Spontaneous pneumothorax in a teenager with prior congenital pulmonary airway malformation

Abstract

Congenital pulmonary airway malformation (CPAM), previously referred to as congenital cystic adenomatoid malformation (CCAM), is a developmental malformation of the lower respiratory tract and the most commonly reported congenital lung lesion. Affected patients typically present with respiratory distress in the neonatal period from expanding cysts and resulting compression of surrounding lung parenchyma. However, some patients also remain asymptomatic until later in life. In this report, we present a case of CPAM requiring emergent left lower lobectomy at the first day of life that remained asymptomatic until the patient developed a spontaneous pneumothorax 18 years later. Our patient's presentation with an isolated spontaneous pneumothorax at age 18 does not appear to have been previously reported. In addition, there are several aspects of this case that represent atypical features of CPAM. After an extensive literature search, few reports exist describing any long-term complications of CPAM following neonatal lobectomy. Chest imaging in our patient demonstrated residual left basilar bullae and there was a moderate fixed obstructive/restrictive defect on pulmonary function testing. His risk for recurrent pneumothorax or infectious complications is unknown based on minimal published information on long-term outcomes or complications in patients with resected CPAM lesions. We conclude that follow up of all CPAM patients should include an evaluation for evidence of residual lung disease both with spirometric testing and chest imaging. Furthermore, concern for infectious complications or symptomatic obstructive lung disease should likewise be considered.

Congenital Cystic Adenomatoid Malformation (CCAM) Type II: A Rare Case of Sudden Infant Death

“…Another important consideration concerns other congenital defects that could be associated with CCAM, for instance, bilateral renal agenesis, hydrocephalus, gastrointestinal or abdominal wall defects (diaphragmatic hernia, jejunal atresia, and tracheoesophageal fistula), spinal deformities (cervical spine/thoracic spine), epiaortic vessels and myocardial anomalies (truncus arteriosus and tetralogy of Fallot), and sirenomelia [ 40 ]. Patients with CCAM may present with clinical features of pneumothorax in the early neonatal period [ 41 , 42 ]. Huang et al reported that different factors are independently associated with a poor outcome; for example, gestational age at birth, birthweight, Apgar score, and the diagnosis time could be considered fundamental to the survival rate [ 38 ].…”

A rare case of congenital pulmonary airway malformation in a 14-year-old male presenting with spontaneous pneumothorax

“…CPAM can manifest in various ways, with respiratory distress being the most common clinical presentation in neonates, regardless of which type of CPAM the neonate has. In about 50 % of affected newborns, CPAM can remain clinically silent until later in life; then, it could manifest as hemoptysis, dyspnea, chest pain, and cough, or it could be discovered coincidentally during imaging for a different reason or while investigating the etiology behind recurrent respiratory infections; spontaneous pneumothorax could also occur with CPAM [ 9 , 12 ]. In our case, the patient had cough, pneumothorax, dyspnea, and chest pain.…”

“…Surgical resection is considered the treatment of choice for CPAM to confirm the diagnosis and avoid the high potential of pulmonary infection and turning into malignancy [ 12 ].…”

A Missed Late Presentation of a Congenital Pulmonary Airway Malformation as a Large Infected Bulla

“…8 Adult patients with undiagnosed CPAMs can present with recurrent pneumonia, pneumothorax, bullae, haemoptysis or dyspnoea. 7,9,10 The differential diagnosis in adults includes acquired cystic lesions (i.e. pulmonary tuberculosis [TB]) or congenital lesions (i.e.…”