Management of AnoRectal Malformations in a Tertiary Level Children ’ s Hospital of Nepal

Introduction: Ano-rectal malformation (ARM) comprises a wide spectrum of diseases that involves the distal anus and rectum as well as the urinary and genital tracts. The factors that need to be considered during treatment are the type of anomaly, associated anomalies and patients general condition. The aim of this study is to analyze the types of anorectal malformations and their management in a tertiary level children’s hospital. Materials and Methods: The medical records of patients with the diagnosis of anorectal malformation managed in the hospital during a period of January 2009 to January 2014 were reviewed. Results: There were 187 cases of ARM 126 (67.37%) males and 61 (32.62%) females. High/Intermediate type was 129 (68.98%). Low ARM was 58 (31.01%). In male high type ARM with recto-urethral fistula was seen in 60 (47.61%). ARM with Rectovestibular fistula(RVF) was seen in 40 (65.5%) female. High and intermediate ARMs were managed initially with colostomy and low ARM with primary anoplasty. ARM with rectovestibular fistula in females were managed with Anterior Sagittal Anorectoplasty without colostomy. Common cloaca in female was managed initially with colostomy followed by posterior sagittal ano-recto-vagino-urethroplasty. Colostomy closure was done after adequate size of anus was achieved with anal dilation. Conclusion: The type of ARM in newborn is diagnosed by perineal examination and X-rays. Low type ARM was managed by primary surgery without colostomy. High anomaly and complex defect was treated with stage surgeries.


Introduction
A no-rectal malformations are congenital malformations in which the distal part of the hindgut fails to develop or develops partially leading to various anomalies.Imperforated anus is one of the commonest congenital ano-rectal malformation in newborn with an incidence of 1 per 4000-5000 live births.Early assessment and accurate diagnosis of the type of anorectal malformation are essential for determining which type of surgery is needed.In addition to the clinical diagnosis different imaging techniques are used like invertogram or prone cross table lateral fi lm, ultrasonography and MRI 1,2,3,4,5 .
The main aim of the management of ARM is t o relieve intestinal obstruction if present; restore anorectal continuity with optimal sphincter function, early postnatal establishment of brain-defecation refl ex, reduction of the physical and psychological stress to the family 6,7,8 .
Wingspread classifi cation in 1984 proposed a working formulation in which this anomaly is classifi ed into three types as high, intermediate and low depending upon the site of the blind rectal pouch in relation to the levator and puborectlis muscle.This classifi cation is more precisely defi ned now in combination with an associated fi stula 9,10,11,12 .
Fistula are found in 80-90% cases of ARM between the lower end of rectum and the perineum, female genitalia or urinary tract.Anorectal malformations can also be associated with other external and internal anomalies especially of genitourinary system 13,14 .

Thapa B et al
High type is more common in male while intermediate more in female.Low type ARM is corrected surgically in a single stage at neonatal period.Colostomy is performed in cases of intermediate and high types followed by anorectoplasty at the later stage and then colostomy closure.With early diagnosis, management of associated anomalies and effi cient surgical repair patient have good functional outcome 15,16,17 .

Materials and Methods
This was a retrospective study where the medical records of patients with the diagnosis of anorectal malformation (ARM) admitted and managed in Kanti Children's Hospital, Department of Surgery, between January 2009 to January 2014 were reviewed.
There were total of 187 new cases of ARM managed during the study period.Three hundred and twenty fi ve different surgeries were performed.The children included in this study were those having anorectal malformation in the neonatal period, those admitted for corrective surgery and those with colostomy closure.The data on sex, types of anomalies and surgical procedures were analyzed.Wingspread Classifi cation and location of perineal fi stula was used to diagnose ARM as high, intermediate and low type.Prone cross table lateral fi lm was done in children without visible fi stula to diagnose the types of ARM.Abdominal ultrasound was done to see any other abdominal pathology associated with ARM like genitourinary anomaly.

Results
Among the 187 new cases of ARM,126 (67.37%) male and 61 (32.62%) female were operated in their fi rst admission.Among them 139 were neonates, male 126 and female 13.Seventy six (60.31%) of total males patients had high and intermediate type ARM.All of them were managed by protective sigmoid colostomy and discharged.Fifty (39.68%) male patients had low type ARM and managed by primary anoplasty.A hundred and ten (87.30%) of total male ARM patients had fi stula.Among them 60 (78.94%) were in high and intermediate type ARM with rectourethral fi stula.Only 16 (21.05%)were without fi stula.A total of 50 (39.68%)male had low type ARM with fi stula in perineum and managed by primary anoplasty.The most common anomaly in female was ARM with rectovestibular fi stula which comprises 40 (65.57%) of total female child.They all were managed by primary anterior saggital anorectoplasty (ASARP) without colostomy.High type ARM in female without fi stula was only seen in 5 (8.19%).Common cloaca were seen in 8 (13.11%) and were managed by colostomy and planned for posterior sagittal ano-recto-vagino-urethroplasty (PSARVUP) later.Similar to males 8 females with low anocutaneous and perineal fi stula were managed by anoplasty.Distal colostogram was done in all patients with colostomy prior to defi nitive surgery.Posterior sagittal anorectoplasty (PSARP) was done in all high or intermediate types and Abdomino Posterior anorectoplasty was done in those high types in which perineal approach was not enough.Colostomy closure was done after two three months of defi nitive surgery.Anal dilation was done in all patients two weeks postoperatively until the desired size of anus.

Discussion
Anorectal malformation is one of the most common congenital anomalies encountered at birth with an absent anus or ectopic anus which is more common in males 1,2 .In our study there were 126 (67.37%%) males and 61 (32.62%) females with male to female ratio of 2:1.This fi gure is close to 65% male given in a series by Penaw 5 .The Alberta congenital anomalies surveillance system 1990-2004 shows an overall rate with male predominance (1.7:1) 8 .
The correct and timely intervention is crucial to determine the immediate future of the child.The basic principles in the management are to prevent or relieve intestinal obstruction, to establish normal defecation and to preserve or restore normal urogenital function.The plan of management depends upon whether these objectives can be realized simultaneously i.e. by single defi nitive surgical procedure or in stages.Low type anomalies in our series including female with rectovestibular fi stula were all managed by single defi nitive surgeries.Others with high, intermediate and complex anomaly were managed with colostomy.
Majority of patients with ARM present early in the neonatal period.Patridge and Gough have reported that 64.9% patients were neonates at presentation 8 .In our series we had higher incidence 139 (74.33%) of neonatal presentations.Those who presented late were mostly female with rectovestibular fi stula and cloaca that were mistaken as normal anus.
Anorectal malformations are used to be classifi ed into three main types depending on whether the blind end is above, within, or below the sphincter muscle complex (Wingspread classifi cation).This classifi cation has now been done with association with male or female to the site of associated fi stula.All cases in our series without visible fi stula underwent prone cross table lateral fi lm and classifi ed into three types with Wingspread classifi cation.In females ARM may be associated with fi stula in the perineum or vaginal vestibule and rarely to vagina.In males this is associated with fi stula to the perineum or urethra or bladder neck.That is the reason perineal examination is very important in anorectal malformation 9,10 .
Invertogram used to be the choice of radiologic investigations to approximate high, intermediate and low anomalies.But now it has been replaced by prone cross table lateral fi lm as it is much easier and less time consuming due to position in prone, hips fl exed in genupectoral position.Patient also feels calm and relaxed in this position showing the full extent of the rectal gas shadow.In our series all ARM cases without visible fi stula underwent prone cross table lateral fi lm and classifi ed.Radiological evaluations do not show the actual anatomy before 24 hours because the rectum is collapsed and it takes time for colonic gas to overcome the muscle tone of the sphincter that surrounds the lower part of rectum.Therefore radiological evaluation done before this time may show very high rectum and yield false diagnosis 11,12 .Few cases in our series referred from other local hospital underwent invertogram before 24 hours showed high ARM.After perineal examination and repeat xray after 24 hours, these showed to be of low types and managed in a single stage.
Ultrasound is also used in many centers to differentiate the types.Hans P Haber et all in their series has measured the distance between the distal blind rectal pouch and the perineum.They classifi ed as high or intermediate with the distance greater than 1.5 cm and as low type with less than 1.5 cm 12 .In our study we are only able to do in 6 cases as ultrasonography is not always feasible in emergency basis.Intra-operative fi ndings of high and low type according to ultrasound measurement in all these six cases were similar.
Pena has reported the presence of the fi stula in female patient to be over 90%.In Liverpool series 83 out of 98 had fi stula in males, 51 out of the 53 had fi stula in females 5,13 .In our series 110 males out of 126 and 56 females out of 61 had fi stula seen pre and intraoperatively.This shows ARM have fi stula with different variants like vestibular, vaginal, anocutaneous and vesicular in females and urethral, vesicular, anocutaneous in male.
The presence of an anocutaneous/perineal fi stula indicates the presence of the low type of ARM Neonates with this type of phenotype do not need further diagnostic procedures on the anorectum and undergo a surgical procedure in the fi rst day of life.In our study 50 males and 8 females had low anomaly and were managed by primary anoplasty (cut back anoplsty, Y-V anoplasty, minimal posterior sagittal anoplasty PSAP).In the children's memorial hospital series of 216 ARM patients 47% had low anomalies and in Liverpool series 74 out of 151 had low anomalies 13 .Low ARM is associated with well developed rectum, anal canal, sphincters and sensory receptors with good postoperative results.
In our series, male 76 (60.3%) and female13 (21.31%) with high or intermediate ARM were primarily managed by colostomy and later with posterior sagittal anorectoplasty PSARP.Though the defi nitive surgery PSARP in advanced centers are done at early infancy.We prefer at around one year of age as the chances of sepsis is higher in early age in our set up, anatomy is clearer while seperating fi stula.Colostomy closure is done after adequate size of anus is reached with anal dilation as many cases come in follo-up without regular anal dilation.Pena and deVires in 1982 gave description of PSARP and anal dilation which is now a landmark of surgery of high ARM 14,15 .
Although three stage surgeries have been in practice for high ARM, recently many authors have practice doing primary PSARP 16,17,18 .A. Mirshemirani et all in their series of 30 newborns with high ARM underwent primary PSARP and showed it to be safe and effective in newborns 19 .In Kanti Children's hospital, Nepal we are not yet able to do as most of the cases come late, dehydrated, with abdominal distension and post operative care for primary PSARP is diffi cult due to high chance of wound infection.
The most common variety of ARM in female in our study was rectovestibular fi stula 40 (65.5%).This anomaly was also done as three stage surgeries before.After the introduction of Primary anterior sagittal anorectoplasty ASARP in 1988, it has become a standard method.All of our cases were managed by primary ASARP and the result was good 20,21 .
In the female the number of opening in the perineum is highly signifi cant.Three openings means that the problem can be managed from perineum as rectovestibular fi stula in our series are all managed in single stage while the presence of two or only one opening (cloaca) means staged surgery.
Various studies have reported the incidence of associated anomalies with ARM to be 30 to 70% cases.The VACTERL syndrome is a specifi c groups of vertebral, anal, cardiac, tracheal, esophageal, and renal and limb anomalies.An overall incidence of 50% has been reported by De Vries.Stephens and Smith found that 60.6% had one or more associated anomalies.In our series only 36 (19.2%) cases had associated anomaly 22,23 .This low incidence in our series may be due to poor screening as our patients undergo the initial surgical treatment without detailed work up for associated anomalies soon after their admission It is because ultrasound, echocardiogram and other screening investigations are not available in our emergency services.Associated congenital anomalies in neonates with ARM is important as survival and prognosis depends upon its number and severity 24 .In our series those cases of ARM associated with esophageal atresia, down syndrome and 2 very low birth weight with suspected congenital heart disease all expired.

Conclusion
Male patients predominate in our study which shows the incidence is high in males.High type anorectal malformations are more frequent in males whereas intermediate type with rectovestibular fi stula is common in femlae.Anorectal malformations can be classifi ed clinically with prone cross table lateral X-ray fi lm and the management protocol can be made on this basis.In males low anomalies are managed with single

Table 1 :
Sex distribution children with ARM.

Table 2 :
Types of ARM and sex distribution.

Table 3 :
Types of ARM in neonates.

Table 5 :
Distribution of male defects in relation to fi stula.

Table 6 :
Distribution of female defects in relation to fi stula Management of Ano-Rectal Malformations in a Tertiary Level Children's Hospital of Nepal

Table 7 :
Associated anomalies in ARM

Table 8 :
Different surgeries performed in ARM