Fetal Encephalocele / Cranium Bifidum

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] Herniated contents + site + associated
   Herniated contents (meningeal sac vs containing brain tissue) + site + associated? (Encephalocele = herniation of intracranial contents through a skull defect (cranium bifidum, an NTD). Meningocele (meninges + CSF only) vs encephalocele (contains brain parenchyma). Site: occipital most common. Fetal MRI is better than ultrasound: assesses herniated contents (brain/cerebellum/ventricle/venous sinus), residual brain, microcephaly, hydrocephalus, associated anomalies.)
   • Meningocele (sac + CSF only, no brain tissue), isolated, no microcephaly/hydrocephalus → Meningocele · relatively good prognosis
   • Contains brain tissue, or microcephaly/hydrocephalus, or associated anomalies (Meckel-Gruber, etc.) → Contains brain tissue/associated anomalies · poor prognosis
2. [End] Meningocele · relatively good prognosis
   Meningocele (meninges + CSF only, no brain parenchyma, isolated, no microcephaly/hydrocephalus): relatively better prognosis; fetal MRI to confirm no brain tissue + check associated anomalies + karyotype/microarray; multidisciplinary prenatal counselling, postnatal neurosurgical repair.
3. [End] Contains brain tissue/associated anomalies · poor prognosis
   Encephalocele (contains brain parenchyma) or with microcephaly/hydrocephalus/associated anomalies: the more herniated brain tissue, the worse the prognosis; check Meckel-Gruber (occipital encephalocele + enlarged cystic kidneys + polydactyly, lethal), Walker-Warburg, Chiari III, amniotic band + karyotype/microarray; widely variable prognosis, thorough informed counselling.

Source guidelines & references

• Fetal encephalocele imaging and associated syndromes (Meckel-Gruber; fetal MRI)

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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