Anesthesia for a patient with Fanconi anemia for developmental dislocation of the hip: a case report

Pregnancy after allogeneic hematopoietic stem cell transplantation in a Fanconi anemia patient

“…Our patient had only one brother (27 years old) who had been the bone marrow donor. The prevalence of the disease is ~3 in 1,000,000 1,3. FA is the result of a mutation in various genes (~18 genes) involving a DNA repair process; it is a multisystem disease that primarily affects the stem cell of the WBC, RBC, and platelets 6.…”

“…Dogan et al1 reported a 2.5-year-old girl with FA who needed surgery because of a hip dislocation. They induced general anesthesia with 8% sevoflurane in 100% oxygen without N 2 O and then performed caudal epidural block.…”

“…These patients may be more sensitive to oxygen-free radicals or ionizing radiation 1. Dogan et al1 preferred using caudal anesthesia, thus avoiding N 2 O, reduction of the amount of anesthetic and analgesic agent, and the provision of postoperative analgesia. Kotera et al14 reported a case of elective C/S in an aplastic anemia patient.…”

“…It is probably best that the IV lines and noninvasive blood pressure cuff should be placed on the lower limbs. As the placement of the arterial line in the radial artery may be difficult and may cause increased complications, it is best to be avoided 1. Similarly, regional blockade of the upper limbs must be performed with caution.…”

“…FA can be inherited in an autosomal recessive manner, an autosomal dominant manner (RAD51-related FA), or an X-linked manner (FANCB-related FA) 1,2. Pregnancy is an uncommon event in patients with FA, since pregnancy in FA is associated with maternal and fetal risks 3.…”