Abordagem cirúrgica intraútero de hidropisia fetal decorrente de malformação adenomatoide cística pulmonar

Abstract

Introduction: Cystic Adenomatoid Lung Malformation (MACP) is characterized by the presence of epithelial adenomatoid hyperplasia of the lung. It is a rare congenital condition, responsible for acute respiratory failure in newborns and is usually neglected as a diagnostic hypothesis. In this sense, intrauterine surgery gains space by being an innovative and resolutive therapeutic alternative. Case description: E.V.D, female, 38 years old, without comorbidities, G2PC1A0, pregnant woman of 22 weeks is referred for a first consultation on 04/08/2019, at the fetal medicine service of Hospital das Clínicas - UFMG, due to pulmonary alterations to fetal ultrasonography (US) (04/03/2019). At this US, it was observed the presence of a complex lobulated cystic area, about 2cm in diameter in the left lung, suggestive of Stocker MACP type I, and small volume ascites, characterizing fetal hydrops. In a new US (04/11/2019) the presence of mild pericardial effusion was verified. Discussion: MACP is characterized by proliferation of terminal pulmonary bronchioles, which coalesce forming cysts in the pulmonary parenchyma. The diagnosis can be performed through prenatal US, in which cystic lung structures are visualized, being generally unilobular and unilateral. Due to their mass effect, they can generate compression of the lung parenchyma, causing hypoplasia, and deviation of the mediastinum, followed by heart failure. In addition, there is damage to venous return, resulting in accumulation of fluid and conditions such as hydrops with pleural effusion and polydramnio. Thus, the first conduct adopted is the follow-up, through serial US and, in the presence of warning signs or extensive lesions, intrauterine surgery is indicated through thoracoamniotic bypass, thoracic puncture and continuous catheter bypass. Conclusion: It is concluded that the performance of US in prenatal is essential for early diagnosis and monitoring of malformations. Moreover, although rare, the MACP should be considered as a diagnostic hypothesis for being responsible for respiratory and cardiac failure at birth. There is still no consensus regarding the management of this condition and the current techniques of intrauterine surgical approach appear as a new therapeutic proposal, aiming to reduce prenatal and postnatal complications, having, in most cases, a curative character.