What is the management approach for chorioangioma (chorioangioma) associated with non-immune hydrops fetalis (NIHF)?

Management of Chorioangioma with Non-Immune Hydrops Fetalis

For chorioangioma causing non-immune hydrops fetalis, fetoscopic devascularization of the tumor is the definitive treatment, with 80% survival when performed for high cardiac output states or hydrops. 1

Pathophysiologic Mechanism

Chorioangiomas cause NIHF through two primary mechanisms that guide management decisions:

• High-output cardiac failure from arteriovenous shunting through the highly vascular tumor, which is the most common pathway to hydrops 2
• Fetal anemia from sequestration and hemolysis within the tumor mass 3
• Hypoproteinemia in some cases, though less common 2

Initial Diagnostic Workup

Comprehensive ultrasound assessment must characterize the tumor and assess fetal status:

• Tumor characteristics: Measure maximum diameter, assess echogenicity (hypoechoic vs hyperechoic), and evaluate vascularity with color Doppler 4
• Fetal echocardiography: Evaluate for cardiomegaly, high cardiac output state, and structural cardiac anomalies 2
• Middle cerebral artery Doppler: Peak systolic velocity >1.5 MoM indicates fetal anemia requiring urgent intervention 2, 5
• Assess for associated complications: Polyhydramnios (present in 70% of cases), fetal growth restriction, and degree of hydrops 3, 1

Maternal laboratory evaluation per SMFM guidelines:

• Indirect Coombs test to confirm non-immune etiology 2, 5
• Complete blood count, Kleihauer-Betke stain for fetomaternal hemorrhage 2
• Parvovirus B19 serology and other infectious workup 2

Genetic testing should be offered via amniocentesis or fetal blood sampling, as aneuploidy confers poor prognosis even with intervention 2, 5

Prognostic Ultrasound Features

The sonographic appearance predicts clinical course and urgency of intervention:

• Hypoechoic, diffusely vascular tumors carry the highest risk: associated with severe polyhydramnios, rapid development of hydrops, and stillbirth without intervention 4
• Hyperechoic, avascular tumors have favorable prognosis: typically associated only with growth restriction and oligohydramnios, with expectant management often successful 4
• Partially vascular, hyperechoic tumors have intermediate risk: moderate polyhydramnios and preterm delivery but generally good outcomes 4

Indications for Fetal Intervention

Fetoscopic devascularization is indicated when:

• Non-immune hydrops develops (immediate indication) 1
• High cardiac output state is documented on echocardiography 3, 1
• Severe fetal anemia (MCA-PSV >1.5 MoM) is present 2
• Progressive cardiomegaly develops 3

Optimal timing: Perform intervention at mean gestational age of 24 weeks when complications develop, before irreversible cardiac decompensation occurs 1

Fetoscopic Devascularization Techniques

Multiple techniques may be required in a single procedure to achieve complete devascularization:

• Bipolar coagulation of feeding vessels 1
• Diode laser ablation using percutaneous ultrasound-guided interstitial laser therapy 3, 1
• Radiofrequency ablation for larger feeding vessels 1
• Surgical clip application to major arterial inflow 1
• Combination approach: Most successful cases use multiple modalities to interrupt all arterial inflow 1

Expected outcomes after successful devascularization:

• Resolution of hydrops in all survivors 1
• Normalization of high cardiac output state 1
• Reduction in polyhydramnios and tumor vascularity 4, 1
• 80% survival rate when performed for hydrops or high output states 1

Alternative Management for Severe Anemia

Intrauterine transfusion is indicated when:

• MCA Doppler confirms severe anemia (PSV >1.5 MoM) 2, 5
• Fetal blood sampling can be performed concurrently with genetic testing 2
• This addresses the anemia component but does not treat the underlying vascular steal 3

Expectant Management Criteria

Conservative management is appropriate only when:

• Tumor is hyperechoic and avascular on color Doppler 4
• No signs of hydrops, cardiomegaly, or high output state 4, 3
• Normal MCA Doppler velocities 2
• Mild or absent polyhydramnios 4

Serial surveillance every 1-2 weeks monitoring for:

• Development of hydrops (skin edema, ascites, pleural/pericardial effusions) 4, 3
• Progression of polyhydramnios 3, 6
• Changes in tumor vascularity (spontaneous regression can occur) 4
• Fetal growth and MCA Doppler 3

Delivery Planning

Timing of delivery:

• Avoid iatrogenic prematurity when possible, as gestational age significantly impacts survival 5
• Elective delivery at 33-36 weeks for cases with controlled complications 4, 3
• Emergency delivery indicated for acute fetal distress, abruptio placentae, or maternal mirror syndrome 7, 6

Delivery location and preparation:

• Tertiary center with Level III/IV NICU capability mandatory 5, 7
• Anticipate neonatal anemia, thrombocytopenia, and need for blood product transfusion 7, 6
• Prepare for postpartum hemorrhage risk from uterine atony due to polyhydramnios and large placental mass 6
• Multidisciplinary team including maternal-fetal medicine, neonatology, and anesthesiology 6

Maternal Monitoring

Monitor for mirror syndrome (maternal preeclampsia developing with fetal hydrops):

• Serial blood pressure checks 5
• Assess for maternal edema, proteinuria, and hypertension 5
• Mirror syndrome necessitates delivery in most cases 5

Critical Pitfalls to Avoid

• Do not delay intervention once hydrops or high cardiac output develops—outcomes deteriorate rapidly with cardiac decompensation 4, 1
• Do not assume all chorioangiomas require intervention—avascular, hyperechoic tumors often have favorable outcomes with expectant management 4
• Do not underestimate neonatal complications—even with successful fetal intervention, anticipate prematurity-related morbidity including respiratory distress, IVH, NEC, and BPD 7
• Do not perform single-modality devascularization—combination techniques achieve better complete devascularization 1