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.     

Parenting children with anorectal malformations: implications and experiences

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. Accepted: 12 September 1997     

Colorectal perforations in neonates with anorectal malformations

 Colorectal perforations in neonates with anorectal malformations (ARM) are rarely reported. Two cases, one each with a low and high ARM is presented. Delayed patient presentation and “closed-loop” intestinal obstruction seem to be possible causes of perforation in these cases. Both patients survived following surgical intervention. The pertinent literature is reviewed to emphasize the overall management of such cases. Accepted: 17 December 1999     

The role of colostomy in the management of anorectal malformations

The role of the colostomy in the definitive correction of anorectal malformations is controversial with regard no only to indications for its use, but also to location and type. We reviewed the records of 272 patients with 307 colostomies and analyzed the frequency of complications related to these controversial points. There were 250 completely divided and 57 loop colostomies, constructed in either the sigmoid or transverse colon. Twenty-five colostomies were associated with complications that included retraction, stenosis, dehiscence, prolapse, infection, bowel obstruction, sepsis, and death. Twelve additional colostomies were placed in positions that impeded the definitive correction of the malformation: 9 sigmoidostomies were too distal and 3 were located in the right-upper abdominal quadrant and did not permit pull-through without tension. Colostomy prolapse and pull-through infection were statistically less likely with a completely diverting colostomy. Thirty-five patients with rectovestibular malformations were encountered in this series. Ten were referred from other institutions after attempted repair without a colostomy and all 10 had complications of infection, dehiscence, retraction, and reopening of the fistula. In contrast, there were no complications in the remaining 25, in whom the repair was preceded by a colostomy, nor after re-repair of the other 10. These findings support the need for a colostomy prior to the repair of this malformation. We recommend a carefully constructed, high, completely diverting sigmoid colostomy prior to the initial definitive repair of anorectal malformations. A right transverse colostomy is preferable preceding redo operations.     

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.     

Neurovesical dysfunction in children after surgery for high or intermediate anorectal malformations

Aim: To evaluate problems with bladder dysfunction in patients operated on for imperforate anus. Methods: All patients (11M, 25F) with high or intermediate imperforate anus seen in this institution between 1987 and 1997 were subjected to the investigation. They had all undergone a posterior sagittal anorectoplasty procedure with the aim of preserving as much as possible of the fistula. Hospital charts were reviewed. A detailed and structured interview regarding micturition habits was performed. If the results of this were abnormal the patients were also subjected to flow registration, measurements of residual urinary volume and in some cases also cystometry. Results: Micturition habits were normal in 30 out of 36 children. The remaining six, considered to have neurovesical dysfunction (NVD), had difficulties in emptying their bladder and exhibited pathological residual volumes. Two were boys with rectourethral fistulae, two were girls with cloacal malformations and two were girls with rectovestibular fistulae. Four out of six children with NVD had sacral anomalies.

Conclusion: NVD may occur even in the absence of sacral anomalies. A 4 h micturition observation is recommended in all newborns with intermediate or high anorectal anomalies to recognize the occurrence of NVD at an early stage.     

先天性直肠肛门畸形盆腔MR三维重建图像及临床意义

目的 建立先天性直肠肛门畸形(anorectal malformations,ARM)盆腔MR三维重建图像,探讨其临床意义.方法 患儿28例,其中先天性直肠肛门畸形组14例,男9例,女5例;年龄3 d1例,2～5个月12例,13岁1例.对照组14例,男8例,女6例;年龄3个月～10岁,平均2.9岁,为无直肠肛管疾患而因其他疾病需行盆腔或臀部MR扫描的患儿.应用Siemens 1.5T MR成像仪,对28例小儿盆腔行横断位、矢状位和冠状位T_1WI和T_2 WI扫描,横断面和矢状面T_1WI加脂肪抑制序列.采用体绘制和3D-Doctor软件进行三维重建;利用三维图像,判断直肠肓端、横纹肌复合体(SMC)位置及发育情况.结果成功建立了28例小儿盆腔的MR三维重建图像,能清晰显示直肠盲端与SMC的关系、瘘管位置.根据三维重建图像上直肠盲端和SMC的关系,14例术前接受检查的ARM患儿中,5例直肠盲端位于SMC以上,诊断为高位闭锁,4例直肠盲端部分穿过SMC,诊断为中位闭锁,5例直肠肓端完全穿过SMC,诊断为低位闭锁,与X线或造影诊断相符,并经手术证实,诊断正确率为100%.每例ARM患儿SMC大小、分布不一致,即使在同一类型的患儿也不同,高位畸形最明显.对照组患儿,SMC边缘整齐,厚度均匀,起于耻骨后面,分两束向后行经直肠两侧并在其后方交汇,形成漏斗样结构,直肠位于中央.结论 盆腔MR三维重建图像能清楚地显示骨盆解剖结构的关系,为ARM诊断和手术提供直观形态学依据,为虚拟手术奠定基础.     

Feasibility of perineal sagittal approaches in patients without anorectal malformations

Perineal sagittal approaches (posterior sagittal anorectoplasty and anterior and posterior sagittal transanorectal approaches) allow complete anatomic exposure of the perineum and lower pelvis. Moreover, they reduce the risk of damaging important structures because the incision is led in the midline. Therefore, many surgeons have used these approaches to treat diseases other than anorectal malformations (ARM), including intestinal dysganglionosis, trauma, pseudohermaphroditism, presacral mass, and rectal duplication. The aim of this study was to describe a small series of patients operated on via these approaches at Gaslini Childrens Hospital over a 5-year period. We retrospectively evaluated 10 patients consecutively operated on via a perineal sagittal approach, with or without sphincteric structure involvement, between January 1997 and December 2001. All of these patients were without ARM. Indications included retrorectal abscesses (two), iatrogenic anal canal stenosis (one), postinflammatory anal canal stenosis (one), internal anal sphincter neurogenic achalasia (one), female pseudohermaphroditism (one), benign sacrococcygeal teratomas (two), malignant sacrococcygeal teratoma (one), and perineal rhabdomyosarcoma (one). Protective colostomy was used in four patients. The parameters that we analysed included technical details, possible complications, perineal cosmetic appearance, and outcome. No complications were experienced. The postoperative cosmetic perineal appearance was excellent in all patients, and continence, when assessed, was always considered satisfactory. All tumours underwent complete gross resection. However, one patient with malignant sacrococcygeal teratoma died as a result of the malignant process 2 years after surgery. Although our study was carried out on a small series of patients, it confirmed that perineal sagittal approaches can be used not only for ARM but also for other conditions involving perirectal pouches, presacral space, and urogenital structures, as these approaches are safe and provide excellent cosmetic results as well as satisfactory functional outcome. Although tumours can be treated via these approaches, outcome remains related to the nature and malignancy of the disease itself.     

Surgical management of anorectal malformations: a unified concept

Posterior sagittal anorectoplasty (PSARP) is a new approach for the treatment of anorectal malformations. It has allowed direct exposure of the anatomy of these defects, enabling the author to describe the spectrum of this malformation. It also permits patients to be grouped on the basis of their potential for continence and the correlation of operative findings with clinical results. Traditional anatomic concepts have proved very inaccurate. The new approach allows separation of rectum from genitourinary tract without damaging important structures. The rectum is located within the limits of the striated muscle structures in a precise manner. This approach has important implications for therapy, evaluation of results, classification, and terminology. All defects can be treated with PSARP. The size of the incision will vary according to the complexity of the malformation, from minimal PSARP in low malformations to full PSARP in higher defects. In addition, approximately 10% of all cases will require a laparotomy. Colostomy is recommended in most patients except for those with low malformations. A new classification is proposed based on practical therapeutic purposes. Patients are also grouped according to their common potential for continence, which is important for the evaluation and comparison of results. The decision to create a protective colostomy is based on clinical facts in 80%–90% of cases and on invertograms and other diagnostic tests in the remaining 10%–20%.     

血液病或肿瘤患儿家长心理状况研究

目的检测血液病或肿瘤患儿家长与普通疾病患儿家长焦虑、抑郁水平和家庭环境的差异。方法采用抑郁自评量表（SDS）、焦虑自评量表（SAS）、家庭环境量表中文版（FES-CV）,对45例血液病或肿瘤患儿家长及52例呼吸系统疾病患儿家长（对照组）进行心理、家庭环境评估。结果血液病或肿瘤患儿家长焦虑、抑郁检出率明显高于对照组（P〈0．05）;血液病或肿瘤组患儿家庭在知识性及娱乐性方面低于对照组（P〈0．05）。结论血液病或肿瘤家长存在较多的不良情绪和家庭环境问题。因此,在治疗原发病的同时,很有必要对患儿及其家长进行适当的心理干预,以提高患儿与家长对血液病或肿瘤疾病长期治疗的配合与依从性。     

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.     

Haemangiomas and vascular malformations of the limb in children

Haemangiomas and vascular malformations of the limb in children are often difficult to manage. The role of surgery and intervention in current management is still not clear. The aim of this study was to review our experience of such patients. Patients were identified using the health board database of inpatient admissions between 1999 and 2003. Clinical notes were reviewed and data collected looking at patient demographics, site of lesion, clinical findings, investigative procedures, intervention and follow-up. A total of 288 patients were identified with a diagnosis of “Haemangioma” or “vascular malformation”. Thirty-eight of these patients were found to have limb haemangiomas or vascular malformations (20 boys and 18 girls). The median age at referral was 1.9 years (0–13.2). The clinical presentation included gigantism, swelling, bruising, bleeding and cosmetic concern. Cosmetic concerns and bleeding being the most frequent. Investigative modalities were used in 19 patients. These included ultrasound, CT, MRI and angiography. Many patients had more then one imaging modality employed. Treatment options included observation, steroids, laser, embolisation and surgical excision. Twenty-five patients (66%) required surgical intervention. Most patients were required one or two procedures, mainly laser or simple excision. However two patients required multiple procedures while one patient with Kasabach–Merritt syndrome required a limb amputation. Patients were followed-up for a median of 3.75 years (0.08–14). Two patients were lost to follow-up. Two patients were noted to have related psychological problems. Haemangiomas and vascular malformations of the limb can be associated with significant functional impairment, especially if they are extending to deeper tissue planes. Such patients are likely to require surgical intervention. For many patients referred to tertiary care, surgery is still the mainstay of treatment and the only curative option. Serious complications were more common in patients with arterio-venous malformations and Kasabach–Merritt syndrome. Two of 38 patients (5.3%) were referred for formal psychological assessment. Given the potential for psychological morbidity we fear this is an aspect of care that may be under-recognised and require further specialist input.     

Long-term bowel function,quality of life and sexual function in patients with anorectal malformations treated during the PSARP era

Anorectal malformations are an important group of congenital anomalies that vary widely in their anatomical characteristics and complexity. Understanding the long-term functional outcomes after modern treatments, and how these compare to the general population, are essential for ensuring that patients receive optimal, evidence-based care. With increasing appreciation of the wider impact of the illness on patients and their families, minimizing social disability from fecal incontinence and enabling normal social integration from the outset are key management concerns. This review summarizes the current knowledge on the functional outcomes by type of malformation, reflecting on the literature, and our institutional experience over a follow-up period of nearly 30 years.     

Risk factors in the primary management of anorectal malformations in Northern India

The purpose of this study was to determine the major factors influencing the survival of babies with anorectal malformation (ARM) during the primary management in the neonatal period in a tertiary referral center. The outcome of 125 neonates with ARM, treated in a single hospital during a 2-year period, was analyzed retrospectively with particular reference to birth weight, time of arrival, and the type of ARM, including pouch colon and major associated anomalies, to see whether any of them had a significant effect on initial survival. High ARM (HARM) was present in 75, low ARM (LARM) in 36, and pouch colon in 14 babies. There were a total of 28 deaths (22%). In babies with isolated HARM weighing >2.5 kg and brought to the hospital within the first 48 h of life, the survival was 100%, which dropped to 80% when brought later than 48 h. Of the babies with birth weight <2.5 kg and isolated HARM but who were brought to the hospital within the first 48 h of life, 80% survived, whereas of those reaching the hospital after 48 h, only 55% survived. Two babies with pouch colon and two of the four babies with LARM and associated anomalies died. In the HARM group, of the 31 babies with major associated anomalies, only 37% with birth weight <2.5 kg and 58% with birth weight >2.5 kg survived. Low birth weight, major associated anomalies, and delay in referral were the three important factors that influenced the outcomes of babies with ARM during the neonatal period.     

学龄期儿童神经性尿频与心理状况关系的临床研究

目的：探讨焦虑、抑郁心理与学龄期儿童神经性尿频发生的关系。方法：选取136例9~12岁神经性尿频患儿为病例组,136例9~12岁健康儿童为对照组。以儿童焦虑性情绪障碍筛查表（SCARED）评价患儿焦虑心理,以儿童抑郁障碍自评量表（DSRSC）评价患儿抑郁心理,比较两组焦虑和抑郁的发生率以及两组SCARED和DSRSC评分,并运用logistic 多元回归分析探讨焦虑和抑郁心理与神经性尿频发生的关系。结果：病例组焦虑和抑郁的发生率均高于对照组,差异有统计学意义（P23分者(即存在焦虑)神经性尿频发病风险为SCARED≤23者的1.224倍;DSRSC筛查≥15分者(即存在抑郁)神经性尿频发病风险为DSRSC<15者的1.148倍。结论：焦虑和抑郁心理参与了学龄期儿童神经性尿频的发病。     

腹腔镜下肛门成形术治疗直肠肛门畸形的并发症分析

目的 探讨腹腔镜下肛门成形术治疗直肠肛门畸形的并发症的产生原因和应对方法.方法 总结分析本中心2010年1月至2015年12月共腹腔镜手术治疗的直肠肛门畸形92例,全部为男性患儿,其中直肠膀胱颈瘘23例,直肠前列腺球部瘘35例,直肠尿道瘘34例.全部患儿均行横结肠造瘘或乙状结肠造瘘的分期手术.术后平均随访时间2.5年(4个月～5年),统计术后并发症和肛门排便情况,其中31例患儿随访大于3年.结果 术后直肠回缩11例,有1例再次手术缝合,另10例保守治疗.术后直肠黏膜外翻7例,均为早期病例,有4例再次手术切除局部外翻黏膜,有3例游离拖出结肠切除部分后再次肛门成形.腹腔残余感染4例,均经抗感染后好转.在已随访3年以上的31例患儿中术后排便失禁3例,其中有1例患儿由于严重瘢痕狭窄予扩肛治疗,另外有2例患儿伴发脊髓栓系.结论 虽然手术并发症难以完全避免,但注意围术期处理和手术技巧,能减少术后并发症的发生.     

Postoperative anorectal manometric evaluation of patients with anorectal malformation

BACKGROUND: Fecal incontinence is a common problem after reconstructive surgery for anorectal malformations. The aim of this study was to investigate the effectiveness of clinical scores and anorectal manometry in patients, who have been operated on for anorectal malformations. METHODS: In total, 18 patients who underwent surgery for anorectal malformation between 1999 and 2004 were investigated for anal continence. For the assessment of the patients' continence, Kelly's clinical scoring, Kiesewetter-Chang scoring, and anorectal manometry were used. RESULTS: In the intermediate level anorectal malformations, average anal resting pressure was found as 58.16 +/- 8.14 cmH(2)O and in high level anorectal malformations was found as 40.16 +/- 17.4 cmH(2)O. In the continence score, good according to Kelly and Kiesewetter-Chang scoring systems was an average anal resting pressure value of 57.92 +/- 8.57 cmH(2)O and in fair or bad was found as 32 +/- 12.83 cmH(2)O. There were significant differences between the scoring systems anorectal malformation level, and average anal resting pressure values (P < 0.05). CONCLUSIONS: Anorectal manometric evaluation of the patients in postoperative period with anorectal malformation can give more realistic information about the patient continence status in anorectal malformations.     

Spectrum of genitourinary malformations in patients with imperforate anus

A retrospective review of 244 cases of imperforate anus demonstrated a 48% incidence of associated genitourinary (GU) anomalies. The incidence and severity of associated GU anomalies was directly related to the level of the fistula between the blind-ending rectum and the GU tract. High-level fistulae to the bladder neck in males and the cloaca in females demonstrated a 90% incidence of associated genitourinary anomalies. In contrast, lower-level fistule to the perineum revealed only a 14% incidence of associated GU anomalies. Classification of anorectal anomalies by fistula level provides an accurate means of predicting the likelihood of an associated GU malformation.     

Window rectostomy: an alternative method of fecal diversion for high anorectal malformations in males without a urinary fistula

This report analyzes our initial experience with window rectostomy (WR) as a new method of fecal diversion for high anorectal malformations (ARM) in 27 males without a urinary fistula between May 1994 and May 1998; total correction was achieved in two stages. In the first stage, during the neonatal period, the dilated rectum was exteriorized as a WR through the left lower abdomen. In the second, after 3–5 months an abdominoperineal pull-through (APPT) procedure was performed in which the window rectostomy was mobilized and taken down to form a new anus. The results were compared with cases of high ARM with urinary fistula that were managed in three stages, i.e., proximal sigmoid colostomy, APPT, and colostomy closure. All 27 cases showed satisfactory results without any mortality or major pelvic infection. The main advantages of WR are that it provides more functioning bowel length after diversion and avoids a colostomy-closure operation and repeated hospitalizations, thus reducing the total cost of treatment. Accepted: 6 October 1999     

腹腔镜辅助高位肛门直肠畸形成形术后中期随访研究

目的 探讨高位肛门直肠畸形患儿腹腔镜辅助肛门直肠成形术(LAARP)的疗效.方法 回顾分析了2002年1月至2005年12月在我院采用LAARP高位肛门直肠畸形10例,并与2002年1月之前行后矢状入路肛门直肠成形术(PSARP)的12例患儿临床情况进行比较.所有患儿均在新生儿期行结肠造口术.LAARP组在3～5岁、PSARP组在6～8岁时,所有患儿的直肠肛门功能通过Kelly排便功能评分法(6分法)以及肛门直肠测压法进行评价.结果 LAARP组行肛门直肠功能评价的平均年龄为(47.2±8.3)个月,低于PSARP组的(74.5±9.3)个月,差异有统计学意义(P<0.05).LAARP组与PSARP组Kelly排便功能评分分别为3.7±0.9与3.4±0.8;二组肛管静息压、肛管高压带长度十分类似,差异无统计学意义(P>0.05),而二组直肠肛门抑制反射差异有统计学意义(P<0.05).结论 中期随访研究表明,高位肛门直肠畸形患儿LAARP可获得满意的排便控制效果,但LAARP与PSARP对长期生活质量的影响还有待于长期随访的结果.