Briefly, all landmarks were labeled on each subject’s face with a surgical marking pen. A total of 23 anthropometric measurements in six craniofacial regions (two on the head, five on the face, four on the nose, five on the eyes, three on the lips and mouth, and four on the ears) per subject were performed using calipers. In this study, our main purpose was to evaluate the craniofacial dimensions of thalassemia major patients and to identify differences by comparing them to those of a control group of comparable age, sex, and ethnicity.Īll subjects or their parents were informed about the craniofacial anthropometric measurements. Craniofacial anthropometry is a useful numerical identification method that assists clinicians to identify deformities and helps surgeons at the stage of reconstructive intervention. The numerical identification of the present disproportion of a morphological facial deformity is the main purpose of these measurements. Craniofacial anthropometry involves the measurement of the skull and face. Īnthropometry is a method used in the measurement of compositions of bones, muscles, and fatty tissues in the human body. In Turkey, the number of homozygote β-thalassemia patients is estimated to be 4000, while the number of carriers of thalassemia major is estimated to be 1,300,000. Thalassemia major is widespread throughout Turkey the frequency of carriers of the disease is 2.1%, and this rate increases to 10% in some areas. Features characterized by prominent frontal and parietal bones, a collapsed nasal bridge, depression of the zygomaticus, and upward-slanted eyes are defined as a slightly mongoloid facial deformity in some patients. The deformations of the “squirrel-like” face are attributed to multi-directional growth, covering the maxillary region in particular, and dental protrusion involving the affected teeth. The craniofacial features of thalassemia major patients include larger cheekbones, a rodent or “squirrel-like” face, a depressed nasal bridge, and a protruding maxilla. Changes in facial and cranial bones have been identified as the overexpansion of the bone marrow results in a typical facial appearance.

The bones become thinner, and pathological fractures may occur.

The orofacial symptoms of thalassemia are due to bone changes associated with ineffective erythropoiesis. Hypertrophy associated with excess erythropoiesis of the bone marrow and the extramedullary region is observed in patients who receive insufficient transfusions. Treatment of the disease requires blood transfusions at regular intervals to prevent heart failure associated with profound anemia. Clinical findings include serious anemia, ineffective erythrocyte production, extramedullary hematopoiesis, and overaccumulation of iron as a result of blood transfusions. Patients present with severe and progressive hemolytic anemia. The clinical onset of disease symptoms occurs in the last six months of the first age. Thalassemia major, also known as “Cooley’s anemia” or “Mediterranean anemia,” is the most severe form of beta-thalassemia thalassemia major patients are homozygotes or compound heterozygotes for beta 0 or beta + genes. Thalassemia major are a group of hereditary blood disorders characterized by absent or reduced β-globin chain synthesis that results in hypochromic microcytic anemia. Thalassemia major is a term used to describe the condition of homozygote or combined heterozygote beta thalassemia patients. Thalassemia is an inherited blood disease demonstrating clinical differences, resulting in hypo chromic microcytic anemia, and is characterized by the lack or deficient production of alpha or beta globin protein chains.

Chipmunk face