- History of Pediatric Urological ReconstructionsDuncan R Morhardt, David A Bloom
- Ethical Consideration in Pediatric Surgical ReconstructionsMindy B Statter, Peter Angelos
- Special Anesthetic Consideration in Infants ToddlersAnnie Amin, Igor Tkachenko
- Postoperative Pain Control and Regional AnesthesiaAshish Gupta, Andrew Wuenstel
- Recent Advances on Long-term Effects of Anesthesia on ChildrenRoshan Patel, Zheng Xie
- Fluid, Electrolyte Balance and Parenteral NutritionSudhir Sriram, Jaideep K Singh
- Basic Surgical Principles of Pediatric SurgeryNikunj K Chokshi, Mark B Slidell
- Plastic Surgical Principles: Skin Flaps/Tissue Transfer/Myocutaneous Flaps in Consideration of Urological ReconstructionsKareem Hassan, David H Song
- Surgical Scars, Prevention, Excisions and RevisionsLydia Williams, Russell R Reid
- Basics of Instrumentation, Suture Material and Equipment for Open SurgeryGursev Sandlas
- Surgical Training in 21st Century (Role of Simulation/Inanimate Models)Thomas G Leffler, Alison C Keenan, Patrick H McKenna
- Role of Simulation in Current Era of Surgical Training
INTRODUCTION
Pediatric urology practice consists of diagnosis, management, and correction of urinary and genital disorders in children with a spectrum of surgical and nonsurgical interventions to rectify urine flow, genital function, and pelvic abnormalities. Reconstruction utilizes native, transferred, or grafted tissue to alter a biologic form through repair, replacement or improvement of abnormal structures.
The goal of this chapter is not to exhaustively review the techniques, but rather to survey the pioneering pediatric urologic reconstructive techniques for grafting and interposing bowel segments, mechanical devices, and artificial substrates. Hypospadias will also be reviewed because of its importance in pediatric reconstructive history. Procedures such as pyeloplasty,1,2 autoaugmentation, bladder neck reconstruction,3 and ureterovesical junction revision that are essential to the pediatric urologic armamentarium are beyond the scope of this discussion and are covered elsewhere in this text.
EARLY PEDIATRIC UROLOGIC RECONSTRUCTION
Complex surgery requires modern anesthetic techniques. Prior to their introduction, urologic interventions to remove bladder calculi, for example, were horrible and often fatal or deals left to specialists called lithotomists. Anesthetic techniques provided sufficient latitude for surgeons to perform lithotomy comfortably and, in time, advanced abdominal and genital procedures.
Bladder ailments, such as tuberculosis and cancer, sorely affected mankind throughout history, but bladder exstrophy represented a particular challenge for pediatric reconstructive surgeons that are still not perfectly met. The need to control the continuous efflux of urine tested surgical ingenuity and the bowel, with its continent sphincter and proximity to the bladder and ureter, was ideal for urinary diversion.
The first reported urologic reconstruction was a simple diversion attempt for a man with bladder exstrophy in 18514 by Eusebius Arthur Lloyd of St Bartholomew and Christ Hospital in London. The patient died one week later presumably from intraperitoneal sepsis. The technique involved passing a silk suture from the ureter directly into the rectum, effecting a fistulous connection, to divert the urine. One year later, John Simon of St Thomas Hospital, London, reported a successful ureterosigmoid diversion5 using the technique described by Lloyd. The patient, a 13-year-old male with bladder exstrophy, had a long recovery complicated by intra-abdominal urine, ileus, and bleeding. The procedure effectively diverted urine to the bowel, although a year later the patient succumbed to sepsis from a pelvic abscess. This, however, was considered the first success with a pediatric urologic reconstruction with bowel.
Gersuny (1892),6 among others, attempted bladder substitution. His method used the proximal and anterior rectum as an extended reservoir in a cystectomy patient. Rutkowski, in May 1898, performed single-stage enterocystoplasty augmentation with a closure of bladder exstrophy, substituting ileum for bladder (Figs. 1A and B).7,8 Roughly three-months later, Mikulicz, famed for his gastroplasty technique, performed a multi-staged enterocystoplasty using twice as much bowel (12 cm) as Rutkowski.8 Mikulicz, who published a brief description of his approach one month earlier than Rutkowski,8 has largely been credited as the key innovator of this technique. In a more detailed follow-up report, Mikulicz noted that after four months follow-up the patient could void up to 100 cc and deemed this a success.4
Aside from forays into cystoplasty with varying colon segments from cecum and sigmoid in Europe,9 the next 50-years saw limited advances in urinary tract reconstruction.
The Ureteroenteric Anastomosis
Ureteroenteric diversion was technically challenging and Tuffier's observation from the late 19th century,10 that refluxing urine mixed with fecal material caused urosepsis, provided a rationale for tunneled anastomosis into colon. Rather than the fistulization method of Lloyd and Simon, a properly sutured anastomosis with trigone or ureter was recognized as a key feature to success. In 1892, Karl Maydl introduced a complete transfer of the trigone to the adjacent sigmoid colon11 which he performed on patients with bladder exstrophy. Maydl's trigonosigmostomy maintained the intrinsic anti-reflux mechanism and provided an easy anastomosis to bowel. This was modified for an exstrophy patient by Tuffier.10 After technical improvement, this approach became relatively successful.12
Burgenheim's ureterorectal anastomosis in 1894 was plagued by leakage. Coffey tunneled ureter into colon.13 As Hendren14 delineates the history, it was not until Nesbit demonstrated an accurate ureteral anastomosis technique for in colon that Leadbetter and Clarke (in 1955)15 could employ a combination of Coffey's tunnel and Nesbit's anastomosis to create the modern anti-refluxing ureteral anastomosis into colon. Underlying its success was compressive tunneling that maintained a one-way valve. This same method was popularized by Goodwin and colleagues16 where the ureter was tunneled under the submucosa and then directly anastomosed to the mucosa. This approach remained in wide use for continent urinary diversions.
EVOLVING PEDIATRIC URINARY RECONSTRUCTION
Modern advances in urologic reconstruction first came from Willard Goodwin following a combination of experimental data and clinical attempts with a series of ileocystoplasty patients at UCLA, followed by other favorable clinical experiences.17
Jack Lapides in 1972 published a landmark paper describing consistent success with clean intermittent catheterization (CIC) for certain lower urinary tract dysfunctions that greatly changed the perception and practice of catheterization.18 Until then, the conventional use of sterile catheterization burdened the management of dysfunctional or reconstructed urinary systems, whereas CIC was convenient and safe.14
Urinary reconstruction expanded once the utility of CIC was evident and all intestinal segments were clinically utilized. The stomach was a late addition. Inspired by a patient's large stomach, Tan Sri Guan Bee Ong and CH Leong performed the first recorded gastrocystoplasty.19 Michael Mitchell and colleagues at Indiana elaborated on the value, and shortfalls, of this technique in children. It remains in occasional use today for patients with limited bowel segments or renal dysfunction.5
The availability of other diversions and recognition of long-term risks of adenocarcinoma displaced ureterosigmoidostomy,20 although it still is an appropriate option for patients in remote or rural locations where the logistics of surveillance are untenable and the cost of catheters rivals the living wages.21 The malignant risk may also be lessened by anatomical separation of the urinary and fecal pooling.
Whereas bladder exstrophy inspired many original efforts in urologic reconstruction, megaureter also stimulated reconstructive urology innovations. Swenson et al,22 after Hendren's telling, drew on experiences from Melnikoff, Nissen, and Goodwin using ileal interposition for megaureter. Hendren23 himself utilized enlarged hydronephrotic segments and dilated urinary systems as a substrate to reconstruct megaureters.
Bowel Conduits
Bricker's ileal conduit technique24 once played an important role in management of pediatric urologic conditions such as neurogenic bladder, malignant tumors, and severe congenital anomalies. Long-term follow-up of ileal conduits in children, however, revealed metabolic derangements, stones, infection, and renal deterioration. Colon conduits gained acceptance as an alternative because of lower experimental pyelonephritis rates.25
Continent Constructions
After favorable adult experience in managing lower urinary tract malignancy, continent urologic diversions and neobladders moved to the pediatric population. The Kock pouch utilized a segment of ileum fashioned with two sets of valves: One proximally to prevent reflux to the kidneys and the other to prevent stomal leakage.26 Indiana and Mainz pouches utilized colon, with the Mainz employing the ileocecal valve as the continence mechanism for the new reservoir although the loss of the ileocecal valve from the alimentary system often resulted in diarrhea.27
In 1980, the French pediatric surgeon Paul Mitrofanoff had the brilliant insight to create a catheterizable continent channel with appendix in a 5:1 tunnel-to-diameter ratio. The Mitrofanoff principle ushered in a new era in urologic reconstruction. Variations of catheterizable channels reconstructed from other bowel segments by Monti and Yang,28,29 as well as Casale30 offered channels for more complex situations. These approach all hinged on CIC.
The Mitrofanoff principle in the Malone antegrade continent enema procedure31 is additionally useful for myelomeningocele and other patients with bowel dysfunction.
Hypospadias
Surgical technique for hypospadias matured in the late 19th century, but persistent complications and suboptimal results exasperated surgeons. Hypospadias continues to test seasoned surgeons, yet the evolution of hypospadias repair shows impressive ingenuity.
Diefenback32 described a method of hypospadias repair but Mettauer33 is considered the first to perform successful reconstruction.34 Weller Van Hook, a Chicago surgeon, in 1896 reviewed hypospadias techniques while introducing his own method and identified other pioneers including Etienne-Frederic Bouisson and Simon-Emmanuel Duplay, both Frenchman who utilized staged curvature repair, glanuloplasty forming the distal neomeatus, and immediate or delayed urethroplasty joining the urethra to the neomeatus using plastic surgical techniques.35 He acknowledged German surgeon Karl Theirsch's overlapping rectangular paraurethral flap innovation36 designed to minimize fistula after neourethra formation.
Carl Beck, a general surgeon with urologic interests who had trained in Prague, explored a urethral advancement approach where the urethra was mobilized and carried through a perforated glans where it was secured. He published his series in 1917.37 Paul Mathieu introduced a technique in 1932 whereby the glans was longitudinally incised to produce a dorsal glanular strip that is matched to a ventral cutaneous strip created proximal to the meatus. This creates a neourethra with a new meatus in the more distal glans,38 perhaps the first modern glansplasty. Graham Humby, in 1941, reported use of buccal mucosal graft to successfully repair a failed hypospadias repair39 but his buccal graft was not widely revisited until the 1990s.40 Sir Denis Browne, the first dedicated pediatric surgical consultant in England, resurrected the Duplay urethroplasty41 with a tubularized urethral strip, but used finer suture material secured with glass beads to reduce fistula formation.
Concomitant management of chordee facilitated the modern single-stage approach. Until the mid-1950s,42 incomplete understanding of the condition led to the practice of ensuring appropriate straightening before the urethroplasty.43 The chordee in most patients 6with hypospadias was presumed to be due to a fibrotic remnant of the corpus spongiosum. Questioning this assumption, John Duckett cataloged a large group of his own patients and noted that in only a minority (13%) were such bands responsible for the chordee. He posited that corporal disproportion with ventral shortening was the predominant pathology. Indeed, simply degloving the penis appeared to correct the chordee in most cases.
Charles Devine and Charles Horton revisited the earlier approaches for a single stage hypospadias repair using a modified Mathieu with a V flap of glans, coupling a meatal advancement with the chordee repair.34 This offered a single-stage correction but a high (~25%) meatal stenosis or fistula rate. Norman Hodgson utilized a combination of inner prepuce for the bridging tubularized graft and the outer preputial layer for shaft skin.44
John Duckett synthesized previous techniques and added his innovation to usher in a modern era of hypospadiology. Asopa in India, in 1971,45 and Standoli in Italy, in 1972,46 offered similar versions of the preputial transverse island technique. From these approaches and innovations of Hodgson, Duckett's horizontal strip of inner preputial transverse island pedicle reduced twisting of the shaft skin and dropped reoperation rates to 7.5–15%.47 Duckett also translated the classic Heineke-Mikulicz plastic technique to develop meatal advancement and glansplasty (MAGPI)48 and with Mark Rich described the technique of hinging the urethral plate by incising the dorsal urethral lumen (Rich 1978)49 based on its capacity to epithelialize without stricture. Warren Snodgrass advanced this notion of hinging50 with the tubularized incised urethral plate.
Artificial Materials to Manage Urologic Problems
Bowel is an excellent resource for urinary tract reconstruction but it has drawbacks. Metabolic complications include long term acidosis with osteoporosis51 and stone formation, electrolyte derangements, jejunal segments bring hyperkalemia. Bacteriuria can lead to ascending infection.52 After gastrocystoplasty, caustic urine secondary to acid production may provoke hematuria-dysuria syndrome.53 Reflux and infection impair renal function. Bowel reconstructive procedures risk dire complications include perforation, anastomotic leak, obstruction, and short gut.
Prior to modern surgical approaches, bladder exstrophy patients employed external urine collection devices to cover the exposed bladder, akin to an early urostomy, reportedly conceived by Andrew Bonn in 1788.4 Attempts to construct artificial mechanical bladders, including efforts by Bogash54 and Friedman,55 failed due to infection and fibrosis. Anthony Atala and colleagues created artificial bladder tissue using a degradable scaffold impregnated with harvested autologous cells and the possibility of genuine bladder replacement gained traction.56 An initial trial with a similarly constructed bladder patch showed promise,57 but the artificial bladder patch was unsuccessful at restoring normal bladder function and attended by severe complications.58 A revival of bladder tissue engineering has seen strides in materials and technologies including silk scaffolds59 and more elaborate stem cell technology.60
In 1990, autologous urethral cells without an artificial underlying scaffold were cultured to form a graft for a multi-stage hypospadias repair,61 although fistula still occurred in one of the two patients. Hypospadias repair with autologous cell seeded acellular matrices in children as well as small intestine submucosa (SIS) produced a fistula rate approaching 30%.62 Recent trials using acellular matrix for urethrocutaneous fistula after hypospadias repair by Subramaniam and colleagues offer additional options for this common complication.63 These early efforts create optimism that this field will see significant improvements and a large leap forward in materials and methods for urinary tract reconstruction.
ACKNOWLEDGMENT
The authors would like to thank Dominic DeSocio for his detailed translations of texts.
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