Bulletin • Hospital for Joint Diseases
Volume 60, Number 2
2001-2002
89
Hydatid Disease of the Left Femur
A Case Report
Yilmaz Tomak MD Nevzat Dabak MD Birol Gulman MD Turgut Nedim Karaismailoglu MD
Tarik Basoglu MD and Lütfi Incesu MD
Abstract
Osseous hydatid disease is a rare but serious condition.
Treatment is difficult because of the progressive course
of the bone involvement and generally admitted algorithm about osseous hydatid disease. We report a sixyear follow-up of a case with involvement of the left femur, treated with an unconnected surgical method and
albendazole. In this patient a 1 cm segment of the cortex
between the trochanteric region to supracondylar area
of the left femur was removed. The medullary cavity of
the left femur was irrigated for 5 minutes with 20% hypersaline solution, and removed without causing any
damage. The medullary cavity was curettaged meticulously and irrigated for 6 minutes with 0.9% saline solution. The bone defect was filled with bone cement.
Albendazole was administered during the postoperative
period. At the sixth year postoperatively, the patient was
pain free. All serological tests were normal. Radiologic
evaluation showed no evidence of disease recurrence.
Meticulous preoperative planning, excision of all the
cysts, and an effective regimen of chemotherapy will reYilmaz Tomak, M.D., is from the Department of Trauma and
Orthopaedic Surgery, Ondokuz Mayis University, Faculty of
Medicine, Samsun, Turkey. Nevzat Dabak, M.D., is from the
Department of Trauma and Orthopaedic Surgery, Ondokuz Mayis
University, Faculty of Medicine, Samsun, Turkey. Birol Gulman,
M.D., and Turgut Nedim Karaismailoglu, M.D., are from the
Department of Trauma and Orthopaedic Surgery, Ondokuz Mayis
University, Faculty of Medicine, Samsun, Turkey. Tarik Basoglu,
M.D., is from the Department of Nuclear Medicine, Ondokuz
Mayis University, Faculty of Medicine, Samsun, Turkey. Lütfi
Incesu, M.D., is from the Department of Radiology, Ondokuz
Mayis University, Faculty of Medicine, Samsun, Turkey.
Reprint requests: Yilmaz Tomak, M.D., Ondokuz Mayis University Medicine Faculty, Department of Orthopaedics and Trauma
Surgery, 55139, Kurupelit - Samsun, Turkey.
duce recurrence. Bone scintigraphy is an important diagnostic method during the follow-up period.
H
ydatid cyst disease is a parasitic disease caused
by a cestode known as echinococci. The genus
Echinococcus includes three species;
Echinococcus multilocularis, Echinococcus vogeli and
Echinococcus granulosus. Echinococcus granulosus is the
most common cause of hydatid disease in man.1-3 The
definitive hosts are dogs, foxes, and other carnivores. The
tapeworms live in the small bowel of these hosts and
infected ova are shed in the feces. When ingested by
intermediate hosts such as man, sheep, or cattle, the larvae
enter the portal circulation. The larvae eventually reach the
liver, where of them most are trapped. Sometimes, larvae
reach the lungs and other areas of the body and form cysts.
The life cycle is completed when the definitive hosts
consume infested viscera of the intermediate host.1,2
The primary hydatid disease of the bone, caused by
Echinococcus granulosus is formed when the scoleces
are localized in the bone, and it is seen in 1% to 2.4 % of
the cases.1,4 The strong structure of osseous tissue limits
the growth of the hydatid cyst, which spreads along medullar and trabecular channels. The trabeculae are slowly
resorbed due to pressure without any cortical extension.
The cysts extend to surrounding soft tissues if the bone
cortex is eroded. The disease affects long bones, vertebral column, pelvis, and costae in order from least to
most affected region.1,5 In general, the patients present
to clinicians with local pain, swelling, or a pathologic
fracture.
Giant cell tumors, solitary bone cysts, aneurysmal
bone cysts, fibrous dysplasia, bone metastasis, neurofibromatosis, and tuberculosis of the bone should be considered in the differential diagnosis of osseous hydatid
disease.4 More recently, special diagnostic techniques
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Figure 2 Coronal T1-weighted MRI of the left femur, showing a
large bone and adjacent soft-tissues hypointensity extending from
the left femoral neck to the whole diaphysis.
Figure 1 Anteroposterior radiograph of the left femur showing
cystic lesions extending from left femoral neck basis to the supracondylar area of the left femur. There are multiple calcifications on the trochanteric region and sclerosis and destructions
along diaphysis.
such as computed tomography (CT) and magnetic resonance imaging (MRI) have been used for the differential diagnosis and accurate assessment of the extent of
the disease. 2,6-8 We present the six-year follow-up of a
case with involvement of the left femur, treated with an
alternative surgical method and albendazole. We also
used bone scintigraphy together with CT and MRI.
Case Report
A 17-years-old female patient was admitted to the outpatient clinic suffering from persistent pain of four
months duration that she described as dull in nature and
extending from the left knee to the hip. A biopsy had
been previously performed at another hospital and the
diagnosis was “hydatid cyst in the bone tissue.” The
patient’s history and the physical examination were normal. The erythrocyte sedimentation rate (ESR) and Creactive protein (CRP) was 80 mm/h (N: 0-15 mm/h),
and was 48 mg/dl (N: 0-6 mg/dl), respectively. Other
biochemical values were normal. The Echinococcus latex agglutination test was 1/256 + titers (N: 0/128). Radiographs showed cystic lesions extending from the left
femoral neck to the supracondylar area of the left femur. Small calcifications in the trochanteric region and
sclerosis as well as destruction of the diaphysis was observed (Fig. 1). Large cystic lesions filling the medullary cavity caused destruction and expansions in the cortex, extending from the trochanteric region to distal of
the diaphysis, as demonstrated on a CT axial section of
the left femur. Coronal T1-weighted MRI showed a large
bone and adjacent soft-tissue hypointensity extending
from the left femoral neck to the entire diaphysis (Fig.
2). Scintigraphy demonstrated that the osteoblastic activity was increased from the left femoral neck to supracondylar area (Fig. 3).
At surgery, the left femur was reached through a lateral longitudinal incision from trochanteric region to the
above-knee level. After retraction of the soft tissues with
Hohman retractors, a 1 cm width of the cortex was removed between the trochanteric region to the supracondylar area of the left femur. The entire medullary
cavity including the femoral neck was irrigated for 5
minutes with 20% hypersaline solution, and was removed
atraumatically without causing any damage to the germinative membrane. The medullary cavity was then
curetted meticulously and irrigated for 6 minutes with a
0.9% saline solution that had been warmed to 90° C.
The defect in the left femur was filled with bone ce-
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91
Figure 3 Bone scintigraphy of the left femur, showing increased
osteoblastic activity extending from the left femoral neck to supracondylar area.
ment. Suction drains were removed 48 hours postoperatively.
Pathological examination revealed that the cuticular
membrane had a lamellar, acellular, homogenous eosinophilic appearance. In addition, soft tissue pieces showing fibroblastic proliferation, inflammatory cells, and infiltrated giant cells were observed. The pathological
diagnosis was “hydatid disease of the bone tissue.”
Postoperative treatment started with albendazole 10
mg/kg/day administered orally. Partial weightbearing
was permitted at the end of second month. Full weightbearing was permitted at end of third month. At the end
of the 18th month administration of albendazole administration was discontinued.
The physical examination performed six years postoperatively was normal. The patient was pain free. On
control radiographs, a hypodense area approximately 1
cm thick (bone cement filling the defective area) was
seen between the trochanteric region and supracondylar
area of the left femur. Destruction was not observed although expansion and thinning were seen in some areas
of the cortex (Fig. 4). Increased osteoblastic activity and
no recurrence of disease can be seen on the bone scintigraphy that was also performed at this six-year followup (Fig. 5). Control ESR 10 mm/h and Echinococcus
Figure 4 Anteroposterior radiograph of the left femur six years
later, showing bony healing and no destructions. Although, expansion and thinning in some areas of the cortex can be seen.
Figure 5 Bone scintigraphy of the left femur six years later,
showing no increased osteoblastic activity or recurrence.
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Volume 60, Number 2
latex agglutination test was 1/ 10 titers.
Discussion
The radiological signs of the osseous hydatid disease
include lucent lesions in the bone that are associated with
bone expansion and thinning of the cortex. In patients
with these signs, soft tissue calcifications are highly suggestive of hydatid disease. 6-8 CT and MRI are important
in determining the accurate extent of the bone and soft
tissue abnormalities.
The literature on bone scintigraphy was reviewed but
no report concerning osseous hydatid disease was found.
However, there are some reports about hydatid disease
in the liver and the chest.9-11 These reports emphasize
that in a great percentage of cases scintigraphy shows
accurate localization, dimension of the cysts, and assists
the surgeon by helping establish the most appropriate
surgical approach to the cyst thus reducing the danger
of spilling its content. According to us, the bone scintigraphy is also the most significant method for the purposes of differential diagnosis; additionally it delineates
the accurate extent of the bone lesions and is also useful
in assessing recurrence during the postoperative period.
Scintigraphy should be employed for the differential diagnosis, preoperative planning, and in the follow-up period of the osseous hydatid disease.
There is no generally accepted treatment algorithm
for osseous hydatid disease. Cases of osseous hydatid
disease are rare and treatment methods are generally
unsuccessful. The literature offers various treatment approaches.2-4,12 Until recently, the basic treatment has been
surgical excision or resection. Unfortunately, the results
have been discouraging.6,8 Surgical excision and curettage are the basic approach for osseous hydatid disease.
Only, macroscopic cysts can be extracted with surgical
excision and curettage. The surgical area might be irrigated with chemical agents in an attempt to kill scoleces
(formalin, 0.5% silver nitrate, hyper-saturated saline
solution, 0.9% saline solution at high temperature). Unfortunately, chemically cleaning with these agents cannot abolish all microscopic female scoleces. This kind
of treatment is generally palliative, and recurrences are
frequent. 2,8
The combination of chemotherapy and surgical treatment has been found to be more efficient than surgical
treatment alone. Mebendazole, albendazole, and
antihelmintic drugs are used for chemotherapy.
Albendazole has been found to be better absorbed than
mebendazole and exhibits superior efficacy against helminths.2,13 Bonifacino and colleagues13 administered oral
albendazole (15 mg/kg/day) in 28-day cycles. The efficacy of chemotherapy was assessed by serological tests
for circulating antigen, immunocomplexes and specific
antibodies, and by radiography and CT scans. They concluded that treatment with albendazole is effective, but
2001-2002
one cycle should be given before operation and six or
more courses afterward.
Uzel and associates 3 stated that Echinococcus
granulosus infestation of the bone is a progressive tumor, similar to a bone tumor. They emphasized that bone
cement and local heating applied after curettage are an
effective method for treating this disease. They applied
this method in the treatment of two cases – one case was
cured after 12 months the other after 30 months. However, these investigators saw the recurrence of fistula
formation in 6 cases in which treatment with chemical
agents and curettage had been applied.
We also think that for achieving successful results in
the treatment of the osseous hydatid disease, this disorder should be considered as a tumor of progressive nature. The treatment algorithm must take into consideration this thesis. The first step in the treatment should
be meticulous preoperative planning that includes special diagnostic techniques such as CT, MRI, and bone
scintigraphy. In this way, the extent of the bone lesions
can be determined precisely. This approach reduces the
possibility of the recurrence. The second step is an aggressive curettage of the affected bone after irrigation
with 20% hyper-saturated saline solution at least 5 minutes and then irrigation for at least 5 minutes with 0.9%
saline solution heated to 90° C. Then, after irrigation,
bone cement is applied to the defect, which has the additional beneficial effect of heating and toxic polymerization. The third and last step is an effective chemotherapy program for at least 12 months. It should not
been forgotten that osseous hydatid disease can recur
any time. Therefore, patients without any symptoms
should be followed for the long-term and serological
tests, radiographs, and bone scintigraphy should be used
periodically to assure that the disease has not recurred.
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