| Abstract|| |
Tracheo-oesophageal fistula (TEF)/oesophageal atresia is one of the most common and serious congenital malformation. Despite progresses made in the field of early diagnosis, surgical techniques, ventilatory support and control of chest infections; morbidity and mortality still remains quite high and differs a lot from one to another centre particularly in the developing countries; as the availability and the level of neonatal care facilities are different. Associated anomalies play a significant role in dictating the outcome, timing of intervention and even the approach to management. The objectives of this review article is to outline the spectrum of associated anomalies, emphasise need of standardised system of documentation of anomalies, prognosis and management issues that would influence timing and approach of TEF repair.
Keywords: Associated anomalies, oesophageal atresia, trachea-oesophageal fistula
|How to cite this article:|
Pal K. Management of associated anomalies of oesophageal atresia and tracheo-oesophageal fistula. Afr J Paediatr Surg 2014;11:280-6
| Introduction|| |
The incidence of various associated anomalies with trachea-oesophageal fistula/oesophageal atresia (TEF/OA) varies between 40% and 55%. ,, Anomalies may be single or multiple, minor or major, detected at birth or later on. The life-threatening anomalies require only counselling and no treatment. Major associated malformations need to be detected and treated in time. The minor ones, with no risk to life, require no immediate intervention. The high incidence of association is due to the early disturbance in organogenesis, which results in OA and other associated anomalies. These anomalies are most common in patients with isolated OA (55-60%) and the least common in cases with H type TEF (25-30%). ,,,, Furthermore, the infants weighing <2000 g have almost three times higher incidence of associated anomalies when compared to those weighing more than 2500 g. 
Infants with OA and other associated congenital anomalies can be divided into following two groups:
Group A: Infants with severe anomalies generally incompatible with survival and constitute about 5% cases presenting with isolated OA. For example, trisomy 15, Potter's syndrome, complex-cardiac anomalies, tracheal agenesis and cerebral hypoplasia. These infants preclude further management
Group B: Infants with OA having major or minor congenital anomalies but salvageable. More than 95% cases fall into this group.
Those likely to complicate the early life of newborns with OA-TEF. Most of them require some form of surgical intervention early in the child's life.
Babies may be affected by a recognised syndrome (e.g., trisomy) or more than one associated anomalies.
| Minor Anomalies|| |
Not likely to be of significant consequence. May or may not need any surgery and if so, it is probably minimal and can be done at a later date.
Problems created by classification and documentation systems not universally employed
Due to considerable difference in the methods used and the interest taken, the incidence of specific types of anomalies varies significantly among different series [Table 1]. Furthermore, the lack of uniformity in the method of investigation and potential associated anomalies (non-uniform application of renal ultrasound with or without micturating cystourethrography and echocardiography) has resulted in incomparable impacts of these anomalies on the prognosis of these infants.
| Recommendations|| |
Myers et al.  analysed the discrepancies in documentation and classification systems of several large series and formulated some recommendations, which adequately takes care of epidemiological, clinical and therapeutic requirements.
- Data should be collected prospectively.
- Routing investigation should include renal ultrasound and echocardiography (as a minimum requirement).
- The number of associated anomalies should be reported as percentage of the total cohort of patients, rather than as a percentage of the anomalies themselves.
- True congenital anomalies should be recorded separately from acquired conditions, for example, intracranial haemorrhage, respiratory distress syndrome, hyaline membrane disease etc.
- Non-life threatening trivial lesions, for example, accessory auricles. Undescended testis, inguinal hernia, umbilical hernia or pre-auricular sinuses should always be specified in the miscellaneous group as consistent reporting of these apparently irrelevant congenital anomalies may ultimately help shed light on the aetiology of OA-TEF itself. In addition, it reduces the likelihood that their inclusion in major categories will distort the overall impact on prognosis.
- Wide variation in the pact of associated abnormalities on the management and long-term outlook in OA should be recognised.
A method of grading of significance of abnormalities shown in [Table 2].
|Table 2: "Signifi cance" of the grading of associated anomalies in OA-TEF|
Click here to view
| Impact and Management of Associated Congenital Anomalies|| |
Major congenital anomalies are responsible for 55% mortality in OA-TEF patients.  Of these, 6-11% have trisomy and/or complex cardiac defects which are incompatible with life thereby precluding any active management. 
Complex cardiac anomalies account for most of deaths (35%). Survival rate falls proportionately with the addition of more defects, for example, 76% survival rate with one additional anomaly and 59% with more than one.  Various series ,, document one or more than one additional congenital anomaly in 50-70% patients with OA-TEF. VACTERL association has high incidence of major cardiac anomalies (78%) and a high mortality rate (24%). ,, Kutiyanawala et al.  also reported more than 70% mortality in their series of 10 cases of TEF cases with the CHARGE association mostly due to severe cardiac anomalies.
System wise associated anomalies are depicted in [Table 3], [Table 4], [Table 5] and [Table 6].
|Table 5: Association between types of anorectal malformations and types OA|
Click here to view
Routine echocardiography is recommended to detect and grade the severity of cardiovascular anomalies. Infants with major cardiac anomalies have 30% risk of mortality.  Most common single cardiac defects; ventricular septal defect (VSD) carries 16% mortality risk. Over 87% VSD closes as the age advances. Congenital heart disease with high pulmonary flow rarely causes a problem in the immediate neonatal period because of the residual high pulmonary vascular resistance.
In patient with patent ductus arteriosus (PDA) dependent anomalies, infusion of prostaglandin E 1 may allow early oesophageal repair. Patients, who do not have improvement, may benefit from initial palliative or corrective cardiac surgery. 
Patent PDA in premature and low birth weight babies causing congestive heart failure usually respond to indomethacin therapy. Non-closure to medical therapy demands PDA ligation through left thoracotomy, pre- or post-TEF repair as the situation might warrant.
Urgent of treatment is determined by the degree of cyanosis and hypoxia
About 13-20% patients with tetralogy of Fallot are associated with right sided aortic arch which will have its own impact on TEF repair.
Right aortic arch anomaly
About 8% infants with OA have aortic arch anomalies.  About 5% infants with OA have right aortic arch (RAA). , Pre-operative detection rate by echocardiogram and chest X-ray is reported in up to 8-10%. , Magnetic resonance angiography and spiral computed tomography scan generally are not part of the routine pre-operative investigations. Three types of RAA have been described: ,,
- Right aortic arch descending on right side or spine with mirror image branching of innominate and subclavian arteries
- Solitary RAA passes over right main bronchus, then returns to left passing behind oesophagus to descend on the left side of vertebra
- Double aortic arch.
Management of RAA is controversial.
A strong correlation has also been described between RAA, long gap atresia and vascular atresia anomalies.  These infants have five folds increase in major postoperative complications including leak and stricture. , Canty et al.  reported high incidence of aberrant left subclavian arteries in infants with long gap (>3 cm) OA-TEF and RAA, requiring left thoracotomy for definitive repair of both the anomalies.
- Infants with OA cardiac anomalies should be immediately put on prophylactic antibiotics to prevent infective endocarditis.
- Cases with severe coarctation of aorta with poor distal perfusion, a less invasive balloon angioplasty is performed prior to OA-TEF repair.
- Recently, authors have described various types of azygos vein anomalies in association with TEF. The presence of anomalous azygos vein denotes a wide gap OA, small upper pouch and the possibility of a upper pouch fistula. 
Pure OA has higher incidence (30%) of anorectal malformations when compared to those with OA and TEF (1%).  Incidence of various types of anorectal malformations and the type of OA are shown in [Table 5].  Non-anorectal gastrointestinal anomalies occur in 6-10% of cases of OA-TEF have been shown in [Table 4].
Oesophageal stenosis is a rare association more commonly found in patients with H type of TEF. A regular practice of probing the distal pouch during the surgical repair of all cases of OA-TEF and the H type in particular, would diagnose such problems early. If detected, demands diversion in the form of an oesophagostomy and a gastrostomy.
Algorithm for investigation of urinary tract in infants with OA-TEF
Beasely et al.  classified urinary abnormalities in four groups according to their impact on the need for management [Table 6].
It is important to recognise that many patients with bilateral renal agenesis do not have features suggestive of Potter's syndrome in the presence of OA probably because the olygohydramnios of renal agenesis is counteracted by the tendency to polyhydramnios with OA. It is thus unlikely that these patients can be identified pre-operatively on clinical grounds alone.
Respiratory system anomalies
A unique form of tracheomalacia is associated with TEF. , The lumen of trachea in the vicinity of fistula (TEF) has a crescent or D-shaped configuration because of an abnormally wide membranous portion of the trachea. The area of TEF is most affected, and the anomalous membranous trachea may extend to a varying distance proximally and involve the bronchi distally. These infants and children have a peculiar barking cough and expiratory stridor, which is prominent than in infants with other forms of malacia.
Algorithm of management of tracheomalacia
Only 33 cases have so far been reported since 1874.  These are uniformly lethal anomalies affecting mostly right lung with right main brochus being replaced by the distal oesophagus. More than 50% have VATER association and/or heart defects. About 50% children are stillborn or else die within the first few months of the life from the concomitant anomalies. Survivors of OA-pulmonary aplasia carry poor survival due to recurrent respiratory infection and failure of solitary lung.
Laryngo tracheoesophageal cleft (symptomatic types II, III, and IV)
Subglotic stenosis, tracheal stenosis, laryngeal atresia require bronchoscopic evaluation of early tracheostomy prior to the repair of OA-TEF. Definitive (tracheo/laryngoplasty) of individual defects is undertaken at a later date.
Congenital diaphragmatic hernia and oesophageal atresia-tracheoesophageal fistula
Only seven cases have been reported till date since the first case of the rare association was reported by Ahmed. ,,, The respiratory distress is usually severe. Each case needs to be individualised depending on the severity of the disease. A pre-operative stabilization is important before repairing the diaphragmatic defect. Both the defects can be repaired simultaneously. However, in babies with severe chest infection, only fistula ligation may be considered as the initial step to prevent reflux pneumonitis.
| Conclusion|| |
Associated anomalies of OA and TEF require comprehensive assessment and uniform documentation. Cognizance of above mentioned issues would enable quick and overall assessment of a child with TEF, better timing of interventions, accurate prognostication and realistic counselling of parents so as to adjust their understanding and expectations.
| References|| |
Holder TM, Ashcraft KW, Sharp RJ, Amoury RA. Care of infants with esophageal atresia, tracheoesophageal fistula, and associated anomalies. J Thorac Cardiovasc Surg 1987;94:828-35.
German JC, Mahour GH, Woolley MM. Esophageal atresia and associated anomalies. J Pediatr Surg 1976;11:299-306.
Holder TM, Cloud DT, Lewis JE Jr, Pilling GP 4 th
. Esophageal atresia and tracheoesophageal fistula. A survey of its members by the surgical section of the American Academy of Pediatrics. Pediatrics 1964;34:542-9.
Chittmittrapap S, Spitz L, Kiely EM, Brereton RJ. Oesophageal atresia and associated anomalies. Arch Dis Child 1989;64:364-8.
Ashcraft KW, Holder TM. Esophageal atresia and tracheoesophageal malformations. In: Holder TM, Ashcraft KW, editors. Pediatric Surgery. Philadelphia: Saunders; 1980. p. 266-83.
Myers MA, Beasley SW, Auldist AW. Oesophageal atresia and associated anomalies: A plea for uniform documentation. Pediatr Surg Int 1992;7:97-100.
Ein SH, Shandling B, Wesson D, Filler RM. Esophageal atresia with distal tracheoesophageal fistula: Associated anomalies and prognosis in the 1980s. J Pediatr Surg 1989;24:1055-9.
Iuchtman M, Brereton R, Spitz L, Kiely EM, Drake D. Morbidity and mortality in 46 patients with the VACTERL association. Isr J Med Sci 1992;28:281-4.
Kutiyanawala M, Wyse RK, Brereton RJ, Spitz L, Kiely EM, Drake D, et al.
CHARGE and esophageal atresia. J Pediatr Surg 1992;27:558-60.
Quan L, Smith DW. The VATER association. Vertebral defects, Anal atresia, T-E fistula with esophageal atresia, Radial and Renal dysplasia: A spectrum of associated defects. J Pediatr 1973;82:104-7.
Spitz L. Esophageal atresia and tracheoesophageal fistula in children. Curr Opin Pediatr 1993;5:347-52.
Mee RB, Beasley SW, Auldist AW, Myers NA. Influence of congenital heart disease on management of oesophageal atresia. Pediatr Surg Int 1992;7:90-3.
Canty TG Jr, Boyle EM Jr, Linden B, Healey PJ, Tapper D, Hall DG, et al
. Aortic arch anomalies associated with long gap esophageal atresia and tracheoesophageal fistula. J Pediatr Surg 1997;32:1587-91.
Harrison MR, Hanson BA, Mahour GH, Takahashi M, Weitzman JJ. The significance of right aortic arch in repair of esophageal atresia and tracheoesophageal fistula. J Pediatr Surg 1977;12:861-9.
Stringel G, Coln D, Guertin L. Esophageal atresia and right aortic arch. Right or left thoracotomy. Pediatr Surg Int 1990;5:103-5.
Stewart JR, Kincaid OW, Titus JL. Right aortic arch: Plain film diagnosis and significance. Am J Roentgenol Radium Ther Nucl Med 1966;97:377-89.
Knight L, Edwards JE. Right aortic arch. Types and associated cardiac anomalies. Circulation 1974;50:1047-51.
Babu R, Pierro A, Spitz L, Drake DP, Kiely EM. The management of oesophageal atresia in neonates with right-sided aortic arch. J Pediatr Surg 2000;35:56-8.
Foker JE. Esophageal atresia and tracheoesophageal fistula. In: Pearson FT, Deslauuriers J, Ginsberg RJ, editors. Esophageal Surgery. New York, NY: Churchill Livingstone; 1995. p. 151-83.
Gupta DK, Arora M, Srinivas M. Azygos vein anomaly: The best predictor of a long gap in esophageal atresia and tracheoesophageal fistula. Pediatr Surg Int 2001;17:101-3.
Gross RE. Surgery of Infancy and Childhood. Vol. 75. Philadelphia: W.B. Saunders; 1953; p. 102.
Beasley SW, Phelan E, Kelly JH, Myers NA, Chetcuti P, Auldist AW. Urinary tract abnormalities in association with oesophageal atresia: Frequency, significance, and influence on management. Pediatr Surg Int 1992;7:94-6.
Brawn WJ, Huddart SN. Tracheoaortopexy via midline sternotomy in tracheomalacia. J Pediatr Surg 1991;26:660-2.
Filler RM, de Fraga JC. Tracheomalacia. Semin Thorac Cardiovasc Surg 1994;6:211-5.
Steadland KM, Langham MR Jr, Greene MA, Bagwell CE, Kays DW, Talbert JL. Unilateral pulmonary agenesis, esophageal atresia, and distal tracheoesophageal fistula. Ann Thorac Surg 1995;59:511-3.
Ahmed S. Right-sided Bochdalek hernia associated with esophageal atresia and tracheo-esophageal fistula. J Pediatr Surg 1970;5:256.
Udassin R, Zamir O, Peleg O, Lernau OZ. Coexisting left diaphragmatic hernia and esophageal atresia. Pediatr Surg Int 1987;2:301-3.
Bowen A. The ventilatory dilemma of coexisting diaphragmatic hernia, esophageal atresia, and tracheoesophageal fistula. Crit Care Med 1983;11:390-1.
Rawlings JS, Shetler PL, Fill WL, Cathcart CF. Concurrent right diaphragmatic hernia and type C tracheoesophogeal fistula. A case report. Clin Pediatr (Phila) 1984;23:518-20.
Dr. Kamalesh Pal
Department of Surgery, Division of Pediatric Surgery, College of Medicine, University of Dammam, King Fahad Hospital of the University, Al Khobar 31952, PO Box 40129
Kingdom of Saudi Arabia
Source of Support: None, Conflict of Interest: None
[Table 1], [Table 2], [Table 3], [Table 4], [Table 5], [Table 6]