Congenital TEF occurs due to a defective separation of the respiratory and digestive tracts during embryogenesis, leading to abnormal communication between the two [6]. The incidence of isolated TEF or H-type TEF is very rare, approximately 1/50,000 per 100,000 live births, with a slightly higher prevalence in males [7]. The clinical diagnosis of a fistula is often quite difficult because it can appear with various symptoms of different severity. Therefore, an early diagnosis is still a challenge in the neonatal period and may be delayed into childhood or even into adulthood [1, 4].

Double TEFs without atresia of the esophagus have a very low incidence rate. There are few reports of patients having more than one fistula [8,9,10,11,12,13,14]. To our knowledge, this is the first case with double H-type TEF in a Tunisian newborn who has been operated on first with a preoperative diagnosis of an isolated H-type fistula located 2 cm above the carina at the level of the third thoracic vertebra. And then, another H-type fistula was located above the level of the first lesion. Among children, this is only the sixth case to be reported in the medical literature. Table 1 summarizes the literature on pediatric double H-type TEFs.

The classical clinical triad of H-type TEF called the triad of Helmsworth et Pryles, includes coughing with cyanosis and choking on feeding, abdominal distention, and recurrent pneumonia [1, 2, 5, 13]. Typically, symptoms start from birth, but the diagnosis is easily missed in the neonatal period [2, 3]. These symptoms are not specific, and they are shared with other disorders like laryngeal cleft, GER, and swallowing disorders [1, 4]. Difficulty in diagnosing double H-type TEF in the literature might be due to extremely low incidence and difficulty in the visualization of fistulae in a single diagnostic study. To make a correct diagnosis, starting with a high index of suspicion is necessary because symptoms are almost the same as in a single H-type fistula. In our case, we found such classical symptoms. They appeared after the first surgery; therefore, the diagnosis of a recurrence or a second TEF was suspected.

Diagnostic methods in suspected H-type fistulae are upper gastrointestinal series and esophagogram proceeded with water-soluble contrast. Their sensitivity ranges from 50 to 73% [7]. CT scan is recommended to assess the pulmonary parenchyma to rule out a fistula. However, it is not the first line study to detect a congenital TEF due to radiation exposure, especially in younger patients. Esophagoscopy is less useful in TEF identification since the esophageal ostium is smaller, and the intraluminal air-pressure during the digestive endoscopy may intermittently close the opening with mucosal folds [3].

Bronchoscopy represents the most reliable tool regarding clinical TEF suspicion. It confirms the diagnosis, establishes the level of the fistula, and identifies associated tracheobronchial anomalies and any additional fistula [3, 15]. Huaying Li et al. [15] stated that the use of a bronchoscopy tracer via esophageal MB injection could be made in the neonate as it is an effective method of confirming the diagnosis and may avoid prolonged suffering. A catheter can be inserted through the fistula to facilitate its intraoperative identification [7]. In our case, the use of a bronchoscopy tracer via esophageal MB injection was able to pick up the first fistula. However, we missed the second one at the first operation. So, the examination of the trachea by bronchoscopy is recommended and must be done carefully. To improve the test’s performance, it is recommended to instill MB using a catheter placed in the esophagus. The presence of a fistula is confirmed if MB passes through the fistula into the trachea.

The treatment of choice is surgical repair. The cervical approach is indicated for proximally located fistula, above the second thoracic vertebra, whereas the thoracic approach is recommended for those at a more caudal level [3, 16]. Surgery consists of ligation and division of the fistula and repair of the tracheal and esophageal walls. In most cases of reported double H-type TEFs, surgical repair was done subsequently. Mattei and al (9) reported the correction of one such fistula in one surgery. The thoracic fistula was corrected by surgical repair, and the cervical fistula was corrected by a balloon catheter. In our case, the double TEF was closed separately; the first was closed by a thoracotomy, and for the second TEF, we performed a cervical approach as it was located high in the thorax.