Recurrent Pneumonia-H Type Tracheoesophageal Fistula , Diagnostic Dilemma

Tracheoesophageal fistula (TEF) without associated esophageal atresia (EA) is a rare congenital anomaly.  Most of the children are treated for episodes of pneumonia prior to definitive diagnosis.  A 5 months infant presented with recurrent pneumonia and diagnosis of H type TEF was made with contrast esophagram. DOI: http://dx.doi.org/10.3126/jnps.v34i1.8517 J Nepal Paediatr Soc 2014;34(1):71-73


Introduction
P neumonia is common cause of childhood mortality and morbidity.During childhood recurrent episode of pneumonia need to be evaluated for predisposing factors.Underlying causes are recurrent aspira on, lung disease (airway stenosis, bronchiectasis and middle lobe syndrome or tracheooesophageal fi stula) and immune defi ciency.In signifi cant number of children no cause may be iden fi ed even a er extensive diagnos c work-up 1 .H-type TEF is one of rare predisposing factor for recurrent respiratory infec ons and accounts for 4% of all congenital tracheoesophageal malforma ons 2 .The clinical features are variable; common being the recurrent respiratory symptoms, aspira on during feeding with cyanosis, abdominal distension.These episodes are treated as recurrent pneumonia with intravenous an bio cs 3,4 .The symptoms are usually present since birth, but the diagnosis is o en delayed and requires high index of suspicion.

The Case
A fi ve months male child presented to Manipal College of Medical Science and Teaching Hospital with symptoms of cough and fever for three days.Baby was born through normal vaginal delivery in Western Regional Hospital Pokhara, weighing 3.05 kg with good APGAR scores.From the second day of life baby was treated for pneumonia with intravenous an bio cs for 10 days.Baby was treated for similar respiratory infec ons at 21 days, 45 days and 4 months.The anthropometric parameters and systemic examina on were essen ally unremarkable.Complete blood count, coagula on profi les and blood chemistry were in normal range.Chest X-ray showed pneumoni s (Fig. 1) Ultrasound abdomen, electrocardiogram and echocardiogram were unremarkable.With history of choking during feed tracheaeosophageal fi stula was suspected.A tube oesophagogram was performed which showed contrast fl owing into tracheobronchial tree through a fi stula between trachea and esophagus at the level of T2-T3 (Fig. 2).Repeat contrast study and bronchoscopy with catheter placement was planned prior to defi ni ve surgery.Pa ent did not turn up for follow up and opera ve fi ndings couldn't be confi rmed.Even if trea ng physician suspects H-type TEF, establishment of diagnosis in developing countries is diffi cult due to lack of inves ga ons.Many diagnos c methods have been advocated for the diagnosis of H-type fi stula.Oesophagogram is usually a reliable method to iden fy congenital H-type tracheoesophageal fi stula, though o en diffi cult, requiring mul ple a empts before the defect is confi rmed.Contrastenhanced studies have the poten al risk of aspira on pneumonia and pulmonary injury.Contrast studies can be performed with adequate arrangements for the emergency resuscita on.Three-dimensional computed tomography and virtual bronchoscopy may be useful techniques in pre-opera ve evalua on 6 .Endoscopic methods like bronchoscopy and oesophagoscopy have the advantage of being diagnos c allowing placement of a catheter across the fi stula to assist in its localiza on during surgery 7 .
Fi y percent of infants with esophageal atresia are nonsyndromic without other anomalies, and the rest have associated anomalies, most o en associated with the VATER or VACTERL (vertebral, anorectal, [cardiac], tracheal, esophageal, renal, radial, [limb]) syndrome 2 .Other associated anomalies includes CHARGE syndrome (coloboma of the eye, central nervous system anomalies; heart defects; atresia of the choanae; retarda on of growth and/or development; genital and/or urinary defects hypogonadism, Goldenhar's syndrome, esophageal stenosis and syndactyly 2,8 .Congenital malforma ons are less common in H-type TEF compared to other varie es of esophageal atresia 8,9 .None of these associa ons were found in the index case.
Diff erent surgical approaches have been described for this anomaly.For proximally located fi stula the approach of choice is cervicotomy and in cases of distal fi stula thoracotomy is usually preferred.Brookes et al reported seven pa ents of H-type TEF and one pa ent with a missed proximal H-type fi stula associated with esophageal atresia 5 .They presented with coughing while feeding, recurrent pneumonia, and episodic cyanosis.A delay in diagnosis was seen in four pa ents and ranged from 2.5 months to 5.9 years.In all pa ents, the diagnosis was made on oesophagogram.The level of the fi stulae was between C5 and T3, and all were successfully repaired via a right cervical approach.Biechlin et al reported a series of 8 cases of H-type TEF, all were repaired through right cervicotomy 10 .An alterna ve thoracoscopic approach in a newborn has recently been reported by Allal et al 11 .Surgery consists of liga on and division of the fi stula and repair of the tracheal and esophageal walls.Most

Conclusion
Though H-type of teacheoesophageal fi stula is a rare congenital anomaly, it should be considered as one of the diff eren al diagnosis for recurrent pneumonia in infants and children.

Fig 1 :
Fig 1: Pneumoni s right middle lobe and upper lobe

Fig 2 :
Fig 2: Contrast oesophagogram showing a contrast within the tracheobronchial tree sugges ve of TEF