Line drawing of proposed mechanisms underlying ependymal denudation and abnormal CSF flow in the Sylvius aqueduct (SA) of spina bifida aperta (SBA) patients. The ependyma of the SA of control foetuses and the non‐altered regions of the SA of SBA patients display a normal expression and a normal transport to the lateral plasma membrane of the junction proteins N‐cadherin and connexin 43 (left panel). This results in normal gap and adherent junctions. In the altered SA regions of SBA patients, N‐cadherin and connexin 43 are expressed but their transport to the lateral plasma membrane appears impaired. Subsequently, N‐cadherin and connexin 43 may abnormally accumulate in the cytoplasm, whereas functional adherent and gap junctions fail (right panel). All together, this may induce: (i) ependymal denudation, aqueduct stenosis and CSF obstruction; (ii) nonsynchronized cilia beating, abnormal CSF flow, and may finally contribute to (iii) hydrocephalus. Abbreviations: AJ = adherent junction; GJ = gap junctions; ER = rough endoplasmic reticulum; Nu = cell nucleus; TGN = trans‐Golgi network.