Congenital Pulmonary Airway Malformation in Children: Advantages of an Additional Trocar in the Lower Thorax for Pulmonary Lobectomy

Aim: To present the use of an additional trocar (AT) in the lower thorax during thoracoscopic pulmonary lobectomy (TPL) in children with congenital pulmonary airway malformation.Methods: For a lower lobe TPL (LL), an AT is inserted in the 10th intercostal space (IS) in the posterior axillary line after trocars for a 5-mm 30° scope, and the surgeon's left and right hands are inserted conventionally in the 6th, 4th, and 8th IS in the anterior axillary line, respectively. For an upper lobe TPL (UL), the AT is inserted in the 9th IS, and trocars are inserted in the 5th, 3rd, and 7th IS, respectively. By switching between trocars (6th↔8th for the scope, 4th↔6th for the left hand, and 8th↔10th for the right hand during LL and 5th↔7th, 3rd↔5th, and 7th↔9th during UL, respectively), vital anatomic landmarks (pulmonary veins, bronchi, and feeding arteries) can be viewed posteriorly. The value of AT was assessed from blood loss, operative time, duration of chest tube insertion, requirement for post-operative analgesia, and incidence of perioperative complications.Results: On comparing AT+ (n = 28) and AT– (n = 27), mean intraoperative blood loss (5.6 vs. 13.0 ml), operative time (3.9 vs. 5.1 h), and duration of chest tube insertion (2.2 vs. 3.4 days) were significantly decreased with AT (p < 0.05, respectively). Differences in post-operative analgesia were not significant. There were three complications requiring conversion to open/mini-thoracotomy: AT– (n = 2; bleeding), AT+: (n = 1; erroneous stapling).Conclusions: An AT and switching facilitated posterior dissection during TPL in children with congenital pulmonary airway malformation enhancing safety and efficiency.

Congenital Central Overinflation with Mainstem Bronchial Stenosis: Pre- and Postnatal Imaging and Outcome—Case Report and Brief Review of Literature

Congenital overinflation of lung is underdiagnosed prenatally as the imaging features of the same are not well described. We describe a very rare case of central variety of congenital overinflation, secondary to right mainstem bronchial stenosis, not previously described in our knowledge, which presented as an enlarged homogenous echogenic/hyperintense lung with cardio-mediastinal shift and was misdiagnosed as congenital pulmonary airway malformation (CPAM). We reviewed imaging features helpful in prenatal diagnosis of this condition on ultrasound and magnetic resonance imaging and discussed an approach for differential diagnosis. The congenital central overinflation may be considered in prenatal detected echogenic lung lesions with the absence of cysts or systemic arterial supply and presence of hypervascularity or dilated bronchi within the lesion.

Unusual Congenital Lesion Masquerading as a Lung Mass in an Adult

Congenital pulmonary airway malformation (CPAM) is a broad spectrum of congenital cystic lung lesions caused by the arrested bronchoalveolar development. Approximately, 80% of CPAMs are diagnosed prenatally or during the neonatal period when patients present with respiratory failure and cyanosis. CPAM is often associated with other organ anomalies and aplasia, and they have poor prognoses. Many CPAMs are detected in infants and school-age children, and infections like pneumonia trigger these diagnoses. It rarely manifests in adults. These often get missed because of the superadded diseases, and hence, it is essential to have a correct approach to their diagnosis to avoid misdiagnosis.

Developmental Pathways Underlying Lung Development and Congenital Lung Disorders

Lung organogenesis is a highly coordinated process governed by a network of conserved signaling pathways that ultimately control patterning, growth, and differentiation. This rigorously regulated developmental process culminates with the formation of a fully functional organ. Conversely, failure to correctly regulate this intricate series of events results in severe abnormalities that may compromise postnatal survival or affect/disrupt lung function through early life and adulthood. Conditions like congenital pulmonary airway malformation, bronchopulmonary sequestration, bronchogenic cysts, and congenital diaphragmatic hernia display unique forms of lung abnormalities. The etiology of these disorders is not yet completely understood; however, specific developmental pathways have already been reported as deregulated. In this sense, this review focuses on the molecular mechanisms that contribute to normal/abnormal lung growth and development and their impact on postnatal survival.