In the Twentieth Century

1.2.1 The Barren Era: 1900-1945

Textbooks of Surgery in 1908 recommended colostomy as a life-saving measure only, otherwise the perineal approach was employed for all other cases [75]. Extension of the long median posterior incision was suggested for those instances associated with a rec-tovesical fistula [105]. Controversy concerning surgical management continued. In 1908, Mastin noted that the AP procedure had little to attract surgeons and could scarcely be viewed as a rational procedure [105]. In 1915, Brenner on the other hand thought that the AP procedure was a novel technique that promised good results in selected cases [14]. By 1922 it was recognized that that the perineal approach was often futile in instances of high rectal atresia [130]. Despite these sporadic observations, by 1926 lapa-rotomy with or without a colostomy was considered a procedure of last resort and was associated with a high mortality [42].

In 1930, Wangensteen and Rice described the radiographic invertogram as a method for determining the level of termination of the rectal atresia and deciding whether a perineal approach was rational [169]. In 1934, Drs Ladd and Gross at the Boston Children's Hospital proposed a classification for ARM (types 1-4) that closely resembled Bodenhamer's work published 74 years earlier [12, 13, 88]. Only one of their 162 cases was managed by a one-stage AP approach and proctoplasty. At the time the success rate for cases of rectovaginal fistula was 50%, that for rectourethral fistula was 20%, and the mortality was in excess of 50%. Dissection was kept close to the hollow of the sacrum, and the external sphincter was divided in lateral halves and resutured in front and behind the proctoplasty [88]. The role of the leva-tors and puborectalis was unknown. In 1936, Stone described transplantation of a low rectovaginal fistula to the anal site without splitting the rectum sagittally. He termed the condition imperforate anus with rec-tovaginal cloaca [161]. In 1938, an early perineal operation was still considered the method of choice by many surgeons [11].

1.2.2 Post World War II Era: 1945-1980

1.2.2.1 A Time of Enlightenment and Continued Controversy

Following World War II, things began to change. The availability of antibiotics and improvements in anesthesia had a positive influence on reducing the septic complications associated with bowel surgery. In 1948, Rhoads and colleagues in Philadelphia rekindled interest in a combined AP approach for cases of imperforate anus and high rectal atresia [134]. In 1950, Denis Browne of Great Ormond Street, London, UK, reclassified the defects associated with rectal agenesis using a thesis originally described by Wood-Jones in 1904 and 1915 [16, 173, 174]. The term "covered anus" became popular, and initial colostomy and subsequent AP pull-through through a hole stretched (not cut) in the pelvic floor was advocated for high lesions. Browne also popularized the "cutback" anoplasty for instances of perineal fistula [16]. In 1953, Douglas Stephens, while working with Denis Browne in London, described the sacroperineal rectoplasty and emphasized the role of the levator ani and downplayed the importance of the internal and external anal sphincters [153, 154]. Two of the four patients undergoing this procedure achieved continence. In 1959, Fritz Rehbein of Bremen reintroduced the endorec-tal pull-through combined with an AP approach for boys with rectourethral fistula [133]. The endorectal concept was described by Hochenneg in Austria in 1889 [63]. Rehbein divided the bowel at laparotomy, stripped the mucosa from the distal rectal atretic end and brought the proximal bowel through the resultant muscular sleeve to the anal dimple to perform an anoplasty [133]. He missed the puborectalis sling in performing this procedure. In 1960, Roumaldi of Italy used the same approach for girls with a rectoves-tibular fistula [140]. In 1961, after extensive dissections, Stephens proposed the importance of the pu-borectalis muscle as the main muscle of continence, but recognized that soiling accidents continued to occur because of mucus leakage from the anal canal due to total or functional absence of the external sphincter [155]. He suggested that this was a problem that needed to be solved by the mother and child. He also advocated the use of the pubococcygeal line on the diagnostic radiographic invertogram to improve identification of the level of rectal atresia [156].

Because of the high incidence of incontinence with the AP approach, in 1963 Kiesewetter of Pittsburgh modified Stephens' operation by performing

Invertogram
Fig. 1.3 A Douglas F. Stephens performed the sacroperineal anoplasty. B E. Durham Smith - working with Dr. Stephens edited the first major modern textbook on ARM

an abdominosacroperineal procedure [79]. He used the abdominal approach to isolate the rectourethral fistula and the sacral route to enter the supralevator space by splitting the pubococcygeus and ileococ-cygeus in the midline. Unlike Stephens, Kiesewetter believed the external sphincter muscle was present and worth saving. Two years earlier (1961), E. Ide Smith and Robert E. Gross had identified a normal-sized external anal sphincter in 15 out of 16 autopsied cases of imperforate anus [150]. Kiesewetter later met with Rehbein and adopted Rehbein's mucosal stripping and endorectal concept, while Rehbein added Stephens' inclusion of the puborectalis sling to his procedure [34]. In 1963, Stephens published the first modern major textbook on the subject [156]. In 1967, Swenson and Donnellan of Chicago described their experience with AP procedures and preservation of the puborectalis sling in the newborn without a co-lostomy [163]. They thought it was advantageous to locate the anoplasty more anteriorly to avoid tension on the bowel as it passed in front of the puborectalis sling. This completely ignored the external sphincter muscle that Swenson (like Stephens) did not think was important in controlling continence [163]. That same year (1967), Kiesewetter and Nixon found the external sphincter muscle present in all nine cases studied at autopsy, but found a gap between the sphincter and the puborectalis muscle [80]. In 1969, Justin Kelly of Australia reported the innovative use of cineradiographic defecography in an attempt to evaluate the function of the puborecatalis following procedures for ARM [76].

In 1970, Cremin et al. reported that the pubococ-cygeal line recommended by Stephens on the radiographic invertogram was an unreliable landmark and suggested using an "M" line, especially in cases with missing sacral segments [27]. In 1973, a prone cross-table radiograph was recommended as an alternate to the classic invertogram for diagnosing the level of rectal atresia [112].

In 1970 a workshop was held at the Royal Children's Hospital, Melbourne, Australia to reclassify ARM. A rather complex, male/female low, intermediate, and high classification was brought into effect. The contents of the workshop were published by Stephens and Smith in 1971 [157]. Stephens and Smith (Fig. 1.3a, b) noted that the functional results of the AP procedure and sacroperineal operation were similar. However, they cited problems with the Kiesewetter/Rehbein operation including mucosal prolapse at the anoplasty site, incomplete use of striated muscle, and loss of rectal storage capacity by using the atretic rectal segment for the muscle sleeve. Yet, they agreed that this procedure was more acceptable than an AP approach alone. The controversy continued, and in 1971, Stephens and Smith recommended the sacrope-rineal approach for redo procedures after an initially inadequate rectoplasty [157]. In 1972, Fowler indicated that since the importance of the puborectalis muscle was recognized, "further finesse in the performance of rectoplasty will center on creating a more functionally efficient sphincter ani" [44]. In 1975, Shafik reported a new concept regarding the anatomy of the anal sphincters and the functional physiology of defecation that he termed the triple loop system [145].

Dissatisfaction with the outcomes of other procedures in 1978 led Mollard to recommend an anterior perineal approach bringing the atretic bowel down in front of the puborectalis sling [110]. Sixty percent of the patients had mucosal prolapse, and soiling continued to be a problem.

Reports recognizing associated anomalies in other midline structures such as esophageal atresia and tracheoesophageal fistula, duodenal atresia, neural tube defects (tethered cord), vertebral anomalies, sacral anomalies, genitourinary anomalies, and instances of congenital heart disease that accompanied imperforate anus became prominent [19, 30, 41, 51, 103, 117, 118, 147, 171]. Acronyms like VATER and VACTERL describe instances of ARM with many of these associated conditions [41, 51, 74, 78, 131]. Certain genetic abnormalities were noted to coexist with ARM, including instances of trisomy 13-15, 16-18, and 21 (Down syndrome) [41, 43, 165]. The absence of associated rectal fistulae in the latter group also became apparent [43, 165]. ARM were also noted to be associated with the cat-eye syndrome (otic atresia and coloboma), Currarino syndrome (rectal stenosis, sacral anomalies, presacral teratoma, or anterior meningocele) and occasionally Hirschsprung disease [24, 29, 41, 86, 89, 98, 104]. It should be noted that although credit has been given to Currarino and associates for recognizing the constellation of defects known as Currarino syndrome, it was Ashcraft and Holder who first described these familial occurrences in 1965 [7, 29]. In India, an unusual subset of patients were identified with imperforate anus and short colon pouch syndrome [8, 17].

The 1970's and early 1980's was a period when children's surgeons reported the results of some of the procedures advocated by Stephens and Smith, Rehbein, Kiesewetter, and others. It became quite clear that different subjective criteria for grading and definitions used by various authors to assess function would make it difficult to compare results. The various studies often focused on the area specifically of interest to the surgeon who advocated the procedure. Comments like excellent, good, fair, and poor characterized the studies, and continence was assessed according to whether one was evaluating puborectalis function or other sphincter activity. Sphincter tone was frequently not mentioned. How motility was evaluated was often in question. Kelly used a scoring system with 6 as a maximum score [77]. Terms like colonic inertia appeared to address the failures and severe constipation that followed the procedure [15, 163]. In 1977, Kiesewetter noted an improvement over time from poor to good in low anomalies and from poor to fair in high anomalies, and that switching from an AP approach to an abdominosacroperi-neal or sacroperineal approach did not improve function [34]. The same year, Nixon and Puri evaluated 47 children with high anomalies; only seven had a good outcome, 28 were fair, 11 poor, and 7 required a permanent colostomy [114]. The seven good outcome patients soiled for 6-17 years of follow-up. Kiesewetter, Turner and Nixon, and Puri suggested that continence was an "evolutionary process" that improved each year during puberty [79, 114]. In 1979, Hecker and Holschneider presented a manometric and functional classification that evaluated external sphincter contraction, propulsive wave frequency and "critical volume," and noted that "an intelligent patient can effect social continence" [54]. The need for a standardized objective testing method to evaluate outcomes was apparent. Mucosal prolapse and stricture of the anoplasty site remained a problem and a variety of secondary plastic procedures were suggested to revise the anoplasty by Mollard and Rowe [111], Nixon [113], Becmeur et al. [9], and Anderson et al. [5]. Incontinence also continued to be a problem, and free muscle transfer, reverse smooth muscle plasty, graci-lis muscle flaps, and artificial sphincters were used in an attempt to correct this problem, with marginal results [34, 64, 66, 67, 149]. Reoperation for a missed puborectalis sling and failed previous procedures was also attempted [81, 87, 127, 149].

1.2.3 The Modern Era: 1980-2005

The 1980s ushered in another procedure, the posterior sagittal anorectoplasty (PSARP), resurrecting the concept initially proposed by Roux [141] in 1834 and subsequently used by Bodenhamer in 1860

[12]. The PSARP operation described by Peter deVr-ies and Alberto Peña and published in 1982 was a new landmark event in the history of ARM, just as influential as the contributions by Amussat and Stephens and was rapidly adopted by many pediatric surgeons throughout the world [4, 33, 153, 154]. They redefined the arrangement of the pelvic muscles and sphincters as a fused sphincter muscle complex. The higher rectal defects were noted to have a wider separation between the subcutaneous and superficial external anal sphincter and the latter muscle from the levators. DeVries and Peña completely divided all the muscles posteriorly in the midline from the anal dimple to the coccyx. They divided the rectourethral fistula from within the atretic segment by separating the mucosa and smooth muscle to avoid urethral and neural damage. The distal atretic segment was tapered to fit within the puborectalis and the divided muscles were sutured posteriorly around and to the neorectum prior to performing the anoplasty. Slight tension was placed on the anoplasty site to draw the skin in and avoid prolapse [120]. The popularity of the PSARP procedure was a reflection of the discontent expressed by many pediatric surgeons with the results obtained after other procedures in the management of intermediate and high ARM. While the procedure allowed a precise anatomic reconstruction, it was not a panacea [55, 148]. In inexperienced hands, urologic injuries were observed during attempted repair of anorectal malformations [68]. Although the rate of continence improved (70% in Peña's hands; Fig. 1.4), it became apparent that many of the children had significant motility disorders, and fecal retention was a major problem [121, 122, 126]. Heightened awareness of this problem led to the establishment of close follow-up programs to assure patient and parent compliance with postoperative anal dilatation and appropriate rectal washouts using enemas [125, 126]. With the advent of the new procedure and continued controversy, an additional conference was held in 1984 at the Wingspread Conference Center in Wisconsin to reevaluate ARM with regard to classification, embryology, anatomy, and treatment. The Wingspread classification simplified the male/female low, intermediate, and high variants decided upon in 1970, transferred many female cloacal variants to separate subgroups and characterized the more uncommon anomalies as rare conditions [34]. Bladder and cloacal exstrophy and the various neuropathies were excluded. The presentations at the Wingspread Conference were edited by Douglas Stephens and E. Durham Smith and were published as a Birth Defects monograph by the March of Dimes in 1988 [158, 159]. The 1980s and early 1990s were periods that were also characterized by children's surgeons paying much closer attention to recognition and repair of cloacal anomalies. Dr. Raffensperger [132] of Chicago, and especially Dr. Hendren of Boston were instrumental in developing methods of repair (Fig. 1.5) [55-62, 97]. Later, Peña (and his associates) employed the posterior sagittal approach to repair cloacal anomalies and developed considerable experience in the management of these patients [92, 93, 124, 128, 151]. Peña also championed the concept of urogenital advancement in the repair of high cloacal defects [124]. Updated information concerning the anatomy and embryology of cloaca was published in a 1996 textbook edited by Stephens, Smith, and Hutson [160].

In 1992, Malone and associates described the antegrade colonic enema concept using an appendicos-tomy on the anterior abdominal wall or hidden within the umbilicus to flush the colon (MACE procedure) as an alternative to traditional retrograde enema washouts from below in children with incontinence or significant fecal retention [99]. Similarly, subsequent reports by Ellsworth et al. [40], Wilcox and Kiely [172], Squire et al. [152], and Levitt et al. [91] found the procedure useful. The Chait percutaneous cecostomy tube [21, 90] and Gauderer sigmoid button [47] were suggested as alternatives. Brent and Stephens [15] had identified the problem of rectal ectasia in 1976 and this was reaffirmed by Cloutier et al. [25], Cheu and Grosfeld [23] and, more recently, Peña and associates [126]. Of interest is that rectal ectasia occurs more commonly in children with low anomalies and may result from longstanding distension of the rectal segment in utero. Some of these patients (including those with megasigmoid) responded favorably to resection of the abnormal bowel [23, 126]. Despite the apparent popularity of the PSARP operation, in the 1990s several articles appeared in the literature recommending anterior sagittal anorectoplasty, anterior perineal anorectoplasty, and other modifications of the Mollard approach [2, 20, 37, 115].

In the 1990s, advances in technology resulted in new methods of assessment of patients with ARM and their associated anomalies. Transperineal ultra-sonography was used to locate the infracoccygeal level of atresia and identify fistulae [52, 82]. Magnetic resonance imaging proved useful in evaluating the pelvic and perineal muscle status and identifying instances of tethered cord, vertebral anomalies, and spinal dysraphic syndromes [96, 137, 166]. Gross and Peña emphasized the value of a pressure-augmented distal colostogram to define the presence and location

Distal Colostogram
Fig. 4 Alberto Peña - advocated the posterior sagittal anorectoplasty (PSARP) procedure for ARM

Fig. 5 W. Hardy Hendren - was a leader in the management of cloacal anomalies of the fistula in patients who required an initial colostomy in the neonatal period prior to definitive repair [50]. Postoperative assessment of sphincter muscle function and the position of the pulled-through segment within the sphincter complex is aided by anal endosonography [73, 162]. The advent of minimally invasive surgical techniques also influenced surgical treatment. Georgeson [48] and others [164] employed a laparoscope-assisted one-stage AP pull-through procedure that provided excellent exposure deep in the pelvis, divided the fistula using a harmonic scalpel or endoscopic clips, identified the sphincter complex and puborectalis muscle, and passed the colon through an intact sphincter to the anal dimple where an anoplasty was constructed in the neonatal period. Laparotomy and transsacral incisions were avoided and postoperative pain was diminished. Iwanaka et al. [71] and Yamataka et al. [175] used a laparoscopic muscle stimulator to accurately locate the sphincter during laparoscope-assisted repair. Adeniran supported a one-stage repair of imperforate anus and rectovestibular fistula in girls [2]. Albanese and colleagues advocated a one-stage correction of high ARM in the male neonate as a method of preserving the neural framework for normal bladder and bowel function that exist at the time of birth [3]. The learning period when neuronal circuitry development takes place resulting in normal or near-normal ano-rectal function occurs early and delayed repair may lose critical time when neural networks and impulses form [3, 45]. The one-stage operation avoids a colostomy and its potential complications, including urinary tract colonization and infection. Results are not yet available to determine whether these modifications will remain enduring and result in favorable long-term outcomes.

Animal models became available to study the embryology and anatomic features of ARM, including cloacal anomalies in pigs [69], mice [84, 85, 95], rats [95, 129], and chick embryos [100]. In mice, defects in sonic hedgehog signaling and mutations in the Gli 2 and 3 genes have been identified that hinder hind-gut development [72, 83, 84, 109]. Careful three-dimensional reconstructive studies of embryos from the Carnegie collection by Paidas [116] and Rogers et al. [139] have led to further observations concerning the human embryology of ARM and cloaca, updating de Vries and Friedland's previous contributions [32].

Constipation Prescription

Constipation Prescription

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