To shunt or not to shunt a pulmonary adenomatoid cystic malformation after 33 weeks of gestation: a case report

Abstract

CONTEXT: Macrocystic adenomatoid malformation of the lung can cause severe mediastinal shift, hydrops and polyhydramnios, thereby increasing the risk of perinatal deaths. After 33 weeks of gestation, repeated puncturing of the cyst is recommended. We present a case in which a cyst-amniotic shunt was placed instead of performing this procedure. CASE REPORT: A cyst-amniotic shunt was placed at 33 weeks of gestation because of a large macrocystic adenomatoid malformation of the lung associated with severe mediastinal shift and polyhydramnios. Although it was confirmed that the catheter was in the correct place, the cyst increased in size again two weeks later, associated with repetition of polyhydramnios. It was postulated that the catheter was blocked, and we chose to place another catheter instead of performing repeated punctures. The cystic volume, polyhydramnios and mediastinal shift regressed progressively. At 38.5 weeks, a 3,310/g male infant was delivered without presenting any respiratory distress. The infant underwent thoracotomy on the 15th day of life. Thus, in the present study, we discuss the possibility of placing a cyst-amniotic shunt instead of performing repeated cystic punctures, even at a gestational age close to full term.

Percutaneous laser ablation under ultrasound guidance for fetal hyperechogenic microcystic lung lesions with hydrops: a single center cohort and a literature review

“…Other prenatal therapeutic options for CCAM with hydrops are still in consideration such as fetal open surgery, thoracoamniotic shunting, and maternal steroids. 22,26,30,45,46 In this situation, therefore, it seems that fetal open surgery is still a good option and steroids seems to be a promising treatment, which need further investigation. However, it is crucial to differentiate between microcystic and macrocystic pulmonary lesions.…”

“…Considering all cases together, median maternal age was 27 (range: [19][20][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35] years. Seven fetuses had microcystic CCAM with hydrops and underwent interstitial laser ablation, while nine fetuses with BPS and hydrops underwent vascular laser ablation.…”

“…[19][20][21]23,24 For this reason, 'minimally invasive' procedures have been proposed such as infusion of intratumoral absolute alcohol to cause vascular sclerosis, intra-fetal laser therapy and maternal steroid administration. 5,15,22,[24][25][26][27][28][29][30][31][32][33][34][35][36][37][38] Herein, we review our experience with laser therapy for congenital microcystic lung lesions associated with hydrops, as well as what was earlier published data.…”

“…For 'macrocystic' chest lesions, cysticamniotic shunting may decompress the mass inside the fetal thorax leading to the regression of fetal hydrops. 5,22 Treatment of 'microcystic' lesions (in association with hydrops) is more controversial. In North America, open fetal surgery has been considered, with a reported 50% of survival.…”

Resultados gestacionais e trombofilias em mulheres com história de óbito fetal de repetição

“…As trombofilias podem ser classificadas em adquiridas e hereditárias e afetam aproximadamente 15% da população geral. As trombofilias hereditárias incluem deficiências das proteínas anticoagulantes (proteína C, proteína S e antitrombina) e mutações nos genes codificadores das proteínas pró-coagulantes (fator V e protrombina) 3 . Tanto a trombofilia adquirida quanto a hereditária podem ser identificadas em 20 a 50% das mulheres que apresentaram um evento tromboembólico durante a gestação ou pós-parto 4 .…”