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Disappearing lumbar hyperostosis in a patient with forestier's diseaseAn ominous sign.

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BRIEF REPORTS
799
DISAPPEARING LUMBAR HYPEROSTOSIS IN A PATIENT WITH
FORESTIERS DISEASE: AN OMINOUS SIGN
ABE CHAITON, ADEL FAM, and BRUCE CHARLES
Forestier's disease, ankylosing hyperostosis of
the spine, or diffuse idiopathic skeletal hyperostosis
(DISH), is a relatively common disorder affecting
middle aged and elderly individuals (1-1 1). It is characterized by a distinctive type of spinal ossification. Extraspinal heterotropic ossifications at sites of ligamentous
and tendinous attachments are not uncommon (2,4,7,91 1). Although frequently asymptomatic, many patients
complain of spinal stiffness and backache (2,4,9,11).
Pathologic and radiologic studies have shown that the
abnormal ossification process is a slowly progressive
one, which may take several years for its completion
(2,5,8,11).
We report here a 77-year-old man who developed progressive backache which was initially thought
to result from advanced diffuse idiopathic skeletal hyperostosis. Serial radiographs, however, showed progressive disappearance of the distinctive lumbar hyperostosis. He was later discovered to have a leaking
abdominal aortic aneurysm.
Case Report. In February 1977 a 77-year-old
man experienced an episode of fever and chills which
cleared spontaneously. He had a second episode a
month later which was associated with low backache.
Radiograms of the thoracolumbar spine showed typical
changes of DISH (Figures 1 and 2) with continuous ossification along the anterior aspect of several contiguous
vertebrae, radiolucency between the deposited bone and
the underlying vertebral bodies, and relative preservation of the intervertebral disc spaces. The sacroiliac
joints were normal. A blood culture grew Bucteroides
From the Division of Rheumatology, Sunnybrook Medical
Centre, University of Toronto, Toronto, Ontario, Canada.
Address reprint requests to Dr. A. G . Fam, Sunnybrook
Medical Centre, 2075 Bayview Avenue, Toronto, Ontario, Canada
M4N 3M5.
Submitted for publication November 2, 1978; accepted in revised form February 21, 1979.
frugih. Because his symptoms resolved spontaneously,
the organism was thought to be a contaminant.
He remained well until October 1977, when the
backache recurred and became more persistent, despite
ASA and indomethacin. A second set of thoracolumbar
films was taken on December 14, 1977. No comparison
was made with the previous films. The radiologic abnormalities and the backache were again attributed to
diffuse idiopathic skeletal hyperostosis. However, as
seen in Figure 3, there is complete disappearance of the
ossification between L4 and L5 vertebrae, partial disappearance of the ossification between L3 and L4, and
extensive destruction of the anterior and inferior portions of L4 vertebral body. There is erosion of L5, particularly anteriorly, but not as advanced as in L4.
On January 4, 1978, the patient had a third episode of fever and chills associated with continuous lumbar pain which also radiated to the right groin. On admission, he was febrile and appeared chronically ill.
Blood pressure was 130/70. The pulse was regular and
the pedal pulses were equal. The abdominal wall veins
were dilated and the flow was cephalad, suggesting
some obstruction to the inferior vena cava. An ill-defined, nonpulsatile, but very tender mass, approximately
6 x 12 cm was palpable in the right upper quadrant.
There was a midabdominal systolic bruit. Lumbar spine
movements were painfully restricted and there was
tenderness opposite the third, fourth, and fifth spinous
processes.
Relevant laboratory investigations revealed a hemoglobin of 9.5 gm/dl, WBC 17,200 per mm3with 86%
neutrophils, BUN 40 mg/dl, creatinine 1.6 mg/dl, and
normal serum calcium, phosphorus, alkaline phosphatase, LDH, SGOT, and bilirubin. A third set of thoracolumbar films showed further erosion and scalloping of
the involved vertebrae. Due to technical difficulties, the
mass failed to visualize on abdominal ultrasonography.
There was no fluid on abdominocentesis. Blood culture
grew penicillin-sensitive Bucteroides frugzXs. Intravenous penicillin G, 12 megaunits per day, was insti-
800
BRIEF REPORTS
liver, inferiorly to the pelvis, laterally to the right perirenal adipose tissue, and medially to the lumbar spine,
causing erosion and cystic degeneration of L4 and L5
vertebral bodies. The aneurysm contained a mural
thrombus. There was no apparent communication between the aneurysmal sac and the venous system. Blood
taken at autopsy again grew Bacteroidesfragilis. There
were no septic foci seen on sections of the aorta, vertebral bodies, or heart valve.
Figure 1. Lateral films of thoracic spine showing continuous ossification along the anterior aspect of several contiguous vertebrae. Note
radiolucency between the deposited bone and the underlying vertebral bodies and relative preservation of the intervertebral disc spaces.
tuted but the patient continued to have spiking fever.
The presence of an abdominal mass, signs of inferior vena cava obstruction, and destructive vertebral
changes suggested an intraabdominal malignancy. Five
days after admission, he suddenly became pale, hypotensive, tachypneic, and restless. The abdomen became
more distended, diffusely tender and bowel sounds were
absent. He died soon after.
At autopsy, there was a large, ruptured arteriosclerotic abdominal aortic aneurysm located just proximal to the bifurcation. At the site of the rupture, there
was a large paraaortic hematoma which resulted from
It ‘Omthe massive bleeding that Occurred
retromunicated with a huge9
more
peritoneal hematoma (secondary “false” aneurysm)
which extended superiorly to the inferior surface of the
Figure 2. Lateral film of the lumbar spine (Feb 1977) showing similar ossification alone the anterior asDect of several vertebrae. The ossification IS not as coitinuous as in t i e thoracic spine. Note the normal
disc heights.
BRIEF REPORTS
Figure 3. Lateral film of lumbar spine (Dec 14, 1977) showing complete disappearance of the ossification between L4 and L5 vertebrae,
partial disappearance of the ossification between L3 and L4, and extensive destruction of the anterior and inferior portions of L4 vertebral body. There is erosion of L5,particularly anteriorly, but not as
advanced as in L4.
Discussion. Based on autopsy studies, diffuse
idiopathic skeletal hyperostosis is estimated to occur in
6-12% of the elderly population (5,8). Although commonly reported as an incidental radiologic finding, the
condition may cause spinal stiffness and backache
which is rarely severe (2,4,9,11). In our patient, the severe recurrent back pain was more likely due to the
leaking aneurysm.
Abdominal aortic aneurysm (AAA) occurs most
commonly in men in the sixth and seventh decades (1214). Arteriosclerosis is by far the commonest cause (13).
Patients are frequently asymptomatic even when the aneurysm is large (12- 14). Most studies cite a 30-60% incidence of rupture of unoperated aneurysms (12,13,15).
Prior to rupture, only 14-23% of patients were known to
have the condition (12,13,16,17).
801
Back pain is estimated to occur in 34-47% of patients with ruptured abdominal aortic aneurysm (17,18).
Recurrent backache extending over a period of several
months, due to slow or repeated leakage from the aneurysm, has also been reported (1 9,20). The autopsy findings in our patient indicated that the recurrent backache
resulted from slow and repeated leakage from the aneurysm.
The diagnosis of ruptured AAA is often overlooked when there is no pulsatile abdominal mass,
when a mass is palpable at a site distant from the aorta,
or when calcification of the aortic wall is not evident on
plain films of the abdomen (17,18,21). All three conditions were operative in this patient and interfered with
antemortem diagnosis. Furthermore, the presence of
signs of inferior vena caval obstruction and erosion of
the vertebral bodies suggested an intraabdominal malignancy.
Aortic aneurysms may produce erosions of the
anterior portions of the vertebrae, most commonly at
the lower thoracic and upper lumbar spine (22). More
extensive erosion of the lumbar spine has been reported
with leaking AAA (19,20). In our case, the exuberant
lumbar hyperostosis did not protect the vertebral bodies
from the erosive forces of the leaking aneurysm. Rather
its disappearance was the earliest radiologic sign of rupture. Diffuse idiopathic skeletal hyperostosis is a slowly
progressive process. Spontaneous regression has not
been reported (2,5,8,11).
Infection of abdominal aortic aneurysms is
thought to increase their propensity to rupture (23). Although recurrent leaks from this type of aneurysm can
cause fever and leukocytosis simulating a septic process
(24), in our patient, Bacteroidesfragilis bacteremia was
documented in two of three febrile episodes. The autopsy failed to determine the source of bacteremia.
There was no demonstrable communication between
the aneurysm or false aneurysmal cavity and the gastrointestinal tract, which is the most common portal of entry (25).
The significance of this case is twofold. First, a
leaking abdominal aortic aneurysm should be considered in the differential diagnosis of severe lumbar back
pain, particularly in a man in his sixth or seventh decade. Secondly, in the presence of DISH, the disappearance of lumbar hyperostosis represents an early
sign of rupture which may occur prior to vertebral body
erosion. The mortality of patients with ruptured AAA is
still high (14,15,17,26), but the chances of survival are
increased by early operation (14,17). The opportunity
BRIEF REPORTS
802
for early diagnoses, before the catastrophic effects of
massive bleeding, is apparent.
Acknowledgments. Our thanks to Ms Pauline
Louis and Mr. James Atkinson for their technical assistance.
REFERENCES
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films. N Engl J Med 265:12-15, 1961
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