Congenital cystic lung disease: contemporary antenatal and postnatal management

Abstract

Congenital cystic lung disease comprises a broad spectrum of rare but clinically significant developmental abnormalities, including congenital pulmonary adenomatoid malformations, bronchopulmonary sequestrations, bronchogenic cysts, and congenital lobar emphysema that result from perturbations in lung and airway embryogenesis. As congenital lung lesions are now more commonly recognized antenatally, mothers require accurate prenatal counseling and appropriate perinatal management. In light of long-term complications of infection and malignancy, there is growing consensus that infants with asymptomatic lesions should undergo elective excision of congenital pulmonary adenomatoid malformation (CPAM) or bronchopulmonary sequestration (BPS). This review will focus on advancements and current practice in the diagnosis and management of CPAM and BPS, identifying aspects of the literature that are confusing or controversial. Although our knowledge and pre- and postnatal management of lung lesions will continue to evolve and improve, there is a compelling need for a unified clinical and pathological classification system that creates a common platform for discussion, clinical management, and research.

Malformações pulmonares congênitas

“…(4,10,(12)(13)(14) Pulmonary sequestrations can be classified as extralobar or intralobar depending on their location in relation to the adjacent normal lung and on their visceral pleural covering. (4,10,11,15) There is a third, rarer, extralobar pulmonary sequestration is composed of irregular bronchi and bronchioles, as well as by alveoli that are twice to five times their normal size. (18) Dilated lymphatic vessels in the subpleural region are found in 85% of cases.…”

“…(5,13,18) Case reports of concurrent extralobar and intralobar pulmonary sequestrations suggest that the two forms share a common embryonic origin. (10) The treatment of choice is surgical resection of the lesion through sequestrectomy or pulmonary lobectomy (3,4,11,12,14,15,20) at the time of diagnosis principally because of the possibility of recurrent infections, hemorrhage, malignant transformation, and other complications. However, there is still controversy as to when resection should be performed.…”

“…However, there is still controversy as to when resection should be performed. (8,(13)(14)(15)18,20,23) Many authors recommend that asymptomatic patients with extralobar sequestration remain under observation only, because such lesions rarely cause symptoms and because there have been no reports of malignant degeneration. However, in such lesions, a mixed component, with a morphology similar to that of CCAM, cannot be ruled out.…”

“…(10) The ratio between intralobar pulmonary sequestration and extralobar pulmonary sequestration is 3:1; intralobar pulmonary sequestration is equally distributed between the genders, (18) and extralobar pulmonary sequestration is more prevalent in the male gender (80% of cases). (10,15,18) Extralobar pulmonary sequestrations are generally located in the lower lobes of the left lung (80%), (4,13,15,19) in close proximity to the costophrenic sulcus (Figure 1), and are less common than are intralobar pulmonary sequestrations, which account for approximately 75% of all cases of pulmonary sequestrations. (9)(10)(11)13,15,18,20) Approximately 10% of all extralobar pulmonary sequestrations are located below the diaphragm.…”

“…(10,15,18) Extralobar pulmonary sequestrations are generally located in the lower lobes of the left lung (80%), (4,13,15,19) in close proximity to the costophrenic sulcus (Figure 1), and are less common than are intralobar pulmonary sequestrations, which account for approximately 75% of all cases of pulmonary sequestrations. (9)(10)(11)13,15,18,20) Approximately 10% of all extralobar pulmonary sequestrations are located below the diaphragm. (18) In intralobar pulmonary sequestrations, there is no lung segment predominance (10) ; however, some authors have reported a greater frequency of intralobar pulmonary sequestrations in the posterior basal segment of the left lower lobe.…”