Journal of Digestive Disorders And Diagnosis
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Case-report Article | Open Access
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  • Unusual Presentation Of Tracheoesophageal Fistula With Meconium Aspiration Syndrome In A Preterm Infant

    Malik Laraib 1
        Khan Maria 2     Aziz Sina 1     Mirza Sadiq 1    

    1 Paediatric unit II, P.I.C.U, Abassi Shaheed Hospital, Karachi, 

    2 medical student Karachi medical and dental college, Karachi 

    Abstract

    Author Contributions

    Received Feb 05, 2017;     Accepted Feb 20, 2017;     Published Mar 28, 2017;

    Academic Editor: Divey Manocha

    Affilation: Upstate Medical University

    Copyright© 2017 Malik Laraib, et al.
    License
    Creative Commons License   This work is licensed under a Creative Commons Attribution 4.0 International License. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

    Competing interests

    The authors have declared that no competing interests exist.

    Funding Interests:

    Citation:

    Malik Laraib, Khan Maria, Aziz Sina, Mirza Sadiq et al. (2017) Unusual Presentation Of Tracheoesophageal Fistula With Meconium Aspiration Syndrome In A Preterm Infant Journal of Digestive Disorders And Diagnosis. - 1(2):8-12
    DOI 10.14302/issn.2574-4526.jddd-17-1454

    Introduction

    Introduction

    Thomas Gibson, is credited for describing the first case of oesophageal atresia (OA) and tracheoesophageal fistula (TEF) and Thomas Hill, noted the second case of OA with an associated anomaly of an imperforate anus. While some newborns may have TEF, about 4% of newborns with TEF do not have OA.1 the incidence of OA is 1 in 2500 live births and 86% of cases are associated with TEF.2 There is 0.5 to 2% risk of recurrence among siblings of affected child.3 However, in Pakistan, only tertiary care centers which have paediatric surgery facility report anomaly cases4.

    Here we present a case TEF with OA, associated with meconium aspiration syndrome (MAS) in an infant. We aim to highlight the importance of conducting a comprehensive antenatal and prenatal workup in newborns to avoid management difficulties after birth and prevent an avoidable fatality. Also, to highlight an early diagnosis if a baby has respiratory distress and ther is failure to pass nasogastric tube and an X-ray which indicates a coiled tube in esophagus and intestinal air suggesting TEF.

    Discussion

    Discussion

    Congenital malformations usually occur during organogenesis resulting in complete or partial absence of an anatomical part or alteration of its normal configuration. Major structural anomalies occur in 2-3% of live births and an additional 2-3% are recognized by the age of 5.4

    This case report presented has shown TEF with blind ending esophageal pouch with a fistula from trachea o distal esophagus.The pathogenesis is poorly understood; the trachea, oesophagus and lungs are foregut derivatives that divide into ventral respiratory and dorsal oesophageal components. Sometimes the oesophagus fails to separate from trachea leading to TEF.

    Mastroiacovo et al5 found that out of 92 malformations studied, 39 malformations including TEF/OA were more common in twins than in singletons and in males. Chromosomal anomalies like trisomy 18 and 21 also predispose to this condition. Even more recently, three genes associated with TEF/OA in humans have been identified.1 In our case, the child was a singleton, female and no other obvious dysmorphic features were seen. CHARGE and VACTERL were excluded. Karyotyping and any other chromosomal analysis couldn t be done due to lack of funding.

    According to Waterston classification our case was type B and according Spitz classification she was falling into category II.6

    The incidence of other anomalies associated with TEF/OA is reported to be 30-60%.7 Children with TEF have a higher incidence of pyloric stenosis 3..The most common anomaly is that of cardiovascular(11-49%) followed by genitourinary(24%), gastrointestinal (24%), musculoskeletal(13%)6. In our case, associated malformation was that of gastrointestinal.

    Sparey et al8 reported that prenatal detection of TEF/OA was possible in 10% of the cases, which is very low compared to other congenital anomalies. The positive predictive value of both polyhydrominos and a small or absent stomach bubble has been reported to be only 56%, making prenatal diagnosis even more difficult,9 same was the scenario in our case: the mother had normal ultrasound scans. This call for more aggressive attempts to conduct prenatal exams and close follow ups of such a pregnancy with delivery in a specialist center.

    In the postnatal period TEF/OA should be considered if newborn has pooling of saliva, feeding and breathing difficulties, and passes nasogastric tube with a pain; however chest x-ray to demonstrate the coiled tube in the esophagus is more confirmatory as was the case in our patient except that she also had Grade 1 MAS. Additional diagnostic modalities include upper pouch esophagogram (UPEG), tracheobronchoscopy and contrast esophagogram with fluoroscopic control. Even endoscopic procedures like bronchoscopy and oesophagoscopy can be used. However, esophagogram with contrast and barium studies offer the best visualization of TEF/OA.10

    Surgical management includes Neonatal Intensive Care Unit and appropriate anesthesia, which is available in developed countries and has an almost 100% survival rate as compared to low-income settings that exist in countries like ours, where all discipline facilities including prompt surgical intervention, ventilator support and prevention of septicemia are lacking, resulting in a high morbidity and mortality ratio.4,11

    The outcome is generally better for term babies 12 than preterm. The death in our case reported was most probably due to low birth weight, prematurity, septicemia and inadequate management modalities.

    Conclusion

    Conclusion

    This case highlights the significance of conducting a comprehensive clinical examination in newborns; meticulous prenatal workup is also necessary to reduce fatalities. TEF/OA should be suspected in any newborn presenting with respiratory distress, persistent drooling, a history of polyhydrominos and inability to pass nasogastric tube. Ventilator support and other management modalities should be mandatory in a tertiary care setup. The parents should also be counseled about future pregnancy as it carries a 1% risk of recurrence.

    Consent: Written informed consent was obtained from the parents of the baby for publication of this case report. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

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