Pediatric Vascular-Osteomegaly Syndrome

Introduction to vascular-bone hypertrophy syndrome in children Angio-osteohypertrophy syndrome (also known as Klippel-Trénaunay syndrome, Parkes-Weber syndrome, Weber syndrome, hypertrophic vasodilation, vasodilator hypertrophy, osteohytrophic, iliac-varicose veins) Hypertrophy, vascular-ostebral hyperplasia, skin spinal hemangioma, etc., this disease is characterized by hemangioma and bone and soft tissue hypertrophy. basic knowledge The proportion of illness: 0.001% Susceptible people: children Mode of infection: non-infectious Complications: edema phlebitis congestive heart failure

Pediatric vascular-bone hypertrophy syndrome

(1) Causes of the disease

The cause of this disease is unknown.

(two) pathogenesis

For different phenotypic irregular hereditary inheritance, there are recessive inheritance in the patients who are married to close relatives. The pathogenesis originates from various hypotheses such as spinal cord, sympathetic nerve, vascular motor nerve and embryonic development abnormality, but they have not been confirmed.

Pediatric vascular-bone hypertrophy syndrome prevention

There are no systematic preventive measures, and attention should be paid to the prevention and treatment of hereditary diseases. Prenatal diagnosis should be terminated.

Pediatric vascular-bone hypertrophy syndrome complications Complications edema phlebitis congestive heart failure

There are often severe edema, phlebitis, thrombosis and ulceration in the area affected by hemangiomas. There are often multiple venous stones in the rectum and colon, often accompanied by venous fistula, and there are reports of congestive heart failure caused by venous shunt.

Pediatric vascular-bone hypertrophy syndrome symptoms common symptoms bone and soft tissue hypertrophy iris defect varicose skin pigmentation deepening eyeball retinal vein variability

Any age can be ill, most cases occur at birth or shortly after birth, more men than women, bone and soft tissue hypertrophy can involve one or several limbs caused by limb hypertrophy, often non-itopic, may be associated with visceral hypertrophy, and refers to ( Toe), spina bifida and skin pigmentation, ocular manifestations of unilateral congenital glaucoma, ocular invagination, conjunctival telangiectasia, iris defect, retinal varicose veins and choroidal hemangioma.

Cardiovascular damage: plaque or cavernous hemangioma can occur in the face, limbs, brain and meninges at birth, in addition to congenital varices, lymphangioma, or any combination of the above.

Examination of vascular-bone hypertrophy syndrome in children

Peripheral blood tests can sometimes have anemia, red blood cell counts, and a decrease in hemoglobin.

X-ray, B-ultrasound, electrocardiogram, CT and MRI should be performed.

1. X-ray examination showed that the affected side of the bone tissue growth and thickening, abdominal plain film can show multiple changes in the rectum and left colon.

2. B-ultrasound examination of the fetus can be seen to have an anechoic zone that represents hemangioma.

Diagnosis and diagnosis of vascular-bone hypertrophy syndrome in children

According to the characteristics of vascular lesions and bone and soft tissue hypertrophy, supplemented by X-ray examination, the growth of the affected side of the bone tissue and thickening, abdominal plain film can show multiple changes in the rectum and left colon, such as multiple venous stones, ultrasound examinations in recent years Prenatal diagnosis is made by understanding whether the fetus has an anechoic area that represents a hemangioma.

The disease must be differentiated from Sturge-Weber syndrome (spleen hemangioma), which is mostly confined to the brain, pia mater and face, and less affected by limbs. Most of the limbs are unilateral and less Involved in the opposite side, can be identified.

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