Pulmonary sequestration is a congenital anomaly resulting in a mass of nonfunctioning lung tissue. Sequestrations can occur as “extralobar,” with their own pleural investments, or, more commonly, as “intralobar,” in which the nonfunctioning mass is surrounded by normal lung tissue. Both forms derive their blood supply from the systemic circulation and are most commonly associated with the lower lobes. Large pulmonary sequestrations can present with potentially fatal respiratory distress in the newborn. Presented is an extralobar sequestration in a newborn occurring in the right upper thorax, a condition not previously described in the literature to our knowledge.
A 2,420-g male product of a 30-week gestation was delivered to a 42-year-old, gravida 4, para 2, diabetic mother. The pregnancy was remarkable for a prenatal sonogram that revealed polyhydra- minose and a cystic mass in the right thorax. No medications were taken during pregnancy. vardenafil 20 mg
Figure 1. Chest roentgenogram demonstrating mass in right upper thorax.
Newborn physical examination results were normal except for low birth weight. The infant did well, and chest roentgenogram (Fig 1 and 2) as well as a repeated sonogram confirmed a cystic mass in the right upper thorax. Barium swallow demonstrated trachea] and esophageal deviation to the left with no invasion (Fig 3).
Exploration was done through a right posterolateral thoracotomy, which revealed normal-appearing right upper, middle, and lower lobes that were compressed by a large, pink, nonaerating mass that filled the upper half of the thorax. The mass had its own pleural investment separate from the adjacent normal lobes and tented the azygos vein and mediastinal pleura laterally and superiorly. An open biopsy was performed with a frozen section diagnosis of normal, nonaerated lung.
FIGURE 2. Lateral roentgenogram demonstrating mass.
Dissection was carried down the right pulmonary artery with identification of all branches to the upper and middle lobes. There were no branches of the pulmonary artery feeding the mass. There was an identifiable pedicle leading to the mass that contained three large arteries of systemic origin. This was divided and the mass removed. The right upper, middle, and lower lobes were allowed to expand, filling the thorax in their normal anatomic position. The remainder of the operation and the hospital course were unremarkable.
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FIGURE 3. Barium swallow demonstrating extrinsic compression of the esophagus with deviation to the left.
Pathologic examination specimen revealed a 21-g mass measuring 5.5 x 3 x 2.5 cm. There was a small area of nonpatent cartilage, 0.2 cm at maximum diameter. The remainder of the specimen consisted of multilobulated cystic tissue composed of lung parenchyma. The histology of the mass with its separate pleura, systemic blood supply and lack of a patent tracheobronchial connection is consistent with the diagnosis extralobar pulmonary sequestration.