Extra-lobar pulmonary sequestration with prenatal diagnosis. A report of 5 cases and review of the literature

Abstract

THE PURPOSE of this work was to study the pre- and postnatal features of extra-lobar pulmonary sequestration (ELPS) and consider their diagnosis and treatment.

Methods: Five ELPS diagnosed prenatally (1986-1992) were reviewed retrospectively.

Results: In 2 cases, prenatal diagnosis was based on the presence of a left suprarenal mass for which tumor markers proved negative postnatally. These 2 infants underwent surgery at 3 weeks of age for supposed neuroblastoma or teratoma. In the other 3 cases, diagnosis was based on the presence of a solid mass at the left base of the thorax. The systemic vessel was visualized in 2 of these cases; mediastinal displacement was noted in one case and hydrothorax (which recurred after puncture) in the other. The latter infant was born at 34 weeks of amenorrhea, and hydrothorax disappeared postnatally after excision of the ELPS. The other two infants were asymptomatic at birth and underwent surgery respectively on the 8th day and during the 6th month of life.

Conclusion: ELPS can take the form of a mass in the abdomen or at the base of the thorax. For subdiaphragmatic ELPS, surgical excision (possibly preceded by percutaneous puncture) is required if the diagnosis is uncertain. Supradiaphragmatic ELPS can be complicated prenatally by hydrothorax or even hydrops, requiring drainage in utero. If the infant is asymptomatic postnatally, systematic surgical excision should be considered. Extra-lobar pulmonary sequestrations (ELPS) are masses of nonfunctional lung tissue vascularized by an abnormal systemic artery and covered with a pleural layer isolating them from the rest of the parenchyma. The diagnosis of these malformations is based increasingly on obstetrical ultrasonography. The purpose of this study was to specify the prenatal features of these malformations and define the diagnostic approach and therapeutic strategy.