Tuberous sclerosis syndrome, or tuberoussclerosis, also known as Bourneville syndrome, Pringle syndrome, Bourneville-Pringle maternal maculopathy, and symmetrical vasodilated fibromatous maternal maculopathy of the face, is a type of neurocutaneous syndrome. It is clinically characterized by symmetrical facial papules in childhood, epileptiform seizures, intellectual disability, neurological and retinal abnormalities. Symptoms of pediatric tuberous sclerosis syndrome: dementia epilepsy and epileptiform seizures papules intellectual disability convulsions coffee spots intracranial hypertension blind spot cysts This syndrome is a multi-systemic impaired disease, clinical manifestations are as follows: 1. skin symptoms early childhood facial sebaceous adenomas are common, a few appear at birth, symmetrical butterfly-shaped distribution on both sides of the nose and forehead, cheeks, eyelids, etc., external shape like a small ball, from They are orange-red papules or nodules that are hard to touch, rich in blood vessels and lipids, and recede when pressed. 2, neurological symptoms more than 90% of children have epileptiform seizures or infantile spasms, often the first symptoms, often occurring from infancy to 3 years of age, 60% to 70% of cases have intellectual impairment, comprehension and memory loss, progressive aggravation, serious cases become dementia, some cases can be caused by intracerebroventricular nodules caused by obstructive hydrocephalus, or intracranial tumors, intracranial hypertension caused by vascular malformations and The corresponding signs. 3. Other abnormalities retinal gray or yellow-white mottling, blind spots in the visual field, retinal crystal tumor, mulberry-like swelling with refraction in the fundus; renal cysts and urological malformations, mixed tumors of the kidney – misshapen tumors; cardiac rhabdomyosarcoma, benign tumors of the gastrointestinal system, etc. Etiology: (I) Pathogenesis The cause of this syndrome is not well understood, but it is generally believed to be an autosomal dominant disorder, and some believe that it is related to embryonic dysplasia. (The typical lesion in the brain is a cortical gray sclerotic plaque, which is a yellowish-white or white nodule, hard as cartilage, and microscopically composed of hyperplastic glial, astrocytic and deformed giant cells, often with calcium deposits. Treatment: (a) Treatment There is no specific treatment for this sign, but mainly symptomatic treatment. Those with infantile spasms or epileptic seizures can be treated with antiepileptic drugs or selective epilepsy surgery depending on the type of seizure. In the presence of brain tumors and intracranial hypertension, surgical resection, dehydration or shunting is indicated. Patients with tumors less than 3.5-4.0 cm in diameter should have ultrasound examination every 6 months. If the tumor is larger than 3.5-4.0 cm in diameter, prophylactic renal artery embolization or surgery (e.g., removal or partial nephrectomy) may be considered. If progressive enlargement of brain giant cell astrocytoma is found, surgery may be performed before the onset of symptoms or local infiltration. Cerebrospinal fluid circulation obstruction can be treated surgically, and cosmetic surgery is feasible for facial sebaceous adenoma. (B) Prognosis The disease progresses slowly and some patients have a poor prognosis, but most patients can survive for decades. The cause of death is mostly due to brain lesions, and cardiac rhabdomyosarcoma is an important cause of death. Survival into adulthood is rare, and the tendency of the affected family to die out is due to the short survival period and low fecundity. Complications such as renal failure, heart failure, persistent epilepsy, and respiratory failure are the main causes of death.