Vertebral hyperostosis, ankylosed vertebral fracture and atlantoaxial rotatory subluxation in an elderly patient with a history of infantile idiopathic scoliosis; a case report
© Al Kaissi et al; licensee BioMed Central Ltd. 2007
Received: 31 January 2007
Accepted: 06 June 2007
Published: 06 June 2007
This is a case report of a 48-year-old-woman with scoliosis since early childhood. Recent radiographic spinal assessment revealed the presence of distinctive spinal abnormalities. To the best of our knowledge this is the first clinical report describing a constellation of unusual changes in an elderly woman with a history of infantile idiopathic scoliosis.
Forestier and Rotes-Querol first described the disease Diffuse Idiopathic Skeletal Hyperostosis (DISH) in 1950 . These authors provided a precise description, separating the disease from discoarthrosis and ankylosing spondylitis. Resnick and Niwayama  described the diffuse nature of the disease and proposed widely used diagnostic criteria. The disease is usually seen in male patients over 45 years of age and characterised by new bone formation at the entheses. Diagnostic criteria of DISH include flowing ossification along at least 4 contiguous vertebrae, preservation of disk spaces, absence of vacuum phenomena or vertebral body marginal sclerosis, and absence of apophyseal joint ankylosis or sacroiliac joint erosions or fusion. The thoracic spine is most commonly involved, but radiographic findings in both the spine and extraspinal structures suggest a generalised disorder of ossification rather than a localised spinal disease. While Diffuse Idiopathic Skeletal Hyperostosis (DISH) is mostly asymptomatic, it can predispose the patient to catastrophic complications. The common potential complications of DISH in the cervical and thoracic spine include fractures, dysphagia, cervical and/or thoracic myelopathy, paraplegia, and dens spinal cord injury resulting from even minor trauma [1–6].
On the other hand the classical risk factors for DISH are known to co-exist in the following conditions; diabetes mellitus type 2, obesity, and hyperuricaemia . Previous reports describing DISH patients with spinal fractures are rare [3–5]. Scoliosis is seemingly not listed among the major risk factors for the development of DISH. The purpose of this case report is to characterise and consider whether idiopathic infantile scoliosis represents an additional risk factor for the development of spinal hyperostosis in elderly people
She was married and her gestational history revealed five pregnancies (two spontaneous abortions in the first trimester and another two ectopic pregnancies, the reason behind these events were not identified). She had one normal male offspring.
Recently, she was identified, as being affected with spinal osteoporosis through the national screening programme. Central (DXA) showed that lumbar spine, T -Score -3.1 SD (osteoporosis), whereas the femoral neck T-Score -1.7 SD (osteopenia). Blood sugar and uric acid levels were normal. Biochemical tests showed mild elevations of serum calcium 2.74 (2.20–265 mmol/l), ionised calcium 1.63 (1.18–1.30 mmol/l), β-crosslaps 1.170 (normal for postmenopausal women is 0.400–1.008 ng/ml).
Diffuse idiopathic skeletal hyperostosis (DISH) is an ossifying, non-inflammatory, non-erosive enthesopathy favouring the dorsal spine but sparing the sacroiliac joints. DISH affects 3–6% of the population over 40 years of age and 11% aged over 70 years [1–4]. A varying proportion of patients with DISH have ossification of the posterior longitudinal ligaments.
DISH leads to acquired narrowing of the spinal canal due to the presence of osteophytes, which sometimes create bony hooks within the spinal canal [1–6]. The cause and pathogenesis of DISH are still unknown. Kiss et al.,  studied the risk factors and the radiographic features of 131(69 males and 62 females) affected with DISH. Obesity, diabetes mellitus, smoking, and hypertension were shown to be the most likely predisposing factors. Some authors have noted the occasional familial incidence of DISH, leading to a suspicion of genetic predisposition 
De Peretti et al , described 48 fractures in 48 patients over a period of 17 years. Twenty patients (mean age 62 years) had ankylosing spondylitis and 28 patients (mean age 81 years) had DISH syndrome. They concluded that spinal fractures in patients with DISH syndrome generally occur spontaneously or after low-energy trauma. None of the reported patients showed a history of scoliosis. They identified 4 types of spine fractures in their series. Our patient manifested type II fracture of the de Peretti et al, classification.
Previous studies have indicated a significant correlation of osteoporosis with idiopathic scoliosis in adults. Cheng and Guo  supported the hypothesis that adolescents with idiopathic scoliosis are at increased risk of osteoporosis compared to the general paediatric population. None of these reports signified the correlation between idiopathic infantile scoliosis and the development of DISH later in life.
Our patient illustrated lateral displacement of the dens by more than 4 mm, the latter was suggestive of atlanto-axial rotatory subluxation (AARS) . The computerised tomogram of the atlanto-axial region demonstrated asymmetrical odontoid-lateral mass distance and confirmed the existence of two healed fractures. It is suggested that the persistence of abnormal dynamics, secondary to the delay in treating AARS, can lead to the development of pathological stickiness between the atlas and the axis, probably because of contractures of peri-articular soft tissue [8, 9]. Evidence supports, that conservative treatment in the elderly population can be managed non-operatively, because few demands are made on the neck. In young adults the story is different. The use of early cranial traction followed by external immobilisation for six weeks is a usual procedure to achieve good long-term stability. If recurrent or irreducible subluxation developed, open reduction and posterior atlantoaxial fusion may be required 
Osteoporotic vertebral compression fractures are a major cause of morbidity and health care cost among elderly patients. In the past, the primary therapy for these fractures has been conservative. Percutaneous vertebroplasty is now a therapeutic option for individuals where medical management has not been successful or for those at risk of developing complications due to long-term immobilization. On the other hand, injectable biomaterials may decrease the incidence of new vertebral fracture e.g. calcium phosphate has been introduced to relieve pain and at the same time is capable of integrating into the bony matrix 
In the light of our findings, we believe that the degree of osteoporosis of the adjacent vertebral bodies, the development of ankylosed spinal hyperostosis, fractures and AARS are a constellation of abnormalities developed in connection with the early onset of idiopathic infantile scoliosis. DISH patients are usually reported to have a history of diabetes mellitus, obesity, and hyperuricaemia. We wish to stress, that scoliosis might be a possible confounder in the relationship between spinal osteoporosis and the development of spinal hyperostosis in the elderly. Further studies are needed to elucidate this sort of correlation.
diffuse idiopathic skeletal hyperostosis
Atlantoaxial rotatory subluxation
atlanto axial subluxation.
We thank Dr. A Z Al-Bahrani Specialist Registrar, Department of Surgery. Hemel Hempstead General Hospital, UK for his technical help. And we thank the patient for cooperation and consent for publishing the data.
- Forestier J, Rotes-Querol J: Senile ankylosing hyperostosis of the spine. Ann Rheum Dis. 1950, 9: 321-30.View ArticlePubMedPubMed CentralGoogle Scholar
- Resnick D, Shaul RS, Robins JM: Diffuse idiopathic skeletal hyperostosis (DISH): Forestier's disease with extraspinal manifestations. Radiology. 1975, 115: 13-24.View ArticleGoogle Scholar
- Paley D, Schwartz M, Cooper P, Harris WR, Levine AM: Fractures of the spine in diffuse idiopathic skeletal hyperostosis. Clin Orthop Relat Res. 1991, 267: 22-32.PubMedGoogle Scholar
- de Peretti, Sane JC, Drane G, Razafindrotsiva C, Argenson C: Ankylosed spine fractures with spondylitis or diffuse idiopathic skeletal hyperostosis: diagnosis and complications. Rev Chir Orthop Reparatrice Appar Mot. 2004, 90 (5): 456-65.View ArticleGoogle Scholar
- Kiss C, Szilagy MM, Pasky A, Poor G: Risk factors for diffuse idiopathic skeletal hyperostosis: a case-control study. Rheumatology. 2002, 41: 27-30. 10.1093/rheumatology/41.1.27.View ArticlePubMedGoogle Scholar
- Hamanishi C, Tan A, Yamane T, Tomihara M, Fukuda K, Tanaka S: Ossification of the posterior longitudinal ligament. Autosomal trait. Spine. 1995, 20: 205-207. 10.1097/00007632-199501150-00014.View ArticlePubMedGoogle Scholar
- Cheng JCY, Guo X: Osteopenia in adolescent idiopathic scoliosis, a primary problem or secondary to the spinal deformity?. Spine. 1997, 22: 1716-1721. 10.1097/00007632-199708010-00006.View ArticlePubMedGoogle Scholar
- Oostveen JC, Van de Laar MA, Tuynman FH: Anterior atlantoaxial subluxation in a patient with diffuse idiopathic skeletal hyperostosis. J Rheumatol. 1996, 23 (8): 1441-4.PubMedGoogle Scholar
- Papadopoulos SM, Dickman CA, Sonntag VK, Rekate HL, Spetzler RF: Traumatic atlantooccipital dislocation with survival. Neurosurgery. 1991, 28: 574-579. 10.1097/00006123-199104000-00015.View ArticlePubMedGoogle Scholar
- Fielding JW, Hawkins RJ: Atlanto-axial rotatory fixation (fixed rotatory subluxation of the atlanto-axial joint). J Bone Joint Surg Am. 1977, 59: 37-44.PubMedGoogle Scholar
- Hardouin P, Grados F, Cotton A, Cortet B: Should Percutaneous vertebroplasty be used to treat osteoporotic fractures? An update. Joint Bone Spine. 2001, 68: 216-221. 10.1016/S1297-319X(01)00265-2.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.