RESUMEN
Acquired hemophilia A can upshot in a life-threatening hemorrhage and airway obstruction. Airway bleeding is a weighty emergency in hemophilia care, necessitating the immediate start of effective hemostatic therapy (porcine factor VIII, the factor eight inhibitor bypassing activity and recombinant factor VIIa) and the decision to undertake proper airway control, such as tracheal intubation and tracheostomy. However, due to the dearth deficiency of effective hemostatic measures we relied upon the use of fresh frozen plasma and cryoprecipitate to gain control of the bleeding despite the precarious threat of infectious disease transmission associated with their use.
RESUMEN
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.