Organs become smaller

Introduction Smaller organs are clinical manifestations caused by diseases such as progressive facial atrophy. Progressive hemifacial atrophy (progressivehemifacialatrophy) is also called Parry-Romberg syndrome, a progressive unilateral tissue dystrophic disease, a small number of lesions involving the limbs or body, called progressive half atrophy Its clinical features are chronic progressive atrophic muscle fibers of focal subcutaneous fat and connective tissue on one side of the face and are not affected by severe cartilage and bone. Most scholars believe that this disease and sympathetic dysfunction are related to various causes of sympathetic nerve damage, causing facial tissue neurotrophic disorders, and finally lead to facial tissue atrophy. Other doctrines involve local or systemic infection damage, genetic degeneration of trigeminal connective tissue disease.

Cause

The cause of this disease is not yet clear. Symptoms of cervical sympathetic disorders, including Horner syndrome, are generally considered to be associated with central or peripheral damage to the autonomic nervous system.

In recent years, some patients have found that some patients often have dysplasia, congenital aneurysm, brain hypoplasia, etc. It is speculated that genetic factors cause abnormal embryogenesis, which may also be related to this disease. In addition, a small number of this disease can be secondary to certain infections such as polio, trauma, autocrine diseases with endocrine dysfunction.

Pathogenesis:

The muscles in the damaged area shrink due to the disappearance of fat and connective tissue, but the muscle fibers are not tired, and the contraction ability is preserved. The subcutaneous fat and connective tissue in the facial lesion are the first to be involved and then involve the skin, subcutaneous tissue, hair and fat. The heaviest gland invades cartilage and bone. Skin and subcutaneous fat other than the face, subcutaneous tissue, soft vocal cords, and other internal organs are also involved.

The ipsilateral cervical sympathetic nerve may have small round cell infiltration in some cases accompanied by atrophy of the cerebral hemisphere may be ipsilateral, contralateral or bilateral individual and accompanied by partial atrophy. Biopsy showed atrophy of the skin papillary layer, fibrosis of the dermis and adipose tissue, and decreased cross-grain of the muscle fibers infiltrated by lymphocytes and plasma cells. In addition, there have been reports in the literature (Henta 1999) facial nerve fiber atrophy and vacuolar degeneration, axonal degeneration can be seen under electron microscope.

an examination

Related inspection

CT examination of liver, gallbladder and spleen in palpation of abdominal organs

The diagnosis is based on the special facial morphology and imaging changes of the disease. When the patient has a typical unilateral atrophy, especially when the subcutaneous fat atrophy occasionally affects the head, neck, shoulders and limbs, the muscle strength is not affected.

1. Adolescents who are born before the age of 20 are occasionally seen in women who are diagnosed within 1 year of age. Insidious onset, slow progress, the process of atrophy can start at any part of the face, such as a side of the cheeks, etc., more common in the upper part and the upper part. The starting point is often strip-shaped and parallel to the midline; the skin is dry and wrinkled and the hair is detached. The lesion is called "knife mark". The lesion develops slowly to half of the face. The forehead of the eyelids, the cheeks of the ankle, the tongue and gums, etc. . Occasionally, it can affect the facial head, neck, shoulders, or other parts of the body to cause pain or sensory disturbances on the cheeks. A few onsets are seizures.

2. The ward is characterized by subcutaneous fat and connective tissue atrophy, skin atrophy and wrinkles, often accompanied by hair loss, hyperpigmentation, leukoplakia, telangiectasia, increased sweat secretion or reduced salivary secretion, sacral frontal bone, etc., and normal skin There is a clear dividing line.

3. Some cases show pupillary changes, iris pigmentation, eyeball retraction or protrusion, eyeball inflammation, secondary glaucoma facial pain or mild side sensation, facial muscle twitching, and endocrine disorders, which may progress with the course of the disease. Facial atrophy and focal lipoatrophy, often accompanied by skin hardening in a certain part of the body involving the limbs and trunk of the lesions, the limbs become thinner and shorter, the breasts become smaller, the bristles become thinner, the organs become smaller, but the muscles The force is normal. Some atrophy invades the contralateral limbs called cross-lateral atrophy.

It is characterized by one or all progressive atrophy of the lateral side tissue.

Women accounted for about 3/5, and those who developed the disease before the age of 20 accounted for 3/4. The left side is more common, the facial atrophy starts from a part, and the progress speed is uncertain. Generally, it develops into a partial atrophy in about 2 to 10 years. 5% of cases involve both sides of the face, in addition to facial atrophy, often involving soft palate, tongue and oral mucosa, occasionally the ipsilateral neck, chest and even the trunk and extremities atrophy (about 10%). The side of the affected area is old and has a sharp contrast with the healthy side. The hair, eyebrows and eyelashes often fall off, and there are white spots and skin blemishes. There may be facial pain or migraine, and sensory disturbances are rare. In addition to the affected side sweat glands and lacrimal gland dysregulation, Horner's syndrome can be seen. A few patients have seizures, and about half of them have paroxysmal activity. Patients with brain atrophy may have hemiplegia, partial sensory disturbance, hemianopia, and aphasia.

Differential diagnosis

Differential diagnosis of small organs:

1, congenital lipodystrophy (congenital lipodystrophy), also known as Lawrence-Seip syndrome This disease mainly manifests the body, limbs or facial scattered fat atrophy autosomal recessive inheritance. Onset in infancy, often complicated by genital hypertrophy, hyperhidrosis, head hirsutism, black acanthosis later developed into diabetes can occur liver, kidney dysfunction or cardiac hypertrophy, and acromegaly.

2, liver shrinkage: "liver atrophy" refers to B-ultrasound or CT detection, liver volume reduction, belonging to traditional medicine "bulging", modern medical qualitative as "cirrhosis", liver reduction at present, there is no other than liver transplantation at home and abroad Effective treatment. In the 1940s, the medical community first proposed the concept of fulminant hepatic failure (FHF), originally called acute liver atrophy or acute liver necrosis.

3, the abdomen depression is a boat-shaped: the anterior abdominal wall is significantly lower than the horizontal plane of the rib to the pubic bone when lying on the back, called the abdominal retraction (abdominal retraction), the full abdominal depression refers to the anterior abdominal wall in the lying position is diffusely significant concave, when the condition is serious The anterior abdominal wall is almost close to the spine, and the rib arch, ankle and pubic symphysis are abnormally revealed, and the whole abdomen is shaped like a boat, which is called a boat-like abdomen.

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