Smoker’s disease is a chronic, progressive bilateral internal carotid artery stenosis of unknown cause, resulting in occlusion with the formation of a vascular network at the base of the brain as a characteristic cerebrovascular disease. Clinically, children and adolescents are characterized by cerebral ischemia and infarction, while adults often have intracerebral hemorrhage as the first symptom.
I. Name
Smoker’s disease is cerebrovascular moyamoya disease. The name “smoky disease” is based on the morphological manifestation of the blood vessels seen on cerebral angiography, i.e., the abnormal network of blood vessels at the base of the brain resembles the smoke exhaled when smoking, and is therefore widely accepted and used. It is an imaging name.
Epidemiology
The disease was first reported by Japanese, and Japanese scholars once thought that the disease was unique to Japanese people. The diagnosis of this disease relies on cerebral angiography, so so far, it is impossible to make an objective survey statistics on its incidence. However, the disease is not uncommon and has been reported in Beijing, Shanghai, Shandong, Henan, Hubei, Anhui, Hebei, and Inner Mongolia, with more and more cases found in Henan.
Third, clinical manifestations
1, the age of onset: mostly in children and adolescents, can also be seen in adults. The literature reports that 4-65 years old, under 10 years old, and 30-40 years old are two high incidence age groups, accounting for 50% and 20% respectively.
2. Gender: Both men and women can develop the disease, with a male:female ratio of 1:1.31.
3. Race: Initially thought to be unique to the Japanese nationality, it is now reported from all parts of the world and all nationalities, but is most frequently reported in Yanzhou, with Japan accounting for the majority of cases.
4. Grouping: Since there are significant differences in the clinical presentation of juveniles and adults, they are generally divided into juvenile and adult groups for ease of analysis.
(1) Juvenile group: ≤15 years old (also positioned ≤19 years old), 95% showed cerebral ischemic symptoms, only 5% with hemorrhage as the main manifestation.
(2) Adult group: 65% of those with hemorrhage as the main manifestation and 24.8% of those with ischemia as the main manifestation.
IV. Treatment
No drug can stop the natural progression of the disease, only surgery is the more effective treatment. It is mainly through direct reconstruction of cerebral vessels, indirect reconstruction and combined direct+indirect reconstruction to change the hemispheric academic work and prevent the development of other ischemic occurrences.
Superficial temporal artery – middle cerebral artery.
Posterior auricular artery – middle cerebral artery.
Occipital artery – middle cerebral artery.
Superficial temporal artery-superior cerebellar artery.
occipital artery-superior cerebellar artery.
extracranial artery-graft vessel-intracranial artery anastomosis.
1.Non-anastomotic vascular bypass surgery
(1) combined temporal muscle-vessel-brain patching.
(2) superficial temporal artery patching: the efficacy is comparable to that of combined temporal muscle-vascular patching, especially if the anastomosis fails.
(3) combined dural-ventricular-arterial vascularization.
(4) combined muscle-vascular-brain procedure.
Advantages: little impact on the original collateral circulation, inconspicuous scalp interval, no impact on cosmetic appearance, little surgical trauma, short duration, and little neurological symptoms produced.
(5) Dural flipping patching: the epidural surface with the middle dural artery is covered on the brain surface.
(6) Other tissue patching: such as capillary tendon membrane, subcutaneous tissue covered on the brain surface.
2.Large omental intracranial graft
The ability of the greater omentum to adhere and establish collateral circulation is particularly strong, with adhesion in a few hours and collateral circulation established in a few days. 1980, Karasawa pioneered the division into intracranial transplantation of tipped greater omentum and intracranial transplantation of free greater omentum (anastomosed vessels, non-anastomosed vessels).
V. Prognosis
It depends on the natural progression of the disease and is related to age, etiology, severity of the disease, degree of cerebral infarction, and whether the subsequent treatment is clinical.
Mortality rate: 1.5% for children, 7.5% for adults, 30% of children can have the power underground, adults with intracranial hemorrhage have a high mortality rate, if the coma time is short, most do not leave sequelae.
The natural course of the disease: the natural course of the disease is from 1 year to several years, and even more than 10 years, once the cerebral base artery is completely occluded, when the collateral circulation has been established, the lesion will stop developing.
In conclusion, the prognosis is still optimistic.