Pediatr Surg Int DOI 10.1007/s00383-015-3749-1
ORIGINAL ARTICLE
The management of anorectal malformation with congenital vestibular fistula: a single-stage modified anterior sagittal anorectoplasty Chen Wang1,2 • Long Li1,2 • Shuli Liu1 • Zheng Chen1 • Mei Diao1 Xu Li1 • Guoliang Qiao1 • Wei Cheng3,4
•
Accepted: 25 June 2015 Ó Springer-Verlag Berlin Heidelberg 2015
Abstract Purpose This study aimed to evaluate the mid-term outcomes of single-stage modified anterior sagittal anorectoplasty (ASARP) for anorectal malformation with vestibular fistula. Method Twenty-six patients with congenital imperforate anus and vestibular fistula underwent single-stage modified sphincter-saving ASARP between January 2008 and December 2012. The ages of the patients at the time of operation ranged from 1 month to 5.1 years. Standard ASARP procedure was modified to avoid the incision of the external sphincter complex. Instead a potential tunnel was created through the center of external sphincter complex under the endoscopic guidance. The patients were evaluated for fecal continence and complications. Result Modified ASARP was successfully performed in all patients. The mean operation time was 52.2 ± 3.5 min (range 47–61 min). The operative blood loss was minimal.
There was no operative complication. Wound infection occurred in 3 patients (3/26, 11.5 %). All patients were followed up for 4.2 ± 1.5 years (range 2–6 years). No patient developed fecal incontinence. Three patients (3/26, 11.5 %) had soiling once or twice per week. Four patients (4/26, 15.4 %) had constipation amenable to diet management. Mucosal prolapse occurred in 1 patient (1/26, 3.8 %). There was no recurrence of fistula, anal stenosis or anterior displacement of the neorectum. Conclusion Mid-term results show that single-stage modified ASARP is an effective and safe option for patients with anorectal malformation and congenital vestibular fistula. Keywords Anorectal malformation Vestibular fistula Anterior sagittal anorectoplasty Colostomy
Introduction
& Long Li
[email protected] & Wei Cheng
[email protected] 1
Department of Pediatric Surgery, Capital Institute of Pediatrics, No. 2 Ya Bao Road, Beijing 100020, People’s Republic of China
2
Graduate School of Peking Union Medical College, Beijing, People’s Republic of China
3
Department of Surgery, Beijing United Family Hospital, Beijing, People’s Republic of China
4
Department of Pediatrics and Department of Surgery, Faculty of Medicine, Nursing and Health Sciences, Monash University, Victoria, Australia
Imperforate anus with vestibular fistula (VF) is the most common form of anorectal malformations (ARMs) encountered in girls. Based on the principle of posterior sagittal anorectoplasty (PSARP), the anterior sagittal anorectoplasty (ASARP) was introduced by Okada in 1992 [1, 2]. This approach provides exact visualization of the fistula between the rectum and the introitus, and precise placement of the rectum within the external sphincter muscle. However, postoperative constipation and soiling are not uncommon. To further preserve sphincter function, single-stage modified sphincter-saving ASARP has been advocated since 2008 in our center. The aim of this study is to describe the technique and evaluate mid-term outcomes of this approach for ARM with congenital vestibular fistula.
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Patients and methods
Surgical technique
Patients
The patients were put on low-residue diet for 3 days before surgery. Oral neomycin was administered. Rectal washout was carried out with normal saline the day before surgery. Prophylactic antibiotic was administered half an hour before the surgery in the operating room. The patient was placed in a lithotomy position. The bladder was emptied using a urinary catheter. The incision was similar to the standard ASARP procedure. A circular incision was made in the mucocutaneous junction at the opening of the fistula (Fig. 1) and extended posteriorly along the median line to reach the anal dimple sited by neuromuscular stimulator. Through the incision, the fistula was dissected out surrounding tissues (Fig. 2). Between the rectum and vagina, there was normally firm connective tissue, a sharp dissection was needed to enter the plane between posterior vaginal wall and rectum. Special care was taken not to damage the sphincter musculature. To prevent rectal retraction, the dissection of rectal tube was extended to the level of the pelvic peritoneal reflection. The neuromuscular stimulator was used to identify
In this study, twenty-six girls with ARM and congenital vestibular fistula underwent single-stage modified ASARP between November 2008 and December 2012. Thirteen patients with acquired vestibular fistula were excluded from the study. The ages of the patients at the time of operation ranged from 1 month to 5.1 years (Table 1). All patients underwent chest X-ray, spine X-ray, abdominal ultrasonography, echocardiography, and fistulogram prior to surgery. The spine MR was performed in suspected cases of vertebral defect. Four patients had associated anomalies (4/26, 15.3 %), including an urachal cyst (1/26, 3.8 %), a renal duplication (1/26, 3.8 %), a patent foramen ovale (1/26, 3.8 %), and a spinal abnormality (1/26, 3.8 %). Ethics approval from the Ethics Committee of Capital Institute of Pediatrics was obtained. Written informed consents were obtained from the parents of the ARM patients prior to the surgery.
Table 1 Patient characteristics Patients
Age at operation (year)
Operative time (min)
Postoperative stay (day)
Associated anomalies
Follow-up (year)
1
4
61
5
None
6
2
1
55
5
Left renal duplication
6
3
2.2
57
5
None
6
4
1
54
5
None
6
5
0.3
50
4
None
6
6
0.4
53
4
None
6
7
0.6
51
5
None
6
8
0.7
57
4
Patent foramen ovale
5
9
0.9
49
4
None
5
10
0.7
52
4
None
5
11
0.5
48
4
None
5
12
0.6
52
4
None
5
13
0.4
49
4
None
4
14
0.6
50
4
None
4
15 16
1.2 2
53 57
4 6
None None
4 4
17
0.1
51
4
None
4
18
2.5
54
4
None
4
19
5.1
57
4
None
3
20
1.2
51
4
Urachal cyst
3
21
1.2
53
5
None
3
22
0.2
47
4
None
2
23
0.6
49
4
None
2
24
0.4
49
4
None
2
25
0.5
47
4
Spina bifida (S1–S2)
2
26
0.5
51
4
None
2
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Result Modified ASARP was successfully performed in all cases. Mean operation time was 52.2 ± 3.5 min (range 47–61 min). Intraoperative blood loss was minimal. No blood transfusion was required. There was no intraoperative complication. Minor wound infection occurred in 3 patients (3/26, 11.5 %) in postoperative period. All patients were followed up. The median follow-up period was 4.2 ± 1.5 years (range 2–6 years). Mucosal prolapse occurred in 1 girl (1/26, 3.8 %), who did not require surgical revision. No recurrence of fistula, acquired anal stenosis or anterior displacement of the neorectum was encountered. According to the Krickenbeck classification, bowel function of the patients older than 3 years was assessed [3]. We considered it a good outcome when the patients were free from soiling or suffer from grade 1 soiling only, and a poor outcome for those presenting with grade 2 or 3 soiling. None of the patients was classified as a poor outcome. Three patients (3/26, 11.5 %) had soiling once or twice per week. Four patients (4/26, 15.4 %) had constipation which was managed by changes of diet. No anal stenosis was observed (Table 2).
Discussion Fig. 1 A probe was used to identify the opening of the fistula
the center of the external sphincter. Under the guidance of endoscopic camera (8F urethroscope, 27030KA; Karl Storz Inc, Tuttlingen, Germany), a tunnel through the center of the sphincter complex was created with artery forceps (Figs. 3, 4). The tunnel was dilated with Hegar probes up to 12 mm. The mobilized rectum was pulled down through the tunnel into the perineal skin without tension. Anoplasty was performed with absorbable interrupted 5-0 PDS (PDS*II,Z148, Ethicon Inc., USA) sutures. The closure of the surgical wound was completed by closing the perineal skin and creation of a perineal body. Oral intake was resumed on postoperative day 1–5. The urinary catheter was removed on postoperative day 2. Parenteral broad-spectrum antibiotics were given for 3–7 days depending on the condition of perineal wound. Local cleaning and care was required. On the postoperative day 14, anal calibration and dilatation was initiated. The anus was dilated twice daily and the size of the dilator was increased weekly until the desired size was reached. All patients were regularly followed up by a resident doctor, fortnightly for the first 3 months; monthly for the next 6 months; every 4 months for the next year; and then yearly.
Imperforate anus with vestibular fistula (VF) is the most common type of ARMs in females. According to Krickenbeck classification, ARM with congenital vestibular fistula is regarded as low malformation [3]. This classification does not distinguish between rectovestibular and anovestibular fistula, since the differentiation does not seem to be important when performing a PSARP or ASARP procedure [4]. This anomaly is often defined as a form of ARM with good prognosis in terms of long-term fecal continence [5, 6]. However, functional complications after surgery, especially constipation and soiling, are not uncommon [7]. Because of this, modification of the surgical treatment is warranted. There have been many procedures described for the management of ARM with vestibular fistula, including cutback operation, V–Y plasty, anal transposition, PSARP, ASARP and their modifications [8, 9]. The PSARP provides excellent exposure and precise placement of the neoanal canal within the muscle complex. However, the external sphincter, muscle complex, and part of the lower portion of the levator mechanism need to be divided [10]. In the procedure of ASARP, only the anterior fibers of external sphincter need to be divided. To maximally preserve the anal sphincter complex, we have modified the standard ASARP procedure. The incision of anterior
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Fig. 2 a–d The rectal fistula was dissected sharply and bluntly little by little from surrounding tissues
Fig. 3 A tunnel through the center of the sphincter complex was created under the guidance of endoscopic camera
external sphincter is avoided in our procedure. A potential tunnel through the center of the sphincter complex is created under the guidance of the endoscopic camera. This study is the first series to evaluate the safety and the efficacy of this technique for children with ARM and congenital vestibular fistula. Compared with traditional anal transposition described by Potts [11], the improvements of our technique include: (1) precise siting of the neorectum within the sphincter
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Fig. 4 The tunnel through the center of the sphincter complex was created
complex. The endoscopic camera was pointed directly at the external sphincter. With amplification of the images, the external sphincter could be observed carefully in the monitor, the potential tunnel was precisely created through the center of the sphincter complex without the damage of the external sphincter. In our group, no patient presented
Pediatr Surg Int Table 2 Postoperative classification
outcomes
according
to
Krickenbeck
n
Rate (%)
Voluntary bowel movements
23
88.5
Soiling (no)
23
88.5
Soiling grade 1
3
11.5
Soiling grade 2
0
0
Soiling grade 3
0
0
22
84.6
Constipation grade 1 Constipation grade 2
4 0
15.4 0
Constipation grade 3
0
0
Constipation (no)
Fig. 5 The fistulography of the megarectum in patient with congenital vestibular fistula
with grade 2 or 3 soiling according to the Krickenbeck classification. (2) Adequate separation of rectum from vagina was performed up to the level of the pelvic peritoneal reflection. No recurrence of fistula, anal stenosis, and anterior migration of the rectum was observed in our series. The separation of the rectal fistula from the vagina is a difficult step. There are some tricks to avoid injury to the vagina during the dissection. (1) Compared with PSARP, the separation of rectal tube from the vagina could be performed under direct vision in ASARP [12]; (2) according to our experiences, there was firm connective tissue between the rectum and the vagina, a sharp dissection was needed to enter the plane between posterior vaginal wall and rectum. The most common complication after PSARP was constipation [13]. Pena proposed the underlying cause of constipation to be a hypomotility disorder of the rectosigmoid (Fig. 5). The sigmoid distension led to mega-sigmoid and overflow incontinence [10]. Lombardi recommended the resection of a significant portion of distal aberrant anorectal canals (fistulas) to achieve better functional results [14]. We followed this practice in our group, only 4 patient (4/26, 15.4 %) had constipation. In our procedure, the rectum was not tapered because this approach resulted in a wider upper portion and a narrower lower portion of intestine [13]. Another possible explanation of low frequency of constipation was that sparing the external sphincter muscle left no subsequent post-operative scarring of the sphincter in modified ASARP procedure. Ideally, a protective colostomy is performed prior to the correction of ARM to prevent wound infection and thus prevent consequent loss of the anal sphincter complex, with the risk fecal continence [15]. The decision to carry out single-stage procedure was somewhat influenced by the practical demand from the parents of the patients, who often come from other provinces, with limited financial resources and affordable time. The colostomy have its own
complications, including peristomal excoriations, prolapse, parastomal hernias, leakages, intraabdominal adhesions or bowel obstruction [16]. In the recent literature, more surgeons tend to do a single-stage procedure for ARM with vestibular fistula. In a prospective study of 72 ARM patients with VF who underwent PSARP without colostomy, all patients had good or fair fecal continence; in only 5 (6.9 %), mild infection was observed [17]. Kumar carried out a retrospective trial of 123 low type ARM patients who underwent single-stage PSARP or ASARP. Only 1 patient (1.85 %) who had associated sacral agenesis had fecal incontinence [18]. In a prospective study, 23 patients with RVF underwent primary ASARP, and 3 patients (13.04 %) developed local wound disruption in the postoperative period [19]. These results indicated that single-stage procedure was feasible and safe for the management of ARM with VF. Wound infection is the common early complication in one-stage ARM correction procedure. The use of perioperative therapeutic antibiotics seems to be necessary for reducing the frequency of wound infection. In our department, all patients were given therapeutic antibiotics (3–7 days), minor wound infection occurred in 3 patients (3/26, 11.5 %), and the incidence was comparable to other reported series [20, 21]. In a prospective study of 35 low ARM patients who underwent primary ASARP or PSARP, 11 % (2/18) patients given therapeutic antibiotics (2–5 days) had wound infection, the result showed a slight but significant lower wound infection rate than that of patients (6/14, 43 %) given no antibiotics or prophylactic antibiotics (24 h) [22]. In our series, all 3 patients suffering from wound infection was fed earlier than postoperative day 3, but no significant difference was noted between early oral intake group (prior to postoperative day 3) and late oral intake group (later to postoperative day 3) in the frequency of wound infection (Fisher’s exact test, p [ 0.05) (Table 3).
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Pediatr Surg Int Table 3 The association between wound infection and the variables
Wound infection (no)
Wound infection (yes)
p value
16 (61.5 %)
3 (11.5 %)
0.54
The time of oral intake Earlier than postoperative day 3 Later than postoperative day 3
7 (26.9 %)
0 (0 %)
The age of the patients [1 year
14 (53.8 %)
2 (7.7 %)
\1 year
9 (34.6 %)
1 (3.8 %)
[52 min
12 (46.1 %)
2 (7.7 %)
\52 min
11 (42.3 %)
1 (3.8 %)
1.00
Operation time
The drawback of our study is that the follow-up period is relatively short. Future manometry study is required to ascertain the benefit of sphincter sparing modification. A larger series would also provide more convincing evidence. Our result indicates that the correction of ARM with congenital vestibular fistula can be performed in patients through single-stage modified ASARP. The approach provides a viable and safe option as a surgical management of ARM with vestibular fistula. Long term follow-up and randomized controlled trials are necessary to confirm our initial findings. Acknowledgments The work was supported by Department of Pediatric Surgery, Capital Institute of Pediatrics, Beijing, People’s Republic of China. The author would like to thank Dr. Long Li and Dr. Wei Cheng for their support and mentorship. Compliance with ethical standards Conflict of interest peting interests.
The authors declare that they have no com-
References 1. Pena A, Devries PA (1982) Posterior sagittal anorectoplasty: important technical considerations and new applications. J Pediatr Surg 17(6):796–811 2. Okada A, Kamata S, Imura K et al (1992) Anterior sagittal anorectoplasty for rectovestibular and anovestibular fistula. J Pediatr Surg 27(1):85–88 3. Holschneider A, Hutson J, Pena A et al (2005) Preliminary report on the International Conference for the Development of Standards for the Treatment of Anorectal Malformations. J Pediatr Surg 40(10):1521–1526 4. Pena A (1995) Anorectal malformations. Semin Pediatr Surg 4(1):35–47 5. Pena A, Hong A (2000) Advances in the management of anorectal malformations. Am J Surg 180(5):370–376 6. Heinen FL (1997) The surgical treatment of low anal defects and vestibular fistulas. Semin Pediatr Surg 6(4):204–216 7. Rintala RJ, Lindahl HG, Rasanen M (1997) Do children with repaired low anorectal malformations have normal bowel function? J Pediatr Surg 32(6):823–826
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8. Dave S, Shi EC (2005) Perineal skin bridge and levator muscle preservation in neutral sagittal anorectoplasty (NSARP) for vestibular fistula. Pediatr Surg Int 21(9):711–714 9. Upadhyaya VD, Gangopadhyay AN, Pandey A, Kumar V et al (2008) Single-stage repair for rectovestibular fistula without opening the fourchette. J Pediatr Surg 43(4):775–779 10. Levitt MA, Pena A (2007) Anorectal malformations. Orphanet J Rare Dis 2:33 11. Potts WJ, Riker WL, Deboer A (1954) Imperforate anus with recto-vesical, -urethral-vaginal and -perineal fistula. Ann Surg 140(3):381–395 12. Kulshrestha S, Kulshrestha M, Singh B et al (2007) Anterior sagittal anorectoplasty for anovestibular fistula. Pediatr Surg Int 23(12):1191–1197 13. Huang CF, Lee HC, Yeung CY et al (2012) Constipation is a major complication after posterior sagittal anorectoplasty for anorectal malformations in children. Pediatr Neonatol 53(4):252–256 14. Lombardi L, Bruder E, Caravaggi F et al (2013) Abnormalities in ‘‘low’’ anorectal malformations (ARMs) and functional results resecting the distal 3 cm. J Pediatr Surg 48(6):1294–1300 15. Pena A, Migotto-Krieger M, Levitt MA (2006) Colostomy in anorectal malformations: a procedure with serious but preventable complications. J Pediatr Surg 41(4):748–756 (discussion 748–756) 16. Patwardhan N, Kiely EM, Drake DP et al (2001) Colostomy for anorectal anomalies: high incidence of complications. J Pediatr Surg 36(5):795–798 17. Menon P, Rao KL (2007) Primary anorectoplasty in females with common anorectal malformations without colostomy. J Pediatr Surg 42(6):1103–1106 18. Kumar B, Kandpal DK, Sharma SB et al (2008) Single-stage repair of vestibular and perineal fistulae without colostomy. J Pediatr Surg 43(10):1848–1852 19. Aziz MA, Banu T, Prasad R et al (2006) Primary anterior sagittal anorectoplasty for rectovestibular fistula. Asian J Surg 29(1):22–24 20. Adeniran JO (2002) One-stage correction of imperforate anus and rectovestibular fistula in girls: Preliminary results. J Pediatr Surg 37(6):E16 21. Upadhyaya VD, Gopal SC, Gupta DK et al (2007) Single stage repair of anovestibular fistula in neonate. Pediatr Surg Int 23(8):737–740 22. Kuijper CF, Aronson DC (2010) Anterior or posterior sagittal anorectoplasty without colostomy for low-type anorectal malformation: how to get a better outcome? J Pediatr Surg 45(7):1505–1508