Keywords
pulmonary torsion - esophageal atresia - postoperative complication
Introduction
Esophageal atresia is an anatomical and physiological disorder of the esophagus requiring
urgent surgical intervention. The most common early complications of esophageal atresia
repair include leakage of the anastomosis, recurrent tracheoesophageal fistula, and
anastomotic stricture. We present a case of atypical postoperative complication in
the form of pulmonary torsion comprising the middle lobe of the right lung.
Case Report
A 3,260 g preterm female neonate, delivered at 38 weeks' gestation, evaluated at 7/8
points in Apgar scale, was referred to our department with prenatally diagnosed esophageal
atresia. On contrast, chest X-ray performed directly after birth the presence of congenital
defect—esophageal atresia with tracheoesophageal fistula—was confirmed (the upper
end of esophagus visible at the level of Th4–5, dilated intestinal loops filled with
air). The child was operated on the second day of life by the right laterodorsal thoracotomy.
The tracheoesophageal fistula was dissected and sutured, and esophago-esophageal anastomosis
was performed. Before closure, the lungs were aerated by forced ventilation, continued
until all collapsed regions fully expanded. During postoperative course, on control
chest X-rays, there was atelectasis in the upper lobe and the middle lobe of the right
lung ([Fig. 1]). On ultrasound examination, no pleural effusion was noted. On the sixth postoperative
day, contrast-enhanced computed tomographic (CT) scan was performed and revealed abrupt
termination of the bronchial airways with atypical course of horizontal interlobal
fissure ([Fig. 2]). Right pulmonary artery was visible, its lobar branches hardly visible; right main
bronchus showed typical diameter for approximately 7 mm from the bifurcation, then
narrowed to approximately 1.5 mm (compression from outside?) and continued as a single
bronchus to the lower lobe. There were neither any branches to the upper lobe nor
to the middle lobe. Thus, bronchography was performed, which showed obstruction of
the upper lobe bronchus and bronchus intermedius and the middle lobe bronchus of the
right lung ([Fig. 3]).
Fig. 1 Chest X-ray, fifth postoperative day.
Fig. 2 Computed tomography of the chest, sixth postoperative day.
Fig. 3 Bronchography, sixth postoperative day.
The patient was qualified for tracheobronchoscopy, which confirmed occlusion of the
upper and middle lobar bronchi (trachea—wide, smooth, site of sutured fistula wide,
patent, tracheal bifurcation normal. Main bronchus: left, normal; right, narrowing
at ∼ 10 mm from bifurcation, with a small fissure in the sagittal plane). The neonate
was qualified for second thoracotomy on the ninth day of life. On exploration, massive
infarction of the upper and middle lobe of the right lung was revealed. Anastomosis
site and site of tracheoesophageal fistula were healed. A 180-degree rotation of the
right middle lobe in a clockwise direction was found, which caused traction and compression
of vessels to the upper lobe. The lobe was untwisted, with return of inflation in
the upper lobe under vision. In the middle lobe after detorsion inflation was seen
only in fragments, however significant decrease of edema and hemostasis was seen,
thus no resection was attempted.
Postoperatively, the patient recovered normal respiratory function (initially ventilation
synchronized intermittent mandatory ventilation with low parameters, from 20th postoperative
day spontaneous respiration, chest drain closed on 3rd postoperative day, removal
of the drain on the 5th postoperative day, feeding via gastric tube from 3rd postoperative
day, discharged home on 35th postoperative day). The patient was then transferred
to a cardiology department for diagnostics of arrhythmia and later, at the age of
5 months, to a specialist pulmonary hospital, where the middle lobe was removed because
of atelectasis.
Discussion
Torsion of a lobe of a lung is a severe, life-threatening event, though extremely
uncommon. It may occur spontaneously after thoracic trauma, thoracic or cardiac surgery.[1]
[2]
[3]
[4]
[5]
[6]
[7] In children, it is considerably less frequent than in adults. So far, according
to our knowledge, only nine cases have been described in pediatric literature, including
one case of lung torsion complicating tracheoesophageal fistula repair.[1]
[2] Mortality in adult patients ranges from 12 to 16%.[8] Pulmonary torsion causes clinical deterioration in the face of normal arterial blood
gases and requires a high index of suspicion for diagnosis. The diagnosis is usually
suggested on chest X-ray and confirmed by CT scans. On chest X-rays consolidation
and opacification of the affected lung fragment, bronchial cutoff or distortion with
mediastinum shift and lobar air trapping can be seen.[9]
[10] The diagnosis was delayed by 14 days in some reports[8]
[11] because of unequivocal results of imaging studies and the fact that clinical findings
may be seemingly inconspicuous. Pediatric patients may present with tachycardia, fever,
tachypnea, abnormal blood tests, reduced breath sounds or chest movements, dullness
to percussion, and increased respiratory effort.[5]
[7]
[8] If pulmonary torsion is suspected basing on clinical history and plain radiographs,
chest CT scan should be performed. CT findings comprise bronchial obstruction or distortion
and abnormal pattern of blood vessels.[3]
[4]
[7]
[9]
[10] Bronchoscopy is a useful tool in the diagnosis of pulmonary torsion.[9]
[10] A complete interlobar fissure, with no parenchymal bridges between the neighboring
lobes, and an absent inferior pulmonary ligament have been described as predisposing
factors.[2] The accepted treatment of pulmonary torsion is detorsion or removal of the affected
lobe.[10]
[11] So far, among the eight cases described in the literature, seven cases have been
operated, with five resections[2]
[4]
[5]
[7]
[12] and two detorsions.[1]
[6] Detorsion of a lung or a lobe of doubtful viability is controversial, as it may
be connected with release of inflammatory mediators and necrotic tissue, leading to
a systemic response and multiorgan failure.[1]
[13]
[14] In the two described cases of detorsion, one patient recovered completely,[1] whereas in the second case the lung remained shrunken and nonfunctioning.[6] In this case, detorsion allowed for better assessment of the lobe viability, and
decrease in edema and hemostasis were observed. In the case of our patient, the torsion
was probably because of an anatomic anomaly—absence of parenchymal bridges between
contiguous lobes. Earlier reoperation would have probably been beneficial, but as
this complication is extremely rare in the pediatric population, diagnostic process
may take longer. Persistent atelectasis of a lung/lobe after thoracotomy should call
attention to a possibility of torsion.
Conclusions
The following can be concluded from this case report:
-
Pulmonary torsion is a severe, life-threatening event, rarely occurring in children.
-
Early diagnosis (X-ray/CT/bronchography/bronchoscopy) may be lifesaving. Clinical
deterioration in the face of normal arterial blood gases should rise a high index
of suspicion for diagnosis.
-
Treatment is surgical, detorsion if possible, or if the lobe is not viable—resection.
