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Eradication of advanced pelvic hydatid bone disease after limb salvage surgery – 5-year follow-up: a case report
© Khan et al.; licensee BioMed Central. 2015
Received: 5 October 2014
Accepted: 10 December 2014
Published: 21 April 2015
Echinococcosis is produced by the larval stage of Echinococcus granulosus; it is a parasitic disease which is seen rarely in humans and has adverse outcomes. We report a case of advanced pelvic hydatid bone disease with successful limb salvage surgery. Our patient had a 5-year follow-up without recurrence which is a rarity as per the literature. Early diagnosis and prompt medical therapy are necessary for effective management whereas delayed diagnosis is always fraught with the risk of recurrence and sepsis.
In 2009, a 30-year-old woman, native of Karachi (Sindhi ethnicity), presented at our clinic with history of a pathological fracture 11 years earlier. Her fracture was initially misdiagnosed and fixed. Subsequently she had persistent disease that progressed with time. Following this she underwent multiple surgeries and the diagnosis of hydatid disease was made but despite multiple debridements and medical therapy she was not cured and finally she was offered a hemipelvectomy (limb sacrifice). On presentation to our hospital she was counseled regarding options of hemipelvectomy versus a limb salvage form of modified internal hemipelvectomy and wide margin resection. She opted for limb salvage. She underwent internal hemipelvectomy with wide margin resection of soft tissue and proximal femur along with postoperative albendazole therapy. She was able to walk again after a very long period. Currently she is 5-years postreconstructive surgery. She is infection free and ambulant without support.
Hydatid bone disease is a rare entity in our part of the world but a careful history and thorough look at the initial images of our patient would have led to the suspicion of pathologic fracture and subsequent early diagnosis of this difficult problem. A second important learning point in this case was the lack of early referral to a center where this difficult problem could have been handled effectively. This could have minimized the physical, mental and financial stress to the patient and her family.
Hydatid cyst disease is a parasitic disease caused by a cestode known as echinococcus. The genus Echinococcus includes three species of which Echinococcus granulosus is the most common cause of hydatid disease in humans [1–3]. The tapeworm resides in the small bowel of the hosts and infected ova are shed in the feces. When ingested by intermediate hosts such as humans, sheep, or cattle, the larvae enter the portal circulation. The larvae eventually reach the liver; where most of them are trapped. Sometimes, larvae reach the lungs and other areas of the body and form cysts. The strong structure of osseous tissue limits the growth of the hydatid cyst, which spreads along medullar and trabecular channels . The disease affects the long bones, vertebral column, pelvis, and costae in order from least to most affected region [1, 5]. Hydatid bone disease is rare, approximately 0.5 to 2.5% of all human hydatidosis . Although long-term survival is possible, the disease is not easy to eradicate and may be impossible to cure [7–9]. Hydatid bone disease is essentially the disease of the young. Early diagnosis is primarily based on X-ray findings which are not specific to the disease. Patients usually present at an advanced stage of the disease and, therefore, treatment is difficult and recurrence is common. It is a serious disease which is difficult to eradicate because of complex and difficult resection. We present a patient who was initially misdiagnosed and underwent multiple surgeries. She is in our follow-up (more than 5 years now) and until now she is infection and disease free.
Hydatid pelvic disease of the bone is considered to be the disease of the young. Diagnosis is primarily based on the findings of X-rays and computed tomography (CT) scans [9–11]. Hydatid bone disease should be considered in any differential diagnosis of osteolytic lesions, especially in endemic regions. The location is mostly hepatic (75%) and pulmonary (15%), and only 10% occur in the rest of the body. Primary skeletal involvement seldom occurs. Bone involvement is seen in only 1 to 2.5% of cases of hydatidosis  and surprisingly musculoskeletal lesions of cystic echinococcosis usually occur as isolated findings and without concomitant hepatic or pulmonary involvement [12, 13]. Nevertheless, the involvement of other organs should be ruled out in any patient with bone hydatidosis. The spine is the most common location for about 50% of osseous hydatidosis, followed by pelvis and hip, the femur, the tibia, the ribs and the scapula . Hydatid disease of bone usually remains asymptomatic over a long period, and it is usually detected after a pathological fracture or secondary infection or the onset of compressive symptoms on adjacent soft tissues. The clinical manifestation may take 10 to 20 years to become obvious, since the cyst grows very slowly. The most common radiological manifestation of skeletal hydatid disease is of a lucent expansile lesion with cortical thinning . The CT appearance of a bone lesion is a well-defined, typically multiloculated osteolytic lesion sometimes with coarse trabeculae within it, giving a honeycomb appearance, which is accompanied by expansion of the bone and thinning of its cortex . The MRI signal intensity pattern of the daughter cysts reflects their contents and may vary in cysts that are dead or alive. The production of hydatid fluid stops when they disintegrate at death . MRI is also helpful in delineating the soft tissue extent of the disease. Immunodiagnosis is useful not only in primary diagnosis but also for follow-up of patients after surgical or pharmacological treatment . Detection of circulating Echinococcus granulosus antigens in serum is less sensitive than antibody detection, which remains the method of choice . Enzyme-linked immunosorbent assay, indirect hemagglutination antibody assay, latex agglutination test, and immunoblot test are the most commonly used immunological methods. Theoretically, surgery with a broad safety margin is the best treatment for bone hydatidosis ; however, most times this recommendation is impossible. For example, in most common sites of hydatid disease of bone, spine and pelvis, radical resection of the lesion is practically impossible . Comparable data have been collected on the outcome of chemotherapy with benzimidazole carbamate (albendazole) that show encouraging results . Albendazole sulfoxide is better absorbed with higher levels of active metabolite in the cysts compared with other benzimidazoles . Treatment with albendazole is effective, but at least one cycle should be given before operation and six or more courses afterwards . In our study, our patient underwent multiple surgeries before presenting to our institute, which reflects the importance of diagnosing the disease early because delay in diagnosis and inadequate management, as happened in this case, leads to further advancement of disease and psychological stress to the patient and his or her family. The natural course of the hydatid disease was explained to our patient including the chances of recurrence of infection. She underwent wide margin excision and stabilization of proximal femur defect with free vascularized fibula along with LCP. She had a follow-up of 60 months and is infection and disease free until now.
Hydatid bone disease is a rare entity in our part of the world but a careful history and thorough look at the initial images of our patient would have led to the suspicion of pathologic fracture and subsequently early diagnosis of this difficult problem. A second important learning point in this case was the lack of early referral to a center where this difficult problem could have been handled effectively. This could have minimized the physical, mental and financial stress to the patient and her family.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Muhammad Shahid Khan: former resident, Department of Surgery, Section of Orthopaedics, Aga Khan University Hospital, Karachi, Pakistan.
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