A computerized vector manometry and MRI study in children following posterior sagittal anorectoplasty

The study was designed to evaluate computerized eight-channel vector manometry (8CVM) and pelvic floor magnetic resonance imaging (MRI) as methods to assess the anal sphincter following posterior sagittal anorectoplasty (PSARP) for anorectal malformations, in particular the functional capacity of the sphincter structures in correlation with postoperative MRI findings. Seventeen children had been operated upon for a rectovesical, -urethral, or -vaginal fistula including one female cloacal malformation; 4 had a secondary PSARP. Mean follow-up was 5.57 years. Continence was evaluated with a modified Kelly score. A CVM technique with an eight-channel perfusion catheter was used. In addition to software-supported data, the manometric parameters included a score-system assessing three different pressure zones of the anal canal qualitatively on the three-dimensional image of the anal sphincter profile. The same procedure was performed on sagittal, oblique axial, and oblique coronal MRI. Furthermore, the thickness of the sphincter muscle was assessed at the level of the maximal mean segmental pressure. All children had decreased absolute vector-volumetry values at rest and on squeezing. Correlation with the clinical score was poor. Correlation of the manometric score with the clinical course was similar to the correlation of MRI score with clinical course (R = 0.425; P = 0.1). Thirteen children demonstrated normal or increased sphincter length; 5 of these had a decreased high-pressure zone (HPZ). The position of the anorectum in the sphincter muscles could be evaluated by the vector-volumetry image as anatomic in 11 cases, nearly correctly positioned in 4, and ectopic in 1 child. MRI detected 2 cases of malposition, 10 anatomic, and 4 nearly-anatomic findings. Correlation of the manometric score with the MRI score and the thickness of the sphincter muscle at the HPZ was significantly high (R = 0.801; P < 0.0001). 8CVM is thus highly sufficient in illustrating the function of the sphincter musculature seen on pelvic floor MRI. Both methods only moderately reflect clinical follow-up, since continence depends on more than sphincter ability. Accepted: 9 March 2000     

Perineal canal : An uncommon entity with good prognosis

This article reports two patients with perineal canal (anovestibular fistula) who were treated at Dept. of Pediatric Surgery, LTMG Hospital, Mumbai. Both the patients had normal anus and a fistula between the anal canal and the vestibule. They were treated by vestibulo anal pull through procedure. The pathogenesis of this condition and the pertinent literature is reviewed.     

Anorectal manometry in newborns

Anorectal manometry was performed in 32 neonates: 18 pre-term and 14 full-term infants, mean ages 6.1 and 7.1 days, respectively. The rectoanal inhibitory reflex (RAIR) was positive in 31 cases, and the relaxation waves produced by rectal distention were recorded along the anal canal and were directly related to the intensity of the stimulus. In the remaining patient the RAIR was negative due to Hirschsprung's disease. The length of the anal canal was 11.3 ± 2 mm in pre-term and 14.7 ± 2.9 mm in full-term infants (P < 0.001). These findings suggest that the presence of a RAIR is independent of the weight, gestational age, and birth age of the infant, and that anal canal length correlates with weight. We conclude that anorectal manometry in the neonatal period is a simple, reliable, and safe method of testing anorectal function, including the diagnosis of Hirschsprung's disease.     

Anorectal anomalies: anorectal manometric function and anal endosonography in relation to functional outcome

To assess the relation between continence and the manometric and endosonographic state of the anorectal segment after surgery for anorectal anomalies (ARA), 33 adolescents operated upon for ARA and 14 controls were examined. Seventeen patients had low and 16 intermediate or high ARA. Fecal continence was recorded, and anal canal manometry was performed by microtransducer. The sphincter muscle complex and its relation to the anal opening was visualized by anal endosonography (ES). Ten patients had perfect continence, 10 had staining, and 13 had soiling. The anal canal resting and squeeze pressures were significantly different in all three groups, and continence function was significantly correlated to anal canal pressures. By anal ES, the internal (IAS) and the external anal sphincters (EAS) were identified with various amounts of scar tissue in all patients. In patients with high or intermediate anomalies the IAS was missing in the lower part of the anal canal, and abundant scar tissue was identified in the EAS in all patients. In patients with good continence function, the EAS was better preserved than in patients with major incontinence. The position of the anus in the EAS muscle complex was assessed, and varying degrees of .eccentrically placed anal canals were identified. Continence function after surgery for ARA is thus correlated to anal canal pressures and ES images. ES, which is painless and suitable for use in children, is a valuable tool for assessing perianal structures, and the findings may serve as a helpful guide for corrective surgery.     

Experimental model for study of anorectal sphincter musculature by manometry and computerized tomography in piglets

There seems to be controversy on the anorectal sphincter presentation and anatomical division, as well as on its functional representation. Evaluation of the anorectal sphincter musculature has been achieved through several methods, including anorectal manometry and computerized tomography, but to date there is no experimental model allowing a detailed manometric study of this muscle complex. In this work, we have developed such a model, which should enable the manometric and radiographic study of the anatomical features and functional mechanisms of sphincteric injuries, as well as the assessment of drug effects on the anorectal musculature upon incontinence and constipation. Twenty-two piglets (aged 25–30 days, weighing 5–7 kg) were studied by anorectal manometry (rectoanal inhibitory reflex and vector volume) and computerized tomography (anorectal angle and anal canal length). The data obtained for the rectoanal inhibitory reflex, represented here as the average and standard deviation, were the following: relaxation duration = 14.75 ± 3.62 s, sphincter basal pressure = 41.58 ± 8.20 mmHg, relaxation index = 87.26 ± 11.52%, speed of relaxation = 5.90 ± 2.10 mm/s, and speed of relaxation recovery = 4.03 ± 1.78 mm/s. As for the vector volume, results were as follows: vector volume = 2692.32 ± 1298.12 mmHg2 cm, sphincter length = 11.82 ± 2.74 mm, high pressure zone length = 5.09 ± 1.34 mm, maximum pressure = 61.50 ± 20.58 mmHg, and asymmetry index = 43.50 ± 10.03%. Radiographic evaluation led to the following results: anal canal length = 9.61 ± 2.14 mm and anorectal angle = 137.91 ± 7.75°. The experimental model designed here allows both anorectal manometry and computerized tomography to be carried out in the same way it is performed in human beings, as long as animal sedation is strictly controlled.     

Internal anal sphincter function after correction of imperforate anus

Thirteen patients with high or intermediate types of imperforate anus have been followed postoperatively by anorectal manometry after surgical correction. Since it is our belief that an internal sphincter anlage is present in the fistula, this was preserved and transplanted to the position of the neoanus. In cases with a blind end and no fistula, thickening of the circular smooth muscle layer was sought and transposed in the same way. Ten of 13 patients had a positive rectoanal inhibition reflex and normal anal pressure, indicating the presence of an internal sphincter. The amplitudes of the relaxations were not different from those of healthy children of the same ages. The children in this study were too young for proper clinical evaluation, but internal sphincter function may be an important factor for their future continence. Offprint requests to: B. Frenckner     

Magnetic resonance images of 91 children with different causes of short stature: pituitary size reflects growth hormone secretion

In order to validate an association between pituitary size and severity of growth hormone deficiency (GHD) we evaluated the magnetic resonance images (MRI) of 107 children with different causes of short stature. Ninety-one MRIs were evaluable (64 male, 27 female; age: 9.1 ± 3.9 years). The levels of insulin-like growth factor-1 (IGF-1) and insulin-like growth factor binding protein-3 (IGFBP-3), and tests of GH stimulation and spontaneous secretion, led to the following sub-groups: severe isolated GHD (SIGHD) (GH < 7 ng/ml) (n = 21); partial, isolated GHD (GH 7–10 ng/ml) (n = 22); multiple pituitary hormone deficiency (MPHD) (n = 13); neurosecretory dysfunction (n = 10); non-classifiable diagnosis (NC) (n = 13); idiopathic short stature (n = 9); and intra-uterine growth retardation (n = 3). Pituitary height (PHT) was measured and hypoplasia was assumed when PHT was <−2 SDS. An ectopic posterior pituitary with missing stalk and a hypoplastic anterior pituitary was present in 12 (57%) SIGHD cases, 12 (92%) MPHD cases and 1 patient from the NC group. An isolated hypoplastic anterior pituitary was observed in 15%−33% of the other groups. PHT (mm; mean, SD) in MPHD (1.7 ± 0.5) was lower than in SIGHD (2.7 ± 1.0, P < 0.05), with PHT of both groups being lower than in all the other groups (3.8 ± 0.9, P < 0.0001). PHT SDS correlates with IGF-I SDS (r = 0.48, P < 0.0001), IGFBP-3 SDS (r = 0.46, P < 0.0001) and the highest peaks in tests of GH stimulation and GH spontaneous secretion (r = 0.36, P < 0.0001). In contrast to all the other groups, no correlation with age was observed in MPHD and SIGHD. Breech delivery was recorded in up to 26% of patients in all seven groups. Surprisingly, only 1 out of 23 patients with an ectopic posterior pituitary was born by breech delivery, suggesting that ectopia of the posterior lobe is not necessarily related to breech delivery. Conclusion PHT is significantly correlated with GH secretion in several types of short stature. Patients with␣ectopic posterior pituitary, missing stalk and hypoplastic␣anterior pituitary either suffer from SIGHD or MPHD, and this anatomical defect is not necessarily related to breech delivery. Received: 1 December 1996 and in revised form: 8 February 1997 / Accepted: 18 February 1997     

Three-flap anoplasty for imperforate anus: results for primary procedure or for redoes.

Three-flap anoplasty was first described in 1987 by Yazbeck for the treatment of rectal prolapse after pull-through operations for imperforate anus, and in 1992 in a case of anterior perineal approach. It is intended to reproduce the normal anatomy of a sensitive anal canal. The purpose of this study is to evaluate 14 children (9 boys and 5 girls) operated for imperforate anus. METHOD: Eleven children had an intermediate or high imperforate anus and 3 had a low imperforate anus. Nine were operated for the first time with a three-flap anoplasty (GrA), and 5 were reoperated with this technique because of fecal incontinence and/or rectal prolapse (GrB). Seven patients had associated malformations: one Hirschsprung's disease, one cloacal defect with renal insufficiency, three complex caudal malformations with tethered cord, one Down syndrome, and two psychological and psychomotor troubles. The patients (average age = 6 years) have been evaluated every year since 1997, with a questionnaire based on a clinical score validated with 30 healthy children, used as a control group. Ten anal manometries were carried out. RESULTS: In 1998, the GrA score was 16.1 (control = 22.5) and the GrB score was 11.5 (p = 0.25). In 1999, GrA and GrB score were approximately the same. The score of those without associated anomalies was 19.6 whereas the score of the children with other malformations or anomalies was 10 (p = 0.02). Anal manometry is almost normal in patients with low or intermediate imperforate anus (rectoanal relaxation reflex for 10 cm H2O, and basal resting pressure more than 40 cm H2O). Even though anal manometry is subnormal in patients with Down syndrome or psychomotor troubles, the clinical score remains low (score = 10). In cases of complex caudal malformations or high imperforate anus, the results of anal manometry and clinical score are bad (score 9.7). CONCLUSION: The three-flap anoplasty can be carried out in newborns without colostomy and often represents the only possible surgical approach to avoid a laparotomy. This plasty, proposed secondarily to correct a defect of continence, can be performed without colostomy, with satisfactory results.     

Role of anal dilatation in treatment of idiopathic constipation in children: long-term follow-up of a double-blind randomized controlled study

Constipation in childhood is a common symptom, with an estimated incidence between 0.3% and 8%. Most of the evidence for the current management of constipation and fecal soiling in children is based on reports of nonrandomized retrospective trials. Anal dilatation has had an established role in the management of idiopathic constipation but has never been evaluated by a randomized study. A double-blind randomized controlled trial was done of children who failed to respond to medical treatment and were admitted for investigation and treatment of idiopathic constipation to Guys Hospital, London, between April 2001 and April 2003. All children had intestinal transit study on admission. They were randomized, using a computer-generated allocation in sealed envelopes, to receive no anal dilatation (control group) or anal dilatation (anal dilatation group). Anorectal manometry and endosonography were done under ketamine anesthesia followed by anal dilatation if necessary under the same anesthesia. Disimpaction of feces from the rectum was done at the end of the procedure under general anesthesia using propofol muscle relaxant to minimize stretching of anal sphincter muscles in the control group. All children had intensification of medical treatment, toilet training, and monitoring of their response to treatment during their hospital stay, which ranged from 3 to 5 days. Outcome was measured using a parents questionnaire of symptom severity at 3 and 12 months of follow-up by one of the authors, who was blinded to randomization. The symptom severity score ranged between 0 and 65 and consisted of scores for the following: delay in defecation (score range 0–10), difficulty and pain with passing stool (0–5), soiling problem (0–10), intensity of laxative treatment (0–10), childs general health (0–5), behavior related to the bowel problem (0–5), overall improvement of symptoms (0–12,) and assessment of megarectum on abdominal examination (0–8). Of 60 neurologically normal children, 31 (19 males) were randomized in the control group and 29 (18 males) in the anal dilatation group. All children had findings consistent with idiopathic constipation and positive anorectal reflex on manometry, no anal sphincter damage on endosonography, and no anal fissure on examination under anesthesia. The median age for control and anal dilatation groups was 7.97 (range 4.1–14.25) years and 7.78 (4–13.25) years, respectively. Both groups were also comparable with regard to median of duration of laxative treatment (32 months vs. 31.5 months), internal anal sphincter thickness on endosonography (0.90 mm vs. 0.80 mm), resting anal sphincter pressure on manometry (51 mmHg vs. 51 mmHg), total rectal capacity on manometry (260 mmHg vs. 260 mmHg), and total symptom severity score before admission (33 vs. 29), respectively. At 12-month follow-up, the median pre-admission symptom severity score had improved significantly, from 33 (range 12–49) in the control group and 29 (16–51) in the dilatation group to 15 (0–51, p<0.0001) and 19 (1–46, p<0.0001), respectively. There was no significant difference between the two groups with regard to symptom severity score improvement at 12-month follow-up (p<0.92). We found a significant correlation between total rectal capacity measured on manometry and symptom severity score before admission and at 12-month follow-up (r=0.30, p<0.01 and r=0.25, p<0.05, respectively). Our results indicate that anal dilatation does not contribute to the management of school-aged children with idiopathic constipation. Admission to hospital for clarification of diagnosis and intensification of medical treatment with disimpaction of stool from the rectum is beneficial.     

一期与分期腹骶会阴肛门成形术治疗高位肛门直肠畸形的疗效比较

目的：比较腹骶会阴肛门成形一期手术与三期常规手术在治疗新生儿高位肛门直肠畸形中的疗效。方法回顾性分析本院收治的48例采用腹骶会阴肛门成形手术的新生儿先天性高位肛门直肠畸形患儿临床资料,分别采用一期手术（A组）和三期手术（B组）进行治疗,比较两组疗效。结果 A组21例均接受一期手术,2例失访,19例术后肛门功能优良率87．72％;B组27例中,肠造瘘后放弃后续手术或失访4例,23例全程完成三期手术,术后肛门功能优良率88．41％,两组疗效比较差异无统计学意义（P＞0.05）。结论选择性一期腹骶会阴肛门成形术治疗高位肛门直肠畸形同样可达到常规三期手术的效果,且有护理方便、病程短、费用低的优点。     

Could lipid infusion be a risk for parenteral nutrition-associated cholestasis in low birth weight neonates?

To assess whether lipid infusion could be a risk factor for parenteral nutrition-associated cholestasis (PNAC) in low birth weight neonates, 22 newborns with cholestasis (29.8 ± 1.6 weeks, 1298 ± 217 g) were compared with 22 without cholestasis (29.5 ± 1.7 weeks, 1286 ± 363 g). The mean level of peak direct bilirubin for the cholestasis group was 4.6 mg/dl compared to 1.2 mg/dl for the noncholestasis group. A univariate analysis revealed that PNAC was significantly related to duration of fasting (p = 0.008) and parenteral nutrition (p < 0.0001), days of antibiotics use (p = 0.025), positive C-reactive protein (p = 0.018) or gastric culture (p = 0.018), and feeding intolerance (p < 0.0001). Total amino acid amount (p < 0.0001), total lipid amount (p < 0.0001), and average daily lipid amount (p = 0.002) were significantly higher in the cholestasis group than in the noncholestasis group. Conversely, prenatal administration of dexamethasone was a significant protective factor of PNAC (p = 0.008). Logistic regression analysis revealed that the cumulative amount of lipid infusion was an independent risk factor for PNAC (p = 0.041; OR 1.174; CI 1.007–1.369). We suggest that decreasing the cumulative load of amino acids and intralipids with early trophic feeding, control of infection, and prenatal administration of dexamethasone could possibly attenuate the severity of PNAC.     

Diversities of H-type anorectal malformation: a systematic review on a rare variant of the Krickenbeck classification

Congenital H-type fistula is a rare congenital rectourogenital connection with an external anal opening in a normal or ectopic position. A systematic review was done to study the anatomical types of congenital H-type fistula, embryology, clinical presentation, relative gender distribution, associated anomalies, investigative modalities, and recent advances in treatment of these lesions. A PubMed search included H-type anorectal malformation; H-type anorectal malformations; H-type anorectal; and H-type congenital anorectal that gave 9;43;76;26 abstracts, respectively. Relevant studies and cited articles were studied omitting duplicate search. The reported incidence is 0.1–16 % of all anorectal malformation. The H-type anorectal malformation is 2.5–6 times more common in females and usually associated with a normal anus. In males, the anomaly is usually a variant with an ectopic anus or a perineal fistula. Anatomical types include anovestibular; rectovestibular; rectovaginal fistula in females and rectourethral (bulbar, prostatic, bladder neck) and rectovesical fistula in males. Variants identified include H-type fistula with perineal fistula, perineal groove, H-type sinus, H-type canal, and acquired H-type fistula. This review compiles the available literature over last six decades. Various surgical corrective procedures have been described. The high recurrence decreases with a learning curve and experience.     

Congenital H-type vestibuloanorectal fistula

Seven girls with a congenital H-type fistula between the vestibule of the vagina and the anorectum are reported. The anus was normally placed in all cases, with the fistula opening into the anterior wall of the lower bowel at the level of the anorectal junction 2–2.5 cm above the anal verge. All seven children underwent surgical repair using a midline perineal approach, a preliminary sigmoid colostomy having been performed in five cases. Satisfactory postoperative results were obtained in six patients. The anatomy and surgical management of this condition are discussed.     

A Prospective Analysis of the Incidence and Risk Factors Associated with Junctional Ectopic Tachycardia Following Surgery for Congenital Heart Disease

This study was designed to evaluate the incidence and risk factors associated with the occurrence of junctional ectopic tachycardia (JET) in patients after congenital heart surgery. We prospectively analyzed cardiac rhythm status in 336 consecutive patients undergoing surgery for congenital heart disease at our institution during a 1-year period. The incidence of JET was 8% (27/336). Repairs with the highest incidence of JET were arterial switch operation (3/13, 23%), atrioventricular (AV) canal repair (4/19, 21%), and Norwood repair (2/10, 20%). Compared to patients with no arrhythmias, patients with JET were more likely to be younger (2.75 ± 2.44 vs 5.38 ± 7.25 years, p < 0.01), have had longer cardiopulmonary bypass times (126 ± 50 vs 85 ± 73, p < 0.01), and have a higher inotrope score (6.26 ± 7.55 vs 2.41 ± 8.11, p < 0.01). By multivariate analysis, ischemic time was the only factor associated with JET [odds ratio, 1.01 (confidence interval, 1.005–1.02); p = 0.0014). The presence of JET did not correlate with electrolyte abnormalities. JET is not necessarily related to surgery near the His bundle or hypomagnesemia. Longer ischemic time is the best predictor of JET. Patients undergoing arterial switch operation, AV canal repair, and Norwood repair are at highest risk of postoperative JET and should be considered for prophylactic therapy.     

Metabolic,inflammatory, endothelial and haemostatic markers in a group of Italian obese children and adolescents

Childhood obesity and its related comorbidities are increasingly recognised in children, predisposing them to early cardiovascular disease and metabolic syndrome. The objective of the study was to investigate markers of metabolism, inflammation and haemostasis in a group of Italian obese children and adolescents. Fifty-nine obese and 40 non-obese subjects were recruited. Fasting glucose and insulin, total cholesterol, HDL and LDL cholesterol, triglycerides, high-sensitivity C-reactive protein (hsCRP), tumour necrosis factor alpha (TNF-α), and adiponectin were measured. Hypercoagulability was assessed by measuring the circulating levels of thrombin-antithrombin complex (TAT), D-dimer, fibrinogen, plasminogen activator inhibitor 1 (PAI-1) and von Willebrand Factor (vWF). A significant degree of insulin resistance was present in obese subjects compared with controls (p < 0.0001). The obese showed higher levels of total cholesterol, LDL cholesterol and triglycerides, and lower levels of HDL cholesterol than controls (p < 0.0001). Circulating levels of hsCRP and TNF-α were significantly higher in obese than in controls while serum adiponectin levels were significantly lower in obese than non-obese subjects (p < 0.001; p = 0.031; p < 0.0001, respectively). vWF, TAT, D-dimer, fibrinogen and PAI-1 levels were significant higher in obese subjects compared with control group (p = 0.02; p < 0.0001; p = 0.0037; p < 0.0001; p = 0.017, respectively). In conclusion, our results suggest that childhood obesity per se is associated with a proinflammatory and prothrombotic state.     

Postoperative internal sphincter function in anorectal malformations — a manometric study

An anorectal manometric assessment of 30 patients with operated high or intermediate anorectal malformations was performed in order to study internal sphincter function in the reconstructed anal canal. In 14 patients (group I, mean age 3.1 years) the rectal blind pouch and fistula, when present, were used in the reconstruction of the anal canal by posterior sagittal anorectoplasty (12 cases) or sacroperineal pull-through (2 cases). In 16 cases (group II, mean age 8.8 years) the anal canal was reconstructed from proximal rectum or sigmoid by sacroabdominoperineal pull-through. In 11 of the 14 patients in group I manometric findings suggested the presence of a functioning internal sphincter. The pattern of anal slow-wave activity and rectoanal inhibitory reflex was normal. None of the patients in group II had a rectoanal inhibitory reflex and the slow-wave activity was of a colonic type. The resting anorectal pressure profiles were significantly higher in group I than in group II (mean 38 cm H₂O vs. 25 cm H₂O). The fecal continence of patients in group I was superior to those in group II despite their much younger ages. The findings of this study indicate that in anorectal malformations there is internal sphincter function in the distal rectal blind pouch and that it can and should be preserved when the anal canal is reconstructed.     

Magnetic resonance imaging in anterior ectopic anus

Different criteria have been used in literature to describe the anterior ectopic anus (AEA) anomaly, resulting in uncertainty over its prevalence, association with constipation and definition of the indications for surgery. It has been recently proposed that the term AEA should be restricted to anomalies in which a normal appearing anal orifice is located in the perineum in a more anterior location than normal, with an anal canal of normal calibre that is shown by electrical stimulation to be surrounded by the voluntary external anal sphincter (EAS). We report about four infants, three females and one male, who presented with constipation and had an anteriorly located anal orifice of normal calibre. The anal position index measured clinically was less than 0.34 in all the female patients and 0.44 in the male patient. In preoperative magnetic resonance imaging (MRI), the EAS was distributed all around the circumference of the anal canal, including the ventral aspect of the anal canal, in all the patients. Preoperative MRI documentation of sphincter distribution is recommended for the diagnosis of AEA, as it would help in better definition of its association with constipation and the results of surgical management.     

Is the anorectal sphincter damaged during a transanal endorectal pull-through (TERPT) for Hirschsprung's disease? A 3-dimensional, vector manometric investigation.

BACKGROUND/PURPOSE: The transanal endorectal pull-through technique (TERPT) for Hirschsprung's disease (HD) exercises considerable traction on the anorectal tissue during dissection. So the question arises as to whether TERPT impairs the integrity of the anorectal sphincter. Computerised 8-channel vector manometry allows a segmental, 360 degrees analysis of muscular defects along the anal canal. Such data after TERPT are not available yet. METHODS: Between 2002 and 2004, 7 children underwent primary TERPT for HD of the rectosigmoid. All could be recruited for follow-up examinations. Stooling pattern, rectal examination, conventional 4-channel and computerised 8-channel vector manometry were assessed; the anal sphincter pressure at rest (ASPR), rectoanal inhibitory reflex (RIR), anal canal length (ACL), high pressure zone (HPZ), maximal segmental pressure (max SP), segmental/total asymmetric index (SAI/TAI), vector volume at rest (VV) were studied. Mean pre- and postoperative values were compared (pre/post). RESULTS: After a mean of 14 months (range 3-21 months) all children had spontaneous bowel movements, with no complaints of encopresis or constipation. 4-channel manometry revealed an unchanged ASPR (48.1/49.2 mmHg). RIR was present in 1/7. Computerised 8-channel comparison revealed no changes for ACL (15.4/16 mm), HPZ (60/53.19 % of ACL), SAI (17.6/18.63 %) and TAI (35.8/35.63 %). A postoperative increase was noted for max SP squeeze (141.4/178.7 mmHg) and VV (38 161/46 680 mmHg/cm (2)). In conclusion, the TERPT for HD preserves the functional integrity of the anorectal sphincter complex and has a favourable clinical and manometric outcome.     

Bowel function after surgery for anorectal malformations in patients with tethered spinal cord

Tethered spinal cord (TC) is an anomaly frequently recognized in association with anorectal malformations (ARM). However, the influence of TC on bowel function in children with ARM remains unknown. Furthermore, there are few studies that have assessed anorectal function in children with ARM and TC. The aim of this study was to evaluate anorectal function in ARM patients with TC using clinical assessment and anorectal manometry. Among 258 patients with ARM, this retrospective investigation included 35 patients who underwent spinal magnetic resonance imaging (MRI) after surgery for ARM. The patients were divided into two groups based on the presence or absence of TC, and bowel function was assessed by Kelly’s clinical score and anorectal manometry. Tethered cord was found in nine of the 35 patients (26%) with ARM. Of the ARM patients, TC was noted in four of 11 (36%) with high type anomalies, one of 8 (13%) with intermediate type anomalies, two of 14 (14%) with low type anomalies, and two of two patients (100%) with cloacal anomalies. Kelly’s clinical score did not significantly differ between the two groups. However, two of the nine patients with TC had poor bowel function (Kelly’s score; 2–0 points). On the contrary, patients without TC did not have poor bowel function. Anorectal manometry did not show a significant difference between patients with and without TC. However, the two patients with TC who had poor bowel function by Kelly’s score had low anal resting pressure, which was essential for achieving fecal continence. In conclusion, the present study showed that tethered cord was more frequently found in patients with more severe anorectal anomalies. Patients with TC were more likely to have poor bowel function, but this did not reach statistical significance. Presented at the 14th International Paediatric Colorectal Club, York, UK, 14–16 July 2007.     

Anal canal duplication in infants and children--a series of 6 cases.

The authors present a series of six anal canal duplications (ACD), duplications of the alimentary tract located along the posterior side of the anal canal, with a perineal opening just behind the anus. Five asymptomatic duplications were diagnosed before the age of one year, by simple perineal inspection. A twelve-year-old girl presented with perineal and anal pains and diarrhoea. Fistulography revealed a tubular structure in five cases and a cystic structure in one case, behind the normal anal canal, in one case communicating with it. A presacral sacrococcygeal teratoma was found in two children and in one case it was visualised by preoperative US in an infant with a lumbosacral myelomeningocele. Surgical excision was performed by a perineal approach in 5 cases, by a combined sacral and perineal approach in the last case, because of the associated teratoma. Non-invasive preoperative investigations, consisting of a pelvic X-ray, US examination, barium enema and fistulography, are sufficient in most cases; MRI is reserved for special indications. Surgical treatment restores a normal perineal aspect, without sequelae, and avoids complications like those described in other types of digestive duplications: infection, ulceration, bleeding, malignant changes during later adult life. Associated anomalies are frequently described in the literature, especially presacral tumours (16%) and anorectal malformations (21%); they can influence the management, the surgical approach and the functional prognosis.