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Congenital isolated ‘H-type’ tracheoesophageal stula (TOF) is a rare disorder which is dicult to diagnose. Clinical presentation is characterised
by a triad consisting of paroxysmal coughing and cyanosis during feeds; recurrent chest infections and failure to thrive; and abdominal
distention secondary to gaseous loading of the bowel. It is oen dicult to diagnose ‘H-type’ TOF because the continuity of the oesophagus is
not interrupted. e diagnosis is oen missed or delayed, leading to complications such as chronic lung disease and failure to thrive.
Keywords. tracheoesophageal stula; H-type isolated stula; polymerase chain reaction; microscopy and culture; Pneumocystis jirovecii
pneumonia; cytomegalovirus; HIV; retroviral disease.
Afr J Thoracic Crit Care Med 2022;28(4):195-197. https://doi.org/10.7196/AJTCCM.2022.v28i4.203
A tracheoesophageal stula (TOF) is an abnormal connection between
the trachea and oesophagus. A TOF arises as a result of failed fusion of
the tracheoesophageal ridge during the third week of embryological
development.[1] e incidence is 1 in 2 500 - 3 000 live births with a
male: female ratio of 1.26:1.[2]
A TOF without associated oesophageal atresia, commonly called
‘H-type’ or isolated stula, is a rare congenital anomaly.[3] e stula
runs from the posterior wall of the trachea downward to the anterior
wall of the oesophagus[4] and accounts for only 4 - 5% of all congenital
tracheoesophageal malformations.[5]
e clinical presentation is characterised by a triad consisting of:
(i) paroxysmal coughing and cyanosis during feeds; (ii) recurrent
chest infections; and (iii) abdominal distention secondary to gaseous
loading of the bowel.[5] It is oen dicult to diagnose ‘H-type’ TOF
because the continuity of the oesophagus is not interrupted; therefore,
the diagnosis is oen missed or delayed.
If le untreated, this condition can lead to multiple complications
such as recurrent pneumonia, failure to thrive and chronic lung
disease. It is advisable to perform prone contrast oesophagraphy as
well as tracheoesophageal bronchoscopy to conrm the diagnosis and
identify the precise location of the stula. Treatment of ‘H-type’ TOF
is an open surgical procedure, either through a cervical approach or
through a thoracotomy or thoracoscopy.[1] e patient presented here
developed recurrent respiratory infections, multiple admissions and
failure to thrive.
Case report
A 6-month-old female infant presented to a local hospital with a 9-day
history of non-productive cough and shortness of breath without
fever. She was admitted at the local hospital for a week and treated
with antibiotics (azithromycin, ampicillin, co-trimoxazole) but did
not respond to antibiotics clinically. She was transferred to our tertiary
hospital owing to unresolving pneumonia. On further inquiry, it was
found that she had had a persistent, non-productive cough since she
was 5 weeks old.
According to her mother, she failed to gain weight despite normal
feeding habits. ere was no history of loose stools, oedema or cyanosis.
She had been admitted thrice since birth, with main complaints
of cough and diculty in breathing. Her initial admission was at
5weeks of life, when she was admitted for 2 weeks with the diagnosis
of bronchiolitis. Her second admission was at 3 months, when she
was diagnosed with pneumonia and managed with antibiotics. Her
third admission was at 5 months, when she was rst diagnosed with
pneumonia and later started on treatment for pulmonary tuberculosis
based on clinical presentation and a chest radiograph.
The infant was born at term by normal vaginal delivery with a
birthweight of 2830 g, Apgar score of 9/10 at one minute and 10/10
at 5minutes. She was retroviral disease-exposed with a negative
HIV-PCR at birth. She missed her immunisations at 10 and 14weeks
owing to a stock shortage at the local clinic. e patient had been
exclusively formula-fed since birth and was on InfaCare formula at
admission. Solid feeds had not been introduced and her milestones
were appropriate for age. ere was no family history of tuberculosis
or asthma. On examination, she looked chronically ill with mild
respiratory distress, and she was on nasal-prong oxygen therapy (2 L/
min). Her vital signs were as follows: respiratory rate, 42 breaths per
minute (tachypnoeic); heart rate, 139 beats per minute (tachycardia);
temperature, 36.7 °C (normal); and blood pressure, 88/44 mmHg. e
infant’s anthropometric measurements were as follows: weight, 4 kg
(below the third percentile); weight-for-length score, below the third
percentile; and she was failing to thrive. ere were no signs of clubbing,
jaundice, oedema, pallor, cyanosis or hypopigmented skin lesions.
On examination of the respiratory system there were subcostal and
intercostal recessions with bilateral crepitation and no deformities.
‘H-type’ tracheoesophageal stula in an infant: A case report
M D Moremi-Letsoalo,1 BSc, MB ChB, FCPaeds, DCH, Dip Allergy, Cert Paed Pulm;
A B (Sebastian) van As,2 MB ChB, MMed, MBA, FCS (SA), PhD; N G Tiva,1 MB BCh, FCPaeds, Cert Paed Pulm;
R M Nchabeleng,1 MB ChB, FCPaeds, MMed; G Ramanyimi,1 MB ChB;
SRisenga,1 BSc, MB ChB, MMed (Paed), DCH, Dip Allergy, Cert Pulm Paed
1 Department of Paediatrics and Child Health, School of Medicine, Faculty of Health Sciences, University of Limpopo, Pietersburg Hospital, South Africa
2 Department of Surgery, School of Medicine, Faculty of Health Sciences, University of Limpopo, South Africa
Corresponding author: M D Moremi-Letsoalo (dietsa.makoma@gmail.com)
196 AJTCCM VOL. 28 NO. 4 2022
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e abdomen demonstrated hepatomegaly without splenomegaly.
Examination of the cardiovascular system demonstrated normal
pulses and no murmurs. ere were no neurological abnormalities.
The initial assessment was that of a 5-month-old female
infant, retroviral disease-exposed with a negative HIV-PCR at
birth, recurrent chest infection, failure to thrive and pulmonary
tuberculosis. e infant was admitted to the paediatric medical ward
to exclude primary immune deciency, cystic brosis, Pneumocystis
jirovecii pneumonia and cytomegalovirus (CMV) infection. She was
started on piperacillin/tazobactam, amikacin, nebulisation with
fenoterol, oral prednisone and tuberculosis treatment.
e following investigations were performed: sputum microscopy,
culture and sensitivity (MC&S) (no growth); GeneXpert test for
tuberculosis (negative); respiratory syncytial virus and adenovirus
(negative); P. jirovecii (negative); CMV DNA viral load (<1000 copies);
SARS-COVID-19 (negative). A full blood count, C-reactive protein
and blood culture were normal. A stool sample for faecal elastase
as well as a primary immune deciency screen were normal. Chest
X-rays showed bilateral inltrates and a chest scan report showed
bilateral multiple consolidation with no bronchiectasis. A small patent
ductus arteriosus (negligible) was observed on echocardiogram.
On observation in the ward, the infant coughed excessively during
feeds, a barium swallow contrast study was performed (Fig.1) and a
stula was identied between the trachea and oesophagus. e patient
was referred to paediatric surgery. Figs2 and 3 are intraoperative
images. Postoperatively, she recovered well and was discharged home
aer 2 weeks. At the time of publication of this report, the infant was
clinically well, gaining weight and no further cough had been observed.
Fig. 1. Barium swallow contrast study of the H-type tracheoesophageal
stula (red arrow).
Fig. 2. Intraoperative image of the TOF (le vascular loop) and the
oesophagus (right vascular loop). e trachea is visible above the TOF.
(TOF = tracheoesophageal stula.)
Fig. 3. Intraoperative image demonstrating the TOF. (TOF =
tracheoesophageal stula.)
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Discussion
Congenital isolated ‘H-type’ TOF is a rare disorder which is dicult
to diagnose. It accounts for 4% of all congenital oesophageal
malformations with an incidence of 1 in 2 500 - 3 000 livebirths.[2] It
was rst described by Lamb in 1873.[1] ‘H-type’ TOF is not associated
with oesophageal atresia and less associated with other congenital
anomalies. A high index of suspicion is necessary for diagnosis in
patients that present with exacerbation of a cough during feeds and
additional signs such as cyanosis, abdominal distension, choking
spells, recurrent respiratory infections and failure to thrive.
Pressure changes between the trachea and oesophagus can cause
entry of air into the oesophagus, or entry of oesophageal content into
the trachea, giving rise to the clinical presentation. Late or missed
diagnosis is due to variability in the anatomy of stulas.[8] ere is no
single investigation with sucient sensitivity and specicity to aid the
accurate diagnosis of ‘H-type’ TOF,[8] although chest radiographs are
helpful to check for signs of aspiration. A prone contrast oesophagram
can aid diagnosis. Tracheoesophageal bronchoscopy is diagnostic and
can also assist with precise location of the stula. Special position
and contrast administration techniques have been described as
aiding the diagnosis. To improve sensitivity, this test should be
performed with a nasogastric tube in situ and the child should be
in the prone position.[6] e nasogastric tube should be withdrawn
slowly while injecting contrast. Contrast-enhanced studies carry a
risk of aspiration pneumonia and should be performed with adequate
emergency resuscitation tools at hand. Bronchoscopy is also helpful in
determining the location of the stula, the presence of a double stula
and the position of the aortic arch.
Magnetic resonance imaging can also be used in making the diagnosis
before birth. Associated congenital abnormalities are more common
with other variants, whereas ‘H-type’ TOF includes least number of
associated congenital abnormalities (30% of cases).[1] e associated
abnormalities reported are vertebral defect, anal atresia, cardiac
defect, TOF, renal anomalies and limb abnormalities (VACTERL
syndrome). Common cardiac abnormalities include ventricular
septal defect, tetralogy of Fallot and chromosomal abnormalities.[9]
e surgical approach for ‘H-type’ TOF has been cervical, thorascopic
and thoracotomy techniques. Identifying the exact level of the stula is
crucial as this will dictate the operative approach. At or above the level
of the T2 vertebra, a trans-cervical approach can be utilised, while
open thoracotomy or a thoracoscopic approach is necessary below the
level of the T2 vertebra. [8]
e real advantage in utilising thoracoscopy over an open approach
remains open for debate.[8] Endoscopic closure of ‘H-type’ TOF has
also been reported with dierent techniques such as glue adhesion,
electrocautery, the use of sclerosant agents and laser ablation.[8]
Endoscopic electrocautery provides a less invasive approach to
management of’ ‘H-type’ TOF. However, this procedure may need to
be performed several times, which necessitates discussion with the
family and a contingency plan in the event of failure.[5]
The most common postoperative complication is recurrent
laryngeal nerve injury, with patients having diculty in breathing,
gastroesophageal reflux disease, vocal cord injury, dysphagia,
wheezing and respiratory tract infections.[2]
Conclusion
Diagnosis of ‘H-type’ TOF can be challenging and is oen missed in
the absence of a high index of clinical suspicion. Complications such
as failure to thrive and chronic lung disease are likely to occur if the
diagnosis is delayed. Early diagnosis and appropriate management
reduce morbidity and improve the prognosis. Tracheobronchoscopy
and prone oesophagram should be performed to confirm the
diagnosis. Surgical repair is the main treatment modality and a
multidisciplinary approach is required for the proper management
of ‘H-type’ TOF.
Declaration. None.
Acknowledgements. None.
Author contributions. Equal contributions.
Funding. None.
Conicts of interest. None.
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Accepted 27 September 2022.
