What Is Larsen Syndrome?

Flat face joint dislocation foot abnormal syndrome is a group of comprehensive deformities characterized by special faces, large joint dislocations and other skeletal abnormalities.

Flat face joint dislocation foot abnormal syndrome

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Flat face joint dislocation foot abnormal syndrome is a group of comprehensive deformities characterized by special faces, large joint dislocations and other skeletal abnormalities.

nickname

Larson syndrome

TCM disease name

Flat face joint dislocation foot abnormal syndrome

Larsen syndrome, Larsen syndrome, Larsen syndrome

ICD: Q89.8

Pediatrics

Flat face joint dislocation foot abnormal syndrome, also known as Larsen syndrome, is a group of comprehensive deformities characterized by special faces, large joint dislocations and other skeletal dysplasia.

1. Special features: prominent forehead, flat cheeks, flat nose bridge, wide eye distance.

2. Joint dislocation was congenital multiple large joint dislocations, mainly elbow, hip and knee joints.

3. Abnormal fingers and hands are shaped like sausages and short sticks; feet are deformed with varus and eversion of horseshoe.

4. Other manifestations of intelligence have no effect, there may be spina bifida, scoliosis, posterior process, condylar abnormalities, cervical segmental abnormalities, congenital heart disease and so on.

The incidence of the abnormalities analyzed by Masson in 1978: 100% dislocation of knee joint; 95% abnormality of foot; 93% facial abnormality; hip dislocation of 60%; hand and finger deformity of 50%; elbow joint dislocation of 47% ; abnormal 37%.

The cause of the disease is unknown.

May be a connective tissue lesion. The genetic type has been reported to be autosomal recessive or dominant, or it can be single.

Diagnosis: According to the above clinical characteristics, combined with X-ray abnormalities for diagnosis. Blood biochemistry, electromyogram, and muscle biopsy were mostly normal.

Laboratory tests: Concomitant infections may have corresponding changes in the hemogram of infection.

Other auxiliary examinations: X-rays, electrocardiograms, and echocardiograms should be performed.

Key points of X-ray diagnosis:

1. Large joint dislocation changes symmetrically, in order of occurrence: knee-tibia is located in front of the femur, hip dislocation is the same as simple congenital hip dislocation, elbow dislocation to the ulnar side, often accompanied by hypoplasia of the lower end of the humerus .

2. 95% of cases of foot abnormalities are accompanied by foot deformities (horse varus foot inversion, valgus deformity, etc.). Additional ossification centers can be found behind the calcaneus, hands, wrists, knees, elbows, etc. This is one of the characteristics of this syndrome .

3. The shape of the abnormal finger of the hand is sausage-shaped, each metacarpal is short and uneven, and the phalanx of the thumb is deformed like a doctor blade.

4. Spinal abnormalities Common cervical segmental abnormalities, cervical thoracic spine at the lateral film lordosis deformity, spina bifida, lateral process and posterior process deformities.

5. The head is abnormally forehead flat, the eyes are too wide, the bridge of the nose is sunken, the chin is small, and the interoccipital bone is in the top occipital region.

6. Congenital heart disease: aortic root dilatation, aortic stenosis and insufficiency.

The symptoms should be distinguished from Rubinstein-Taybi syndrome, Ehlers-Danlos syndrome, and Otopalatodigital syndrome. These syndromes have only 1 or 2 malformations of the syndrome No major multiple joint dislocations were found.

Large joint dislocation and foot deformity can be considered surgical correction, no special treatment.

With multiple deformities, in addition to facial and skeletal deformities, there are congenital heart disease and spina bifida.

Prognosis: The symptoms have no significant effect on life.

Prevention: The cause is unknown. Refer to the preventive measures for hereditary diseases.

First described by Larsen in 1950, Silvermanl reported 33 cases in summary in 972, and Liu Aiqin in China reported 2 cases in 1988.

The cause is unknown, and preventive measures for hereditary diseases can be followed.