Worth syndrome, also known as benign form of Worth hyperostosis corticalis generalisata with torus platinus, autosomal dominant osteosclerosis, autosomal dominant endosteal hyperostosis or Worth disease, is a rare autosomal dominant congenital disorder that is caused by a mutation in the LRP5 gene. It is characterized by increased bone density and benign bony structures on the palate. Causes
Worth syndrome is caused by a mutation in the LRP5 gene, located on human chromosome 11q13.4. The disorder is inherited in an autosomal dominant fashion. This indicates that the defective gene responsible for a disorder is located on an autosome (chromosome 11 is an autosome), and only one copy of the defective gene is sufficient to cause the disorder, when inherited from a parent who has the disorder. Diagnosis
Treatment
History
The condition was first reported by H. M. Worth in 1966. In 1977, two doctors, R.J. Gorlin and L. Glass, distinguished the syndrome from van Buchem disease. In 1987 a group of Spanish doctors pointed out that the condition may not be benign, and may sometimes cause nerve damage. References


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Treatment
Non-surgical
Early treatment for mild cases of hallux rigidus may include prescription foot orthotics, shoe modifications (such as a pad under the joint, and/or a deeper toe box to take the pressure off the toe and/or facilitate walking), specialized footwear (rocker-sole shoes), medications (anti-inflammatory drugs) or injection therapy (corticosteroids to reduce inflammation and pain). Physical therapy programs may be recommended, although there is very limited evidence that they provide benefit for reducing pain and improving function of the joint. Surgical
The goal of surgery is to eliminate or reduce pain. There are several types of surgery for treatment of hallux rigidus. The type of surgery is based on the stage of hallux rigidus. According to the Coughlin and Shurnas Clinical Radiographic Scale:Stage 1 hallux rigidus involves some loss of range of motion of the big toe joint or first MTP joint and is often treated conservatively with prescription foot orthotics. Stage 2 hallux rigidus involves greater loss of range of motion and cartilage and may be treated via cheilectomy in which the metatarsal head is reshaped and bone spurs reduced. Stage 3 hallux rigidus often involves significant cartilage loss and may be treated by an osteotomy in which cartilage on the first metatarsal head is repositioned, possibly coupled with a hemi-implant in which the base of the proximal phalanx (base of the big toe) is resurfaced. Stage 4 hallux rigidus, also known as end stage hallux rigidus, involves severe loss of range of motion of the big toe joint and cartilage loss.