Recurrent Wheezing in Pre-school Age: Not Only Airway Reactivity!

CASE REPORT published: 17 March 2020 doi: 10.3389/fped.2020.00101 Recurrent Wheezing in Pre-school Age: Not Only Airway Reactivity! Marco Roversi 1 , Federica Porcaro 2*, Paola Francalanci 3 , Adriano Carotti 4 and Renato Cutrera 2 1 Academic Department, University of Rome Tor Vergata, Rome, Italy, 2 Paediatric Pulmonology and Respiratory Intermediate Care Unit, Sleep and Long-Term Ventilation Unit, Academic Department of Paediatrics, Research Institute, Bambino Gesù Children’s Hospital, Rome, Italy, 3 Department of Pathology, Research Institute, Bambino Gesù Children’s Hospital, Rome, Italy, 4 Unit of Pediatric Cardiac Surgery, Research Institute, Bambino Gesù Children’s Hospital, Rome, Italy Background: About a fifth of all mediastinal masses are primary cysts arising in the absence of other underlying pathology. Bronchogenic cysts, although rare, are the most frequent type responsible for lower airways compression as they often develop in the peripheral branches of the tracheobronchial tree. Edited by: Michele Torre, Giannina Gaslini Institute (IRCCS), Italy Reviewed by: Giselle Cuestas, Hospital Pedro de Elizalde, Argentina Pierre Goussard, Stellenbosch University, South Africa *Correspondence: Federica Porcaro Case presentation: We report the case of a 6-months-old child admitted for acute respiratory distress and wheezing not responsive to asthma treatment. Digestive and airway endoscopy proved a mild and a marked reduction of the esophageal and tracheal lumen, respectively. The nocturnal polygraphy showed an underlying obstructive disorder and the chest CT scan confirmed the presence of a wide mediastinal cyst compressing the trachea. The mass, later identified as a bronchogenic cyst, was surgically removed with complete resolution of the patient’s respiratory symptoms. Discussion: Our case shows that differential diagnosis of wheezing in pre-school aged children should encompass causes others than airway reactivity, thus prompting further evaluation and management. Keywords: pediatrics, wheezing, asthmatic bronchitis, bronchogenic cyst, airways abnormalities BACKGROUND Specialty section: This article was submitted to Pediatric Pulmonology, a section of the journal Frontiers in Pediatrics Received: 24 November 2019 Accepted: 27 February 2020 Published: 17 March 2020 Citation: Roversi M, Porcaro F, Francalanci P, Carotti A and Cutrera R (2020) Recurrent Wheezing in Pre-school Age: Not Only Airway Reactivity! Front. Pediatr. 8:101. doi: 10.3389/fped.2020.00101 Frontiers in Pediatrics | www.frontiersin.org Despite being one of the most common finding in infants and children, wheezing never ceased to be an alarming symptom for both the parents and the physician. It consists of a continuous sound heard during normal expiration or inspiration when airways obstruction is severe (1). A wheezing sound is usually caused by turbulent airflow passing through a narrowed medium-sized airway. Particularly under pre-school age (<6 years), a heterogeneous group of diseases, ranging from a self-limited viral process to a life-threatening disease, can be responsible for this symptom (2, 3). Diagnosis and treatment of young children with wheezing can thus be challenging and assessment of any kind of wheezing should always include a careful examination and detailed medical history, comprising the time of onset and the concurrent clinical manifestations (Figure 1). A chronic wheezing unresponsive to any treatment should prompt further evaluation with advanced imaging as to exclude congenital anomalies of the tracheobronchial tree, comprising vascular rings and slings (4), or a mediastinal mass (Table 1). We discuss the case of an infant with persistent wheezing and acute respiratory failure due to a large mediastinal mass compressing the lower airways. 1 March 2020 | Volume 8 | Article 101 Roversi et al. Wheezing Without Bronchial Hyperreactivity FIGURE 1 | Flow chart on differential diagnosis of wheezing in children. The main causes of acute wheezing are highlighted in red, whereas the conditions underlying a chronic wheezing are colored in blue. their surroundings. Blood tests and microbiologic analysis on respiratory secretions were negative. The echocardiography was limited by a poor acoustic window (due to significant air trapping) and was inconclusive for vascular rings. Based on the history of recurrent symptoms partially responsive to inhaler short term β2 agonists, the patient underwent airway and digestive endoscopy, which revealed a severe tracheomalacia at the T2-T3 level. Antero-posterior compression with a 1:1 ratio between the cartilage rings and the pars membranacea was observed. Anteriorly, the trachea appeared to be compressed by a pulsating mass. No abnormal communications between the airways and the digestive tract were found. Given the airway compression, a nocturnal polygraphy with overnight oximetry was carried out and proved the underlying obstructive disorder. In order to define the extrinsic compression and quantify the tracheal collapse, a dynamic chest CT scan with contrast enhancement was carried out and revealed a 4.0 cm wide mediastinal mass closely adherent to the anterior profiles of the first five thoracic vertebra, both compressing and dislocating the trachea and esophagus to the front and to the right, respectively (Figures 2A,B). Integration with ultrasound imaging directed at the jugulum confirmed the presence of a thin walled anechoic cyst. The patient underwent median sternotomy followed by opening of the pericardium and lateralization of the great vessels; total thymectomy was also made necessary to access the mass. On lowering the right pulmonary artery, the voluminous mass was appreciated, tightly adherent to the pars membranacea of the trachea and easily detachable from the esophagus. The cyst, filled with clear liquid and not communicating with the foregut, was therefore punched to reduce its size and facilitate dissection. Histopathological analysis later identified a cystic formation covered by respiratory epithelium and without smooth muscle in the walls, namely a bronchogenic cyst. The postoperative endoscopy revealed complete resolution of the tracheal compression at the T2-T3 TABLE 1 | Causes of recurrent/chronic wheezing in children. Tracheo-bronchomalacia* Vascular compression/rings* Tracheal stenosis/web* Extrinsic compression of trachea-bronchial tree (cyst or tumor, lymphadenopathy, cardiomegaly) Asthma Gastroesophageal reflux, aspiration Not recognized foreign body Bronchopulmonary dysplasia Cystic fibrosis Primary ciliary dyskinesia Immunodeficiency Bronchiolitis obliterans *These alterations tend to be present in pre-school aged children. CASE PRESENTATION A 6-months-old child was admitted at our hospital for acute respiratory failure. Her family history was positive for atopy. She was born at term from a vaginal birth and an uncomplicated pregnancy. Weight at birth was 2,790 g and respiratory distress in t (...truncated)