JUNE, 1973

OSTEOPATHIA STRIATA-VOORHOEVE’S DISEASE*

REVIE\V OF THE ROENTGEN MANIFESTATIONS
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By JOHN A. GEH WEllER, Ml)., WILEY R. BLANI), M.D., TERRENCE S. CARDEN, JR., M.D.,
and RICHARD H. DA1lNER, Ml).
DURHAM, NORTH CAROLINA

O S’I’EOP.VI’I-iIA Stniata is a rare roent- roen tgenograpbic changes diagnostic of this

genograph i C en ti tv cii arac terized by disorder, to review the literature and to add
hne linear striations seen primarily in the 2 new cases. One of tile cases is unique in
metaphvses and diaphyses of long bones in that it is the only instance of unilateral
asvmptornatic patients. The finding has skeietal involvement to be reported.
been described ill all bones except those of
the skull and the clavicles. Patients in R E P0 RT 0 F C A S ES
whom the entity has been described have CASE 1. Au 8 year old white girl has been
110 known associated physical abnormali- followed for years at the I)uke tniversity Medi-
ties or characteristic laboratory findings. cal Center (I)tMC) for speech therapy and
Our purpose is to describe ill detail the mental retardation. ihe diagnosis of osteo.

FIG. I. Case I. (A and B) Frontal roentgenograms of the knees at the age of 19 years. Linear striations in
the distal femora and proximal tibiae parallel to the axis of the bone shafts. These changes are more
prominent in the femora. Note tibialosteochondroma on the right (4).

* From the l)epartment of Radioloiv, l)uke University Medical Center, Durham, North Carolina.

450
 VOL. ii8, No. I Osteopathia Stniata-Voorhoeve’s Disease 45’

pathia striata was made at the age of 8 years

when roentgenograms of tile pelvis and knees
were made because of vague left hip and leg
pain. Incidental note was made of a small osteo-
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chondronia of tile proxinial right tibia.

Roentgenograms of the long bones taken at
the age of 19 years show linear striations in the
ends of the femora, tibiae and proximal humeri
(Fig. I, 4 and B; and 2). A small osteochon-
droma projects medially from the proximal
right tibial metaphysis. Striations are also
noted in the first metatarsals (Fig. 3, 4 and B).
Views of the pelvis show stniations in the iliac
wings (Fig. 4), ischia and pubic ranii. There
are zones of bone condensation in the acetab-
ular roofs and the humeral epiphyses. Changes
of osteitis pubis are also present.

CASE II. A 24 year old white woman was ad-

nlitted to the psychiatric service of DUMC in
May, 1971. Aillaboratory and physical findings
were normal.
During her hospitalization she swallowed an
open safety-pin. Plain roentgenograni of tile
abdomen, obtained to localize the pin, revealed
abnormal striations in the right ilium. A sub-
sequent bone survey showed unilateral changes
of osteopathia striata limited to the right os
coxa (Fig. 5), femur, tibia, fibula (Fig. 6, 4
and B), and calcaneus (Fig. 7, 4 and B). All
other bones were normal.

HISTORIC REVIEW
FIG. 2. Case I. Proximal left humerus in anteropos-
The changes of osteopathia striata were tenor projection. Note that the striations are
first described by Voorhoeve, who reported present in the epiphysis, metaphysis and diaphy-
3 cases in 1924.8 The entity went unnamed, sis. A few areas of bone condensation are seen in
however, until 1935, when Fairbank made the epiphysis.
the current designation.’ Our review of the
literature reveals a total of only 5 case re- linear stniations. In long bones, the stnia-
ports,”28 but additional cases undoubtedly tions are nearly uniform and are seen pri-
have been observed because the entity is ii- manly in the metaphyses and diaphyses.
lustrated in some of the standard text- They run parallel to the axis of the shaft of
books”7 dealing with disorders of the skele- long bones and occasionally cross into the
tat system. However, the texts give no in- epiphyses. The stniations apparently vary
formation about the patients. in length in direct relationship to the
growth rate of the involved bone. The long-
ROEN’I’GEN FEATURES
est stniations, therefore, are found in the
The diagnosis of osteopathia stniata is femora. In the iliac wings, a fan-shaped
based entirely on roentgen findings. No pattern is seen (Fig. ; and ). This finding
pathologic correlation exists because none has been more striking in cases reported by
of the patients described had a bone biopsy. others. Involved bones are normal in shape,
The hallmark of the disorder is fine, dense, density and cortical thickness.
 452 Gehweiler et al. JuNE, 1973
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1i. . (ise I. (.1 and R) \nteroposterior roentgenograms of the feet. laint striations are seen in the first
Iisetattrsais, hei ng nore Pt 01111 I1fl t on tile left (1?).

DIFFEREN’I’I.AL DIAGNOSIS

Ihere have been reports of bOne “stnia-

tiofls other ill diseases, including osteo-
poi ki losi s, osteope trosi s, dy schondroplasi a
(Oilier’s disease) , melorheostosis, Paget’s
disease, po1’ostotic fibrous dsplasia and
neurofibromatosis.’ 4,6 However, in all of
these entities the striations are dissimilar
to tiiose found it-i osteopathia stniata in

11G. 4. Case I. Anteroposterior roentgenogram of the

Pelvis and ilips. Striations are fan-shaped in the
iliac wings. Some striations are also seen in the
pubis and ischium I)iiateraiiV. Both ischial tu-
I)erosities are more radiopaque than normal and
there are ZOOCS of increased density in the acetabu-
lar roofs.
 VOL. ii8, No. 2 Osteopathia Striata-Xoorhoeve’s I)isease 453

their location, length, width, or in the num-

ben of bones involved. Fairbank, in a per-
sonal communication to Bloor,1 contended
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that the only other condition that “can

give rise to widespread and uniform stnia-
tion” is celiac disease. A brief review of tile
cases of celiac disease available to us failed
to reveal any patients with bone stniations.
However, few of these patients had com-
plete roentgenographic studies of their
skeletal systems.

DISCUSSION

‘fiie etiology and pathogenesis of this

disorder are unknown. It is thought to be
an error in modeling of bone and is classi-
fled by Rubin among the metaphyseal dys-
FIG. 5. Case II. Pelvis and hip Joints at 24 sears of
plasias.7 However, the author states ciearl’
age show striations on the rigilt side in tile iliac
that it does not fit his classification of bone \ving and proXiIllal right femur. Zones of bone
dysplasi as. c0Ildens1tion are seen in the right acetabular roof.
Four of the 7 patients whose cases have
been reported were females. The only pos- iloeVe, which included a father, son, and
sible hereditaryor familial influence was daugilter who showed similar bone cilanges.
present in the 3 cases reported by Voor- In all patients except our Case II, in-

p...)

1IG. 6. Case II. (A an(1 B) Lateral roentgenograms of the knee joints show striations in the feniur, tibia and
fibula on the right. The left side is normal (B).
 454 Gehweiler et al. JUNE, 1973
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lic. 7. Case ii. (A and B) lateral roentgenograms of the feet. Faint linear striations are present in the
calcaneus On the right as well as in the distal right tibia (4).

volvement was bilateral. Fairbank, in his could be seen in osteopoikilosis, but he

original paper,’ also reported a case of uni- pointed out that these streaks were innegu-
lateral involvement in a 12 year old boy, lan and shorter than those found in osteo-
but stniations eventually became apparent pathia stniata.
bilaterally and the author corrected his Hurt6 also raised the question of a rela-
findings in a later report.4 tionship to osteopetrosis. One of his pa-
Several authors have postulated a rela- tients showed changes typical ofthat entity
tionship with other diseases. Voorhoeve in the skull and ribs and changes of osteo-
believed that the entity was a congenital pathia stniata in the long bones. His case
defect related to dyschondroplasia and Os- resembled that reported by Bloor1 in which
teopoikilosis. Fairbank stated that the the patient had bone sclerosis at the base
entity could not be considered a form of of the skull and thickening of the frontal
dyschondroplasia because the changes in bone. This patient did not have the bone
the hands and feet in that disorder could condensations seen in the other 6 cases re-
not be considered stniations and there was viewed here.
no uniform involvement of the major met- Thus, it can be seen that there has been
aphyses-as in osteopathia stniata. a significant debate and postulation about
Hunt#{176}suggested that osteopathia stniata the nature of this entity and the undenly-
may be a form of osteopoikilosis in which ing disorder, but no definitive information
the stniations predominate. Evidence for has become available. Since patients show-
this theory is the finding in 6 of the 7 re- ing the skeletal changes of osteopathia
ported cases of bone condensations some- stniata have no clinical or laboratory ab-
what suggestive of osteopoikilosis. Hurt normalities, there is no indication for bone
speculated that force applied to long bones biopsy or other more aggressive diagnostic
involved by the underlying entity could maneuvers that might help to explain the
produce changes typical of either disorder. pathogenesis. Failing this, the disorder re-
Fairbank3 agreed that occasional streaking mains a purely roentgenographic entity.
 VOL. ii8, No. I Osteopathia Stniata-Voorhoeve’s Disease 455

SUMMARY & 7oint Surg., 1954,36-B, 26 1-265.

2. FAIRBANK, H. A. T. Case ofunilateral affection of
Osteopathia striata is a rare skeletal dis- skeleton ofunknown origin. Brit. 7. Surg., 1925,

order manifested roentgenographically by 12, 594-599.

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fine, linear striations primarily in the long 3. IAIRBANK, H. A. T. Generalized diseases of skele-
ton. Proc. Roy. Soc. Med., Clinical section I,
bones.
1935, 28, I, 611.
There are no associated clinical or lab-
4. FAIRBANK, H. A. T. An Atlas of General Affec-
oratory abnormalities. tions of the Skeleton. E. & S. Livingstone Ltd.,
A review of the literature reveals only Edinburgh, 1951, pp. 111-117.
5 reported cases. 5. GREENFIELD, G. B. Radiology of Bone Diseases.
Two new cases, including I with unique
J. B. Lippincott Company, Philadelphia, 1969,
pp. 236-238.
unilateral involvement, are reported.
6. HURT, R. L. Osteopathia striata-Voorhoeve’s
Several hypotheses about the etiology of disease: report of case presenting features of
the disorder are discussed. osteopathia striata and osteopetrosis. 7. Bone
& 7oint Surg., 1953,35-B, 8o-96.
John A. Gehweiler, M.D. 7. RUBIN, P. Dynamic Classification of Bone Dys-
Department of Radiology plasias. Year Book Publishers, Inc., Chicago,
Duke University Medical Center 1964, 387-388.
Durham, North Carolina 27710 8. VOORHOEvE, N. L’image radiologique non encore
d#{233}crited’une anomalie du squelette: ses rap-
ports avec Ia dyschondroplasie et l’osteopathia
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