Thoracoamniotic Shunts: Fetal Treatment of Pleural Effusions and Congenital Cystic Adenomatoid Malformations

Abstract

Objective: To determine whether fetuses that underwent thoracoamniotic shunt placement for treatment of pleural effusion (PE) or macrocystic congenital cystic adenomatoid malformation (CCAM) have an improved outcome as compared with an untreated population. Methods: A retrospective review from a single tertiary center was performed using thoracoamniotic shunt placement to treat PE or macrocystic CCAM between 1998 and 2001. Thoracoamniotic shunts were used on 26 occasions in 19 pregnancies. Results: The average gestational age at the diagnosis of PE and CCAM was 22 + 4 and 20 + 0 weeks, respectively. Shunts were offered in pregnancies complicated by hydrops or at significant risk for pulmonary hypoplasia. Shunts were placed at 26 + 2 weeks (average) and 23 + 1 weeks (average) in the PE and CCAM groups, respectively. In CCAM patients, the mean pre- and postshunting mass volumes were 50.5 and 25.7 cm³, representing a 51% reduction in mass volume following shunt placement. In the PE group, the average delivery age was 33 + 5 weeks, with an average shunt placement to delivery time of 7 + 3 weeks. In the CCAM group, the average delivery was 33 + 3 weeks, with an average shunt placement to delivery time of 10 + 2 weeks. The postnatal survival rates were 67% (6/9) and 70% (7/10) in the PE and CCAM groups, respectively. Conclusions: (1) Thoracoamniotic shunts should be considered as a treatment option for selected PE or macrocystic CCAM fetuses with hydrops or a significant risk for pulmonary hypoplasia; (2) the neonatal survival with shunting was similar for PE and CCAM groups and was improved as compared with literature reports, and (3) fetuses with CCAM presented earlier with hydrops than those with PE. Successful shunting resulted in a prolongation of pregnancy into the 3rd trimester in both groups.

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

“…4 In the present case, we decided to place this shunt at 33 weeks because we observed that the cyst had developed recently and acutely and that it was responsible for severe mediastinal shift leading to polyhydramnios and preterm labor. Our first procedure gave a good result for one week.…”

“…It has been suggested that, at a later stage, a better therapeutic option might be prenatal puncturing of the adenomatoid cyst. 4 However, multiple punctures might be needed, which might increase the risks of…”

“…At a later stage, prenatal puncture of the adenomatoid cyst may be preferred. 4 We report here a case of macrocystic CCAM in which a cyst-amniotic shunt was performed later, leading to reduction of the tumor mass and vaginal delivery without respiratory distress. The importance of this case report may lie in opening a discussion on fetal therapeutic options in such cases after the gestational age of 33 weeks.…”

“…2,3 In cases of macrocystic CCAM with large cysts or associated with hydrops, cyst-amniotic shunt may reduce the cyst size and thus decrease the risk of perinatal death. 4 This procedure is usually performed before the gestational age of 33 weeks. At a later stage, prenatal puncture of the adenomatoid cyst may be preferred.…”

Nuevos desafíos en cirugía fetal

“…Los fetos con lesiones con un quiste dominante en los cuales se desarrola hidrops antes de las 32 semanas de gestación, pueden ser consideradas para tratamiento en útero con drenajes toracoamnióticos, con resultados que alcanzan el 70% de sobrevida 13 . En los casos de lesiones microquisticas grandes con desarrollo de hidrops antes de las 32 semanas, y que no mejoran con la administración materna de esteroides, se debería considerar toracotomía fetal para resección de la lesión (figura 1).…”