65
STUDIES IN CALIFORNIA PALEOPATHOLOGY:
IV. AN ACHONDROPLASTIC DWARF FROM THE AUGUSTINE SITE (CA-Sac-127)
by
J. Michael Hoffman
with a radiographic interpretation by Dr. James 0. Johnston
66
67
INTRODUCTION
That dwarfs, especially the achondroplastic variety, have occupied a promin-
ent place in paleopathological studies for many decades cannot be denied. Numerous
reports of prehistoric examples of dwarfs in general and achondroplastics specifically,
represented by indirect artistic and/or direct skeletal evidence, have been summarized
for both the Old World (Brothwell 1967; Janssens 1970; Ruffer 1921; Warkany 1971;
Wells 1964, et al.) and the New World (Coe 1965; Corson 1972; Linne 1943; Proskouria-
koff 1949; Snow 1943; Thompson 1970; and von Winning 1974, among others).
Although the New World contains as many if not more artistic representations
of dwarfs than the Old World, the latter has provided us with the majority of skeletal
remains that can be considered prehistoric achondroplastics. Snow's (1943) review of
prehistoric Native American dwarf skeletons indicates that this phenomenon was
probably not so rare, given the number of specimens described. But as Brothwell
(1967:434) notes, all specimens have been lost except for the two Moundville, Alabama,
individuals reported in detail by Snow himself (1943).
There has recently been brought to light a previously unreported prehistoric
achondroplastic dwarf from central California, now housed in the collections of the
Lowie Museum of Anthropology (LMA), University of California, Berkeley. Since this
individual apparently represents only the third extant prehistoric achondroplastic
dwarf skeleton from the New World, a full description, interpretation and comments
bearing upon its importance are presented herein. This paper, then, represents the
detailed reporting of this specimen first mentioned by Hoffman (1975: 3) in a prelim-
inary note.
Provenience
The specimen (LMA catalogue #12-6670) was excavated in 1938 by a field
crew from Sacramento Junior College under the overall direction of Prof. J. B. Lillard.
The locality, known as the Augustine Site (CA-Sac-127), is generally located one mile
south of Sloughhouse, Sacramento County, California, or about 18 miles southeast of
the city of Sacramento. The Augustine Site was initially excavated in 1933 by Sacra-
Inaento Junior College which continued archaeological work at the site every year until
1938. Although a great deal of skeletal material was recovered during this period of
time, the records relating to the individual under discussion are virtually nonexistent.
The original field notes from the excavation (assembled by Franklin Fenenga in 1941
and now in the possession of the Archaeological Research Facility, UC-Berkeley, as
MIs. 42) make no mention of the recovery of a dwarf except as the title for a photograph
Of the post-excavated, articulated bones at the .end of the manuscript, "Sac-127. Final
burial from 1938 diggings. 12-6670. " An examination of all the written notes from the
1938 field season fails to disclose any additional information, except for a comment
dated May 21, 1938, by Lillard himself, "We found holes (test pits) made five years
ago and excavated a few fragmentary and one well-placed burial" (Ms. 42, p. 169). We
68
cannot, however, assume this was the dwarf, even though May 21 was the last date in-
cluded in the field notes. The end result is the inability to assign any chronological
placement for the dwarf based on direct archaeological associations. However, two
other indirect lines of evidence may help in the dating problem.
As reported by Grady (1969), the original burial complex at the Augustine
Site excavated by Sacramento Junior College during the 1933-1938 field seasons has
been associated with the Late Horizon, Phase II (A. D. 1500-1800), of the central
California archaeological sequence. Although we have no definitive documentation to
support this, we might presume that the dwarf is part of that original complex. Addi-
tionally, an examination of the general condition of the dwarfed skeletal remains is
consistent with other known Late Horizon material from the Augustine Site (Dr. James
Bennyhoff, personal communication), and not with more recently excavated material
from the Rooney Extension (CA-Sac-127R) which supposedly comes from Middle Horizon
components of the site (Grady 1969). We may very tentatively, then, posit that the
dwarf is from the Late Horizon and not the Middle Horizon as originally reported
(Hoffman 1975: 3). We must note, almost parenthetically, that since this specimen is
so unique and valuable no destruction of the skeleton proper can be justified for
absolute dating purposes at this time.
'Rediscovery' of the dwarf in the Lowie Museum
It is difficult to ascertain the history of this skeleton after its arrival at the
Lowie Museum in the 1940's. Original cataloguing information indicates that it was
recognized as a dwarf at the time of accession but it apparently elicited no great interest
for it was never, to our knowledge, formally reported in any publication. Discussions
with museum personnel and other osteologists who have worked with the museum's
skeletal collections indicate that various people knew of its existence at intermittent
times over the last decade or more; given this knowledge it is still difficult to under-
stand why such a unique and valuable individual was never formally reported. In
addition, at some point in the past (probably since its arrival at the Lowie Museum),
the individual was reconstructed and fully articulated, possible for display purposes.
When the skeleton was first brought to my attention in early spring, 1975,
the results of the prior reconstruction were still quite evident. Individual plaster inter-
vertebral discs had been made and the entire vertebral column glued together into four
or five major segments. Various reconstructions were done in plaster, for the entire
right innominate, portions of both femora and other miscellaneous areas. The three
major long bones of each extremity were glued together in approximate anatomical
position, as was the complete, partially reconstructed pelvis and the partial left tarsus.
Before the morphological examination and description was begun, the skeleton was
completely disarticulated by gently separating the vertebral elements from their plaster
discs and soaking all glued parts in acetone to loosen and remove the polyvinyl-based
cement. The reconstructed portions of the individual bones were left intact; these show
up in the photographs as pure white areas and in various radiographs as solid radiopaque
densities.
69
MOBRPHOLOGICAL DESCRIPTION
In the following descriptions the general outline presented by Snow (1943) is
followed; this aids the comparative study of the Moundville and Augustine dwarfs. They
are, in general, very similar.
Cranium
The cranial vault is a large, rounded structure overshadowing a small face.
Snow's (1943: 14) description of "bulging" is an apt designation. There is mild lamb-
doid flattening, probably postural in origin. The cranial shape in norma verticalis
most closely approximates the sphenoid category (Comas 1960, after Martin). Suture
patterns vary from simple to complex. All sutures are intact exocranially except for
the sagittal, of which only a one centimeter remnant remains anteriorly, and the left
occipitomastoid which is obliterated. The frontal is high and broad with a very slight
slope above the continuous brow ridges which are joined at a moderately developed
glabellar eminence; there is moderate frontal bossing. The parietals are large with
medium bossing, weakly developed temporal lines and a very small parietal foramen
on the right side. The parieto-occipital articulations bilaterally are low when viewed
laterally and give an appearance of a flattened (side to side) occipital.
The occipital has a good curve from lambda to opisthion. The nuchal region
is moderately rugged with a broadly-thickened area in the region of the external occipi-
tal protuberance. The external occipital crest is well developed anterior to the inferior
nuchal lines and is flanked by deep depressions (attachment sites for the rectus capitis
Posterior minor mm.). The foramen magnum is very small, 3. 0 x 1. 8 cm (a-p x lat),
having an ovoid shape posteriorly and virtually straight borders on either side anter-
iorly. The cranial base around the foramen magnum is rather thick, especially at
basion. The occipital condyles are curved and angled slightly outward posteriorly.
There are bilateral, deep oval depressions situated posteriorly and somewhat lateral
to the condyles; these form sites of secondary articulation of the atlas with the occipital,
there being a bilateral, bulging development of the lateral masses of the atlas, postero-
laterally, which fits nicely these post-condylar depressions. (See description of atlas
below.) The depression on the left shows a smooth, round articular surface on its
anterolateral aspect which corresponds to same on the left lateral mass of the atlas.
The basilar portion of the occipital is markedly foreshortened and terminates in a rela-
tively narrow basilar suture which is obliterated. In addition to being foreshortened,
the cranial base is also compressed from side to side. Therefore all the normally
well-spaced canals and foramina of the cranial base exhibit severe crowding and gen-
erally smaller size; there is nothing unusual about their morphological appearance
except the crowding and reduced size of the individual structures.
The sphenoid shows a reduced body size, shallow yet broad pterygoid fossae,
and broad greater wings; there is a small posterior nasal aperture. The temporals
show small squamae, moderately developed mastoid processes and relatively large,
oval, deep mandibular fossae. The moderately thick tympanic plates enclose oval-
shaped, straight external auditory meati; the latter are devoid of exostoses. The
suprameatal crests are moderately developed and extend to the parietomastoid sutures;
zygomatic processes are rather thick. The zygomatic bones are also relatively thick
and show moderate anterior and lateral projections.
The suborbital fossae are deep with shallow lateral notches. The intact
nasal bones are flat, short and broad with a transverse depression running across their
middle, thus giving a concave appearance when viewed laterally. The left nasal sill is
fairly sharp, the right dull; there is a small, partially eroded, nasal spine. The nasal
aperture is short, broad and roughly oval in outline. Alveolar prognathism is pronounced,
while the upper face appears recessed. As Snow (1943: 14) notes, "This, along with the
depressed nasal root, gives the general facial profile a concavity, typical of this type
of dwarf (Dish-faced)."
The mandible is of medium size with a well-formed mentum and moderate
alveolar prognathism. The two genial tubercles are small. There are well-developed
mylohyoid ridges and the mylohyoid grooves are bridged bilaterally. The mandibular
foramina are large. The gonial regions show rather strong muscle markings inside
and outside; there is moderate gonial eversion.
Dentition
The following chart summarizes the maxillary and mandibular dentitions.
Right                                                            Left
a                                 a  a
ad     ad ad ad                      ad ad ad
8    7   6  5A   3--2    1           1  . 2  3  A4  5  6  7  .8
8 7 6 5 4 3 21                       1 2 3 4 5 6 7 8
ad ad      ad ad ad   s            s  a  ad         ad
s                   s
X - tooth lost antemortem
/ - tooth lost postmortem
a - periapical abcess
ad - alveolar dehiscence
s - shovelling
There is good edge-to-edge bite, but it is noted that the maxillary dental arcade has a
slightly smaller transverse diameter overall than the mandibular arcade. There is
mild-moderate alveolar resorbtion of both arcades. The anterior mandibular teeth
show moderate crowding. There are no obvious caries, but slight calculus deposits on
most teeth, and moderate-marked occlusal attrition with secondary dentine formation
on most teeth.
71
Cranial pathologies
There is an elevated, blister-like lesion, three centimeters in diameter, in
the left posterior aspect of the frontal bone, circular to oval in outline. While the inner
table is intact, the elevated outer table has been eroded away in the central portion of
the lesion; the edges are irregular and rough and show very fine porosities extending
1-2 mm from the edge. The cavity of the lesion shows fine porosities throughout its
surface. The cavity is deepest and undermines the outer table in an anteromedial
direction; it is fairly smooth in overall outline except for a small part of its anterior
wall which shows a slight ridge. Just medial to the elevated outer table is a slight,
round depression, ca. 5-6 mm in diameter, which shows a pitted concavity. The major
differential diagnostic possibilities include an epidermoid inclusion cyst or cranial
hemangioma.
Fine to relatively large porosities occur over much of the skull, the most
prominent being in the nasal bones, the posterior parietals and the interparietal part
of the occipital. Those in the latter two areas are generally 1-2 mm in diameter and
expose the diploic bone of the vault.
Dental pathologies are summarized above.
Vertebral column
The vertebral column is intact and contains the normal complement of verte-
brae per region (7-12-5-5); there are no coccygeal vertebrae present. Generally the
thickness of the individual vertebral bodies is normal thus producing a total column
length which probably would have been within normal limits for this population were it
not for the gross pathologies it manifests. Mild to moderate degenerative disc disease
exists throughout, manifested by roughening and erosion of the articular surfaces of
the centra with some spicular bone formation.
The cervical vertebrae are generally unremarkable, with the following excep-
tions. The atlas shows bulging posterolateral extensions of the lateral masses; that on
the left shows a round accessory articular surface on its lateral aspect for articulation
with the cranium (see description of cranium above). These bulging extensions of the
lateral masses form a hood over the sulci for the vertebral arteries as they leave the
transverse foramina and course posteromedially to cross over the posterior arch and
enter the foramen magnum. The tuberosities for the transverse ligament of the atlas
are very prominent, extending well into the vertebral canal and thus possibly restricting
the rotary motion between the atlas and axis. The sixth cervical vertebrae has abifid
transverse foramen on the right side. The seventh cervical has a small articular
facet on the right superolateral surface of the centrum, probably for an anomalous
unilateral cervical rib; correspondingly there is no right transverse foramen, only an
unabridged groove. There is no cervical osteophytosis but the articular surfaces of
the centra are generally concave and have erosions with bony spicules (indicating mild
disc degeneration).
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The first through the eighth thoracic vertebrae are generally unremarkable
except for the concave articular surfaces of the centra and small spicules indicating
some disc degeneration. T9 shows very mild lipping of the antero-inferior border of
the centrum. T10 has mild anterior lipping of both the superior and inferior borders
of the centrum; there is slight collapse of the centrum anterolaterally on the left which
would have contributed to a slight kyphoscoliosis. Tll has a partial wedge-shaped
centrum; there is moderate lipping on the superior border and marked on the inferior,
the latter forming a broad platform with undulating edges. T12 is apparently hypoplastic
(see radiographic interpretation by Dr. Johnston), having a true wedge-shaped centrum;
there is marked lipping of both the superior and inferior borders on the right side which
fuse together anteriorly.
The first lumbar has a partial wedge-shaped centrum with marked osteophytic
growth from the right anterolateral aspect of the superior border; there is mild lipping
of the inferior border. L2 is partially wedge-shaped; L2 and L3 show mild anterior
lipping. The combination of partial wedge-shaped and hypoplastic bodies of T10 through
L2 produces a kyphogisof approximately 900 in the thoracolumbar region. The posterior
surfaces of the centra of L4 and L5 are strongly concave. The inferior border of L5
is tilted upward and backward producing a centrum with a reversed wedge-shape, i. e.,
anterior body thicker than posterior; this, in combination with the angulation of the Si
articular surface (see below) would have produced an extremely exaggerated lumbosacral
lordosis.  Overall there is a decreasing interpediculate space from Li through L5,
accompanied by some increase in the anteroposterior diameter of the vertebral canal:
thus the shape of the canal changes from flat and broad to long and narrow from Li
through L5. See Table 1. The markedly constricted lumbar vertebral canal is further
accentuated by the close approximation of the laminae of the posterior arch.
TABLE 1
Lumbar Vertebral Canal Dimensions, in mm
Interpediculate             Anteroposterior
Vertebra              distance                    diameter
Li                   20                            9
L2                   18                            9
L3                   15                           10
L4                   15                           10
L5                   14                           11
The 5-segment sacrum is virtually uncurved in profile. A portion of the right
auricular surface and adjacent ala are missing (with a partial plaster reconstruction
attempted by previous workers). The sacrum is long and narrow overall with a promin-
ent promontory. When held with the anterior surface in a true vertical position the Si
articular surface slopes backward approximately 450 (or with the Si articular surface
in a horizontal plane the sacrum slopes backward 450); this Si angulation helps produce
73
the exaggerated lordosis noted above. The Si articular surface is also strongly concave
and shows evidence of disc degeneration. The posterior sacral surface is deeply pitted
at attachment sites of the sacroiliac ligaments. The left auricular surface is concave,
especially inferiorly, rather undulating and with irregular borders.
Pelvis
The left innominate is intact; the right is represented only by a portion of the
iliac crest. (We should remember that a right innominate had been reconstructed of
plaster and the entire pelvic structure, innominates plus sacrum, glued together as a
unit by previous workers.) This reconstituted pelvic structure demonstrated a pelvic
inlet having a kidney-shape that was flattened anteroposteriorly with the sacral promon-
tory so far advanced into the pelvic canal that vaginal childbirth would have been pre-
cluded if this were a female.
That this individual probably was a female is attested to by the following
mnorphologic features of the pelvis: a very wide subpubic angle; a wide and broadly-
curved greater sciatic notch; a very long pubis when compared to the ischium; and the
presence of the three female criteria established by Phenice (1969): ventral arc,
subpubic concavity and medial ridge of the ischiopubic ramus.
There is a very prominent pubic tubercle with a sharp spine directed super-
iorly. The left ilium is rather upright with little tendency toward lateral flaring. Of
the three pelvic elements the ilium appears most greatly reduced in size; this is a
general size reduction but appears most marked in the posterior ilium where there is
an extremely small auricular surface. The acetabulum is shallow and ovoid in outline
with a deep acetabular fossa and a somewhat flattened acetabular roof. Muscle attach-
ments sites are well marked. When the sacrum is articulated with the left innominate,
and the latter placed in approximate anatomical position, the sacrum assumes a nearly
horizontal position. This again is part of the complex leading to an exaggerated lumbar
lordosis.
Sternum
The large manubrium, partially damaged, is broad and unfused to the body
(although it is presently glued to the latter). The body is short and broad, especially in
the inferior half, and strongly curved when viewed laterally, thus presenting a convex
anterior surface. All costal notches are deeply impressed. There is no xiphoid.
Ribs
The ribs are generally short and strongly curved, thus reducing the antero-
posterior thoracic diameter. The sternal ends are broad and flaring. There are strong
mnuscle markings.
74
Clavicles
These are essentially normal in appearance, but rugged and perhaps slightly
short. They are well-curved and possess strong muscle and ligament attachment sites.
Scapulae
Both scapulae are intact except for slight, minor damage (the right coracoid
process and portion of the glenoid cavity have been previously reconstructed in plaster).
Although small they are rugged with strong muscle attachment sites. The left scapula
has a deep scapular notch, the right a rather large foramen. The vertebral borders
are moderately convex, with axillary borders moderately concave. The glenoid
cavities are very shallow and roughly teardrop-shape in outline.
Post-cranium
The long bones show the most dramatic disturbances; they are short but with
stout shafts, deformed, and with well-developed muscle markings. The left and right
bones are essentially mirror images (reflecting the generalized, symmetrical nature
of the dysplasia) so that the specific descriptions can be generalized to both bones.
Humeri
Both humeri are intact and can be characterized as having extremely short,
robust shafts and flaring distal ends; their general appearance is similar to those of
the Moundville individuals. The inferior borders of the humeral heads extend down-
ward, thus giving a mushroom appearance to the inferior half of the heads where they
are attached to the anatomical necks. The greater and lesser tuberosities are promin-
ent, separated by a broad, shallow intertubercular groove. The groove, as it continues
down the shaft, is bordered by broad, rugged crests for the attachment of teres major,
latissimus dorsi and pectoralis major muscles along the anteromedial and anterolateral
borders of the upper shaft. The large deltoid tuberosity on the posterolateral border
of the shaft terminates just at midshaft. These large, rugged crests and the deltoid
tuberosity give the proximal shaft a greatly expanded diameter compared to the more
diminutive distal shaft. Additionally, the shafts are bowed in such a way as to present
a concavity (especially in the distal part) when viewed anteriorly which serves to thrust
the whole distal articular end forward away from the shaft.
There are no radial fossae and only small coronoid fossae, the latter on the
right humerus being more deep impressed than the left. The olecranon fossae are
narrow, oval slits, very deep and extending somewhat superiorly. The trochlea are
broad and shallow anteriorly and as they contiinue posteriorly become deep V-shaped
grooves. The capitular surfaces become narrowed inferiorly and generally show less
articular surface thani in normal individuals. The most inferior surface of the capi-
tulum shows a smaill (10 x 6 mimii) shallow oval depressioa in which fits the mredial edge
75
of the radial head when the radius is in the standard anatomical position, 1. e., supinated;
apparently the radial head was dislocated normally, a not uncommon clinical finding in
achondroplasia. The medial epicondyles are prominent, show a constricted base infer-
iorly, and greatly add to the flaring of the distal hurneral extremities.
UTlnae
Both ulnae are present and intact. The olecranon processes are short and
thick with broad superior aspects which taper backward and downward; the anterior spines
are sharp but because of the narrow, restricted opening of the deep olecranon fossae
they do not project very far into the latter structure. This bony obstruction leads to the
inability to fully extend the forearm at the elbow; the carrying angles in the living indi-
vidual are estimated at 1350 on the left, 1300 on the right. The trochlear notch is
sharply divided by a median ridge into a large medial and smaller lateral aspect. The
lateral aspect of the trochlear notch, at a line separating the olecranon and coronoid
components of the notch, presents a discontinuity representing a nonarticular surface.
This discontinuity is a groove which extends posteriorly, and somewhat laterally, to end
in a deep oval pit situated just above the posterior end of the rather flat, poorly defined
radial notch.
The supinator crest is moderately developed on the right ulna, mildly on the
left. Anterior to each crest is an oval depression, well-marked on the right, less on
the left, which Snow (1943: 28) stated was for the origin of the supinator muscle.
Normally only the more posterior aspect of this depression is for the origin of the
supinator. In this instance when the humerus, ulna and radius are all articulated the
depression comes to hold the very prominent radial tuberosity when the radius is brought
into pronation.
The upper 1/4 to 1/3 of the shafts posteriorly possesses a sharp, crest-like
border most prominent superiorly. Generally the shafts are moderately well developed
but with only poorly developed interosseous crests. The medial surface of the distal
shaft shows a prominent, posteriorly curving crest for the attachment of the pronator
quadratus. The posteriorly positioned styloid processes are nearly as large as the
ulnar heads and are separated therefrom by a deep notch.
Radii
The two malformed radii are intact except for the loss of the right radial head
and neck. The circumference of the left radial head is roughly circularly in outline. The
superior articular surface is very shallowly depressed overall with a deeper central
depression which comnprises hardly more than a quarter of the diameter of the head.
Anterolaterally the articular surface angles downward some 45 0  The whole radial head
is angled backward 300 and slightly laterally, the angulation reflecting the direction the
radial neck takes from the main shaft. The neck itself is very short. The large,
prominent radial tuberosity has its greatest diameter in a transverse direction and the
76
prominence is accentuated by the angulation of the neck on the shaft. In the anatomical
position the radial tuberosity projects roughly anteromedially.
The shafts exhibit moderate lateral bowing which again serves to accentuate
the prominence of the radial tuberosity and the angulation of the head and neck. Both
shafts exhibit an anteroposterior flattening with prominent medial and lateral borders in
the second fourth of their length. Distal to this flattening the shafts are prismatic and
gradually expand in size as they progress distally.
The distal ends of the radii seem overly large compared to the short relatively
modest shafts. There are prominent tubercles for attachment of the brachioradialis
and extensor carpi radialis brevis muscles. The ulnar notch is well developed but
shallow. The prominent styloid process serves to give a moderate curve to the distal
articular surface.
Metacarpals
Three metacarpals are present: left second, and right second and third. All
are reduced in length some 15-20% and have thin shafts. The normal longitudinal con-
cavity of the palmar surfaces is rather exaggerated and is especially noticeable in the
distal 1/2 to 1/3 where the curvature becomes greatest. Although the metacarpals are
more curved than normal, it is much more apparent in the palmar concavity than in the
dorsal convexity. This is due in part to the overall shortening of the shaft which accen-
tuates the concavity more because the radius of the concavity is reduced proportionately
more than the radius of the convex dorsal surface.
Femora
Both femora are nearly intact except for the following features which have been
previously reconstructed in plaster: the tip of the left trochanter, the right femoral head
and neck and a small portion of the shaft distal to the lesser trochanter.
The proximal ends are distinguished by the gross development of muscle and
tendon attachment sites and short femoral necks. The left neck-shaft angle is 1350. The
femoral head does not seem overly large given the reduced size of the shaft. As in the
Moundville dwarfs, the femoral heads appear mushroomed along their entire free border
except for a small extension of the head anterolaterally onto the femoral neck. As noted
the femoral neck is short and of relatively small diameter except where the extension of
the head is applied to the neck. The left foveal depression is of normal size and thus
appears relatively large due to the small femoral head upon which it is impressed.
The greater trochanters are markedly developed with very deep trochanteric
fossae. Anteriorly the greater trochanter ends in a well developed tubercle where the
lateral band of the iliofemoral ligament attaches. There is hardly a suggested presence
of an intertorchanteric line which consists of a small inferomedial roughness coming off
77
the tubercle mentioned above. Directly inferior to this tubercle is a shallow, circular
depression about one centimeter in diameter. Posterior to the greater trochanter is a
mildly developed quadrate tubercle which is connected to a greatly overdeveloped lesser
trochanter via the elevated intertrochanteric crest. As Snow (1943: 41) notes, "the
lesser trochanters extend medially as sharply defined finger-like processes. "
Both femoral shafts are mildly bowed posteriorly and roughly quadrilateral
in cross-section with the larger side anterior. Posteriorly the gluteal tuberosities are
mnoderately developed and extend along the posterolateral border of the shaft rather
than swinging medially to join the spiral (pectineal) line at the apex of the linea aspera.
The spiral line itself is mildly developed and extends to where the.linea aspera would
normally begin. There is no true linea aspera, only the posterior surface of the quad-
rilateral shaft. This posterior surface is boundedmedially and laterally by the inferior
extensions of the spiral line and gluteal tuberosity respectively. Inferiorly this pseudo-
linea aspera broadens into the large, concave, distally-flaring popliteal surface.
The distal femoral extremities are large, flaring, malformed structures.
Most notable is the gross over development of the medial condyle (nearly twice the size
of the lateral) which is separated from the lateral by a deep, narrow intercondylar fossa
(slightly wider in its posterior than anterior aspect) which is discernible as much in an
anterior view as a posterior one. The superior aspect of the patellar articular surface
is greatly lengthened and there appears a depression in its uppermost part which is
bordered superiorly by an overhanging lip of bone from the adjacent nonarticular meta-
physis. The normal depression which separates the patellar articular surface into
medial and lateral parts and which continues onto the inferior surface of the condyles
is wholly confined to the superior half of the anterior condylar surfaces. The whole
appearance of the distal extremity one receives is that of the entire condylar end being
rotated 900 antero-inferiorly, thus bringing the intercondylar fossa forward and extend-
ing the patellar surface superiorly. The medial epicondyle is broad and extensive while
the lateral is relatively diminished in size (although fairly prominent in its lateral
projection) with a prominent popliteal sulcus inferiorly.
Tibiae
Both tibiae are wholly intact and are distinguished by very prominent, flaring
extremities and pronounced attachment areas for muscles, tendons and ligaments. As
with the Moundville material the proximal ends of both tibiae show pronounced posterior
retroversion or backward overhaDg. The condylar surfaces are intact with the medial
being larger than the lateral. The medial condyle presents a fairly normal shape (very
slightly concave) but the lateral is strongly convex in an anteroposterior direction such
that the most anterior portion of the lateral condyle faces directly forward instead of
Upward. In both tibiae the superior surface of the lateral condyle is some 2-3 mm
above the superior surface of the medial condyle.
78
The tibial tuberosities are very prominent with the medial end of the left
tuberosity ending in a small dimpled depression. There are well developed oval
articular facets (1 x 1. 5 cm) on the posterolateral aspect of the inferior surface of the
backward overhanging lateral condyle; these are for the fibular heads which fit very
nicely.
The shafts show slight bowing anteriorly, are of normal shape and just
slightly smaller in diameter than normal. The soleal (popliteal) lines on the posterior
surface of the upper shaft are moderately developed. The distal 1/3 of each shaft is
curved very slightly medialward, possibly resulting in a slight varus deformity at the
ankle.
The distal extremities are relatively large with well developed, broad medial
malleoli which appear to extend further medially than normal. The inferior articular
surfaces are smooth with somewhat exaggerated normal undulations; the left has a deep,
conical pit in the middle. The fibular notches bilaterally are well-formed, deep im-
pressions with prominent anterior and posterior boundaries that form tubercles.
Fibulae
Both fibulae are intact and longer than their respective tibiae. They have well
developed heads which are rather normal in appearance. They possess strongly
ridged shafts with very prominent interosseous borders. The well developed distal
extremities have deep malleolar fossae but no obvious lateral malleolar sulci for
peroneal tendons except for very slight impressions on the most inferior part of the
malleoli.
Talus
Only the left talus is present. It is generally unremarkable other than its
slightly reduced size from normal (it would probably fall within the lower range of
normal size for females). The neck appears to be slightly shortened in its anteropos-
terior length. The articular surfaces are of normal arrangement and configuration.
There is a slight porosity and roughening in the central region of the navicular articular
surface which probably represents beginning degenerative joint disease at the talon-
avicular joint.
Calcaneus
Only the left calcaneus is present and like the talus, is rather unremarkable
except for a slightly reduced size. Comparison with other calcanei indicate that the
reduced length is accounted for primarily by reduction of the body, the posterior element
of the calcaneus. The peroneal trochlea is very prominent in its lateral projection.
The configuration of the articular surfaces is within the normal range of variation.
79
Cuboid
The slightly reduced in size left cuboid is of normal configuration. There is
an articular facet on the tubercle for the sesamoid bone sometimes found in the peron-
eus longus tendon.
Metatarsals
Only the left fifth metatarsal remains; it is moderately shortened but of
normal configuration and is not bowed like the metacarpals.
RADIOGRAPHIC INTER PRETATION
The following radiographic description, interpretation and differential diag-
nosis is by Dr. James O. Johnston, Chief, Department of Orthopedics, The Permanente
Medical Group, Oakland, California. Dr. Johnston's time and interest in this case are
gratefully acknowledged and appreciated.
On a lateral projection of the skull, not seen is the typical appearance of
hydrocephaly and frontal bossing that one would like to see for a classical
achondroplastic diagnosis. However, there is a fair amount of hypoplasia
of the basilar portion of the skull, especially through the maxillary area
and basis sphenoid area, suggesting achondroplastic diagnosis. An A/P
projection provides a better case for achondroplasia, with bitemporal bossing
and a small hypoplastic alveolar ridge with a narrow transverse dimension
which frequently results in malocclusion problems when it attempts to
articulate against the normal mandible, which is relatively larger than the
opposing upper maxillary area.
On the film of the mandible, it is indeed larger in its transverse dimension
and shows that this individual did have a malocclusion problem, which is
typical of achondroplasia. The clavicles on the same film are small and
quite compatible with achondroplasia. I cannot really comment on the sternum.
A lateral view of the mandible shows a condylar area which is unusually
short for articulation with the temporal bone. On this same film the ribs
are short and certainly compatible with achondroplasia. Another bone on this
film looks like os calcis and has a short tuberosity compatible with
achondroplasia.
On a lateral view of the cervical and lumbar spine, these appear reasonably
healthy. Of note is the odontoid process which is of good length and is well
articulated with the ring of C-i. This tends to go against a diagnosis of
Morquio' s and the severe spondyloepiphyseal dysplastic dwarfs that might
be considered. The lateral view of the lumbar spine reveals characteristic
posterior concave deformity of the vertebral bodies, producing a "dumb bell"
80
myelogram (if such a study could have been done on this individual). The
disc heights are reasonably well preserved and there is minimal deformity
in most of the vertebrae except for the upper one, which is probably the
portion that articulates with the kyphotic area seen on another film. This-
upper lumbar vertebra (probably L-2) has a definite wedge configuration and
evidence of degenerative spurring because of its closeness to the severe
kyphotic deformity.
. . . a lateral x-ray at the lumbodorsal junction shows a rather severe gibbus
deformity of nearly 900 angulation with a considerable amount of secondary
degenerative spurring in front, as the result of advanced degenerative disc
disease resulting from this kyphotic deformity. This unusual manifestation of
adult achondroplasia has been recorded in the literature, especially in the
type of achondroplastic dwarf having a fair amount of ligamentous laxity and
joint dislocation tendency. This type of gibbus deformity is more typically
seen in the severe epiphyseal dysplastic group such as Morquio's and Hurler's
or in other forms of dwarfism associated with collagen deficiency and result-
ant ligamentous laxity and scoliosis. This atypical vertebra is sometimes
referred to as a hypoplastic vertebra but if one follows the child from infancy
to adulthood it becomes apparent that the kyphosis is progressively develop-
mental, secondary to laxity of the ligaments at the lumbodorsal junction and
not truly a hypoplastic condition of the vertebra; more immediately evident at
birth or shortly thereafter. Also seen on this lateral projection is the
classical concave configuration of the posterior surface of the vertebral body,
also typical of achondroplasia and not seen in pseudoachondroplastic conditions
of the epiphyseal variety.
On a lateral view, the thoracic spine appears quite normal except for some
hypoplasia of the posterior elements, including the facets and pedicles which
seem short and certainly compatible with achondroplasia. The spinous
processes appear unusually long, which I do not fully understand.
An A/P x-ray of the sacrum shows the classical findings of an achondroplastic
type of dwarf, with the obvious restricted distance between the pedicles at the
S-1 level and progressive stenosis (viewed in a caudal direction). This
evidence of spinal stenosis is probably the most diagnostic feature of the
achondroplastic dwarf and tends to rule out all other forms of short-limbed
dwarfism, includingallepiphyseal dyplasias such as Hurler's, Morquio's and
severe forns of pseudoachondropla'stic spondyloepiphyseal dysplasia.
Unfortunately, the lateral projection of the pelvis is difficult to interpret
because good acetabulum detail cannot be seen. However, I can say that the
sciatic notch is fairly sharp due to the inadequacy of the triradiate cartilage
during the developmental years of the individual, typical of achondroplasia.
On this film the fairly hypoplastic appearing scapula has no really specific
81
features on which to comment.
X-ray of the humerus, radius and ulna shows considerable dwarfism, especially
of the humerus. This typical form of achondroplasia, more amplified in the
proximal long bones than in those of the distal bones, is referred to as the
rhizomelic form of dwarfism as compared to the acromelic form seen in the
Ellis-van Creveld syndrome. Also seen is an excellent example of muscle
effect on the humerus, with a dilated diaphyseal area at the mid-shaft of the
humerus where the deltoid muscle tendon inserts, producing a rather rec-
tangular shape to the upper metaphysis, typical of achondroplasia. The
epiphyses appear well structured and strong, ruling out the severe epiphyseal
Pseudoachondroplastic forms of dwarfism. The ulna is short with a slight
degree of bowing. The adjacent radius is more severly deformed, with
considerable distortion of the proximal half due to the abnormal insertion of
the biceps tendon, with a prominent bicipital tuberosity similar to the prom-
inence of the lesser trochanter on the upper end of the femur. The radial
head itself is deformed and strongly suggests that this was partially dislocated
from the capitulum of the humerus, which is not unusual in this condition.
The distal end of the radius and the ulna are transversely oriented, typical
Of achondroplasia and tending against the mucopolysaccharide group and the
severe epiphyseal dysplastic dwarfs.
On x-ray, the femur is quite short with a narrow diaphysis of excellent cor-
tical structure and a flared metaphyseal area on both ends, which is typical
Of achondroplasia. The epiphyses of this bone appear quite normal and well
Structured with good strength, which goes against a diagnosis of a pseudo-
achondroplastic form of dwarfism. There is a normal angle of the femoral
neck to the shaft, which is typical of achondroplasia and tends to go against
the diagnosis of epiphyseal dysplasia such as Morquio's. Prominence of the
lesser trochanter is not atypical for achondroplasia. In fact, all the areas
Of tendinous insertion tend to be prominent and more elevated than in normal
bones. The other bone seen on this x-ray is probably the fibula. Had it been
X-rayed with the tibia one could see the excessive overgrowth of the fibula
as compared to the tibia, which is typical of the achondroplastic dwarf. How-
ever, holding the x-ray of the fibula next to that of the tibia, one can see that
the fibula is longer than it should be (assuming the tube length from the subject
is the same for both of these x-rays and assuming these are bones from the
saine specimen); strongly compatible with a diagnosis of achondroplasia.
X-ray of the tibia also shows the characteristic narrow diaphysis with a
flared metaphyseal area. There is no unusual sloping of the tibial plateau
Of valgus deformity of the ankle mortise as seen in epiphyseal dysplasias,
again pointing strongly toward achondroplastic diagnosis. The smaller bones
Il this x-ray are undoubtedly metatarsal bones, as can been seen by the
styloid process which represents the fifth metatarsal. Again, the pattern is
82
typical of achondroplasia, with shortening and a narrow diaphysis and flared
metaphyseal area with no deformity of the distal epiphyses, suggesting a
strong, normal joint articulation with the proximal phalanx, typical of
achondroplasia. There is no proximal tapering of the metatarsals, which
goes against a diagnosis of the epiphyseal dysplastic group, including Hurler's
and Morquio's.
It is obvious this particular individual was of good, solid structure as far as
functional capability. The individual had good joints, with no evidence of
arthritis, and was obviously able to get around quite well. However, one
thing that may have "done this dwarf in" is the rather severe lumbodorsal
kyphosis, known to cause serious problems of cauda equina compression,
with resultant weakness of the lower extremities and very difficult to treat
surgically by posterior decompression. This individual may have become
very weak in the lower extremities and died as the result of paraplegia.
However, the structure of the bone in the lower extremities is quite good and
does not show any significant degree of disuse osteoporosis that would suggest
paraplegia of any duration. It is possible the individual developed an acute
paraplegia following some relatively minor injury that might well have caused
a neurogenic bladder syndrome, resulting in death from urological complica-
tions.
All in all, you have a good case for a true achondroplastic dwarf. I think we
can safely rule out other forms of dwarfism because of the adequate x-ray
evaluation. I have mentioned the differential diagnostic possibilities and
need go no further. It is most fascinating to go over a case such as this. As
you know, achondroplastic dwarfism is ancient, dating back 5000 years when
dwarfs were known to be part of our society, as described by inscriptions
in Egypt.
DISCUSSION
General characteristics of achondroplasia
Parrot (1878) first coined the term 'achondroplasia' (literally meaning without
cartilage formation) to distinguish disproportionate dwarfism, i.e., dwarfism of the
short-limbed type, from the proportionate type due to rickets. In 1892 Kaufmann
substituted the term 'chondrodystrophia foetalis' to imply a deficient growth of the
cartilaginous epiphyseal growth plate. These were broadly inclusive terms which, as
the understanding of the specific deficit became clearer and other similar entities
became differentiated, gradually took on a very specific meaning. Achondroplasia is
the term in near-universal use today; synonyms include: chondrodystrophia, chondro-
dystrophia fetalis, chondrodystrophic dwarfism, chondrodysplasia fetalis, micromelia,
and achondroplastic dwarfism.
83
Achondroplasia is the most commnon type of dwarfism and has been character -
ized as an "hereditary, congenital, familial disturbance in epiphyseal chondroblastic
growth and maturation" (Aegerter and Kirkpatrick 1975: 101). It is inherited as an
autosomal dominant characteristic whose incidence is variable and ranges from 1 in
every 3000 to 9500 births, with approximately 80% of all live births dying in the first
year (Warkany 1971: 768). If, however, the achondroplastic survives the first year
or two of life he has a relatively good chance of reaching and surviving into adulthood.
During later middle age, though, adult achondroplastics are apt to suffer from the
symptoms caused by pressure upon the spinal cord and nerves (produced by the skeletal
deformities of the vertebral column) with resultant backaches, sciatica, paresthesia
and even paraplegia (Aegerter and Kirkpatrick 1975: 109; Greenfield 1975: 197) with
all the attendant complications of the latter. The mental and sexual development of
achondroplastics is usually normal.
Achondroplasia is characterized as a rhizomelic, micromelic type of dwarf-
ism. This means the dwarfism displays overall short limbs (micromelia) which show
a greater reduction in the proximal segments of the limb (rhizomelia) than in the distal
segments, i. e., in the upper arms and thighs.
As Aegerter and Kirkpatrick (1975:. 102) note, the relative frequency of
achondroplasia is kept rather low because of the high rate of intrauterine deaths of the
malformed fetus and to the difficult parturition in affected mothers because of the dis-
torted pelvic canal. With an autosomal dominant mode of inheritance one would expect
a much higher incidence of this type of dwarfism in the general population. Obviously,
in countries with modern medical facilities this feto-pelvic disproportion can be obviated
through caesarean section delivery of the fetus. In fact, the relative frequency of
achondroplasia is probably higher than expected because of the ability of achondroplastic
mothers to successfully deliver nonvaginally. In effect, then, the contemporarily
reported frequencies of achondroplasia probably reflect a balance between high frequencies
based on an autosomal dominant mode of inheritance and adequate medical care and the
tendency to lowered frequencies based on fetopelvic disproportions, stillbirths and
newborn inviability. The frequency of living achondroplastics in prehistoric times
Would obviously be much lower.
Achondroplasia has been classified as one of numerous skeletal dysplasias
characterized by a disturbance in chondroid production related to an abnormal matura-
tion of growth plate chondroblasts of unknown etiology, i. e., "an idiopathic disturbance
Of growth plate chondroblast maturation" (Aegerter and Kirkpatrick 1975: 101). The
defective division and maturation of these growth plate chondroblasts results in a de-
ficient chondroid lattice and subsequent zone. of provisional calcification. Without an
adequate calcified chondroid latticework, there is little opportunity for normal osteoid
to be laid down (Aegerter and Kirkpatrick 1975: 101-109). The result is a marked de-
crease in the linear growth of endochondral bones. Since the defect is localized in bones
of endochondral origin, actually in the chondroblasts themselves, intramembral and
Periosteal (appositional) bone formation are normal. The apparent abnormalities in the
84
latter osteogenic modes of development are probably the result of their accomodation to-
the malformations of adjacent endochondral bone. As Greenfield (1975: 197) and others
have noted there may be periosteal strips of fibrous tissue which seal the epiphyseal
line and thus negate further growth in a length of the bone. With normal periosteal
ossification, appositional growth at the ends of long bones proceeds faster than epiphy-
seal growth and this, according to Edeiken and Hodes (1973: 65), is what gives these
bones flared extremities. Aegerter and Kirkpatrick (1975: 109), however, imply that
the stress on the epiphysis, in the face of a strong cortex with a deficient underlying
spongiosa, may be the cause of the flaring.
The following account of the histological changes in achondroplastic growth
plates summarizes very well how the disturbed epiphyseal chondroblastic maturation
accounts for the major characteristics seen in this dysplasia.
In the achondroplastic child the iliac-crest growth plate is histologically
nearly normal whereas the growth plate of the fibula, and presumably of all
the long bones, is very deranged. Similarly, bone growth is normal in the
iliac crest and is very stunted in the fibula. Recent work in our laboratory
indicates that the iliac-crest cartilage of achondroplastic patients contains
normal amounts of hydroxyproline and some increase in amounts of galactos-
amine and glucosamine. The proteinpolysaccharides, however, which can
easily be extracted from the iliac-crest cartilage of normal children, are
difficult to extract from the cartilage of many of our achondroplastic patients.
Perhaps the proteinpolysaccharides form large aggregates in the cateilage
of the achondroplastic patients which could interfere with the formation and
resorption of the growth plates more in the long bones than in the iliac-crest,
possibly owing to the differences in cartilage matrix, collagen organization,
and manner of ossification of these two structures.
The cartilage-cell columns in the fibular epiphyseal plates of achondro-
plastic children are disarranged and are separated by wide cartilaginous septa
rich in fibers. Visualized with polarized light, these seem to be collagen
fibers, and with the electron microscope many wide, well banded fibrils are
seen in these septa. This fibrotic cartilage appears to resist erosion by the
invading vascular processes. Presumably, the splittingof the abnormal
proteinpolysaccharide molecules, necessary for calcification and ossification,
proceeds very slowly or is even blocked at some sites. Thus, vascular pene-
tration of the plate and bone formation are very stunted and irregular. On
the other hand, the matrix abnormality in the child with achondroplasia does
not interfere with the type of calcification and ossification found in the iliac-
crest at the termination of the large septa rich in collagen fibers. Here the
process resembles intratendinous ossification, which is normal in children
with achondroplasia as seen by their well developed trochanters, tuberosities,
and epcondyles. Periosteal and intermembranous ossifications are also normal.
Bone forms in all these sites withoutbreakdown of collagen fibers or matrix.
85
Assuming that the two growth plates of the vertebral bodies resemble the
growth plate of the iliac crest in children with achondroplasia as they do in
normal children, the discrepancy between the nearly normal growth in height
of the vertebrae and iliac wings of these patients and the very short bones of
their extremities can be attributed to the different structure of the respective
growth plates.
Several authors have observed that, in children with achondroplasia,
fibrous connective tissue extends from the perichondriumn into the epiphyseal
plate. The hyaline cartilage at the periphery of the fibular plates of some
of our patients contained many collagen fibers which often seemed to extend
through the septa to the metaphysis. Abundant normal periosteal bone spread
out in a funnel-shaped fashion underneath the peripheral margin of the plate
and accounted for the flaring of the metaphysis (Ponseti 1970: 710-713).
Stature increase in achondroplastics is slow after birth and generally termin-
ates prematurely; overall height rarely exceeds 140 cm (55 in), with an average in the
neighborhood of 48-50 in (Warkany 1971: 774). The normal dentitional and other physical
developmental milestones tend to be retarded to some degree. The onset of walking
(at about 20 months) is accompanied by a rapidly developing lumbar lordosis, protuber-
ant abdomen and prominent buttocks (Bailey 1973: 62-64) characteristic of this type of
dwarfism. Good discussions of the developmental growth stages found in achondroplasia
are presented in Bailey (1970, 1973), Langer et al. (1967), and Ponseti (1970), and
need not be recounted here.
Interpretation of bony abnormalities
If we remember that the defect in this skeletal dysplasia directly affects only
endochondral bone, many of the bony abnormalities seen in this individual become
readily understandable. The basic problem in the cranium results from the fact that
most bones of the cranial base (occipital, sphenoid, petromastoid portion of the temporals)
are developed exclusively or primarily from cartilage while the vault and facial struc-
ture (parietals, frontal, temporal squama, nasals, maxillae, and mandible) are pre-
formed in membrane bone. The result is a markedly foreshortened cranial base and
reduction in its overall size accompanied by a recessed upper face and a relatively
large vault which appears to bulge out in all directions over the diminutive base upon
which it rests. There is a general lack of growth along the inter-sphenoid and spheno-
occipital synchondroses. Whereas in the normal individual the four principle epiphyseal
Ossification centers of the cranial base normally close in early adulthood, they may
Ossify in utero or in infancy in achondroplasia. This accounts for the small cranial
base, the decreased diameter of the foramen magnum, the prominent brachycephaly,
and the depressed nasal bridge (Aegerter and Kirkpatrick 1975: 102-103). The brain is
thus obliged to grow upward, forward and outward resulting in the characteristic high,
bulging forehead, and frontal and parietal bossing. The bulging forehead also helps
accentuate the saddle nose (Warkany 1971: 774).
86
Although most authors attribute the presence of the depressed nasal region to
the generally foreshortened cranial base in concert with the bulging forehead, this is
not a wholly satisfactory explanation. The nasal recession actually involves more than
just the flattened nasal bones; it also involves the frontal processes of the maxillae such
that the whole upper face, i.e., the nasomaxillary region, is actually recessed and
flattened. Since the cranial base is not in direct contact with this niasomaxillary com-
plex, its foreshortening probably only contributes indirectly to the problem. A more
specific explanation probably lies in the fact that the ethmoid, upon which the naso-
maxillary region is buttressed (directly and indirectly via the lacrimals) is of endo-
chondral origin, being derived from the cartilaginous embryonic nasal capsule. The
lacrimals themselves ossify from the membrane overlying the nasal capsule and thus
might be expected to be generally reduced also. The probably diminished development
of the cartilage-derived ethmoid, then, appears to be a more direct explanation for the
depressed nasal bridge, albeit aided and accentuated by the bulging frontal bone and a
generally shortened cranial base.
Since the mandible enlarges by both endochondral and intramembranous
ossification (primarily the latter), it shows a relatively normal (although still somewhat
small) development which makes it appear relatively large compared to the diminished
facial structure; this also contributes greatly to the prominent prognathism of achondro-
plastics. These craniofacial features are generally so constant that most achondro-
plastics look like members of the same family (Aegerter and Kirkpatrick 1975: 102-103;
Warkany 1971: 774).
Although the vertebral column is of cartilaginous origin, there is generally
less inhibition of trunk growth than of the limbs (Aegerter and Kirkpatrick 1975: 103).
In fact, the trunk may approach or even reach the limits of normal length. This may be
due, in part, to the complex, intersegmental origin of the individual vertebrae and/or
the overall segmented nature of the vertebral column, resulting in less general depres-
sion of vertebral body growth than if the trunk were composed of a single, longitudinal
element (cf. Ponseti 1970: 712). Generally, though, there is some reduction in vertebral
height, and overall development is usually abnormal to some degree. (Given the fact
that vertebrae do arise from a cartilage model, it is surprising the trunks are not more
malformed.)
In addition to a general reduction in size the vertebrae may be wedge-shaped,
with the development of thoracolunmbar kyphosis as we have seen in this individual. The
posterior vertebral elements are usually more affected, producing a restricted neural
canal which may compress the cord and/or spinal nerves. This is especially likely in
the thoracolumbar region where a gibbus deformity may develop or in the lumbar region
where the interpediculate spaces become progressively narrow. See Caffey (1958).
Although this particular prehistoric Californian manifests this progressive diminution of
the interpediculate distance, there may be some compensation here because the antero-
posterior diameters of the vertebral canal increase slightly in the more inferior lumbar
vertebrae (see Table 1). However, we should note that the pedicles are much shorter
87
than usual so that the overall vertebral canal is greatly reduced when compared to normal.
In addition, the compression of the spinal cord and/or spinal nerves may not be due just
to reduced interpediculate distance, short pedicles, or kyphotic deformity, but may also
be contributed to by the close apposition of the laminar elements of the vertebral arch.
The pelvis of achondroplastics is generally reduced with a kidney-shaped
inlet greatly reduced in its anteroposterior diameter, a result of the very prominent
sacral promontory and the short caudal segment of the ilium. The pubis is nearly
normal in size and configuration but may appear abnormal as it adapts to the more
greatly reduced ischium and especially the ilium.
It is the long, cylindrical bones of the limbs which are most dramatically
affected in achondroplasia, a direct result of their being entirely dependent upon endo-
chondral growth for increase in length. The exaggerated bony prominences are easily
understood as the result of a near-normal muscle mass being applied to a reduced, mal-
formed skeletal framework. Possible explanations for the flaring bone ends were pre-
sented above and need not be repeated here. Since periosteal ossification is not affected
in achondroplasia, the diaphyses generally show a near-normal cortical thickness
associated with greatly reduced shaft lengths.
The inability to fully extend the elbow was noted above in the morphological
description. Bailey (1971, 1973) has speculated on the causes of this sudden block to
full extension and, on the basis of clinical and radiographic examinations, lists the
following possible causes:
1) deformities of the radial head,
2) subluxation or dislocation of the radial head,
3) humeral impingement of the tip of the olecranon,
4) incongruous fit of the bones of the olecranon fossa,
5) abnormal bowing of the distal end of the humerus, and
6) tight soft tissue structures about the elbow (Bailey 1971: 77, 1973: 64).
Although Bailey has the advantage of numerous clinical experiences in trying to explain
this phenomenon, we have an advantage here in being able to directly examine the bony
relations at the elbow. It is easily seen that the major contributor to the full extension
block is the extremely constricted opening of the olecranon fossa which allows no more
than the extreme tip of the olecranon process to intrude, even though the fossa itself is
quite deep. In effect, the lateral borders of the fossa so constrict the opening that the
lateral aspects of the anterior tip of the olecranon process are blocked from further
extension.
In addition, the small depression noted on the inferior surface of the capitulum
(which accepts the edge of the radial head) may indicate that the dislocated radial head
contributes to this extension block, i. e., the radial head only fits this depression when
the elbow is in its fullest possible extension. Even if the above bony malformations were
88
normal, the anteriorly displaced distal articular end of the humerus may contribute to
the apparent lack of full extension, i. e. , the humero-ulnar articulation may actually be
in full extension but, because of the anterior displacement of the distal articular end
of the humerus, may appear to enclose a less obtuse angle. Obviously we cannot eval-
uate the contributions of tight soft tissue structures.
A tabulation of the osteometric data for this individual appears in Appendix A.
Measurements follow those presented in Snow (1943) so they may be directly compared
to the female and male achondroplastic skeletons from Moundville. In general, the
measurements are quite comparable to those of the Moundville dwarfs, especially the
craniometric data.  The Augustine Site female, however, has shorter long bones than
even the Moundville female (which are shorter than the Moundville male) and this
observation helps support the probable diagnosis of female.
An accurate age estimation is difficult to make, given the abnornial bony
development which probably affects the expression of those criteria normally used in
skeletal aging. We can say the individual is an adult (all permanent dentition erupted,
all epiphyses fused, spheno-occipital synchondrosis fused) and, given the amount of
dental attrition, was probably in the third or fourth decade of life. Although not without
its problems, the degree of exocranial suture closure generally confirms this. Pubic
symphyseal aging was not done because the pelvic distortion (and muscular stresses
placed upon it) and slight damage to the symphyseal face would have precluded meaningful
results.
Differential diagnosis
In his radiographic analysis and interpretation, Dr. Johnston mentions several
of the more prominent differential diagnostic possibilities to rule out a skeletal dysplasia
of this kind. In general, the problem is one of distinguishing between the many forms of
dwarfism; specifically, we are concerned with the short-limbed dwarf types, or those
processes resulting in disproportionate short stature. To fully discuss the total array
of possibilities would severely overburden the general anthropological audience to which
this paper is directed. For those interested in this aspect of the problem, excellent
reviews of the differential diagnosis of achondroplasia can be found in Aegerter and
Kirkpatrick (1975), Bailey (1973), Caffey (1958), Edeiken and Hodes (1973) -- an
especially good discussion, Greenfield (1975), and Jaffe (1972) among others.
Anthropological significance
In summarizing the importance of finding a dwarf (? chondrodistrophia hyper-
plastica) from medieval Yugoslavia, Farkas and Lengyel (1971/72: 207) note the
following:
The point of interest of the paleopathological diagnosis that can be regarded
probable on the basis of the deformations described lies, on the one hand, in
89
the rarity of pathological deformation (Nemeskeri-Harsanyi, 1959), and on
the other hand, in the fact, interesting in itself, that the individual examined,
in spite of the anomalies of osteogenesis, had lived up to a senile age.
The rarity of the phenomenon described and the attainment of adulthood are also points
of interest in the present case of prehistoric achondroplasia.
Although contemporary frequencies would indicate that the birth of an
achondroplastic individual is not so rare an event today, we briefly discussed above the
possibility that the frequency would be much lower in prehistoric times, mainly on the
basis of lack of access to modern medical care. An additional component of this prob-
ably lower frequency in prehistoric times relates to the possibility of infanticide, at
least among those groups that practiced it on the basis of a newborn "appearing different"
at birth. However, unless we have some sort of documentation (artistic, artefactual,
etc.) to support this, it can only be conjectured and suggested as one of the many pos-
sibilities for the very infrequent recovery of this kind of prehistoric skeletal material.
Indeed, we should probably feel very lucky that such a complete individual has been able
to be recovered and studied. (Compared to the Moundville and other Old World speci-
Imens this may be the most completely preserved extant prehistoric achondroplastic
individual.)
Given the health problems to which achondroplastics are prone during their
lifetime, the attainment of at least the third or fourth decade of life in the individual
Under discussion is remarkable. It was noted above that 80% of liveborn achondro-
plastics die within the first year of life, but if they can survive the infant-childhood
years they have a good chance of reaching adult status. Today, with adequate medical
attention they can expect to achieve normal life spans, or at least a close approximation.
Dr. Johnston has so well summarized the major possibilities that led to the death of
this Augustine site individual that we can say little else that would add to his discussion,
except to recommend that the reader go back to the final part of Dr. Johnston's report.
Since dwarfs and hunchbacks have apparently played important roles in the
New World (at least Mesoamerica), as evidenced by their frequent portrayal in arte-
factual remains (see discussion above), we might wonder whether this hunchbacked
dwarf from prehistoric California was also accorded special status among the Augustine
Site inhabitants. Although we could plausibly argue for such, on the basis of the Meso-
amnerican archaeological and ethnographic record (Corson 1972: 318-325), to do so
Would be an exercise in vacuo since we have no associated artefactual remains in this
inlstance. It would be interesting in this regard to learn of the existence of ethnographic
reports that mention dwarfs in California, either factual accounts or in the folklore or
raythology of California groups,
We would also like to know what general role this individual may have played
in the economic support of the group, if any. If no special status was accorded this
'different' member of the group, was she able to carry out the normal daily activities
appropriate to her sex? Even if she were not paralyzed for any length of time her spinal
deformity (of many years' duration) would have precluded certain physical tasks. Perhaps
there were very specific tasks that were accorded to her and not others. But, again,
this is only speculation. In any event, we can probably say that she did suffer some
physical handicap, but the fact that she was able to live to adulthood would argue for the
social group at the Augustine Site to have made various adjustments and accomodations
to her disability. What these were we cannot specify.
The widespread depiction of dwarfs and/or hunchbacks in the literature of
Mesoamerican archaeology brings up another point of anthropological interest that
supports the presentation of such detailed osteological evidence as we have done in this
report. And that concerns the actual medical diagnosis of the abnormalities depicted.
That 'a full understanding of the biological base of achondroplasia (and other skeletal
disorders for that matter), and the resultant, specific abnormalities manifested, is
necessary before attempting to diagnose artistic representations can be illustrated with
the following example. In his excellent book, The Shaft Tomb Figures of West Mexico,
von Winning (1974) portrays a figurine (Figure 14, p. 98) diagnosed as "Syphilitic
dwarf," the syphilis diagnosed pi-imarily on the presence of a sunken nose. The figurine
has other elements, in addition to the depressed nose, that strongly favor achondroplasia
as the correct diagnosis (without having to allude to the enigmatic problem of pre-
Columbian New World syphilis): bulging cranial vault, with a protruding forehead, and
micromelia of the rhizomelic variety. Von Winning (personal communication) did not
make the diagnosis of syphilis and has communicated that "this is questionable because
it [the sunken nose] is also a symptom of achondroplasia. " The point to be made here
is that diagnoses so often made in the past have not entertained differential diagnostic
possibilities that may be more reasonable, if not more exciting. (See Stewart [1975]
for another example of this general problem.) Without actual skeletal and/or mummified
remains that are much less equivocal, we should always be very careful in our pronounce-
ments of the presence of specific disease entities.
A final point of anthropological interest, that also bears upon the detailed pre-
sentation of such rare skeletal abnormalities, concerns the identification and interpre-
tation of faunal remains from archaeological or other contexts, By presenting detailed
descriptions and photographs of such material, the faunal analyst should be able to
specifically identify this process if the occasion ever arises. With the uncovering of a
nearly complete skeleton, with cranium, this shouldn't present any problem as to its
identification. But suppose only a few fragments of limb bones were available -- would
the identification be so easy? Probably not! (In fact, several individuals have remarked
on the very general similarity of the limb bones to various sea mammals. But when the
skull and pelvis were introduced, these general appearances quickly dissipated.) In a
sense, then, this is just one more item in the range of human (and vertebrate) skeletal
variability that should always be kept in mind.
91
Medical significance
We have been primarily concerned in this paper with the anthropological
aspects of a very rare paleopathological phenomenon. Can such a study contribute to the
health sciences as well? Yes, in several different ways. First, we have an additional
documentation from prehistory in a previously unreported region (the western United
States) of a very rare genetically-based abnormality of skeletal growth. This geographic
extension helps support the notion that geographic (hence, environmental) variability
does not affect the manifestations of this growth abnormality. This is perhaps a minor
point, but one which has important implications if the environment were seen to affect
the expression of this genetic trait. Additionally, we have further evidence that this
hereditary trait has remained stable in its expression over time; the implications of
this fact parallel that mentioned above.
As should be apparent from the numerous citations from the medical literature,
achondroplasia has elicited a great deal of interest among health professionals, especially
the orthopedists who must deal directly with the problems arising from the bony abnor-
malities. In trying to interpret and understand these abnormalities the osteological
paleopathologist has a distinct advantage over the clinician in that the former can directly
examine the bony material itself, whereas the latter must rely on clinical information
alone (unless he happens to be fortunate enough to acquire autopsy material for dissection).
For the most part, this may not make any difference in the management of the patient
but it can help in the overall understanding of why the problem exists. For example,
Bailey (1971: 75) notes, "The surgical implications of deformities of the upper limbs in
achondroplasia are minimal but the diagnostic significance, particularly of their elbow
deformities, is great.' It is hoped the present paper has contributed to that understanding.
SUMMARY
We have presented in this paper a full report on the third extant prehistoric
achondroplastic dwarf skeleton (adult, female) from the New World, previously reported
in a brief note (Hoffman 1975: 3). The nearly complete individual comes from the
Augustine Site (CA-Sac-127), Sacramento County, California, and more likely dates from
the Late Horizon, Phase II (AD 1500-1800), not the Middle Horizon as originally reported.
No specific archaeological context could be determined. A detailed morphological des-
cription was presented and accompanied by a radiographic analysis and interpretation
by Dr. James 0. Johnston, Oakland, California.
The general biological characteristics of achondroplasia were presented as
background for an interpretation of the bony abnormalities seen in this individual.
Among the specific features noted were:
1) a more specific explanation for the depressed nasomaxillary region than
had previously been reported,
2) a specific explanation for the lack of full elbow extension,
92
3) the observation that the interlaminar distance of the lumbar vertebrae is
reduced which further contributes to the spinal stenosis in achondroplasia,
and
4) the first reporting of an accessory atlanto-occipital articular facet in
achondroplasia.
The anthropological significance of this individual was discussed and the
following points noted:
1) the rarity of finding achondroplastic skeletal material,
2) the unusual attainment of adult age for a prehistoric achondroplastic,
3) the possible social position of the individual at the Augustine Site,
4) the problems of diagnosing diseases from artistic representation, and
5) its possible confusion with other faunal remains.
The paper concluded with some general comments on the importance of such
paleopathological remains to the health professions, specifically:
1) a further demonstration of the temporospatial constancy of a known genetic
trait, and
2) the unique contributions that osteological paleopathologists can made to our
understanding of health-related problems through their direct examination of
bony abnormalities.
93
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1970  Orthopaedic aspects of achondroplasia. Journal of Bone and Joint
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1971  Elbowand other upper limb deformities in achondroplasia. Clinical
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1973  Disproportionate Short Stature. Philadelphia: W. B. Saunders.
Bass, William M.
1971 Human Osteology: a Laboratory and Field Manual of the Human Skeleton.
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1967
Caffey, John
1958
Coe, Michael D.
1965
Comas, Juan
1960
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Edeiken, Jack and Philip J. Hodes
1973 Roentgen Diagnosis of Diseases of Bones, 2nd. ed. Volume One.
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Farkas, Gyula and mre Lengyel
1971/72 Skeleton of a medieval dwarf from Ludos-Csurgo (Yugoslavia).
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94
Grady, Mark Allen
1969 An osteological analysis of selected burials from the Rooney extension
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Greenfield, George B.
1975 Radiology of bone diseases, 2nd. ed. Philadelphia: J. B. Lippincott.
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1975 A prehistoric achondroplastic dwarf from California: a preliminary
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Jaffe, Henry L.
1972 Metabolic, Degenerative and Inflammatory Diseases of Bones and
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Janssens, Paul A.
1970   Paleopathology. Diseases and Injuries of Prehistoric Man. New
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1892  Untersuchungen uber die Sogenannte foetale Rachitis (Chondrodystrophy
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Linne, S.
1943   Humpbacks in ancient America. Ethnos 8: 161-186.
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1959
and L. Harsanyi
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1878
Sur la malformation achondroplasique et le dieu Phtah.
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Proskouriakoff,
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Tatiana
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University of Chicago Press.
Snow, Charles E.
1943 Two prehistoric Indian dwarf skeletons from Moundville. Alabama
Museum of Natural History, Museum Paper 21. University, Alabama.
Stewart, T. D.
1975   Cranial dysraphism mistaken for trephination. American Journal of
Physical Anthropology 42: 435-438.
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1971
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Congenital Malformations. Notes and Comments. Chicago: Year
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96
APPENDIX A. Osteometric data from achondroplastic dwarf from CA-Sac-127
(all measurements in mm)
CRANIUM
Glabello-occipital length                      170
Maximum breadth                                148
Basion-bregma height                           138
Minimum frontal b.                              96
Basion-nasion 1.                                85
Basion-prosthion 1.                             80
Horizontal circumference                       517
Nasion-opisthion arc                           385
Bizygomatic b.                                 126
Midfacial (bimaxillary) b.                      92
Total facial h.                                108
Upper facial h.                                 61
Nasal h.                                        44
Nasal b.                                        25
Orbital h.                                      34L 34R
Orbital b. (maxil-front)                        42L 42R
Orbital b. (dacryon)                            36L 36R
Anterior interorbital b.
(maxil-front)                             20
(dacryon)                                 28
Biorbital b.                                   101
Maxillo -alveolar 1.                            49
Maxillo-alveolar b.                             57
MANDIBLE
Condylo-symphyseal 1.                           95
Bicondylar b.                                  116
Bigonial b.                                     97
Height of symphysis                             34
Mandibular h., right                            56
Min. b. -ascending ramus                        32
Mandibular angle                               116
97
INDICES
Cranial                                          87.1
Length-height                                    81. 2
Breadth-height                                   93. 2
Mean height                                      86.8
Cranial module                                  152
Frontal-parietal                                 64.9
Total facial                                     85.7
Upper facial                                     48.4
Nasal                                            56. 8
Orbital (max-front)                              81. OL 81. OR
Orbital (dacryon)                                94. 4L 94. 4R
Palatal                                         116.3
Mandibular 1-br                                  81.9
Mandibular 1-ht                                  58.9
HUMERUS
Maximum 1.                                      157    159
Midshaft circumference                           56     55
Max. diam. head                                  36     36
Max. diam. midshaft                              21     20
Min. diam. midshaft                              20     20
Index robustness*                                35.7   34.6
Humeral-femoral index                            70.7   70.4
RADIUS
Maximum 1.                                      127     --
Midshaft circumference                           32    (34)
Index robustness                                 25.2   --
Humeral-radial index                             8009   --
ULNA
Maximum 1.                                      148    156
Midshaft circumference                           38     38
Index robustness                                 25.7   24.4
SCAPULA
Maximum l.                                      --     130
Maximum b.                                       83     85
Length of spine                                 __      99
CLAVICLE
Maximum l.                                      114    117
Midshaft circumference                           32     32
index robustness                                 28.1   27.4
Clavicular-humeral idex                          20.4   20.4
FEMUR
Maximum 1.
Midshaft circumference
Bicondylar 1.
Max. diam. head
Subtroch. a-p diam.
Subtroch. lat. diam.
Midshaft a-p diam.
Midshaft lat. diam.
Platymeric index
Index robustness
TIBIA
Maximum l.
Midshaft circumference
Nutrient a-p diam.
Nutrient lat. diam.
Midshaft a-p diam.
Midshaft lat. diam.
Platycnemic index
Index robustness
Tibio-femoral index
FIBULA
Maximum 1.
Midshaft circumference
SACRUM
Height
Breadth
Index
INNOMINATES
Height
Breadth
Index
. .            .  ~~~~midshaft circumference
* All robusticity indices follow Snow, r. . =m
maximum length
comparable; this does not follow the formulae presented in Bass 1971.
. to keep indices
98
Left
222
67
211
37
20
25
18
21
80.0
30.2
Right
(226)
(66)
(219)
18
28
19
20
64.3
(29.2)
194
57
23
19
19
16
82.6
29.4
85.8
207
43
193
58
23
20
19
16
87.0
30.1
86.9
210
39
96
(99)
(103. 1)
154
122
126.2
ILLUSTRATIONS
Plate 1.
Plate
Plate
Plate
Plate
Plate
Plate
Plate
Plate
Plate
2.
3.
4.
5.
6.
7.
8.
9.
10.
Partially reconstructed anchondroplastic dwarf prior to its
disassembly
Skull of achondroplastic dwarf
Close-up views of cranium
Vertebral column elements
Pelvis
Thoracic and upper limb bones
Lower limb bones, plus metacarpals
Radiographs of cranium and vertebral column
Radiographs of miscellaneous bones
Radiographs of limb bones
99
Plate I. Part.ially reconstructed dwarf prior to its disassembly
Top:         Left - achondroplastic dwarf szkeleton-from CA-Sac-127, sans
ribs and small bones of hands and feet
Middle - complete verteb'ral column from C1 through, L5
Right -vertebrae T9 through T12; note plaster intervrertebral
disc reconstructions
-Bottom:     Left - anterior view of lower limb bones; note reconstructed
:. ~~right femoral head and excessive tarsal extension of both fibulae
Middle- right - anterior view of upper limb long bones; note
lack of fulexte-nsion at elbo'w
- ~-Lower right -left anterosuperior view of reconstructed pelvis
-       ~~(right innominate is plaster mirror imxage of the left); note
broad, shallow, pelvic inlet (see Plate 5)
Plate I
103
Plate 2. Skull of achondroplastic dwarf
Top:        Left - anterior vriew of cranium
Right - left lateral view of cranium; note lesion (at arrows) in
posterior frontal region (see Plate 3)
Middle:      Left - basal view of cranium
Right - left lateral view of entire skull; note recessed nasal
and upper maxillary regions which accentuates the prognatEhism
Bottom:      Left - posterior view of craniu:m; note extreme biparietal
bossing and foci of osteoporosis
Right - superior view of mandible
Plate 2
105
Plate 3. Closeup views of cranu
Top:         crania base; note small, angular foramen magnum and the
foreshortened, laterally compressed base as a whole; arrows
-point to accessory articular facet for expanded left lateral
mass of atlas (see Plate 4)
Bottom:      the lesion. involving the left posterior frontal region, possibly
an epidermoid inclusion cyst or hemangioma
Plate 3
-AS0 I, '   j;f.E ' .:;.3i.'' ';iSiW f
107
iPlate 4. Verteb-ral columan elements
'Top:
L1eft - superior view of articulated atlas and a-xis; note expanded
lateral masses of atlas with accessory articular facet on left
(at arrows)
M!2iddle - right superolateral view of C7; note absence of right
costal element (results in a notch for the vertebral artery rather
than a closed transverse foramen); arrows point to articular
facet for a right cervical rib; there is a partially bridged trans-
verse foramen on the left
Right - superior view of IL5; note extremely contracted vertebral
canal, as much a result of the narrowed interlaminar distance as
the reduced interpediculate distance; the actual length of the
pedicles is reduced as well as the interpediculate distance.
IL,eft - right lateral view of 'T11 through ILA; note anterior
wedging of all vertebrae (producing a prominent kyphotic deform-
ity) and extensive anterior osteophytosis
Right - left lateral view of 'T11 throulgh IIA; the extreme wedging
( ? hypoplasia) of 'T12 is quite evident
L1eft - anterior view of sacrum; note partial reconstruction of
sulperior asp-ect of right ala
Right - left lateral view of sacru:m with ]EL5 in normal anatomical
position; note complete lack of anterior sacral curvrature, the
exaggerated lumbosacral lordosis, and the reduced, deeply-
concave auricular surface
M iddle:
:Bottom:
]Plate 4
109
Plate 5. Pelvis
Top:        Left - superior view of reconstructed pelvis; note contracted,
kidney-shaped inlet with markedly reduced anteroposterior
diameter
Right - inferior view of reconstructed pelvis; note large, ex-
pansive pelvic outlet
Middle:      Left - posterior view of reconstructed pelvis; note deep pitting
on sacrum for ligamentous and tendinous insertions
Right - anterior view of left innominate; note prominent pubic
tubercle, fClattened acetabular roof, reduced ilium and ischium
with a relatively normal pubis
Bottom:     Left - lateral view of left innominate; note wide, shallow greater
sciatic notch
Right - medial view of left innominate; note small auricular
surface
Pl a te 5
illl
Plate 6. Thoracic and upper limb bones
Left -clavicles, scapulae, and sternum sans xiphoid
Right - right first rib (top) and nine left ribs; note exaggerated
curvrature of most ribs, producing a constricted anteroposterior
thoracic diameter
upper limb bones; from left to right - anterior left humerus,
posterior right humerus, medial left ulna, lateral right ulna,
anterior left radius, posterior right radius; note extremely
constricted opening to olecranon fossa, the depression anterior
to the supinator crest on the right ulna (arrows) and the
extremely short, angulated radial neck
lateral view of left elbow region; the left photo depicts the
bony relations with forearm supinated (note dislocated radial
head); the right photo depicts the forearm pronated to show how
the radial head swings into the depression anterior to the
supinator crest; in both photos the elbow is extended as fully
as possible, illustrating the decreased carryling anlgle; the
distal radial-ulnar articulations (not seen) are normal
Top:
Middle:
Bottom:
Plate 6
ow
113
Plate 7. Lower limb bones, plus metacarpals
Top:         from left to right - anterior left femur, posterior right femur,
anterior left tibia, posterior right tibia, lateral left fibula,
medial right fibula
Bottotrl:    Left - left cuboid and calcaneus
Middle - left fifth -metatarsal and talus
Right (top to bottom) - left second metacarpal, right second
and third metacarpals
Plate 7
115
Plate 8. Radiographs of cranium and vrertebral column (see text for description)
Top:         Left - lateral radiograph of cranium; note shortened cranial
base, hypoplastic upper face, and sclerotic borders surrounding
the lesion on the frontal bone (posterior aspect)
Right - anteroposterior radiograph of cranium
Middle:      (from left to right) - cervical vertebrae 1 through 7, thoracic
vertebrae 1 through 5, thoracic vertebrae 6 through 10, Tll
through Ll
Bottom:      Left - lu:mbar v7ertebrae 2 through 5
Right - anteroposterior view of sacru:m
Plate 8
117
Plate 9., Radiographs of miscellaneous bones
Top:        mandible, three ribs, left calcaneus
Bottom:     Left -mandible, clavicles, sternurn
Right - left innominate, scapulae
Plate 9
119
Plate lO. Radiograph,s of-limb b-ones  .:.~
Top:      -   ihmeri, ulnae, radii  :-:-       :    ;
Bottom:   X   .Left -;:. -femor, fibulae;''note plaste'r.-reconstruction in right
*...fem,ur                             .:
:Right -tibiae., left f-ifth. me taretarsal aind thre metacarpals
plte 10.