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Total debranching thoracic endovascular aortic repair is useful for avoiding neurological complications in cases where cardiopulmonary bypass is difficult and for devising an intraoperative cervical branch reconstruction method.This report describes two patients with INPPL1- related skeletal dysplasia diagnosed prenatally. A literature review is conducted to find out if high-lethality is associated with particular pathogenic variants in INPPL1 gene. Prediction of lethality in the prenatal setting has an impact on perinatal management.