Journal of Indian Association of Pediatric Surgeons
Journal of Indian Association of Pediatric Surgeons
                                                   Official journal of the Indian Association of Pediatric Surgeons                           
Year : 2019  |  Volume : 24  |  Issue : 1  |  Page : 65--67

Split-appendix technique for simultaneous use in the mitrofanoff principle and posterior urethral substitution in a bladder exstrophy–epispadias complex patient


Yogesh Kumar Sarin, Parveen Kumar 
 Department of Pediatric Surgery, Maulana Azad Medical College, New Delhi, India

Correspondence Address:
Dr. Parveen Kumar
Department of Pediatric Surgery, Maulana Azad Medical College, New Delhi - 110 002
India

Abstract

We describe the case of a bladder exstrophy–epispadias complex (BEEC), in which a “split” appendix was used simultaneously as a posterior urethral replacement (for strictured Mitchell's repair) as well as a Mitrofanoff channel. Split appendix had been used before for simultaneous Mitrofanoff and Malone's antegrade contrast enema channels to manage neurogenic bladder and bowel. To the best of our knowledge, this is the first reported case for the use of split appendix for urethral reconstruction in a child with BEEC.



How to cite this article:
Sarin YK, Kumar P. Split-appendix technique for simultaneous use in the mitrofanoff principle and posterior urethral substitution in a bladder exstrophy–epispadias complex patient.J Indian Assoc Pediatr Surg 2019;24:65-67


How to cite this URL:
Sarin YK, Kumar P. Split-appendix technique for simultaneous use in the mitrofanoff principle and posterior urethral substitution in a bladder exstrophy–epispadias complex patient. J Indian Assoc Pediatr Surg [serial online] 2019 [cited 2021 Apr 18 ];24:65-67
Available from: https://www.jiaps.com/text.asp?2019/24/1/65/247909


Full Text



 Introduction



Bladder exstrophy–epispadias complex (BEEC) is still a challenging congenital pediatric urological anomaly, after about 150 years of surgical attempts to create “normal” anatomy. The treatment is directed at various goals of achieving social continence, preservation of upper tracts, and cosmetically acceptable abdominal wall and external genitalia. In cases with small bladder plate and dehiscence of primary repair, the treatment depends on bladder augmentation with clean intermittent catheterization (CIC) for achieving social continence. This leaves the patient with a urinary stoma on the abdominal wall.

Our patients usually come from the low socioeconomic group. Many of them are uncomfortable with abdominal stoma and demand for a urinary stream or at least ejaculation from below. This has prompted us in looking for alternatives for urethral substitution, especially after failed primary repair.

 Case Report



We describe a child with BEEC in whom a “split” appendix was used simultaneously as a posterior urethral replacement (for strictured Mitchell's repair) as well as a Mitrofanoff channel. A 1-day-old male neonate underwent a modern-staged reconstruction procedure. The bladder plate was closed within first 48 h. Mitchell's urethroplasty was accomplished at the age of 9 months, which got complicated by urethral stricture and penopubic fistula. At the age of 3 years, it was planned to close the penopubic fistula and to create a Mitrofanoff stoma (as a vent lest the urethral stricture might affect the upper tracts). Intraoperatively, a long (~12 cm) appendix was encountered with pliable mesentery. The appendix could be divided into two halves with intact blood supply to both the halves [Figure 1]. The proximal half was used to create the Mitrofanoff stoma, and the distal half was used to substitute the posterior urethra after excising strictured bladder neck and urethral plate [Figure 2]. We could bring the appendix out through the corporal bodies to create a neomeatus at penoscrotal junction. Concomitant bilateral ureteric reimplantation was done using the Politano–Leadbetter technique. The child was discharged after teaching the parents CIC through both the stomas.{Figure 1}{Figure 2}

The child did not follow up for 2 months and returned with stricture at both stomas resulting from noncompliance with CIC. Investigations revealed bilateral mild-to-moderate hydronephrosis with normal kidney function tests. The parents were recounseled and the stomas were revised. The child was dry on regular CIC and night drainage through the Mitrofanoff stoma. Subsequent investigations revealed no worsening of upper tracts and no vesicoureteral reflux.

With the need for the increased frequency of CIC (small-capacity bladder) to achieve social continence, at the age of 4 years, the patient underwent augmentation cystoplasty. Since then, the child had been in regular follow-up, doing CIC 3 hourly with night drainage and preserved upper tracts. At the age of 13 years, the child showed his concern for being hypospadiac and underwent urethroplasty. Now, 2 years post urethroplasty, the child can urinate in a single stream. To the best of our knowledge, this is the first reported case for the use of split appendix for urethral reconstruction in a child with BEEC. As our patient had pubic diathesis, it was easy to mobilize the appendix in the desired place. However, in other patients, it may require transpubic approach of dissection.

 Discussion



Appendix had long been considered a vestigial organ, but it is not so in the present era. Its use as interposition, both internally in the form of biliary conduit and urinary conduit and externally in the form of appendicovesicostomy and appendicocecostomy, has been well published. Büyükünal et al. in 1995 were the first ones to describe the use of appendix interposition in the treatment of severe posterior urethral injuries.[1] They did animal experiment on rabbits and suggested that the pedicled appendiceal flap technique could be used for the treatment of severe forms of posterior urethral injuries.

In 1999, Koshima et al. published their technique of free vascularized appendix transfer for reconstruction of penile urethras with severe fibrosis in two cases.[2] In 2002, Aggarwal et al. demonstrated the use of pedicled appendix graft for substitution of urethra in posttraumatic recurrent urethral strictures in two patients.[3] They reported gratifying results at the 3-year follow-up, without any complications.

One interesting mention of the use of ileocecal segment for bladder augmentation and appendix for urethral reconstruction has also been reported in patients with BEEC; the appendix was laid in the urethral lumen as the neourethra following urethral demucosation.[4] The use of two-staged, modified substitution urethroplasty using appendix free flap on microvascular anastomosis, for posturethra stricture, has been reported.[5]

Split-appendix technique has been used by many urologists for appendicovesicostomy and appendicocecostomy. This technique relies on the chance favor of lengthy appendix, in which divided length of appendix is used for both stomas albeit at precarious blood supply. The meticulous harvesting of appendix blood supply remains cornerstone for split technique.

In this patient, as there was pubic diathesis, it was easy to mobilize the appendix in the desired place. However, in other patients, it may require transpubic approach of dissection. Our patient represents a long period of 11 years of follow-up, postuse of split-appendix technique for posterior urethral substitution in BEEC.

 Conclusion



Appendix is a versatile organ that may be used for a variety of substitution procedures and no more a vestigial organ. This adds another option to the pediatric surgeon's armamentarium aiming at near-normal social life for a patient with exstrophy bladder.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Büyükünal SN, Cerrah A, Dervişoğlu S. Appendix interposition in the treatment of severe posterior urethral injuries. J Urol 1995;154:840-3.
2Koshima I, Inagawa K, Okuyama N, Moriguchi T. Free vascularized appendix transfer for reconstruction of penile urethras with severe fibrosis. Plast Reconstr Surg 1999;103:964-9.
3Aggarwal SK, Goel D, Gupta CR, Ghosh S, Ojha H. The use of pedicled appendix graft for substitution of urethra in recurrent urethral stricture. J Pediatr Surg 2002;37:246-50.
4Amirzargar MA, Yavangi M, Ghorbanpour M, Hosseini Moghaddam SM, Rahnavardi M, Amirzargar N. Reconstruction of bladder and urethra using ileocecal segment and appendix in patients with exstrophy-epispadias complex: The first report of a new surgical approach. Int Urol Nephrol 2007;39:779-85.
5Hiradfar M, Shojaeian R, Sharifabad PS. Two staged modified substitution urethroplasty using appendix-free flap. BMJ Case Rep 2015;2015. pii: bcr2015210771.