Patients most often present during the third through sixth decades of life.  Retropharyngeal calcific tendonitis can be misdiagnosed as retropharyngeal abscess, traumatic injury, or infectious spondylitis. Symptoms include neck pain, dysphagia, odynophagia, and low-grade fever.

The diagnosis is made radiographically by calcification anterior to C1–C2 and prevertebral soft-tissue swelling. Prevertebral fluid collection/effusion has been described and is considered nearly pathognomonic of this entity. The prevertebral collections tend to be smooth, linear/lenticular and do not contain an enhancing wall (which helps distinguish it from an abscess).

Le Fort I fractures, also known as Guerin fractures or ‘floating palate’, usually involve the inferior nasal aperture. The fracture extends from the nasal septum to the lateral pyriform rims, extends horizontally above the teeth apices, crosses below the zygomaticomaxillary junction, and traverses the pterygomaxillary junction to interrupt the pterygoid plates. May occur in isolation or in association with le Fort II and III fracture.

Le Fort II fractures have a pyramidal shape and extend from the nasal bridge at or below the nasofrontal suture through the frontal processes of the maxilla, inferolaterally through the lacrimal bones and inferior orbital floor and rim through or near the inferior orbital foramen, and inferiorly through the anterior wall of the maxillary sinus; it then travels under the zygoma, across the pterygomaxillary fissure, and through the ptergoid plates

Le Fort III fractures follow impact to the nasal bridge or upper maxilla. These fractures start at the nasofrontal and frontomaxillary sutures and extend posteriorly along the medial wall of the orbit through the nasolacrimal groove and ethmoid bones. The fracture continues along the floor of the orbit along the inferior orbital fissure and continues superolaterally through the lateral orbital wall, through the zygomaticofrontal junction and the zygomatic arch. Intranasally, a branch of the fracture extends through the base of the perpendicular plate of the ethmoid, through the vomer, and through the interface of the pterygoid plates to the base of the sphenoid.

Polyostotic disease can be associated with abnormal skin pigmentation (ipsilateral to the osseous lesions) and endocrinopathies. The constellation of polyostotic fibrous dysplasia, skin pigmentation and precocious puberty has the eponym McCune Albright Syndrome. Mazabraud Syndrome is fibrous dysplasia associated with soft tissue myxomas. Malignant degeneration is rare but know complication.

Radiographically, Fibrous dysplasia appears as a well circumscribed intramedullary lesion with a ground glass matrix and a narrow zone of transition with no periosteal reaction, cortical thickening or soft tissue mass. Bony deformity can result in so called “shepherd’s crook” deformity of long bones.  On MRI Fibrous dysplasia shows variable appearance depending on the amount of fibrous and ossified tissue present but typically show areas of low signal on both T1 and T2 WI.

Diagnostic criteria for Sjogren’s Syndrome typically includes: Schirmer test, Keratoconjunctitis, Xerostomia, Extensive lymphocytic infiltrate on minor salivary gland biopsy, Laboratory evidence of a systemic autoimmune disorder.

Ultrasound shows enlarged salivary glands with multiple scattered cysts and increased vascularity on doppler. MRI shows inhmogeneous honeycomblike internal septation pattern, bilateral cysts. B Cell-Lymphomatous transformation, typically low grade, occurs in approximately 5 to 10 percent of patients.

SICCA SYNDROME (Mikulicz disease) : xerophthalmia and xerostomia.