|Year : 2017 | Volume
| Issue : 2 | Page : 96-100
Simultaneous single-staged repair of anorectal malformation with tracheoesophageal fistula: Lessons learned
Ajay Narayan Gangopadhyay, Vaibhav Pandey
Department of Paediatric Surgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India
|Date of Web Publication||22-Mar-2017|
Ajay Narayan Gangopadhyay
House No 161, Lane No 15, Ganeshpuri, Susuwahi, 145 Nasirrpur, Varanasi - 221 011, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Introduction: Anorectal malformation (ARM) associated esophageal atresia (EA) with tracheoesophageal fistula (TEF) spawns special therapeutic propositions. The outcome of these patients banks on numerous factors. We performed this study with an aim to compare the outcome of single-staged simultaneous primary repair of both anomalies versus staged repair of these disorders.
Materials and Methods: Retrospective review of cases with ARM and associated EA with TEF managed over a period of 5 years from July 2010 to June 2015 after ethical approval was undertaken. Patients were split into two groups based on whether they underwent staged repair (Group A) or single-staged simultaneous primary repair of ARM with TEF (Group B). Patient's records were analyzed for demography, weight, gestational age, associated anomaly, preoperative and postoperative sepsis screen results, early and late postoperative complications (at least up to 1 year of age). Patient's Kelly score for continence at the age of 3 years or more was compared.
Observation: A total of 28 were included in the study. Among these, 17 were managed with staged procedure for ARM (Group A), whereas 11 underwent simultaneous single-stage repair of ARM with TEF (Group B). No difference in continence score was observed in outcome between the two groups (P = 0.96). Overall mortality in Group A at 1-year follow-up was 52.9% and in Group B was 43.4%.
Conclusion: The simultaneous single-staged primary repairs result in better long-term outcome in our setup.
Keywords: Anorectal malformation, anorectal malformation with tracheoesophageal fistula, esophageal atresia
|How to cite this article:|
Gangopadhyay AN, Pandey V. Simultaneous single-staged repair of anorectal malformation with tracheoesophageal fistula: Lessons learned. J Indian Assoc Pediatr Surg 2017;22:96-100
|How to cite this URL:|
Gangopadhyay AN, Pandey V. Simultaneous single-staged repair of anorectal malformation with tracheoesophageal fistula: Lessons learned. J Indian Assoc Pediatr Surg [serial online] 2017 [cited 2022 May 20];22:96-100. Available from: https://www.jiaps.com/text.asp?2017/22/2/96/202682
| Introduction|| |
Anorectal malformations (ARM) are one of the most frequent congenital defects with reported incidence between 1/1500 and 1/5000 live births., ARM presents with a wide panorama of defects, ranging from relatively simple low malformations to very complex cloacal anomalies. Over the past three to four decades, the posterior sagittal anorectoplasty (PSARP) has emerged as procedure of choice for the management of these anomalies. The advances in management have piloted these patients to excellent functional outcome and virtually no postoperative mortality except patients with other major congenital anomalies. One such association is ARM along with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF). Although the survival of these patients has reached to about 80%–90% in western countries, in developing countries, the mortality rates are still high. The prime reasons for high mortality are low birth weight and delayed presentation (with pneumonitis) because of late referrals., ARM conjoined with EA ± TEF spawns special therapeutic propositions. The outcome of these patients banks on numerous factors, most importantly associated anomalies.,, Further, these patients foster rapid and progressive abdominal distension. The resulting regurgitation of gastric contents into lungs leads to rapid deterioration of these patients. Managing these patients on ventilators preoperatively is a greater challenge. The resulting progressive distension of bowel pushes up the diaphragm leading to ever increasing ventilatory demand. Hence, prompt surgical intervention is mandatory for improved outcome of these children. Our previous experience with single-stage repair of ARM and its distinctive advantages in our setup prompted us to treat the patients of ARM with EA + TEF in single stage. Since then, we have been treating the patients of ARM with EA + TEF in a staged manner as well as single-staged simultaneous repair of both the disorders. We undertook this study with an aim to appraise the advantages and disadvantages of single-staged simultaneous repair of both anomalies versus staged repair of these disorders.
| Materials and Methods|| |
We executed this retrospective review of cases with ARM and associated EA with TEF managed over a period of 5 years from July 2010 to June 2015. The ethical endorsement was taken from the institute ethical committee. All cases were operated by a single surgeon ( first author). Patients of ARM with pure EA without TEF were excluded from the study. Male patients with perineal fistula and female cases with common cloaca were excluded from the study. Patients who were lost to follow-up for at least 1 year were excluded from the study. Patients were split into two groups based on whether they underwent staged repair (Group A) or single-staged simultaneous repair of ARM with TEF (Group B). The main factors which decided single-stage repair were birth weight at presentation (>2.0–2.5 kg), economic background of parents, and their unwillingness regarding staged procedures. Patient's records were analyzed for demography, weight, gestational age, associated anomaly, preoperative and postoperative sepsis screen results, early and late postoperative complications (at least up to 1 year of age).
Group A patients underwent babygram in erect posture with k-91 catheter in upper esophageal pouch, cross-table lateral view in prone position for ARM, and echocardiography. All these cases were subjected to single-stage, single-layer primary esophageal anastomosis using absorbable suture after ligation of fistula with diversion colostomy for ARM.
Group B patients underwent similar preoperative investigations. They were subjected to single-stage, single-layer primary esophageal anastomosis using absorbable suture after ligation of fistula as well as single-stage repair of ARM, i.e., PSARP or abdomino-PSARP depending on the site of fistula.
All our ARM patients are assessed for functional outcome at age of 3 year or more. The functional assessment following ARM repair was carried out using Kelly's method. Patients were divided into three groups according to the score: Group 1 - good (score 5–6), Group 2 - fair (score 3–4), and Group 3 - poor (score 0–2). Statistical analysis was done by SPSS 16 version. Data are presented in the form of number and percentage for qualitative variables and mean, standard deviation, and range in case of quantitative variables.
During the study period, 956 neonates of ARM and 365 neonates of EA ± TEF were admitted. Fifty-four neonates had both ARM and EA ± TEF. A total of 28 were included in the study. Among these, 17 were managed with staged procedure for ARM (Group A), whereas 11 underwent simultaneous single-stage repair of ARM with TEF (Group B).
Mean age at presentation in Group A and B was 3.18 ± 1.07 days and 2.91 ± 0.83 days, respectively (P = 0.437). Mean weight at presentation in Group A and B was 2.21 ± 0.22 kg and 2.38 ± 0.09 kg, respectively (P = 0.02). Males were more common than females in both the groups. Mean age at last follow-up in group A was 3.56 ± 1.22 years and in Group B was 4.22 ± 1.98 years. Mean weight and height at last follow-up in Group A were 12.45 ± 2.98 kg and 91.56 ± 6.98 cm, respectively, whereas in Group B, it was 13.24 ± 1.78 kg and 95.67 ± 8.82 cm, respectively.
In Group A, out of 17 patients, 9 (52.9%) had severe cardiac anomaly, 4 (23.5%) had no anomaly, and other 4 (23.5%) could not be screened. On the other hand, in Group B, out of 11 patients, 2 (18.2%) had severe cardiac anomaly, 6 (54.5%) had no anomaly, and other 3 (27.3%) could not be screened (P = 0.14). Most common anomalies were ventricular septal defects, atrial septal defects followed by combination of complex cardiac anomalies preoperative sepsis screen were positive in 10 (58.8%) of Group A cases and 5 (45.5%) of Group B cases (P = 0.48).
Mean operating time in Group A and B was 90.45 ± 10.23 min and 115 ± 13.23 min (P = 0.63). Postoperative ventilatory time, inotropic support, and anastomotic leak among Group A and B have been highlighted in [Table 1].
|Table 1: Postoperative ventilatory requirement, inotropic support, and anastomotic leak among Groups A and B|
Click here to view
Types of ARM in Group A and B have given in [Table 2]. Mean age at PSARP in Group A was 4.24 ± 1.23 months and mean age at colostomy closure was 9.45 ± 2.33 months. Male patients in both group with rectobulbar and prostatic fistula were managed with PSARP. Patients with rectovesical fistula underwent abdomino-PSARP. Female patients with vestibular fistula underwent anterior sagittal anorectoplasty and patients with rectovaginal fistula were managed by PSARP.
Postoperative complication analysis
Five patients (29.4%) in Group A died at first admission, whereas the mortality at first admission in Group B was 4 (36.4%). At 1-year follow-up, another 4 patients of Group A (23.5%) died, three - due to colostomy diarrhea, dehydration, and delayed presentation, and one after PSARP due to pneumonitis and septicemia. Overall mortality in Group A at 1-year follow-up was 52.9%, whereas in Group B, one (9%) patient died due to pneumonia and sepsis in follow-up resulting in an overall mortality of 43.4% at 1 year. Long-term complication in the form of esophageal stricture developed in 3 (25%) patients out of 12 who were discharged after 1st surgery in Group A and 2 (28%) out of 7 in Group B (p = 0.55). Two patients of Group A could not undergo staged PSARP at the age of 1 year because of monetary problems.
Out of 14 patients (Group A: 8 and Group B: 6), 10 completed follow-up of 3 years or more. These patients (Group A: 4 and Group B: 6) were evaluated for continence. No difference was observed in outcome between the two groups (P = 0.96). Details have been given in [Table 3]. One patient (Group B) had mucosal prolapse requiring excision.
| Discussion|| |
The management of ARM has seen a sea change over the past three decades. Improved neonatal care and nutritional support have shifted priority from survival in these patients to better long-term structural and functional outcome. Operative procedures have shifted from classical procedures to PSARP, and now to minimal invasive procedures., Furthermore, now, single-stage neonatal repair has been advocated by different authors across the globe with excellent early outcome. Primary neonatal repair has also achieved equivalent long-term functional outcome when compared to stage procedure.
About 50% of children with ARM have associated congenital anomalies. The incidence of type of reported anomalies is different in different series. Genitourinary tract anomalies (40%–50%) are most commonly reported followed by cardiovascular disorders (30%–35%), spinal cord tethering (25%–30%), gastrointestinal anomalies (5%–10%), and VACTERL (4%–9%) anomalies.,,, The mortality of children with ARM is now mostly limited to group of patients associated with severe cardiac anomalies.
The patients of ARM with EA have higher risk of mortality because of higher incidence of associated other congenital anomalies.,, Byun et al. have reported a mortality rate of 24.1% in patients with ARM with EA ± TEF in spite of excellent survival of patients with EA ± TEF alone.
Excellent survival rates of children with EA ± TEF have also been reported from our country., The improved outcomes are mainly due to advances in better antenatal care, neonatal intensive care, nutritional support, and improvement of anesthetic and surgical techniques. Along with these advances, concepts of the prognosis and therapeutic strategy for EA management have been changing. Different reports have questioned the predictive validity of the Waterston and Spitz classification.,, Different authors have reported that only severe cardiac disorders and extremely low birth weight to an extent are predictors of higher mortality rates in these patients. However, different studies from various centers of our country have proposed that other factors are also important for the final outcome of these patients. Factors such as delayed presentation and pneumonitis at presentation may be implicated for poor prognosis., Most of our patients are of low birth weight, have pneumonitis, and present late at tertiary center. Hence, the much higher rates of mortality of children in both the groups at first admission in our study may be explained by the interplay of higher incidence of complex heart disease with other factors mentioned above.,,
Changing the position of the baby after EA and TEF repair to the prone jackknife position requires team effort and vigilant staff to take care of the endotracheal tube, transanastomotic tube, and central line. Once patient is stabilized in the standard jackknife position, free abdominal movement is critical. In one patient, there was dislodgment of transanastomotic tube while repositioning.
Further, the mortality rates in our study are also high in follow-up. This can be mainly implicated to the poor postoperative care given to our patients at home once they are discharged. Poor feeding practices may be a causative factor for higher incidences of diarrhea and recurrent aspiration pneumonitis, especially lack of propped up nursing. Studies need to look into and highlight these factors as well.
The reported incidence of anastomotic leakage of esophagusranges from 15% to 20%., In our study, the incidence of leak was 17%–18% in both the groups. Minor leak resolved spontaneously with conservative management, i.e., chest tube drainage, appropriate antibiotic coverage, and total parental nutrition. One patient in each group with anastomotic leak died in the postoperative period.
Anastomotic stricture is the most common complication following the surgical repair of EA and observed in 20%–40% after successful repair., The incidence of esophageal stricture was 25%–30% in the present series which was comparable to other series. There was no difference in the occurrence of esophageal stricture between the two groups.
Analysis of data in the present study has shown that the early and short-term results of both the groups were comparable. Various study from our center and all across the world have proven the advantages of single-staged repair of ARM not only in short-term but also in regard to long-term continence of these patients. The long-term problems of staged repair such as colostomy associated complications, risk of repeated surgical interventions and above all, the financial burden over the family provides distinct advantage of simultaneous single-staged repair in our setup.
| Conclusion|| |
ARM associated with EA and TEF poses special therapeutic challenge. Simultaneous single-staged primary repair of both the anomalies has similar immediate and early outcome of these patients in our setup. We acknowledge the limitations of our study with small number of cases and also retrospective nature of the study. Larger multiinstitutional prospective study from our country is required to formulate the guidelines for management of this special group of children.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Pena A, Levitt M. Anorectal malformations. In: Stringer M, Oldham K, Mouriquand PD, editors. Pediatric Surgery and Urology: Long Term Outcomes. 2nd
ed. Cambridge: Cambridge University Press; 2007. p. 401-5.
Rintala RJ, Lindahl HG. Posterior sagittal anorectoplasty is superior to sacroperineal-sacroabdominoperineal pull-through: a long-term follow-up study in boys with high anorectal anomalies. J Pediatr Surg 1999;34:334-7.
Pena A. Posterior sagittal anorectoplasty: Results in the management of 332 cases of anorectal, malformations. Pediatr Surg Int 1988;3:94-104.
Tönz M, Köhli S, Kaiser G. Oesophageal atresia: what has changed in the last 3 decades? Pediatr Surg Int 2004;20:768-72.
Spitz L. Esophageal replacement. In: Grosfeld JL, O'Neill JA Jr., Coran AG, Fonkalsrud EW, editors. Pediatric Surgery. 6th
ed. Philadelphia, PA: Mosby Elsevier; 2006. p. 1093-106.
Shaw-Smith C. Oesophageal atresia, tracheo-oesophageal fistula, and the VACTERL association: review of genetics and epidemiology. J Med Genet 2006;43:545-54.
Depaepe A, Dolk H, Lechat MF. The epidemiology of tracheo-oesophageal fistula and oesophageal atresia in Europe. EUROCAT working group. Arch Dis Child 1993;68:743-8.
Gangopadhyay AN, Pandey V, Gupta DK, Sharma SP, Kumar V, Verma A. Assessment and comparison of fecal continence in children following primary posterior sagittal anorectoplasty and abdominoperineal pull through for anorectal anomaly using clinical scoring and MRI. J Pediatr Surg 2016;51:430-4.
Kelly JH. The clinical and radiological assessment of anal continence in childhood. Aust N
Z J Surg 1972;42:62-3.
Elbatarny A, Elafifi M, Ismail K, Metwally M, Hashish A, Elmahalawy M, et al
. Evaluation of one-stage posterior sagittal anorectoplasty for the repair of high and intermediate anorectal malformations. Ann Pediatr Surg 2009;1:36-45.
Endo M, Hayashi A, Ishihara M, Maie M, Nagasaki A, Nishi T, et al.
Analysis of 1,992 patients with anorectal malformations over the past two decades in Japan. Steering Committee of Japanese Study Group of Anorectal Anomalies. J Pediatr Surg 1999;34:435-41.
Watanabe Y, Ando H, Seo T, Kaneko K, Katsuno S, Shinohara T, et al.
Three-dimensional image reconstruction of an anorectal malformation with multidetector-row helical computed tomography technology. Pediatr Surg Int 2003;19:167-71.
Byun SY, Lim RK, Park KH, Cho YH, Kim YH. Anorectal malformations associated with esophageal atresia in neonate. Pediatr Gastroenterol Hepatol Nutr 2013;16:28-33.
Tandon RK, Sharma S, Sinha SK, Rashid KA, Dube R, Kureel SN, et al.
Esophageal atresia: Factors influencing survival – Experience at an Indian tertiary centre. J Indian Assoc Pediatr Surg 2008;13:2-6.
] [Full text]
Singh S, Wakhlu A, Pandey A, Singh A, Kureel SN, Rawat J, et al.
Esophageal atresia associated with anorectal malformation: Is the outcome better after surgery in two stages in a limited resources scenario? J Indian Assoc Pediatr Surg 2012;17:107-10.
] [Full text]
Spitz L, Kiely EM, Morecroft JA, Drake DP. Oesophageal atresia: at risk groups for the 1990s. J Pediatr Surg 1994;29:723-5.
Spitz L. Esophageal atresia. Lessons I have learned in a 40-year experience. J Pediatr Surg 2006;41:1635-40.
Osia S, Hadipour A, Moshrefi M, Mirzapour M. Esophageal atresia: 13 years' experience in Amirkola Children's Hospital, North of Iran. Caspian J Pediatr 2015;1:22-4.
Chittmittrapap S, Spitz L, Kiely EM, Brereton RJ. Anastomotic stricture following repair of esophageal atresia. J Pediatr Surg 1990;25:508-11.
Gangopadhyay AN, Shilpa S, Mohan TV, Gopal SC, Gupta DK, Sharma SP. Single-stage management of all pouch colon (anorectal malformation) in newborns. J Pediatr Surg 2005;40:1151-5.
[Table 1], [Table 2], [Table 3]