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REVIEW ARTICLE |
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| Year : 2005 | Volume
: 10
| Issue : 2 | Page : 80-85 |
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Assessment of postoperative results in anorectal malformations
V Bhatnagar
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
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0971-9261.16466
 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 |
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-à-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 | |  |
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 | | |
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 | | |
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 | | |
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 | | |
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-à-vis the muscle complex - these can also be corrected surgically. [18],[19],[20],[21]
| Ultrasonography | | |
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 | | |
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 | | |
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 | | |
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.
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Tables
[Table - 1]
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