An audit of neonatal colostomy for high anorectal malformation: the developing world perspective

Abstract A high divided sigmoid colostomy has been recommended for staged management of high anorectal malformation. We audited our cases of neonatal colostomy for high anorectal malformation to assess its effectiveness. A retrospective study was carried out of all surgical newborns admitted with high imperforate anus as the single diagnosis at our centre between December 1998 and December 2000. Morbidity and mortality were analysed after retrospective stratification into two groups (group A: birth weight >2.5 kg; group B: birth weight <2.5 kg). The chi square test was used to test the statistical significance in terms of outcome in the two groups. Overall mortality was 16%. Group A consisted of 34 babies: 30 with divided sigmoid colostomy and four with transverse loop colostomy. One baby with a divided sigmoid colostomy died from wound complications and septicaemia (mortality 2.9%). All four babies with transverse loop colostomy done under local anaesthesia survived, despite being sick on arrival. Group B consisted of 16 babies: 15 with sigmoid colostomy and one with transverse loop colostomy, with seven deaths (44%). None of the five babies with transverse loop colostomy done under local anaesthesia died, despite being sick on arrival, whereas all eight babies who died had undergone sigmoid colostomy under general anaesthesia. The difference in the outcomes of babies in groups A and B is highly significant (p <.01). Sick, small (<2.5 kg) and septic babies arriving late to the unit do not appear to tolerate general anaesthesia and divided sigmoid colostomy well, despite that procedures long-term advantages. Divided sigmoid colostomy has produced excellent results in babies >2.5 kg, but in the context of the developing world and limited critical care availability, transverse loop colostomy under local anaesthesia may save lives.     

Scenario of neonatal surgery in West Bengal with reference to cases of anorectal malformation

Pediatric Surgery, though a super specialty does not enjoy the glamour and importance like other specialities, though, dealing with the most delicate of mankind, the children. The reasons for this are manifold and the results of this, brutal. This is a retrospective study carried at the major institutes of West Bengal where departments of pediatric surgery exist. We have observed a gross discrepancy between the number of patients admitted for surgically correctable congenital malformations and the standard state/ national frequency of these disorders. We focus on the plight of a child not able to reach the leval III health care system with a pediatric surgical back up and analyze the pros and cons with constructive criticism of the existing system.     

Management of anorectal malformation in neonates

Anorectal malformations are one of the commonest anomalies in the new born. Major advances have been made in the last decade in operative techniques to reconstruct this abnormality. The final outcome in these babies is dependent on careful planning and operative intervention in the neonatal period. The purpose of this paper is to discuss the varied presentations of this anomaly, initial assessment and operative management with reference to our own experience.     

Colostomy for high anorectal malformation: an evaluation of morbidity and mortality in a developing country

Colostomy is a life-saving procedure in newborns with high anorectal malformations (ARM). However, the procedure may be attended by complications, particularly in resource limited settings. This is an evaluation of the morbidity and mortality following colostomy for ARM in newborns in two paediatric teaching centres in a developing country. A retrospective review of 61 neonates who had colostomy for high ARM in 4 years is conducted. The babies were categorised into Group A (weight at presentation < 2.5 kg) and Group B (weight at presentation > 2.5 kg). There were 47 boys and 14 girls aged 18 h to 28 days (median 6 days). There were 23 babies in Group A; 18 had colostomy under local anaesthetic (LA), 5 of whom died while 5 had the procedure done under general anaesthetic (GA), 3 of whom died (mortality 8/23, 34.78%). Group B consisted of 38 babies, 18 had colostomy under GA, 3 died, while in 20 the procedure was under LA, 1 of who died (mortality 4/38, 10.5%). The difference in mortality between groups A and B was statistically insignificant (p < 0.056). There were no significant differences in outcome between the two groups when the type of anaesthesia or types of colostomy were considered. Surgical site infection was the most common 12/61, 19.7%. Of the 12 babies that died, 7 were due to overwhelming infections, 4 respiratory insufficiencies and 1 cyanotic heart disease. The overall procedure related mortality was therefore 7 (11.5%). None of the centres had adequate neonatal intensive care services during the period of this report. Morbidity and mortality following colostomy for ARM in newborns is still high in this setting, due largely to infective complications, particularly in babies < 2.5 kg.     

Anorectal malformation with ileal atresia

A low-type anorectal malformation associated with ileal atresia is described. It was very difficult to classify the anorectal malformation due to the presence of ileal atresia. Such anomalies are rare. A review of the literature on previously reported cases in Japan is presented. The possibility of other alimentary obstructions should be considered when anorectal anomalies are present.     

肛门直肠畸形术后患儿行为问题的研究

目的探讨肛门直肠畸形术后患儿行为问题的状况.方法随访67例肛门直肠畸形术后患儿,在肛门功能临床评分基础上应用Achenbach's儿童行为问题量表调查其行为问题状况.结果67例肛门直肠畸形术后患儿中,13例(19.4%)存在行为问题,显著高于对照组.9例重度便失禁(肛门功能临床评分0～2分)患儿中,6例存在行为问题,明显高于其他患儿.结论严重便失禁影响肛门直肠畸形患儿行为问题,应进行长期随访,提高其生活质量.     

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.     

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.     

Mayer-Rokitansky syndrome and anorectal malformation

Mayer Rokitansky Kuster Houser syndrome (MRKH syndrome) is characterized by Mullerian duct structures agenesis, vaginal atresia being the commonest variant. It can be associated with renal, skeletal, spine and other malformations. Patient with Mayer Rokitansky syndrome has a varied presentation from newborn period to adolescence. Thorough investigations are required for classification of the syndrome and diagnosis of associated anomalies. The MRKH syndrome patient may require complex vaginal reconstructive surgery and a detailed counseling about the potentials of menstruation and fertility. Here we are presenting a patient having association of anorectal malformation, Mullerian duct agenesis and renal anomaly.     

Wnt5a在肛门直肠畸形患儿末端直肠组织中的表达及意义

目的：通过检测Wnt5a在肛门直肠畸形（ARM）患者末端直肠壁的表达,探讨其与ARM之间的可能关系。方法：应用免疫组化和Western blot方法检测20例ARM患儿、7例后天性肠瘘和6例非胃肠道疾病患儿(对照组)末端直肠壁Wnt5a的表达并对比分析。结果：对照组Wnt5a表达于直肠壁内肌间神经丛、黏膜层及黏膜下层,ARM组的末端直肠壁内Wnt5a表达较对照组减少,尤其高位畸形组减少更为明显。Western blot蛋白印迹结果：ARM高位组、中位组和低位组Wnt5a蛋白表达均明显低于对照组和后天肠瘘组（P<0.01）。高位组和中位组Wnt5a蛋白表达明显低于低位组（P<0.01）。后天瘘组与对照组比较差异无统计学意义。结论：Wnt5a在ARM末端直肠的表达水平减低,可能与ARM的发生密切相关。     

A rare association of diphallus,colonic duplications,ileal atresia,and an anorectal malformation

Diphallus is a rare congenital anomaly that is often associated with various other anomalies of the midline structures of the posterior portion of the body. We report a unique case of a 1-day-old male with complete diphallus, incomplete bilateral tubular duplications of the colon, ileal atresia, and an anorectal malformation.     

先天性肛门直肠畸形儿童生存质量影响因素研究

目的 分析影响先天性肛门直肠畸形(anorectal malformation,ARM)儿童术后生存质量(quality of life,QOL)的相关因素,为进一步制定综合性干预措施提供依据,以提高ARM患儿的QOL.方法 用中文版PedsQLTM4.0量表,对100例2岁和5岁ARM的儿童进行问卷调查,计算QOL总分及各维度得分,采用单因素及多因素分析QOL的影响因素.结果 2岁ARM患儿QOL单因素分析结果显示:临床分型、排便障碍和家庭居住地对患儿QOL各维度得分以及总分有不同程度的影响(P＜0.05);5岁ARM患儿QOL单因素分析结果显示:临床分型、排便障碍和母亲文化程度对患儿QOL各维度得分以及总分有不同程度的影响(P＜0.05).2岁ARM患儿QOL多元逐步回归分析结果显示:家庭居住地和排便障碍是影响ARM儿童生理功能得分的主要因素(P＜0.05),排便障碍是影响社交功能得分的主要因素(P＜0.05),排便障碍和家庭居住地是影响QOL总分的主要因素(P＜0.05);5岁ARM患儿QOL多元逐步回归分析结果显示:母亲职业和临床分型是影响ARM儿童生理功能得分的主要因素(P＜0.05),排便障碍是影响情感功能得分的主要因素(P＜0.05),临床分型和排便障碍是影响社交功能得分的主要因素(P＜0.05),母亲职业是影响学校表现的主要因素(P＜0.05),排便障碍和临床分型是影响QOL总分的主要因素(P＜0.05).结论 2岁ARM患儿的QOL受到家庭居住地和排便障碍的影响,5岁ARM患儿的QOL受到母亲职业、临床分型和排便障碍的影响,应针对这些影响因素给予积极干预,从而提高患儿的QOL.     

Management of anorectal malformation without ligation of fistula: An approach preventing posterior urethral diverticula

ObjectiveThe posterior urethral diverticulum is a common urologic complication requiring reoperations in anorectal malformation cases (ARM). We present a series of 24 cases of male ARM managed without ligation of fistula.Material and methodsA prospective study was conducted between July 2010 and June 2012 including male neonates with ARM, where rectobladder neck and rectoprostatic fistula were approached by the abdominal route. The fistulous tract was dissected to the distal-most possible length and was excised flush with the urethra without its ligation. A per-urethral catheter was placed in situ. A record was made of any features of urinary leak and a micturating cystourethrogram was performed at the 1-year of follow-up.ResultsTwenty-four cases of ARM, 16 with rectobladder neck fistula and eight cases with rectoprostatic fistula were included. Of these, 12 had single-stage primary abdominoperineal pull-through and 10 were managed by primary posterior sagittal anorectoplasty. Two cases with colostomy during the neonatal period were managed by laparoscopic assisted anorectoplasty at 6 months. None of the cases had a urinary leak during the postoperative period. All had a normal micturating cystourethrogram at 1 year.ConclusionsThe approach of dividing fistula without ligation may prevent posterior urethral diverticula, but larger long-term follow-up studies are needed.     

Vitamin-K status beyond the neonatal period

Vitamin K-dependent clotting factors (FII, FVII, FX) and PT and TT were studied prospectively in 113 healthy newborns during the first 6 weeks of life. No vitamin K prophylaxis was given. Infants were divided into three groups: breast fed, formula fed or combination. The method of nutrition could not be found to influence the coagulation status.     

Necrotizing enterocolitis beyond the neonatal period

Necrotizing enterocolitis (NEC) is usually considered to be a neonatal disease, and is rarely described beyond the newborn period. During the last 15 years, 19 infants from the Negev region, Israel, with NEC were beyond the neonatal age group (range=34–616 days, median-90 days). Of this group only 16% were born prematurely, and only 16% had perinatal or neonatal pathology. Diarrhea and dehydration preceding NEC were common as was malnutrition. Bacteremia was detected in 42%. The mortality rate was 90%. Malnutrition, supposedly contributed to the formation of NEC and to the high mortality rate among these infants.     

Acute retention of urine due to prolapsing stoma in a case of anorectal malformation with bladder neck fistula

This case report describes an extremely premature infant who was born with a high anorectal malformation requiring a colostomy soon after birth. He later developed multiple episodes of acute urinary retention complicated by bilateral hydronephrosis and acute renal failure. The cause of the retention was found to be the prolapsing stoma, which was kinking the bladder neck.     

Repair of anorectal anomalies in the neonatal period

In a 3-year period between 1986 and 1988, 147 patients with anorectal anomalies were seen in our department; 93 were neonates, of which 35 had low malformations treated by anoplasty soon after birth. Fifty-eight had high and intermediate types; 26 of these had defunctioning colostomies. One of the 26 patients had the definitive repair done in the neonatal period, the other 32 were operated on with posterior sagittal anorectoplasty (PSARP) as a one-stage procedure in the neonatal period. The majority (85%) were operated on within 4 days of birth. There was no operative mortality. The commonest complication was wound infection, which was mild in 15 patients and severe in 2. There were no anal strictures. The functional results were good in 30 and average in 3 patients. A comparison with 60 older children who had a similar operation done as a staged procedure in the same period showed equally low morbidity and good functional results. Based on these results, it was found that one-stage repair of high and intermediate types of anorectal anomalies in the neonate using PSARP is safe and feasible.     

Covered exstrophy associated with an anorectal malformation: A rare variant of classical bladder exstrophy

We report the case of a male newborn with covered bladder exstrophy, high anorectal malformation, and rectourethral fistula. The child had a split symphysis and diverging rectus muscles in the infraumbilical region. The ventral part of the bladder was covered with thin, fragile skin and some portions of the bladder bulged out as abdominal-wall hernias. Two of these hernias were located just above the penis, and the overlying skin showed a resemblance to scrotal skin. The penis was small and slightly laterally displaced, but otherwise normal; the child also had unilateral reflux into a dysplastic left kidney. The bladder neck and posterior urethra were patulous, but there was no urinary incontinence. The child underwent a singlestage reconstruction of the exstrophic lesion and a staged repair of the anorectal malformation. The clinical significance of this entity is discussed and the literature reviewed.