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Early reported rectal sensation predicts continence in anorectal anomalies
Abstract
Background:
Straining at stool is an automatic reflex in babies and implies the presence of rectal sensation. We hypothesised that early reported rectal sensation would predict future continence in children with anorectal anomalies.
Aim of the study:
The aim of this study is to determine if early straining at stool was a useful predictor of future continence in infants born with high anorectal malformations.
Methods:
A retrospective case note review of prospectively collected clinical information was performed with institutional review board approval. All patients with intermediate/high anorectal malformation operated on by a single surgeon from 1984 to 2010 were included. After stoma closure, parents were asked: The responses were noted within the first year of stoma closure and then all patients were followed up until they were at least 3 ½years old and continence could be assessed using the Krickenbeck outcome classification. Data were compared using Fisher's exact test and sensitivity, specificity and positive predictive value (PPV) were calculated.
Main results:
Forty-eight patients were included in the study. Sixteen (33%) were female (12 cloacal malformation, 3 rectovaginal fistula, 1 rectal atresia) and 32 (66%) were male (6 rectovesical fistulae, 22 rectourethral fistulae, 4 no fistula). Median follow-up was 9.7years (range 3.5-17.9). Twenty-one children were noted by their parents to exhibit early straining at stool after stoma closure. Twenty of them achieved long term continence. The sensitivity of early straining as a predictor for long term continence was 77%, specificity 95% and positive predictive value 95%.
Conclusion:
The presence of early rectal sensation reported by parents is a good predictor of long term continence. This allows more informed discussion with families in the early years of life.
... Knowing the anatomic subtype of anorectal malformation along with the quality of the sacrum and the spine allows for an understanding of future continence, the key question on parents' and their family's minds. These factors, when considered, have improved the clinician's ability to explain things to families even in the newborn period [35]. For patients with Hirschsprung disease, surgery that preserves the anal canal and the sphincter mechanism should allow for voluntary bowel movements in the future. ...
... In most females, except for those with a cloaca, a primary operation without the need for a stoma, even in the newborn period, is possible. This decision must be based on the surgeon's experience and the clinical circumstances in which they find themselves [34][35][36]. In the case of Hirschsprung disease, a primary repair is usually possible, either in the newborn period or delayed for several months with the patient carrying out daily colonic irrigations at home. ...
The treatment of patients with colorectal disorders requires care from a wide variety of medical and surgical specialties over the course of their lifetime. This is ideally handled by a collaborative center which facilitates the assessment and development of patient care among multiple specialties which can enhance the quality and implementation of treatment plans, improve communication among different specialties, decrease morbidity, and improve patient satisfaction and outcomes. This collaborative approach can serve as a model for other parts of medicine requiring a similar multi-disciplinary and integrated method of care delivery. We describe the process, as well as the lessons learned in developing such a program.
... The self-control rate of patients with rectoperineal fistula was the highest and that of patients with cloacal ectopia was the lowest. 23 This is the same as most of the previous studies that the more complex and higher location ARM, the poorer postoperative bowel movement autonomy. [24][25][26][27] For low-type ARMs (recto-perineal fistula and rectovestibular fistula), the patients often have more severe constipation. ...
... (3) Lesions of the spinal cones, such as spinal cord embolism, could be a risk factor for postoperative FDD, because the spinal cones are also implicated in the regulation of defecation. (4) In addition, pre-sacral spinal bulges may also cause constipation by directly compressing the rectum.However, some studies have concluded that the spinal/spinal cord malformations and other related malformations have no significant effect on intestinal functions.3,23 Therefore, larger sample sizes or further studies are needed to determine the specific effects between other systemic malformations and bowel functions. ...
Anorectal malformation (ARM) is a common congenital gastrointestinal malformation in neonates, which has complex pathological changes and unclear etiology. The only treatment modality currently available for ARMs is through surgical treatment to correct the deformity of the anal morphology and restore normal defecation function. In addition, the mortality rates of children with ARMs have significantly decreased as a result of recent advances in medical technology. It is important to note that despite the treatment of perianal anatomical deformities, approximately one‐third of ARM patients continue to experience functional defecation disorder (FDD), which includes fecal incontinence and constipation and negatively impacts the patient's quality of life. There are still numerous questions regarding the exact cause of FDD in ARM patients following surgery. It is generally accepted that the occurrence of FDD may be related to clinical staging, structural changes in the perianal rectal muscles, anomalies of the enteric nervous system, surgical injury, etc. However, some of these factors are still controversial and require additional study to be confirmed. In this paper, we review the risk factors for FDD following surgery in ARMs, which will help us to subsequently develop appropriate interventions to enhance the anal function and improve quality of life of children with ARMs.
... Skerritt et al. looked into "straining" as an early predictor of continence in ARM patients. [82]. They hypothesized that straining during defecation in early period after reconstruction of ARM was an indicator of rectal sensation. ...
... Twenty of them achieved long-term continence. They concluded that early straining was a predictor for long-term continence with a sensitivity of 77%, specificity of 95%, and positive predictive value of 95% [82]. ...
The introduction of posterior sagittal anorectoplasty (PSARP) by deVries and Peña in the early 80s has impacted to the treatment of patients with anorectal malformations (ARM). It gained great recognition worldwide in a very short time, and since then, surgeons dealing with the treatment of this complex malformation could achieve tremendous progress in contemporary management of this anomaly. Despite the growing experience and body of information globally, the treatment of ARMs continues to be a challenge to the pediatric surgeons due to the nature and the variability of the anomaly, and short- and long-term problems continue to exist even after nearly 40 years of the PSARP era. Today, knowing more about it, pediatric surgeons are committed to do more for their ARM patients to have them as physically and socially healthy individuals.
A basic premise in the care of complex patients is that experience, increased volume of cases, and an integrated, multi-disciplinary approach yields improved outcomes. Is this true using the example of the care of children with colorectal and pelvic reconstructive needs? This review gives a brief historical context on how care for this patient group evolved, delineates the key elements to create a collaborative care model, and describes multiple advances that have been developed, based on the model, which have improved patient care and quality of life.
LEVEL OF EVIDENCE
Review
Caring for children with colorectal and pelvic reconstructive needs is complex and requires a lifelong commitment from clinicians devoted to the field. There has been a myriad of advances that have improved care and it has become clear that an integrated, multi-disciplinary approach maximizes the goal of improving the quality of life of children afflicted with these conditions. The purpose of this review is to briefly discuss the history of this field and to describe the key advances that have improved patients’ lives.
This is a brief presentation of the most common types of anorectal malformations. It has been written for the practicing pediatrician. It is meant to be a practical guide to identify and manage these pediatric problems from birth until adult life. It emphasizes the importance of suspecting the associated defects as well as to try to avoid the most common errors observed in the management of these malformations. It also includes information that will allow the clinician to establish the functional prognosis for each specific types of defect. The management of the sequelae is considered an important part of this chapter.
This is a brief presentation of the most common types of anorectal malformations. It was written for the practicing pediatrician. It meant to be a practical guide to identify and manage these pediatric problems from the newborn until adult life. It emphasizes the importance of suspecting the associated defects, as well as to try to avoid the most common errors observed in the management of these malformations. It also includes information that will allow the clinician to establish the functional prognosis for each specific types of defect. The management of the sequelae is considered an important part of this chapter.
To determine the anorectal function in patients with anorectal malformations (ARM) in order to facilitate patient counseling and follow-up.
Data were collected by the German network for urorectal malformations (CURE-Net) according to the International Krickenbeck consensus. Questionnaires on bowel function and a defecation protocol were completed by the families/patients. The clinical findings were assessed from the patients' clinical records.
Two hundred and ninety-seven patients with ARM were assessed, 175 patients gave complete data on continence, 52 of them were excluded due to mental retardation, age, and earlier type of pullthrough. Complete continence was found in 27 %, perineal fistula in 40 %, rectourethral/vesical in 10 %, vestibular in 24 %, cloaca in 0 %. Krickenbeck grade 1 soiling: 42 %, grade 2 and 3: 31 %. Forty-nine percent of the incontinent patients practiced bowel management, reaching continence in 19 %. The statement of constipation (67 %) was validated with the last clinical findings, showing coprostasis in 46 %, "Not suffering constipation" was confirmed in 61 % and falsified in 29 %.
ARM patients in Germany, as assessed by independent researchers, show a high rate of fecal incontinence and insufficiently treated constipation. Parents should be counseled accordingly and motivated to engage in consequent follow-up. Intensified efforts in the conservative treatment of constipation and fecal incontinence are crucial to improvement.
Anorectal malformations (ARM) are common congenital anomalies seen throughout the world. Comparison of outcome data has been hindered because of confusion related to classification and assessment systems.
The goals of the Krinkenbeck Conference on ARM was to develop standards for an International Classification of ARM based on a modification of fistula type and adding rare and regional variants, and design a system for comparable follow up studies.
Lesions were classified into major clinical groups based on the fistula location (perineal, recto-urethral, recto-vesical, vestibular), cloacal lesions, those with no fistula and anal stenosis. Rare and regional variants included pouch colon, rectal atresia or stenosis, rectovaginal fistula, H-fistula and others. Groups would be analyzed according to the type of procedure performed stratified for confounding associated conditions such as sacral anomalies and tethered cord. A standard method for postoperative assessment of continence was determined.
A new International diagnostic classification system, operative groupings and a method of postoperative assessment of continence was developed by consensus of a large contingent of participants experienced in the management of patients with ARM. These methods should allow for a common standardization of diagnosis and comparing postoperative results.
Background/purpose
Posterior sagittal anorectoplasty (PSARP) has become the standard surgical treatment for males rectourethral fistula (RUF) at most surgical centers worldwide. We aimed to define the long-term bowel functional outcomes following PSARP for RUF at our institution between 1983 and 2006, with comparison to age- and gender-matched controls.
Methods
Patients were invited to answer a detailed, previously validated Bowel Function Score (BFS) questionnaire by post. Respondents were matched by age and gender to three controls from the general population who had answered identical questionnaires. Case records were reviewed retrospectively for operative details. Ethical approval was obtained. Social continence was defined as soiling or fecal accidents < 1/week and no requirement for changes of underwear or protective aids.
Results
Of 34 (79%) respondents (median age 19 (range, 4–29) years), 74% had voluntary bowel movements (VBMs), 24% were reliant on anterograde continence enema (ACE) washouts, and 1 patient had a colostomy. Impairment of bowel function was significantly higher in all aspects of fecal control among patients than controls (p < 0.001). A statistically significant decline in fecal accidents and soiling was observed with age (p ≤ 0.03). Thirty-one percent of patients with VBMs had constipation managed with diet or laxatives (vs 2% of controls, p = 0.0002). Of patients with VBMs followed up for > 12 years (n = 20), 50% were completely continent (vs 73% of controls; p = NS). Overall, 76% of respondents were socially continent with or without artificial means in the form of ACE washouts. By BFS score, 39% had a good functional outcome, 27% had a moderate outcome, 9% had a clearly poor score and 24% were living with an ACE.
Conclusions
Our results suggest that in the long-term, functional symptoms remain highly prevalent among patients treated for RUF with PSARP. However, the majority can be expected to achieve social continence, although for some this will require intervention with ACE bowel management. Approximately one third may report VBMs and complete continence.
Purpose:
Longitudinal follow-up of changes in bowel function in children with anorectal malformations (ARMs) with or without spinal cord pathology and neurogenic bladder dysfunction (NBD) as they grow. Another purpose was to identify predictors influencing bowel functional outcome.
Material and methods:
The study included 41 patients with ARM, excluding perineal fistula (21 boys and 20 girls). Bowel function was evaluated at ages 5, 10 and 15 years using a structured questionnaire and a three-week registration of number and time of bowel movements, episodes of fecal leakage and soiling. Additional bowel treatment with enemas and stool softeners and use of diapers were recorded. A group of 52 healthy boys and girls was used as control.
Results:
A successive improvement in functional outcome with age in children with ARM and normal spinal cord was seen with respect to continence, soiling and constipation. Continence was achieved earlier in girls than in boys (at 10 years: girls 80%, boys 36%). Soiling and constipation decreased with age both in grade and frequency (at 10 years low grade soiling: girls 53%, boys 64%). Boys with spinal cord malformation with NBD in combination with prostatic/bladder neck fistula (PRF/BNF) and sacral agenesis had the worst functional outcome with minimal possibility of improvement over time. Functional outcome in girls with NBD and tethered cord did not differ significantly from those without NBD and with a normal spinal cord. Psychosocial co-morbidity, neuropsychiatric disorders, developmental delay and megarectosigmoid were also risk factors impeding the functional outcome.
Conclusion:
In the present study there was a successive improvement in bowel function during childhood and adolescence in ARM children, but they did not achieve the level of healthy children. NBD, spinal cord malformation, sacral malformation and PRF all were negative predictive factors for bowel score at 5 years.
Limited information exists on what constitutes normal bowel function in children and adolescents. This information is essential for determining outcomes of surgery for anorectal malformations and other colonic disorders.
The aim of this study was to define bowel function and fecal continence in a large sample of individuals aged 4 to 26 years.
This investigation is a cross-sectional cohort study.
Five hundred ninety-four individuals aged 4 to 26 years were randomly selected from the population register of Finland. Participants anonymously answered a previously validated 7-item postal questionnaire on bowel function.
The primary outcomes measured were bowel function score, fecal continence, stooling frequency, constipation, and social problems in relation to bowel function.
Recognition of the need to defecate was well established by age 4, but the ability to hold back defecation improved with age. Fecal accidents decreased from 11% in preschool children to 2% by 13 to 17 years (p = 0.02). Fecal staining of underwear was common (33%); the prevalence of soiling decreased with age. Stooling frequency was normal in 92%. Constipation was more common in females. Problems restricting social life in relation to bowel function were rare (0.5%).
The fine-tuning of fecal continence continues to develop during childhood. Minor soiling is common in healthy individuals. Because of the changes with age, the evaluation of functional outcomes in patients who have undergone surgical treatment of anorectal malformations or other colonic disorders should include comparison with data from age-matched controls.
Constipation in anorectal malformations (ARM) is extremely common, particularly in the lower types. Failure to adequately treat it can lead to significant morbidity.
From our series of over 2000 patients with ARM, we reviewed 398 with good prognosis for bowel control and a tendency toward constipation; rectoperineal fistula (63), rectovestibular fistula (114), rectobulbar urethral fistula (104), imperforate anus with no fistula (46), rectal atresia or stenosis (9), and cloaca with a common channel below 3 cm (62). Those lost to follow-up, not yet toilet-trained (<3 years old), or with poor prognostic features were excluded. We compared morbidities in patients we operated on and managed primarily (group A, n = 268) to those managed at other institutions who suffered from constipation or incontinence and were referred to us for treatment (group B, n = 130). Those we managed primarily were subjected to an aggressive senna-based laxative program, started after their primary repair or after colostomy closure.
Morbidities associated with constipation were higher in the referral group and included fecal impaction (7.8% vs 38.5%), overflow pseudoincontinence (4.9% vs 33.8%), and megacolon (14.6% vs 54.6%). A loop or transverse colostomy (4.9% vs 9.2%), stoma or anorectal stricture, or a stenotic fistula (2.2% vs 28.5%) were contributing factors. Adequate laxative treatment with, in certain cases, resection of a megarectosigmoid (2.6% vs 23.1%) enabled many pseudoincontinent children to achieve bowel control (reported previously). Unneeded colorectal biopsies (1.9% vs 16.2%), Hirschsprung's-type pullthroughs (0% vs 3.1%), and, in retrospect, unneeded antegrade continent enema procedures (0% vs 3.1%) were higher in Group B. Overall, 19.8% of Group A and 66.2% of Group B experienced constipation-related morbidities.
The morbidity of constipation in ARM includes fecal impaction, megacolon, incontinence, and performance of unneeded surgeries. Inadequate treatment, the type of the original colostomy, and postoperative anal or stomal stricture as well as stenotic fistulae were key contributing factors. Children with ARM and good prognosis for bowel control are at the greatest risk for severe constipation and its consequences. With recognition and aggressive, proactive treatment, we have found that these morbidities can be reduced.
Purpose:
Megarectum complicating surgery for anorectal malformation (ARM) has implications for long-term continence. Factors influencing continence and defecation include intact rectal reservoir, innervation/proprioception of the anorectal muscle complex, functioning anorectal inhibitory reflex (AIR), and intact perception at the anal margin. We studied outcomes after surgery for ARM with emphasis on megarectum; particularly as to whether altered rectal proprioception from anatomic sacrococcygeal anomalies affect incidence. We also assessed whether an abnormal AIR could trigger passive rectal dilatation without mechanical obstruction.
Methods:
Eighty six infants (53 male) with ARM over 20 years were included. Demographics, surgical history, pathology, defecation patterns, imaging, manometry, and morbidity were analyzed. Incidence of sacrococcygeal malformations in children with and without megarectum was compared using Fisher exact test. Manometry results were evaluated for integrity of AIR and correlated to megarectum occurrence.
Results:
There were 23 high/intermediate and 63 low ARMs. Fourteen (16%) developed a megarectum: 6 of 23 in high and 8 of 63 in low anomalies (P = .33). Twelve patients underwent megarectum resection at a median of 2.6 years (7 months to 10 years); 2 received bowel management protocols. Fifty-seven percent (8/14) of children with and 7% (5/72) without megarectum had sacrovertebral anomalies (P = .0001). Patients with preoperative manometry (n = 5) demonstrated an intact AIR. Colonic manometry demonstrated hyperactive colons (n = 2). Constipation was the predominant preoperative symptom; 3 patients suffered from incontinence after resection. All the specimens showed normal innervation and thickened muscularis on pathology.
Conclusions:
Sacral anomalies, which are more prevalent in children who developed megarectum, may result in abnormal rectal proprioception contributing to this pathology. Innervation anomalies may coexist, although preoperative manometries showed normal AIRs. Rectal dysmotility may lead to stool retention with subsequent dilatation, and patients who underwent colonic manometry had diffuse colonic hypermotility. Further physiologic and cellular studies are needed to elucidate the causes of this significant complication after surgical ARM repair in the absence of obstruction.
Anorectal malformations (ARMs) and Hirschsprung disease (HD) are the most common congenital colorectal defects in the newborn. The outcomes of HD and ARMs have improved significantly because of improved understanding of the pathologic anatomy and physiology of these defects and of the modern surgical techniques. Still, many patients suffer from defective bowel control even as adults. Some of these also have problems with urinary control and sexual functions. The functional problems are more pronounced in patients with ARMs. Compared with healthy people, both patients with ARMs and those with HD have limitations in their quality of life. Inferior quality of life is more common in patients with ARMs. There are very few published data on long-term outcome of adults with ARMs and HD. The effect of aging on the functional outcome and quality of life remains unclear, although some preliminary data suggest that the bowel function and quality of life may deteriorate with aging.
Anorectal malformations (ARMs) affect 1 in 4000 to 5000 births. The Krickenbeck conference developed a classification based on anatomical and functional criteria to better compare treatment outcome.
The aim of this study is to evaluate the functional outcome in patients 10 years following standardized surgical treatment of ARM related to the Krickenbeck classification. .
Anatomical anomalies were classified as above. Children and carers were followed closely in a multidisciplinary clinic. Data were collected using a functional outcome questionnaire for a minimum of 10 years after surgical reconstruction. Outcome measurements were related to the Krickenbeck classification.
There were 53 children in the study group (29 male, 24 female). Krickenbeck anatomy: perineal fistula, 36%; vestibular fistula, 26%; rectourethral fistula, 36%; rectovesical fistula, 2%. All children were treated by posterior sagittal anorectoplasty. In children with perineal fistula, continence was achieved in 90%. Grade 2 constipation was noted in 21%. One child had a Malone antegrade continence enema (MACE) procedure. In children with vestibular fistula, continence was achieved in 57%. Grade 3 constipation was noted in 28%. One child had grade 1, and one child had grade 2 soiling. Two children had a MACE procedure. In children with rectourethral fistula, continence was achieved in 58%. One child had grade 3 soiling. Grade 3 constipation was found in 42% of children and grade 2 constipation in 1 child. A MACE procedure was performed in 36%. The only child with a bladder neck fistula had a MACE procedure for intractable soiling.
The outcome for patients with ARM is related to the severity of the anomaly. The uniform application of the Krickenbeck classification should allow rational comparison of treatment outcome.
From October 1980 to November 1981, 34 patients with anorectal anomalies have been operated upon by a sagittal midline approach. The skin incision extends from the sacrum to the perineum (ventral aspect of the anal dimple). The superficial and deep layers of the external sphincter are identified by electrostimulation and split, with the coccyx, in the midline. Ileo- and pubococcygeal portions of the levator dorsally and then the striated muscle complex of the external sphinctor, pubococcygeus and the presumed puborectalis are split ventrally to the urethra. In no cases has the ventral portion of the levators been separated from the thick ventral portion of the external sphincter, hence the term "striated muscle complex." When the terminal bowel is dilated (congenitally ectatic), the bowel is tailored prior to reconstruction of the sphinctors. The posterior sagittal approach provides an excellent exposure for evaluation and mobilization of the terminal bowel. It enables one to construct an anal canal, suture the bowel wall to the striated musculature and the mucosa to the skin, thereby reducing or avoiding the complications of prolapse and stenosis. In males with ectasia and a rectourethral fistula, transrectal closure of the mucosa at the fistula site, leaving the rectal longitudinal smooth muscle insertions on the prostatic capsule, avoids damage to the nerves and genital structures.
Parents play a crucial role in the life of a child suffering from an anorectal malformation (ARM), since their guidance contributes to the degree to which the child learns to cope with his or her disability. We investigated whether they experience stress in parenting such a child and also attempted to identify somatic or behavioral characteristics in the child that influence the stress of parenting. The parents of 109 children (69 males, 40 females; median age 5.9 years, range 1-18 years) with an ARM (58 low, 10 intermediate, 41 high) were studied. The Nijmegen Questionnaire on Child-rearing Situations (NQCS) was used to investigate the existing parenting situation. Behavioral characteristics of the children were studied by means of the Child Behaviour Checklist (CBCL) and the Teacher Report Form (TRF). In a semi-structured interview, we investigated how parents experienced the implications of the disability in everyday life with their child. Our study showed that as far as the perception of parenting stress is concerned, parents of children with an ARM do not differ from those with healthy primary-school children. Within the group of parents with ARM-afflicted children, the parents of older, incontinent children experienced relatively more stress, especially when the child concerned was male. With regard to the children's behavior, the parents and teachers under investigation did not report a higher than normal incidence of deviant behavior. However, when individual parents observed difficult behavior in their child, they found it harder to deal with than the incontinence for feces. Regarding the implications of the disorder for their everyday lives, parents were concerned and indicated a need for specific counselling. We conclude that having a child with a somatic affliction, in this case an ARM, does not automatically imply that the parents experience child-rearing problems. However, certain groups of parents are more at risk, i.e., parents with older, incontinent sons and parents with children exhibiting behavioral problems. In addition, our study shows that parents do have difficulties in coping with the implications of the disorder and express a need for support. We feel that patient care can be improved if aid is tailored to these specific problems.
It is unclear which surgical method offers best long-term functional results in patients with high anorectal anomalies. The purpose of this study was to compare the long-term outcome of sacroperineal-sacroabdominoperineal pull-through (SP-SAP) to that of posterior sagittal anorectoplasty (PSARP).
Only boys with high anorectal anomalies (rectourethral fistula) were included in the study to get fully comparable patient groups. From 1975 to 1987, 36 consecutive patients underwent anorectal reconstruction: 19 had SP-SAP (1975 to 1983) and 17 PSARP (12 with internal sphincter-sparing technique, 1983 to 1987). The late bowel function (age at follow up, SP-SAP, 19 years; range, 15 to 22; PSARP, 13 years; range, 10 to 19) was evaluated by clinical interview and examination, and anorectal manometry.
Six (35%) of the PSARP patients and one (5%) of the SP-SAP patients (P < .04) were always clean without any adjunctive measures. Three PSARP patients and two SP-SAP patients stayed clean with daily enemas. In the PSARP patients with soiling, the median frequency of soiling episodes in a month was four (range, 1 to 16), in the SP-SAP patients, 20 (range, 2 to 28, P < .001). None of the SP-SAP patients but 8 of 17 of the PSARP patients had constipation requiring diet or oral medication. Two PSARP patients and four SP-SAP patients had occasional faecal accidents. The median daily bowel movements in the PSARP group was one (range, one to four) and in the SP-SAP group, three (range, one to five, P < .001). The PSARP patients had significantly higher anorectal resting and squeeze pressures and voluntary sphincter force (cm/H2O, PSARP: mean resting, 47+/-9; mean squeeze, 106+/-29; mean voluntary sphincter force, 60+/-22; SP/SAP: mean resting, 27+/-10; mean squeeze, 68+/-22; mean voluntary sphincter force, 41+/-17; P < .01). Thirteen (76%) of the 17 PSARP patients and none of SP-SAP patients had positive rectoanal reflex indicating functional internal sphincter.
In boys with high anorectal anomalies, PSARP clearly is superior to sacroperineal and sacroabdominoperineal pull-through in terms of long-term bowel function and faecal continence.
Constipation is a major complication in patients who have undergone posterior sagittal anorectoplasty (PSARP) operation for a high anorectal malformation. Overflow incontinence is the main cause of fecal soiling in these patients. The aim of this study was to outline the natural history of constipation in patients with high anorectal malformations and relate this to the functional outcome at the end of the patient's growth period.
The study group consisted of 22 pubertal or postpubertal patients (median age 15; range, 13 to 25) with high or intermediate anorectal malformations repaired by PSARP procedure. The patients have been followed-up since birth. Constipation was defined as a need to use medical treatment or diet to ensure bowel emptying. Continence was classified as follows: grade 1, no soiling in any circumstances; grade 2, staining less than once a week, no fecal accidents; grade 3, staining more than once a week, no fecal accidents; grade 4, daily soiling or accidents, need for regular enemas, or the antegrade colonic enema procedure. All patients underwent anorectal manometry and magnetic resonance imaging of the spine and spinal cord.
At the time of the study 2 (9%) of the 22 study group patients had constipation, but 15 (68%) had been constipated before puberty. Eleven patients (50%) were fully continent (grade 1) without constipation. Six of those had a history of constipation associated soiling. Three patients (14%) had occasional staining (grade 2) and no constipation. Two of them had been constipated with significant soiling before the onset of puberty. In the 5 (22%) patients with frequent staining (grade 3) the degree of soiling had decreased after the disappearance of constipation. Two of the 3 patients with poor outcome (grade 4) require regular enemas for recalcitrant constipation. Spinal cord anomalies were detected in 4 and abnormal sacrum in 15 patients. Of the anorectal manometric parameters, only the force of voluntary sphincter squeeze correlated with the functional result.
In the majority of patients who underwent PSARP procedure for high anorectal malformation, constipation disappears at adolescence, and this is associated with improved fecal continence outcome.
Megarectum in association with anorectal malformation contributes to chronic constipation and fecal incontinence. Resection of megarectum in anorectal malformation improves bowel function, but neuropathy and poor sphincter quality may affect the outcome of fecal continence adversely. The aim of this study was to evaluate the benefits of resection of megarectum in anorectal malformation and to ascertain the impact of anal sphincter quality and neuropathy on the outcome.
We studied 62 children with intractable fecal incontinence after repair of anorectal malformation between January 1991 and January 2005. All patients were investigated with anorectal manometry and anal endosonography under ketamine anesthesia. On endosonography, an intact or scarred internal anal sphincter (IAS) was classified as good and a fragmented or absent IAS as poor. On manometry, a resting anal sphincter pressure equal to or more than 30 mm Hg was classified as good and a lower pressure as poor. Functional assessment of fecal continence was done before and after excision of megarectum using a modified Wingfield scores.
Sixteen children had excision of megarectum with median age of 9 years (range, 2-15 years) and postoperative follow-up of 5 years (range, 1-10 years). Seven had formation of antegrade continent enema stoma before excision of megarectum. Children were classified into three groups of anomalies: low (n = 6), intermediate (n = 4), and high (n = 6). All children were incontinent of feces. After excision of megarectum, of the 9 children with good IAS and no neuropathy, 7 became continent of feces. Of the remaining 7 children, 4 had poor IAS and 3 had neuropathy, 5 of whom required an antegrade continent enema stoma to be clean.
Excision of megarectum in children who had previous repair of anorectal malformation results in fecal continence in the presence of a good IAS and absence of neuropathy. Patients with a poor IAS or neuropathy will often require artificial means of fecal continence.