Cretinism - Congenital hypothyroidism

 


Definition
The condition that results from inadequate secretion of thyroid hormones during fetal life or early infancy. The brain and skeleton fail to develop properly, resulting in mental retardation and dwarfism.
 
Radiographic Appearances
In cretinism, the base of the skull is foreshortened, the face is wide and short, the mandible underdeveloped, and the maxilla overdeveloped. This can be identified both clinically and with lateral cephalometric radiographs. The eruption of primary and permanent teeth is delayed.
The long bones may be thickened and short, the epiphyses appear late and are often irregular and deformed.
 
Pathology
Cretinism is a condition of stunted body growth and impaired mental development. The symptoms, which appear during early infancy, are the gradual development of a characteristic coarse, dry skin, a slightly swollen face and tongue, umbilical hernia, and an open mouth that drools. The baby is usually listless, slow-moving, constipated, and a slow feeder. Cretinism is the result of a congenital deficiency in the secretion of the hormone thyroxine from the thyroid gland. In some cases, this is thought to be caused by an insufficient amount of iodine in the diet of the child's mother during pregnancy. Thyroid testing on newborns to detect cretinism is now a routine test in many countries.
 
Treatment:
Treatment with thyroid hormone promotes normal physical and mental development. It is essential that treatment be started during the first six weeks of life or irreversible changes may take place.

 

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