Congenital Pulmonary Airway Malformation (CPAM) Fetal · CVR

Step-by-step decision

Choose step by step as prompted; reaching an endpoint gives the management recommendation. You can go back a step or restart anytime.

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Full pathway

1. [Decision] CVR and hydrops/dominant cyst assessment
   CVR and presence of fetal hydrops/dominant large cyst? (CPAM = congenital pulmonary airway malformation. CVR = lesion volume (L x W x AP x 0.523)/head circumference (corrects for gestational age). Fastest growth 20–26 weeks, mostly regresses after ~28 weeks. Macrocystic (≥5 mm) vs microcystic/solid (more steroid-responsive). Fetal MRI for characterization + differentiating pulmonary sequestration (systemic supply).)
   • CVR <1.6, no dominant large cyst, no hydrops → CVR <1.6 · weekly follow-up
   • CVR ≥1.6, no hydrops → CVR ≥1.6 no hydrops · maternal steroids
   • CVR ≥1.6 with fetal hydrops, or a dominant cyst enlarging acutely → With hydrops → shunt/fetal surgery
2. [End] CVR <1.6 · weekly follow-up
   CVR <1.6, no dominant large cyst, no hydrops: good prognosis (hydrops risk <2–3%); weekly ultrasound follow-up to the ~26-week plateau; postnatal CT, resect if symptomatic (elective resection still usually advised even if asymptomatic).
3. [End] CVR ≥1.6 no hydrops · maternal steroids
   CVR ≥1.6, no hydrops (hydrops risk up to 80%): twice-weekly close ultrasound monitoring + maternal betamethasone (2 doses) (survival near 100% after steroids in high-risk without hydrops); fetal MRI for characterization, follow shrinkage.
4. [End] With hydrops → shunt/fetal surgery
   CVR ≥1.6 with hydrops (or a dominant cyst enlarging acutely): fetal hydrops = critical; maternal steroids (may repeat the course, ~40% survival with hydrops); large cyst → thoraco-amniotic shunt; refractory → open fetal surgery; very large with airway compression → EXIT; multidisciplinary.

Source guidelines & references

• CPAM volume ratio CVR and prenatal management (CVR ≥1.6 steroids/thoraco-amniotic shunt)

This pathway is our own synthesis of the decision logic in the guidelines above (not the guideline verbatim); thresholds and workflows change as guidelines update — in practice follow the latest guideline, your institution's protocol and the individual patient.

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