Anesthesia in Che'diak-Higashi syndrome--case report.

Che'diak-Higashi syndrome (CHS) is a lethal, progressive, autosomal recessive, systemic disorder associated with oculocutaneous albinism, photopobia, nystagmus, massive leukocyte inclusions (giant lysosomes), histiocytic infiltration of multiple body organs, development of pancytopenia, hepatosplenomegaly, recurrent or persistent bacterial infections, and a possible predisposition to development of malignant lymphoma. This rare disorder of children characterized by impaired resistance to bacterial infection leading to early demise. This syndrome is rarely seen. We are presenting this case report to discuss a patient with Che'diak-Higashi syndrome, who was scheduled for splenectomy in our clinic.