Dr. Ebraheim’s educational animated video describes the condition of Vertebral Plana Eosinophilic Granuloma.
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Eosinophilic Granuloma Vertebral Plana
Vertebra Plana is a flattened vertebra in the spine, and it is associated with eosinophilic granuloma. Eosinophilic granuloma is a tumor like condition due to proliferation of the histiocytes. It is called histiocytosis X or Langerhans histiocytosis X. Eosinophilic granuloma can occur in any bone in the skeleton. It can also occur in the skin and in the skull. Eosinophilic granuloma also has a visceral involvement. Eosinophilic granuloma is called the great imitator. It looks similar to many lesions such as osteomyelitis, Ewing’s sarcoma, leukemia, lymphoma, and fibrous dysplasia. The condition of eosinophilic granuloma is common in the spine, and it may cause flattening of the vertebra called “vertebra plana”. Vertebra plana has many names such as “pancake vertebra” or “coin on edge vertebra”. The vertebral body loses its height anteriorly and posteriorly, and it is most common in the thoracic spine. This complete compression of the vertebra can lead to back pain or neck pain and kyphosis. If this magnitude of compression occurs in the elderly patient, usually it is due to osteoporosis. If it occurs in the younger patient (usually between 2-10 years old), then it is due to eosinophilic granuloma. It is usually a single vertebra is affected. With collapse of multiple vertebrae, look for other reasons such as lymphoma, Gaucher disease, mucopolysaccharides and metastatic disease. When eosinophilic granuloma is seen in bones, it usually appears as a focal destruction of bone and is usually seen in children under the age of 10 years. The lesion may appear very aggressive; however, it is benign, self-limited, and it will heal spontaneously. It affects the vertebral body more than the posterior elements of the spine. Biopsy is usually not needed, especially if the patient has the characteristic history and x-ray appearance. The disc height is maintained and no involvement of the posterior element. There will be large cells. The histiocytes have a clear cytoplasm and a single large, oval nucleus or “bean shaped” nuclei. These cells do not have nuclear atypia or nuclear mitosis, so it is not malignant cells. These cells are called Langerhans cells. The Langerhans cells are grouped “coffee bean shaped” nuclei with abundant cytoplasm. You also find eosinophils (smaller cells) with bilobed nuclei and eosinophilic cytoplasm (pink). The eosinophils may be found in large numbers. You may also find birbeck granules under electron microscopy (tennis racket shaped structures) inside the Langerhans cells. Ewing’s sarcoma will have round, blue nuclei. Osteomyelitis will have mixed inflammatory cells. Eosinophilic granuloma is self-limited, usually managed conservatively. The spine lesions usually resolve spontaneously. Bracing may prevent progressive deformity of the spine and correct the deformity in the majority of cases. Vertebral lesions usually regain about 50% of its height back. If the patient has no neurological deficit, then follow the lesion and treat it nonoperatively. 10% of the patients with a spine lesion may need surgery for deformity correction or to relieve severe neurologic deficit. Low dose radiation (about 500-900 rads) may be indicated if there is neurological deficit, especially if surgical decompression of the lesion is not possible.
