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8 The Kelly Procedure
135
from the venous plexuses at the base of the prostate, which is easily controlled with bipolar cautery.

Tension-Free Bladder Neck Construction

The bladder is opened in the midline proximally from the urethral neo-meatus. The ureteric orices are cannulated with a 4 or 6 Fr feeding tubes, and if required a Cohen type ureteric reimplantation is performed. The location for the construction of the bladder neck is just proximal to the verumontanum, an area that exhibits characteristic longitudinal linear folds (Fig.8.5). Triangles of bladder mucosa are marked and removed, and a 1- or 2-layer tension-free orthotopic bladder neck reconstruction is performed over an 8 Fr silicone stent with interrupted 4/0 Monocryl sutures. We do not use a measure of the width of the remaining urethral plate, nor do we measure the nal circumference of the reconstructed bladder neck. Instead, we use our judgement and experience to aim that rst layer of sutures captures an adequate amount of detrusor muscle to ensure that the urethral stent can be moved with a degree of resistance. The proximal end of the catheter is secured within the bladder with a 6/0 Monocryl suture. A 10 or 12 Fr suprapubic catheter is inserted into the bladder for postoperative drainage. The bladder is closed with a single layer with interrupted 4/0 Monocryl sutures. Ureteric stents, when used, can be brought out through the midline closure or laterally through separate puncture sites.
Fig. 8.5 Operative image showing orthotopic location of bladder neck construction, with marked mucosal triangles for excision
136
Fig. 8.6 Original hand drawing by Justin Kelly showing the IPGAM procedure
N. S. Johal et al.
Urethral Reconstruction andGlans Closure
The urethral plate is tubularised with interrupted 5/0 or 6/0 PDS sutures on the exist­ing 8Fr silicone urethral stent. An IPGAM procedure is performed distally to form a more ventrally placed urethral meatus (Fig.8.6). Triangles of mucosa are excised from the dorsal aspect of the glans, lateral to the central urethral plate. The glans is reconstructed over the urethra with 1 or 2 layers of interrupted 6/0 PDS sutures. The urethra is retracted ventrally, and the corporal bodies are approximated in the mid­line with 1 or 2 layers of interrupted 5/0 PDS sutures, either with or without a rota­tion of the corpora to correct the dorsal chordee.
Wound Closure andPenile Skin Reconstruction
The rectus muscle closed in layers commencing with interrupted 2/0 Vicryl for the rectus muscle and 0 or 1 Vicryl for symphyseal region. The skin is closed in 2 lay­ers, with Vicryl for the subcutaneous fat and Monocryl for skin. An umbilicoplasty can be performed at the skin closure stage if requested by the family. Skin coverage of the penis is always a challenge, and excision of the dartos tissue from the penile
8 The Kelly Procedure
skin can facilitate this step. Anchoring sutures between the dartos tissue and the base of the corpora can enhance the visible penile length. The urethral stent is secured to the glans penis with 4/0 Prolene. A compression dressing is placed around the reconstructed penis.
137
Kelly Procedure inFemales (Video 2)
The basic tenets of the procedure are the same as in the male patient. The incision continues inferiorly to expose both the clitoral corporal bodies leaving intact the labia minora skin aps. This enables subsequent clitoral hood skin reconstruction following the bladder neck and urethral reconstruction. As in the male, the pelvic muscular oor is exposed in the extraperitoneal plane to access the neurovascular bundle, the obturator internus muscle and the superior aspect of the levator ani below and lateral to the vagina. Each clitoral body is separated from the surrounding fat of the labium majus. As in the male, the clitoral bodies can be detached from the ischiopubic ramus with identication and mobilisation of the pudendal neurovascu­lar bundles. However, this step is not always necessary and is determined by the degree of pubic diastasis, which if signicant can hinder a tension free bladder neck construction and approximation of the clitoral bodies. After the bladder neck is reconstructed, the muscle of the urogenital diaphragm below and lateral to the vagina can be used as a wrap over the bladder neck and urethral repair. The abdo­men is closed as in the male. The mons pubis is constructed by approximation of the subcutaneous fat from either side to achieve a good cosmetic appearance.

Postoperative Management

Postoperatively the patient can return to the in-patient ward and can commence feeds almost immediately. Postoperative analgesia is delivered via an indwelling epidural catheter for the initial 4–5days, which we have previously shown results in a reduced hospitalisation following primary closure [5]. Broad spectrum intrave­nous antibiotics such as co-amoxicav are continued for 48h and then converted to an oral dose for a total of 7 days after which prophylactic dose antibiotics are administered. If the patient has undergone ureteric reimplantation, the ureteric cath­eters are removed after 7days. The penile compression dressing is removed on day 7, the urethral stent on day 14 and the suprapubic catheter after day 21 once effec­tive urethral voiding is established.
Complications andOutcomes
In our experience, major complications following a Kelly procedure are rare. Glans ischaemia and atrophy are widely muted as a potential complication, supposedly related to the mobilisation of the pudendal neurovascular bundle. Over the past
138
N. S. Johal et al.
20years, the exstrophy team has performed in excess of 400 Kelly procedures and has witnessed hemi-glans atrophy in 2 patients. Less serious and more common complications would include urethrocutaneous stula formation, penile skin dehis­cence, infrapubic incisional hernia and urethral meatal stenosis. Factors responsible for good outcomes are a high volume of cases (related to centralisation of BEX surgery to 2 centres in United Kingdom), collaborative working arrangements, spe­cialist anaesthesia and postoperative analgesia, and highly experienced specialist urology nursing care.
Our institution made a conscious decision 25years ago to seek an alternative surgical strategy for children with BEX, having concluded that the staged approach of neonatal bladder closure, Cantwell-Ransley epispadias repair and Young-Dees bladder neck reconstruction was not conferring good outcomes. The Kelly proce­dure was introduced and became the operation of choice for all BEX cases within a few years and has remained so to this day. We are highly satised with the improved penile cosmetic outcomes, reduced bladder augmentation rates and a signicant increase in the number of patients who can achieve spontaneous urethral voiding without the need for continence pads or underwear. We are required to submit annual outcome data to the national funding organisation for highly specialised ser­vices, such as BEX care, and the number of children who are dry by day and voiding per urethra at 5years of age is 50–60%. We know that this gure increases with age and specically with biofeedback therapy, which commences at around 8years of age. We remain condent that the decision we made to adopt the Kelly procedure for our BEX patients was correct and that it has provided our cohort of patients with improved outcomes.
It is wrong in our view to simplify urinary continence in terms of dryness by day and night, because in BEX patients it is a much more complex issue, that should take into account voiding intervals and uid intake. There is a wide diversity of phenotypes in BEX, and outcomes are unpredictable and at times unexpected. To compare the outcomes of BEX surgeries and institutions by means of urinary conti­nence is divisive and overlooks many other health outcome measures, which are equally and, in some cases, more important.
The Kelly procedure is not complex, but does require a high surgical skillset, courage and a large cohort of patients to become procient. We are condent that the centralisation of BEX care that we have witnessed in the United Kingdom and that is now underway with the establishment of consortia in the United States will continue to enhance our understanding of what are the right goals and aspirations in the management of BEX.
8 The Kelly Procedure
139

References

1. Siffel C, Correa A, Amar E, Bakker MK, Bermejo-Sanchez E, Bianca S, Castilla EE, Clementi
M, Cocchi G, Csaky-Szunyogh M.Bladder exstrophy: an epidemiologic study from the inter-
national clearinghouse for birth defects surveillance and research, and an overview of the lit-
erature. Am J Med Genet. 2011;157:321–32.
2. Burki T, Hamid R, Duffy P, Ransley P, Wilcox D, Mushtaq I.Long-term followup of patients after
redo bladder neck reconstruction for bladder exstrophy complex. J Urol. 2006;176(3):1138–41.
discussion 1141–2
3. Kelley JH, Eraklis AJ.A procedure for lengthening the phallus in boys with exstrophy of the
bladder. J Pediatr Surg. 1971;6(5):645–9.
4. Kelly JH, Taghavi K, Mushtaq I, Justin H.Kelly and his procedure for bladder exstrophy and
epispadias. J Pediatr Surg. 2022;57(2):314–21.
5. Mushtaq I, Garriboli M, Smeulders N, Cherian A, Desai D, Eaton S, Duffy P, Cuckow P.Primary
bladder exstrophy closure in neonates: challenging the traditions. J Urol. 2014;191(1):193–7.
Scrotoperineal Approach toBladder Exstrophy Repair
S.N.Kureel, ArchikaGupta, andKanoujiaSunil

Introduction

Bladder exstrophy is one of the most serious urological birth defects involving the lower abdominal wall, bladder, urethra, and penis with certain associated anomalies and mostly normal upper tracts. The classic description of this birth defect was given by O’Neill as “if one blade of a pair of scissors were passed through the urethra of a normal person; the other blade was used to cut through the skin, abdominal wall, anterior wall of the bladder and urethra, and the sym­physis pubis; and the cut edges were then folded laterally as if the pages of a book were being opened” [1] (Fig.9.1). Trendelenburg in 1906 described that despite the severity of the defect, all neurophysiological components necessary for continence are present in exstrophy [2]. Therefore, any strategy/approach for reconstruction must aim to create a low-pressure reservoir of adequate capacity, create an outlet, reconstruct the bladder neck with internal sphincter, and recon­struct the striated muscle of the external sphincter with preserved innervation. While aiming for satisfactory continence, it is of paramount importance that upper tracts do not suffer any damage from high pressure or the inevitable vesi­coureteric reux. Reconstruction of the outlet must be aimed at creating dynamic resistance with capacity to relax on command of the pontine micturition center and remain contracted in the lling phase to avert leakage of urine (Fig.9.2). Therefore, outlet reconstruction should be accomplished such that innervation
9
Supplementary Information The online version contains supplementary material available at
https://doi.org/10.1007/978- 3- 031- 91238- 2_9.
S. N. Kureel (*) · A. Gupta Department of Pediatric Surgery, King Geroge’s Medical University, Lucknow, India
K. Sunil Chandan Institute of Pediatric and Neonatology, Lucknow, India
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2025 A. R. Shukla, R. S. Joshi (eds.), Bladder Exstrophy and Epispadias,
https://doi.org/10.1007/978-3-031-91238-2_9
141
142
Fig. 9.1 Classic exstrophy bladder (male) showing open bladder plate with epispadias with preputial hood on ventral aspect of glans penis
S. N. Kureel et al.
remains preserved. Other desirable goals are preservation of the cavernosal nerve and an adequately sized phallus (Fig.9.3). The technique of radical cor­poral detachment for penile lengthening must ensure absolute preservation of the neurovascular bundle of corpora and vessels supplying the glans.
The task of bridging the abdominal wall defect is achieved by bringing the recti toward the midline after doing the osteotomy [35]. The aesthetic appearance is further enhanced with umbilicoplasty, abdominoplasty, and penile skin coverage (Fig.9.3).
The midline scrotoperineal approach of bladder exstrophy reconstruction with innervation-preserving sphincteroplasty and radical corporal detachment realizes all the aforementioned goals [68].
9 Scrotoperineal Approach toBladder Exstrophy Repair
Fig. 9.2 Schematic diagram to show salient points of normal micturition. The lling phase under control of sympathetic (red) and emptying under control of parasympathetic (green). It is controlled by paracentral lobule of cerebral cortex through sacral micturition center (S2, 3, 4)
143
A brief review of the relevance of surgical anatomy, especially in the context of bladder exstrophy here, would be useful in understanding the technical nuances of the procedure. This would include:
1. Anatomy of the bony pelvis
2. Anatomy of the urogenital diaphragm in exstrophy
3. Anatomy of fascial planes and vessels in the epispadiac penis
4. Relevant anatomical details of open bladder-plate
144
Fig. 9.3 Appearance after reconstruction of bladder exstrophy showing adequate-sized phallus, umbilicus, and well­aligned lower abdominal skin crease
S. N. Kureel et al.
Anatomy ofBony Pelvis
The bony conguration of the pelvis in bladder exstrophy has been described by Sponseller and associates using computerized tomography (CT) pelvis and three­dimensional reconstruction [9]. In a cross-sectional view of the bony pelvis at the level of acetabulum and triradiate cartilage, the intertriradiate distance is the length of an intertriradiate line joining the triradiate cartilage of the right and left sides across the pelvic cavity. This line also divides the pelvis into anterior and posterior segments (Fig.9.4). There has been shown to be a mean external rotation of 12 degrees in the posterior pelvis on each side, retroversion of the acetabulum, a 31% increase in intertriradiate distance, a mean 18° external rotation of the anterior pel­vis, and a 30% shortening of superior pubic rami compared to the control popula­tion. This shortening of superior pubic rami along with external rotation of the pelvis contributes to a varying degree of diastasis of the pubic symphysis. Our study on diastasis of the pubic bone in patients with bladder exstrophy has demonstrated that the shape of the gap between the ischiopubic rami, where the urogenital dia­phragm is located, varies from rectangular to trapezoid [10] (Fig.9.5a, b). Stec etal. have described that the sacrum in exstrophy patients has a 42.6% larger volume and 23% more surface area compared to the control population [11].
9 Scrotoperineal Approach toBladder Exstrophy Repair
145
12˚
Fig. 9.4 Shows the magnitude of external rotation of pelvis, retroversion of acetabulum, increased intertriradiate distance, and external rotation of anterior pelvis with shortened superior pubic rami. Abnormal anatomy (yellow) is superimposed with normal anatomy of cross-section of pelvis (ochre yellow)
Fig. 9.5 (a) Three- dimensional image of CT pelvis perineal view showing rectangular shape of space between ischiopubic rami. I— medial and lowermost point of ischial tuberosity; P—Superior and medialmost point of pubic bone. (The cartilaginous segment of ischiopubic ramus is not ossied.). (b) Three-dimensional image of CT pelvis perineal view showing trapezoid shape of space between ischiopubic rami. I—Medial and lowermost point of ischial tuberosity; P—Superior and medialmost point of pubic bone
30%
31%
a
b
Anatomy ofUrogenital Diaphragm inBladder Exstrophy
It has been conclusively demonstrated that all the normal muscles of the urogenital diaphragm are present in patients with bladder exstrophy [12]. Normally, the uro­genital diaphragm between bilateral ischiopubic rami and the base of the urogenital triangle is triangular. However, in bladder exstrophy, the urogenital diaphragm may be trapezoid or rectangular depending upon the degree of pubic diastasis [10]. The