Successful surgical management of intralobar pulmonary sequestration in Ghana

Abstract

Pulmonary sequestration occurs when a portion of lung tissue receives its blood supply from an anomalous systemic artery. Three main presentations, intralobar, extralobar and communicating bronchopulmonary foregut malformations, have been described. It is the second most common congenital lung anomaly. The intralobar variant is the most common type seen in 75% of cases, especially in late childhood. Imaging of choice for diagnosis are computed tomography scan and magnetic resonance imaging. Management involves surgical resection with ligation of the aberrant blood supply via thoracotomy or thoracoscopy. Endovascular therapy with coil embolization of the aberrant anomalous systemic artery as a standard therapy or as a hybrid therapy is an option. We present our successful surgical management of an infant diagnosed prenatally with congenital lung abnormality and confirmed postnatally as intralobar pulmonary sequestration.

Presenting a Case of an Extralobar and Extrapleural Pulmonary Sequestration in a Four-Month-Old Infant

“…It receives its blood supply from the systemic circulation with or without a separate venous drainage and comprises 0.15 to 6.4% of all congenital lung malformations [2,3]. It was first described in 1861 by Rokitansky via a fraction theory in which there is a separation of normally developing lung tissue from the rest of the lung during development which subsequently becomes non-functional [4][5][6]. Pryce then described it in 1946 via a traction theory whereby traction on lung tissue by maturing primitive systemic vasculature results in sequestration when an aberrant early interruption of pulmonary arterial development occurs [4,[6][7].…”

“…It was first described in 1861 by Rokitansky via a fraction theory in which there is a separation of normally developing lung tissue from the rest of the lung during development which subsequently becomes non-functional [4][5][6]. Pryce then described it in 1946 via a traction theory whereby traction on lung tissue by maturing primitive systemic vasculature results in sequestration when an aberrant early interruption of pulmonary arterial development occurs [4,[6][7]. With this theory, sequestration can be extralobar if traction occurs early enough in development to cause the growth of visceral pleura around the sequestered lung tissue or intralobar if traction occurs after the lung pleura has already formed [8].…”

“…With this theory, sequestration can be extralobar if traction occurs early enough in development to cause the growth of visceral pleura around the sequestered lung tissue or intralobar if traction occurs after the lung pleura has already formed [ 8 ]. Pulmonary sequestrations occur in the bronchopulmonary malformation spectrum, which includes other malformations like congenital lobar hyperinflation, bronchogenic cyst, congenital pulmonary airway malformation (CPAM), bronchial atresia, and arteriovenous malformation [ 6 , 9 ] based on various aberrations encountered during embryogenic development.…”