X-linked ichthyosis presenting with cryptorchidism for orchidopexy: A rare anesthetic encounter and case report

Clin Case Rep. 2024 Aug 5;12(8):e9245. doi: 10.1002/ccr3.9245. eCollection 2024 Aug.

Abstract

Key clinical message: Cutaneous scaling and associated clinical syndrome displayed in X-linked ichthyosis mandates multidisciplinary care. Patient with ichthyosis confronts a numerous challenge to an anesthesiologist and demands a rigorous management. As these patients are very vulnerable perioperatively, meticulous care and support are utmost.

Abstract: Ichthyosis is a group of genetic conditions distinguished by the appearance of hyperkeratotic scales on the skin's surface. X-linked ichthyosis results from a mutation in the steroid sulfatase (STS) gene, which encodes the steroid sulfatase enzyme. Here we report a case of a 6-year-old child with X-linked ichthyosis. He presented to our operation theater for correction of left-sided undescended testis and underwent surgery uneventfully. To handle X-linked ichthyosis perioperatively, meticulous planning and efficient anesthetic administration are critical.

Keywords: anesthesia; cryptorchidism; ichthyosis; steroid sulfatase.

Publication types

  • Case Reports