Oesophageal atresia with tracheo-oesophageal fistula in a preterm neonate in Limbe, Cameroon: case report & brief literature review

Aminde et al. BMC Research Notes 2014, 7:692 http://www.biomedcentral.com/1756-0500/7/692 CASE REPORT Open Access Oesophageal atresia with tracheo-oesophageal fistula in a preterm neonate in Limbe, Cameroon: case report & brief literature review Leopold N Aminde1,2*, Veronica N Ebenye2, Walters T Arrey1,2, Noah F Takah2 and George Awungafac2 Abstract Background: Oesophageal atresia is a congenital anomaly in which there is interruption of the oesophageal lumen resulting in an upper and lower segment. We present the case of a rare sub-type of Oesophageal atresia with proximal trachea-oesophageal fistula associated with Meconium Aspiration Syndrome. This is the first case reported in literature in the South West Region of Cameroon. Case presentation: A 2 day old preterm male baby who presented as an emergency with difficulty breathing, fever and refusal to feed. Initially managed as early onset neonatal sepsis from meconium aspiration syndrome in which a diagnosis of oesophageal atresia was finally made. Conclusion: A high index of suspicion for Oesophageal atresia/trachea-oesophageal fistula should prevail when faced with a neonate with the triad: respiratory distress during feeds, regurgitation and persistent frothy salivation. The case discusses the diagnostic dilemma and management difficulties in a preterm neonate with the above association in a low income setting. Keywords: Oesophageal atresia, Trachea-oesophageal fistula, Diagnosis, Preterm, Cameroon Background Congenital malformations and their associations are not so rare and contribute to neonatal and infant morbidity and mortality [1]. Their prevalence ranges from 1% to over 4% depending on the place and population studied [2,3]. We report the case of Oesophageal atresia (OA) with proximal trachea-oesophageal fistula (TOF) associated with Meconium Aspiration Syndrome (MAS) in a preterm infant. Though, a not so common finding, this re- iterates the importance of thorough clinical examination of new borns, especially preterm, keeping in mind congenital abnormalities. This seems to be the first reported in literature from the South West Region of Cameroon. Case presentation A 2 day old preterm (30 weeks) male baby was admitted as an emergency due to difficulty in breathing, fever and refusal to feed. Mother had 3 antenatal visits (no * Correspondence: 1 Department of Paediatrics, Regional Hospital Limbe, Limbe, Cameroon 2 Faculty of Health Sciences, University of Buea, Buea, Cameroon ultrasound scan), no illness during pregnancy and denies any fever during labour. Past history significant for prolonged labour with excess amniotic fluid (meconium stained) at delivery of male preterm of weight 1.9 kg and APGAR 8, 9 at 1 and 5 minutes respectively. On examination, the child was in respiratory distress (RR = 65 cycles/ min) febrile to touch (Temp = 38°c). He had yellowish stain on nails (probably from meconium) and pale bluish extremities and no jaundice. Chest exam revealed thoraco-abdominal asynchrony, tachycardia (160b/min), no murmur and crackles in both lungs. Abdomen was apparently normal and anal canal was patent. A working diagnosis of Early onset neonatal sepsis following meconium aspiration syndrome (MAS) was made. Due to financial constraints only a Full Blood Count (which showed leukocytosis) and Chest X-Ray (which showed hyperinflation of the lung with patchy infiltrates in lower lung fields) could be afforded. Arterial blood gases were not done. Suctioning of oropharynx was done and the child placed on oxygen, kept warm and intravenous hydration and antibiotic therapy started. After 2 days of treatment, there was improvement in © 2014 Aminde et al.; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Aminde et al. BMC Research Notes 2014, 7:692 http://www.biomedcentral.com/1756-0500/7/692 general condition. Day 4 of admission was marked by choking and regurgitation following commencement of feeds but no fever. A repeat FBC was normal. Mother was re-assured and treatment continued. Day 5 was marked by persistence of regurgitation, difficulty breathing during feeds with bluing of periphery and frothy salivation for which mother reports to have been present since birth. No abdominal distension and the child passed stool. The differential diagnosis now included; Oesophageal atresia, oesophageal web, oesophageal stricture, respiratory failure. Nasogastric tube inserted which coiled returning towards oral cavity and a repeat Chest and Abdominal X-ray with tube in place showed mild pulmonary infiltrates with no air in the stomach and duodenum respectively. This was suggestive of an oesophageal atresia with proximal trachea-oesophageal fistula. The child was re-examined thoroughly and no evidence for other congenital anomaly was seen. A good samaritan offered for the Abdominal and Cardiac echography which were normal. The neonate was then referred to Paediatric surgeon at a referral centre for management. Due to low birth weight and inadequate facilities, child was managed conservatively by parenteral nutrition, antibiotics and upper pouch suction pending eventual surgical correction when considered to be low risk. We lost the child 16 hours after arrival at referral centre. Discussion The year 1697 saw the first description of Oesophageal atresia and Tracheo-oesophageal fistual by Thomas Gibson. Oesophageal atresia (OA) is a congenital malformation in which the oesophageal lumen is found to be interrupted, resulting in an upper and lower segment. While a vast majority of patients (92%) usually have a trachea-oesophageal fistula (TOF), about 4% of patients with TOF do not have OA [4]. This occurs in 1/2500 – 4500 live births [4-6]. The aetiology of oesophageal atresia is poorly understood. The trachea, oesophagus and lung are foregut derivatives, and during the fourth week of embryonic life, the foregut divides into ventral respiratory and dorsal oesophageal components. It is suggested that there is an alteration in the migration of the lateral folds or growth arrest at the time of evagination. Most of the time, the posterior oesophagus does not completely separate from the trachea, leading to distinct varieties of trachea-oesophageal fistula (TOF). This occurs between the third and sixth week of gestation [7]. The birth of an infant with OA/TOF in a family without a previous history of this condition is associated with a recurrence risk of ~ 1%. Twin concordance rate for OA/TOF is about 2.5% [4]. The above information suggests that genetic factors play a minor role in th (...truncated)