HUMAN OSTEOLOGICAL METHODS CHRONIC DISEASES. Version 19/12/2011. Manual til kroniske sygdomme ADBOU 1

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1 HUMAN OSTEOLOGICAL METHODS CHRONIC DISEASES Version 19/12/2011 Manual til kroniske sygdomme ADBOU 1

2 Table of content Chronic diseases 1. Leprosy 2. Syphilis 3. FOS 4. Tuberculosis Manual til kroniske sygdomme ADBOU 2

3 Chronic diseases 1. Leprosy Baggrund Infektionssygdommen spedalskhed forårsages af bakterien Mycobacterium leprae. Sygdommen, der fandtes i Østen og Mellemøsten før Kristi fødsel, spredtes til Sydeuropa og op gennem Europa med Romerne og nåede til Vesteuropa med folkevandringerne i 400- og 500-årene e.kr. De analyser, der foreligger på skandinavisk skeletmateriale, indikerer, at sygdommen fandtes i Norden fra denne tid (Arcini og Artelius, 1993), men den gængse teori er, at spedalskhed først blev bragt til Danmark og Norden med vikingerne fra deres handels- og plyndringstogter i Europa omkring år Spedalskhed var dog med sikkerhed en udbredt sygdom i middelalderen. Fra midten af 1200-tallet oprettedes Skt. Jørgensgårde, der var spedalskhedshospitaler, hvor man forsøgte at isolere de syge. Isoleringen af de syge var et effektivt middel, og sygdommen var, i begyndelsen af 1500-tallet, hvor der fandtes i alt 31 hospitaler af denne type i Danmark, næsten udryddet. Spedalskhedssmitte sker ved indånding af bakterien, kontakt via hud eller indtrængen gennem slimhinder. Bakterien formerer sig i kroppens køligste dele i de yderste ekstremiteter og i ansigtet. Påvirkningen af nervetrådene resulterer i, at den motoriske kontrol og følesanser tabes, og herudover forårsager bakterien kredsløbsforstyrrelser. Når immunforsvar svækkes, kommer sygdommen i udbrud, hvilket resulterer i, at den smittede ikke mærker kulde, varme og skader og således pådrager sig sår og sekundære infektioner udefra. Skeletforandringerne findes primært i ansigtsskelettet, hvor der sker en nedbrydning af næsehulens kant, i ganen samt fortil i overkæben i knoglestykket mellem fortænderne og næsehulens åbning; i hånd- og fodknogler, der deformeres og nedbrydes samt i fibula, hvor der dannes exostoser og sker fortykkelse af knoglen, da der dannes ekstra periosteal knoglebelægning. Tibia kan inficeres sekundært, hvor der dannes forandringer på ydersiden. Manual til kroniske sygdomme ADBOU 3

4 Registration The location and scoring guidelines for the seven leprosy related lesions used in the likelihood based diagnostic system are described. It is important to stress that none of the lesions described on their own are sufficient to make a valid diagnosis. Edge of the nasal aperture (edge of the nasal aperture) Location: The vertical part of the edges of the nasal aperture in both sides from the most inferior point on the nasal-maxillary suture to approximately 1 cm lateral of the anterior nasal spine. Scores: /: No information 0: Unaffected: Normal (sharp) edge of the bone lateral to the nasal aperture 1: Affected: Remodeled edge of the nasal aperture (i.e. at least ½ cm of this edge is rounded) The figures below show three faces with changes of the edge of the nasal aperture ranging from none to severe. It is unlikely that the individual illustrated in ill. 1 suffered from leprosy and it is unlike that the individual in ill. 3 did not suffer from leprosy, but this is not only due to the changes illustrated here. The ½ cm criterion for rounding of the edge of the nasal aperture might appear scanty. It was on purpose chosen to be inclusive in the positive state because it will permit scoring of a number of skeletons where the maxilla has been separated from the clavarium ill. 1 ill. 2 ill. 3 Manual til kroniske sygdomme ADBOU 4

5 Anterior nasal spine (anterior nasal spine) Location: The most anterior point of the medial-sagittal plane of the nasal aperture where the two lateral edges meet. Scores: /: No information 0: Unaffected: The nasal spine is pointed 1: Affected: Nasal spine is missing or well rounded and this is clearly a condition acquired during life. If it is even a remote possibility that the rounding of the nasal spine could be post mortem then No information (-) is scored The figures below show three faces with changes to the anterior nasal spine ranging from none (a clearly pointed spine) over a mild affection (a just rounded spine) to severe (a spine which is completely gone). It is unlikely that the individual illustrated in ill. 4 suffered from leprosy and it is unlikely that the individual in ill. 6 did not suffer from leprosy, but this is not due to the changes illustrated here, alone. ill. 4 ill. 5 ill. 6 Manual til kroniske sygdomme ADBOU 5

6 Alveolar process of the pre-maxilla (aveolar process) Location: The anterior part of the upper jaw; basically the bone around the roots of the upper incisors. Scores: /: No information 0: Unaffected: Normal alveolar process 1: Affected: Intra vitam destruction of the alveolar process (i.e. Prosthion has retreated above a straight line connecting the most prominent point of the alveolar process between the lateral incisor and the canine in both sides of the upper jaw) The figures below show three faces with changes of the alveolar process of the pre-maxilla. These range from none to severe. It is unlikely that the individual illustrated in ill. 7 suffered from leprosy and it is unlikely that the individual in ill. 9 did not suffer from leprosy, but this is not due only to the changes illustrated here. ill. 7 ill. 8 ill. 9 Manual til kroniske sygdomme ADBOU 6

7 Palate (palate) Location: The palatine process of the maxilla, i.e. the flat or arched area between the sutura incisivum and the transverse palatine suture not including the maxillary alveolar processes. Scores: /: No information due to poor preservation of the of the relevant bone 0: Unaffected: Normal palate with no or little porosity 1: Affected: Pitted or perforated palate. Over ½ of the palate (palatine process of the maxilla) is porous (i.e. covered by densely packed small holes) and/or the palate is perforated in one or more locations by holes measuring at least 2 x 2 mm. The figures below show three faces with changes the palate. These range from non porosity over clustered small wholes to a large perforation of the palate. It is unlikely that the individual illustrated in ill. 10 suffered from leprosy and it is unlike that the individual in ill. 12 did not suffer from leprosy, but this is not due only to the changes illustrated here. ill. 10 ill. 11 ill. 12 Manual til kroniske sygdomme ADBOU 7

8 Fibula Subperiosteal exostoses (Fibula Subperiosteal exostoses) Location: The medial plane and the inter-osseous crest of the middle two thirds of fibula Scores: /: No information 0: Unaffected: Normal, smooth bone 1: Affected: Two or more rounded, pointed or even jagged exostoses on the middle part of fibula. 2 or more of the exostoses must be at least 2 mm long and the surface must have a bark-like structure for this scoring The figures below show three fibulae with varying degrees of formation of subperiostal exostoses. These range from no exostoses to a fibula that is grossly deformed by subperiostal exostoses. It is unlikely that the individual illustrated in ill. 13 suffered from leprosy and it is unlike that the individual in ill. 15 did not suffer from leprosy, but this is not due only to the changes illustrated here. ill. 13 ill. 14 ill. 15 Manual til kroniske sygdomme ADBOU 8

9 Fibula hypertrophy (swelling) (fibula swelling) Location: The medial plane and the inter-osseous crest of the middle two thirds of fibula Scores: /: No information 0: Unaffected: Normal, smooth bone 1: Affected: Hypertrophy, presence of new-formed porous bone. There is a distinct swelling of the bone surface. The swollen areas have a porous surface and small and even large drainage holes are frequently seen The figures below show three fibulae with varying degrees of hypertrophy. These range from a fibula with no hypertrophy over one showing a limited area with a little hypertrophy to a fibula with extensive hypertrophy. It is unlikely that the individual illustrated in ill. 16 suffered from leprosy and it is unlikely that the individual in ill. 18 did not suffer from leprosy, but this is not due only to the changes illustrated here. In other papers using this approach to study the pathology and epidemiology of osteological leprosy the terms porotic hyperosteosis or general swelling have been used for this lesion. ill. 16 ill. 17 ill. 18 Manual til kroniske sygdomme ADBOU 9

10 5 th metatarsal bone (5 th metatarsal) Location: The metatarsal bone proximal to the little (5 th ) toe. Scores: /: No information 0: Unaffected: Normal bone 1: Affected: Any changes on the bone from mild periosteal reactions on the palmar face of the bone to grossly deformed and shortened bone The figures below show three 5 th metatarsal bones with varying degrees of leprosy related changes. These range from a normal, smooth 5 th metatarsal bone over one that has small irregular exostoses on the palmar surface of the bone to one that is severely shortened and deformed. It is unlikely that the individual illustrated in ill. 19 suffered from leprosy and it is unlike that the individual in ill. 21 did not suffer from leprosy, but this is not due only to the changes illustrated here. ill. 19 ill. 20 ill. 21 Manual til kroniske sygdomme ADBOU 10

11 Fused phalanges (fused phalanges) Location: hand phalanges Scores: /: No information (less than 4 joints preserved) 0: Unaffected: Normal bone as seen in ill : Affected: At least one joint is fused in a hook-like position as seen in ill. 23. Only the 2 nd, 3 rd, 4 th and 5 th phalanges on both hands are scored. Only the joints between proximal and intermediate and between intermediate and distal hand phalanges are scored. In total that adds up to a maximum of 16 joints, of which 4 must be preserved to score this trait. ill. 22 ill. 23 Manual til kroniske sygdomme ADBOU 11

12 2. Syphilis Baggrund Treponematose er betegnelsen for de fire sygdomme pinta, yaws, bejel og syfilis, der alle enten er forårsaget af den samme mikroorganisme Treponema pallidum eller er forårsaget af fire forskellige mikroorganismer, som ved mutation har ændret sig fra den oprindelige treponemabakterie. Sygdommene er begrænset forskelligt geografisk, klimatisk og sociokulturelt og forårsager hermed forskellige symptomer. Syfilis er en seksuelt overført sygdom, men smitten kan overføres i fosterstadiet. I dette tilfælde fører sygdommen til abort, dødfødsel eller medfødt syfilis. Syfilis er den eneste af de fire typer, der findes overalt i verden. Det formodes, at være den sygdom vi kender i Danmark fra slutningen af middelalderen og frem 1. Skeletfund tyder dog på, at en treponematose type var til stede i tidligere perioder. Knogleforandringer, der minder om syfilis er observeret, men er mindre voldsomme, og angriber sjældent kraniet, som ved syfilis. Hos den smittede udvikles den klassiske syfilis i tre stadier 2 over flere år, hvor bakterien skiftevis er aktiv og inaktiv. Skelettet menes at blive påvirket i 1,5-20 % af syfilistilfældene, og af disse udgør forandringer i kraniet, omkring næsehulen samt i skinnebenene ca. 70 %. Knoglepatologien viser sig i kraniet som små afrundede ormehuller, der remodellerer og efterlader et arret udtryk. Herudover ses dybe nekroser med en diameter på 1 1½ cm, der har skarpe eller afrundede kanter. I remodelleringsfasen dannes knogle henover nekrosen, der får et stjerneformet udtryk. Til slut efterlades den ophelede knogle med et bulet udseende. I ansigtsskelettet nedbrydes næseåbningens kant, næsehulens knogler og ganen. I det postkranielle skelet sker en fortykkelse på indersiden af tibia, der får den såkaldte sabelform. Ydermere påvirkes især fibula, femur, clavicula, humerus, radius og ulna, men stort set 1 Det er således foreslået at syfilis blev bragt til Europa med Columbus mænd, da de i 1493 vender tilbage fra Amerika efter den første tur til det nyopdagede kontinent. En anden teori fremsætter det syn, at syfilis fandtes i den gamle verden før Columbus besøg i Amerika, men beviset for dette syn besværliggøres af, at det er vanskeligt at skelne syfilis fra andre sygdomme i denne tidlige historiske tid, der alle betegnes som spedalskhed. Meget tyder dog på, at nogle typer treponematose fandtes i både den ny og gamle verden før 1500, hvilket isolering af DNA-strengen for treponematose bakterien har bekræftet (Aufderheide og Rodríguez-Martín, 1998; Ortner, 2003). 2 Primærstadiet viser sig, efter en inkubationsperiode på nogle uger, som sår på kønsorganerne. Sekundærstadiet udvikles mellem to og 10 år efter smitte. Her spredes bakterien i kroppen via blodbanerne og forårsager hudkløe samt læsioner i hud og slimhinder. Tertiærstadiet eller slutstadiet af syfilis er karakteriseret ved lokal vævsødelæggelse i bl.a. hjerte, blodkar og centralnervesystemet samt skeletinvolvering. Manual til kroniske sygdomme ADBOU 12

13 alle postkranielle knogler kan vise sygdomsrelaterede forandringer. Registration Ossa frontales, parietales and occipitales (frontal bone external surface) (parietal bone external surface) (occipital bone external surface) Scores: /: Less than half of the bone is preserved. 0: Unaffected: Normal bone as seen in ill : Affected: An area with wormholes that covers more than 2 cm 2 (ill. 25) or at least two necroses are present (ill. 26). Remodelling with or without new lesions can also be present (ill. 27). Ill. 24 Ill. 25 Ill. 26 Ill. 27 Manual til kroniske sygdomme ADBOU 13

14 Clavicula and ulna (proximal half of the diaphysis) Scores: /: Less than half of the bone is preserved. 0: Unaffected: Normal bone as seen in ill. 28 and 31. 1: Affeted: At least ¼ of the relevant bone is swollen with a bumpy appearance and shows a smooth or microporos surface (ill. 29 and 32). In young individuals necroses can be present both as active lesions and in a remodelled stage (ill. 33). Ill. 31 Ill. 28 Ill. 29 Ill. 32 Ill. 30 Ill. 33 Manual til kroniske sygdomme ADBOU 14

15 Ulna Olecranon process and Humerus - epicondyl Scores: /: Less than half of the relevant part of the bone is preserved. 0: Unaffected: Normal bone as seen in ill. 34 and 37. 1: Affected: The bone has a worm eaten appearance (ill. 35, 36 and 38). Later on irregular deep necroses will occur and most of the joint will be destroyed. Ill. 34 Ill. 37 Ill. 35 Ill. 38 Ill. 36 Manual til kroniske sygdomme ADBOU 15

16 Humerus and femur (distal 1/3 of bone, posterior) Scores: /: Less than half of the relevant part the bone is preserved. 0: Unaffected: Normal bone as seen in 39 and 42. 1: Affected: The relevant bone is swollen with a slightly rough appearance (ill. 40 and 43). In young individuals necroses can be present both as active lesions and in a remodelled stage (ill. 40). In some cases the entire bone can be swollen (ill. 41 and 44). Ill. 39 Ill. 40 Ill. 41 Ill. 42 Ill. 43 Ill. 44 Manual til kroniske sygdomme ADBOU 16

17 Tibia Scores: /: Less than half of the bone is preserved. 0: Unaffected: Normal bone as seen in ill : Affected: New bone formation on the medial side crooks the bone and forms sabre shin (ill. 46). Later on the entire bone will get a swollen and bumpy appearance (ill. 47). In young individuals necroses can be present both as active lesions and in a remodelled stage (ill. 48). Ill. 45 Ill. 46 Ill. 47 Ill. 48 Manual til kroniske sygdomme ADBOU 17

18 3. Focal Osteolytic Syndrome (FOS) Baggrund Denne patologiske tilstand er nylig erkendt og dette ind til videre kun med sikkerhed i dansk middelalderlig skeletmateriale 3. Knogleforandringerne kan forekomme i alle skelettets knogler, og læsionerne findes både i det kompakte og det trabekulære (spongiøse) knoglevæv, enten som runde eller aflange osteolytiske (knoglenedbrydende) forandringer. Læsionerne optræder enten uden knogledannelse langs kanterne eller med knogledannelse langs kanterne, hvilket viser sig som en rand af nydannet knogle. Den sidstnævnte type læsion bekræfter tilstandens patologiske natur, idet individet må have været i live, da dannelsen af knogle i tilknytning til læsionen skete. De patologiske forandringer i forbindelse med fokal osteolytisk syndrom kan ellers let forveksles med post mortale forandringer pga. bl.a. planterødders omdannelse af knogle i jorden. Da sygdommen ikke er beskrevet eller kendt i moderne medicinsk forskning, kendes intet til den patogene agent, der ligger bag syndromet, eller hvordan syndromet påvirker den smittedes væv og organer. Ud fra registreringer af skeletter med de patologiske forandringer kan prevalsen af smittede med fokal osteolytisk syndrom ikke direkte konkluderes, da sygdommen nok som andre kendte knoglepatologiske sygdomme ikke har 100% knogleinvolvering. De analyser, der indtil nu er udført på skeletmateriale med sygdomsforandringerne, viser dog at hyppigheden af læsioner ikke er ens hos danske middelalderlige skeletpopulationer med forskellig geografisk placering, datering og forskelle i den socioøkonomiske baggrund for de gravlagte. Endvidere er læsioner med forbindelse til fokal osteolytisk syndrom observeret i tyske forhistoriske skeletter, svenske middelalderskeletter, samt muligvis hos amerikanske indfødte dateret til 1600 tallet og jordanske skeletter dateret til ca f.kr. 3 Den patologiske natur for syndromet er første gang erkendt af Jesper Boldsen og Ulla Freund i skeletsamlingen ved Syddansk Universitet blandt skeletter fra den tidlig middelalderlige ødekirke Nordby beliggende i Viby ved Århus. En registrering af sygdommen i flere middelalderlige skeletpopulationer er efterfølgende blevet igangsat, hvilket har dannet grundlag for en beskrivelse af de patologiske forandringer (Pedersen, 2008). Manual til kroniske sygdomme ADBOU 18

19 Registration Substantia compacta. The flat bones of the calvarium and facial skeleton and the diaphyses of the humerus, ulna, radius, femur, tibia and fibula. More than half of the cortex has to be preserved for the bone to be registered. The highest score possible is given. Scores: /: Not enough is preserved for the bone to be registered. 0: Unaffected: Normal bone without ante-mortem lytic lesions. If lesions are present they are clearly of post-mortem nature as seen in ill. 49 and 52. 1: Affected: Clear, elongated or round lytic lesion with sharp edges and without any signs of new bone formation as seen in ill. 50 and 53. 2: Affected: Clear, elongated or round lytic lesion with either rounded edges or new bone formation present seen as a rim at the margins and/or in the cavity of the lesion as seen in ill. 51 and 54. The surrounding bone tissue can be affected seen as either new bone formation and/or bone resorption. ill. 49 ill. 50 ill. 51 ill. 52 ill. 53 ill. 54 Manual til kroniske sygdomme ADBOU 19

20 4. Tuberculosis Baggrund Infektionssygdommen tuberkulose forårsages af to forskellige bakterier; Mycobacterium bovis, der overføres fra kvæg til mennesker bl.a. gennem mælkeprodukter fra kvæg, og Mycobacterium tuberculosis der overføres mellem mennesker, hvilket overvejende sker via luftveje, hvorved lungerne påvirkes hurtigt efter smitte. Hvis den primære infektion i lungerne ikke heles, kan bakterien overføres til blodbanerne og dermed føres til andre organer og væv. Her kan sygdommen komme i udbrud flere år efter smitte, bl.a. hvis den smittede oplever en periode med svækket immunforsvar, evt. pga. fejlernæring eller andre sygdomme. Tuberkulose har i løbet af de senere år haft en tiltagende udbredelse især i den fattige del af verden, hvor den er en følgesygdom i forbindelse med udviklingen af AIDS hos HIV-smittede. Tuberkulosesmitte fører sjældent til involvering af skelettet, hvilket gør det svært at estimere sygdommens udbredelse i middelalderen ud fra skeletmateriale alene. De to typer tuberkulose, knogle- samt lungetuberkulose, menes dog at have hver sit udtryk i knoglerne. Knogletuberkulose ses i skelettet som nedbrydning og sammenfald i ryghvirvlerne, der skaber en pukkel i ryggen, og herudover ses makroporøsitet i knoglerne, især i de store led. Knogleinvolvering i forbindelse med lungetuberkulose forekommer sjældent men kan ses som dannelse af lungepanser eller pleurapanser, der er forkalkninger af betændelse i lungehinden om lungerne. Ydermere kan der i forbindelse med lungetuberkulose dannes belægninger på indersiden af ribbenene. Registration Tuberculosis can affect every bone in the skeleton, but it seems to prefer the vertebrae and the weight bearing joint. The lesions can be scattered in various bones, or combined in a single joint. Symmetrical lesions where a joint or bone is affected in both sides of the skeleton are rarely seen. The manual serves as a collection of the most likely affected areas and the presence of a single lesion is thus not enough to make a valid positive diagnosis. Due to tuberculosis related pathology the bones may seem to have osteoarthritis, and eburnation in the articular surface can be present in young individuals. Eburnation is NOT scored, it is only a part of the gen- Manual til kroniske sygdomme ADBOU 20

21 eral description of the affected skeletons. Sacro-iliac Location: Facies auricularis of the os coxae and of os sacrum are scored. The lesion is most often located at the posterior half of the joint surface. At least 75 % of each facies auricularis has to be preserved in order to score the area. Scores: /: The bone is missing, or less than 75 % of the bone is preserved. 0: unaffected: Normal smooth bone with no pathological changes as described below. There may be other lesions present related to postmortem damages or other pathological conditions. 1: affected: Presence of a single erosive area or a cluster of pits, which can be more or less grained (ill. 55). The bone surface may be perforated and an oval, circular or coalesced shaped hole is seen resulting in the trabecular bone being exposed. Bony bumps and irregularly, rounded exostoses (ill. 56) can be seen in connection to the holes. The reactive bone growth which causes the exostoses also tends to smoothen out the lesions edges. Manual til kroniske sygdomme ADBOU 21

22 Ill. 55 Ill. 56 Manual til kroniske sygdomme ADBOU 22

23 Knee Location: Femur and Tibia are scored. In children and young people, the described lesions will often occur at the area between the metaphysic and epiphysis. At least 75 % of each of the areas to be scored has to be preserved in order to score the area. Scores: /: The bone is missing, or less than 75 % of the bone is preserved. 0: Unaffected: Normal smooth bone with no pathological changes as described below. There may be other lesions present related to post-mortem damages or other pathological conditions. 1: affected: Presence of a single erosive area or a cluster of pits, which can be more or less grained (ill. 57). The bone surface may be perforated and an oval, circular or coalesced shaped hole is seen resulting in the trabecular bone being exposed. It is important that the edges of the hole and the trabecular bone are sharp and have the same colouration as the surrounding bone, or the lesion will be due to post-mortem changes and not tuberculosis. Often there will be no reactive bone growth in connection to the lesion, except for a slight smoothening of the exposed trabecular bone. Manual til kroniske sygdomme ADBOU 23

24 Ill. 57 Vertebrae, Foramen nutritium Location: All thoracic and lumbar vertebrae are scored. At least 75% of the wall of 50 % of the corpus vertebra has to be preserved in order to score the area. Scoring: /: Less than 75% of the thoracic vertebrae is preserved. 0: Unaffected: The walls of the vertebrae looks normal. 1: Affected: Vertebrae with hypervascularity and foramen nutritium larger than 3 mm are scored. The area around the largest of the foramen nutritium can have a depressed appearance, and the opening of the foramen nutritium will be funnel shaped. Manual til kroniske sygdomme ADBOU 24

25 Vertebrae destruction Location: All vertebrae except the cervical vertebrae and the first five of the thoracic vertebrae are scored. The lesions can be located at the central or anterior part of corpus vertebrae both at the superior and inferior surfaces. The lesions on the vertebrae are the classic location of tuberculosis related lesions. At least 75% of the corpus Vertebrae has to be preserved in order to score the area. Scores: / The bone is missing, or less than 75 % of the bone is preserved. However if a large part of the corpus vertebrae is destroyed due to lesions described below, the corpus vertebrae is scored as 1. 0: Unaffected: The bone does not have any lesions as described below. Manual til kroniske sygdomme ADBOU 25

26 1: Affected: Presence of a single erosive area or a cluster of pits, which can be more or less grained (ill. 58 and 59). The bone surface may be perforated and an oval, circular or coalesced shaped hole is seen resulting in the trabecular bone being exposed. The lesion may expand in the trabecular bone, where it creates a round or bowl shaped cavity. If the cavity gets large enough or more cavities fuse, the vertebrae body can be damaged and collapse This creates a kyphosis at the spine also known as morbus Pott or Pott s disease. ill. 58 ill. 59 Manual til kroniske sygdomme ADBOU 26