Fetal Surgery for Congenital Pulmonary Airway Malformation Periprocedural Care

Updated: Nov 05, 2019
  • Author: Eric Bradley Jelin, MD; Chief Editor: Hanmin Lee, MD  more...
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Periprocedural Care

Patient Education and Consent

Extensive counseling for families with fetuses with lesions amenable to fetal resection is mandatory. The risks and benefits of fetal intervention to both mother and fetus must be discussed frankly.


Preprocedural Planning

The assembly of a multidisciplinary team that includes an expert sonologist, an anesthesiologist, and a fetal surgeon is critical for intervention success in catheter-based therapies, fetal resections, and ex-utero intrapartum treatment (EXIT) procedures.

With ultrasonography (US), finding an appropriate window that does not traverse the placenta is a key first step in catheter-based therapies. Thoracoamniotic shunts must be placed with exquisite precision to ensure that the double-pigtail shunt has one limb in the dominant cyst and one limb in the amniotic cavity.


Patient Preparation


Maternal anesthesia for catheter-based therapies consists of conscious sedation augmented by subcutaneous local anesthesia. Oral tocolysis with indomethacin or nifedipine should be used.

Maternal anesthesia for fetal resection consists of deep general anesthesia and uterine relaxation. Fetal anesthesia is achieved with an intramuscular shot of “the fetal cocktail,” which includes opiates and paralytics.

Deep general anesthesia with isoflurane for uterine relaxation is used for EXIT procedures. A delicate balance between uterine tone and maternal blood pressure must be maintained. Maternal/fetal oxygenation must also be optimized. [28]


Monitoring & Follow-up

Catheter-based therapies

After catheter-based therapies, serial sonograms are taken to ensure continued shunt patency, appropriate shunt position, and fetal well-being. Vaginal delivery is possible but must be pursued only after a complete obstetric evaluation. It is critical that the obstetrics and pediatrics teams be aware of the shunt so that it can be clamped, attached to suction, or removed immediately after birth.

Steroid administration

Surveillance US should be performed to document the response to therapy. If hydrops persists, successive rounds of steroids can be considered.