HUMAN OSTEOLOGICAL METHODS
CHRONIC DISEASES
Version
19/12/2011
Manual til kroniske sygdomme – ADBOU
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Table of content
Chronic diseases
1. Leprosy
2. Syphilis
3. FOS
4. Tuberculosis
Manual til kroniske sygdomme – ADBOU
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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 1000. 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.
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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:
/:
0:
No information
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
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ill. 3
4
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
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ill. 5
ill. 6
5
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
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ill. 8
ill. 9
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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
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ill. 12
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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
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ill. 14
ill. 15
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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
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ill. 17
ill. 18
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5th metatarsal bone (5th metatarsal)
Location: The metatarsal bone proximal to the little (5th) 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 5th 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
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ill. 20
ill. 21
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Fused phalanges (fused phalanges)
Location: hand phalanges
Scores:
/:
No information (less than 4 joints preserved)
0:
Unaffected:
Normal bone as seen in ill. 22.
1:
Affected:
At least one joint is fused in a hook-like
position as seen in ill. 23.
Only the 2nd, 3rd, 4th and 5th 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
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ill. 23
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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 frem1. 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 stadier2 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.
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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. 24.
1:
Affected:
An area with ‘wormholes’ that covers more
than 2 cm2 (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. 26
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Ill. 25
Ill. 27
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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.
28
Ill. 31
Ill. 29
Ill. 30
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Ill. 32
Ill. 33
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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
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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
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Tibia
Scores:
/:
Less than half of the bone is preserved.
0:
Unaffected:
Normal bone as seen in ill. 45.
1:
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
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3. Focal Osteolytic Syndrome (FOS)
Baggrund
Denne patologiske tilstand er nylig erkendt og dette ind til videre kun
med sikkerhed i dansk middelalderlig skeletmateriale3. 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. 3000 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).
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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
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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-
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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
mortem
present
damages
or
related
other
to
post-
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.
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Ill. 55
Ill. 56
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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.
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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.
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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.
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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
Manual til kroniske sygdomme – ADBOU
ill. 59
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