|Year : 2020 | Volume
| Issue : 2 | Page : 401-408
Two-stage repair for delayed presentation of anorectal malformation with vestibular and perineal fistulae in females
Mohamed Ahmed Negm1, Mohamed Mahmoud Khedre2
1 Pediatric Surgery Unit, Qena Faculty of Medicine, South Valley University, Qena, Egypt
2 Pediatric Surgery Unit, Faculty of Medicine, Minia University, Minia, Egypt
|Date of Submission||19-Dec-2019|
|Date of Acceptance||17-Jan-2020|
|Date of Web Publication||27-Apr-2020|
MD Mohamed Ahmed Negm
Pediatric Surgery Unit, Qena Faculty of Medicine, South Valley University, Qena, 83523
Source of Support: None, Conflict of Interest: None
Background/aim In spite of advances in knowledge and techniques in the management of anorectal malformation (ARM), delayed presentation of female ARM with vestibular and perineal fistulae is quite common especially in developing countries. In this literature, there is no standardized surgical algorithm for late presentation of these female ARM. This study aimed to present the experience of two pediatric surgery tertiary centers regarding delayed presentation of vestibular and perineal fistulae using two-stage procedures.
Patients and methods This prospective study was conducted on 28 female patients with late presentation of vestibular and perineal fistulae from November 2014 to April 2019. Lower contrast study was done for all patients. Laxative, frequent rectal wash enemas, and Hegar’s dilators were used for 2–3 weeks preoperatively. All patients were repaired by anterior sagittal anorectoplasty (ASARP) with covering high sigmoid loop colostomy, and colostomy closure was performed after 2–3 months. Krickenbeck continence score was used for assessment of anal continence.
Results Of 28 patients included, 25 patients completed the study. There were 14 patients with perineal and 11 with vestibular fistulae. The age of patients ranged from 8 months to 18 years. The main reasons for delay were socioeconomic, unawareness, and overlapping causes. Sphincter-saving ASARP was used in 15 patients and the classic ASARP was used in 10 patients. Tapering proctoplasty was needed in one patient. Minor wound infection developed in three patients. Constipation occurred in 11 (44%) patients. Follow-up period ranged from 6 to 50 months (median 30 months). Good continence was obtained.
Conclusion Although delayed presentation of vestibular and perineal fistulae in female patients is accompanied by comorbidities, with good surgical management, successful results can be obtained by using two-stage procedure.
Keywords: anal continence, anorectal malformation, anterior sagittal anorectoplasty, constipation, delayed presentation, vestibular fistula
|How to cite this article:|
Negm MA, Khedre MM. Two-stage repair for delayed presentation of anorectal malformation with vestibular and perineal fistulae in females. Egypt J Surg 2020;39:401-8
|How to cite this URL:|
Negm MA, Khedre MM. Two-stage repair for delayed presentation of anorectal malformation with vestibular and perineal fistulae in females. Egypt J Surg [serial online] 2020 [cited 2020 Sep 28];39:401-8. Available from: http://www.ejs.eg.net/text.asp?2020/39/2/401/283196
| Introduction|| |
Anorectal malformations (ARM) are congenital correctable anomalies with good outcome in most cases . In female patients, imperforate anus with rectovestibular fistula, rectoperineal fistulae, and cloacal anomalies are the most common ARM ,.
Despite advances in knowledge and techniques in surgical correction of ARM, in developing countries, delayed presentation of vestibular and perineal fistulae in female patients is still quite common .
In this literature, there is controversy in the lines of surgical treatment of late presentation of ARM with vestibular or perineal fistula in female patients. The purpose of the present study was to present the outcome of two tertiary pediatric surgery centers in the surgical management of delayed presentation of vestibular and perineal fistulae in female patients older than 6 months using two-stage procedure.
| Patients and methods|| |
This prospective study included female patients with late presentation of vestibular and perineal fistulae. The study was conducted in the period from November 2014 to April 2019 in two referral pediatric surgery tertiary centers in Upper Egypt. The study was carried out after the Local Ethical Committee reviewed and approved the study protocol in both institutions. The parents of all patients signed a detailed written informed consent form before enrollment in the study. The following data were collected: the age at presentation, cause of delay, associated anomalies, previous attempts of repair, rectal dilatation on contrast study, procedure selected for surgical correction, operative and postoperative complications, continence assessment, and follow-up period data for at least 6 months.
Design of the study
Inclusion criteria were female patients with ARM with vestibular and perineal fistulae who first presented after the age of 6 months ([Figure 1]).
|Figure 1 Different patients with delayed presentation (a) an 18-year-old patient with vestibular fistula; (b, c) contrast enema show the fistula and rectal dilatation; (d) perineal fistula in a 8-year-old patient with history of previous unsuccessful surgery; (e, f) contrast enema of the same patient showing rectal dilatation; (g) a 15-month-old patient with vestibular fistula; (h) perineal fistula in a 12-month-old patient.|
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Exclusion criteria were patients who presented with colostomy.
The included patients were subjected to the following
- Thorough history taking and examination to detect the cause of delay and associated anomalies.
- Contrast enema to measure fistula length and the degree of rectal dilatation ([Figure 1]).
- Preoperative preparation with frequent rectal wash enemas and Hegar dilatation for all patients 2–3 weeks before surgery to decrease rectal dilatation.
- All patients were repaired by anterior sagittal anorectoplasty (ASARP) with covering high sigmoid loop colostomy, and colostomy closure was done after 2–3 months later.
- During the follow-up period, cooperative children were advised to perform pelvic floor exercise.
- Anal continence assessment was done using Krickenbeck continence score  1 year after surgery and/or patient’s age older than 3 years ([Table 1]).
All patients were repaired either by classic ASARP as described by Okada et al.  in patients with rectal dilatation or sphincter-saving ASARP in patients without rectal dilatation (in which the mobilized rectum pass through the center of intact sphincter complex).
All patients were operated under general anesthesia with caudal analgesia.
Regarding position of the patients, lithotomy position (or supine position with hanging and supporting the legs) was used, with insertion of urethral catheter.
Using muscle stimulator (Peña stimulator) or low-current diathermy (needle diathermy cutting mode), the position of the neoanus was marked at the center of the sphincter muscle complex, traction sutures were applied around the fistulous opening with circumferential incision, and strict midline perineal incision was done for careful dissection of perineal muscle. Meticulous dissection was needed for separation of the rectum from posterior vaginal wall, especially in these late cases owing to presence of fibrosis and adhesions.
Then mobilization of the rectum to reach the perineum without tension was done. This was followed by passing the rectum in the center of muscle complex and finally perineal body reconstruction and anoplasty. In vestibular fistula, more meticulous dissection was needed for separation of the rectum from posterior vaginal wall.
In sphincter-saving technique, the center of the intact sphincter complex was identified using Peña stimulator and then with gradual dilatation by Hegar’s dilator without its incision or laceration. The mobilized rectum then was passed through the center of sphincter complex, and finally, anoplasty and perineal body reconstruction was done , ([Figure 2]).
|Figure 2 Classic and sphincter-saving ASARP technique. (a) Patient aged 11 months with perineal fistula; (b) rectal mobilization and separation from posterior vaginal wall; (c) ASARP before anoplasty; (d) sphincter-saving ASARP in another patient with the rectum passed through the intact sphincter; (e) the colostomy before closure with no related colostomy complications except for mild pigmentation; (f) the perineum after complete healing. ASARP, anterior sagittal anorectoplasty.|
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Loop colostomy at the junction between the descending and sigmoid colon was done at the end of surgery.
Oral feeding was started on the second postoperative day, and urethral catheter was removed after 5 days. Discharge from the hospital was done on the sixth day after training the parent on how to deal with both stoma and perineal wound.
Anal dilatation was started after complete wound healing. Colostomy closure was done after 6–8 weeks after complete healing of the perineal wound.
| Results|| |
Of 28 patients included in the study, one patient (aged 20 years with vestibular fistula) refused surgery and two girls with vestibular fistula presented with colostomy (done elsewhere), so they were excluded from this study. A total of 25 patients fulfilled the inclusion criteria. There were 14 patients with perineal and 11 patients with vestibular fistulae.
By reviewing the medical record in both centers, it was found that 28 patients included in this study presented with late presentation among 200 female patients with ARM who presented to both centers during the study period. In the first year of this study, of 44 cases of female ARM, 11 (25%) cases of them were late presentations. The presentation of these late cases gradually decreased and now has become one to two cases per year.
As shown in [Table 2], the age of studied patients ranged from 8 months to 18 years (median 3 years) at presentation. Five patients presented initially by fecal impaction with accidental discovery of perineal fistula. Causes of delay are summarized in [Table 2], which shows the associated anomalies and causes of delayed presentations. Laxatives, rectal wash enemas, and Hegar’s dilators were done 2–3 weeks before surgery, which was successful for improvement of rectal dilatation in all except one patient. Sphincter-saving ASARP was used in 15 patients without rectal dilatation, and the classic ASARP was used in 10 patients with rectal dilatation, as shown in [Figure 3]. Tapering proctoplasty was needed in one patient owing to dilated rectum, and this patient developed superficial wound infection. There was no reported rectal injury, and vaginal injury occurred in two patients with immediate intraoperative repair. Operative time ranged from 2.5 to 4 hr, with a median of 3.25 h. Hospital stay ranged from 4 to 6 days (median 5 days). Superficial wound infection occurred in three patients with vestibular fistula (one of them tapering proctoplasty was done), with improvement with local wound care. Anal stenosis developed in three patients (two vestibular and one perineal): two patients improved with regular anal dilatation, and only one patient (with perineal fistula) needed anoplasty. One patient needed mucosal excision before colostomy closure. Colostomy closure was done 2–3 months after the first stage. The only complication related to the colostomy in this study was skin excoriation especially in older patients ([Figure 3]). Only one patient needed blood transfusion in whom tapering proctoplasty was needed. There was no mortality reported in this study. Continence was assessed by Krickenbeck continence score  done in 18 (72%) patients. The result of postoperative continence is shown in [Table 1]. Soiling occurred in 10 (55.5%); fortunately, those patients showed improvement with time during the follow-up period. Constipation occurred in 11 (44%) patients and was controlled by diet, but three patients needed laxatives. All patients maintained regular follow-up by outpatient visits or phone calling. The follow-up ranged from 6 to 50 months (median 36 months). The overall outcomes of the studied patients were satisfactory.
|Figure 3 Preoperative, early and long-term follow-up in patients aged 6 years. (a) Perineal fistula with cicatrization due recurrent inflamation; (b,c) contrast study through the fistula to asess rectal dilatation; and (d) 3 months after anterior sagittal anorectoplasty with mucosal prolapse on right side; (e) the same patient before colostomy closure with skin excoriation arround the stoma; (f,g) two-year follow-up of the same patient after closure of colostomy.|
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| Discussion|| |
In female patients, imperforate anus with rectovestibular fistula, rectoperineal fistula, and cloacal anomalies are the most common ARM ,. Although ARM can be easily detected in the neonatal period by proper clinical examination after resuscitation, there is still delayed diagnosis. This not only occurred in low-resource countries but also in developed one but with low percentage ,. This delay is usually associated with more morbidity than early presentation ,.
In this literature, delayed presentation is defined as ‘patient who presented after 7 days of birth, except for a female patient, with low-type ARM, where presentation beyond 6 months of age was considered as delayed’ . However, other authors considered presentation more than 48 h after birth as delayed diagnosis . The higher incidence of delayed presentation of ARM in female than males may be owing to colostomy, which was done as an emergency procedure in neonatal period for males with high and intermediate ARM, with the need for definitive treatment thereafter. Poverty, inadequate knowledge or experience about female ARM, and lack of advanced health care are other causes .
In this study, the incidence of delayed presentation was 25% in the start of this study, but now, it has decreased owing to increased awareness in the community. According the result of this study, the reasons of late presentation were unawareness and accidental discovery of abnormal anal opening. This is the main cause of delayed presentation in perineal fistula. The main concern of parents came for consultation was the abnormal anal opening and its effect on marriage, especially those presented at adolescence age. The main problem of interest was the affection of abnormal anal opening on the future marriage. Unfortunately, one patient refused surgery assuming that she is continent and surgical correction may cause problems with her current marriage, as she was already engaged.
In a study by Rawat et al. , illiteracy, wrong advice, and poverty were the main causes of late presentation. They added that their patients came from villages with low social and economic standard. In another study, Sinha et al.  stated that ‘the reasons for a delayed presentation were either delayed awareness on the part of parents or poor access to the appropriate facilities.’
Major congenital anomalies sometimes may be the cause of delay in the current study. A total of three (12%) patients had congenital heart disease that needed correction before definitive surgery for low ARM. Mittal et al.  reported that associated congenital anomalies were found only in one patient (10%).
In this literature, female ARM with vestibular and perineal fistula according to Krickenbeck classification are corrected either by one or three stages . Many authors prefer correction of early presentation of vestibular and perineal fistula in the neonatal period or beyond the neonatal period ,. Peña  proposed surgical correction at 2–3 months. However, there is no universal standard of surgical management of late presentation of perineal and vestibular fistulae.
Most of the reported studies used either one or three stages for surgical correction. According to this study and the previous experience of our centers, two-stage repair is more convenient for these patients with late presentation. Colostomy first performed in the three-stage repair, can be replaced by preoperative good rectal wash to decrease rectal dilatation. One-stage repair carries the increased risk of perineal wound complications with decreasing the chance of continence especially in late presenter. Moreover, repair with covering colostomy offers a safe option for decreasing perineal wound complication with good perineal and sphincter muscle function thereafter. This agreed with Khalifa et al.  in their study comparing one-stage vs two-stage procedure for rectovestibular fistula in that two-stage procedure was safer than one stage, although the age of their patients ranged from 3 months to 2 years.
Gupta et al.  compared the outcome of one-stage vs conventional three stages on female patients with perineal and vestibular fistulae, with variable ages up to 14 years. They concluded that primary one-stage definitive surgery had significant higher complications.
Although colostomy has many complications , according to this study, a covering colostomy is a safer way to avoid complications of perineal wound after correction of ARM particularly in those presented late for definitive surgery. In a study by Sham et al. , they did correction of 13 female patients and their age ranged from 13 to 32 years; primary ASARP repair was done in only three cases and staged repair was done in other patients.
In this study, tapering proctoplasty was done only in one patient. Postoperatively, this patient had mild constipation and soiling. The constipation was controlled by diet modification, whereas the soiling was improved after one year and became apparent only during the attacks of diarrhea. In a study by Rawat et al. , tapering proctoplasty was needed in three cases using PASARP owing to severe rectal dilatation. Soiling and constipation occurred in these cases. The authors provided explanation that this may be due to the development of dilatation and redundancy in the rectosigmoid region with time.
In this study, three patients developed superficial wound infection that improved by local wound care; two patients were adolescents with vestibular fistula with history of previous surgery, and the third patient underwent tapered proctoplasty. In a study by Sharma and Gupta , 33 female patients with age ranged from 5 months to 14 years underwent one-stage PASARP. Superficial wound infection developed in two patients and reoperation was not required. In the present study, constipation was the main problem among the studied patients, which occurred in 11 (44%) patients; however, it was controlled with diet modification in eight (32%) patients and only three (12%) patients needed laxative.
Continence was assessed by different scores used to assess the surgical results after correction of ARM . In this study, patients were assessed by Krickenbeck continence score . During follow-up period, children and adolescent patients continued wearing diapers for at least 5 months with gradual improvement. With follow-up, good results were obtained in most of patients.
Limitation of this study
This study considered short-term and intermediate-term outcomes; however, long-term follow-up of these cases is needed, and this will be done in future study. This study included vestibular with perineal fistulae as one group of patients, although they have different pathology and presentation; however, the plan of surgical correction is nearly the same to some extent. Moreover, this study targeted to discuss causes of late presentation and surgical outcome after two-stage repair.
| Conclusion|| |
Although delayed presentation of ARM with vestibular and perineal fistulae in female patients is accompanied by comorbidities, with good surgical management, successful results can be obtained. Two-stage repair offers a safe option for surgical correction, has good continence outcomes, and decreases the morbidity of multistage procedure.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Sinha SK, Kanojia RP, Wakhlu A, Rawat JD, Kureel SN, Tandon RK et al.
Delayed presentation of anorectal malformations. J Indian Assoc Pediatr Surg 2008; 13:64–68.
Rosen NG, Hong AR, Soffer SZ, Rodriguez G, Peña A. Rectovaginal fistula: a common diagnostic error with significant consequences in girls with anorectal malformations. J Pediatr Surg 2002; 37:961–965.
Breech L. Gynecological concern in patients with anorectal malformations. Semin Pediatr Surg 2010; 19:139.
Sharma S, Gupta DK. Delayed presentation of anorectal malformation for definitive surgery. Pediatr Surg Int 2012; 28:831–834.
Holschneider A, Hutson J, Peña A, Beket E, Chatterjee S, Coran A et al.
Preliminary report on the International Conference for the Development of Standards for the Treatment of Anorectal Malformations. J Pediatr Surg 2005; 40:1521–1526.
Okada A, Kamata S, Imura K, Fukuzawa M, Kubota A, Yagi M et al.
Anterior sagittal anorectoplasty for rectovestibular and anovestibular fistula. J Pediatr Surg 1992; 27:85–88.
Elsawaf MI, Hashish MS. Anterior sagittal anorectoplasty with external sphincter preservation for the treatment of recto-vestibular fistula: a new approach. J Indian Assoc Pediatr Surg 2018; 23:4–9.
] [Full text]
Negm MA, Arafa MA, Elshimy KM. Short-term outcome of one-stage sphincter-saving anterior sagittal anorectoplasty in vestibular and perineal fistulae in female infants. Egypt J Surg 2020; 39:199. [Full text]
Acosta Farina D, Ortiz-Interian CJ, Acosta Vasquez CE. Imperforate anus, delayed presentation in a 7-year-old girl. J Pediatr Surg 1993; 28:962–964.
Kumar V, Chattopdhay A, Vepakomma D, Shenoy D, Bhat P. Anovestibular fistula in adults: a rare presentation. Int Surg 2005; 90:27–29.
Haider N, Fisher R. Mortality and morbidity associated with late diagnosis of anorectal malformations in children. Surgeon 2007; 5:327–330.
Turowski C, Dingemann J, Gillick J. Delayed diagnosis of imperforate anus: an unacceptable morbidity. Pediatr Surg Int 2010; 26:1083–1086.
Sham M, Singh D, Phadke D. Anorectal malformations: definitive management during and beyond adolescence. J Indian Assoc Pediatr Surg 2012; 17:120.
] [Full text]
Rawat J, Singh S, Pant N. Anorectal malformations in adolescent females: a retrospective study. J Indian Assoc Pediatr Surg 2018; 23:57–60.
Mittal A, Airon RK, Magu S, Rattan KN, Ratan SK. Associated anomalies with anorectal malformation (ARM). Indian J Pediatr. 2004; 71:509–514.
Upadhyaya VD, Gopal SC, Gupta DK, Gangopadhyaya AN, Sharma SP, Kumar V. Single stage repair of anovestibular fistula in neonate. Pediatr Surg Int 2007; 23:737–740.
Short SS, Bucher BT, Barnhart DC, Van Der Watt N, Zobell S, Allen A, Rollins MD. Single-stage repair of rectoperineal and rectovestibular fistulae can be safely delayed beyond the neonatal period. J Pediatr Surg 2018; 53:2174–2177.
Pena A. Surgical treatment of female anorectal malformations. Birth Defects 1988; 24:403–423.
Khalifa M, Shreef K, Al Ekrashy MA, Gobran TA. One or two stages procedure for repair of rectovestibular fistula: which is safer? (A single institution experience). Afr J Paediatr Surg 2017; 14:27.
] [Full text]
Gupta A, Agarwala S, Sreenivas V, Srinivas M, Bhatnagar V. Primary definitive procedure versus conventional three-staged procedure for the management of low-type anorectal malformation in females: a randomized controlled trial. J Indian Assoc Pediatr Surg 2017; 22:87.
Peña A, Migotto-Krieger M, Levitt MA. Colostomy in anorectal malformations: a procedure with serious but preventable complications. J Pediatr Surg 2006; 41:748–756.
Brisighelli G, Macchini F, Consonni D, Di Cesare A, Morandi A, Leva E. Continence after posterior sagittal anorectoplasty for anorectal malformations: comparison of different scores. J Pediatr Surg. 2018; 53:1727–1733.
[Figure 1], [Figure 2], [Figure 3]
[Table 1], [Table 2]