Bladder outlet obstruction causes fetal enterolithiasis in anorectal malformation with rectourinary fistula

Extraluminal calcified meconium is found frequently by prenatal ultrasound in cases with bowel perforation and meconium peritonitis. Intraluminal intestinal meconium calcifications are rarely seen in prenatal sonography. Meconium calcifications result from a mixture of meconium and urine that indicates a connection between intestinal and urinary tract.We report a case of a male newborn prenatally diagnosed with intraluminal echogenic calcifications at 23 weeks of gestation, suggesting an anorectal malformation (ARM) with rectourinary fistula. At birth, the child presented with a complex ARM including high anal atresia with both perineal and rectourethral fistula. Furthermore, a bladder outlet obstruction due to a urethral stenosis was diagnosed. Vesicostomy was performed as an emergency procedure followed by colostomy during neonatal period. Posterior sagittal anorectoplasty was performed at the age of 4 months.Prenatal echogenic calcifications within bowel should raise the suspicion of ARM with rectourinary fistula and bladder outlet obstruction.     

Robotic-assisted anorectal pull-through for anorectal malformations

Background/purpose

Many reports have addressed the feasibility and safety of using robotic surgery in children. To our knowledge, no published report has described the use of a surgical robot in the repair of anorectal malformations (ARMs).

Methods

Included children underwent robotic-assisted repair of ARMs with rectourethral fistula between April 2006 and March 2010 at King Khalid University Hospital, Riyadh, Saudi Arabia, using the da Vinci Surgical System. Their medical records were reviewed with respect to demographic data, associated anomalies, techniques and operative procedures, complications, outcomes, and follow-up.

Results

Five male infants (mean age, 6.6 months) underwent robotic-assisted repair of ARMs with rectourethral fistula using the Georgeson technique. The fistulae were divided and ligated in 4 patients and was left open in 1. All procedures were successfully completed without conversion to an open technique. One patient developed left-sided epididymo-orchitis postoperatively. All the patients had their colostomy closed. The follow-up ranged from 6 to 36 months. Fecal continence was difficult to assess in 2 patients. Two patients have voluntary bowel movements without soiling. One infant has fecal soiling and is on a laxative/enema for constipation.

Conclusions

Robotically assisted repair of ARMs with rectourethral fistula is feasible and safe. It offers a good alternative to the criterion standard, posterior sagittal anorectoplasty (PSARP), for repair of ARMs with rectourethral fistula. More patients and a longer follow-up period are needed for further evaluation of this novel approach.     

Laparoscopy and its use in the repair of anorectal malformations

Introduction

Laparoscopy has been used for the treatment of anorectal malformations (ARMs) in an attempt to be less invasive and with the hope that it would result in a better functional outcome. There remains a significant debate about whether these expectations have been fulfilled.

Methods

Seventeen patients with ARM for whom laparoscopy was used were retrospectively reviewed. Six were operated on primarily by the authors, and 11 cases were referred after a laparoscopic repair performed elsewhere. In addition, a literature review was performed looking for evidence of less invasiveness and improved functional results in patients operated on laparoscopically.

Results

The diagnosis was imperforate anus with a rectobladder neck fistula in our 6 cases with the fistula ligated laparoscopically in each case. In 1 patient, the malformation was repaired entirely using laparoscopic technique. The other 5 patients had a laparoscopically assisted repair because we had to open the abdomen to taper a dilated rectum in 2, mobilize a very high rectum in 2, and take down a distal colostomy stoma in 1. Eleven patients were referred with a variety of problems after a laparoscopic repair done elsewhere for rectal stricture (5), rectal prolapse (4), recurrent rectourethral fistula (3), rectal mislocation (3), failed attempted repair leading to fecal incontinence (1), and a posterior urethral diverticulum (1). Our literature review included 47 references (involving 323 patients) published between 1998 and 2010. All studies showed that laparoscopic repair of ARMs is feasible. The review, however, did not provide evidence of less invasiveness or improved functional results.

Conclusions

Laparoscopy for ARM is a less invasive procedure when compared with those operations that would have previously required a laparotomy (rectobladder neck fistula). In cases of rectoprostatic fistulae, the laparoscopic approach is feasible and avoids a lengthy posterior sagittal incision. There is no evidence that the laparoscopic approach is a less invasive procedure for other types of ARMs. In cases of rectobulbar fistula, congenital anal stenosis, perineal fistula, ARM without fistula, the evidence suggests that it may be lead to more complications. There is no evidence in the literature demonstrating better functional results in cases of ARM operated on laparoscopically.     

The posterior sagittal trans-sphincteric approach

The posterior sagittal, transphincteric approach to treat different pelvic problems has been known since last century. Although some surgeons have embraced it and have enthusiastically advocated it s use, it has never become an overly popular technique. The purpose of this study is to evaluate the advantages and disadvantages of the approach, both from an historical perspective and from the authors experience. The international literature on the subject was reviewed since 1877 up to the present date. A retrospective evaluation of the authors experience was conducted, and the results reviewed. Specific attention was paid to the final result obtained in the treatment of the original condition, surgical complications and the effect of the surgical approach on bowel and urinary control. The experiential review included 114 cases. They were divided into two groups. A included 85 patients who underwent a posterior sagittal transphincteric approach that included 49 cases of Hirschsprung s disease (primary 21, secondary 28), 15 presacral masses; 10 rectaltumors; 7 acquired recto-genito-urinary fistulae; and 4 cases of idiopathic rectal prolapse. Group B included 29 patients who underwent a posterior sagittal trans-anorectal approach, in which the anterior wall of the rectum and the sphincter was divided as well.. This group included 12 cases of urogenital sinuses; 8 acquired urethral stricture or atresia after trauma; and 9 posterior urethral masses. Post-operative bowel control was normal all cases except in those patients whose basic condition had resulted in fecal incontinence, or who had sustained an irreversible injury prior tothe operation. Urinary control was normal except in cases with pre-operative incontinence. Complications included recurrence of recto-genitourinary fistulae in 3 cases, recto-cutaneous fistula in 3 Hirschsprung s patients and 2 partial wound dehisences. The posterior sagittal trans-sphincteric approach represents a useful technical alternative. It seems to be particularly useful to treat complications after surgery for Hirschsprung s disease, pre-sacral masses, acquired recto-genito-urinary fistula and idiopathic rectal prolapse. The transanorectal approach provides excellent exposure to the posterior urethra and vagina. Boweland urinary control are not compromised.     

腹腔镜辅助高位肛门闭锁手术的初步经验

目的探讨腹腔镜技术在治疗小儿高位肛门闭锁中的应用价值。方法本组高位肛门闭锁患儿共6例,其中2例合并直肠尿道瘘,1例合并一穴肛畸形。术中在腹腔镜下游离直肠,结扎离断直肠尿道瘘(阴道瘘)。找到耻骨直肠肌收缩中心并加以扩张,将直肠盲端自此隧道中拖出与会阴部皮肤缝合。3例并发球形结肠患儿,在腹外切除病变结肠,将近端结肠自耻骨直肠肌中心拖出行括约肌成形术。结果本组6例的手术均顺利完成,腹腔镜操作时间70～110 min(平均82 min),出血量10～20ml(平均14.5ml)。1例行臀大肌瓣转移外括约肌成形术,术后发生感染,改行横结肠单口造瘘术。本组无尿道瘘(阴道瘘)、憩室及切口感染的发生。术后随访6例3个月～3年,肛门无狭窄,大便控制良好。结论腹腔镜辅助便于处理直肠尿道瘘(阴道瘘),可准确辨认耻骨直肠肌收缩中心,减少了术后大便失禁的发生。     

The Gonzalez hernia revisited: Use of the ischiorectal fat pad to aid in the repair of rectovaginal and rectourethral fistulae

Introduction

During the development of the posterior sagittal approach to anorectal malformations a vital technical challenge was a precise midline dissection, which if off, allowed for the ischiorectal fat pad to bulge into the wound. This occurrence became affectionately known as a “Gonzalez hernia”, after a trainee of Dr Pena’s (and a co-author of this paper). We describe here an innovative use of the ischiorectal fat pad to aid in the repair of acquired rectovaginal and rectourethral fistulae.

Methods

Patients with recurrent vaginal or urethral fistulae were selected for review. The ischiorectal fat pad was deliberately mobilized (via a posterior sagittal or transanal approach) and used to buttress the repair of the posterior vagina or urethra.

Results

The ischiorectal fat pad technique was used in 9 patients. All had an acquired fistula (6 rectovaginal fistula, 3 rectourethral fistulas). We used the posterior sagittal approach in 7 and in 2 the transanal approach. Six patients had had at least two prior attempts at fistula repair. Six patients had a stoma, and 3 did not. There were no recurrences in greater than six month follow-up.

Discussion

The ischiorectal fat pad is easily visualized and mobilized, either via a posterior sagittal or transanal approach, providing excellent coverage with native, well-vascularized tissue, in an area that is difficult to heal. It is an excellent option for recurrent rectovaginal and rectovaginal fistulae and may have other additional creative applications.     

Dartos flap interposition in the surgical repair of rectourethral fistulas

BACKGROUND: Multilayer closures are important techniques in urethral fistula repair. We report our experience with dartos flap interposition in the repair of rectourethral fistula. METHODS/RESULTS: A tip of a V-shaped scrotal skin flap is mobilized and de-epithelialized to develop a dartos flap. Two patients with a rectourethral fistula underwent fistula closure covered with this dartos flap. The surgical repair was successful in both cases and neither patient showed any late complication. CONCLUSIONS: A scrotal dartos flap is well vascularized and easy to mobilize. We believe this technique is helpful in preventing postoperative complications following complex posterior urethral surgery.     

Complex urethral disruptions: In pursuit of a successful reconstruction

OBJECTIVES: We analyzed the methods and outcomes of urethroplasty in men with complex urethral disruptions. METHODS: The medical records of 40 men with complex urethral disruptions were analyzed. Surgical methods were individualized according to stricture location, severity and length of the stricture, bladder neck characteristics and presence of complicating factors. Patients were divided into four groups based on the above characteristics. RESULTS: End-to-end urethroplasty performed in six patients with short bulbar strictures (<3 cm) was successful in all. Elaborated perineal repair was performed in 10 patients with intermediate (3-6 cm) strictures with or without complicating factors. Elaborated perineal repair with urethral substitution was performed in nine patients with long segment stricture (>6 cm). Abdominal transpubic repair was successfully applied to patients with rectourethral fistula or lacerated bladder neck. Success rate of anastomotic urethroplasty was 95% while over all success rate was 85%. CONCLUSION: Guidelines for urethral reconstruction of complex urethral disruptions are predicated on stricture length, location, bladder neck characteristics and associated complicating factors. End-to-end urethroplasty with stricture excision is highly reliable for short strictures for which previous operative repair have failed. Elaborated perineal repair is extremely versatile for intermediate and longer strictures with associated complicating factors. Abdominal transpubic urethroplasty is effective for patients with rectourethral fistula or lacerated bladder neck.     

Diagnosis and management of congenital rectourethral fistula in a child with long tubular duplication of the colon and Klippel-Feil syndrome

We describe a unique association of congenital rectourethral fistula with long tubular duplication of the colon in a boy with Klippel-Feil syndrome and Sprengel deformity. He presented with a rectourethral fistula after surgical repair of a tubular duplication of the terminal ileum, colon, and proximal rectum. Preoperative identification of the fistula was challenging and was only achieved after cystoscopy with injection of methylene blue under pressure through Foley catheters placed into the anus and distal stoma of a colostomy. Surgical repair was performed through the posterior sagittal approach. The patient is doing well after 4 years of follow-up.     

Transanorectal approach for the treatment of urogenital sinus: preliminary report.

The treatment of the urogenital sinus with normal rectum still represents a challenge. A perineal approach with or without a skin flap seems to be effective for those patients with a low implantation of the vagina. However, in patients with a high vaginal implantation, this treatment frequently fails to provide a good, functional vagina due to a narrow, strictured vaginal opening. Based on previous experience in the treatment of more than 80 patients with a persistent cloaca, a posterior sagittal transanorectal approach with a protective colostomy was performed in three patients with urogenital sinus and normal rectum. The pelvis was approached through a midsagittal posterior incision; the coccyx was split and the entire anorectal sphincteric mechanism was divided in the midline. The rectum was bivalved in the midline including both posterior and anterior rectal walls. This provided excellent exposure to the urogenital sinus. The vagina was then fully separated from the urogenital sinus (as described in cases of persistent cloacas), and then mobilized and sutured to the perineum. The rectum and sphincteric mechanism were meticulously reconstructed. A midline incision assures the preservation of anorectal innervation, and provides excellent exposure to the pelvis. Anal dilatations are not necessary to maintain a patent and supple anorectal opening because the rectum has two suture lines, one in front of the other. After the colostomy was closed, all patients had appropriate bowel control for their age; two of them are fully continent for urine and the third one still has a suprapubic cystostomy tube waiting for a repair of an additional urethral malformation.     

The management of old urethral injury in young girls: analysis of 44 cases

Background/purpose: Traumatic urethral injury in girls is rare, and there is no consensus on its management. The authors report their 22-year experience.Methods: Forty girls presented with urethrovaginal fistula. Twenty-six girls presented with cystostomy tube in place, whereas 17 girls presented with complete urinary incontinence. Incision and dilatation of the obliterated urethra was carried out in 7 patients. Vaginal repair of urethrovaginal fistula was performed in 4 patients. Transpubic reconstruction of the urethra using a modified Young-Dees-Leadbetter procedure with simultaneous repair of the urethrovaginal fistula was performed in 35 patients (once in 27, twice in 5, and 3 times in 3 patients).Results: Follow-up in 40 girls averaged 3.5 years. Twenty-nine patients have regained normal urinary control, and 11 patients have mild stress urinary incontinence. Four patients were lost to follow-up.Conclusions: Simple dilation of the obliterated urethra can reestablish satisfactory urethral patency if the obliterated segment is short. The vaginal approach to urethrovaginal fistula may be successful in patients without concomitant urethral stricture or in those with stricture amenable to simple dilation. The transpubic approach remains the method of choice for repairing complete urethral disruption and severe urethral stricture, especially when associated with urethrovaginal fistula.     

Perineal subcutaneous dartos pedicled flap as a new technique for repairing urethrorectal fistula

PURPOSE: Urethrorectal fistulas are rare, and the etiology is usually traumatic or iatrogenic (postoperative). Several operative approaches and techniques have been used for fistulous repair but no procedure has proved to be the best or universally acceptable. We present a new technique for repairing urethrorectal fistulas. MATERIALS AND METHODS: We successfully treated 12 male patients 7 to 65 years old who presented with urethrorectal fistula from 1990 to 1997 using the perineal subcutaneous dartos pedicled flap procedure. Urethrorectal fistulas resulted from crush pelvic injury in 6 cases and gunshot in 2, and developed after prostatectomy in 4. The fistula was associated with urethral stricture in 4 cases. A perineal approach was used in all cases of urethrorectal fistula and combined with the transsymphyseal approach in the 4 patients with posterior urethral stricture. We interposed a subcutaneous dartos pedicled flap as a vascularized tissue flap between the repaired rectum and urethra. RESULTS: The results of our technique were excellent in all cases. No leakage or perineal collection developed and there was no fistula recurrence. In 1 patient urethral stricture was managed by visual internal urethrotomy. Loss of the internal and external sphincters resulted in urinary incontinence in 4 cases, involving gunshot injury (2), crush pelvic injury (1) and prostatectomy (1). Followup ranged from 9 to 42 months. CONCLUSIONS: Our technique of a perineal subcutaneous dartos pedicled flap fulfills all principles of the successful repair of urethrorectal fistula. We consider it to be an ideal solution to this urological dilemma.     

Urethroplasty and vaginoplasty by a single small bowel interposition graft: a novel technique for cloacal reconstruction

Despite the advances in surgical management of anorectal malformations, repair of cloacal malformations remains a challenging condition for many pediatric surgeons. Posterior cloaca is present where urogenital sinus deviates posteriorly to form a cloaca with rectum. Often, achieving adequate urethral and vaginal length can be challenging. We describe a novel technique where a loop of small bowel on a single mesenteric blood supply could be constructed in 2 functional tubular structures: one for urethral and the other for vaginal replacement.     

Posterior sagittal anorectoplasty: important technical considerations and new applications

Posterior sagittal anorectoplasty (PSARP) is a new technique for the repair of high anorectal malformations. It is based upon complete exposure of the anorectal region by means of a median sagittal incision that runs from the sacrum to the anal dimple, cutting through all muscle structures behind the rectum. It was learned through this procedure that the external sphincter is a functionally useful prominent structure. No puborectalis sling, as such, could be identified. It was possible, however, to recognize a muscle continuity from the skin to the sacral insertion of the levator ani. Since it is impossible to pull the generally ectatic rectum through without destroying the muscle structures present, the rectum must be tapered to allow suturing the muscle behind it. In all these anomalies, the rectum and urethra (or vagina) are very closely joined, sharing a common wall, and their separation calls for extensive exposure. A number of technical details clarified in the course of applying the procedure in 54 patients are fully discussed. This approach also proved to be very successful in the management of two patients with rectocloacal fistula, two with rectal atresia and two with stenosis. Colostomy has been closed in 27 patients and fecal continence may be described as excellent, except in those patients with severe sacral anomalies, and unquestionably superior to that obtained by us with other techniques.     

Posterior sagittal anorectoplasty for failed imperforate anus surgery: lessons learned from secondary repairs

PURPOSE: The aim of this study was to evaluate secondary operations using a posterior sagittal approach in patients with fecal incontinence and impaction after primary repair of anorectal malformations. METHODS: Twenty patients (14 boys, 6 girls) who had previous failed surgery for imperforate anus underwent secondary operations. The indications for surgery included fecal incontinence (n = 16) and fecal impaction (n = 4). Patients ranged in age from 2 to 30 years (mean, 11 years), with 4 over the age of 20 years. The primary procedures included abdominosacroperineal (n = 7), sacroperineal (n = 10), and perineal (n = 3) pull-throughs. At surgery, none of the patients underwent a diverting colostomy. The rectum was mobilized from the surrounding structures through a posterior sagittal approach. The surgical findings included anteriorly displaced anus (n = 17), laterally displaced anus (n = 3), mesenteric fat surrounding the rectum (n = 4), mega-rectosigmoid (n = 2), and others (n = 3). The rectum underwent reconstruction, which involved relocation of the rectum and anus to surround them with the muscle complex. RESULTS: Patients underwent follow-up for periods ranging from 8 months to 6 years after surgery (mean, 3 years). To evaluate the functional results, fecal continence scores (Templeton and Ditesheim) were calculated for incontinent patients. Of the 16 incontinent patients, 12 achieved continence and 4 some improvement. Of the 4 patients with fecal impaction, 2 achieved daily voluntary bowel movement, whereas the other 2 have mild constipation and need occasional enemas. CONCLUSIONS: Our study suggests that (1) a secondary operation through a posterior sagittal approach can restore fecal continence and is efficacious even in adolescents and adults and (2) a posterior sagittal procedure can be safely performed without a diverting colostomy.     

尿道下裂术后尿道狭窄继发附睾炎的诊治

【摘要】〓目的〓探讨尿道下裂术后尿道狭窄继发附睾炎的临床特点与疗效。方法〓2005年1月至2011年2月收治的尿道下裂术后尿道狭窄46例患者中8例继发附睾炎,对该类患者予抗感染及对症治疗后,尽早行尿道探查、尿道狭窄段切开或切除及组织瓣移植尿道成形术等方法修复尿道狭窄。结果〓8例尿道狭窄继发急性附睾炎患者术后8天内附睾炎症状基本消失。1例患者阴茎伤口感染致阴茎中部尿瘘于3月后成功行尿瘘修补术,余7例患者伤口愈合良好,无尿瘘发生,排尿通畅,尿线粗。随访3~12月,8例患者均无尿道狭窄复发,无急性附睾炎复发,慢性附睾炎者临床症状较术前明显改善。结论〓本组患者中,尿道下裂术后尿道狭窄继发附睾炎见于严重的尿道狭窄患者及年龄偏大患者;尿道下裂术后尿道狭窄应积极处理,一旦出现急性附睾炎时应在抗感染等保守治疗前提下,尽早行尿道成形修复狭窄段尿道,恢复正常尿流。     

Conservative therapy of rectourethral fistula: five-year follow-up

A case of rectourethral fistula is described, occurring after radical prostatectomy and adjuvant radiotherapy for pathologic Stage C carcinoma of the prostate. Urethral instrumentation for stricture disease immediately preceded development of the stricture. Conservative management for five years has resulted in the development of osteomyelitis of the pubis, but with antibiotic suppression the patient remains active and asymptomatic. Predisposing factors for the development of rectourethral fistula following radical prostatectomy may include adjuvant radiotherapy and recurrent urethral instrumentation. Most patients will require definitive repair but occasionally a patient may be managed conservatively.     

Transanal repair of rectourethral fistula after radical retropubic prostatectomy: a case report

A 65-year-old man underwent a radical retropubic prostatectomy for prostate cancer, and 5 days later fecaluria and serous diarrhea appeared suddenly. Cystourethrography domonstrated the flow of contrast material into the rectum through the fistula, so we diagnosed a rectourethral fistula. We first attempted conservative management, but the fistula did not close spontaneously. So we performed the transanal repair of rectourethral fistula about 2 months after surgery. This repair was effective, and the patient was alive without fistula recurrence at about 2 years after the repair surgery. This approach is simple and does not require a new incision, but it is only useful for low rectourethral     

The transpubic approach for the correction of complex anorectal and urogenital malformations

Background

The transpubic approach has been used mainly to treat urethral injuries and prostate cancer. There are no reports describing this approach in anorectal malformations.

Methods

Forty-two patients who underwent a transpubic approach for their genitourinary/colorectal reconstruction were reviewed. Indications, complications, and follow-up were analyzed. A midline infraumbilical incision was used. The pubic cartilage was divided with needle cautery. Institutional review board approval was obtained (IRB# 2008-1317).

Results

The cases included complex malformations (16), covered exstrophy (15), long urogenital sinus with normal rectum (6), and reoperations in cloacas which had been left with persistent fistulae between vagina and urinary tract with normal rectum (5). Excellent exposure was achieved in all cases, allowing successful anatomical reconstruction. Functional results varied depending on the specific type of defect but were not expected to be good due to severe congenital or acquired anatomic defects. There were 3 complications related to the transpubic approach: pubic dehiscence, suspected osteomyelitis, and bleeding.

Conclusion

The transpubic approach should be considered for the repair of complex anorectal and urogenital malformations, especially when adequate exposure cannot be achieved with an abdominal, perineal, or posterior sagittal approach. Another ideal indication is in patients with a normal rectum who were born with a complex urogenital sinus or underwent a failed attempted repair but were left with problems requiring reoperation in a scarred and fibrotic pelvis.     

'High' anorectal malformation in boys: need for clarity of definition and management

Posterior sagittal anorectoplasty is purported to be the approach of choice for the treatment of both intermediate and high variety of anorectal malformations. The authors describe their devastating experiences with 2 cases of high variety of anorectal malformations in which defects were repaired thorough the posterior sagittal approach. In the first case, because of the high level of the rectal pouch, complete urethrovesical disconnection was done along with inadvertent mobilization and pull-through of urinary bladder. The patient subsequently required major reconstructive procedures. In another case, the bladder was partially mobilized before the mistake was realized; the high-lying rectum was later identified, and a pull-through was performed without any ill consequences. The authors infer that only the patients with rectal pouches ending caudal to the third sacral vertebra should have their definitive surgery performed through posterior sagittal approach. They suggest that PS3 line should be the watershed in management of anorectal malformation in boys.