- Case report
- Open Access
- Open Peer Review
Leontiasis ossea and post traumatic cervical cord contusion in polyostotic fibrous dysplasia
© Maramattom; licensee BioMed Central Ltd. 2006
- Received: 29 January 2006
- Accepted: 15 August 2006
- Published: 15 August 2006
Leontiasis ossea (leonine facies) or cervical canal stenosis are rare complications of polyostotic fibrous dysplasia (PFD). This case report documents dramatic leontiasis ossea in PFD as well as post traumatic cervical cord contusion due to hyperextension injury in a patient with generalized PFD involving the cranio-facial bones, axial skeleton and entire spine with secondary cervical canal stenosis. Cervical cord contusion has not been reported earlier in PFD.
- Fibrous Dysplasia
- Diffuse Idiopathic Skeletal Hyperostosis
- McCune Albright Syndrome
- Facial Deformity
Fibrous dysplasia (FD) is a rare skeletal developmental disorder whereby the medulla of bone is replaced by fibrous tissue leading to distortion of bony architecture, expansion and weakening of bones, easy fractures, joint subluxations or dislocations and compressive symptoms . Four varieties of FD are recognized; the monostotic form (single bone involvement), polyostotic form (PFD) [multiple bones are affected], craniofacial form (multiple craniofacial bones are affected) or a cherubic form (maxilla and mandible alone are affected). PFD has a predilection for the long bones, ribs, spine and craniofacial bones. It is sometimes associated with the McCune Albright syndrome where café au lait spots and endocrinopathies (particularly precocious puberty) coexist. Rarely, other endocrine dysfunction such as hyperthyroidism, growth hormone excess, Cushing syndrome or primary hyperparathyroidism can also be associated with PFD. Although FD is linked to an activating mutation in the gene that encodes the subunit of stimulatory G protein (Gs) located at 20q13.2–13.3, it is a non-heritable congenital developmental disorder.
Fibrous dysplasia predisposes the spine to atlanto-axial instability , odontoid fractures , compression fractures, spinal cord compression via expansile lesions [4, 5] sarcomatous transformation  or scoliosis . Although PFD can produce spinal canal stenosis with consequent pathological implications, cervical cord contusions have never been reported before with this disorder.
I would like to report a case of PFD affecting the entire spine, producing cervical canal stenosis and post traumatic cervical cord contusion. This report is also noteworthy for its dramatic depiction of leontiasis ossea, a peculiar facial deformity sometimes associated with PFD.
PFD is a rare cause of CCS because it afflicts the lumbar, thoracic, sacral and cervical vertebrae in descending frequency. Nearly 70% of the lesions involve only the posterior aspects of the spine . The most common abnormality seen in PFD is scoliosis (~50% of patients). PFD has rarely been reported to cause pathologic compression fractures of the lumbar spine . Our case is remarkable in that PFD involved the entire vertebral column producing cervical canal stenosis predisposing the patient to cervical cord contusion during hyperextension injury. Another remarkable feature was the leontiasis ossea involving the entire craniofacial skeleton which has rarely been reported .
Although the term 'leontiasis ossea' is widely used for localized swellings of the face including those involving the jaw, it should be restricted to a generalized homogenous swelling that implicates most facial bones . True leontiasis ossea is a rare facial deformity that is encountered in polyostotic FD, Albright's syndrome and rarely with Paget's disease, uremia with secondary hyperparathyroidism or acromegaly. Leontiasis ossea can be associated with progressive proptosis, visual impairment or nasal obstruction. Our patient had a relatively asymptomatic leontiasis ossea of long duration with only mild obstructive nasal symptoms, snoring and cosmetic disfigurement.
In conclusion, we present an unusual presentation of PFD with diffuse involvement of the cranio-facio-vertebral skeleton with leontiasis ossea and a post traumatic cervical cord contusion secondary to hyperextension injury of the cord in a compromised cervical canal.
"Written consent was obtained from the patient for publication of study".
- Leet AI, Chebli C, Kushner H: Fracture incidence in polyostotic fibrous dysplasia and the McCune-Albright syndrome. J Bone Miner Res. 2004, 19: 571-577. 10.1359/JBMR.0301262.View ArticlePubMedGoogle Scholar
- Perlick L, Rolf V, Wallny T, Schmitt O: Atlanto-axial instability presenting a rare complication in fibrous dysplasia – a case report. Unfallchirurg. 2000, 103: 73-75. 10.1007/s001130050011.View ArticlePubMedGoogle Scholar
- Stompro BE, Alksne JF, Press GA: Diagnosis and treatment of an odontoid fracture in a patient with polyostotic fibrous dysplasia: case report. Neurosurgery. 1989, 24: 905-909.View ArticlePubMedGoogle Scholar
- Mezzadri JJ, Acotto CG, Mautalen C, Basso A: Surgical treatment of cervical spine fibrous dysplasia: technical case report and review. Neurosurgery. 1999, 44: 1342-1346. 10.1097/00006123-199906000-00110.PubMedGoogle Scholar
- Montoya G, Evarts CM, Dohn DF: Polyostotic fibrous dysplasia and spinal cord compression. Case report. J Neurosurg. 1968, 29: 102-105.View ArticlePubMedGoogle Scholar
- Yalniz E, Er T, Ozyilmaz F: Fibrous dysplasia of the spine with sarcomatous transformation: a case report and review of the literature. Eur Spine J. 1995, 4: 372-374. 10.1007/BF00300303.View ArticlePubMedGoogle Scholar
- Leet AI, Magur E, Lee JS, Wientroub S, Robey PG, Collins MT: Fibrous dysplasia in the spine: prevalence of lesions and association with scoliosis. J Bone Joint Surg Am. 2004, 86: 531-537.PubMedGoogle Scholar
- Hoffman KL, Bergman AG, Kohler S: Polyostotic fibrous dysplasia with severe pathologic compression fracture of L2. Skeletal Radiol. 1995, 24: 160-162. 10.1007/BF00198085.View ArticlePubMedGoogle Scholar
- Viljoen DL, Versfeld GA, Losken W, Beighton P: Polyostotic fibrous dysplasia with cranial hyperostosis: new entity or most severe form of polyostotic fibrous dysplasia?. Am J Med Genet. 1988, 29: 661-667. 10.1002/ajmg.1320290325.View ArticlePubMedGoogle Scholar
- Evans J: Leontiasis ossea; a critical review, with reports of four original cases. J Bone Joint Surg Br. 1953, 35: 229-243.PubMedGoogle Scholar
- Dantas M, Costa RS, Jorgetti V: Facial leontiasis ossea: a rare presentation of hyperparathyroidism secondary to chronic renal insufficiency. Nephron. 1991, 58: 475-478.View ArticlePubMedGoogle Scholar
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.