Download Extensive hydatidosis of the femur and pelvis with pathological

International Journal of Infectious Diseases (2009) 13, e480—e482
http://intl.elsevierhealth.com/journals/ijid
CASE REPORTS
Extensive hydatidosis of the femur and pelvis with
pathological fracture: A case report
Ramchander Siwach a, Roop Singh a,*, Virender Kumar Kadian a,
Zile Singh a, Mantu Jain a, Harnam Madan a, Sunita Singh b
a
b
Department of Orthopaedic Surgery, Paraplegia and Rehabilitation, Pt B.D. Sharma PGIMS, Rohtak 124001, Haryana, India
Department of Pathology, Pt B.D. Sharma PGIMS, Rohtak, Haryana, India
Received 9 September 2008; received in revised form 11 December 2008; accepted 26 December 2008
Corresponding Editor: William Cameron, Ottawa, Canada
KEYWORDS
Hydatid cyst;
Pelvis;
Femur
Summary
Background: Hydatid cysts caused by Echinococcus sp can produce tissue cysts anywhere in the
body. Skeletal cystic lesions are rare, yet because of their unusual presentation diagnosis can be
missed.
Case report: We report a case of extensive hydatidosis of the femur with pathological fracture
and involvement of the pelvis bone, without involvement of abdominal viscera, in a 51-year-old
woman. The patient presented with swelling and deformity of the upper and middle third of the
left thigh. The diagnosis was confirmed clinico-radiologically and the patient was treated with
hindquarter amputation and chemotherapy. The patient died of sepsis and extensive bedsores
one month after surgery.
Conclusions: Orthopedic surgeons should be alert to this morbid condition and this disease should
be suspected in cystic lesions affecting any organ of the body in pathological fractures with nonunion, especially in endemic areas of the world. Early diagnosis helps in eradication and salvage of
the bone; misdiagnosis and delayed diagnosis are always fraught with the danger of amputation,
recurrence, and sepsis.
# 2009 International Society for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.
Introduction
Hydatid disease (echinococcosis) is endemic in many parts of
the world, particularly in sheep-raising districts of Australia,
South America, Central Asia, the Middle East, and the Mediterranean countries. In cases of hydatid cysts the incidence
* Corresponding author. Tel.: +91 1262 213171.
E-mail address: [email protected] (R. Singh).
is 59—75% in the liver, 27% in the lung, 3% in the kidney, and
1—2% in the brain.1 Bone involvement in hydatidosis occurs in
less than 1% of the patients,2,3 yet it is the most debilitating
form of echinococcosis in humans. Although compatible with
long-term survival, the disease is not easy to eradicate and
perhaps impossible to cure.2 Skeletal involvement is usually
secondary to hepatic or pulmonary hydatidosis, however it
may on occasion occur as the primary disease. Cases have
been reported in the vertebrae, the femur, the tibia, and the
pelvis.4—7 Intraosseous lesions usually began at the epiphysis
1201-9712/$36.00 # 2009 International Society for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.
doi:10.1016/j.ijid.2008.12.017
Extensive hydatidosis of the femur and pelvis
e481
and may be polycystic or may occur, though less frequently, in
the form of a solitary hydatid cyst.2 The polycystic type
occurs because the cyst is unable to expand and fragments,
causing diffuse spreading of the daughter cyst and scolices
along the bone canals owing to bone rigidity. In both types of
hydatid cyst, destruction of bone occurs through three
mechanisms: compression, ischemia, and osteoclast proliferation around the compressed bone tissue causing thinning
and fracture and extension into soft tissues.2 We present in
this paper a case of extensive hydatidosis of the femur and
pelvis with pathological fracture of the femur.
Case report
A 51-year-old female was referred to our tertiary center with
complaints of pain, swelling and deformity of the upper and
middle third of the left thigh of two-year duration. She was
anemic, in poor general condition, and had a sacral bedsore.
Local examination revealed flexion contracture of the left
hip and knee. Diffuse swelling was present at the left mid
thigh; painful abnormal mobility was present in the left mid
thigh. X-ray revealed a segmental pathological fracture of
the left femur with multiple osteolytic lesions with honeycomb appearance, narrow transitional zone without reactive
bone in the whole of the left femur, and left hemipelvis with
complete resorption of the femoral head and neck. Soft
tissue shadows with multiple punctuate opacities were also
noted (Figure 1).
Abdominal sonography did not reveal hepatic or other
visceral involvement. Ultrasound of the neck showed no mass
lesion in the thyroid or parathyroid. A computed tomography
scan revealed multiple lytic expansile lesions involving the
left hemipelvis and sacrum, the spinal canal, and the whole
of the left femur with pathological fracture. A break in the
cortex was seen at multiple places in the pelvis and femur
(Figure 2).
Laboratory findings showed raised eosinophils with an
erythrocyte sedimentation rate of 35 in the first hour and
hemoglobin of 8 g%. A hemagglutination test was positive.
Hindquarter amputation was performed and histopathology
of the resected specimen revealed the characteristic trila-
Figure 1 Plain radiograph showing intraosseous cystic lesions
in the left pelvis and femoral bones, destroyed hip joint, and
pathological fracture of the femur.
Figure 2 Computed tomography scan showing multiple cystic
lesions involving the pelvic cavity and ileum.
mellar hydatid cyst wall and scolices of Echinococcus granulosus scattered amidst fragments of bone and bone marrow
(Figure 3). The patient was given albendazole 10 mg/kg/day
and measures were taken to improve her general condition.
However, the patient died after one month due to sepsis and
extensive bedsores.
Discussion
Hydatid disease is caused by the tapeworm Echinococcus.
Species of the genus Echinococcus include Echinococcus
vogeli, Echinococcus granulosus and Echinococcus multilocularis. In man and domestic animals, this parasitic infection is
most commonly caused by the larval stage of Echinococcus
granulosus.2,3 The adult worm resides in the intestine of the
canine, which functions as a definitive host. Ingestion of ova
passed with the feces from the definitive host by man and
domestic animals as intermediate hosts, hatch in the small
intestine and enter the blood circulation, locating in different
tissues where they produce hydatid cysts.3 These cysts commonly occur in the lungs and liver, but can be found in any other
organ or tissue including bones, spleen, heart, eye, brain, and
genitourinary tract. Anatomoclinical changes are peculiar to
localization in the bone.2 From the anatomopathologic stand
Figure 3 Microscopy confirms the diagnosis and reveals classic
hydatid cyst (H&E, 200).
e482
point, this localization marks the torpid, insidious progression
of the parasite into the bone tissue, leading to a diffuse,
extensive, invasive process; so from the clinical stand point,
wherever it is localized, its complete surgical eradication is
rarely possible.2 Hydatid cysts of bone remain asymptomatic
over a long period, and are usually detected after a pathologic
fracture or secondary infection, or following the onset of
compressive myelopathy in cases of vertebral lesions.4 However, a definite preoperative diagnosis without histological
examination is often difficult as there are no pathognomonic
signs, radiological findings may be confused with those of other
tumoral lesions, and serological tests are of limited value.2
When a pathological fracture occurs in long bones due to
hydatid cyst, non-union is common. The present case might
have been harboring a symptomatic infestation for quite a long
time and it was only the pathological fracture with non-union
that brought the patient to us for treatment. Echinococcus
joint disease is usually due to secondary extension from an
adjacent bone. Transarticular extension from the pelvic bone
to the femur or sacrum, similar to the present case, has been
reported in the literature.8,9
The threat of anaphylactic reaction to the cyst fluid and
spread of infection to the surrounding structure and soft
tissues, sometimes by the seeding of hooklets, adds anxiety to both open biopsy and definitive surgery. Difficulty in
both diagnosis and management are hallmarks of hydatidosis of bone. The disease mimics chronic osteomyelitis,
fibrous dysplasia of bone osteosarcoma, benign cystic
lesion of bone, brown tumor (hyperparathyroidism), and
various other neoplastic lesions.10—12 It should be considered in the differential diagnosis of expansile osteolytic
lesions, especially in endemic areas.12,13 A conclusive
diagnosis of hydatidosis has only been reached in half of
the cases preoperatively.4 The indirect hemagglutination
test is more reliable in diagnosing the condition than the
Casoni skin test or Weinberg’s complement fixation
test.8,14 Histopathological examination is confirmatory.12
The anaphylactic nature of the cyst fluid demands strict
precautions and skillful handling during biopsy and definitive surgery.
Osseous hydatidosis should be treated with radical resection with a wide margin of healthy tissue. This may be
difficult, but incomplete removal results in recurrence.15
Radical resection in the pelvis and hip is extremely challenging,4,8,15 and total eradication of parasitic osteitis is almost
impossible.15 Reconstructive surgeries after radical excision
are almost technically impossible in the pelvis and hip,
although in the past, hip arthroplasty3,7 and custom-made
prosthesis4,16 have been tried. Extensive surgical
approaches are always accompanied by the dangers of
recurrence and infections. Even patients in a good general
condition may not tolerate such surgeries. Sepsis may be a
cause of death.7,15 Overall, a review of the literature reveals
a poor prognosis if the disease is extensive in the pelvis and
femur.10,11,13,15
R. Siwach et al.
The purpose of this article is to alert orthopedic surgeons
of this morbid condition and to emphasize the fact that this
disease should be suspected in cystic lesions affecting any
organ of the body in pathological fractures with non-union,
especially in endemic areas of the world. Early diagnosis
helps in eradication and salvage of the bone; misdiagnosis
and delayed diagnosis are always fraught with the danger of
amputation, recurrence, and sepsis.
Conflict of interest
No grants have been received for this study. No people or
organizations are associated with this study.
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