British Joumal of Plastic Surgery U97::\), 31,244-249

THE REPAIR OF EXSTROPHY OF THE BLADDER

By J. C. H. VANDER MEDLEN, M.D., and G. G. THOMAS, F.R.C.S., F.R.C.S.E. Department of Plastic and Reconstructive Surgery, Academisch Ziekenhuis Rotterdam-Dijkzigt, Rotterdam, The Netherlands and the Department of Paediatric Urology, Academisch Ziekenhuis

Rotterdam-Sophia Kinderziekenhuis, Rotterdam, The Netherlands

OvER the last ro years, 25 patients with exstrophy of the bladder have been treated at this centre. Many had received initial surgical treatment elsewhere and were referred for correction of remaining deformities. Usually no attempt had been made to recon­struct the abdominal wall and pubic area by adequate transposition of skin nor to correct the dorsal penile contracture by release of the corpora cavernosa. All such patients had intensive scarring in the pubic area and all were unable to achieve normal erection. As a result of our collaboration and the experience we gained in treating these secondary deformities, and as more cases were referred to us at birth, we have developed the integrated treatment to be described.

While there may be cases in which urinary diversion is the only approach, we felt that every attempt should be made to close the bladder and reconstruct the abdominal wall as soon as possible after birth. The penis is also deformed and we must restore natural erection, replace the missing skin, reconstruct the urethra and reconstruct the glans. As many of these procedures as possible should be carried out at the first operation. Finally, at a later age, urinary continence must be restored.

FIRST OPERATION

Within days of birth if the bladder is not closed, metaplasia of the bladder mucosa occurs and the bladder wall becomes oedematous and ultimately fibrotic. The ideal time for the first operation is as soon after birth as the infant is fit. Skin has its maximum extensibility at this age and this is an added reason for early operation. Wherever possible all of the following procedures are carried out at this operation.

Reconstruction of the bladder. When the exstrophy is large, it is not difficult to close the bladder which is readily mobilised from the surrounding abdominal wall. The only point where a little difficulty may be encountered is at the site of the bladder outlet where the tissue is fibrous and tends to be fixed. The bladder is then closed over a fine uretlual splint and a small suprapubic cystostomy.

When the bladder is small, we still attempt to close it although many authorities advocate urinary diversion in such cases. The bladder resembles an inverted purse which cannot be everted because the fibrous ring at the mucocutaneous junction is too tight. When this ring is excised, the bladder can be stretched and especially when treatment has been delayed, extra capacity can be created by longitudinal incisions in the bladder muscle down to mucosa and again stretching the bladder. This manoeuvre can create sufficient material to close the bladder with a small cavity and return it to the abdomen. Again difficulty is encountered at the bladder outlet and rather than

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THE REPAIR OF EXSTROPHY OF THE BLADDER 245

tighten this area, a free graft of anterior rectus sheath is employed as part of the anterior bladder oudet.

It is important that at the initial closure no attempt is made to produce tightness at the bladder oudet. It must be accepted that the sphincter is deficient and an overlap of tissue to create a sphincteric action results in narrowing which will result in obstruc­tion, infection and stone formation. No attempt is made to bring the pubic rami together as even if bilateral sacral osteotomies are performed and the rami sutured together, separation of the rami recurs within weeks.

In recent years, it has become obvious that the long term results of urinary diversion are not good in that there is progressive deterioration of the upper urinary tracts even in uncomplicated cases. As a result, we feel that it is worthwhile to attempt to conserve the bladder remnant and to close all cases primarily even when the bladder is small.

Restoration of a normal penile erect position and reconstruction of the urethra. If untreated, the penis when erect lies on the abdominal wall making inter­course impossible. This is due to the shortage of skin and the short mucosal strip on the dorsum. Reconstructing the urethral canal on the dorsum does nothing to help and may increase the deformity (Hanna and Williams, 1972). It is preferable to construct the urethra on the ventral aspect. The mucosal strip is raised from the corpora cavernosa thus pardy releasing them for natural erection, the corpora are split in the midline (an

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FIG. 2. The authors' modification of the scrotal visor flap.

246 BRITISH JOURNAL OF PLASTIC SURGERY

easy manoeuvre in a young child) and the mucosal strip passed ventrally and attached to a tubular invagination of the lining of the prepuce (Fig. I). This also allows the bladder neck to slip back thus improving the chance of later continence.

The skin shortage on the dorsum may be replaced by the excess skin on the ventral aspect, but this is valuable material for further urethral reconstruction and we prefer a modified scrotal visor flap (Khanna, 1973) (Fig. 2). The circumferential incision around the base of the penis further frees the corpora for natural erection.

FIG. 3· The umbilicus lies at the upper border of the bladder illustrating that the skin defect is bigger than first expected.

Restoration of the abdominal wall and pubic area. At first we under­estimated the shortage of skin in the lower abdominal and pubic area. In most patients the umbilicus is found at the upper border of the everted bladder (Fig. 3) and con­sequently the entire skin of the central part of the lower abdominal wall is lacking. Initially, in order to bring skin to the pubic area, use was made of a scrotal visor flap. This flap did much to improve the appearance but little to correct the existing shortage, being too small to cover the entire pubic area. A groin flap was first used unilaterally but later when it had become clear that these flaps are extremely suitable for this purpose, bilateral flaps were used in exactly the same manner as later described by Ship and Belzer (1972) and Owsley and Hinman (1972). Despite the improvement which was obtained in this way, we still felt that in some patients even more skin was needed. This was solved by a large Z-plasty and a Z-plasty and 2 groin flaps are now used routinely for the reconstruction of the abdominal wall and pubic area (Fig. 4). A typical operative series is shown in Figure 5·

THE REPAIR OF EXSTROPHY OF THE BLADDER 247

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Reconstruction of the glans. This should never be forgotten as the spatulated appearance is the only stigma which is easy to correct. Much better cosmesis can be obtained by closing or by resecting the groove.

SUBSEQUENT OPERATIONS

Lengthening the urethra. Since the foreskin is still virtually intact, the urethra may be readily lengthened to as near the tip as possible by the technique previously described (van der Meulen, 1977).

Restoration of continence. Incontinence is the greatest single problem in these children as the bladder outlet and the urethra can never be made completely normal. Even in highly selected groups in which only the large bladder extrophies are closed, the continence rate ranges from only 30 to 50 per cent (Williams, 1969; Jeffs, 1972).

No attempt is made to produce continence at the initial closure. Even though the sphincter is abnormal or absent, it is possible to create continence later by procedures which artificially elongate the urethra. The most usual method is that of Young-Dees Leadbetter, in which the urethra is elongated approximately 3 em by utilising flaps from the base of the bladder (Leadbetter, 1964). At the same time, it is usual to re­implant the ureters, both to control reflux, which is common in these cases, and also to facilitate the construction of a new urethra from the bladder base. This procedure requires a bladder of adequate size, and at present, whether the bladder which is small at the initial closure will grow enough, is not known. So far, the rate of growth of our small bladders is encouraging.

If these methods fail, then urinary diversion must be performed, but it is better to delay this as long as possible provided there is no deterioration of the upper urinary tract.

SUMMARY AND CONCLUSIONS

Exstrophy of the bladder is a complex deformity, best treated from the outset by collaboration between plastic surgeons and paediatric urologists. Operation should be carried out as early in life as possible and the first stage should include closure of the

248 BRITISH JOURNAL OF PLASTIC SURGERY

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FIG. 5· Typical operative series. (a) and (b ) Dorsal and ventral views. (c) The scrotal viso.r flap, the 2 g.roin flaps and the Z-plascy have been designed. (d) The bladder has been closed and the flaps .raised. (e) The visor flap has been transposed following the translocation of the mucosal st.rip. (f) Only sub­cutaneous sutures are used fo.r closure. (g) and (h) Postoperative views to show the position of the urethral

opening in the preputial lining and the freedom of urinary flow.

bladder, reconstruction of the abdominal wall, ventral transposition of the urethral strip, replacement of the skin defect on the dorsum of the penis and repair of the spatulate glans.

At secondary operations the urethra is lengthened and restoration of continence attempted.

The early results of this integrated regime are encouraging.

THE REPAIR OF EXSTRO PHY OF THE BLADDER

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REFERENCES

HANNA, M. K. and WILLIAMS, I. D. (1972). Genital funct ion in males with vesical extrophy and epispadias. British Journal of Urology, 44, 169.

}EFFS, R. D. ( 1972). Primary closure of the extrophied bladder, in Current Controversies in Urologic Management, edited by Saunders & Co., Philadelphia, p. 235.

:E<liM'NA, N. N. (1973). A technique for epispadias repair. Plastic and Reconstructive Surgery, 52, 365.

LEADBETTER, L. W. (1964). Surgical correction of total urinary incontinence. Journal of Urology, 91, 261.

VANDER MEULEN, J. C. H. (1977). The treatment of hypospadias. Plastic and Reconstruc­tive Surgery, 59, 206.

SHIP, A. G. and PELZER, R. H. (1972). Reconstruction of the female escutcheon in exstrophy of the bladder. Plastic and Reconstructive Surgery, 49, 6.

OWSLEY, ]. Q. and HINMAN, ]R., F. (1972). One-stage reconstruction of the external genitalia in the female with exstrophy of the bladder. Plastic and Reconstructive Surgery, so, 3-

WILLIAMS, D. I. (1969). Epispadias and extrophy. Proceedings of the Royal Society of Medicine, 62, 1079.

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