Bronchopulmonary Sequestration

doi:10.1378/chest.57.5.454

Chest

Volume 57, Issue 5, May 1970, Pages 454-459

https://doi.org/10.1378/chest.57.5.454 Get rights and content

Intralobar pulmonary sequestration is a cystic intrapulmonary mass supplied by an aberrant systemic artery with venous drainage usually to the pulmonary veins. Most cases reported have been in adults. This report presents the findings in 11 patients, nine of whom are less than 11 years of age. Radiographic evidence of an inflammatory process or mass in the basilar segments of the lower lobes, presence of a continuous murmur over the thorax and history of repeated pneumonia or lower respiratory tract infections should alert the pediatrician to suspect this diagnosis. Definite diagnosis can be established by aortic angiocardiography. Surgery is indicated because the sequestered lobe is a site for repeated infections.

Section snippets

INTRODUCTION

Pryce¹ coined the term “intralobar pulmonary sequestration” in 1946. Most reports since that time have been in adults beyond the second decade of life. There are few reports in the pediatric literature2, 3, 4, 5, 6, 7, 8, 9 particularly of children under ten years of age regarding this embryologic anomaly. For this reason we are presenting the clinical profile, roentgenographic findings, surgical management and pathologic data in 11 pediatric patients. Nine patients were 11 years or less in age.

MATERIAL AND METHOD

Eleven patients have been seen at University Hospital in Iowa City, Iowa, between 1954 to 1968 in whom the diagnosis of intralobar pulmonary sequestration was established by surgery or at autopsy. Their ages ranged from seven days to 17 years. There were eight boys and three girls (Table 1).

HISTORY

Seven patients were referred for evaluation of recurrent respiratory infections (cases 2, 3, 4, 5, 6, 9, and 11, Table 1). Six of these patients had had more than one roentgenographic documented episode of pneumonia and four had pneumonia at the time of admission. One of these seven patients (case 4) also had a continuous murmur which was heard over the involved lung segment and less prominently over the anterior thorax.

Another patient (case 1) who was asymptomatic was referred for evaluation

CARDIAC MURMURS

Two patients (cases 1 and 4) had continuous murmurs heard maximally over the posterior thorax, Table 1. In the remaining nine cases no murmur was audible and if present was felt to be innocent.

ELECTROCARDIOGRAMS

Only four patients had electrocardiograms. Two tracings were normal, one was suggestive of combined ventricular hypertrophy and one showed left axis deviation.

RADIOGRAPHIC FINDINGS

The bronchopulmonary sequestration was confined to the posterior basilar portions of the lower lobes. In eight cases the left lower lobe was involved and in three the right lower lobe (Table 1).

A wide spectrum of plain chest film findings was noted. In case 1, the PA and lateral chest radiographs were normal. In two patients (cases 3 and 8) dense airless masses were noted (Fig 1a, b). In two patients (cases 2 and 7) the lesion was uniformly hyperlucent (Fig 2a, b). In one patient (case 5) there

SPECIAL RADIOGRAPHIC STUDIES

Bronchography was performed in nine patients and revealed failure of the contrast material to enter the sequestrated lung segment (Fig 4).

ANGIOCARDIOGRAPHY

Four patients had cardiac catheterization and aortic angiocardiograms (Table 1). Right and left heart catheterization demonstrated no abnormalities.

Aortograms demonstrated the aberrant vessel (Fig 5) from the aorta entering the sequestered lung segment. Subsequent to this there was opacification of the pulmonary veins and left atrium. Case 4 showed numerous vessels from dilated intercostal arteries entering the sequestered lung segment.

SURGERY

Ten of the 11 patients had an operation for removal of the abnormal pulmonary tissue. Four patients had a left lower lobectomy and two, right lower lobectomy. The basilar segments alone were removed from the left in two patients and from the right in one. One patient had a left pneumonectomy (case 7). The object of each operation was to remove the diseased part of the lung and be as conservative as possible. In all patients systemic arteries entered the abnormal segment of the lung usually from

PATHOLOGY

Eleven specimens were available for examination and consisted of either a lower lobe or basilar segment except in case 7 in which a pneumonectomy was done.

Adequate sections of the aberrant vessels were obtained only in the last four cases. All were elastic arteries, and all showed patchy intimal fibrosis, but no other change. No aberrant veins were seen except in case 4.

One specimen (case 1) showed no cystic change grossly or microscopically, while the remaining ten cases were multicystic. The

COMMENT

The characteristic feature of intralobar sequestration is a cystic intrapulmonary mass, supplied by an aberrant artery of systemic origin. Usually, the cystic area is confined to the posterior basal portion of the lower lobe, and the segmental bronchus to that area is absent. Most often the left lower lobe is involved. Occasionally, the lesion involves tire upper lobe.¹⁰ The cyst may be single or multiple and is usually lined by respiratory epithelium, often with cilia. Smooth muscle is almost

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