4].[310] This may also occur secondary to meatal stenosis. This can be avoided by using optimum size of skin strip for urethroplasty. The treatment of this complication, once it occurs is surgical longitudinal excision of the urethral circumference. Even the skin cover may require excision to avoid redundant and unsightly penile skin.

Figure 4

Megalourethra with receding urethra

Localised diverticulum usually occurs proximal to a long standing stricture. The diverticulum and its track should be excised along with management of the stricture. A bulbous diverticulum may be associated with a fistula. All of them should be managed together.

Residual chordee

The ventral curvature of the penis may be inadequately corrected [Figure 5a]. One should aim for a straight penis in these patients. In clinical practice 10-150 of residual flexion is acceptable because normal urination and coitus are possible with this. Larger residual chordee will be functionally unfavourable and aesthetically unacceptable. This occurs because of the left over chordee tissue or failure to address the short corporal tissue.

Figure 5

(a) Urethrocutaneous fistulae with residual chordee. (b) Post-operative result after correction of chordee with dorsal corporal placation and closure of fistula

Management includes meticulous surgical excision of chordee and correction of the curvature by dorsal plication [Figure 5b]. Rarely a ventral dermal patch is required to lengthen the ventral corpora.

Retrusive meatus

With many of the older techniques, the urethra was reconstructed only upto corona glandis. If reconstructed glanular urethra fails then the urethral opening will remain at corona.[3] In these cases, the urinary stream will be broad and it may deviate ventrally. With newer techniques, this unfavourable result is relatively uncommon. The management is akin to that of distal hypospadias. This sometimes requires a classical re-do urethroplasty to achieve the objectives of hypospadias repair.

Hair growth from urethra

If hair bearing scrotal skin is used for urethroplasty, the hair may grow inside the urethra. Sometimes the hair may be visible through the fistula or even the meatus, which is aesthetically disturbing [Figure 6]. This may also become a nidus for secondary urethral calculus.

Figure 6

Hair from urethrocutaneous fistula

The management of this complication is excision of hair bearing urethral wall and replacement with preputial skin or buccal mucosal graft. Some surgeons perform preoperative laser hair removal from the scrotal skin and use it for urethral reconstruction.[1112] It is better to avoid the use of scrotal skin for urethra reconstruction as laser epilation may not be 100% effective, and the remaining hair follicles may grow hair. This is not a very reliable solution.

Balanitis xerotica obliterans

This is one of the most dreaded unfavourable results of hypospadias surgery. The cause is not well known. This is believed to be a chronic inflammation causing hypopigmentation and scarring [Figure 7]. This results in recurrent severe stenosis and stricture and a difficult condition to manage.[1314] When local steroid injection fails, surgical excision of BXO lesion is recommended.

Figure 7

Balanitis xerotica obliterans

Penile torsion

Post-operative penile torsion is a disturbing unfavourable result of hypospadias urethroplasty. Torsion may be an inherent problem which is usually anti-clock to the left. Postoperative penile torsion is common with pedicle and island preputial flaps. It is more with pedicle flap. In the evolution of newer techniques, the preputial flap is islanded to reduce the risk of abnormal penile torsion. Most of the right handed surgeons tend to rotate the flap from the right side of penis; hence the postoperative torsion is mostly clockwise. With newer techniques like TIP and Bracka's urethroplasties the torsion is much less.

Torsion of 20-30° in flaccid state may get corrected on erection and may not cause functional problems. However rotation beyond 30° requires surgical correction. Degloving of penis, release of soft tissue causing torsion and resuturing of skin in reverse rotated position corrects the torsion to a large extent.

Microphallus

Microphallus or micropenis is anatomically normally formed penis. When the stretched length of the penis is 2.5 standard deviation (SD) below the norm for a particular age, then it is labelled as micropenis.[15] The definition excludes hypospadias and ambiguous genitalia. However, after chordee correction and urethroplasty if the penis is short then it can be labelled as micropenis.

In our series of 36 patients of hypospadias, in 23 (65%) patients stretched penile length was 2.5 SD below the norm for the age, amounting to microphallus. Also, the penile length had no relationship with the severity of hypospadias.

In hypospadias, if microphallus is present it is an inherent abnormality and it is congenital. This occurs because of inadequate androgen stimulation of the target organs or because of insensitivity of the target organ to the available androgen.

The treatment should begin early. It is treated with local and systemic testosterone. There are reports that 90% patients of micropenis gain normal penile length with testosterone if started on time. If there is no response to hormonal therapy then surgical options are considered.

Penoscrotal transposition

In proximal hypospadias, the scrotum overrides the base of penis [Figure 8]. In our series of 108 patients, 5 of them had penoscrotal transposition. This was addressed by bilateral V-Y advancement, Z-plasty or interpolation flap.

Figure 8

Penoscrotal transposition with proximal hypospadias

Bifid scrotum

Scrotum is divided in two parts by the midline groove in proximal hypospadias. After urethroplasty the scrotum may remain bifid in some cases [Figure 9a]. This is managed by designing appropriate Z-plasty. In many cases there may be a need to sacrifice part of scrotal skin in the groove. Otherwise, it may remain redundant. The midline septum is reconstructed by approximating the medial surfaces of the tunica vaginalis and the scrotal skin is sutured incorporating Z-plasty. This is a simple procedure and is always effective.

Figure 9

(a) Adherent graft after first stage Bracka's repair. (b) Intraoperative picture of completion of urethra reconstruction. (c) Post-operative 2nd stage Bracka's repair with a urinary stent and drain in situ. (d) Fistula free post-operative result

Hooded prepuce and redundant penile skin

It has always been taught that one should preserve the tissues during hypospadias urethroplasty as in case of complications the available tissue will be useful. This dictum usually leaves redundant skin over the penis. This occurs more often in preputial flap and Byer's techniques. Similarly, one is likely to leave the hooded prepuce which is a hallmark of hypospadias. This leads to aesthetically unfavourable results.

With newer techniques these problems are seen less often. However, these require sacrificing the extra tissue so as to achieve normal looking penis.

Psychosocial problems

The primary aim of hypospadias reconstructive surgery is to provide functional and aesthetic penis. Any abnormality of the external genitalia has a significant bearing on the psychology of the patient. The parents on the other hand are concerned about the sexual capabilities and the ability to discharge normal sperms for reproducing a child. This has been observed in all cases of hypospadias especially in those who have undergone reconstruction late or those who have repeated complications.[16]

A well designed and thorough counselling protocol should be extended to all patients and parents by the surgeon himself and also by a trained counsellor. The child should develop a positive body image. The details of the cause and the future impact of this congenial abnormality should be discussed with the parents so that they could provide effective support to the child.

ASSESSMENT OF SURGICAL OUTCOME

These are the majority of unfavourable results or complications which should be anticipated with hypospadias surgery. Holland and Smith 2001 developed hypospadias objective scoring evaluation system to assess the surgical outcome of hypospadias surgery. Meatal location, meatal shape, quality of urinary stream, angulation on erection and fistula are five parameters used for assessment.[17] This objective scoring facilitates the evaluation of various techniques. Its use has also been suggested to improve communication between everyone involved in the management including parents.

TIPS TO MINIMISE UNFAVOURABLE RESULTS

As a hypospadias surgeon one should aim at achieving all the objectives of hypospadias surgery. Following tips may minimise the unfavourable results:

Pre-operative and postoperative counselling to patients and parents should be given during every visit to the clinic. Details of deformity, possible causes, and surgical procedures available and to be done, surgical outcome and long term results should be part of regular discussion.

Urethral diameter: One should plan to reconstruct optimum diameter of urethra-neither too narrow nor too wide as both have their own problems as mentioned.

Urethra should be devoid of folds, valves or rugosity

Length of urethra: Neourethra should be of optimum length. Too long urethra will cause folds, megalourethra and generate turbulence causing failure of urethroplasty.

Urethral stability: Reconstructed urethra should be well adherent to the ventral surface of corpora cavernosa. This is automatically achieved with TIP and Bracka's urethroplasty. Probably this is a major and significant difference between newer successful techniques vis a vis the older preputial flap techniques [Figure 9a, 9b].

Water proofing: Preputial dartos flap, tunica vaginalis flap, spongiosum flap and scrotal dartos flaps have been used for water proofing. This separates the urethra from the skin and also gives ventral support to the reconstructed urethra.

Use of tumescence and haemostasis: Tumescence with 1:200,000 IU adrenaline saline with lignocaine gives effective haemostasis. This obviates the need for tourniquet. This also facilitates dissection of the flaps.

Use of drain: In case of bleeding at the end of surgery, a drain should be placed subcutaneously to avoid haematoma formation. The author has used tube drain in 21% of his hypospadias cases, which is removed after 24-48 h [Figure 9c].

Urinary diversion: Perurethra PVC tube is used as stent and for urinary drainage for 7-10 days. Suprapubic cystostomy is avoided as it has its own morbidity and causes unwanted scar without improving the overall result.

Aesthetic considerations: While performing urethroplasty due consideration is given to avoid dog ear or unwanted tag of skin [Figure 9d]. Majority of the elements of aesthetic considerations are taken care of while performing the primary surgery itself. One should achieve normal looking glans with or without prepuce with vertical slit like urethral meatus on the tip of the glans [Figure 10].

Figure 10

Aesthetically acceptable penis after distal hypospadias urethroplasty with vertical slit like urethral opening

Choice of urethroplasty: This is based on the surgeon's training and experience. The technique followed is well drawn in the algorithm [Figure 1]. Secondary surgeries are decided based on the problems and assessment. Some of the guidelines have been mentioned in Figure 2.

CONCLUSION

The complications and unfavourable results of hypospadias surgery are unending. The increasing list of urethroplasty techniques and their modifications by every surgeon proves that the problem is continuing. The developments during the past two decades have reduced these complications and unfavourable outcome to a large extent. One should aim at achieving cent per cent complication free urethroplasty, to provide a normal looking functional penis and an individual with normal self-esteem and positive body image.