%0 Journal Article
%T Placental steroid sulphatase deficiency: an approach to antenatal care and delivery
%A Frieda-Elsje Dreyer
%A Ganiy Opeyemi Abdulrahman
%A Gareth Waring
%A Kim Hinshaw
%J Archive of "Annals of Saudi Medicine".
%D 2018
%R 10.5144/0256-4947.2018.445
%X Placental steroid sulphatase deficiency (SSD) is an X-linked inborn error of metabolism. Congenital X-linked ichthyosis (XLI) is a genetic disorder of keratinisation caused by steroid sulphatase (STS) deficiency, which results in a scaling skin condition in male infants shortly after birth. It may be associated with failed induction of labor and prolonged labor leading to cesarean delivery due to ¡®cervical dystocia¡¯. We present two cases of congenital ichthyosis. Thorough counselling of women with a previously affected pregnancy during the antenatal period should include discussion about mode of delivery and a critical review of the complexities of prenatal diagnosis in this condition. We propose a clinical management pathway to offer women with a previous pregnancy affected by this rare condition
%U https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6302985/