Roentgen Studies of the Maxillae and Mandible in Sickle-Cell Anemia

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Abstract
There are a number of excellent reports describing the roentgenographic changes in the skull and long bones in sickle-cell anemia (1–14). No reference, however, has been made to the jaws. The purpose of this preliminary report is to describe the roentgenographic findings in the mandible and maxillae in patients with this type of anemia. Features of Sickle-Cell Anemia Definition and Incidence: Sickle-cell anemia is an hereditary and familial form of chronic hemolytic anemia with excessive red blood cell destruction, as well as excessive red blood cell formation as evidenced by hyperplasia of the bone marrow. The disease occurs almost solely in Negroes, and is without sex preference. Elongated crescent or sickle-shaped erythrocytes approximately 15 microns in length are found in the blood stream. The sickle-cell trait, a dominant asymptomatic finding, was present in 7.3 per cent of a series of 8,000 Negroes (15). It is transmitted in accordance with the mendelian law and becomes manifest clinically only when the number of abnormal erythrocytes is great enough. One out of 40 American Negroes with the sickling trait showed evidence of sickle-cell anemia (15). Symptoms and Signs: The symptoms vary considerably, but in general the patient may complain of easy fatigability, weakness, shortness of breath, epigastric and hypochondrial pain, nausea, vomiting, and muscle and joint pains. The heart is usually dilated, as in most prolonged anemias. There is frequently a systolic murmur at the apex, representing relative mitral insufficiency. The spleen is commonly enlarged, although this is not a constant finding. Bone deformities such as kyphosis, scoliosis, saber shin, and the tower-shaped skull have also been described (7). During the “crisis” the patient becomes very ill, often writhes with pain, has a septic type of temperature and a high leukocyte count. The disease in its varied manifestations simulates many surgical conditions. Pathologic Findings: The red bone marrow is soft, jelly-like, and uniformly darkred or purplish-black in the fiat bones of the trunk and calvarium and in the long bones. Microscopically, it is hyperplastic, as evidenced by increased cellularity and congestion. In addition to the abnormal hematopoiesis and blood stasis, there is evidence of degenerative and reparative processes in the marrow. The findings include thrombosis, infarction, necrosis, hemorrhage, granular and crystalline pigment deposits, hyalinization, fibrosis, abnormal calcification, and new bone formation. The bone marrow lesions are similar to those in other organs of the body. Roentgenographic Findings. Long Bones: The roentgenographic findings in the bones of the extremities have received the most attention. In children, the long bones may show widening of the medullary cavity, thinning of the cortex, prominent trabeculations, and occasionally periosteal elevation.
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