Clinical symptoms and treatment of smoker’s disease

  Smoky disease is a cerebrovascular disease characterized by chronic progressive stenosis or occlusion of the terminal internal carotid arteries and the beginning segments of the anterior and middle cerebral arteries bilaterally, and secondary to the formation of a characteristic abnormal vascular network at the base of the skull (which looks like smoke on imaging, hence the Japanese name Moyamoya). The cause of the disease is still unknown. In the early stage, the patient mainly shows symptoms of cerebral ischemia due to vascular stenosis, and in the later stage, due to the proliferation of a large number of smoke-like vessels, the proliferating vessels are prone to rupture due to dysplasia, and the symptoms of cerebral hemorrhage are the main symptoms, while cerebral ischemia can be combined.  Clinical manifestations: Ischemic type: unexplained headache, dizziness and dizziness are common, and the symptoms can be mild or severe; transient language disfluency, numbness and weakness of the limbs can also occur; in addition to the above-mentioned manifestations, pediatric patients can also show fainting episodes after anger, crying or activity, which can be easily overlooked or misdiagnosed.  Hemorrhagic type: Adult patients mostly present with spontaneous ventricular hemorrhage and intracerebral hemorrhage. It manifests as sudden headache, nausea and vomiting, coma, hemiparesis, etc.  Hazards of smoker’s disease: Patients can have sudden cerebral infarction or cerebral hemorrhage at any time, resulting in limb paralysis, vision loss, coma, etc. It can be seriously disabling or dead!  Examination methods: Head and neck CT angiography (CTA), head and neck magnetic resonance angiography (MRA), cerebral angiography DSA any of the three tests can make a clear diagnosis.  Indications for surgery: All patients with a clear diagnosis of smog. (Except for coma or complete hemiparesis. Surgery can be considered in January after recovery from ventricular hemorrhage or cerebral hemorrhage) Surgery: There is no curative treatment for this disease. But it can be improved by early surgery —- intracranial and extracranial revascularization to improve cerebral ischemia, reduce the pressure of smoke-like vessels and reduce the probability of cerebral hemorrhage. And the earlier the surgery is performed, the better the results. Since the underlying cause of smog is caused by cerebral ischemia, early surgery to introduce extracranial vessels into the skull to supply brain tissue is the most effective method of treatment.  Especially for patients with spontaneous ventricular hemorrhage and their families, who can have no discomfort at all after the hemorrhage is absorbed, they are reluctant to undergo intracranial and extracranial vascular bypass surgery due to the fear of surgery, and they regret it only when the second hemorrhage occurs, when the opportunity is often lost and regret is left. In clinical practice, we often encounter patients who do not receive timely bypass surgery after the first bleeding, and then suffer further or repeated bleeding disability or death.  Direct intracranial and extracranial revascularization (superficial temporal artery-middle cerebral artery branch anastomosis): The superficial temporal artery in front of the ear screen outside the cranium is anastomosed with a branch of the middle cerebral artery inside the cranium to introduce extracranial blood supply into the ischemic intracranial arterial system and improve cerebral ischemia. (See the bottom 5 color pictures, the 5th color picture shows that the anastomosed vessels are open in the 6-month postoperative review) Indirect intracranial and extracranial revascularization (mainly temporal muscle patching + dural reversal + superficial temporal artery patching): The extracranial temporal muscle, superficial temporal artery and meningeal vessels will be patched on the brain surface, and the previously ischemic brain tissue will induce vascular proliferation of the dura and temporal muscle to supply blood to the brain tissue and improve cerebral ischemia. This procedure usually takes 3-6 months.  Number and cost of surgery: Most patients with smoker’s disease require bilateral surgery, while some patients require surgery on only one side. The specific surgical plan depends on the location of cerebral ischemia, cerebral angiography, and the patient’s symptoms as shown by the patient’s imaging.  Generally, the most serious side of the cerebral vasculature is operated first (for example, if the patient has recurrent numbness in the left hand, the right side of the brain is operated first); the other side is decided after 3-6 months by rechecking the cerebral angiogram.  The cost of doing one side is about 45,000 yuan in our hospital (including all the costs from admission to discharge, of course, the medical insurance can also reimburse part of it, the actual patient pays about 35,000 yuan. The following is a comparison of cerebrovascular DSA angiogram before and after surgery in a patient with smog.  The first two films show the early and late stage of preoperative right-sided extracranial angiography, showing no blood vessels supplying blood to the intracranium; the last two films show the right-sided postoperative extracranial angiography at 6 months showing a significant increase of extracranial vessels such as the superficial temporal artery supplying blood to the intracranial brain tissue.  Here is a case receiving direct + indirect intracranial extracranial vascular