Abstract
Congenital insensitivity to pain with anhidrosis (CIPA), also known as hereditary sensory and autonomic neuropathies (HSAN I-V), is an exceptionally rare autosomal recessive disorder. The pathogenesis of CIPA remains not fully elucidated. Clinical manifestations primarily include the absence of pain perception, painless injuries to the extremities, oral mutilation, anhidrosis accompanied by hyperthermia, and delayed intellectual development. Given the autonomic dysfunction in CIPA patients, previous reports on anesthetic management for this rare disorder have predominantly depended on general anesthesia or neuraxial anesthesia, both of which pose significant challenges. This case report presents a unique approach to anesthetic management in a child with CIPA, who successfully underwent hollow nail internal fixation for femoral neck fracture under orthopedic navigation using ultrasound-guided femoral nerve block combined with lateral femoral cutaneous nerve block. The patient was sedated with dexmedetomidine and did not require any opioids during the procedure. Her vital signs remained stable throughout the surgery, and her recovery was uneventful, with discharge occurring 5 days post-operation. This case contributes to the medical literature by demonstrating a safe and effective anesthetic strategy in a pediatric CIPA patient, highlighting the potential of regional anesthesia techniques as a viable alternative to general anesthesia or neuraxial anesthesia in such high-risk cases.