Evaluation of outcome of anorectal anomaly in childhood: the role of anorectal manometry and endosonography

The aim of this study was to evaluate role of anorectal manometry (ARM) and anal endosonography (ES) in assessment of the internal anal sphincter (IAS) quality on continence outcome following repair of anorectal anomalies (ARA). We devised a scoring system to evaluate the quality of the IAS based on ARM and ES and correlated the scores with clinical outcome, using a modified Wingfield score (MWS) for faecal continence. We also assessed the implication of megarectum and neuropathy on faecal continence. Of 54 children studied, 34 had high ARA and 20 had low ARA. Children with high ARA had poor sphincters on ES and ARM, and also poor faecal continence compared to those with low ARA. The presence of megarectum and neuropathy was associated with uniformly poor outcome irrespective of the IAS quality. The correlations between MWS on one hand, and ES and ARM scores for IAS on the other hand were weak in the whole study group, ES r = 0.27, P < 0.04, and ARM r = 0.39, P < 0.004. However, the correlations were strong in those who had isolated ARA without megarectum or neuropathy, ES r = 0.51, P < 0.02 and ARM r = 0.55, P < 0.01, respectively. In conclusion, the ARM and ES are valuable in evaluation of continence outcome in children after surgery for ARA and those with good quality IAS had better faecal continence. The IAS is a vital component in functional outcome in absence of neuropathy and megarectum.     

Postoperative psychological status of children with anorectal malformations

Our purpose was to clarify factors that influence the level of depression of Japanese children with anorectal malformations (ARM). The subjects comprised 66 children with ARM, aged 0–16 years, and their mothers. Patients were divided into three groups (Group 1: aged 0–5 years, Group 2: 6–11 years, and Group 3: 12–16 years). The level of depression of the children aged 6–16 years was investigated by Kovac’s children’s depression inventory (CDI). The psychological status of their mothers was assessed by Spielberger’s state-trait anxiety index (STAI) and Zung’s self-rating depression scale (SDS). The classification of ARM and the clinical condition were also investigated. Defecation scores were assigned for the degree of bowel dysfunction. The level of depression in the patients was more marked in Group 3 than in Group 2 according to the CDI score. Significant correlations between the CDI score of the child and the STAI-1, STAI-2 and SDS scores of the mother were observed in Group 2, but not in Group 3. In Group 2, the STAI-1 score of the mothers was significantly influenced by the degree of bowel dysfunction in her child. In Group 3, the CDI score was significantly correlated with the constipation score. This study revealed that bowel dysfunction is the important factor that influences the level of depression of the children with ARM. In the period of childhood during which the child attends primary school, bowel dysfunction indirectly influences the level of depression of children through the psychological status of mothers. However, bowel dysfunction directly influences the level of depression of children in adolescent patients.     

Utility of spinal MRI in children with anorectal malformation

Background  The association between spinal cord anomalies and imperforate anus is well recognized. Until now, the incidence of tethered cord has been assumed to be higher in patients with high-type imperforate anus. However, recent reports suggest that tethered cord is as common in patients with a low lesion as in those with a high lesion. Objective  To review the incidence of spinal cord anomalies in those with a low lesion and those with a high (including intermediate) anorectal malformation (ARM), and to determine the best diagnostic imaging strategy. Materials and methods  A group of 50 consecutive patients with postoperative ARM and in whom spinal MRI had been performed were identified retrospectively. We reviewed and compared the following factors between those with a high lesion and those with a low lesion: (1) clinical symptoms, (2) spinal cord anomalies, and (3) vertebral anomalies. Results  The incidence of spinal cord anomalies was no different between those with a high lesion and those with a low lesion, and spinal cord anomalies were present regardless of the presence of vertebral anomalies or symptoms. Conclusion  Owing to the high incidence of spinal cord anomalies in patients with imperforate anus, MRI is the best imaging tool for detecting such anomalies regardless of the level of the lesion.     

Secondary posterior sagittal anorectoplasty for anorectal malformations

Sixteen patients who had undergone secondary posterior sagittal anorectoplasty (PSARP) for fecal incontinence were clinically followed for 3 to 10 years (mean 5.8). In addition to preoperative manometric evaluation, postoperative anorectal manometry was performed on all patients 1–3 times. Fourteen of the 16 patients underwent postoperative biofeedback training. At the last follow-up visit, the mean age of the patients was 18.3 years (range 16–26). Fectal continence improved in 13 patients. Manometrically measured, both the squeeze and resting pressures in the anal canal were significantly increased. Biofeedback facilitated the improvement of continence in 10 of the 14 patients. However, at the last follow-up, only 4 had good fecal continence with voluntary bowel movements and only occasional soiling. Three patients had voluntary bowel movements but frequent soiling; 8 used daily enemas to stay clean and 1 has a permanent colostomy. Secondary PSARP improves fecal continence in patients with intractable incontinence following primary repair of anorectal anomalies, but in adulthood it is good in only a minority of cases. Most patients require adjunctive measures to maintain socially acceptable bowel function.     

Long-term outcome of internal sphincter myectomy in patients with internal anal sphincter achalasia

Background  

Internal anal sphincter achalasia (IASA) is a condition with presentation similar to Hirschsprung’s disease (HD), but with the presence of ganglion cells on rectal suction biopsy (RSB). The diagnosis is made on anorectal manometry (ARM) by the absence of the rectosphincteric reflex on rectal balloon inflation. Internal sphincter myectomy (ISM) is the treatment of choice for patients with IASA. Recently, botulinum toxin has been used to treat IASA patients. The purpose of this study was to assess the long-term bowel function in patients with IASA following ISM.     

Evaluation of dysfunction following reconstruction of an anorectal anomaly

To evaluate the utility of anorectal manometry (ARM) and magnetic resonance imaging (MRI) with an endocoil in the assessment of dysfunction in children with repaired anorectal anomalies (ARA), 15 patients aged 1 to 15 years with repaired ARAs and chronic faecal incontinence or constipation were prospectively recruited. They underwent clinical assessment using a modified Wingfield score (MWS). ARM and MRI with an endocoil and conventional external coil were carried out. The results of ARM alone, MRI alone, and a combination of ARM and MRI were correlated with the MWS. Manometric internal anal sphincter (IAS) scores determined from sphincter length and activity correlated with MWS (r = 0.56, P = 0.02); manometric scores of rectal peristaltic activity did not. Overall manometric score (IAS and rectal scores combined) showed a correlation with MWS (r = 0.55, P = 0.02). Endoanal MRI sphincter scores did not correlate with MWS, but the presence of a megarectum on MRI did (r = 0.44, P = 0.05). Overall MRI score did not correlate with MWS. Minor neurosacral anomalies were shown on MRI in 3 children who had poor functional scores. Combined manometric and MRI scores showed a correlation with MWS (r = 0.58, P = 0.01). ARM and MRI are potentially useful in the assessment of dysfunction of children with repaired ARAs. Both modalities require refinement and further assessment in the context of directing management.     

肛门直肠畸形人类及大鼠神经系统的并发症及神经支配研究进展

先天性肛门直肠畸形(anorectal malformations,ARM)是最常见的小儿消化道畸形,占消化道畸形的1/4.尽管长期以来ARM的手术方式得到不断改进,但长期随访发现仍有许多中高位ARM患儿术后存在不同程度的排便功能障碍.研究人员意识到术后肛门直肠功能不良取决于许多因素,骶尾椎畸形以及骶尾部脊髓发育不良已成为影响术后排便功能的重要因素.研究人员通过对人类胚胎以及动物实验研究发现,腰骶椎异常是ARM常见的伴发畸形,腰骶髓神经细胞存在减少的现象.人类和大鼠胚胎发育过程中涉及肛门直肠和盆底肌神经支配的相关因素繁多.该文介绍人类及大鼠肛门直肠畸形神经系统并发症以及神经支配的异常,总结对该病合并神经支配异常的认识和研究发展过程,并提出了关于肛门直肠畸形神经系统研究的新方向,探索改善肛门直肠畸形手术预后的新策略.     

肠无神经节细胞症患儿术后肛门直肠测压的应用研究

目的：探讨经肛门 Soave 巨结肠根治术后患儿排便功能及直肠肛管测压的应用价值。方法回顾性分析经术后病理检查证实的61例肠无神经节细胞症（Hirschsprung＇s disease,HD）患儿临床资料,均行经肛门 Soave 巨结肠根治术,其中腹腔镜辅助手术16例。术后平均随访1年（3个月至2年）,根据手术年龄分为婴儿组（0～1岁）及幼儿组（1岁以上）。对患儿术后大便控制能力、并发症情况及肛门直肠测压结果进行比较。结果婴儿组术后排便控制情况与幼儿组比较,差异无统计学意义（P ＞0.05）。肛门直肠测压检查显示术前直肠肛管抑制反射总阴性率为95.1％（58／61）,术后均未恢复;两组直肠静息压、肛管静息压比较,差异无统计学意义（P ＞0.05）。结论经肛门 Soave 巨结肠根治术治疗小儿肠无神经节细胞症,术后可获得良好的排便控制功能,可作为首选术式;肛门直肠测压的术前诊断价值较高,术后短期随访作用有限。     

Associated anomalies with anorectal malformation (ARM)

Objective : To find the prevalence of associated anomalies in children with anorectal malformation (ARM).Methods: One hundred and forty patients (80 males and 60 females) with expand were studied to detect associated anomalies and to find their prevalence. High and low type of ARM was seen in 52.14% and 47.86% of patients respectively. Associated anomalies were more common with high type of ARM (78.08%) than in patients with low type of ARM (37.31%). 58.57% patients had associated anomalies which included those of urinary system (37.14%), vertebral system (34.28%), skeletal system other than vertebral (15.17%), genital system (14.29%), cardiovascular system (12.14%), gastrointestinal tract (10.7%) and spinal cord (10%).Results : 37.43% patients had 3 or more than 3 components of VACTERL association. Two patients had all six components of VACTERL. Most common association was vertebral, anal and renal anomalies seen in 16 patients.Conclusion : Patients with ARM should undergo a detailed general physical, systemic and radiological examination (infantogram, echocardiography, US of urogenital system) in neonatal period to detect associated anomalies in early period     

Anorectal malformations and their impact on survival

Objective : To evaluate the incidence, types and the effect on outcome of associated anomalies in neonates with anorectal malformations (ARM).Methods : This retrospective study was carried out on all neonates with ARM admitted to the neonatal surgical intensive care unit (NSICU) from 1998 through 2003.Results : Of the 754 neonates admitted to the NSICU during the study period of 6 years, there were 124 (16.4%) neonates with anorectal malformations. Of these 110 were included in the study. 73 % were male and 27% female. 86% of these were high ARM (HARM) while only 14% were low ARM (LARM). Associated anomalies were seen in 68% of patients. The incidence was 72% for HARM and 50% for LARM. The major associated anomalies consisted of esophageal (13%), gastrointestinal (GIT) (11%), genitourinary (GUT) (32%), skeletal (26%), cardiac (33%) and miscellaneous 26%. The overall survival rate was 84% (82% for HARM and 94% for LARM). The survival among those with associated esophageal anomalies was 43%, GIT 67%, GUT 80%, cardiac 61%, skeletal 76% and miscellaneous 79% respectively. This difference in survival was significant only for those with esophageal (p=0.004) and cardiac anomalies (p=0.0026). The survival rates among those with one, two or more than two organ systems involved with associated anomalies were 88%, 82% and 58% respectively. This difference was significant only for more than two organ systems involvement (p=0.003).Conclusion : Associated anomalies are common in neonates with ARM, the incidence being similar for HARM and LARM. The survival depends upon the number and severity of associated anomalies both in patients with LARM and HARM. Neonates with more number of organ systems involved have a poorer survival specially when associated with esophageal and cardiac anomalies. All neonates with ARM merit a meticulous search for associated anomalies so that the management can be tailored for each baby.     

先天性无肛畸形儿MR成像及其与排便功能关系研究

目的评估先天性肛门直肠畸形儿（anorectal malformations，ARM）尾端发育情况及横纹肌复合体（sphincter muscle complex，SMC）发育状态与排便功能的关系。方法39例ARM，男28例，女11例，应用不同序列体部相控阵列线圈或头部线圈MRI显示ARM直肠闭锁水平、骶尾椎、骶髓、泌尿生殖系的发育情况，并对SMC从多个层面进行定量分析，评估其发育状态与临床评分和肛门功能客观检查的关系。结果39例患儿中，MRI T1WI、T2WI扫描判断畸形水平的正确诊断率为100％，T2WI扫描瘘管检出率为96．9％。骶尾椎和骶髓的异常占41．0％，泌尿生殖系统畸形占20．5％。当耻骨直肠肌宽度的相对值PRWR〈0．18，外括约肌宽度的相对值EASWR〈0．15时，71．4％的ARM术后出现肛门失禁。当PRWR〉0．18，EASWR〉0．15时，91．3％的ARM术后排便功能好。结论MR检查能清楚地显示ARM类型、闭锁水平、SMC发育状态、脊柱脊髓及泌尿生殖系统存在的伴发畸形，从MR影像中找到了评价SMC的定量指标。     

Congenital funnel anus in children: associated anomalies,surgical management and outcome

Funnel anus (FA) is a rare and distinct type of anorectal malformation characterized by a skin-lined deep anal funnel, missing transitional epithelium and stenosis secondary to fibrotic internal sphincter. We aimed to characterize associated anomalies, surgical management and outcome of children with FA. The hospital records of 11 consecutive children (7 boys) treated for FA between 1992 and 2006 were screened. The collected data included the type of anorectal malformation, surgical management, associated anomalies, results of diagnostic investigations and outcome. Only one patient was free of any associated malformation. Six patients had a complete Currarino syndrome. Seven patients had a hemisacrum (scimitar) and tethered cord was present in two cases. Six patients underwent excision of a benign presacral teratoma. Anal stenosis associated with FA was managed by serial dilatations. Subsequent resection of the megarectosigmoid secondary to refractory constipation was performed on five occasions. Three patients underwent coloanal pull-through for Hirschsprung’s disease (HD). The level of aganglionosis was at the rectosigmoid junction in two cases and low in the rectum in one. One additional patient had hypoganglionosis. Of the three patients with HD two also had Down’s syndrome. After median follow-up of 6.5 (0.3–13.5) years four patients have normal bowel function and four suffer from soiling. Two patients with HD and Down’s syndrome and one patient with an undefined syndrome are fecally incontinent. Associated anomalies are common and diverse in children with FA. Pelvic MRI, sacral radiography, evaluation of the urinary tract and rectal biopsies are recommended as routine investigations in cases of FA. Surgical care of these patients is demanding and should be confined to dedicated centers.     

Long-term outcome of anorectal malformations: the patient perspective

To assess the long-term outcome of surgery for anorectal malformations (ARM) from the patient’s perspective. One hundred and sixty seven children were operated for ARM between 1982 and 2000. Disease impact questionnaires to assess both clinical and psychological outcomes were sent to 137 eligible families. Statistical analysis was performed using SPSS version 11.5. The response rate was 61% (n = 84). Sixty six percent had soiling. There was no significant difference in the incidence of soiling between genders or between the younger child and adolescent. Soiling was significantly increased in high (86%) and intermediate (79%) compared to low (43%) malformation (P = 0.001). Constipation was seen in 62% and abdominal pain in 49%, with no significant difference between malformation levels. Overall, 71% had associated anomalies. Although 44% had a documented urological abnormality, clinical significant problems were seen in only 30%. Eighty percent of the children had one or more behavioural problems and 15% expressed suicidal thoughts. ARM had a negative impact on the social life of the child in 52% and on family functioning in 50%. Soiling (P = 0.000), presence of associated anomalies (P = 0.001), constipation (P = 0.005), level of ARM (P = 0.015) and abdominal pain (P = 0.039) correlated significantly with psychosocial morbidity. Despite these findings, 62% of adolescents and 71% of children below 12 years with their parents reported above average global hopefulness score, remaining hopeful for the future. Children with ARM have ongoing physical and social morbidity, indicating the need for continuing multidisciplinary review and support to optimise their quality of life.     

Electrical stimulation and biofeedback exercise of pelvic floor muscle for children with faecal incontinence after surgery for anorectal malformation

We report our experience of electrical stimulation and biofeedback exercise of pelvic floor muscle for children with faecal incontinence after surgery for anorectal malformation (ARM). Electrical stimulation and biofeedback exercise of pelvic floor muscle were performed on children with post-operative faecal soiling following repair of intermediate or high type ARM. Children under the age of 5 years or with learning difficulties were excluded. They had 6 months supervised programme in the Department of Physiotherapy followed by 6 months home based programme. Bowel management including toilet training, dietary advice, medications and enemas were started before the pelvic floor muscle exercise and continued throughout the programme. Soiling frequency rank, Rintala continence score, sphincter muscle electromyography (EMG) and anorectal manometry were assessed before and after the programme. Wilcoxon signed rank test was performed for statistical analysis. From March 2001 to May 2006, 17 children were referred to the programme. Twelve patients (M:F = 10:2; age = 5–17 years) completed the programme. There was a trend of improvement in Rintala score at sixth month (p = 0.206) and at the end of programme (p = 0.061). Faecal soiling was significantly improved at sixth month (p = 0.01) and at the end of the programme (p = 0.004). Mean sphincter muscle EMG before treatment was 1.699 μV. Mean EMG at sixth month and after the programme was 3.308 μV (p = 0.034) and 3.309 μV (p = 0.002) respectively. After the programme, there was a mean increase in anal sphincter squeeze pressure of 29.9 mmHg (p = 0.007). Electrical stimulation and biofeedback exercise of pelvic floor muscle is an effective adjunct for the treatment of faecal incontinence in children following surgery for anorectal malformation.     

Perineal lipomas associated with anorectal malformations

Newborns with anorectal malformations may have associated perineal lipomas. These lesions are rare but may make the repair of the anorectal malformation more complex. Knowledge about the functional consequences of these lesions is scanty. The purpose of this study was to review the experience of anorectal malformations with perineal lipomas in two Scandinavian paediatric surgical centres. Six patients with perineal lipomas and anorectal malformation treated in the two centres from 1991 to 2005 were retrospectively reviewed. Bowel function was evaluated in patients more than 4 years old. Three girls and three boys (age 4 months to 14 years) with anorectal malformations and perineal lipoma were included in the study. The boys all had high anorectal malformations with rectourethral or rectovesical fistulas. Two girls had a rectovestibular fistula and one girl had a cloaca. All patients had perineal lipomas, in one associated with an anterior meningocele. The lipomas were excised at time of anorectal reconstruction. One of the patients still has a colostomy, and two colostomies were recently closed. The bowel function was evaluated in three patients. Two patients have an ACE stoma to control constipation and soiling and one is soiling despite regular washouts. In conclusion, perineal lipomas associated with anorectal malformations are rare but may distort sphincter anatomy. Excision is best performed at time of anorectal reconstruction. Although our experience is limited, bowel function seems to be compromised by these lesions.     

脊髓拴系松解术后脊柱裂患者的肛管直肠功能研究

目的：与临床对于脊柱裂致膀胱功能障碍的大量研究报道相比,关于脊柱裂患者的肠道功能障碍研究资料非常有限。该文拟研究脊柱裂患者行脊髓拴系松解术后的肛管直肠功能。方法：采用多通道肛管直肠测压技术对因排尿功能障碍而来就诊的21例脊柱裂患者进行肛管直肠功能检测,入选患者皆已于至少2年前行脊髓拴系松解术。结果：脊柱裂患者的最大肛管静息压低于对照组,但二者没有统计学差异(P=0.372)。在嘱脊柱裂患者行最大限度收缩肛门动作时,绝大多数患者肛管压力没有任何升高。在行模拟排便动作时,19例(90.5%)患者表现为盆底功能紊乱型肛管压力变化。直肠肛管抑制反射在所有受检者均存在,诱发该反射所需最小直肠气囊容量在脊柱裂患者组和对照组间差异无显著性(P=0.725);诱发持续性直肠肛管抑制反射所需直肠气囊容量在脊柱裂患者组显著性高于对照组(P<0.001)。直肠感觉阈值在脊柱裂患者显著高于对照组(P<0.0001)。结论：大多数脊柱裂患者不能自主收缩肛门外括约肌,排便时表现为盆底功能紊乱型直肠肛管压力曲线,同时直肠感觉功能也受到严重损害。直肠肛管抑制反射在所有脊柱裂患者均存在,该反射可能受到中枢神经系统的调控。     

先天性肛门直肠畸形术后合并便秘患儿排便功能评定及病因探讨

目的便秘是先天性肛门直肠畸形(anorectal malformation,ARM)术后常见的并发症,其病理改变复杂,病因尚不清楚。本研究利用多种客观检查方法对ARM术后便秘患儿肛门直肠功能和神经功能进行评定,并对其病因进行探讨。方法利用同位素排便造影、直肠肛管测压、肌电图和肛门括约肌神经电生理等方法对49例ARM患儿和31例正常儿童的排便功能进行全面、系统和动态的评价,49例ARM患儿根据畸形位置分为中低位组和高位组,每组再根据是否发生便秘分出两个亚组,并对上述测量指标进行统计分析。结果同位素排便造影结果显示,ARM术后便秘组半排时间[中低位:(13.45±8.35)s;高位:(20.59±4.26)s]与术后无便秘组[中低位:(4.69±6.86)s;高位:(7.66±6.38)s]相比明显延长,ARM术后便秘组排空率[中低位:(29.35±14.84)s;高位:(33.00±9.04)s]与术后无便秘组[中低位:(61.70±23.01)s;高位:(60.31±30.38)s]相比明显降低,差异具有统计学意义(P<0.05)。直肠肛管测压检测结果显示,直肠感觉阈在所有ARM组中均明显高于正常组(P<0.05),ARM便秘组[中低位:(53.57±9.45)mL;高位:(57.50±9.14)mL]高于ARM无便秘组[中低位:(46.32±14.61)mL;高位:(47.27±8.76)mL]。ARM便秘组感觉收缩时间[中低位:(2.79±0.39)s;高位:(3.51±1.93)s]明显长于ARM无便秘组[中低位:(1.97±0.67)s;高位:(2.11±0.43)s],差异具有统计学意义(P<0.05)。肌电图结果显示,反映排便动力的痉挛指数在术后合并便秘组均明显高于未合并便秘组。神经电生理结果显示会阴-肛门反射潜伏期在ARM患儿组均明显延长,其中ARM合并便秘组[中低位:(66.04±16.20)ms;高位:(70.41±17.91)ms]延长更加明显,与ARM无便秘组[中低位:(38.51±16.92)ms;高位:(49.91±9.45)ms]相比存在统计学差异(P<0.05)。结论ARM术后便秘患儿直肠感觉功能和排便动力存在明显异常,其严重程度与支配盆底肌肉的神经功能异常有关。治疗前应进行详细、系统的肛门直肠和神经功能的客观检查,明确排便功能障碍的具体病因和病理改变。     

Sacral development in anorectal malformations and in normal population

Background: The sacral ratio (SR) was proposed by Alberto Pe?a in 1995 as a reliable tool to evaluate sacral development in anorectal malformations (ARM). The SR is obtained by comparing sacrum size with fixed bony parameters of the pelvis. In previous studies, the average normal SR was calculated to be 0.74 for the anteroposterior view (APSR) and 0.77 for the lateral view (LSR). Objectives: (a) To evaluate the range of variability of the SR in a normal population with age as a possible variable factor. (b) To evaluate the reliability of the SR as a prognostic factor in patients with ARM. Materials and methods: We studied pelvic radiographs of 147 normal children in order to calculate a normal range of values for the SR. In 59 children with ARM, clinical parameters of faecal and urinary continence, spinal cord anomalies (as detected by MRI) and rectal innervation patterns (studied by enzymo-histochemistry) were correlated with SR values by Snedecor's ’F' test and Bravais-Pearson's ’r' correlation index. In 25 cases (15 children with ARM and 10 controls), multiple measurements of SR were obtained at different ages. Results: Normal values for APSR ranged from 0.52 to 1.12, with an average of 0.74. Normal values for LSR ranged from 0.52 to 1.18, with an average of 0.75. Low SR values (below 0.52) correlated well with spinal cord anomalies and with unfavourable functional prognosis in children with ARM. APSR and LSR values increased with age in the normal population, while they decreased in patients with ARM. Conclusions: The SR has a wide range of normal values, and should be considered truly pathological when lower than 0.52, in either the AP or lateral views. Age has a significant influence on SR values. Received: 15 October 2000/Accepted: 7 July 2001     

Evaluation and treatment for spinal cord tethering in patients with anorectal malformations.

BACKGROUND: It has recently been recognized that there is a close relationship between spinal cord tethering (SCT) and congenital anorectal malformation (ARM). PATIENTS AND METHODS: We evaluated spinal MRI examinations of 28 patients with ARM (14 boys and 14 girls) aged 5 months to 9 years. All patients diagnosed with SCT subsequently underwent operation. Patients were divided into high and low type ARM groups. We reviewed the relationship between SCT and ARM, and evaluated the untethering surgery. RESULTS: We evaluated 14 boys (high, 9; low, 5) and 14 girls (high, 4; low, 10). Of these 28 patients, 13 had SCT on MRI. Five out of 13 patients with high type ARM and 8 out of 15 patients with low type ARM had SCT. Seven out of 10 girls with low type ARM had SCT. Ten of these 13 patients with SCT experienced bowel/urological/orthopedic symptoms. SCT symptoms progressed prior to operation in the 2 patients who underwent untethering surgery a few years after their initial MRI examination. Postoperatively, orthopedic symptoms disappeared completely in all patients, but other symptoms did not. CONCLUSIONS: Based on the results of this study, we recommend routine MRI examination of patients with ARM and early untethering surgery in cases with SCT.     

Low anorectal malformation associated with ‘ano-urethro-cutaneous’ fistula

Anorectal malformations are one of the common congenital anomalies encountered in the newborn period. The plethora of anomalies described makes it a complex subject for embryological explanations. Fistulous communications between the blind rectum and the urinary tract are common in boys with high/intermediate anomalies, but it is uncommon in children with low anorectal malformations, more so a double fistula. We are reporting a case of ‘ano-uerthro-cutaneous fistula’ associated with a low ano rectal malformation.