Most infants with EA with TEF have proximal atresia with distal TEF. They are easily diagnosed soon after birth as to apparent clinical features, nevertheless H-type TEF are not diagnose early because esophagus is patent. Many diagnostic methods have been advocated for the diagnosis of H-type fistula. Esophagram is usually a reliable method to identify congenital H-type tracheoesophageal fistula, though often difficult, requiring multiple attempts before the defect is confirmed. Furthermore, contrast-enhanced studies have the potential risk of aspiration pneumonia and pulmonary injury and should be performed with adequate neonatal emergency resuscitation at hand. Endoscopic methods like bronchoscopy and esophagoscopy have the advantage of being diagnostic allowing placement of a catheter across the fistula to assist in its localization during surgery [2-4]. H-type TEF is associated with other malformations in about 30% of cases, including VACTERL/VATER, CHARGE syndrome, Goldenhar’s syndrome, esophageal stenosis, and syndactyly . The index case has none of these associations.
Different surgical approaches have been described for this anomaly. For proximally located fistula the approach of choice is cervicotomy and in cases of distal fistula thoracotomy is usually preferred. Biechlin et al reported a series of 8 cases of H-type TEF, all were repaired through right cervicotomy. An alternative thoracoscopic approach in a newborn has recently been reported by Allal et al. Surgery consists of ligation and division of the fistula and repair of the tracheal and esophageal walls. Brookes et al reported seven patients of H-type TEF and one patient with a missed proximal H-type fistula associated with esophageal atresia. They presented with coughing while feeding, recurrent pneumonia, and episodic cyanosis. A delay in diagnosis was seen in 4 patients and ranged from 2.5 months to 5.9 years. In all patients, the diagnosis was made on esophagram. The level of the fistulae was between C5 and T3, and all were successfully repaired via a right cervical approach [6-9].
In present case cervical approach was chosen with preservation of recurrent laryngeal nerve. The outcome in present case was satisfactory as baby discharged home on 10th POD in stable condition. A high index of suspicion in cases of cyanosis and choking on first feed and recurrent respiratory symptoms even when esophagus is patent, indicate H-type TEF until proved otherwise. Such patients must be thoroughly investigated to demonstrate the anomaly.