Spontaneous resolution of plastic bronchitis in a patient post hemi-Mustard/bidirectional Glenn atrial switch procedure in the double-switch operation for congenitally corrected transposition of great arteries after course of Augmentin

Abstract

We report the case of a five-year-old girl with plastic bronchitis after repaired complex congenital heart disease, who became asymptomatic after a short course of Augmentin. We report the disease regression as response either to antibiotic or as coincidental with spontaneous resolution.

Introduction

Plastic bronchitis is a rare disease of unclear etiology characterized by the formation of airway casts that may be coughed up, found at bronchoscopy, or in surgical specimens [1,2]. It is associated with an underlying cardiac or pulmonary pathology which can lead to life-threatening airway obstruction [2,3].

Several therapies have been used for patients with plastic bronchitis, including medical and interventional procedures, but recurrences are common and mortality remains high [4]. We report our case of disease regression as either response to antibiotic or coincidental with spontaneous resolution.

Case report

Our patient is a five-year-old girl with complex congenital heart disease diagnosed with corrected transposition, ventricle septal defect, and pulmonary stenosis. She underwent palliative Blalock–Taussig shunt at two months of age, and corrective surgery at one year of age, in a double switch operation using hemi-Mustard/bidirectional Glenn atrial switch and right ventricle to pulmonary artery conduit size 14 mm (Contegra®). After surgical operation, the patient developed chylothorax with right-sided effusion but despite medical and supportive treatment, the chylothorax persisted. The patient subsequently underwent thoracic duct ligation and improved. One month later, however, she presented with right-sided chylothorax again, where she was treated for six weeks with fat free formula (Monogen®), fat free diet and chest drain. She was asymptomatic after that with regular follow-up at our center. Two months after surgery, she underwent diagnostic cardiac catheterization. Her hemodynamic monitoring showed patent Glenn circulation, with mean pulmonary artery pressure at 12 mmHg, oxygen saturation at 90% in room air. At follow-up, her oxygen saturation ranged between 88% and 92%.

At four years of age, the patient was admitted to a local hospital coughing up large rigid branching casts (Fig. 1). She was treated at the local hospital with bronchodilators, but with no response. Two months later, she became febrile with flu-like illness and was started on a course of Augmentin, 500 mg, three times daily for seven days. She improved significantly after the antibiotic course, and her productive cough ceased. We maintained follow-up, and eight months later, our patient continues to be asymptomatic.

Figure 1.

A large branching cast expectoration.

Discussion

Plastic bronchitis is a rare association of congenital heart disease, which has poor prognosis and a mean reported mortality of 33% (14–50%) [5].

The prevalence of plastic bronchitis in patients who have undergone the Fontan procedure is estimated to be as high as 4–14% [6].

Hemodynamic stabilization, residual obstruction exclusion, thoracic duct ligation, dietary modification and rhythm optimization were suggested treatment options for children with congenital heart disease and plastic bronchitis [5].

Oral macrolide antibiotics are described as treatment options [7]. However, Augmentin was used with our patient and a fast response ensued, which we think is worth reporting for possible future inclusion in medical therapy trials.