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Intraoperative endosonography enhances laparoscopy-assisted colon pull-through for high imperforate anus.

PURPOSE: The authors used ultrasonographic endoprobes during laparoscopy-assisted colon pull-through (LACPT) for the repair of high imperforate anus to confirm the pull-through canal was surrounded symmetrically by pelvic floor muscles. METHODS: Six patients with high imperforate anus were treated by LACPT (mean age at LACPT, 8.2 months). An endoscopic (12-MHz, 2.5-mm in diameter) and proctoscopic (7.5-MHz, 12-mm in diameter) probe were inserted into the proposed route of dissection intraoperatively to measure the thickness of the surrounding muscle tissue at at least 3 levels: the external anal sphincter, the levator ani muscle sling, and the intervening muscle complex. RESULTS: The average thickness of the external anal sphincter was 2.3 +/- 0.4 mm anteriorly, 2.4 +/- 0.4 mm on the left, 2.4 +/- 0.5 mm posteriorly, and 2.6 +/- 0.6 mm on the right. The average thickness of the muscle complex was 2.3 +/- 0.6 mm anteriorly, 2.2 +/- 0.5 mm on the left, 2.1 +/- 0.4 mm posteriorly, and 2.2 +/- 0.5 mm on the right. The average thickness of the left crus of the levator ani muscle was 1.8 +/- 0.3 mm, the right crus was 1.9 +/- 0.4 mm, and the rim located posterior to the rectum was 2.0 +/- 0.3 mm. No statistically significant difference was found between the measurements taken at each level. CONCLUSION: Intraoperative endosonography during LACPT can greatly enhance the precision of positioning the pull-through canal.

Anus, Imperforate↗

Position of rectal fistula in relation to the hymen in 46 girls with imperforate anus.

Forty-six records of infant girls with so-called imperforate anus have been reviewed to determine the position of the rectal opening in relation to the hymen. Eight of these children proved to have the cloacal deformity, in which the rectum enters high into a single tube and just behind the opening of a double or septate vagina, with the urethra entering anteriorly at the same level. No normal hymen was visible in these eight children. Seventeen patients had a normal-appearing hymen, and no rectal opening on the perineum. In each of these 17 children the rectal opening was found above the tissue flap overlying the posterior vestibule. In the remaining 21 patients, the hymen was visible and appeared normal, and there was a rectal opening somewhere on the perineum between the vestibule and the normal position for the external sphincters. These findings suggest that in the presence of a normal-appearing hymen, and in the absence of a normal anus, the rectal opening will be either in the posterior part of the vestibule or on the perineum. Where no hymen was visible, we have found in a limited experience that the rectal opening was high in the pelvis in some degree of the so-called cloacal deformity.

Anus, Imperforate↗

Cloaca, the most severe degree of imperforate anus: experience with 195 cases.

OBJECTIVE: To provide a follow-up of 195 patients with cloacal malformations seen by the author from 1959 to 1998. SUMMARY BACKGROUND DATA: Cloaca, which occurs in approximately 1 of 50,000 births, is the most complex type of imperforate anus with confluence of the rectum, vagina, and bladder in a urogenital sinus. Functional results for the bowel, the genital tract, and the urinary tract were formerly poor. Cloacal exstrophy, which is an even more complex spectrum of malformations, was uniformly fatal until 1960. In addition to imperforate anus, these babies have an omphalocele, two exstrophic bladders, between which there is an open cecum, and a blindly ending colon hanging down in the pelvis from the cecum. Although both of these diagnoses contain the word "cloaca," which is Latin for sewer, they are really two separate entities in terms of surgical management. Cloaca and cloacal exstrophy in most cases are very different anatomic problems. However, there are variants that are like a hybrid, which is the rationale for reporting together an experience with both entities. METHODS: Records were reviewed of 154 patients with cloaca and 41 patients with cloacal exstrophy to assess anorectal function, urinary continence, and sexual function where available. RESULTS: Follow-up was available in 141 cloaca patients: 82 have spontaneous bowel movements and satisfactory control, 38 use enemas to evacuate, 9 have a colostomy, 7 have fecal soiling, and 5 are too recently operated to evaluate. Regarding urinary control, 83 void spontaneously, 40 catheterize to empty, 4 have urinary diversion, 1 has a continent diversion, 5 patients are wet, and 8 are too recently operated to judge. Twenty-four patients are now adults, 17 of who have experienced coitus and 7 have not. Seven have had babies, all except one by cesarean section. Results of surgery for cloacal exstrophy are not as good, but are encouraging nonetheless for an anomaly that was uniformly fatal before 1960. Of the 41 cloacal exstrophy patients being followed, 7 have not undergone surgery. Fifteen have a colostomy; 19 had pull-through of the colon, but 3 were subsequently reversed for fecal incontinence. Most depend on enemas to evacuate. Urinary dryness was attained in 30 patients, usually by intermittent catheterization of the bladder, which was augmented with small bowel or stomach or both. Only three void voluntarily. Fifteen of the completed long-term patients wear no bag. Only three of the completed patients wear two bags. The rest have one bag. CONCLUSIONS: Imperforate anus and associated malformations in cloaca and cloacal exstrophy are not hopeless problems. A reasonable lifestyle can be achieved for most of these children with comprehensive surgical planning.

Anus, Imperforate↗

Double bladder and urethra with imperforate anus and ureterorenal reflux: a case presentation with review of the literature.

A 23-month-old girl with an imperforate anus, which was corrected surgically when she was 1 day old, presented with a 2-month history of recurrent urinary infections. Evaluation revealed a single kidney and ureter draining into duplicate ipsilateral bladders, which in turn were drained by separate side-by-side urethras. Right ureterorenal reflux also was noted. This condition could easily have been undiagnosed if infection with symptoms had not been present. Any patient with an imperforate anus and any urinary symptomatology should undergo complete urological evaluation.

Abnormalities, Multiple↗

Earlier appearance and higher incidence of the rectoanal relaxation reflex in patients with imperforate anus repaired with laparoscopically assisted anorectoplasty.

BACKGROUND: This study aimed to evaluate clinically and manometrically the anorectal function of patients with imperforate anus after repair with laparoscopically assisted anorectoplasty (LAR), as compared with the function of patients after undergoing the conventional method, posterior sagittal anorectoplasty (PSARP). METHODS: The defecation status and anorectal manometry of patients with high or intermediate type imperforate anus repaired with LAR ( n = 9) and age-matched patients repaired with PSARP ( n = 13) were assessed and compared during the first year of postoperative follow-up evaluation. The defecation status was classified by the frequency of bowel openings (<1, 1-4, and >5 times per day). Manometric assessment was performed by an open-tip hydraulic capillary infusion system. The presence of the rectoanal relaxation reflex was determined, and the resting sphincteric pressure and resting rectal pressure were measured. RESULTS: Seven of nine LAR patients had an "acceptable" frequency of one to four bowel openings per day, in contrast to 7 of 13 PSARP patients. The difference in the presentation of daily stooling is not significant ( p > 0.05). A positive RAR was detected in 88.9% (8/9) of the LAR patients, and in only 30.8% (4/13) of the PSARP patients ( p < 0.01). The presence of a rectoanal relaxation reflex also significantly correlated with an acceptable frequency of bowel opening (1-4 times per day) in both LAR and PSARP patients ( p < 0.05). Moreover, a rectoanal relaxation reflex was detected significantly earlier in LAR than in PSARP patients (4.9 +/- 1.2 vs 10.1 +/- 2.5 months; postoperatively p < 0.0001). Both the LAR and PSARP patients had a similar resting sphincteric pressure (21.5 +/- 4.7 vs 25.4 +/- 6.2 cm H2O; p > 0.05). By contrast, the resting rectal pressure was significantly lower in LAR than in PSARP patients (7.7 +/- 1.5 vs 11.5 +/- 1.3 cmH(2)O; p < 0.05). CONCLUSIONS: In the early postoperative stage, patients repaired with LAR had more favorable findings in anorectal manometry than patients repaired with PSARP. Long-term follow-up studies to confirm a superior defecation continence achieved with LAR are warranted.

Abnormalities, Multiple↗

Laparoscopically assisted anorectal pull-through for high imperforate anus: three years' experience.

PURPOSE: We describe our three-year experience with the laparoscopically assisted anorectal pullthrough for high imperforate anus using laparoscopic muscle electrostimulation. MATERIALS AND METHODS: From March 2001 to January 2004, 7 patients with a diagnosis of high anorectal malformation underwent laparoscopically assisted anorectal pull-through. The patients, all males aged from 4 to 9 months (mean age, 5.8 months), presented with a rectourethral fistula. The associated malformations noted were sacral malformation, laryngeal stenosis, urethral duplication, multicystic kidney, nonpalpable testis, and esophageal atresia. All patients were treated with a colostomy in the newborn period followed by a delayed laparoscopically assisted anorectal pullthrough. Laparoscopy included stimulation of the puborectal muscle, using a modified Peña electrostimulator introduced through a trocar. All patients underwent a postoperative period of anal dilatation. RESULTS: In 6 cases the laparoscopically assisted anorectal pull-through was successful; there was 1 conversion to the open technique, due to strong tension from the colostomy. CONCLUSION: Although longer follow-up to evaluate continence is to come, laparoscopically assisted anorectal pull-through should be considered for the correction of the high imperforate anus and, according to our experience, it represents the gold standard. It offers the advantage of good visualization of the fistula and the surrounding structures and minimally invasive abdominal and perineal wounds. With the laparoscopic Peña stimulator the direct observation of the contraction of the puborectalis sling allows an evaluation of the functional contractility and an accurate colonic pullthrough in the center of the muscle complex.

Anus, Imperforate↗

Anterior perineal anorectoplasty for intermediate and high imperforate anus.

Anterior perineal anorectoplasty is a new technique for the repair of high imperforate anus. This technique allows direct access and clear visualization of the puborectalis sling and rectal pouch; division of the fistula and mobilization of the rectum can be precisely performed under direct vision, thereby reducing injury to surrounding structures; the pull-through of the rectum is readily facilitated; it obviates the need to change the position of the patient during the procedure; and mobilization of the rectum required to pull the rectum through is minimal, hence the late complication of mucosal prolapse is avoided. Details of the operative procedure are described herein.

Anal Canal↗

Enterolithiasis with imperforate anus: report of a case.

A three-year-old child presented with imperforate anus. A local perineal procedure was performed at birth, possibly without repairing the fistula. The child later presented with severe anal stenosis, which required a divided sigmoid colostomy. The child later presented at the age of two years with multiple radio-opaque shadows in the pelvis. These proved to be enteroliths, which had developed in the distal rectal stump possibly due to a large associated recto-urethral fistula with associated urinary stasis.

Anus, Imperforate↗

Hyperchloremic acidosis in an infant with imperforate anus and rectourethral fistula.

A case of hyperchloremic acidosis in an infant with imperforate anus and rectourethral fistula, showing lethargy, tachypnea, vomiting, and dehydration, is reported. Surgical correction by abdominoperineal pull-through and division of the rectourethral fistula was performed after doing a cystocutaneostomy to eliminate the diversion of urine into the rectum.

Acidosis↗

Transient ureteric dilatation in neonates with imperforate anus: a report of 4 cases.

In a series of 64 patients with imperforate anus, 4 were identified with ureteric dilatation without vesico-ureteric reflux. The appearance resembled that of the primary obstructive megaureter. However, since the dilation resolved spontaneously in each case, it is concluded that the condition represents a separate entity, the cause of which has not yet been elucidated.

Anus, Imperforate↗

Effect of loperamide on faecal control after rectoplasty for high imperforate anus.

The effect of loperamide on faecal control after Stephens rectoplasty for high imperforate anus was studied in eight patients more than 8 years after surgery. Clinical assessment and anorectal manometry were performed before and after administration of loperamide for 6 to 8 weeks. Decreased faecal soiling was noted in seven of the eight cases. Obstipation forced two patients to stop the medication. The improved faecal control was verified by an increase in squeeze pressure at anorectal manometry in four patients. When incontinence persists after Stephens rectoplasty, loperamide can improve the faecal control in some cases.

Adolescent↗

Laparoscopically assisted anorectal pull-through for high imperforate anus--a new technique.

BACKGROUND/PURPOSE: This report describes a new technique of laparoscopically assisted anorectal pull-through (LAARP) for repair of high imperforate anus. The procedure utilizes minimal perineal dissection, preservation of the distal rectum, and accurate placement of the rectum within the levator ani and external anal sphincter muscle complex. METHODS: Sharp dissection and cautery was used laparoscopically to expose the rectal pouch down to the urethral or vaginal fistula, which was clipped distally and divided. The pelvic floor musculature was then assessed and the levator sling identified. Externally, electrostimulation was used to define the center of the anal dimple. An 8-mm skin incision was made, centered at the strongest cephalad contraction. Using a hemostat, minimal blunt dissection on the perineum was guided by transillumination from the laparoscopic light source. A trocar, consisting of a radially expandable sheath over a Varess needle, was passed through this defined plane in the external sphincter muscle complex and advanced into the pelvis between the 2 bellies of the pubococcygeus muscle, guided by laparoscopic visualization. This perineal trocar therefore formed a passage through the center of the striated muscle complex and levators. The rectal fistula, which had been dissected out laparoscopically, was grasped using the perineal trocar and exteriorized to the perineum. Anorectal anastomosis was performed with absorbable interrupted suture. RESULTS: Seven patients were treated with initial colostomy in the newborn period followed by delayed LAARP 2 to 12 months later. In 4 newborn infants, the LAARP was performed as a primary procedure without prior colostomy. Laparoscopic mobilization has been possible on all cases attempted. All of the patients have a brisk and symmetric anal contraction with perineal electrostimulation. CONCLUSIONS: Lack of long-term follow-up precludes accurate assessment of the potential for fecal continence. However, short-term experience has been that this new method of pull-through for imperforate anus offers many advantages, including excellent visualization of the rectal fistula and surrounding structures, accurate placement of the bowel through the anatomic midline and levator sling, and minimally invasive abdominal and perineal wounds.

Anus, Imperforate↗

Anal sphincter function after surgery for high imperforate anus--a long term follow-up investigation.

37 children were treated for high imperforate anus during the years 1944-1965. Eighteen of these 37 participated in a follow-up examination at the age of 18-35 years. The examination consisted of an anorectal manometry, EMG of the external sphincter, a digital anal examination, and an interview. Special attention was paid to anal continence. Fourteen patients did not admit any major anal problems, and eight of them had a good continence. Anorectal manometry showed an absence of internal sphincter function in all patients. During voluntary squeeze the continent patients increased anal pressure significantly more than the incontinent ones, and they were also aware of significantly smaller distensions of the rectal ampulla. The present investigation shows the necessity of well-functioning striated sphincter muscles to achieve good continence, and therefore also the importance of careful dissection and identification of the striated sphincter muscles during the operation.

Adolescent↗

Primary vesicoureteric reflux in neonates with imperforate anus.

Cystography, performed in 26 out of a total of 33 consecutive neonates with imperforate anus, revealed primary vesicoureteric reflux in 12 cases. The reflux was usually pronounced, and was particularly common in females and in infants with urinary tract infections. The incidence of reflux was unrelated to the severity of the anorectal anomaly, and reflux was often demonstrated in patients in whom an intravenous pyelogram had shown an undilated upper renal tract.

Anus, Imperforate↗

Isolated imperforate anus in monozygotic twins: case report and implications.

The authors report a case of isolated imperforate anus with perineal fistula in monozygotic twins. Only 4 other well-documented cases of monozygotic twins concordant for isolated anorectal malformation have been reported in the literature. In all these cases the defect occurred below the levator ani muscle. This case confirms previous reports on the incidence of low anorectal anomalies in monozygotic twins where the defect was isolated. A review of the literature relating to this condition in twins and siblings points toward low anomalies and more severe malformations having different genetic backgrounds.

Adult↗