Anorectal Malformations in Males: Pros and Cons of Neonatal versus Staged Reconstruction for High and Intermediate Varieties.

BACKGROUND: High and intermediate types of anorectal malformations (ARMs) in male neonates may be managed either by primary neonatal reconstruction without colostomy cover or by traditional policy of staged reconstruction after neonatal colostomy. Posterior sagittal anorectoplasty (PSARP) is the current widely practiced reconstructive technique with varied results. AIM: To assess our functional results of PSARP without colostomy in male neonates with high and intermediate ARMs compared to 3-stage (neonatal colostomy - PSARP - colostomy closure) methodology in a high volume tertiary care institution of a developing country. PATIENTS AND METHODS: The number of colostomies performed for male high/intermediate anomalies and the number of babies who completed 3-stage reconstruction during a 10-year period is analyzed. The outcome of primary neonatal PSARPs during the same period was analyzed. Eighty primary PSARPs were compared to 81 staged reconstructions for outcome analysis, using Kelly score.
RESULTS: A total of 453 colostomies were performed, but only 253 of them completed all stages of reconstruction (52%). Good continence was achieved in 45% of cases of primary PSARP versus 26% in 3-staged surgery.
CONCLUSIONS: Primary PSARP in neonatal period without colostomy is a good option for high and intermediate ARMs in males if the treating surgeon is reasonably skilled in neonatal surgery and PSARP procedures.

Introduction

Anorectal malformations (ARMs) are one of the most common conditions in pediatric surgical practice. A lot has been written about the techniques of management and currently Pena's posterior sagittal anorectoplasty (PSARP) occupies the center stage.[12] There are many scoring systems which are in vogue to measure the continence results and quality of life scores.[34567891011] The anomalies may be treated by conventional three stages[12] (neonatal colostomy – PSARP or abdominoperineal pull-through [APPT] – colostomy closure) or accomplished by reconstruction in neonatal period without colostomy.[13141516171819202122] There are many proponents of single stage reconstruction in the literature in female anomalies also.[2324] The aim of this study is to present a large series of single stage reconstruction in the neonatal period without colostomy and compare the results with 3-stage reconstruction performed during the same period in the same circumstances, in male ARMs.

Patients and Methods

The number of neonatal high divided sigmoid colostomies for male high/intermediate ARMs performed in our department over a 10-year period from 2006 to 2016 was counted. The number of patients completing subsequent 2 stages of management (PSARP or APPT and colostomy closure) during the same period was documented. The neonates who underwent primary PSARP without colostomy cover after initial cross table prone lateral (CTPL) radiograph[25] were analyzed during this period (Group A). It is the senior authors’ practice to perform primary PSARP in all neonates whose CTPL radiograph shows air in the presacral region. Survival figures and functional results (Kelly score)[6] in this group were compared with all babies who underwent 3-stage reconstruction by 2 consultant surgeons (PM, KLNR) (Group B). Continence achieved in different locations of recto-urinary communication was analyzed. Three years follow up for neonatal PSARP and 2 years follow up after the completion of all 3 stages was accepted as minimum requirement for assessing continence. Statistical significance for continence results between two groups was calculated using Chi-square test (Pearson value) with a P < 0.05 taken as significant. Congenital pouch colons[2627] and low ARMs referred after initial colostomy were excluded from this study.

Results

A total of 453 colostomies were performed for high and intermediate variety of ARMs in males in our department during the study period [Table 1]. However, all of them did not translate into definitive reconstruction with only 253 (52%) patients completing all the stages of definitive surgery. Group A comprised 80 neonates and Group B had 81 babies. Table 2 outlines continence scores in both groups. Significant “good” scores were achieved in primary PSARP group. The location of the recto-urinary tract fistula did not affect the continence rates significantly [Table 3].

Table 1

Number of colostomies versus posterior sagittal anorectoplasty/abdominoperineal pull-throughs performed for high and intermediate anorectal malformations in males

Table 2

Anorectal malformation: Comparison of fecal continence in primary versus staged surgery groups

Table 3

Continence versus location of fistula

Discussion

Despite considerable interest of surgeons, the ultimate results of high and intermediate ARMs as measured by continence results are not completely satisfactory. The reported continence rates vary from 2% to 84% (!).[2829303132] Neonatal high divided sigmoid colostomy in a distended abdomen of a sick baby is not devoid of complications and various authors have confirmed this.[333435] In developing countries, especially patients from low economic strata, the conventional policy of colostomy - PSARP – colostomy closure effectively means long duration of treatment, significant costs in terms of time and resources, difficult social environment, mental trauma to parents and ultimately, questionable continence results. Patients not coming for follow up after a colostomy are well known to all pediatric surgeons. This may be due to loss of life due to diarrhea, associated anomalies or dis-inclination to continue treatment. In our center, this happened in 219 of 453 colostomies over a 10-year period (48% loss) despite most babies going home initially. It may be argued that some of these babies may be undergoing subsequent reconstruction elsewhere, but it is also true that being a tertiary care center, we perform secondary reconstructive surgery for many babies whose initial colostomy had been performed elsewhere. These 2 factors should reasonably neutralize each other for statistical purpose.

We presented here one of the largest series of male neonatal PSARPs for high and intermediate varieties in the literature (Group A). The initial mortality figures of 15%, mainly due to congenital heart disease, ventilator related issues or sepsis appear to be a drawback; however, cognizance has to be taken of the ultimate loss of 48% of babies in staged reconstruction. The ethical, philosophical, or reputational issues may have to be taken into consideration. Counseling of the family is essential and may help. Apart from providing the family, a one-time surgery solution, the continence rate is better in this group (45% good continence compared to 26% in staged reconstruction; P = 0.045). The authors do not underestimate the requirement of technical surgical skills required, logistics of neonatal reconstruction in terms of hospital resources (timings of available operation theaters for the neonates etc.). But once accomplished, this policy would conserve hospital resources especially valuable operation theater time of a busy hospital for other needy patients. The surgeon's skill is of paramount importance.[36]

The advantages of neonatal single stage PSARP may be summarized as follows: For the parents, avoidance of two additional anesthesia/surgeries, burden of colostomy management under difficult social conditions and lesser time spent in the hospital and economic cost. For a busy hospital, saving resources of theatre and bed occupancy time is significant. For the patient, better continence rates and avoidance of entry into abdomen for colostomy and colostomy closure may enhance the quality of life. Better continence rates may be explained by better brain-anal reflexes development right from the time of birth.[1314] Technically, it is quite easy to find and dissect the meconium filled, distended rectal pouch at birth during primary PSARP procedure.

What are the reasons for not so complimentary results in these high ARMs? Poorly developed muscles of continence and neural deficiency are primary factors. It is possible that the puborectalis muscle, once divided and even if adequately reconstructed may end in fibrosis and may not function optimally. Disuse atrophy for many months or years of waiting for the reconstructive process to complete may partly be responsible. High incidence of constipation may be due to excess use of diathermy on the rectum resulting in denervation or due to colonic motility problems. Laparoscopy assisted pull through has been proposed in the recent years.[3738] However, the authors’ personal exposure to the patients treated by this method did not inspire confidence in this technique. The “master” of the technique, Peña and Hong, in the world's largest series of 1192 patients[2] has reported 37.5% full continence, 37.5% soiling and 25% total incontinence. As a rule of thumb, 1/3 of these children may achieve good continence, 1/3 fair and 1/3 may be fully incontinent.[39] Ultimately for achieving “social continence”, bowel management program or antegrade colonic enemata are a necessary part of rehabilitation in these children.[40] Rintala and Lindahl[41] have reported the improvement of continence at adolescence as constipation disappears.

Conclusion

If the treating surgeon is reasonably skilled in neonatal surgery and PSARP procedures, the authors would recommend consideration of primary neonatal PSARP in these anomalies. Although there is higher initial mortality, ultimately more babies survive with better continence rates in the long term. Availability of operation theater logistics for performing these emergency procedures on an elective basis is essential.

Limitations of the study: It is not a randomized study. Most of the primary neonatal PSARPs were done by senior experienced surgeons and some amount of surgical skill bias cannot be eliminated.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.