Chiari-Frommel Syndrome

Chiari-Frommel syndrome

Chiari-Frommel syndrome is a rare disease characterized by abnormal development of the genital organs in women.

This syndrome was first described by the Austrian gynecologist Hans Chiari and the German gynecologist Richard Frommel in the late 19th and early 20th centuries.

The main manifestations of Chiari-Frommel syndrome:

  1. Agenesis or hypoplasia of the uterus and vagina is incomplete or abnormal development of these organs.

  2. Anomalies in the development of the kidneys and urinary tract.

  3. Heart defects.

  4. Spinal abnormalities such as scoliosis.

  5. Limb defects.

The causes of the syndrome are not completely clear. It is assumed that there are disturbances in the functioning of genes during embryonic development.

Diagnosis is based on identifying characteristic anomalies during examination. Treatment depends on the manifestations and is aimed at correcting existing disorders.

The prognosis for Chiari-Frommel syndrome varies from relatively favorable in mild forms to serious in severe malformations of internal organs.



Chiari–Frommelsytondme (syndrome) is a congenital pathology accompanied by persistent cerebrovascular accident due to compression (squeezing) of the spinal cord by a hard bone formation in the skull. Normally, the spine and spinal cord are located between hard, protective bony plates that form the cranium. However, when there is a malformation of the spine or skull, the upper cervical vertebrae deviate forward, bending the back wall of the skull. Somewhat higher, they compress the spinal cord, which with its upper end goes into the foramen magnum. The abnormal location of the upper cervical vertebrae can be caused by both hereditary reasons and a lack of microelements and birth trauma.