View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Springer - Publisher Connector Eur Spine J (2007) 16:1129–1135 DOI 10.1007/s00586-007-0342-x ORIGINAL ARTICLE Diffuse idiopathic skeletal hyperostosis in ancient clergymen J. J. Verlaan Æ F. C. Oner Æ G. J. R. Maat Received: 28 November 2006 / Revised: 24 January 2007 / Accepted: 26 February 2007 / Published online: 28 March 2007 Ó Springer-Verlag 2007 Abstract Diffuse idiopathic skeletal hyperostosis (DISH) adults was 36.8 years. Seventeen adult individuals (40.4% is a common but often unrecognized systemic disorder of all adults), displayed ossifications of at least four con- observed mainly in the elderly. DISH is diagnosed when tiguous spinal levels and/or multiple enthesopathies of the the anterior longitudinal ligament of the spine is ossified on appendicular skeleton and were therefore, assigned the at least four contiguous spinal levels or when multiple diagnosis DISH. The mean age of these individuals was peripheral enthesopathies are present. The etiology of 49.5 ± 13.0 years. In at least three of these individuals, DISH is unknown but previous studies have shown a strong DISH had led to extensive ossification and subsequent association with obesity and insulin-independent diabetes ankylosis of axial and peripheral skeletal structures. In this mellitus. DISH can lead to back pain, dysphagia, mye- population of (presumably) clergymen and high-ranking lopathy, musculoskeletal impairment and grossly unstable citizens, DISH was observed in unusual high numbers at a spine fractures after minor trauma. In archeological studies relatively young age. Some of the examined cases suggest a high prevalence of DISH has been demonstrated in that DISH may be a seriously incapacitating disorder when ancient clergymen. The present study describes the path- the more advanced stages of the disease have been reached. ological changes of human remains excavated from It is hypothesized that ‘‘a monastic way of life’’ can pre- the abbey court (Pandhof) in the city of Maastricht, The dispose to DISH. Present demographic trends in obesity Netherlands. Human remains of 51 individuals buried and diabetes mellitus as potential co-factors for the between 275 and 1795 CE were excavated and examined. development of DISH warrant further study to investigate The remains were investigated according to a standardized its future prevalence. physical anthropological report and individuals demon- strating ossification of spinal ligaments and/or multiple Keywords Diffuse idiopathic skeletal hyperostosis Á peripheral enthesopathies were included in the study group. Osteoarchaeology Á Paleopathology Á Spinal ankylosis The authors reviewed all available material and after reaching consensus, each abnormality found was given a diagnosis and subsequently recorded. After examination, Introduction 28 individuals were considered to be adult males; 11 adult females; three adults of indeterminate sex and nine indi- Diffuse idiopathic skeletal hyperostosis (DISH) is a com- viduals were of sub adult age. The mean age at death for mon but often unrecognized systemic disorder observed mainly in the elderly [1]. DISH is diagnosed when ossifi- cation of the anterolateral aspect of the anterior longitu- J. J.Verlaan (&) Á F. C. Oner Department of Orthopaedics, University Medical Center Utrecht, dinal ligament is present on at least four contiguous spinal Heidelberglaan 100, 3584 CX Utrecht, The Netherlands levels without involvement of the intervertebral disk space e-mail: [email protected] and/or apophyseal (facet) joints [2]. The disease can manifest itself also at extraspinal locations leading to G. J. R. Maat Barge’s Anthropologica, Leiden University Medical Center, peripheral entheseal ossification and bony spurs [3, 4]. Leiden, The Netherlands Studies investigating the demographic characteristics of 123 1130 Eur Spine J (2007) 16:1129–1135 DISH have found a prevalence ranging from 2.9% in the The ‘‘Onze Lieve Vrouwe’’ Church (OLVK; Church of Korean population, to 10.0% in a Caucasian population Our Lady) in Maastricht is one of the oldest churches in with a male/female ratio of 2:1 in most investigations [5, The Netherlands with some parts of its structure dating 6]. All papers pertinent to DISH demonstrate a consistent back to the eleventh century [24]. It was built on the and marked increase of the disease with advancing age [7]. foundation of an early medieval Merovingian Church, Its etiology is unknown but several publications have found which in turn was built on the remains of a Roman fortress. correlations with metabolic disorders, especially high body When an architectural rearrangement was planned for its mass index and insulin-independent diabetes mellitus [8– garden, the abbey court (‘Pandhof’) of the OLVK was 10]. The difference in prevalence between ethnic groups temporarily available for archaeological investigation. also suggests genetic factors being involved in the etiology Following initial fieldwork it was discovered that part of of the disorder [5, 6, 11]. the ‘Pandhof’ had been used as a burial place (Fig. 1)[25]. From paleopathological studies it becomes apparent that After excavation and archaeological investigation was DISH is not a disorder from recent history. Its features have completed, the human remains of 51 individuals were sent for example been demonstrated in the excavated remains of to our department for physical anthropological examina- an Italian female individual who lived six centuries CE tion. During this examination, an unusual high number and (Common Era); in the Egyptian pharaoh Ramses II (1302– particularly strong expression of DISH was noted in the 1213 BCE); and in hominid specimens as old as the skeletal material with some individuals demonstrating se- Neandertal Shanidar I living approximately 50,000 years vere ossification of spinal ligaments and multiple periph- ago [12–15]. Signs of DISH have even been shown in eral enthesopathies. In this study individuals with DISH are dinosaurs [16]. Although the radiological manifestations of presented to demonstrate its high prevalence and describe DISH can sometimes be quite dramatic, displaying exten- some aspects of the wide range of abnormalities resulting sive ossification of ligamentary structures, its clinical from the disease. Furthermore, three cases are highlighted symptoms are usually relatively mild [17]. Some authors to demonstrate some of the specific musculoskeletal have described severe dysphagia, progressive airway pathology. obstruction, obstructive sleep apnea and difficult endotra- cheal intubation due to anterior cervical bony protuber- ances while others have noted minor stiffness or mild pain Methods when the abnormalities were located in the thoracic or lumbar spine [18, 19]. The presence of DISH has been An archaeological investigation of the Pandhof site was associated with unstable spinal fractures, often complicated performed and the artifacts found were dated by stratigra- by permanent neurological deficit, following minor trauma phy (dating based on geological superposition of artifacts), [20, 21]. Still other studies could not confirm these findings and/or seriation (dating using the change of ‘fashion’ of and concluded that DISH is a strict radiological observa- artifacts over time to estimate the year of burial). After tion without clinical relevance [22, 23]. Presently, medical completion of the archaeological examination the exca- or surgical intervention is considered necessary for symp- vated human remains were packed individually and sent to tomatic treatment in rare cases only. our department. After the bones of each individual were Fig. 1 Photograph of the excavation at the Onze Lieve Vrouwe Kerk in the city of Maastricht 123 Eur Spine J (2007) 16:1129–1135 1131 reconstructed, the joints were articulated and the human of indeterminate sex and nine individuals were of sub adult remains investigated according to a standardized physical age. The mean age at death of adults was 36.8 ± 15.9 years, anthropological report [26]. For all excavated individuals, with a range of 20–69 years, based on N = 38 comprising of attempts were made to assess sex, age at death and stature. 27 males, eight females and three individuals of indeter- Subsequently, they were examined for pathological con- minate sex. Seventeen adult individuals (17/42 = 40.4% of ditions and individuals demonstrating spinal ligament the adult population; ten males, five females and two indi- ossifications of four or more contiguous vertebral levels viduals of indeterminate sex) displayed key features of and/or extensive peripheral enthesopathies were included DISH (ossification of spinal longitudinal ligaments without in the study group [3]. Two authors reviewed all available involvement of disk space/facet joints and/or the presence material and after reaching consensus, each abnormality of multiple peripheral enthesopathies) and were included in found was given a diagnosis and recorded. Furthermore, all the study group. The individuals diagnosed with DISH had archaeological records were retrieved to obtain information been buried in the interval 450–1795 CE. The mean age of about estimated year of burial and possible clues to social these individuals was 49.5 ± 13.0 years with a range of 23– status or identification. 69 years. Table 1 lists the demographic characteristics of individuals from the study group and their specific patho- logic conditions. Results Three individuals, from grave 25, 43 and 44