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REVIEW ARTICLE
Year : 2005  |  Volume : 10  |  Issue : 2  |  Page : 80-85
 

Assessment of postoperative results in anorectal malformations


Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India

Correspondence Address:
V Bhatnagar
Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9261.16466

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   Abstract 

Surgery for the correction of anorectal malformations (ARM) is performed by almost all pediatric surgeons. A number of operative procedures are practiced. The posterior sagittal anorectoplasty procedure has found wide acceptability and although it provides the most accurate anatomical reconstruction of the neoanorectum and the sphincters, the results are not in keeping with this technical advantage. In addition, there is no uniformity in describing the results of surgery and hence comparative evaluation of different series is difficult. This review describes the various methods that are available for the assessment of postoperative results following surgery for ARM, with a special emphasis on clinical methods keeping in mind the limitations in various parts of the country. Clinical examination and clinical scoring systems hold an important place in the post-operative evaluation of these patients. Imaging modalities are useful in the diagnosis of misplaced bowel and damage to the muscle complex and are necessary before re-do surgery is planned. Objectivity in the evaluation is provided by anorectal manometry combined with electromyography and these corelate well with clinical scoring systems. A consensus is required for uniformity in the methods of assessment.


Keywords: Anorectal malformations, anorectal manometry, clinical scoring, fecal continence


How to cite this article:
Bhatnagar V. Assessment of postoperative results in anorectal malformations. J Indian Assoc Pediatr Surg 2005;10:80-5

How to cite this URL:
Bhatnagar V. Assessment of postoperative results in anorectal malformations. J Indian Assoc Pediatr Surg [serial online] 2005 [cited 2017 Apr 23];10:80-5. Available from: http://www.jiaps.com/text.asp?2005/10/2/80/16466


The correction of anorectal malformations (ARM) forms a significant part of every pediatric surgeon's practice. These anomalies with an estimated incidence of 1 in 5000 live births and a preponderance in males seem to be fairly well distributed all over the country (although the incidence varies in different areas and some malformations, e.g., the pouch colon appear to be more common in North India).[1]

Over the last half century or more, the treatment of ARM has evolved from a simple cutback/translocation anoplasty to abdominoperineal pull through, sacroperineal pull through, and its modifications to the currently practiced procedure of posterior sagittal anorectoplasty (PSARP). All operative procedures for the correction of ARM aim at providing a portal for the discharge of feces from the perineum and establishing a working relationship between the bowel and sphincter. Although PSARP allows better understanding of the muscular anatomy vis--vis bowel orientation, a significant proportion of patients have postoperative fecal incontinence.[2]

The act of defecation and fecal continence are complex physiological functions depending on a variety of factors including the rectum, internal and external sphincters, pelvic floor muscles, sensory and motor functions, and integrity of neural (both autonomic and somatic) pathways.

The methods employed for the assessment of surgical results have included:

  • Clinical evaluation and scoring systems;
  • Imaging - magnetic resonance, contrast-enhanced CT, ultrasound, defecography;
  • Pressure/electrical activity - manometry and electromyography (EMG);



   Clinical evaluation Top


Clinical examination with or without imaging studies is necessary for diagnosing the coexistence of other anomalies - the VATER and VACTERL associations are well known to all pediatric surgeons. Some of the more common and serious associated anomalies are as follows:

Vertebral, sacral 10-25%

Spinal

Tethered cord, syringomyelia, lipoma 15-40%

Currarino triad

ARM, presacral mass, sacraldefect Rare

CVS

TOF, VSD, TGA, hypoplastic, left heart

syndrome 10-25%

GI

TEF, malrotation, atresia-duodenal,

small/large bowel, aganglionosis 10%

GU

VUR, renal agenesis/dysplasia, cryptorchism,

hypospadias, genital anomalies, cloaca 20-60%

Some of these anomalies can be life threatening and need to be treated even before the correction of ARM. Others can seriously affect the quality of life and the ultimate prognosis.[3],[4]

A good clinical examination will also help in detecting genital tract problems, which could lead to postoperative genital, fertility, and sexual problems. In females, almost half the patients may have inadequate genital function due to genital anomalies and vaginal scarring. Also, a high majority may require caesarian section for delivery and may report a worsening of continence during pregnancy or following vaginal delivery.[5],[6] In males, on the other hand, more than half the patients may develop infertility due to ejaculatory duct obstruction, retrograde ejaculation, and erectile dysfunction.[7],[8]

Clinical examination also helps in the evaluation of complications, e.g., wound infections/dehiscence, retraction of neoanus, iatrogenic fistulas, voiding dysfunction, anal stenosis, higher strictures, rectal mucosal prolapse, constipation, psychological disturbances, and incontinence.

Digital examination can assess and predict the level of continence, e.g., a good squeeze pressure on command is associated with good results.[9] In small babies, digital examination may neither be feasible nor desirable due to the narrow caliber of the neoanus. In such situations, it may be more prudent to use the anal dilator, although it may only give information on the caliber of the neoanus. The desirable anal/anorectal caliber with respect to age should be:[10]Hegar's #

1-4 months 12

4-8 months 13

8-12 months 14

1-3 years 15

3-12 years 16

>12 years 17


   Clinical scoring systems Top


A number of clinical scoring systems are in use currently. For a scoring to be popular, it should be simple, and easy to apply and yet be able to differentiate between subtle grades of continence.

The Kelly's score of continence

This system of scoring awards points for three basic parameters. An overall score of 5-6 is considered good, 3-4 fair, and 0-2 poor.[11] It is by far the simplest of all scoring systems and the easiest to apply even in the office setting.

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

The Pena's criteria for assessment of continence

These criteria do not award points/scores but only classify three grades of continence.[12]

Voluntary bowel movement feels defecation urge, capacity to express the need, and ability to hold bowel movement.

Soiling

I Occasional, <2/week, no change of underwear

II Frequent, 1/day, change of underwear sometimes

III Constant

Constipation

I Manageable with diet

II Manageable with laxatives

III Manageable with enemas

Urinary incontinence

I Mild dribbling day and night

II Complete incontinence

The Templeton score of continence

In this system of scoring, scores are awarded for six parameters, and the scores are classified as good (4-5), fair (2-3.5), and poor (0-1.5).[13]

Toilet training for stool

Successful 1.0

Occasionally successful 0.5

No awareness of impending stool 0

Accidents

None or rare 1.0

3/week or less 0.5

More than 3/week 0

Extra underpants/liners

Never 1.0

Only when diarrhea 0.5

Always 0

Social problems

None 1.0

Infrequent odor, attends school, no dates, etc. 0.5

Frequent odors affecting school and play 0

Activity restriction

None 0.5

Avoids swimming, sports 0

Rashes

No current problems 0.5

Some current problems 0

The Holschneider score of continence

This system is a little more detailed than the previous one and gives a score of 10-14 for good, 5-9 for fair, and 0-4 for poor continence.[14]

Frequency of defecation

Normal (1-2/day) 2

Often (3-5/day) 1

Very often 0

Fecal consistency

Normal 2

Loose 1

Liquid 0

Soiling

No 2

Stress/diarrhea 1

Constant 0

Rectal sensation

Normal 2

Defective 1

Missing 0

Ability to hold back defecation

Minutes 2

Seconds 1

Missing 0

Discrimination between formed loose or gaseous stool

Normal 2

Defective 1

Missing 0

Need for therapy (enemas, drugs, napkins)

No 2

Occasional 1

Always 0

The Rintala score of continence

This scoring system goes into more details and offers a maximum score of 20 but does not classify into good, fair, or poor.[15]

Ability to hold back defecation

Always 3

Problem <1/week 2

No voluntary control 1

Feels/reports urge to defecate

Always 3

Most of the time 2

Uncertain 1

Absent 0

Frequency of defecation

Every other day-2/day 2

More often 1

Less often 0

Soiling

Never 3

Staining, <1/week, no underwear change 2

Frequent, often underwear change 1

Daily, protective aids 0

Accidents

Never 3

<1/week 2

Weekly, often protective aids 1

Daily, protective aids day and night 0

Constipation

Nil 3

Manage with diet 2

With laxatives 1

With enemas 0

Social problems

Nil 3

Sometimes 2

Deterioration in social life 1

Severe social/psychological problems 0

The number of scoring systems in use is testimony to the fact that a comprehensive and yet simple system has not been devised yet. Variations in reported results are due to the small number of cases in individual series, use of different scoring systems, individual steps of surgery not being detailed, and height of atresias not being considered.[16]


   Imaging Top


Imaging studies in the postoperative assessment of ARM patients serve two major functions - for the evaluation of associated malformations and the assessment of the causes for fecal incontinence including the complications of surgery. The commonly employed imaging methods include defecography, contrast-enhanced computerized tomography (CT) scan, MRI scan, and ultrasonography including endorectal sonography.


   Defecography Top


Defecography is a modification of the barium enema, which provides a dynamic evaluation of the anorectal function under fluoroscopy. Lateral films are taken during the act of defecation. The salient features are that at rest the anal canal should be closed and the anorectal angle should be 90 or less and during defecation the anal canal opens and the anorectal angle straightens out. In abnormal situations, the anal canal may remain open or the anorectal angle maybe obtuse.[17] A dedicated radiologist and an X-ray room in which a patient can defecate are the prerequisites. Standard barium enemas are useful in the evaluation of constipation to rule out stenoses, strictures, and posterior shelfing in the anal canal or re-dilatation in cases of pouch colon.


   CT and MRI scans Top


Contrast-enhanced CT scans and MRI scans have been used to assess the status of the lumbosacral vertebrae and spinal cord and the integrity of the levator ani/striated muscle complex. In general, MRI scans are comparatively better with regard to the resolution of images of the muscle complex and particularly that of the vertebrae and spinal cord. Abnormalities of the vertebrae and spinal cord resulting in fecal incontinence carry a poor prognosis. On the other hand, damages to the muscle complex are potentially repairable. In addition, these studies may reveal an excess of fat outside the neoanorectum, which prevents it from functioning as a cohesive unit, with the external sphincters or a misplaced bowel vis--vis the muscle complex - these can also be corrected surgically. [18],[19],[20],[21]


   Ultrasonography Top


Endorectal sonography has also been employed for the identification of the muscle complex during laparoscopy-assisted pull through for high imperforate anus. However, the experience is limited.[22] This technique has the potential to be used postoperatively also.


   Anorectal manometry and EMG Top


Functional results of surgical treatment of ARM can be evaluated with anorectal manometry, which assesses the functional compliance of the muscles responsible for the act of defecation and continence, and the electrical activity of muscle contraction of the external sphincter can be studied using EMG.

The patient should be sedated and in the supine position. A balloon-tipped probe is inserted in the neoanus and EMG needles are placed on either side of the neoanus. Baseline and squeeze pressures, anorectal reflex, and anal canal pressure profile in response to rectal dilatation are measured.[23]

Langemeijer and Molenaar[24] have devised a scoring system on the basis of manometry, which has therapeutic implications:

[Table - 1]

In a comparative study on 41 patients, we have found that the clinical outcome depends on the type of anomalies (continence rates: low - 90%; high - ~50%). Anorectal manometry assesses functional compliance, correlates well with Kelly's score, can predict long-term results, and should be used for decision regarding re-operation. Electromyography activity assesses external sphincter status.[25] Other studies using anorectal manometry have also shown the objective utility in assessing postoperative results.[26],[27]

Anorectal manometry can also demonstrate objective evidence of improvement with biofeedback therapy. In these patients, the prerequisites are that the sphincter should not be hypoplastic, and the bowel should not be misplaced.[28],[29]


   Other functional studies Top


Fecodynamic studies

Fecoflowmetry and saline enema test help in obtaining clinical indicators for bowel function by assessing the motor activity of the pelvic floor muscle and providing qualitative and quantitative evaluations of the anorectal motor activity.[30],[31] The test is performed by instilling saline in a clean rectum and then measuring the flow and amount of saline passed out in a manner similar to mictiometry.

Urodynamic studies

A number of studies have shown urodynamic abnormalities ranging from dysfunctional voiding to frank urinary incontinence in patients with ARM.[32] The incidence of postoperative urinary incontinence in these patients ranges from 0 to 10% in patients with low ARM, up to 28% in patients with high ARM in the pre-PSARP period and significant reduction in the post-PSARP period (8-10%). Recent studies have shown that a high percentage of preoperative patients with ARM have occult neurovesical dysfunction (small capacity bladder, uninhibited detrusor contractions, poor compliance, etc.) even in the absence of sacral or spinal defects, and changes in capacity, compliance, and detrusor contractions may occur in the postoperative period.[33],[34],[35]


   Plan of management Top


An algorithm has been suggested for the postoperative assessment of patients with ARM.[36] Following surgery for ARM, all patients should be put through toilet training, and a fecal continence scoring done at 3 years of age. If the score is fair or poor, then the patient should receive dietary management, incentives, pharmacological intervention, and enemas (singly or in combination) till a re-scoring at the age of 5 years. Persistently poor scores should prompt investigations to rule out neurogenic bladder and bowel, sacral/spinal defects, and poor pelvic muscles - in which case the patient should be advised a permanent colostomy. If the investigations suggest a misplaced bowel or other correctable conditions, then an appropriate re-operation should be carried out and the patient reassessed at the age of 10 years.

In conclusion, the aim of postoperative assessment is to categorize the patients into three groups that have different treatment options for the management of postoperative problems:[3]

Group I - these patients have poor anatomy, flat bottom, poor quality muscle, sacral defect, and urinary incontinence. The treatment options for such patients range from biofeedback, antegrade colonic enema, enemas/suppositories, muscle transfer operations, and permanent colostomy.

Group II - these patients have good quality muscle and good quality sacrum but misplaced bowel. The treatment options in these patients are either repositioning the bowel or defattening the pulled through colon.

Group III - in these patients, constipation is the persisting problem. These patients can be managed with enemas, suppositories or anterior resection.



 
   References Top

1.Chatterjee SK. Anorectal Malformations. A Surgeon's Experience. Delhi: Oxford University Press; 1991. p. 1-3.  Back to cited text no. 1    
2.Pena A, Hong A. Advances in the management of anorectal malformations. Am J Surg 2000;180:370-6.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Kiely EM, Pena A. Anorectal malformations. In, O'Neill JA Jr, Rowe MI, Grosfeld JL, Fonkalsrud EW, Coran AG, editors. Pediatric Surgery. 5th Ed. St Louis: Mosby; 1998. p. 1425-48.  Back to cited text no. 3    
4.Smith ED, Saeki M. Associated anomalies in anorectal malformations in children: Update 1988. Birth Defects 1988;24:501-49.  Back to cited text no. 4  [PUBMED]  
5.Hall R, Fleming S, Gysler M, McLorie G. The genital tract in female children with imperforate anus. Am J Obstet Gynecol 1985;151:169-71.  Back to cited text no. 5  [PUBMED]  
6.Matley PJ, Cywes S, Berg A, Ferreira M. A 20 year follow up of children born with vestibular anus. Pediatr Surg Int 1990;5:37-40.  Back to cited text no. 6    
7.Rintala R, Mildh L, Lindahl H. Fecal continence and quality of life in adult patients with an operated high or intermediate anorectal malformation. J Pediatr Surg 1994;29:777-80.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]
8.Pryor JP, Hendry WF. Ejaculatory duct obstruction in subfertile males: Analysis of 87 patients. Fertil Steril 1991;56:725-30.  Back to cited text no. 8  [PUBMED]  
9.Shandling B, Gilmour R, Ein S. The anal sphincter force in the evaluation of postoperative imperforate anus. J Pediatr Surg 1991;26:1369-71.  Back to cited text no. 9  [PUBMED]  [FULLTEXT]
10.Pena A. Surgical Management of Anorectal Malformations. New York: Springer Verlag; 1989. p. 15.  Back to cited text no. 10    
11.Stephens FD, Smith ED. Anorectal Malformations in Children. Chicago: Year Book Medical Publishers; 1971.  Back to cited text no. 11    
12.Pena A. Anorectal malformations. Semin Pediatr Surg 1995;4:35-47.  Back to cited text no. 12  [PUBMED]  
13.Templeton JM, Ditesheim JA. High imperforate anus: Quantitative result of long term fecal continence. J Pediatr Surg 1985;20:645-52.  Back to cited text no. 13    
14.Holschneider AM. Elektromanometrie des Enddarms. Munich: Urban and Schwarzenberg; 1983. p. 213-8.  Back to cited text no. 14    
15.Rintala R, Lindahl H. Is normal bowel function possible after repair of intermediate and high anorectal malformations? J Pediatr Surg 1995;30:491-4.  Back to cited text no. 15    
16.Holschneider AM, Jesch NK, Stragholz E, Pfrommer W. Surgical methods for anorectal malformations from Rehbein to Pena - critical assessment of score systems and proposal of a new classification. Eur J Pediatr Surg 2002;12:73-82.  Back to cited text no. 16  [PUBMED]  [FULLTEXT]
17.Langemeijer RATM, Molenaar JC. Continence after posterior sagittal anorectoplasty. J Pediatr Surg 1991;26:587-90.  Back to cited text no. 17    
18.Minocha A, Gupta AK, Mitra DK. Computed tomography of sphincteric muscle complex in anorectal malformations. Department of Paediatric Surgery, AIIMS, New Delhi, M.Ch. Dissertation, 1995.  Back to cited text no. 18    
19.Taccone A, Marzoli A, Martucciello G, Salomone G, Magnano GM, Dodero P, et al . Computed tomography vs magnetic resonance in the diagnosis of anorectal anomalies. Radiol Med (Torino) 1991;82:638-43.  Back to cited text no. 19    
20.Tsuji H, Okada A, Nakai H, Azuma T, Yagi M, Kubota A. Follow up studies of anorectal malformations after posterior sagittal anorectoplasty. J Pediatr Surg 2002;37:1529-33.  Back to cited text no. 20  [PUBMED]  [FULLTEXT]
21.Taccone A, Martucciello G, Fondelli P, Dodero P, Ghiorzi M. CT of anorectal malformation - a postoperative evaluation. Pediatr Radiol 1989;19:375-8.  Back to cited text no. 21  [PUBMED]  
22.Yamataka A, Yoshida R, Kobayashi H, Lane GJ, Kurosaki Y, Segawa O, et al . Intraoperative endosonography enhances laparoscopy assisted colon pull through for high imperforate anus. J Pediatr Surg 2002;37:1657-60.  Back to cited text no. 22    
23.Bhat NA, Bhatnagar V. Anorectal manometric evaluation of patients with anorectal anomalies. Department of Paediatric Surgery, AIIMS, New Delhi, M.Ch. Dissertation, 2001.  Back to cited text no. 23    
24.Langemeijer RATM, Molenaar JC. Defecation problems in children: Anatomy, physiology and pathophysiology of the defecation mechanism. Neth J Surg 1991;43:208-12.  Back to cited text no. 24    
25.Bhat NA, Grover VP, Bhatnagar V. Manometric evaluation of postoperative patients with anorectal anomalies. Indian J Gastroenterol 2004;23:206-8.  Back to cited text no. 25    
26.Heikenen JB, Werlin SL, DiLorenzo C, Hyman PE, Cocjin J, Flores AF, et al . Colonic motility in children with repaired imperforate anus. Dig Dis Sci 1999;44:1288-92.  Back to cited text no. 26    
27.Martins JL, Pinus J. Clinical and manometric postoperative evaluation of posterior sagital anorectoplasty in patients with upper and intermediate anorectal malformations. Sao Paulo Med J 1996;114:1303-8.  Back to cited text no. 27  [PUBMED]  
28.Iwai N, Nagashima M, Shimotake T, Iwata G. Biofeedback therapy for fecal incontinence after surgery for anorectal malformations: Preliminary results. J Pediatr Surg 1993;28:863-6.  Back to cited text no. 28  [PUBMED]  [FULLTEXT]
29.Hibi M, Iwai N, Kimura O, Sasaki Y, Tsuda T. Results of biofeedback therapy for fecal incontinence in children with encopresis and following surgery for anorectal malformations. Dis Colon Rectum 2003;46:S54-8.  Back to cited text no. 29  [PUBMED]  
30.Kayaba H, Hebiguchi T, Yoshino H, Mizuno M, Yamada M, Chihara J, et al . Evaluation of anorectal functions of children with anorectal malformations using fecoflowmetry. J Pediatr Surg 2002;37:623-8.  Back to cited text no. 30    
31.Yagi M, Iwafuchi M, Uchiyama M, Iinuma Y, Kanada S, Ohtaki M, et al . Postoperative fecoflowmetric analysis in patients with anorectal malformation. Surg Today 2001;31:300-7.  Back to cited text no. 31    
32.Rintala RJ. Anorectal malformations: An overview. In, Stringer MD, Oldham KT, Mouriquand PDE, Howard ER, editors. Pediatric Surgery and Urology: Long Term Outcomes. WB Saunders Co. Ltd., 1998. p. 357-75.   Back to cited text no. 32    
33.Hulthen de MV, Mellstam L, Amark P, Frenfner B. Neurovesical dysfunction in children after surgery for high or intermediate anorectal malformations. Acta Pediatr 2004;93:43-6.   Back to cited text no. 33    
34.Kumar A, Agarwala S, Grover VP, Mitra DK. Occult neurovesical dysfunction in children with anorectal malformations prior to anorectoplasty. Presented at the 30th National Conference of the Indian Association of Pediatric Surgeons, Jabalpur, 2004.   Back to cited text no. 34    
35.Jindal B, Bhatnagar V. Pre and post-operative urodynamic assessment in children with ano-rectal anomalies. Unpublished data (ongoing study), 2004.   Back to cited text no. 35    
36.Ditesheim JA, Templeton JM. Short-term v long-term quality of life in children following repair of high imperforate anus. J Pediatr Surg 1987;22:581-7.  Back to cited text no. 36    


Tables

[Table - 1]


This article has been cited by
1 Anorectal malformations: Definitive management during and beyond adolescence
Sham, M. and Singh, D. and Phadke, D.
Journal of Indian Association of Pediatric Surgeons. 2012; 17(3): 120-123
[Pubmed]



 

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    Abstract
    Clinical evaluation
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    Imaging
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    Plan of management
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