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Obstetric anesthesia for harlequin ichthyosis: a unique challenge

Thea Rosenbaum1, Eric R Rosenbaum, Kristen L Lienhart

  • 1From the *Department of Anesthesiology, University of Arkansas for Medical Sciences, Little Rock, Arkansas; and †Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, Arkansas.

A & a Case Reports
|January 23, 2015
PubMed

Harlequin ichthyosis (HI) is a rare disorder of defective lipid transport resulting in severe epidermal hyperkeratosis producing large plate-like scales. Although mortality is high, improved treatments have allowed some with HI to survive into their third and fourth decades. However, until this case, there have been no known reports of pregnancy followed by birth of a healthy neonate to a mother with HI. We report one of the only approximately 25 known current HI survivors worldwide unique in having carried a pregnancy to full term and outline challenges for the anesthesiologist during labor and delivery.

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