Gingival fibromatosis facial dysmorphism

Gingival Fibromatosis with Facial Dysmorphism is a rare genetic condition characterized by the overgrowth of the gingiva (gum tissue) and distinctive facial features. This condition falls under the broader category of Gingival Fibromatosis, which involves the proliferation of gum tissue, but is distinguished by its association with facial dysmorphism.

Symptoms and Characteristics

The primary symptom of Gingival Fibromatosis with Facial Dysmorphism is the excessive growth of the gum tissue, which can begin in childhood or adolescence. This overgrowth can cover the teeth partially or completely, leading to difficulties in speaking, chewing, and maintaining oral hygiene. The condition is often progressive, with the severity of the gum overgrowth increasing over time.

Facial dysmorphism associated with this condition includes distinctive facial features such as a broad nose, a wide mouth, and a prominent forehead. Other features may include macrocephaly (an abnormally large head), hypertelorism (an increased distance between the eyes), and a high arched palate. These features can vary in severity among individuals with the condition.

Causes

Gingival Fibromatosis with Facial Dysmorphism is a genetic condition. It is often inherited in an Autosomal Dominant manner, meaning that only one copy of the altered gene in each cell is sufficient to cause the disorder. However, cases have been reported where the condition appears without any known family history, suggesting the possibility of new mutations or a complex inheritance pattern.

The specific genes involved in Gingival Fibromatosis with Facial Dysmorphism have not been fully identified, making the genetic basis of the condition an area of ongoing research.

Diagnosis

Diagnosis of Gingival Fibromatosis with Facial Dysmorphism is primarily based on clinical examination. The presence of gingival overgrowth along with the distinctive facial features can lead to a diagnosis. In some cases, a biopsy of the gum tissue may be performed to confirm the diagnosis of fibromatosis. Genetic testing may also be offered to identify any known genetic mutations associated with the condition, although the absence of a known mutation does not rule out the diagnosis.

Treatment

Treatment for Gingival Fibromatosis with Facial Dysmorphism focuses on managing the symptoms. Surgical removal of the excess gum tissue (gingivectomy) may be necessary to expose the teeth and improve oral function. This procedure may need to be repeated if the gum tissue regrows. Orthodontic treatment may also be required to address any dental abnormalities.

Management of the facial dysmorphism may involve consultation with a multidisciplinary team, including a geneticist, a dentist or oral surgeon, and possibly a plastic surgeon. Supportive therapies, such as speech therapy, may be beneficial for some individuals.

Prognosis

The prognosis for individuals with Gingival Fibromatosis with Facial Dysmorphism varies. While the condition does not typically affect overall life expectancy, the severity of the symptoms and their impact on quality of life can vary widely. Regular dental care and appropriate surgical interventions can help manage the symptoms and improve oral health and function.

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