Congenital Lung Disease

Pediatric Cystic Deep Neck Mass Presenting With Stridor and Dysphagia

“…Accordingly, foregut duplication cysts are subclassified as bronchogenic cysts, esophageal duplication cysts, or enteric duplication cysts, depending on the epithelium. 2,3 Bronchogenic cysts are lined with ciliated respiratory epithelium, which may demonstrate squamous metaplasia and seromucinous glands, with underlying smooth muscle and hyaline cartilage resembling a bronchus. These are the most common, comprising more than 50% of mediastinal cysts.…”

“…Enteric duplication cysts are less commonly connected to the esophagus, but may be closely associated with vertebrae, and are lined with gastric or intestinal mucosa with an overlying muscle layer. 3 Of note, the cysts often demonstrate mixed mucosal types and may not represent the typical mucosa found at the anatomic level of the cyst. 3 In the described case, pathologic findings demonstrated mixed squamous and ciliated respiratory epithelium with stroma containing both smooth and skeletal muscle and benign mucosal glands, most consistent with bronchogenic duplication cyst.…”

“…3 Of note, the cysts often demonstrate mixed mucosal types and may not represent the typical mucosa found at the anatomic level of the cyst. 3 In the described case, pathologic findings demonstrated mixed squamous and ciliated respiratory epithelium with stroma containing both smooth and skeletal muscle and benign mucosal glands, most consistent with bronchogenic duplication cyst. The pathogenesis of foregut duplication cyst development is unclear; however, the predominant theory of development is that the foregut fails to detach from the notochord at the appropriate time point in embryogenesis, as proposed by Veeneklaas in 1952 4 and supported by animal studies from Qi et al 1 Alternatively, the split notochord theory suggests that abnormal adherence of endoderm to the neural tube ectoderm could lead to herniation through the notochord, causing a posterior neuroenteric fistula.…”

“…2,5 Importantly, definitive diagnosis is histopathologic. 2,3,5 Management may consist of observation, aspiration, or surgical resection. While foregut duplication cysts are benign lesions, surgical resection is the treatment of choice.…”

Congenital Lobar Emphysema in Early Adulthood

“…A posteroanterior chest X-ray should be the initial workup in patients with respiratory symptoms. Hyperlucency of the affected lobe can be seen accompanied by atelectasis of adjacent lobes and mediastinal shift to the opposite side [ 8 ]. Nonetheless, chest CT scan remains the gold standard for the diagnosis of CLE, as it allows evaluation of adjacent lobes and contralateral lung.…”