Laparoscopic-Assisted Anorectal Pull-Through for High Imperforate Anus: 14 Years Experience in a Single Center

Abstract

Purpose:

To report our 14 years experience with the laparoscopic-assisted anorectal pull-through (LAARP) for the treatment of male neonates with high imperforate anus.

Materials and Methods:

We reviewed all medical charts of patients who underwent LAARP from January 2001 to January 2015 collecting information regarding demographic data, associated anomalies, type of fistula, pelvic floor muscles tropism, surgery (operative time, conversion to open technique, and complications), and follow-up. Follow-up data were obtained through the use of validated questionnaires that aim to assess the intestinal function in terms of constipation and continence.

Results:

At the Department of Pediatric Surgery of the University of Bologna, 13 male patients were operated in the study period (our protocol consisted of colostomy at birth followed by delayed LAARP). Mean age at operation was 4.75 months (range 1–14). There was one conversion to open technique due to a strong tension from the colostomy (this patient was excluded). Functional results were acceptable at a minimum 2-year-long follow-up.

Conclusions:

LAARP is a good choice for the correction of the high imperforate anus. Short-term outcomes are similar to those after posterior sagittal anorectoplasty (PSARP). Long-term outcomes should be better assessed.

Introduction

Anorectal malformations (ARMs) include a wide spectrum of diseases occurring in 1:5000 newborns¹ and they have a male preponderance.² The most frequent defect in male patients is the rectourethral fistula.²

Posterior sagittal anorectoplasty (PSARP) has been the procedure of choice for ARM correction since 1980s.³ In the last two decades, there has been a growing interest in the use of a laparoscopic-assisted anorectal pull-through (LAARP). This approach has been shown to be safe and efficient.^4–6 However, there is an ongoing discussion regarding which are the indications and it seems that we still have to better interpret long-term outcomes and functional advantages.⁷

In this article, we describe our 14 years experience with the laparoscopic repair of high ARMs in males focusing on long-term functional outcomes.

Materials and Methods

We reviewed all the medical charts of male patients with high ARMs treated by LAARP at the Department of Pediatric Surgery of the S. Orsola-Malpighi Hospital (Bologna, Italy) from January 2001 to January 2015. We included patients with prostatic, bladderneck, and recto-bulbar fistula. We collected information regarding demographic data, associated anomalies, type of fistula, pelvic floor muscles tropism, surgery (operative time, age and body weight at operation, conversion to open technique, and complications), and follow-up.

The protocol we followed for the management of these patients was based on the algorithm proposed by Levitt and Peña.⁸ Initial management included a careful evaluation of the patient to determine the anatomy and plan the definitive repair (perineal inspection as first step and radiological evaluation to exclude associated anomalies). In this initial assessment, we obtained the evaluation of perineal muscles (levator ani muscle group and external anal sphincter) with magnetic resonance imaging. We grouped our patients depending on the development of these aforementioned muscles (well developed or underdeveloped).

We then performed a three-stage repair starting from the creation of a preliminary loop-colostomy on the descending colon in the neonatal period followed by LAARP several months later and then the colostomy closure as third step. The LAARP was performed as previously described.⁹ Once the transperineal dissection was completed, the passage through the perineal plane in the muscle complex and fascial plate was created with a trocar under laparoscopic surveillance. The laparoscopic electrostimulator helped us with the identification of the proper site of penetration. LAARP was successfully performed in 12 patients who were considered in this series, while we had one conversion because the colostomy was too low and we couldn't pull through the rectum.

After LAARP, we kept in situ a urinary catheter for 5 days. Feeding was gradually reintroduced when bowel function resumed. Two weeks after the repair, the patient was started on a protocol of anal dilatations, and parents were instructed to dilate the neo-anus at home.

All patients were evaluated at our outpatient clinic at 1, 3, and 6 months after surgery and then every year or more frequently, as needed. Follow-up data were obtained through the use of two questionnaires: the validated Rintala questionnaire¹⁰ and the Kelly scoring system.¹¹ These questionnaires aim to assess the intestinal function in terms of constipation and continence. The Kelly system (Table 1) gives points for continence, staining of underclothes, and quality of sphincter squeeze for a total of 6 points (5–6 good, 3–4 fair, and 0–2 poor). The Rintala scoring system evaluates seven aspects offering a maximum score of 20 (Table 2). Informed consent was obtained for all patients participating in the study.

Table 1.

The Kelly Continence Score Assessment of Bowel Function

Continence	Normal, no soiling = 2
	Occasional accidents, feces/flatus escape = 1
	No control, frequent accidents = 0
Staining	Always clean = 2
	Occasional staining = 1
	Always stained = 0
Sphincter	Strong and effective squeeze = 2
	Weak and partial squeeze = 1
	No contraction = 0

Table 2.

The Rintala Continence Score Assessment of Bowel Function

Ability to hold back defecation	Always = 3
	Problems <1/week = 2
	Weekly problems = 1
	No voluntary control = 0
Feels/reports the urge to defecate	Always = 3
	Most of the time = 2
	Uncertain = 1
	Absent = 0
Frequency of defecation	Every other day—twice a day = 2
	More often = 1
	Less often = 1
Soiling	Never = 3
	Staining <1/week, no change of underwear required = 2
	Frequent staining/soiling, change of underwear often required = 1
	Daily soiling, requires protective aids = 0
Accidents	Never = 3
	Less than 1/week = 2
	Weekly accidents, requires often protective aids = 1
	Daily, requires protective aids during day and night = 0
Constipation	No constipation = 3
	Manageable with diet = 2
	Manageable with laxatives = 1
	Manageable with enemas = 0
Social problems	No social problems = 3
	Sometimes (foul odors) = 2
	Problems causing restriction in social life = 1
	Severe social and/or psychic problems = 0

We further analyzed long-term functional results grouping our series according to age (younger or older than 3 years) to obtain information regarding continence after the age of toilet training.

Results

In 14 years, we performed 12 LAARPs to treat male patients with bladderneck (1/12), prostatic (5/12), and bulbar (6/12) fistula. Characteristics of our series are summarized in Table 3. The mean age at anorectoplasty was 4.75 months (range 1–14 months). Sacral vertebral anomalies were found in seven patients (four sacral agenesis and three coccygeal agenesis), and two had spine defects that required surgery at 1 and 2 years of age because of progressive enlargement of the myelomeningocele and symptomatic tethered cord. Underdeveloped perineal muscles were identified in four patients (one with bladderneck fistula and three with bulbar fistula). The mean sacral ratio was 0.56.

Table 3.

Characteristics of Our Patients (N = 12)

Gestational age at birth (weeks)	39 (range 36–40)
Body weight at birth (g)	3013 (range 2300–4070)
Underdeveloped perineal muscles (No. of patients)	4
Age at surgery (months)	4.75 (range 1–14)
Body weight at surgery (g)	6192 (range 4068–9800)
Operation time (minutes)	262 (range 140–435)
Postoperative stay (days)	8.5 (range 6–12)
Length of follow-up (years)	8 (range 2–13)
Sacral ratio	0.56 (range 0.3–1)
Sacral vertebral anomalies (No. of patients)	7 (4 sacral agenesis and 3 coccygeal agenesis)
Spine (No. of patients)	2 (tethered cord and myelomeningocele)
Associated anomalies	No. of patients (type)
Cardiac	3 (2 VSD, 1 PDA)
Renal	2 (VUR, MCKD)
Genital	3 (Hypospadias)
Perineal masses	3 (Lipomas)
Other	1 (Esophageal atresia)

MCKD, mutlicystic kidney disease; PDA, patent ductus arteriosus; VSD, ventricular sepral defect; VUR, vesico-ureteral reflux.

The mean operative time and weight at surgery were, respectively, 262 minutes and 6192 g. The mean length of hospital stay was 8.5 days. Postoperative course was uneventful in all our patients.

It is to notice the finding of three patients with perineal masses (that turned out to be lipomas) in our series. They were all large lesions outside the sphincter muscles, easily removed at the time of anoplasty.

Table 4 resumes functional results of our patients (in terms of voluntary bowel movements, use of enemas or laxatives, soiling, and Kelly and Rintala scores) compared to type of fistula, age at follow-up, sacral ratio, presence of perineal muscles underdevelopment, and vertebral/spinal anomalies. The mean duration of follow-up was 8 years. The overall mean functional score at long-term follow-up was 11.17 (Rintala) and 3.75 (Kelly) (Rintala range 4–16 and Kelly range 2–5). The Kelly and Rintala scores were, respectively, 3.6 and 11.3 considering only patients older than 3 years of age. Younger patients obtained mean scores of 10.5 and 4.5, respectively.

Table 4.

Functional Results Related to Type of Fistula, Age, Spinal Anomalies, Sacral Ratio, and Perineal Muscles Development

Pt	Type of fistula	Age (years)	Vertebral/Spinal anomalies	SR	PMU	VBM	Laxatives	Enemas	Soiling (frequency: times per week)	Kelly score	Rintala score
1	RB	12.7	Yes (sacral agenesis)	0.5	No	Yes	Yes	Yes	Yes (1–2)	4	11
2	RP	14	Yes (sacral agenesis)	0.48	No	Yes	No	No	Yes (1–2)	4	16
3	RP	12.6	Yes (sacral agenesis)	0.3	No	Yes	No	Yes	Yes (1–2)	3	10
4	RP	10.3	Yes (coccigeal agenesis)	0.7	No	Yes	No	No	Yes (4–5)	5	16
5	RB	10.8	Yes (sacral agenesis, myelomeningocele)	0.3	Yes	Yes	Yes	Yes	Yes (every day)	2	4
6	RB	9	No	0.3	Yes	Yes	No	Yes	Yes (every day)	2	4
7	RP	9	No	0.6	No	Yes	No	Yes	No	4	16
8	RP	5.3	No	0.72	No	Yes	Yes	No	Yes (1)	4	13
9	RB	2.8	No	0.76	No	Yes (diaper)	No	No	Yes (every day)	5	10
10	RB	3.2	No	0.68	No	Yes	No	No	Yes (1–2)	5	12
11	RBla	3.4	Yes (coccigeal agenesis, tethered cord)	1	Yes	Yes	No	No	Yes (every day)	3	11
12	RB	2.4	Yes (coccigeal agenesis)	0.4	Yes	Yes (diaper)	No	No	Yes (every day)	4	11

PMU, perineal muscles underdevelopment; RB, recto-bulbar; RBla, recto-bladderneck; RP, recto-prostatic; SR, sacral ratio; VBM, voluntary bowel movements.

Regarding complications, we observed two patients with rectal prolapse (respectively, Nos. 4 and 8): one with asymptomatic minimal hemicircumferential rectal prolapse and the other one with evident circumferential rectal prolapse. Patient No. 2 developed high rectal tone. The boy with persistent evident prolapse required surgical excision at 2 years of age. The other patient with hemicircumferential prolapse was treated conservatively: after fecal disimpaction, he was started on a bowel management program with improvement. High rectal tone disappeared after a period of rectal dilatations. One patient with recto-prostatic fistula developed asymptomatic blind ending urethral fistula (occasionally identified during ultrasound [US] evaluation and confirmed at micturating cystourethrogram [MCUG]) that disappeared over time.

Patients with recto-prostatic fistula obtained the highest functional scores (mean Kelly score 4 and mean Rintala score 14.2) and all of them had normal perineal muscles. Among the four patients with sacral ratio below 0.4, three had perineal muscles hypoplasia. They are all patients with rectourethral fistula with a mean Kelly score of 3.6 and a mean Rintala score of 8.6.

Discussion

The treatment of ARMs with urinary fistula represents a challenge for pediatric surgeons. Indeed, there are technical difficulties that should be overcome to reduce complications¹² and there are problems related to the malformation itself that is associated with a poor functional prognosis.^1,7,8,13 The most commonly used surgical technique to treat these anomalies is the posterior sagittal approach. LAARP was popularized by Georgeson at the beginning of the 21st century⁹ after its first description in 1998.¹⁴ Laparoscopy permits excellent visualization of the rectal fistula and surrounding structures and accurate placement of the pull-through segment at the center of the levator sling.^1,9,15 It is estimated that LAARP is required in about 10% of boys with ARMs.¹⁶

In particular, the presence of a recto-bladderneck fistula is a clear indication for LAARP since it avoids the laparotomy.^1,2,7,17 The laparoscopic approach in case of prostatic fistula is an accepted procedure when the surgeon has experience in the field of minimally invasive surgery and he might find the posterior sagittal operation technically demanding. LAARP is a questionable indication to treat bulbar fistulas. Indeed, according to some authors, the presence of a long common wall between the rectum and urethra in bulbar fistula makes laparoscopy not recommended^7,17 as it requires an extensive distal dissection and it is associated with an increased risk of urethral complications (recurrent fistula and posterior urethral diverticulum).⁷

In our series, we performed LAARP in six patients with bulbar fistula. Our choice was based on the ease of the laparoscopic approach in the hands of well-trained laparoscopists. In these patients, we decided not to close the fistula to reduce the risk of diverticula, as suggested by Van der Zee et al.,¹² followed by 5-day-long urethral catheterization.

Concerning urologic outcomes, two patients had urinary incontinence related to neurologic bladder. Except from these two patients, we never documented urinary symptoms, even if it is questionable the fact that we do not routinely perform MCUG. We accidentally found a urethral blind-ending residual fistula in one patient 5 years after the surgery. The patient was born with a recto-prostatic fistula corrected by LAARP. Despite the presence of the residual fistula, he was asymptomatic so we followed him conservatively.

Urethral diverticula are found in 2% of patients after ARM repair¹⁸ and it seems that they are detected more frequently after the laparoscopic approach, probably because of the limited view of the common wall between rectum and urethra.^19,20 The indication for resection is controversial, and active surveillance of patients with urethral diverticulum is advocated in case of asymptomatic patients without obstructive urinary signs.^18,19 In our patient, there was a blind-ending residual fistula more than a “real diverticulum.” This was another reason for conservative management, along with the fact that the patient was asymptomatic. The patient underwent US evaluation and subsequent MCUG to better investigate the detection of a cystic structure. We do not routinely perform MCUG to reduce the radiation exposure in small children.

Overall, we reported a few complications, confirming that the technique is good in the hands of experienced operators. The most commonly reported complications include: rectal prolapse, anal stenosis, urethral diverticulum, and incorrect placement of the neo-anus. The incidence of rectal prolapse is reported between 8.8% and 46% in different series.²¹ Hypothesized explanations are the inadequate fixing of the rectum to the presacral fascia, extended dissection, or the early use of the anus.^17,21 It is also to say that high ARMs with poor sacral and pelvic musculature are more commonly associated with rectal prolapse.²¹ In our series, the incidence of rectal prolapse was 16.6% (2/12 patients, one of them with perineal muscles underdevelopment), but just one patient required surgical correction. We never tried to anchor the rectum to the fascia. A limited but adequate rectal dissection might be sufficient to prevent prolapse. If not, a minimal or moderate rectal prolapse may resolve spontaneously in the first year after surgery with an adequate bowel management program. As suggested by our colleagues, surgery should be reserved for symptomatic patients with negative effects of the prolapse on their daily lives.²²

Recent articles show that outcomes after LAARP are still unclear because of lacking randomized studies and the influence of other elements on future function.^7,18,21,22 In a recent article, Peña and colleagues call us to improve the quality of reporting starting from a standardization process.⁷ The need for standardization of outcome reporting was also advocated by Al-Hozaim et al. in 2010.¹⁵

In light of these recent considerations, we decided to review our series. We obtained acceptable long-term functional results, although we observed patients with fairly low scores. We noticed that low scores were more common in patients with associated spinal or congenital anomalies (one patient with myelomeningocele and neurologic bladder obtained a Kelly and Rintala score of 2 and 4, respectively) confirming the assumption that there are complex malformations associated with poor prognosis despite the technique chosen for the correction. The group that obtained the lowest score was the one with poor associated prognostic factors (such as Sacral ratio below 0.4 and underdeveloped perineal muscles). Low scores were also observed in patients we operated on at the beginning of our activity confirming the need for a well-structured experience.

However, it seems that we still have to better interpret long-term outcomes and functional advantages, evaluated with clinical, manometric, and radiological studies.^21,23,24

In the context of this ongoing discussion, concerning the comparison between laparoscopy and PSARP, we share the assumptions of some colleagues who suggest that it is not a matter of which technique is more important or better, but it comes to choosing the right procedure in each single case.^12,16

Conclusions

Laparoscopic repair of ARMs with urinary fistulas is safe, simple, and feasible. We do not pretend to have better functional results compared to PSARP, but we consider LAARP a valid alternative when PSARP is a surgical challenge. We believe that the right procedure should be chosen in every single case on the basis of the type of malformation and the surgeon's attitude.

Footnotes

Disclosure Statement

No competing financial interests exist.

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