Double H-type TEF is Rare

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Double H-type tracheoesophageal fistula which is rare to see


Double H-type TEF is Rare


Congenital ‘isolated’ tracheoesophageal fistula (TEF) is a rare variant of esophageal atresia, also termed H-type TEF. Even rarer is a double H-type TEF. Here, we report a two-week-old female, with double H-type TEF – one identified pre-operatively, the other during surgery.

Pre-operative laryngotracheal bronchoscopy (LTB) with guidewire cannulation is considered key to definitive TEF repair, to aid in both pre- and intra-operative identification of the fistula(e). In our experience, only one H-type TEF was identified at the planned pre-operative LTB, with the second H-type fistula identified at the time of cervical dissection.

Both fistulae were repaired during the same operation, via a cervical approach. This represents the first reported case of a double H-type TEF, repaired primarily via a single cervical incision.

Double H-type TEF is Rare

The first case reported in the literature was a post-mortem autopsy report by Hübner, in 1943. Congenital ‘isolated’ tracheoesophageal fistula (TEF) without esophageal atresia (EA), also termed H-type TEF, is a rare variant of the EA spectrum.

The H-type TEF has been reported to occur in approximately 3–4% of patients within the EA spectrum. Since then, contemporary cases have reported double H-type TEF in survivors.

The report is about a case of a two-week-old female, which represents the first reported case of a double H-type TEF, repaired primarily via a single cervical incision.

Double H-type TEF is Rare

Case report

A two-week-old female was admitted to the neonatal intensive care unit for investigation of poor weight gain, noisy breathing after feeding, and episodes of cyanosis related to feeding. The patient was born via an emergency low uterine segment cesarean section, indicated for fetal bradycardia.

Her Apgar scores at one and five minutes, were nine and ten, respectively. Her gestational age was 39 + 2 weeks, and her birth weight was 2.8 kg (3.5% centile, z score −1.8), as compared to her admission weight of 2.74 kg (2.8% centile, z score −1.9).

This patient was initially investigated with an upper gastrointestinal contrast study, which showed a possible but equivocal appearance for H-type TEF.

She subsequently underwent a formal tube esophagogram, which did not confirm the finding of an H-type TEF. However, due to ongoing clinical suspicion, she was investigated further with laryngotracheal bronchoscopy (LTB) to examine for a fistula. Again, initially, no H-type TEF was seen.

However, following the injection of methylene blue into the esophagus, a fistulous connection to the airway was identified and the presence of an H-type TEF confirmed.

On the day of H-type TEF repair, the neonate underwent LTB, at which a clearly visible H-type TEF was cannulated with a 0.32″ guidewire, prior to exploration via a right cervical (supraclavicular) approach. However, during cervical exploration, two distinct H-type TEF were identified and dissected.

The more cranial TEF was encountered first and, notably, had no guidewire in situ.

This TEF demonstrated the wider caliber of the two, and its identity was confirmed by the passage of a 6Fr feeding tube into the esophagus with the aspiration of gastric contents, as well as by a secondarily placed 0.28″ guidewire into the trachea. Image intensification was utilized to confirm that both the originally placed 0.32″ and secondarily placed 0.28″ guidewires were in situ (in the trachea cranially and esophagus caudally).

The more caudal TEF was identified 8–10 mm caudally and contained the primarily inserted 0.32” guidewire. Both H-type TEF were divided and closed with interrupted 5/0 PDS (Polydioxanone) sutures.

Of note during the post-operative course was that extubation was delayed until 13 days post-repair due to suspected tracheal swelling.

Prior to discharge from the hospital on full breastfeeds, the patient was screened for the VACTERL (vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, limb abnormalities) association. Whilst no VACTERL anomalies were identified per se, the patient was shown to have sensorineural hearing loss in the right ear.

Subsequent to discharge from the hospital, the patient has had regular surgical and respiratory outpatient assessments. Over the ensuing four years, she has had no issues with feeding, gastroesophageal reflux, nor esophageal stricture.

A contrast study was performed at three years of age, in the setting of new symptoms of intermittent stridor and upper airway secretions. This showed an irregular esophageal mucosal appearance at the level of the repair, albeit with no evidence of a recurrent TEF

Double H-type TEF is Rare

An uncommon presentation of double H type of tracheoesophageal fistula

Abbreviations: TEF, tracheoesophageal fistula; VACTERL,
vertebralanalcardiovascular renal and limb
Tracheoesophageal fistula (TEF) is an abnormal connection
between the trachea and the esophagus.1
Most TEF abnormalities
are diagnosed at birth since 98% of them are associated with
esophageal atresia.2
The incidence is approximately 1 in 4000
live births and in some cases, it may associate with VACTERL
(Vertebral, Anal, Cardiovascular, Renal, and limb) anomalies.3 H type
of the tracheoesophageal fistula is rare and comprises only 4% of the
cases of TEF.4
In the H type, the fistula runs from the posterior wall
of the trachea downwards to the anterior wall of the esophagus.4 Double H
type of fistula is even less common with only a few cases reported.5
There is no associated esophageal atresia. These cases are difficult to
diagnose early because of nonspecific symptoms.6 If untreated it leads
to multiple complications such as recurrent pneumonia, lung abscess,
ARDS, acute lung injury, poor nutrition, bronchiectasis from recurrent
aspiration, respiratory failure, and death.1 Here we present a case of an
eight-year-old who presented with recurrent lower respiratory tract
infection mimicking asthma and was found to have double H type of
Case report
An 8-year-old boy came with a history of recurrent cough, breathing
difficulty since he was 8 months of age. Present episode was for 5
days with fever, purulent sputum, and breathlessness with a wheeze. The
child came for evaluation of refractory asthma to a pulmonary physician.
His mother had polyhydramnios in pregnancy. She delivered him by
full-term normal vaginal route. Following birth, he had tachypnea, and breathing difficulty and nasogastric tube could not be passed. He
was diagnosed with tracheo-oesophageal fistula and was operated.
Surgical closure of the upper fistula was done by a pediatric surgeon. The postoperative period was uneventful.
He was then asymptomatic for 8 months. At 8th month of age,
he was first diagnosed with pneumonia, admitted, and treated with
antibiotics. After 1 year of the previous episode, he again had a respiratory
infection for which he was treated. Thereafter, since 5 years of
age, he had been having recurrent episodes of cough with purulent
sputum, breathlessness with wheeze every 2 months for which he was
diagnosed to have Asthma and treated with inhaled bronchodilators
and inhaled steroids with not much relief. On examination, he was
febrile with pulse oximetry saturation 90%. He had tachycardia and
tachypnea. He was poorly built for his age. Respiratory examination
showed bilateral diffuse polyphonic rhonchi and coarse crepitations.
Another system examination was within normal limits.
Since the above presentation was not typical of Atopy and
Asthma, Computed Tomography of thorax with contrast was asked. It
showed the presence of tracheo-oesophageal fistula (TEF) with bilateral
pneumonia and bronchiectasis. Double H type of fistula was seen
with the upper fistula forming a pouch and the lower fistulous connection
persisting between the trachea and the esophagus. The diagnosis of recurrent
respiratory infection secondary to TEF was made. This case is unique
because the recurrence of TEF post-surgical correction is uncommon.
It also highlights the importance of a detailed birth history and its
clinical correlation.

TEF occurs due to the abnormal development of Tracheoesophageal
ridges in the fetus. There is a failure of lateral septation of the two tracts,
namely digestive and respiratory, resulting in the formation of an abnormal
communication between the two3
It usually occurs between the fourth to
the fifth week of development.7
It was first described in 1929 by Negus.
It was then an incidental postmortem finding in an adult who died
of lung carcinoma.3
Isolated H type of TEF is a rare disorder and is
hence challenging to diagnose.1
H type of fistula is not associated
with esophageal atresia.3 If the defect is large, then these cases present
early with respiratory symptoms due to aspiration and abdominal

distension.3 Depending on the pressure difference between two
structures, the air can enter into the esophagus or food particles can
enter into the trachea, thus causing symptoms.1 The common complaints
are recurrent respiratory infections, aspiration, and bloating of
the abdomen.8
Our patient had recurrent respiratory tract infection with
fever, purulent sputum, and bronchiectasis. This complication leading
to chronic lung disease in the form of bronchiectasis in later life is
because of repeated lung infection secondary to aspiration.9
A survey by Killen and Grenlee showed that 43% of cases were
diagnosed in 1st month while 83 % of cases were diagnosed in 1st year.10
Early diagnosis is considered up to 4 years of age.3
Our patient was
diagnosed and operated at birth. He however had a recurrence and
presented at a later age of 8 years which is rare.TEF antenatally
presents with polyhydramnios due to inability of the fetus to swallow
amniotic fluid. The incidence of polyhydramnios is variable. Some
studies have found an incidence of 61%, while a few others have
reported up to 91%.7
This is rarely seen in H type pf TEF.8
The mother
of a child had polyhydramnios in pregnancy. At the time of our
diagnosis, he was found to have H type of TEF.
The various modalities of diagnosis include esophagogram,11
Tc-99m sulfur colloid scintigraphy,12 Computed tomography13 and
Magnetic resonance imaging.14 The gold standard for diagnosis
however, is combined bronchoscopy and esophagoscopy with
passing of a fine catheter through the fistula.15 In our case, Computed
tomography of the thorax was able to pick up the fistula and its
associated complications.
The treatment is by surgical correction. This can be done by
the cervical route or thoracic route depending on the location of the
fistula.5,16 Surgery involves ligation of the fistula with correction of
the walls of the trachea and esophagus.1
Alternatively, closure of
the fistula has been attempted by the endoscopic approach using tissue
adhesives, electrocautery, and Nd:YAG laser.17 Few cases have been
reported were Double H fistula has been reported and subsequently
corrected by surgery. Mattei reported the correction of one such fistula
in one operation. The thoracic fistula was corrected by surgical repair
and the cervical fistula was corrected by a balloon catheter.18
This case highlights the importance of looking for a second fistula
while correcting H type of TEF. Everything that wheezes is not asthma.
In cases of refractory wheeze, detailed evaluation should be done for
cases that can mimic asthma and appropriately treated.