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ORIGINAL ARTICLE
Year : 2019  |  Volume : 12  |  Issue : 2  |  Page : 122-130  

Anorectal malformations: Early outcome analysis from a high-volume tertiary care institute


Department of Paediatric Surgery, SMS Medical College, Jaipur, Rajasthan, India

Date of Submission13-Apr-2018
Date of Acceptance05-Jul-2018
Date of Web Publication25-Mar-2019

Correspondence Address:
Rahul Gupta
Department of Paediatric Surgery, SMS Medical College, Jaipur, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mjdrdypu.mjdrdypu_59_18

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  Abstract 


Context: Associated malformations in anorectal malformation (ARM) are of important implication with regard to final outcomes since the anorectal lesions that cause intestinal obstruction are correctable with operative intervention. Aims: To study the presentation, types of anomalies, associated malformations, and procedures performed in relation to the type of anomaly and early outcomes analysis of ARM patients in the neonatal period. Settings and Design: A retrospective study was performed from January 2016 to December 2016. Subjects and Methods: The study included all patients with ARM admitted in the Neonatal Intensive Care Unit. Results: There were 216 neonates having ARM with 173 males and 43 females (m:f = 4:1). High-type ARM was seen in 177 (81.94%) cases, while low type in 39 (18.06%) neonates. Associated malformations were documented in 67 (31.02%) neonates. Esophageal atresia (EA) was the most common (51) associated anomaly. Among the 200 procedures undertaken for ARM, left transverse loop colostomy was the most common (132). Only 83 (38.42%) neonates were diagnosed on the 1st day of life. There were 67 (31.02%) deaths, 145 (67.13%) survivors, and 4 (1.85%) cases left against medical advice. There were only 9 (17.65%) survivors among those associated with EA. Cardiovascular was the most common (39) cause of mortality followed by septicemia (30). Conclusions: An overall high mortality rate of 31.02% and an extremely high rate (74.51%) among those associated with EA were present. In spite of the progress made in the field of neonatal care, associated malformations compounded with a high volume of patients in our resource-limited institution negatively influence the outcome of ARM in the newborn period. Strict infection control measures for prevention of septicemia and investigations for other associated malformations are recommended to improve the outcomes.

Keywords: Anorectal malformation, associated malformations, colostomy, early outcome analysis, high volume


How to cite this article:
Gupta R, Gupta AK, Shukla AK, Chaturvedi V, Sharma P, Tanger R. Anorectal malformations: Early outcome analysis from a high-volume tertiary care institute. Med J DY Patil Vidyapeeth 2019;12:122-30

How to cite this URL:
Gupta R, Gupta AK, Shukla AK, Chaturvedi V, Sharma P, Tanger R. Anorectal malformations: Early outcome analysis from a high-volume tertiary care institute. Med J DY Patil Vidyapeeth [serial online] 2019 [cited 2019 Jun 16];12:122-30. Available from: http://www.mjdrdypv.org/text.asp?2019/12/2/122/254777




  Introduction Top


Anorectal malformations (ARMs) comprise wide spectrum of diseases, affecting both sexes.[1] ARM is diagnosed because of the absence of an anus or the presence of an ectopic anus at birth.[1] It is essential to diagnose accurately all the associated malformations in ARM as soon as possible, as these have important implication with regard to final outcome of the neonate since the anorectal lesions (majority of cases) that cause intestinal obstruction are correctable with operative intervention.[2] Optimal surgical management of a neonate with ARM is directed according to the level of anomaly as well as the presence or absence of a fistula between the anorectum and urinary tract or vagina.[3] We aimed to study the presentation, types of anomalies, associated malformations, radiological investigations in the diagnoses, procedures performed in relation to the type of anomaly and associated malformations, and early outcomes analysis of ARM patients in the neonatal period at our high-volume tertiary care institute.


  Subjects and Methods Top


All neonates with ARM admitted in the Neonatal Intensive Care Unit (NICU) of the department of pediatric surgery over a period of 1 year from January 2016 to December 2016 were retrospectively studied. A detailed history and careful physical examination was completed. An invertogram was performed in whom the examination did not reveal the type of ARM. In female patients with cloacal malformation/vestibular fistula in whom the diagnosis was established by perineal examination, a babygram was performed to diagnose the presence of congenital pouch colon (CPC). ARM was classified into high and low types according to the Krickenbeck classification.[4]

Ultrasonography of the abdomen was performed to evaluate for cloacal malformation. Routine use of ultrasonography for urological anomalies was not contemplated due to resource limitations. Associated anomalies that were either obvious or life-threatening requiring urgent intervention were identified. As the resources were restricted (nonavailability of bedside echocardiography [Echo]), all the patients were not subjected to Echo for complete cardiac evaluation. VACTERL association was defined as follows: (a) if the patient had 3 or more anomalies of the vertebral, anorectal, cardiac (excluding patent ductus arteriosus, patent foramen ovale), tracheoesophageal fistula (TEF), renal/genitourinary, and limb systems and (b) if the patient had both the “core” features, i.e., ARM and TEF.[5] There should be absence of any clear evidence for an alternate, overlapping diagnosis.

Intravenous fluids, nasogastric tube placement, and antibiotics were continued postoperatively. Gradual feeds were initiated after postoperative passage of meconium or when colostomy started functioning. In cases associated with esophageal atresia (EA), before starting feeds, a contrast study was undertaken on the 7th postoperative day.

Patient's age at presentation, sex, clinical presentation, and type of anomaly, preoperative investigations undertaken for establishment of diagnosis, associated malformations, intraoperative findings (operative records), postoperative complications, and treatment outcomes were analyzed and charts were prepared. EA was classified according to the gross anatomic classification.[6]

Exclusion criteria included

  1. Female ARM patients presenting beyond the neonatal period (e.g., female ARM with vestibular fistula or low-type anomalies)
  2. Males with high- or low-type ARM presenting beyond the neonatal period
  3. Patients with cloacal exstrophy, sirenomelia syndrome, and caudal regression syndrome
  4. Patients presenting with colostomy or other operative interventions performed outside the institution were also excluded from the study.


The indications for admission for vestibular fistula in NICU were either abdominal distension following failure to pass meconium or the presence of life-threatening associated malformations.

Technique

Usually, the colostomy performed for high-type ARM was either left transverse or high sigmoid loop colostomy. (a) In case of left transverse colostomy, a transverse incision was made in the left upper abdomen slightly above the mid-point between the umbilicus and costal margin. Transverse colon was appreciated just below the incision, recognized by the presence of greater omentum. (b) In case of high sigmoid colostomy, a left oblique incision was made in the left lower abdomen midway between the umbilicus and anterior superior iliac spine. Small bowel loops were retracted; sigmoid colon was recognized by the presence of its mesentery (sigmoid mesocolon) and taeniae coli and subsequently sigmoid colon was delivered.

In both transverse and sigmoid colostomy procedures, colon was exteriorized (brought out) as a loop and elevated using a nontraumatic rubber anchoring tube which was passed through a window in the mesentery (beneath the mesenteric border of the bowel). This prevented the loop from receding intraoperatively and also resulted in good bridge formation and prevention of retraction in early (3–5 days) postoperative period [Figure 1]. One or two seromuscular sutures were applied between proximal and distal end at the base of the loop. Meticulous anchoring, fixing the loop with peritoneum and fascia, followed by formation of good bridge between the two loops, was performed. After fixation, the loop was opened with the diathermy and edges of the stoma were stitched with the skin. We made sure that a good bridge is created between the two loops [Figure 1]. Distal loop wash with normal saline was done at the end of the procedure to remove the meconium from the distal loop. Breastfeeding was started after colostomy started functioning. Colostomy care was explained to the mother. Distal loop wash was also performed during postoperative period (before discharging the patient from the hospital) and during follow-up visits to prevent the fecaloma formation.
Figure 1: Nontraumatic rubber anchoring tube (ventriculoperitoneal shunt tubing) preventing retraction of colon intraoperatively and in early postoperative period (a-d); Left transverse loop colostomy with good bridge between proximal and distal loops (e-i)

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In patients with CPC, fistula ligation and either (a) pouchostomy (in Type 1 and 2) or (b) excision of pouch and end colostomy (and end ileostomy) were performed. Low-type malformations were perineal fistula, bucket handle anomaly, covered anus, and anal stenosis.

The patients were prescribed prophylactic antibiotics for prevention of urinary tract infection/reflux nephropathy. All neonates were followed up for 1 month and early complications were recorded. Various parameters were reviewed to analyze the risk factors associated with the morbidity and mortality. Long-term follow-up was not included in the study as compliance is very poor in our setup.


  Results Top


The results of the study are summarized in [Table 1], [Table 2], [Table 3], [Table 4], [Table 5], [Table 6], [Table 7]. There were 216 neonates having ARM with 173 males and 43 females (m:f = 4:1) as shown in [Table 1]. High-type ARM was seen in 177 (81.94%) cases, while low type in 39 (18.06%) neonates [Table 1]. The average birth weight was 2250 g (range 900–3250 g). Associated malformations were documented in 67 (31.02%) neonates (more than one anomaly was present in some cases). The percentage of associated malformations was more in females (44.19%) than males (27.75%) as shown in [Table 2]. Associated malformations were present more in high-type anomaly (31.64%) than low-type ARM (28.20%) as shown in [Table 3]. High-type male ARM (with/without rectourinary) was the most common variety constituting 134 (62.04%) neonates, followed by low-type anomaly (16.36%) in males [Table 1]. Persistent cloaca was the most common indication for admission (6.07%) in the female group [Table 1]. CPC was confirmed in 18 patients (Type 1 = 2, Type 2 = 3, Type 3 = 2, and Type 4 = 11), of which 2 were seen in persistent cloaca, 3 with female high-type ARM, and 13 with male high-type ARM.
Table 1: Type of anomalies in the present study

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Table 2: Percentage of associated malformations as per sex distribution

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Table 3: Percentage of associated malformations as per the type of anomaly

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Table 4: Summary of associated malformations in the present study

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Table 5: Summary of procedures performed for anorectal malformations

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Table 6: Summary of procedures undertaken for various associated malformations in the present study

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Table 7: Outcome of patients as per the day of admission and its association with or without esophageal atresia in the present study

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A total of 101 anomalies were identified, with gastrointestinal anomalies being the most common association. EA (51) was the most common individual anomaly associated with ARM in our patients; duodenal atresia was the second most common (7) malformation among the gastrointestinal group [Table 4]. Of 51 cases of EA, Type C – 44, Type A – 6, and esophageal web – 1 were present. Thirty-one patients with high-type male and three cases with high-type female ARM had EA; seven cases of low-type ARM were associated with EA. Of 13 patients with cloacal malformation, there were 3 neonates who had associated EA, while there were 7 cases of vestibular fistula associated with EA. Triple atresia was present in 4 neonates (Type C – 3 and Type A – 1) in the study. There were 51 patients with VACTERL association. EA, DA, and low ARM along with hydrocephalus suggestive of “VACTERL-H” association were seen in one of our female patients.

Invertogram was performed in all the patients in the study; also, a babygram was performed in cases suggestive of CPC and those neonates with associated EA (red rubber catheter in situ) and other malformations. Cardiovascular anomalies could be documented in 12 (5.55%) neonates among all the patients. Only 25 patients could be evaluated with Echo due to resource limitations; thus, 48% (12) of cases had cardiovascular anomalies among those evaluated with Echo. Urogenital malformations were identified in 12 (5.55%) neonates. Skeletal malformations were seen in 8 (3.7%) neonates; severe scoliosis due to multiple vertebral defects was present in 4 cases in the study. Other anomalies are summarized in [Table 4].

Among the 200 procedures undertaken for ARM [Table 5], left transverse loop colostomy [Figure 1] was the most common (132) for high-type anomaly. It was followed by posterior sagittal anoplasty in 34 neonates with low ARM. A total of 18 patients received end stoma in patients with CPC. Other important procedures performed for high type were fistula ligation, excision of CPC and end colostomy in 14 and sigmoid loop colostomy in 7 cases. All procedures were performed between 2 and 48 h following diagnoses and under the supervision of the consultants. Of 216 patients, 4 patients left against medical advice (LAMA). Five patients died before any intervention could be contemplated. In 7 patients with vestibular fistula associated with EA, only thoracotomy was performed (for primary correction of esophageal pathology).

Intraoperative surgical complications during repair of ARM include bleeding (3) and serosal tears in intestine during sigmoid loop colostomy (3). Three patients had pneumoperitoneum due to bowel perforation; two patients had perforation in the colon, while in the remaining case, it was present in the pouch. The total number of procedures performed for associated malformations was 58 [Table 6]. Only 83 (38.42%) neonates were diagnosed on the 1st day of life. The rest (61.58%) were diagnosed late, ranging from 2nd to 15 days of life [Table 7].

Postoperative complications among the survivors (including during 1-month follow-up) were septicemia with thrombocytopenia (11), pneumonia (6), wound infection (5), wound dehiscence (2), colostomy prolapse (2), and colostomy retraction (1). There were 67 (31.02%) deaths, 145 (67.13%) survivors, and 4 (1.85%) cases with LAMA [Table 7]. There were only 9 (17.65%) survivors among those associated with EA, while there were 136 (82.42%) survivors among those without EA [Table 7]. The survival outcomes (on the basis of the day of admission) among those without EA than with EA were statistically significant (P < 0.01). Of 67 deaths, 38 were associated with EA and 29 were those without EA. Among the patients who died, 53 neonates were male and 14 were female; 59 belonged to high-type ARM and only 8 were low type. Most cases (28) died between 24 and 72 h (2–3 days) after surgery, followed by 18 cases within first 24 h (<1 day), 14 neonates beyond 72 h (4–7 days), and 7 patients beyond 1st week after surgery. More than one reason could be attributed to the cause of mortality. Cardiovascular was the most common (39 cases) cause of mortality. It was followed by septicemia (30), fluid overload (8), feeds before admission, aspiration pneumonia (5), and intra-operative surgical complications (4).


  Discussion Top


ARM comprises a complex group of diseases (1:5000 live births) involving the anorectum as well as genitourinary system.[1],[2],[3] It is a spectrum disorder with minor defect to complex malformation (persistent cloaca) with poor functional outcomes.[7] High-type ARM constituted 81.94% cases in our study, similar to other studies.[8] The most common defect in males is imperforate anus with a rectourethral fistula.[7],[8],[9] Perineal fistulae in both sexes, and male scrotal fistulae, are regarded as external fistulae and classified as low-type anomaly. Low-type ARM was present in 1/5th of the patients in our study, while more than half of the patients constituted low-type in a European study.[10]

The most common defect in females is vestibular fistula.[7] It's percentage was low (5.61%) in our study as only those cases with either life-threatening associated malformations requiring (urgent) surgical intervention or those presenting with subacute obstruction due to nonpassage of meconium were admitted in NICU. Vestibular fistula is being managed with primary posterior sagittal anorectoplasty (PSARP) at a later date.

A patient with persistent cloaca presents with a single perineal orifice; a lump representing distended vagina (hydrocolpos) may be present in up to 50% of patients [Figure 2].[7] In our study, persistent cloaca was present in 6.07% (13) of patients, which was similar to a large (over 20 years) European series.[10] Furthermore, hydrocolpos was present in two cases for which vaginostomy was performed. CPC was documented in 2 of 13 cases with persistent cloaca. Urological anomalies may be present in 90% of patients with persistent cloaca, and obstructive uropathy must be dealt in the neonatal period.[7],[11] There was one patient with a rectovaginal fistula with two separate openings (urethral and vaginal) in the perineum, although Levitt and Pena disagree on this entity.[7]
Figure 2: Clinical photographs showing (left) small genitalia in a patient of persistent cloaca (with single perineal opening, as seen in the inset image); (right) distended vagina (hydrocolpos) presenting with suprapubic lump below and transverse loop being delivered in the wound (above) is seen

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An “invertogram” or “prone cross-lateral film” is performed in 10%–20% neonates when clinical signs do not reveal the type of ARM by 24 h.[9],[12],[13] In our series, all the neonates were subjected to invertogram and babygram to diagnose associated gastrointestinal and other malformations and also because of high incidence of CPC in our geographical area.[14],[15]

Associated malformations in ARM may occur in approximately 50%–60% of patients.[16] The incidence of associated anomalies is higher in patients with high ARM than in those with low ARM.[7],[16] In our study, 31.02% of patients had associated anomalies (more in high than low type). In one Asian study, 63.8% of the patients had at least one associated anomaly,[17] while in a European study, 58% patients had major associated anomaly.[18] In our study, the yield was low (31.02%) owing to underevaluation of urological anomalies. Urogenital anomalies are the most common and may present in 50%–60% of cases with high-type ARM and 15%–20% of patients with low-type ARM.[19] Various studies have shown that the vesicoureteral reflux (VUR) is seen in one-third of cases, ranging from 19% to 47% in patients with ARM.[9],[11] VUR is one of the most common genitourinary anomalies in ARM.[8],[9] Urogenital anomalies could not be evaluated in our study due to resource limitations and poor compliance in follow-up. Cardiovascular anomalies are present in 1/3rd of cases with ARM,[20] but in our study, only 5.55% were detected with the malformation.

In the present study, gastrointestinal anomalies were seen in 27.31% (59) patients, while it was 20% in one study.[18] VACTERL association refers to the nonrandom co-occurrence of vertebral anomalies (V), anal atresia (A), congenital heart defects (C), TE, renal anomalies (R), and limb defects (L).[5] VACTERL association was documented in 23.61% of patients in our study, while in other study, it was 8.9%.[21] The percentage in our study would have increased if more evaluation of cardiac and urological malformations was possible in our recourse-limited setup. Furthermore, VACTERL-H association was appreciated in one of our patients. This patient was a female, which is in contrast to previous reports in the literature.[5]

In all patients under the study, the presence of EA was sought by passing No. 10 sterile, blunt-tipped, soft, red rubber catheter into the esophagus. Eleven patients were admitted primarily for EA (referred with diagnosis of EA) and ARM was diagnosed after admission, while in 40 patients, the diagnosis of EA was confirmed later by performing red rubber catheter test. Thus, importance of red rubber catheter test cannot be overemphasized. The number of patients with EA in our study was very high (23.61%) compared to other studies with reported percentage of 6.9%–10%.[17] In a 7-year period from 2010 to 2016, a total of 1554 neonates with EA were managed at our institute.[22]

Posterior sagittal anoplasty is the surgical method of choice for a newborn whose rectal pouch terminates close to the perineal skin (low-type anomaly), whereas colostomy is usually chosen for cases with a high-lying rectum and/or intermediate type (internal fistula).[9]

Reconstruction in high-type ARM needs more extensive surgical procedure and is typically delayed until the infant gets older.[9] Colostomy is essential and is performed as a temporary procedure soon after birth in high-type male ARM and especially in females with persistent cloaca and rectovaginal fistula.[1],[2],[3] Even though a new trend of primary repair without colostomy in high-type ARM has been reported, most pediatric surgeons prefer a protective colostomy before the definitive surgery.[3] Colostomy itself helps investigate the malformation, as level of anomaly, rectourinary fistula, and bladder silhouette can be visualized by well-tempered pressure augmented distal colostogram.[3],[23]

Two varieties of colostomy are generally performed in high-type ARM, i.e., loop and divided colostomies. In a divided colostomy, both ostomies are placed wide enough apart so that a stoma cap can be placed on the afferent loop without covering the efferent loop.[18] Divided colostomies are not performed in our institution and only loop colostomies are performed. Although divided colostomy is ideal and also recommended by Pena and other authors to achieve complete defunctioning as it helps in preventing passage of fecal matter between proximal and distal limb which may lead to fecal impaction, megarectum, bacterial overgrowth, and fecal contamination of urinary tract (due to rectourinary fistula),[3],[7] But divided colostomy requires meticulous technique, time-consuming compared with loop colostomy, especially when the patient presents late with septicemia and marked abdominal distension. In simple loop colostomy, the bowel is sutured to the abdominal wall and is not completely divided. Simple loop colostomy with or without spur works quite well to relieve the obstruction, particularly in emergency.[24],[25]

The most commonly used sites are the high sigmoid colostomy/descending colon followed by left transverse colostomy.[18],[26]

High sigmoid colostomy

Approaching the sigmoid colon and identifying its proximal and distal parts is difficult when a patient presents late with abdominal distension and markedly dilated small bowel (especially when the child has been fed and is having septicemia). This procedure causes excessive handling of the bowel resulting in serosal tears. With this procedure, there is larger average size of the incision and more time for the procedure. Furthermore, placement too distally (faulty placement of sigmoid colostomy near recto-sigmoid region) may result in insufficient length and interfering with the mobilization of the rectum during the reconstruction and necessitate a revision of the sigmoid colostomy [Figure 3].[26] A more proximal stoma needs to be created with closure of earlier colostomy, followed by abdominoperineal pull-through or PSARP.[26] In our experience, this revision due to defective distally placed sigmoid colostomy is a major technical error that compromises the vascularity of distal pull-through segment and results in more bowel handling and postoperative adhesions.
Figure 3: Distal colostogram before the 2nd stage definitive procedure showing rectourethral fistula (red arrow) joining with bulbar urethra (yellow arrow); short distal segment is seen (a) with faulty placement of sigmoid colostomy near rectosigmoid region (b); very short distal segment (c and d) with wide rectobladder neck fistula (blue arrow); (B = Bladder, R = Rectum)

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Left transverse colostomy

Transverse colostomy is performed in the left upper abdomen; transverse colon is usually present below the incision. When not appreciated, a single moist gauze piece was inserted cranially to take out the omentum along with it, followed by delivery of transverse colon into the incision. If in spite of these maneuvers, transverse colon is not seen, and a large dilated loop was seen on preoperative abdominal radiograph, then diagnosis of CPC (Type 1–3) should be considered (personal experience). The transverse colostomy is easy to perform, smaller size of the incision, lesser intraoperative time, and fewer intraoperative complications. Also, maintaining hygiene of the stoma site is easier with it than high sigmoid colostomy. The indications of left transverse colostomy are suspicion of wide bladder neck fistula (the presence of both meconuria and pneumaturia) and Type 4 CPC.[27] The disadvantage with left transverse colostomy is that it carries the high risk of prolapse because the transverse colon is more mobile than the sigmoid colon.[7],[18]

In our study, a significantly large percentage (61.11%) of neonates underwent transverse loop colostomy, while sigmoid loop colostomies were performed in 3.24% of neonates only.

In our study, overall postoperative complication rate was 12.5% among patients and 18.62% (27) among the survivors (145); in a Western study, complication rate was 23%.[18] Prolapse was seen equally (one each) in both sigmoid and transverse loop colostomies in the present study. The prolapse was minor and reducible (both the cases) and not necessitating colostomy revision. This complication was very rare in our series as compared to European study. Prolapse can be avoided by creating a small fascial defect, applying seromuscular sutures between the loops, and meticulously anchoring the loop with fascia (personal experience). Prolapse is more in transverse loop colostomy as compared to sigmoid loop colostomy due to relative mobility of transverse colon, which has also been observed in few studies.[18],[26],[27],[28] The proximal limb prolapses in transverse loop colostomy and distal limb in sigmoid loop colostomy. In our series, only 3 patients required colostomy revision due to colostomy retraction (1) and wound dehiscence (2). Pericolostomy excoriation was not part of the study because of the inconsistency in defining this complication (skin excoriation). One study mentions a very high incidence of skin excoriation, while only a small percentage of cases required hospital admission.[29] Pericolostomy excoriation was seen in our patients and our patients received prebiotics, topical application of zinc oxide, steroid creams, etc., for its management. Thus, mild cases of skin excoriation having less clinical importance were not included in the complications. The other complications mentioned in the literature are intestinal obstruction (adhesion, volvulus, and intussusception).[26] Urinary tract infection rate was not calculated in the present study owing to 1-month follow-up only.

Mortality in our series was high (31.02%) as compared to Western studies (6%).[18] Mortality rate may range from as low as 0.1% to 11%.[28],[29] The reasons for very high mortality rate were due to delayed referral owing to delayed diagnoses (lack of trained workforce and limited resources in peripheral health centers). These neonates usually present late with markedly distended abdomen with respiratory distress (owing to markedly distended abdomen) to our tertiary care center (Indian subcontinent). Furthermore, there is a history of feeding before admission along with pneumonia, especially those associated with EA (our high-volume tertiary care institute is dealing with an increased number of patients with EA), undiagnosed other associated malformations (cardiovascular anomalies), and VACTERL association which increases the morbidity. Associated cardiovascular anomalies were the most common cause of mortality in our study (diagnosis by exclusion, the presence of cardiac murmur, and VACTREL association).

There was a high incidence of associated EA (23.61%) in our series and low survival rate (17.65%) among them. Mortality in our series was not directly related to the type of stoma/colostomy and which was also reported by a recent study.[18]

Compounded with above factors, there is a high volume of patients and overcrowding in NICU which leads to cross infection and high incidence of septicemia, leading to increased mortality rate in our setup. In one Western study involving over 10 years, 200 cases were analyzed,[2] while in one study from Iran, only 105 patients underwent surgery over a 10-year period.[8] We are managing over 200 neonates in a single year, which is a very high volume of neonates with ARM at our center.

Recommendations for improving the favorable outcomes are as follows:

  1. Improvement of facilities for the diagnoses of associated malformations, particularly cardiac anomalies with the help of bedside Echo and urological malformations with abdominal ultrasonography
  2. Round-the-clock neonatal care under neonatologist (not available in our setup)
  3. Strict infection control measures
  4. Improvement in the nursing care delivery system
  5. As most of the genital malformations are detected by careful clinical examination, urologic anomalies entail additional evaluation for their detection. Ultrasonography of abdomen and microbubble contrast ultrasound must be performed for all the cases with ARM. Diagnosis of associated anomalies can improve prognosis of the patients with ARM. Thus, prospective study to evaluate the frequency of urogenital and other associated anomalies is further recommended to improve the final outcomes of patients with ARM.



  Conclusions Top


Approximately 2/3rd of cases were high-type male ARM, and associated major anomalies were documented in approximately 1/3rd of patients. Left transverse loop colostomy is more effective and easy to perform in neonates presenting late with markedly distended abdomen and has fewer complications as compared to sigmoid loop colostomy. An overall high mortality rate of 31.02% and an extremely high rate (74.51%) among those associated with EA were present. Survival outcomes were better in ARM without associated malformations. In spite of the progress made in the field of neonatal care, associated malformations especially EA and VACTREL association compounded with a high volume of patients in our resource-limited institution negatively influence the outcome of ARM in the newborn period. Strict infection control measures for prevention of septicemia and investigations for other associated malformations, especially cardiovascular and genitourinary, are recommended to improve the outcomes.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
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    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1], [Table 2], [Table 3], [Table 4], [Table 5], [Table 6], [Table 7]



 

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