General anesthesia for a pregnant patient with PAPA syndrome

A 31-year-old female, with 22 weeks of pregnancy, presented with sudden onset of severe headache. CT scan showed diffuse subarachnoid hemorrhage. A cerebral angiogram showed dissecting aneurysm of right cerebral artery. To obliterate the aneurysm and prevent rupture, the patient underwent coil embolization via an endovascular approach under general anesthesia because the procedure under sedation with local anesthesia was too risky for re-bleeding. The patient has been diagnosed as PAPA syndrome. Although the arthritis was now stable and she was taking no drug, remarkable osteoarthritis was observed. The cervical spine X ray demonstrated no cervical ankylosis. As patient was sedated with propofol, airway examination could not be done except noticing thyromental distance of seven centimeters. Patient's trachea was intubated using Macintosh size #3 laryngoscope blade and a 7.0 non-styletted tracheal tube at the first attempt without any problems (Cormack grade I). Anesthesia was maintained with sevoflurane, fentanyl and remifentanil. After the end of endovascular surgery, the patient was transferred to the intensive care unit under mechanical ventilation. She was weaned from mechanical ventilation 2 days later but consciousness was unclear. Right incomplete paralysis was also observed. MRI revealed vasospasm on the bilateral internal carotid artery. The patient underwent percutaneous tansluminalangioplasty coil and intraarterial injection of fasudil hydrochloride under local anesthesia. The consciousness recovered fully and the paralysis was improved. The patient delivered the baby by Caesarean sections under combined spinal and epidural anesthesia at 36 weeks without any problems with both the mother and baby.