Acta Radiologica

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Hyperostosis cranii
Radiography and scintigraphy compared

H. Jacobsson & M. Haverling

To cite this article: H. Jacobsson & M. Haverling (1988) Hyperostosis cranii, Acta Radiologica,
29:2, 223-226, DOI: 10.3109/02841858809174997

To link to this article: https://doi.org/10.3109/02841858809174997

Published online: 07 Jan 2010.

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FROM THE DEPARTMENT OF DIAGNOSTIC RADIOLOGY, KAROLINSKA SJUKHUSET, S-10401 STOCKHOLM,
SWEDEN.
HYPEROSTOSIS CRANII
Radiography and scintigraphy compared
H. JACOBSSON
and M. HAVERLING
Abstract
The bone scintigrams of 52 women with radiographically prov-
en hyperostosis cranii were examined specifically with regard to
the endosteal lesions. It was confirmed that hyperostosis cranii
exhibits a characteristic appearance at bone scintigraphy that is
usually easy to recognize. By scoring the degree of hyperostotic
changes and their scintigraphic activity according to a semiquan-
titative scale, an inverse relation between radiographic changes
and scintigraphic activity was observed but could not be statisti-
cally proven. A tendency of more pronounced radiographic
changes and a tendency of decreasing activity at bone scintigra-
phy with increasing age were also found. It is suggested that
hyperostosis cranii starts in younger women and that the initial
osseous turnover of the lesions is high. Successively, the meta-
bolic activity decreases while the endosteal lesions increase,
tending towards a stabilization of the process with increasing age.
Key words: Skull, hyperostosis; radiography; radionuclide
studies.
Hyperostosis cranii (HC) was first described by MOR-
GAGNI in 1719 (8). Since then it has been studied by a large
number of authors, and its symptom complex has been
referred to as Morgagni syndrome, Stewart-Greeg-Morel
syndrome, Henschen syndrome, craniopathia metabolica
or craniopathia neuroendocrinica (4, 7). The condition,
which is of unknown etiology and significance, and prefer-
ably affects women, has been reported in association with
various metabolic, endocrinologic and psychiatric mani-
festations (5, 6, 11, 12). Its morphology has been studied
by several authors, including MOORE(7) who suggested a
classification system based on radiographs, and DIHL-
MANN (1) who modified this classification on the basis of
computed tomograms. The frequency of the condition has
been analysed in Iarge materials, and attempts have been
made to register its development with radiographs of indi-
viduals at different age (5, 7, 11).
Acta Radiologica 29 (1988) F a x . 2
Bone scintigraphy is a sensitive and widely used meth-
od to detect and follow skeletal metastases. Although the
technique is principally unspecific, the experienced exam-
iner often can sort out areas of focally increased activity
as not being caused by malignant bone processes, e.g.
serial rib fractures, osteoarthritis, dental lesions of the
jaws. Although little has been reported on the appearance
of HC at bone scintigraphy, we have found that this
condition usually exhibits an easily recognized pattern. In
addition, we have encountered a number of HC cases
exhibiting an intense uptake in lesions with rather limited
radiographic changes. We have also seen several cases of
radiographically advanced HC with low metabolic activity
at bone scintigraphy.
In the present investigation the apparent lack of correla-
tion between radiographic findings and bone scintigraphy
is further studied. The information obtained by combining
these two diagnostic modalities might throw some light on
the development of HC.
Material and Methods
The study comprised 52 women with radiographically
proven HC, all in the form of hyperostosis frontalis in-
terna or hyperostosis fronto-parietalis, according to
MOORE’Sclassification (7). The mean age was 62 years
(range 32 to 85 years). All were part of a larger group of
patients with mammary carcinoma undergoing radiogra-
phy and bone scintigraphy in the search for possible bone
metastases. Patients with suspected metastatic involve-
ment of the skull were excluded from the study.
Examination procedures. The radiographic examination
Accepted for publication 17 October 1987.
223
224 H . JACOBSSON A N D M . HAVERLING

Fig. 1. Lateral view of the skull of a 53-year-old woman with Fig. 2. Lateral view of the skull of a 73-year-old woman with
hyperostosis cranii. a) Radiograph showing moderate frontal le- hyperostosis cranii. a) Radiograph showing very marked fronto-
sions (2+). b) Bone scintigram showing marked frontal activity parietal lesions ( 5 + ) . b) Bone scintigram showing moderate activ-
(4+). ity corresponding to the lesions (2+).

Scintlyraphlc
included at least one frontal and two lateral views. The score
scintigraphic examination was performed as an analogous
registration with a General Electric 400 Maxicamera 2 to 3
hours after the intravenous injection of 500 MBq 9 9 T ~ m -
methylendiphosphonate. The examination included at
least one lateral registration from each side. The time
interval between the two examinations never exceeded
two weeks. The radiographs and the scintigrams were
evaluated independently. The degree of hyperostotic
changes and the radionuclide activity of the lesions were

.
scored according to a semiquantitative scale 1+ to 5+,
+
where 1 represented mild (‘incipient’) changedactivity,
and 5+ very marked changedactivity (Figs 1, 2). By
including only cases with frontal and fronto-parietal hy-
perostosis in the study, a reasonable correspondence be-
tween the areas evaluated by the two modalities could be
ensured.
Fig. 3. Relation between radiographic changes and activity at
Statistical methods. When analysing the relation be- bone scintigraphy in hyperostosis cranii as assessed by a semi-
tween radiographic and scintigraphic score (Fig. 3) rank- quantitative scale. An inverse relation is suggested, but the corre-
correlation was used. When comparing the age of the lation is not statistically significant.
 HYPEROSTOSIS CRANII 225

. ..
Radiographic
score
Mean age Standard
deviation
Results

1
5. During the present study it was confirmed that HC
66 106 exhibits a characteristic appearance at bone scintigraphy
4.
that is usually easy to recognize and distinguish from
metastases. HC typically appears as symmetric frontal or
fronto-parietal lesions of variable extent, flaring laterally
3. from and sparing the midline.

. ..
By plotting the scintigraphic score against the radio-

I
2. . . a .
graphic score, as illustrated in Fig. 3, a negative correla-
tion between radiographic and scintigraphic score is sug-

.
50 105
gested but cannot be statistically proven.
1. The radiographic score plotted against the age of the
patient is seen in Fig. 4. There was a significant tendency
L, .
30 40 50 60 70 80 90 Age(years)
of more marked hyperostotic changes with increasing age.
The relation between age and scintigraphic activity was
Fig. 4. Relation between age and radiographic changes in hyper- plotted in the same way as illustrated in Fig. 5 This shows
ostosis cranii as assessed by a semiquantitative scale. The small a significant tendency of decreasing activity of the lesions
number of patients scored I + and 5+ are added to the groups of with increasing age.
2+ and 4+ respectively. By separately comparing the three
groups thus obtained, the only significant difference (p<O.Ol) of
age which could be demonstrated was group 1 +2 versus group 3 Discussion
and group 4+5, respectively. Thus, at higher ages the endosteal
Reports on scintigraphy of HC are scarce in the litera-
lesions are more prominent.
ture and series of cases have not been published. Tran-
sient diminution of activity in the frontal areas at brain
scintigraphy in cases of hyperostosis frontalis interna has
Sclntigraphlc Mean age Standard
score deviation been reported (3). Strongly increased accumulation of
bone-seeking tracer in the frontal lesions of hyperostosis
frontalis interna has been described in a single case, on
the basis of which the ‘classical’ scintigraphic findings of
the entity have been defined (9). By the present study it is
confirmed that HC usually can be recognized at bone
scintigraphy. However, the scintigram does not parallel
the radiographic findings. The tendency of an opposite
development of radiographic changes and activity at bone
scintigraphy with increasing age explains the lack of posi-
tive correlation between the two diagnostic techniques. A
negative correlation may exist but could not be proven in
this material. However, since bone scintigraphy reflects
metabolic activity and vascularity rather than radiograph-
4/ 3’0 4b 60 6’0 7’0
Age(years1
ic lucency, this discrepancy between radiographic and
scintigraphic findings is not inconsistent (2).
Fig. 5. Relation between age and scintigraphic activity of the
endosteal lesions in hyperostosis cranii as assessed by a semi-
The semiquantitative scoring system used is based on
quantitative scale. The small number of patients scored 5+ are subjective criteria, but if applied by the same interpreter
added to the group of 4 + . By separately comparing the four they should be useful measures for evaluation. Similar
groups thus obtained, the only significant difference (p<O.Ol) of quantitation systems have been used by other investiga-
age which could be demonstrated was group 4+5 versus group 1, tors for mapping the radiographic extent of HC (6, 7). The
group 2 and group 3, respectively. Thus, at higher ages the
scintigraphic activity is lower. age variations between most groups of Figs 4 and 5 were
small due to a large variation within the groups, presum-
ably caused by a considerable interindividual variation of
the rate of development of the changes and of the age at
the onset of the process. The natural history of HC is
different radiographic and scintigraphic score groups (Figs unknown. In earlier reports HC was claimed to be a
4, 5) one-way analysis of variance followed by a multi- progressive condition which increases with age (1 1). Lat-
comparison procedure was carried out. The distribution er, it has been claimed not to be an age phenomenon,
of age within groups was checked for assumptions re- reaching a maximum at 40 to 60 years, and then rapidly
quired for the use of analysis of variance. diminishing (5). It has also been concluded that HC is a

15-888082
226 n. JACOBSSON
process of irregular course; once started it may progress
but it can also be stabilized or show regression (6). These
studies were based on large series of single radiographic
examinations of women of different age. Thus, an appar-
ent decrease with increasing age could be due to the
affected individuals not reaching old age owing to patho-
logic processes associated with HC ( 5 ) . Since the present
cases have been selected for HC from a larger material,
they do not allow conclusions concerning prevalence fig-
ures at different ages. However, the tendency of more
pronounced radiographic changes with increasing age sup-
ports the view of HC being a progressive condition that
does not undergo regression at higher ages. This is also
consistent with the decreasing scintigraphic activity with
increasing age, since bone resorption and bone remodel-
ling would give rise to increased activity at bone scintigra-
phy (2, 10).
Since the cases investigated were all part of a larger
series of patients with mammary carcinoma, the present
material obviously represents a selection, even though
cases with suspected metastases of the skull were ex-
cluded. Consequently, as a matter of principle, conclu-
sions should be restricted to this group of patients. How-
ever, considering the fact that the present cases exhibited
a radiographic appearance identical with that of HC in
general, and this being a frequent condition, our findings
permit us to formulate a working hypothesis for future
testing on a population without potential systemic bone
affection.
Thus, it is proposed that HC starts in younger or middle
aged women, and that in earlier phases of the develop-
ment the osseous turnover is high. Successively, the
metabolic activity decreases while the deposits grow very
AND M . HAVERLING
slowly, tending towards a stabilization in the process with
increasing age.
Requesf for reprints: Dr Hans Jacobsson, Department of Diag
nostic Radiology, Karolinska Sjukhuset, P. 0. Box 60500, S-
10401 Stockholm. Sweden.
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