[Anesthesia in a patient with alkaptonuric ochronosis for total hip arthroplasty].

Alkaptonuric ochronosis, caused by a deficiency of homogentisate 1,2-dioxygenase, is a rare, autosomal recessive, metabolic disorder. Accumulation of homogentisate acid (HGA) at the connective tissue destructs the spine and large joints, and cardiac valvular disease is prominent. In this report, we describe a case of alkaptonuric ochronosis for anesthetic management. A 75-year-old female patient with the disease was scheduled for a total-hip arthroplasty. We avoided applying general anesthesia for her valvular regurgitations. Spinal anesthesia was achieved successfully, and resulted in a hypesthesia level at T12. Although a epidural catheter was indwelled with no leak of cerebrospinal fluid, an accidental dural puncture appeared later during the surgery, suggesting a subdural catheterization. She had an uneventful perioperative course without any symptoms. In the patient of alkaptonuric ochronosis, the dura and arachnoid membrane could be damaged made vulnerable by HGA. In addition, since the clinical findings resemble ankylosing spondylitis, degenerative changes such as a narrowing of the disk space and spine fusion would make the regional technique unsuccessful. In term of anesthesia, alkaptonuric ochronosis requires ingenuity since there are a number of factors associated with prevention of untoward complications. Each case is to be evaluated individually and managed carefully.

[Diagnosis of intracranial hypotension syndrome by a bolus or continuous epidural injection of normal saline solution].

A 75-year-old man had neck sprain by traffic accident. He had headache but not orthostatic at first. He also had dysesthesia and pain of upper limbs, neck pain, and nausea. His symptoms vanished immediately after 10 ml bolus thoracic epidural injection of normal saline for about one hour. Succeeding continuous epidural injection of normal saline (10 ml x h(-1)) made him symptom free until stopping injection. If headache of a patient vanished after epidural normal saline injection, a diagnosis of intracranial hypotension syndrome can be made. This patient became pain free after one trial of cervical epidural blood patch of 12 ml. Quantity of blood to be injected into the cervical epidural space can be decided by flood of injected blood from thoracic epidural vent tapped in advance.