【Overview】 Hemifacial hypertrophy refers to the progressive hypertrophy of one side of the face, which is called hemihypertrophy when the hypertrophy involves half of the limbs and trunk. The disease was first reported by Friedreich and Curitiu in 1862 and 1925, respectively, so it is also known as Friedreich’s disease or Curitiu’s syndrome. The main manifestation of the disease is the onset of infancy, slow progression, and the development of one side of the face, limb or trunk that stops on its own in young adulthood. The disease is relatively rare. The etiology and pathogenesis of the disease is unknown and may be related to autonomic function or to endocrine dysfunction. Microscopically, tissue hyperplasia of the skin, subcutaneous tissue and bone can be seen, but without edema, inflammatory and hyperplastic changes. Diagnosis】 Imaging X-rays may reveal thickening of bones or teeth on the side of the lesion, CT and MRI may suggest hypertrophic changes in subcutaneous connective tissue, bones and other organs on the side of the lesion, and ultrasound may assist in detecting organ enlargement on the side of the lesion. Differential diagnosis The disease occurs in infants and young children and progresses slowly, discontinuing spontaneously at least at an early age. The main manifestation is progressive hypertrophy of the face on the side of the lesion, or hypertrophy of the ipsilateral limb, and the diagnosis is confirmed by X-ray examination showing significant thickening of the bone on the side of the hypertrophy. However, the disease requires the following differential diagnosis. 1. Normal asymmetry on both sides Normal people can have incomplete symmetry of the face, limbs or trunk on both sides. Sometimes, in addition to mistaking the small side for hemifacial atrophy, the large side is mistaken for facial hypertrophy. However, physical examination did not reveal hypertrophy of the facial skin, subcutaneous tissue and bones on the larger side. The imaging examination also did not suggest abnormal performance. 2, facial lateral atrophy In the early stage of the disease, when one side of the face atrophy, the other normal side shows relatively large, at this time, attention should be paid to avoid mistaking the normal side for hypertrophy. The distinction is mainly made by local skin changes and X-rays showing bone changes. Treatment overview】 All patients can stop the disease on their own after it has progressed to a certain level. There is no specific treatment for this disease, however, decompression is possible when excessive hypertrophy produces compression symptoms. The disease is also known as Parry-Romberg syndrome. It is a progressive unilateral dystrophic disorder of the facial tissues, with a few lesions involving the limbs or trunk, called progressive hemifacial atrophy. The clinical features are chronic progressive atrophy of subcutaneous fat and connective tissue on one side of the face focally muscle fibers are not involved, and in severe cases invade cartilage and bone Most scholars believe that the disease is related to sympathetic nerve dysfunction and various causes of sympathetic nerve damage, causing neurotrophic disorders in facial tissues, and finally leading to facial tissue atrophy. Other theories involve local or systemic infections and injuries, trigeminal neuritis, genetic degeneration of connective tissue disease, etc. The rate of progression of the disease varies Most cases tend to remit after several years to more than 10 years of progression but the accompanying epilepsy may continue. Epidemiology: Facial deviation atrophy (Parry-Romberg syndrome) starts mostly in childhood and adolescence. It is usually between 10 and 20 years of age, but there is no absolute age limit. More female patients have not been identified more comprehensive incidence statistics Clinical manifestations: 1. Prevalent in adolescents before the age of 20 years occasionally seen within 1 year of age, more common in women. The onset of the disease is insidious, slow progression, the atrophy process can start in any part of the face, mostly one side of the cheek frontal, etc. The upper orbital zygoma is more common starting point is often striped, slightly parallel to the midline; dry skin, wrinkled hair loss, called “knife marks” lesions slowly develop to half of the face severe forehead orbit, ear zygoma, cheek, tongue, gums and other tissues. Occasionally, atrophy of the tongue and gums can spread to the opposite side of the face, cranium, neck, shoulders, or involve other parts of the body, partly with pain or sensory disorders in the cheeks. 2. The disease area shows limited subcutaneous fat and connective tissue atrophy skin atrophy, wrinkling, often accompanied by hair loss, hyperpigmentation, white spots, capillary dilation, increased or reduced sweat secretion, reduced saliva secretion, cheekbones, forehead bones and other sunken and normal skin with a clear line of demarcation. 3, some cases and present pupillary changes, iris pigment reduction, eye sunken or protruding eye inflammation secondary glaucoma facial pain or mild diseased side hyperalgesia, facial muscle twitching, and endocrine disorders, etc., can progress with the course of the disease facial lateral atrophy and focal lipodystrophy is also often accompanied by skin sclerosis of a part of the body. When the lesioned limb and trunk are involved, the limb becomes thinner and shorter, the breast becomes smaller, the axillary hair becomes sparser and the organs become smaller, but the muscle strength is normal. In some cases, the atrophy invades the contralateral limb and is called crossed lateral atrophy. Complications: Only some patients have concomitant seizures or migraine, but about half of the patients have paroxysmal activity recorded on the EEG Diagnosis: The diagnosis is based on the specific facial morphology and imaging changes of the disease. The diagnosis is not difficult when the patient presents with typical unilateral facial atrophy, especially subcutaneous fat atrophy, occasionally spreading to the cranium, neck, shoulders and limbs without affecting muscle strength. Differential diagnosis: Only in the early stages, the following diseases need to be differentiated: 1. congenital lipodystrophy (congenitallipodystrophy) also known as Lawrence-Seip syndrome This disease mainly manifests The trunk, limbs or face scattered distribution of fat atrophy autosomal recessive inheritance in infancy is often complicated by vulvar hypertrophy sweating, head hirsutism black acanthosis. The later development of diabetes can appear liver, kidney insufficiency or cardiac hypertrophy, as well as the combination of acromegaly. 2, limited scleroderma disease at the beginning of the disease may produce confusion but the head and face is not a good site for scleroderma and skin scleroderma and the following tissue adhesions are very tight not easy to pinch up, but also no knife scar type distribution can help to identify. The disease is usually self-limiting and treatment is still limited to symptomatic treatment. Some people use camptothecin (camptothecin hydrobromide) 5ml mixed with saline 10ml for facial acupuncture injection, which can be effective for mild cases. Acupuncture, physiotherapy and tui na can also be used. Those with epilepsy, migraine, trigeminal neuralgia, and eye inflammation should be treated accordingly, and cosmetic surgery is feasible in severe cases. Prognosis: There are no effective preventive measures, but the main thing is to prevent the possible primary cause of the disease.