1 - Serviço de Cirurgia Pediátrica; Hospital de Dona Estefânia, CHULC, EPE
2 - Serviço de Cirurgia Pediátrica do Hospital Central do Funchal
3 - Unidade de Pneumologia Pediátrica; Departamento de Pediatria, CHULC, EPE
- E-Poster – 20th International Congress on Pediatric Pulmonology, 24 -27th June 2021 (online)
Introduction: Congenital pulmonary airway malformation (CPAM) is a rare abnormality of lung development with an incidence about 1,5 cases per 10,000 live births. CPAM can be diagnosed on antenatal ultrasounds or appears clinically as acute respiratory distress, recurrent pulmonary infection, asymptomatic incidental discovery, and rarely as a pneumothorax. We report a rare case of a health’s baby who suffered a pneumothorax as the initial manifestation of CPAM at 5 weeks of age.
Case report: A 5-week-old female child came to the emergency department of a peripheral hospital for sudden onset of breathing difficulties, without fever or other symptoms. From personal history, it was a monitored pregnancy, with no reported anomalies on the antenatal ultrasounds; born at 39 weeks gestation by cesarean section; hospitalization at 20 days of life for late sepsis without isolated agent. On physical examination, she was tachycardic, polypneic without cyanosis, with intercostal retraction and decreased breathing sounds in the left hemithorax. Blood tests without changes. Research for adenovirus and respiratory syncytial virus was negative. Arterial blood gas test with mild respiratory acidosis and chest X-ray revealed hypertransparent image on the left hemithorax looking like intrathoracic bowel, pulmonary atelectasis and right mediastinal shift suggestive of left diaphragmatic hernia. The child was transferred to our hospital center and underwent an exploratory laparotomy using a left-side subcostal incision; the operative findings revealed only a small left diaphragmatic eventration. We performed a diaphragmatic plication and placed a chest tube with improvement of ventilatory parameters. Chest X-ray after surgery confirmed partial pulmonary expansion. Postoperative CT scan revealed secondary pneumothorax due to cystic pulmonary malformation in the left lower lobe (Stocker type I). Extubated on the 9th day and discharged on the 17th day of hospitalization. Currently she has 3,5 months-old, is asymptomatic and is awaiting an elective left inferior lobectomy.
Discussion/Conclusion: CPAM is characterized by an overgrowth of immature primary bronchioles localized at a segment of the bronchial tree. Stocker classifies CPAM into five types that differ by location, cystic structure, size, and epithelial lining. The type I lesion, as in our child, is the most common and consists of multiple large cysts (> 2 cm in diameter) of varying sizes. This type of lesion has the best prognosis. The postnatal clinical spectrum of CPAM ranges from the neonate requiring mechanical ventilation, to the child presenting recurrent CPAM-related pneumonias, to the asymptomatic adult. The presentation as pneumothorax in early period of life is rare and may be caused by spontaneous rupture of a dominant cyst probably enlarged by the child respiration. The diagnosis in neonates and infants is made by chest radiographs or CT scan in lesser obvious cases because lesions with large number of cysts are easily confused with appearance of congenital diaphragmatic hernia, as in our case. With this case, we learn two things: 1) any child who presents with spontaneous pneumothorax should be investigated for an associated lung malformation as CPAM; 2) Confirm the diagnosis of diaphragmatic hernia with another exam beyond chest X-ray because some conditions can mimic its radiological findings.
Palavras-Chave: congenital pulmonary airway malformation; pneumothorax; infant