VANGL2 and craniorachischisis: In yet another type of digenic cause, high frequencies of craniorachischisis are found in mutants that are double heterozygotes for mutations at two PCP genes of different gene families (e.g., Vangl2;Celsr1, Vangl2;Dvl2, Vangl2;Scrib, or Celsr1;Scrib) [10,185].