Will this patient need any diagnostic procedures prior to coming to the OR?

Would you obtain any imaging? Why? What studies? What if they require an anesthetic? Does this patient need any specific laboratory evaluation?

Should the patient be transferred to the ICU for stabilization, intubation, and airway securement and sedation?

Would you anticipate any comorbidity?

Is this an unusual problem? Common? Relatively common for neonatal surgical emergencies? For neonatal thoracic surgical emergencies?

Would your considerations be any different if this was a congenital lobar emphysema or a pulmonary sequestration with regard to planning the anesthetic?

Eighty percent of affected neonates present with some degree of respiratory distress due to pulmonary compression or hypoplasia. As a developmental outgrowth of the tracheobronchial tree, these anomalies may become overdistended due to air trapping. Secondary causes of respiratory insufficiency are mediastinal shift and spontaneous pneumothoraces. Typical signs and symptoms include tachypnea, grunting, retractions, use of accessory muscles, cyanosis, and failure to thrive. Impaired clearance of sections due to impaired air flow can result in infectious processes such as pneumonia or lung abscesses. It sounds like this situation is turning into an acute emergency after 2 days, contraindicating diagnostic procedures beyond those already done. The patient needs to come directly to the operating room and have the worsening respiratory status treated before any further compromise. Preoperative evaluation may require an echocardiogram to rule out congenital heart disease and may also be useful for identifying patients with (1) decreased cardiac output due to increased intrathoracic pressure from a large lung lesion and (2) pulmonary sequestrations with a large blood supply, in which high-output cardiac failure develops.

If there is time for a portable chest x-ray, it might be worthwhile to look for evidence of worsening intrathoracic volume of the CCAM and mediastinal shifting. Blood work, such as arterial blood gases, will not help at this point. If there is no time for a CXR, then air trapping and mediastinal shifting with a possible pneumothorax should be anticipated and preparations made for chest tube drainage and respiratory support. If time permits, more thorough preoperative evaluation is necessary to determine the patient’s current pulmonary status, the impact of the anticipated surgery, and the ability to tolerate OLV if needed.

The patient should come directly to the operating room for definitive surgical treatment.

Comorbidities may be congenital or due to acutely compromised physiology. CCAM is a lung lesion that develops from adenomatous overgrowth of terminal bronchioles. These lesions can be cystic, solid, or mixed intrapulmonary masses that lack bronchial cartilage and glands. Communication with the tracheobronchial tree is usually maintained and vascular supply and venous drainage are derived from the pulmonary circulation. Approximately 20 % of patients with CCAM have an associated congenital anomaly, with renal agenesis and cardiac defects being the most common. When the lesions are large, pulmonary growth may be compromised leading to pulmonary hypoplasia in normal contiguous lung areas.

CCAMs comprise approximately 25 % of all congenital lung malformations, with an estimated incidence of 1:25,000–1:35,000.

Each congenital lung lesion has several important features. Congenital lobar emphysema (CLE) involves abnormal emphysematous lung that communicates with a bronchus; overexpansion is a major concern. CCAM tissue does not participate in gas exchange, but may communicate with the tracheobronchial tree and therefore the same concern remains with regard to air trapping. Pulmonary sequestration lesions involve nonfunctional lung tissue without a bronchial connection, and the blood supply is from anomalous systemic arteries.