Figure 10.5. Approximately 5% of neonates with a congenital diaphragmatic hernia will have an extralobar BPS, which is usually an incidental intraoperative finding. It is widely believed that extralobar pulmonary sequestrations are a result of prenatal pulmonary malformation while intralobar pulmonary sequestrations can develop due to recurrent pulmonary infections in adolescents and young adults. Katrin Karl, Rabih Chaoui, in Twining's Textbook of Fetal Abnormalities (Third Edition), 2015. Clinical presentation Patients usually present before the third decade with recurrent infection. The majority of these fetuses have a very good outcome. These fetuses should be followed closely, delivered near term, and the pulmonary sequestration removed surgically after birth — often the removal is an elective proceedure in early childhood. Associated defects—is there another problem or a cluster of problems (syndrome). According to Price, such abnormal pulmonary tissue had previously been said to be ectopic or dislocated [11]. Extrapulmonary sequestration is completely enclosed in its own pleural sac. Extralobar BPS has a predominance in males (3:1), is more common on the left side, and can be associated with conditions such as congenital diaphragmatic hernia, vertebral deformities, and congenital heart disease. Venous drainage is usually via the systemic venous system. Ultrafast fetal magnetic resonance imaging (MRI) may help differentiate CCAM from BPS.42 Furthermore, there are prenatally diagnosed lung masses that display clinicopathologic features of both CCAM and sequestration-hybrid lesions, which suggests a shared embryologic basis for some of these lung masses.43–45 The ability to differentiate intralobar and extralobar sequestration before birth is limited unless an extralobar sequestration is highlighted by a pleural effusion or is located in the abdomen (usually close to the left adrenal gland). Usually the sequestration is removed after birth via surgery. BPS was first described in 1861 by Rokitansky [10]. These are the only fetuses who are candidates for treatment before birth. Gregory Hammer, ... Peter J. Davis, in Smith's Anesthesia for Infants and Children (Eighth Edition), 2011. No communication with the tracheobronchial tree occurs. This condition is usually diagnosed in children and is generally thought to be congenital in nature. Computed tomography (CT) and MRI are the most valuable tools for imaging [1,9,26]. A multitude of somatic anomalies have been associated with sequestration, most commonly diaphragmatic hernia. Children with BPS may be asymptomatic but they may also have important respiratory distress and severe congestive heart failure secondary to the volume loading from a large systemic artery for the sequestered segment [9]. Patrizia Dall’Igna, ... Piergiorgio Gamba, in The Complete Reference for Scimitar Syndrome, 2017. We use cookies to help provide and enhance our service and tailor content and ads. However, CT allows sharper delineation of thin-walled cysts and emphysematous changes than MRI. On prenatal ultrasound, BPS usually appears as a solid, homogenous, hyperechogenic mass with at least one feeding vessel from the thoracic or abdominal aorta (Fig. Figure 10.6. It is mandatory to identify and ligate the feeding systemic arterial vessel(s), which usually is found within the inferior pulmonary ligament. Venous drainage is usually via the systemic veins. Air-fluid levels due to bronchial communication can be seen. Fetuses with pulmonary sequestrations, identified by an abnormal artery on ultrasound, usually do well with surgical removal after birth. Patients present with signs and symptoms of, It is believed that sequestrations become infected when. The mass may shrink in size before birth. Intralobar sequestration (ILS) in which the lesion is located within a normal lobe and lacks its own visceral pleura.

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