Table of Contents  
Year : 2015  |  Volume : 8  |  Issue : 3  |  Page : 354-357  

H-type tracheo-esophageal fistula in a very low birth weight infant: An unexpected and diagnostic challenge for neonatologist

1 Department of Neonatology, Sunrise Hospital, Hyderabad, India
2 Department of Neonatology, Fernandez Hospital, Hyderguda, Andhra Pradesh, India
3 Department of Neonatology, ACPM Medical College, Dhule, Maharashtra, India

Date of Web Publication15-May-2015

Correspondence Address:
Deepak Sharma
Fernandez Hospital, Hyderguda, Hyderabad, Andhra Pradesh - 500 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0975-2870.157085

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Tracheo-esophageal fistula (TEF) without associated esophageal atresia or H-type fistula is a rare congenital anomaly. H-type fistula is usually missed in the neonatal period as the presenting symptoms are either of recurrent pneumonia or gastro-esophageal reflux which always lead to delay in diagnosis and infant undergoes unnecessary treatment. We report a case of H-type of TEF, diagnosed within 12 days of birth based upon choking and cyanosis on the first trial of spoon feeds. Diagnosis was confirmed with contrast esophagogram. The infant was operated for it and was successfully discharged.

Keywords: Esophagogram, fiber optic bronchoscopy and esophagoscopy, gastro-esophageal reflux, H-type fistula, recurrent pneumonia, tracheo-esophageal fistula

How to cite this article:
Parakh H, Kapoor MS, Sharma D, Pandita A, Shastri S. H-type tracheo-esophageal fistula in a very low birth weight infant: An unexpected and diagnostic challenge for neonatologist. Med J DY Patil Univ 2015;8:354-7

How to cite this URL:
Parakh H, Kapoor MS, Sharma D, Pandita A, Shastri S. H-type tracheo-esophageal fistula in a very low birth weight infant: An unexpected and diagnostic challenge for neonatologist. Med J DY Patil Univ [serial online] 2015 [cited 2023 Sep 22];8:354-7. Available from:

  Introduction Top

A trachea-esophageal fistula (TEF, or TOF); is an unusual connection which occurs between the esophagus and the trachea. Congenital TEF occur as a result of the failure of fusion of the tracheo-esophageal ridges during the 3 rd week of embryological development. [1] Of all varieties of TEF, H-type fistula accounts for about 4% of the total cases and has been reported with an incidence of around 1:50 000-80,000 births. [2] The classical triad of symptoms of H-type fistula includes recurrent respiratory symptoms, aspiration during feeding with cyanosis, and abdominal distension. In spite of recent advances in imaging modalities for diagnosis of H-type TEF, it still remains a rare entity, and few case reports are there in the literature where it was reported in adulthood. [3],[4],[5],[6] The late presentation of these fistulas is in the form of chronic lung disease, which results because of repeated aspiration leading to recurrent infection and bronchiectasis. [5]

This is a case report of a term intrauterine growth retardation baby who presented to nursery on day-1 with respiratory distress and was diagnosed as H-type TEF on day 12 of life. The novelty of our case report is early diagnosis and the birth weight of the neonate. The neonate was only 1500 g in birth weight, which is a bad prognostic factor, but in spite of this we were successful in the discharge of the neonate in well condition.

  Case Report Top

A female baby was born to Gravida2 Para1 Live1 mother whose pregnancy was complicated by severe pregnancy-induced hypertension. Mode of delivery was an emergency caesarean section in view of abnormal Doppler study and oligohydramnios. Gestational age was 38 weeks with birth weight was 1500 g (<10 th centile), length 43 cm (<10 th centile) and head circumference was 30 cm (<10 th centile) with Ponderal index of 1.91. Apgar scores were 8, 9, and 9. She was admitted to the nursery with complaints of respiratory distress. Chest radiograph done was suggestive of transient tachypnea of the newborn. Respiratory distress subsided within 12 h, after which oxygen was stopped. Orogastric feeds started on day-1 of life which baby tolerated well. Days 2 of the life spoon feeds tried upon which baby had an episode of cyanosis. Baby was investigated with sepsis screen that was positive. Hence, antibiotics started and the baby continued on Ryles tube feeds were continued as no feed intolerance was documented. Feeding was slowly advanced to full feeds by day 9 of life. On day 9 of the life spoon feeds were tried, but the cough was present with the same. Chest was clear and chest radiograph was normal. Gastro-esophageal reflux (GER) was considered, and antireflux measures were started. On day 11 of life upon the spoon feeds baby had an episode of cyanosis and desaturation followed by seizures for which phenobarbitone was given. Sepsis screen was repeated, which showed increasing platelets and decreased C-reactive protein. Electroencephalography did borderline abnormal hence baby was continued on maintenance phenobarbitone. On day 12, after the 24 h seizure free interval tube feeds restarted, which baby tolerated well, but the cough persisted in attempting spoon feeds. In view of recurrent episodes of desaturation and cyanosis on spoon feeds, Gastrograffin study was done. Gastrograffin was given through a nasogastric tube into the esophagus at different levels. TEF was noted between upper esophagus and the mid trachea (H-type) with trachea bronchial tree opacification. Fistula measured 3.5 mm long and 2 mm width with Grade III GER [Figure 1],[Figure 2] and [Figure 3]. Baby was operated for the fistula. Postoperative course was uneventful, and the baby was discharged and now in regular follow-up.
Figure 1: Gastrograffin study showing H-type fistula with grade III gastro esophageal reflux

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Figure 2: Gastrograffin study showing H-type fistula with grade III gastro esophageal reflux

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Figure 3: Gastrograffin study showing H-type fistula with grade III gastro esophageal reflux

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  Differential Diagnosis Top

  • Recurrent pneumonia.
  • GER.

  Discussion Top

Congenital TEF without esophageal atresia (EA) (H-type) is an extremely rare type of esophageal anomaly, which usually presents after neonatal period and is very difficult to diagnose. The reported incidence in the literature varies from a range of 1.8% to 4.2% [7],[8] among all types of TEF. Neonates with H-type TEF generally present with choking episodes and cyanotic spells during feeding, which was reproducible during a "test feed" as seen in our case. H-type TEF usually presents with symptoms from neonatal periods, though the presentations usually vary among different patients. These symptoms are usually confused with GER disease or with recurrent pneumonias. In the majority of the cases, infants may become symptomatic only when they are older with recurrent or multiple pneumonias. The H-type fistula is usually located in the cervical region. It has the lowest incidence of associated congenital malformations and have the best prognosis among all varieties of TEF. Usually, TEF present antenatally with polyhydraminos, but this finding is rarely seen with H-type of TEF and fetus are usually appropriate for gestational weight. Clinically, it is characterized by the classic triad which is usually seen in patients.

  • Recurrent episodes of choking and coughing with feeding,
  • Recurrent lower respiratory tract infections.
  • Abdominal distension as air passes through the fistula into the stomach.

Patients of TEF must be screened for associated malformations including VACTERAL associations (vertebral defects, imperforate anus, cardiac defects, TEF with EA, renal dysplasia or defects and limb anomalies. [9],[10]

A review of reported cases by Killen and Greenlee noted that the diagnosis was made within the 1-month of life in 43% and within the 1-year in 83%. [11] The diagnosis requires a high index of suspicion. Chest radiograph is nonspecific and may show evidence of aspiration and gastric dilatation. The diagnosis can be established by contrast study like esophagogram, which will be able to diagnose fistula in 80% of cases. [12] A noninvasive technique to diagnose an H-type TEF is Tc-99 m sulfur colloid scintigraphic technique. [13] The gold standard for the diagnosis includes combined fiber optic bronchoscopy and esophagoscopy with passage of fine balloon catheter from trachea to esophagus. [14] Computed tomography scan and magnetic resonance imaging can also be used for diagnosis and localization of H-type TEF safely and accurately in a sick preterm infant seen as a hypo intense structure due to air content. [15],[16] H-type TEF fistulas are managed surgically with ligation of the fistula, and generally transcervical approach is preferred but in few rare cases, a transthoracic surgical approach is done, depending on the location of the fistula. Due to this reason, accurate documentation of the location of the fistula is extremely important for preoperative planning. [17],[18] Long-term postoperative complications of TEF fistula include anastomotic site leakage, growth retardation, dysphagia, recurrent pneumonia, obstructive airway disease, airway hyperreactivity, GER disease, tracheomalacia, recurrent TEF, recurrent infections, esophageal stenosis and wheezing illness. [19],[20]

Choudhury et al. in their study of survival of patients with EA: Influence of birth weight, cardiac anomaly, and late respiratory complications reported that the mortality rate among 240 patients with congenital EA/TEF between 1990 and 1997 was 9.2%. Thirty-nine percent had an early, in-hospital deaths, which were a result of EA/TEF, and the rest were due to associated congenital malformations, especially cardiac defects. Sixty percent of late deaths, which were recorded till 2 years of age following repair, were due to respiratory causes, including aspiration, tracheomalacia, GER, sudden infant death syndrome, and reactive airways disease. [21]

Somppi et al. in their study to evaluate the outcome and late sequelae of patients with EA or TEF reported that out of 60 patients treated, 33% of them reported having impaired quality of life due to various reasons like respiratory infections, dyspnea, and difficulties in swallowing and coughing at night, whereas 18% had GER symptoms. Tracheo-bronchoscopy showed tracheal narrowing and inflammation in one-third; in histopathological analysis, however, the rate of inflammation was more than doubled. Histological examination showed esophageal inflammation in 51%, Barrett's esophagus in 6% and a  Helicobacter pylori Scientific Name Search on in 21% of cases. [22]

  Learning Points/Take Home Messages Top

  • H-type TEF is a rare anomaly with a better prognosis if diagnosed earlier.
  • A high index of clinical suspicion and classical symptoms are key to diagnosis.
  • Contrast esophagogram can be considered investigation of choice for the diagnosis of H-type TEF.

  References Top

Clark DC. Esophageal atresia and tracheoesophageal fistula. Am Fam Physician 1999;59:910-6, 919.  Back to cited text no. 1
Crabbe DC. Isolated tracheo-oesophageal fistula. Paediatr Respir Rev 2003;4:74-8.  Back to cited text no. 2
Ng J, Antao B, Bartram J, Raghavan A, Shawis R. Diagnostic difficulties in the management of H-type tracheoesophageal fistula. Acta Radiol 2006;47:801-5.  Back to cited text no. 3
Holman WL, Vaezy A, Postlethwait RW, Bridgman A. Surgical treatment of H-type tracheoesophageal fistula diagnosed in an adult. Ann Thorac Surg 1986;41:453-4.  Back to cited text no. 4
Newberry D, Sharma V, Reiff D, De Lorenzo F. A "little cough" for 40 years. Lancet 1999;354:1174.  Back to cited text no. 5
Waterston DJ, Carter RE, Aberdeen E. Oesophageal atresia: Tracheo-oesophageal fistula. A study of survival in 218 infants. Lancet 1962;1:819-22.  Back to cited text no. 6
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.  Back to cited text no. 7
Zacharias J, Genc O, Goldstraw P. Congenital tracheoesophageal fistulas presenting in adults: Presentation of two cases and a synopsis of the literature. J Thorac Cardiovasc Surg 2004;128:316-8.  Back to cited text no. 8
Upperman JS, Gaines B, Hackam D. H-type congenital tracheoesophageal fistula. Am J Surg 2003;185:599-600.  Back to cited text no. 9
Kirk JM, Dicks-Mireaux C. Difficulties in diagnosis of congenital H-type tracheo-oesophageal fistulae. Clin Radiol 1989;40:150-3.  Back to cited text no. 10
Killen DA, Greenlee HB. Transcervical repair of h-type congenital tracheo-esophageal fistula: Review of the literature. Ann Surg 1965;162:145-50.  Back to cited text no. 11
Laffan EE, Daneman A, Ein SH, Kerrigan D, Manson DE. Tracheoesophageal fistula without esophageal atresia: Are pull-back tube esophagograms needed for diagnosis? Pediatr Radiol 2006;36:1141-7.  Back to cited text no. 12
Vatansever U, Acunas B, Salman T, Altun G, Duran R. A premature infant with h-type tracheoesophageal fistula demonstrated by scintigraphic technique. Clin Nucl Med 2006;31:451-3.  Back to cited text no. 13
Nagata K, Kamio Y, Ichikawa T, Kadokura M, Kitami A, Endo S, et al. Congenital tracheoesophageal fistula successfully diagnosed by CT esophagography. World J Gastroenterol 2006;12:1476-8.  Back to cited text no. 14
Little DC, Rescorla FJ, Grosfeld JL, West KW, Scherer LR, Engum SA. Long-term analysis of children with esophageal atresia and tracheoesophageal fistula. J Pediatr Surg 2003;38:852-6.  Back to cited text no. 15
Gunlemez A, Anik Y, Elemen L, Tugay M, Gökalp AS. H-type tracheoesophageal fistula in an extremely low birth weight premature neonate: Appearance on magnetic resonance imaging. J Perinatol 2009;29:393-5.  Back to cited text no. 16
Karnak I, Senocak ME, Hiçsönmez A, Büyükpamukçu N. The diagnosis and treatment of H-type tracheoesophageal fistula. J Pediatr Surg 1997;32:1670-4.  Back to cited text no. 17
González Temprano N, Viguria Sánchez N, Ayuso González L, Pérez Martínez A. H-type tracheoesophageal fistula in neonates: Different therapeutic approaches. An Pediatr (Barc) 2014;81:e50-1.  Back to cited text no. 18
Kovesi T, Rubin S. Long-term complications of congenital esophageal atresia and/or tracheoesophageal fistula. Chest 2004;126:915-25.  Back to cited text no. 19
Shah AR, Lazar EL, Atlas AB. Tracheal diverticula after tracheoesophageal fistula repair: Case series and review of the literature. J Pediatr Surg 2009;44:2107-11.  Back to cited text no. 20
Choudhury SR, Ashcraft KW, Sharp RJ, Murphy JP, Snyder CL, Sigalet DL. Survival of patients with esophageal atresia: Influence of birth weight, cardiac anomaly, and late respiratory complications. J Pediatr Surg 1999;34:70-3.  Back to cited text no. 21
Somppi E, Tammela O, Ruuska T, Rahnasto J, Laitinen J, Turjanmaa V, et al. Outcome of patients operated on for esophageal atresia: 30 years′ experience. J Pediatr Surg 1998;33:1341-6.  Back to cited text no. 22


  [Figure 1], [Figure 2], [Figure 3]

This article has been cited by
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[Pubmed] | [DOI]


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