EXTRA-LOBAR PULMONARY SEQUESTRATION WITH PRENATAL-DIAGNOSIS - A REPORT OF 5 CASES AND REVIEW OF THE LITERATURE

The purpose of this work was to study the pre- and postnatal features of extra-lobar pulmonary sequestration (ELPS) and consider their diagn osis and treatment. Methods: Five ELPS diagnosed prenatally (1986-1992 ) were reviewed retrospectively. Results: In 2 cases, prenatal diagnos is was based on the presence of a left suprarenal mass for which tumor markers proved negative postnatally. These 2 infants underwent surger y at 3 weeks of age for supposed neuroblastoma or teratoma. in the oth er 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 t hese cases; mediastinal displacement was noted in one case and hydroth orax (which recurred after puncture) in the other. The latter infant w as born at 34 weeks of amenorrhea, and hydrothorax disappeared postnat ally after excision of the ELPS. The other two infants were asymptomat ic at birth and underwent surgery respectively on the 8th day and duri ng 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 ELP S, surgical excision (possibly preceded by percutaneous puncture) is r equired if the diagnosis is uncertain. Supradiaphragmatic ELPS can be complicated prenatally by hydrothorax or even hydrops, requiring drain age in utero. If the infant is asymptomatic postnatally, systematic su rgical excision should be considered. Extra-lobar pulmonary sequestrat ions (ELPS) are masses of nonfunctional lung tissue vascularized by an abnormal systemic artery and covered with a pleural layer isolating t hem from the rest of the parenchyma. The diagnosis of these malformati ons is based increasingly on obstetrical ultrasonography. The purpose of this study was to specify the prenatal features of these malformati ons and define the diagnostic approach and therapeutic strategy.