U p i n t h e A i r : A P a t h o l o g i c a l P u z z l e

A. Diaz, J. Burnham

Central Michigan University's Leonard Lieberman Laboratory

An isolated pathological specimen, a fused femur and os coxa, has been in the teaching collection at Central Michigan Univers ity for thirty years with no known contextual information. The pathology includes osteoarthritis and total fusion of the acetabular joint at an angle of a pproximately 125°. This would suggest that the individual must have been immobilized in a seated position for an extended period of time. We undertook a st udy to determine age, sex and possible pathologies to explain this condition. Osteometric and morphological analyses indicated that the individual was an adult male. Radiographs reveal that the fusion was complete with no evidence of trauma, though a traumatic dislocation cannot be ruled out. We also c onducted a literature search of diseases that could cause this pathology and we present several likelihoods, which include: rheumatoid arthritis, Legg -Perthes’ disease and septic arthri-tis. We also considered the possibility that this fusion may have been a symptom of an adjacent spinal pathology. Due to the lack of skeletal remains, an ul-timate diagnosis may not be attainable, but we invite opinions regarding other possible causes of this unusual pathology.

A b s t r a c t

The pathologically fused femur and os coxa in the teaching collection of the Leonard Lieberman Laboratory at Central Michigan University has

piqued the interested of students and professors alike for decades because of its complete lack of context or background info rmation. The authors

decided to give this individual the study he deserves. What follows is their dedicated attempt to tell his story and discover his long-kept secrets.

I n t r o d u c t i o n

O b s e r v a b l e P a t h o l o g i e s

The necrosis of the femoral head and ace-tabulum implies that there was a loss of blood, which eventually led to the destruc-tion of the joint. It could have occurred from either a disease or trauma, though a femoral neck fracture is notorious for cut-ting off the blood supply to the head and leading to necrosis. See figure 7.

Ankylosis, by definition, is stiffness or immobility in a joint due to bone fusion. It occurs from disease or trauma, or sometimes both. It can be fibrous or bony, also called false or true ankylosis respectively. In this case, the fusion is described as a bony ankylosis of the hip joint. For such excessive remodeling, Dr. Hartman, D.C. (personal communication) estimates that a 10-year period was needed, though disease could have exasperated the growth. Ankylosis leads to immo-bility and a great deal of muscle tension that misshapes bones. The af-fected femur is heavier and more dense than normal, with a thickened neck (Bennett, 1874). See Figures 8 and 7.

Ank ylosis

The more perplexing aspect of this individual’s mor-phology is the angle of fusion. The leg must have been at this inclination for an extended period of time. The in-dividual’s position during fusion is likely the one that he found the most comfortable and where the majority of his muscles were relaxed (Little, 1843). One likely hy-pothesis is that the individual was seated and leaning over due to a spinal injury, pathological condition or be-cause of the muscle tension associated with ankylosis. See figures 5, 2 and 10.

T h e A n g l e Necrosis

The pubic symphysis is damaged and only the inferior portion

remains; using the Suchey-Brooks Method, an age range of 23-

57 years (Phase IV) was concluded (Haas, 1994). The distal fe-

mur’s epiphysis is completed closed. Furthermore, ankylosis to

this excessive degree does not occur until 25-30 years of

age (Little, 1843). See figure 6.

A g e : A d u l t

The fol lowing features’ morphology were assessed to ascer tain sex: the g reater sciatic notch, the preauricular surface and the three components of the Phenice Method (the ventral arch, subpubic con-cavity and the ischiopubic ramus). See figure 3.

S e x : M a l e

Arthritis is one of the most common pa-thologies found on the skeleton. It is caused in a joint by bone-to-bone motion (Aufderheide, 1998). From it’s presence on the distal femur, one could infer that this in-dividual was at one time, mobile and active. It is also a sign that the pathology was not congenital. See figure 9.

Ar thr i t is

Though the bone has no historical context, the condition does.

Many cases of hip joint ankylosis in early medical journals

(dating late 1800s) are similar to this bone. The bone shows no

sign of corrective procedures, which was rare before the 1900s

(Little, 1843). Inconclusive but interesting, this information gives

some insight into a possible contextual age.

Contextual Age: Pre -1950s

-Pronounced spiral line, gluteal line and linea aspera -Defined muscle markings on intertrochanter-ic crest, but little sign of actual crest -No sign of the quadrate tubercle -Presence of third trochanter -Thicken cortical bone; loss of bowing -Anterior-posterior length is greater than the medial-lateral length; oblong shape

-Pronounced facet of lateral epicondyle -Marked rugosity along the edge of the medial supracondylar line/ridge -Arthritic lipping on the condyles and around the epiphyseal rim, seeping into the intercondylar fos-sa -Roughened and grainy patellar surface

-Head fused to os coxa; head of femur lat-erally and superiorly rotated -Neck shortened, thickened and not clearly defined -Displaced intertrochanteric line -Bony overgrowth on greater trochanter; many muscle markings -Defined muscle markings on the lesser trochanter -Deep trochanteric fossa

-Muscle markings/lines of growth around circumference -Excessive hyperostosis in whole area -No sign of acetabular notch; ossified -Spongy bone on posterior portion of what would have been the acetabulum; seeps into the lateral side of obtura-tor foramen -Various abnormal grooves and pits -Indentations on fusion bone area

What follows is a list of noteworthy features that have been observed in the femur and os coxa. Some seemingly abnormal at-

tributes may be due to variation, muscle at attachments, pathology or trauma.

P a t h o l o g i c a l F e a t u r e s

-Posterior inferior iliac spine not well defined -Pronounced, winged anteri-or inferior iliac spine, with ‘square’ indentation -Irregular muscle markings on posterior iliac blade, near crest -Misshapen auricular surface and tuberosity; arthritic lip-ping, including winged ossifi-cation -Weak inferior and anterior gluteal lines -Completely distorted ili-opubic ramus -Thin ischiopubic ramus -Lack of lesser sciatic notch

Proximal Femur

Distal Femur Fusion

Shaft of the Femur Os Coxa

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This study brought up many interesting hypotheses about the pathologi-

cal remains from the laboratory, but it is the authors’ contention that the

fusion was a result of a traumatic dislocation and its subsequent arthritis.

Muscle tension from this ankylosis forced the man to bend forward,

leaving his femur at the angle it was eventually fused at. This study’s

main obstacle was the lack of uniform osteological adjectives; the au-

thors hope to one day rectify this. In the future, further analyses on mus-

cle attachments are planned as well as trying to give the bone more con-

text by finding its origin. Test cases such as this are vital to pathological

study to remind those that study it to never abandon the basics: studying

the bone’s morphology and completing an in-depth literature review.

C o n c l u s i o n

Possible Diagnosis

Additional causes of bony ankylosis, noted by physicians in the 1800s, to be gout, syphilis, tuberculosis, coxagia, typhus, synovitis or scarlet fever (Gross, 1882 and Little, 1843). Hemophilia and diabetes were also discussed over the course of this study.

A vertebral pathology would explain

the angle of fusion if it was severe

enough to force the individual to lean

over. If this fusion was related to dis-

ease, it’s possible that it spread from

the spine. The examples given are as-

sociated with hip disease and ankylo-

sis.

Ankylosing Spondylitis, Pott’s Deformity and Spinal Kyphosis

An inflammatory disease of the

joints and connective tissues.

-Leads to bony ankylosis

(Gross, 1882)

-Dislocation/subluxation

often occurs because of

tension in muscles

-Onset from 20-50 years

-Also affects spine

Rheumatoid Arthritis

“circulatory disturbances of the

epiphysis (Mann & Murphy,

1990)”

-Avascular necrosis

-Displaced femoral head

-Thickened, shortened fem-

oral neck

-Exuberant bony over-

growth (Ortner & Putschar,

1981)

Legg-Perthes’ Disease

An infection of the joint that

leads to joint destruction.

-Leads to bony ankylosis

and dislocation

-Common from 20-40 year

olds; debilitated older indi-

viduals susceptible

-Effects similar to rheuma-

toid arthritis

Septic Arthritis

Vastus Muscles: extensor of the knee Adductors Longis, Brevis and Magnus: adductor and flexors of the hip -Roughened attachment at linea aspera Iliofemoral Ligament: limits motion and prevents hyper-extension -No sign of point that anchors (Spiral Line) Iliopsoas Tendon: complete flexion of the hip Iliacus Muscle: hip flexor Psoas Major Muscle: hip flexor -Markings at insertion point on lesser trochanter Obturator Externus: outward rotation of the hip Piriformis: outward rotation of the hip -Deep insertion point at the trochanteric fossa

Gluteaus Maximus: extension and outward rotation of the hip Gluteus Medius: abduction and outer rotation of the hip Gluteus Minimus: abduction and inner rotation of the hip -Weak attachment of gluteal muscles on iliac fossa -Robust insertion point for part of the gluteus maxi-mus on third trochanter -Broad and robust insertion site for gluteus mini-mus and gluteus medius muscles on greater trochanter -Increased thickness and rugosity on attachment point for part of gluteus maximus on posterior superior iliac spine Erector Spinae: extends the vertebral column -Abnormal attachment on iliac crest

Sacrotuberous Ligament: motion and stability of the sa-croiliac joint -Poorly defined area of attachment on posterior inferior iliac spine Obturator Internus: outward rotation of the hip -Lack of area where passes through the lesser sciatic notch Gastrocnemius Muscle: flexes leg -Pronounced attachment point on facet of lateral epicon-dyle Gemelli Muscle: involved in outward rotation of the hip -Possibly caused spine posterior to sciatic notch -Deep insertion point in trochanteric fossa

A c k n o w l e d g e m e n t s

We would like to give a special thanks to the individuals that offered us their time, support and expertise. Our gratitude

to Dr. Steven K. Hartman of Mt. Pleasant Chiropractic Clinic who graciously donated his time and clinic x-ray ma-

chine. To Dr. Sergio Chavez, Dr. Rachel Caspari and Dr. Cathy Willermet, all professors of Anthropology at CMU,

we give a special thank you for their seemingly endless assistance. No one could have a better faculty than Central Michi-

gan University.

Almost every muscle attachment area in this bone is abnormal, but those that are excessively accentuated are the following:

M u s c l e A t t a c h m e n t s

Aufderheide, Arthur C., Conrado Martin, and Odin Langsjoen. The Cambridge Encyclo-pedia of Human Paleopathology. New York: Cambridge University Press, 1998. Bennett, Edward H. “Article X: Examples of True Ankylosis.” The Dublin Journal of Medical Science 57 (1874): 489-505. Brothwell, Don R., and A. T. Sandi-son. Diseases in Antiquity; a survey of the diseases, injuries, and surgery of early populations,. Spring-field, IL: C.C. Thomas, 1967. Grant, J. C. Boileau, and James E. Ander-son. Grant's Atlas of Anatomy. 7th ed. Balti-more: Williams & Wilkins, 1978. Gross, Samuel David. A System of Surgery: Pathological, Diagnostic, Therapeutic, and Opera-tive Volume 2, 6th edition. Philadelphia: Henry C. Lea’s Son & Co., 1882.

Haas, Jonathan, Jane E. Buikstra, Douglas H. Ubelaker, and David Af-tandilian. Standards for Data Collection from Human Skeletal Remains: proceedings of a seminar at the Field Museum of Natural History, organized by Jonathan Haas. Fayetteville, Arkansas: Ar-kansas Archeological Survey, 1994. Little, William John. On Ankylosis; or Stiff-Joint: a practical treatise on the contractions and deformities resulting from diseases of the joint. Lon-don: Longman, Brown, Green, and Long-mans, 1843. Mann, Robert W., and Sean P. Mur-phy. Regional atlas of bone disease: a guide to path-ologic and normal variation in the human skeleton. Springfield: Thomas, 1990. Salter, Robert Bruce. Textbook of Disorders and Injuries of the Musculoskeletal System: an introduc-tion to orthopedics, fractures and joint injuries, rheu-matology, metabolic bone disease and rehabilitation.

2. ed. Baltimore: Williams & Wilkins, 1984. Steinbock, R. Ted. Paleopathological Diagnosis and Interpretation: bone diseases in ancient human populations. Springfield: Thomas, 1976. Ortner, Donald J., and Walter G. J. Putschar. Identification of Pathological Conditions in Human Skeletal Remains. Washington, D.C.: Smithsonian Institution Press, 1981. White, T. D., and Pieter A. Folkens. Human Osteology. 2nd ed. Toronto: Academic Press, 2000. Wigley, Fredrick. “Case Rounds - Case Round #4.” The Johns Hopkins Arthritis Cen-ter.

References

“Ankylosis of this joint cannot exist, even in a comparatively slight degree, or for any length of time, without being followed by more or less distortion of the pelvis and loins, in the form of com-pensating curves; the muscles of the thigh and leg become waster and rigid: and the knee is eventually rendered stiff and useless. (Gross, 1067)

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