International Journal of Infectious Diseases 18 (2014) 57–61
Contents lists available at ScienceDirect
International Journal of Infectious Diseases journal homepage: www.elsevier.com/locate/ijid
Treatment experiences of pelvic bone hydatidosis§ Qiuzhen Liang a, Hao Wen b, Akbar Yunus a, Zheng Tian a, Fei Jiang a, Xinghua Song a,* a b
Department of Orthopaedics, First Affiliated Hospital of Xinjiang Medical University, Urumqi, Xinjiang 830054, China Department of General Surgery, First Affiliated Hospital of Xinjiang Medical University, Urumqi, Xinjiang, China
A R T I C L E I N F O
S U M M A R Y
Article history: Received 3 June 2013 Received in revised form 10 September 2013 Accepted 11 September 2013
Background: Hydatid diseases of the pelvic bone are rare, generally incurable, and have high rates of recurrence. Methods: We report nine cases of osseous hydatidosis involving the pelvis. Patients were treated through different surgical options, including simple debridement, bone cement filling with or without internal fixation, reconstruction using a pedicle screw-rod system, and hemipelvic replantation after liquid nitrogen devitalization combined with femoral prosthesis replacement. The therapy was completed by medical treatment or radiotherapy. Results: The average follow-up was 7.3 years (range 2–19 years). Whilst five patients were symptomfree at the last follow-up, the remaining patients suffered from hip pain or productive sinuses. Conclusions: Patients must be treated at the early stages of the disease. Total exeresis and individual reconstruction is necessary when osseous and soft tissue involvement is not extensive. Internal fixation should not be used when surgical eradication is uncertain. ß 2013 The Authors. Published by Elsevier Ltd on behalf of International Society for Infectious Diseases. All rights reserved.
Corresponding Editor: Eskild Petersen, Aarhus, Denmark Keywords: Bone hydatidosis Pelvis Treatment
1. Introduction
2. Patients and methods
Hydatid disease is a rare pathology, but relatively common in the Mediterranean, the Middle East, Central Asia, and East Africa.1 It may develop in almost any part of the body, although most hydatid cysts occur in the liver or in the lung.2,3 Bone involvement is rare, and accounts for only 0.5% to 4% of all locations.3 The most commonly described sites for hydatid cysts of the bone are the vertebrae, the long bones of the lower limbs, and the pelvis.4 According to Zlitni et al.,5 echinococcosis of the pelvis accounts for 28% of all cases of osseous hydatidosis. The only curative treatment for osseous hydatidosis is surgery, and the surgical options for this disease better resemble oncologic therapy than the usual surgical treatment for visceral hydatid cysts.5 Patients often refuse hemipelvectomy because of the potential for functional loss. Thus, different surgical methods for individual reconstruction have been developed.6 We report here our experience in treating nine patients with osseous hydatid disease of the pelvis.
We treated nine patients with hydatid disease involving the pelvis between 1995 and 2011 (Table 1). The patients were three men and six women, with a mean age of 32.0 years (range 14–52 years). Plain radiography, computed tomography (CT), and magnetic resonance imaging (MRI) were used in all of the patients to establish a diagnosis and to assess the status of their visceral organs. Laboratory testing for echinococcosis was performed using eight immunodiagnosis tests, with ELISA and the gold-label method (dot immunogold filtration assay, DIGFA). These tests can detect four types of antigens, namely Echinococcus granulosus crude hydatid cyst fluid antigen (EgCF), E. granulosus protoscolex antigen extract (EgP), hydatid cyst fluid native antigen B (EgB), and Echinococcus multilocularis metacestode laminated layer extract (Em2). The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level were also determined for the nine patients. Three patients presented with infection of the ilium, one presented with infection of the acetabulum, one presented with infections of the ilium and sacrum, another presented with infections of the ilium and sacroiliac joint, two presented with infections of the ilium and acetabulum, and one presented with infections of the hemipelvis and greater trochanter. The majority of the patients presented with pain in the hip or gluteal region, and one patient (patient 7) presented with sciatica. Four patients (patients 6–9) presented with restricted hip motion.
§ This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial-No Derivative Works License, which permits non-commercial use, distribution, and reproduction in any medium, provided the original author and source are credited. * Corresponding author. Tel.: +86 15199189693; fax: +86 991 4366792. E-mail address: [email protected] (X. Song).
1201-9712/$36.00 – see front matter ß 2013 The Authors. Published by Elsevier Ltd on behalf of International Society for Infectious Diseases. All rights reserved. http://dx.doi.org/10.1016/j.ijid.2013.09.010
Q. Liang et al. / International Journal of Infectious Diseases 18 (2014) 57–61
58 Table 1 Characteristics of the patients Patient
Age, years/sex
Location
Treatment
1
29/M
Ilium, ischium, pubis, hip, and greater trochanter
2 3
26/F 24/F
Ilium Ilium and acetabulum
4 5
45/F 31/M
Acetabulum Ilium
6 7
52/M 14/F
Ilium Ilium and sacrum
8 9
35/F 32/F
Ilium and acetabulum Ilium and sacroiliac joint
One debridement, one hemipelvis replantation after liquid nitrogen devitalization with femoral prosthesis replacement, chemotherapy Four debridements and chemotherapy Two debridements and one bone cement filling, chemotherapy Internal fixation with bone cement filling Two debridements with radiotherapy, chemotherapy Numerous debridements Numerous debridements, one screws and rods fixation, chemotherapy Five debridements and chemotherapy Three debridements with radiotherapy, chemotherapy
Status of liver and lung
Outcome
7
Uninfected
Symptom-free
9 5
Hepatic infestation Uninfected
Symptom-free Symptom-free
2 6
Uninfected Uninfected
Symptom-free Symptom-free
7 19
Hepatic infestation Uninfected
7 4
Hepatic infestation Uninfected
Hip pain Walking difficulty, productive sinuses Hip pain Walking difficulty
Follow-up, years
F, female; M, male.
Five patients underwent simple debridement (patients 2, 5, 6, 8, and 9) because of the extent of disease involvement and the destruction of soft tissue barriers prior to admission to our hospital. Patient 4 underwent reconstruction with two plates fixed to the lateral parts of the iliac ala and three cortical bone screws fixed to the bone defects along the long axis combined with bone cement filling. Two patients (patients 3 and 4) received bone cement filling after local excision. Patient 1 was diagnosed with hydatid disease after the first debridement in 2005. Imaging studies performed in 2009 revealed multiple cystic images of the right pelvis, including the ilium, acetabulum, ischium, pubis, and greater trochanter; the sacroiliac articulation remained intact. Wide resection was carried out on the involved muscles, and a hemipelvectomy was performed on the lateral parts of the sacroiliac articulation and symphysis pubis combined with femoral osteotomy over the lesser trochanter. The resected hemipelvis was immersed in liquid nitrogen for 20 min. Detected lesions were excised completely after thawing. Four plates and screws were used to stabilize the hemipelvis. A femoral prosthesis was carefully inserted into the acetabulum, which was filled with bone cement (Figure 1). Patient 7 was diagnosed with sacrum echinococcosis disease when she was 14 years old. Numerous debridements were performed on the patient because of the frequent recurrence of the disease. We used a pedicle screw-rod system on the patient in 2004 to reconstruct bone defects and stabilize the pelvic ring (Figure 2). The patients were administered postoperative chemotherapy with albendazole (10 mg/kg/day) for 6–12 months with close observation of the liver enzymes. Two patients (patients 5 and 9) received radiotherapy (Dose of total = 6900 cGy/23 f/30 days). 3. Results All patients underwent MRI scanning, and five out of the nine cases were identified as having hydatid disease. The liver was involved in three patients (Table 1). Nine patients (100%) were positive in the eight immunodiagnosis tests. The average ESR was 18 mm/h (range 3–48 mm/h), and the average CRP was 5.8 mg/l (range 2.3–61.4 mg/l). The final diagnosis was confirmed by histopathological examination of the resected tissue. Resection margins were wide in one patient (patient 1), marginal in two patients (patients 3 and 4), and intralesional in the remaining patients. The mean follow-up was 7.3 years (range 2–19 years). Five patients (patients 1–5) were symptom-free, had no recurrence, and were able to walk unaided at the latest followup; the remaining patients suffered from hip pain, limitation of
movement, or productive sinuses. Five patients required several debridement procedures in combination with chemotherapy or radiotherapy. One of these five patients (patient 7) developed chronic lesions with sinus formation in the lumbosacral region 4 years after reconstructive surgery with a pedicle screw-rod system; she received another debridement with removal of the internal fixator in 2011 (Figure 2). A hemipelvectomy was proposed for patients 6 and 8, but both refused and instead opted for repeated debridement procedures. Radiography of patient 1 in January 2013 showed that the femoral prosthesis and pelvis were in good alignment and osteotomy sites were healed, with no sign of recurrence for 3 years (Figure 1). None of the patients died during the follow-up period. 4. Discussion The progression of hydatid disease is slow, and the symptoms of pelvic localization usually only become evident in adulthood in the form of pain, swelling, claudication, sinus formation, vague abdominal pains, or pressure symptoms involving the adjacent organs.2,5–7 Similar to our observations, the literature reports that sciatica is the first clinical symptom that appears when the pelvic hydatid cyst compresses the lumbosacral nerve plexus.8 It must be emphasized that there are no specific symptoms for pelvic hydatid disease; the mode of presentation is determined by the size of the cyst and any attendant complications. Diagnosis of hydatid disease from pelvic localization by radiological detection may be difficult, and symptoms are often nonspecific. Whilst serological tests are important for the differential diagnosis, their reliability is not 100%.3 The eight immunodiagnosis tests include ELISA and DIGFA. The overall sensitivity of DIGFA is 80.7% for human cystic echinococcosis and 92.9% for human alveolar echinococcosis. The highest specificity was 93.4% with EgB extract for cystic echinococcosis, and 90.3% with Em2 antigen for alveolar echinococcosis when cystic echinococcosis versus alveolar echinococcosis cross-reactivity was excluded. The major advantages of this method include its rapidity, convenience, and ability to provide an initial diagnosis. Differentiation between cystic echinococcosis and alveolar echinococcosis is also possible in approximately 80% of all cases in either the clinical or community setting.9 Blood testing for inflammatory markers has been observed to lack specificity. A definitive diagnosis may be obtained only by histopathological examination of the resected tissue. The only curative treatment for osseous hydatidosis is surgery.5 The treatment of patients by different surgical options has been reported in the literature, including simple drainage or
Q. Liang et al. / International Journal of Infectious Diseases 18 (2014) 57–61
59
Figure 1. A 33-year-old male patient with hydatid disease of the right hemipelvis and ipsilateral greater trochanter. (a) Radiograph of the pelvis in 2009 showing a multilocular lytic lesion involving the right pelvis and greater trochanter. (b) MRI scan showing a shift in signals from the inferior part of the right iliac region to the acetabulum and a lobulated cyst in the soft tissue around the right hip region, as well as the iliac fossa, iliacus and internal obturator muscle. (c) Appearance of the resected hemipelvis and the liquid nitrogen treatment. (d) Most of the scolexes aspirated from lesions in the bone before liquid nitrogen treatment could not be stained for direct microscopic examination (40, eosin stain). By contrast, all of the scolexes obtained after liquid nitrogen treatment were stained (200, eosin stain). (e) Radiograph performed 3 years after surgery showing the femoral prosthesis and pelvis in good alignment.
debridement, complete excision, total hip replacement, large bone grafts, femoropubic arthrodesis, megaprosthetic replacement, massive arthroplasty, osteosynthesis, and hemipelvectomy.10–12 Simple drainage or debridement is suggested as a treatment option that is readily accepted by many patients; however, incomplete removal results in early recurrence and dissemination. The basic technique involves exposure of the lesion and protection of the adjacent normal tissue with gauze soaked in 20% NaCl solution, combined with burnishing of the interior wall of the residual cavity using a high-speed burr. Dexamethasone (10– 20 mg) is injected during the operation to prevent allergic shock. The hydatid peel is removed by aspiration, and the compressed normal tissue or ‘capsule’ wall is cleaned with alcoholic phenyl and soaked in 20% hypertonic NaCl solution for 10 min. Reduction of contamination is achieved by carefully performing curettage and using operative instruments separately. Patient 6 in our study was misdiagnosed with tuberculosis in 2006 and received the first debridement without the protection of adjacent tissues. She has so far undergone eight debridements, and involvement of the sacroiliac articulation was observed at her last follow-up. Patient 1 was initially diagnosed with hip arthritis for right hip pain but was confirmed to have cystic echinococcosis by pathological evaluation after surgical debridement. Imaging studies performed after 4 years revealed an important osseous change, with multiple cystic images in the right pelvis and greater trochanter. Thus, basic techniques must be followed when the diagnosis of echinococcosis cannot be ruled out before operation. Bone cement filling has been proven to be an effective method for reducing the recurrence rate of cysts because the elevation in temperature in the polymerizing cement has a necrotizing effect that may kill daughter cysts.13 No recurrence in the ilium region
was observed in patients who had been treated with bone cement, and their recoveries appeared excellent. Several surgical methods for reconstruction are available. Patient 7 in our study underwent reconstruction with a pedicle screw-rod system. This method is a simple reconstructive procedure that does not require lengthy preoperative customization. However, it cannot guarantee complete cutting of the edge around the sacroiliac joints, and surgery sometimes results in disease recurrence or worse conditions. Martinez et al.6 reported that a hip replacement should probably not be attempted when the destruction of the hip joint is extensive. Neelapala et al.14 proposed that subtotal excision of the cyst along with joint replacement is an acceptable treatment option and that loose components can be successfully revised by this method to achieve good clinical outcomes. Wirbel et al.12 showed that megaprosthetic replacement for hydatid disease results in restricted but acceptable limb function. Kapoor et al.15 reported the formation of bone defects after debridement of the ilium, along with filling of the proximal femur with bone cement and augmentation of the femur using an intra-medullary nail. Eradication of the parasite was not the goal of this surgical treatment; instead, the procedure aimed to promote maintenance of limb function. Gdoura et al.11 observed a case of pelvic bone hydatidosis that required broad surgical resection and reconstruction by femoropubic and sacral arthrodesis; however, the fixation fractured 7 months after surgery. Some complications observed appear to be associated with the large bone defects that occur after radical resection. Thus, we formulated an unusual combination procedure for patient 1, involving replantation of the resected hemipelvis after devitalization and replacement of the femoral prosthesis. We believe that such a combination can provide a desirable bone stock and retain
60
Q. Liang et al. / International Journal of Infectious Diseases 18 (2014) 57–61
Figure 2. A 37-year-old female patient with hydatid diseases of the ilium and sacrum. (a) CT of the pelvis showing an important osseous change of the sacrum in 1999. (b) Radiograph performed 7 years after reconstructive surgery using a pedicle screw-rod system. (c) MRI scan performed in 2012 showing recurrent lesions around the sacrum and pelvic cavity. (d) Appearance of the lumbosacral area in 2012 showing a surgical scar with sinus formation.
sufficient limb function without redundant procedures. The sacroiliac articulation was left intact, and invasion by parasitic formation in the surrounding soft tissues was not extensive. Considering the barriers presented by the pelvic muscles, iliectomy of the healthy bone may give hope of complete surgical eradication. The advantages of liquid nitrogen treatment include simplicity, desirable bone stock, sufficient biomechanical strength, maintenance of osteoinductive and osteoconductive properties, and absence of harmful denatured substances.16,17 Hildreth et al.18 demonstrated the destruction of E. multilocularis eggs by the application of liquid nitrogen for 15 min via in vitro and in vivo experiments. Colli and Williams19 found that eggs from E. granulosus remained infectious for 24 h when stored at 50 8C, but were not infectious when stored at 70 8C. Utilization of liquid nitrogen as a cryogenic agent to sterilize Echinococcus cysts in the bone has yet to be reported. Bone hydatid cysts are different from visceral hydatid cysts; the former lack a fibrous capsule and manifest osteolytic bone destruction, thereby allowing the easier penetration of liquid nitrogen to devitalize the parasite in the damaged bone. In our study, we compared the results of eosin staining before and after treatment with liquid nitrogen by microscopy and found that Echinococcus cysts in the bone may be devitalized by the application of liquid nitrogen for 20 min (Figure 1). The prognosis for pelvic bone hydatidosis depends on whether or not the coxofemoral and sacroiliac articulations are affected. The prognosis is usually poor when the disease is widespread in the basin; surgical eradication becomes uncertain in these cases and extensive mutilation must be accepted.5 Siwach et al.10 reported a case of extensive hydatidosis of the femur and pelvis bone, in which the patient died of sepsis and extensive bedsores 1 month after a hindquarter amputation. A hemipelvectomy was recommended for patients 6 and 8 in our study, but both refused this option because of the potential for functional loss. Therapeutic dilemmas can arise in cases of extended disease, with many muscles or muscle layers in different sites of the body communicating through fistulas.14 One patient in our study received reconstruction with a pedicle screw-rod system, but developed chronic sinus formation after 4 years that did not cease
until the internal fixation had been removed. Therefore, internal fixation should not be used when lesions are hard to resect completely. Most surgeons concur that treatment of osseous hydatidosis better resembles oncologic therapy than the usual surgical treatment of visceral hydatid cysts.5 The wide resection of the involved bone together with the surrounding soft tissue is advocated because incomplete resection confers a great risk of recurrence.20 We believe that the Enneking system for staging malignant musculoskeletal tumors is also instructively significant for pelvic bone hydatidosis because the pelvic regions (including types I, II, III, and IV) indicated for disease onset and malignant tumor progression are similar to those of echinococcosis. However, pelvic bone hydatidosis cannot be fully equated with pelvic malignant tumors because of the following: (a) The invasiveness of malignant tumors is much higher than that of hydatid disease. For example, Mankin et al.21 reported that the mean survival rate of patients with high-grade malignant pelvic tumors is approximately 50%, whereas hydatid disease is rarely life-threatening even with multiple-organ involvement. Masse et al.22 believed that, even without complete eradication, the slow extension of hydatid disease allows a hypothetically wide margin for surgical excision. (b) The age of onset of hydatid disease is generally low and most cases occur in the 15–35 years range; most malignant tumors develop after 45 years of age. As such, most of the patients in this study opted for debridement or reconstruction instead of hemipelvic resection even when the destruction was extensive. A custom-made hemipelvic prosthesis has previously proven to be an effective treatment for malignant tumors, however it shows a relatively short service life in hydatidosis compared with devitalization and replantation. Although surgery is the most effective treatment for hydatidosis, the combination of preoperative albendazole therapy, surgery, and postoperative albendazole therapy has been proven to be an effective regimen. Medical treatment aims at reducing the size of the cysts and sterilizing their content before and after the surgery to allow the treatment of small cysts that are virtually undetectable;23 drug therapy is generally not used as the primary treatment except in cases where the patient is not fit for surgery.
Q. Liang et al. / International Journal of Infectious Diseases 18 (2014) 57–61
Radiotherapy is used as an adjunct to surgery and appears to be a good alternative for patients with contraindications to medication. In conclusion, pelvic bone hydatidosis is a rare disease even in endemic countries. Surgery of the pelvis is difficult and requires meticulous technical preparation. Early diagnosis helps in the eradication and salvage of the bone. The surgeon should be aware that incomplete resection exposes the patient to a greater risk of recurrence than a complete one. We also advocate that treatment of the patient be done during the early stages of the disease. Total exeresis and individual reconstruction is necessary when the osseous and soft tissue involvement is not extensive. Internal fixation should not be used when surgical eradication is uncertain. Conflict of interest: No conflict of interest to declare. References 1. Sapkas GS, Stathakopoulos DP, Babis GC, Tsarouchas JK. Hydatid disease of bones and joints. 8 cases followed for 4–16 years. Acta Orthop Scand 1998;69:89–94. 2. Terek MC, Ayan C, Ulukus M, Zekioglu O, Ozkinay E, Erhan Y. Primary pelvic hydatid cyst. Arch Gynecol Obstet 2000;264:93–6. 3. Song XH, Ding LW, Wen H. Bone hydatid disease. Postgrad Med J 2007;83:536– 42. 4. Duran H, Ferrandez L, Gomez-Castresana F, Lopez-Duran L, Mata P, Brandau D, et al. Osseous hydatidosis. J Bone Joint Surg Am 1978;60:685–90. 5. Zlitni M, Ezzaouia K, Lebib H, Karray M, Kooli M, Mestiri M. Hydatid cyst of bone: diagnosis and treatment. World J Surg 2001;25:75–82. 6. Martinez AA, Herrera A, Cuenca J, Herrero L. Hydatidosis of the pelvis and hip. Int Orthop 2001;25:302–4. 7. Seenu V, Misra MC, Tiwari SC, Jain R, Chandrashekhar C. Primary pelvic hydatid cyst presenting with obstructive uropathy and renal failure. Postgrad Med J 1994;70:930–2. 8. Sanal HT, Kocaoglu M, Bulakbasi N, Yildirim D. Pelvic hydatid disease: CT and MRI findings causing sciatica. Korean J Radiol 2007;8:548–51.
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9. Feng XH, Wen H, Zhang ZX, Chen XH, Ma XD, Zhang JP, et al. Dot immunogold filtration assay (DIGFA) with multiple native antigens for rapid serodiagnosis of human cystic and alveolar echinococcosis. Acta Trop 2010;113:114–20. 10. Siwach R, Singh R, Kadian VK, Singh Z, Jain M, Madan H, et al. Extensive hydatidosis of the femur and pelvis with pathological fracture: a case report. Int J Infect Dis 2009;13:e480–2. 11. Gdoura F, Trigui M, Zribi W, Ellouze Z, Bouzidi R, Ayedi K, et al. Pelvic bone hydatidosis. J Orthop Traumatol Surg Res 2010;96:85–9. 12. Wirbel RJ, Schulte M, Maier B, Mutschler WE. Mega-prosthetic replacement of the pelvis. Function in 17 cases. Acta Orthop Scand 1999;70:348–52. 13. Yildiz Y, Bayrakci K, Altay M, Saglik Y. The use of polymethylmethacrylate in the management of hydatid disease of bone. J Bone Joint Surg Br 2001;83: 1005–8. 14. Neelapala VS, Chandrasekar CR, Grimer RJ. Revision hip replacement for recurrent hydatid disease of the pelvis: a case report and review of the literature. J Orthop Surg Res 2010;5:17. 15. Kapoor SK, Kataria H, Patra SR, Bharadwaj M, Vijay V, Kapoor S. Multi-organ hydatidosis with extensive involvement of the hemi-pelvis and ipsilateral femur. Parasitol Int 2013;62:82–5. 16. Tanzawa Y, Tsuchiya H, Shirai T, Hayashi K, Yo Z, Tomita K. Histological examination of frozen autograft treated by liquid nitrogen removed after implantation. J Orthop Sci 2009;14:761–8. 17. Tsuchiya H, Nishida H, Srisawat P, Shirai T, Hayashi K, Takeuchi A, et al. Pedicle frozen autograft reconstruction in malignant bone tumors. J Orthop Sci 2010;15:340–9. 18. Hildreth MB, Blunt DS, Oaks JA. Lethal effects of freezing Echinococcus multilocularis eggs at ultralow temperatures. J Parasitol 2004;90:841–4. 19. Colli CW, Williams JF. Influence of temperature on the infectivity of eggs of Echinococcus granulosus in laboratory rodents. J Parasitol 1972;58:422–6. 20. Herrera A, Martinez AA. Extraspinal bone hydatidosis. J Bone Joint Surg Am 2003;85:1790–4. 21. Mankin HJ, Hornicek FJ, Temple HT. Malignant tumors of the pelvis: an outcome study. Clin Orthop Relat Res 2004;425:212–7. 22. Masse A, Parola PG, Brach del Prever EM, Gallinaro P. Hydatidosis of the pelvis: a case report with a 25-year follow-up. Arch Orthop Trauma Surg 2004;124:203– 5. 23. Merkle EM, Schulte M, Vogel T, Tomczak R, Reiber A, Kern P, et al. Musculoskeletal involvement in cystic echinococcosis report of eight cases and review of the literature. AJR Am J Roentgenol 1997;168:1531–4.
Contents lists available at ScienceDirect
International Journal of Infectious Diseases journal homepage: www.elsevier.com/locate/ijid
Treatment experiences of pelvic bone hydatidosis§ Qiuzhen Liang a, Hao Wen b, Akbar Yunus a, Zheng Tian a, Fei Jiang a, Xinghua Song a,* a b
Department of Orthopaedics, First Affiliated Hospital of Xinjiang Medical University, Urumqi, Xinjiang 830054, China Department of General Surgery, First Affiliated Hospital of Xinjiang Medical University, Urumqi, Xinjiang, China
A R T I C L E I N F O
S U M M A R Y
Article history: Received 3 June 2013 Received in revised form 10 September 2013 Accepted 11 September 2013
Background: Hydatid diseases of the pelvic bone are rare, generally incurable, and have high rates of recurrence. Methods: We report nine cases of osseous hydatidosis involving the pelvis. Patients were treated through different surgical options, including simple debridement, bone cement filling with or without internal fixation, reconstruction using a pedicle screw-rod system, and hemipelvic replantation after liquid nitrogen devitalization combined with femoral prosthesis replacement. The therapy was completed by medical treatment or radiotherapy. Results: The average follow-up was 7.3 years (range 2–19 years). Whilst five patients were symptomfree at the last follow-up, the remaining patients suffered from hip pain or productive sinuses. Conclusions: Patients must be treated at the early stages of the disease. Total exeresis and individual reconstruction is necessary when osseous and soft tissue involvement is not extensive. Internal fixation should not be used when surgical eradication is uncertain. ß 2013 The Authors. Published by Elsevier Ltd on behalf of International Society for Infectious Diseases. All rights reserved.
Corresponding Editor: Eskild Petersen, Aarhus, Denmark Keywords: Bone hydatidosis Pelvis Treatment
1. Introduction
2. Patients and methods
Hydatid disease is a rare pathology, but relatively common in the Mediterranean, the Middle East, Central Asia, and East Africa.1 It may develop in almost any part of the body, although most hydatid cysts occur in the liver or in the lung.2,3 Bone involvement is rare, and accounts for only 0.5% to 4% of all locations.3 The most commonly described sites for hydatid cysts of the bone are the vertebrae, the long bones of the lower limbs, and the pelvis.4 According to Zlitni et al.,5 echinococcosis of the pelvis accounts for 28% of all cases of osseous hydatidosis. The only curative treatment for osseous hydatidosis is surgery, and the surgical options for this disease better resemble oncologic therapy than the usual surgical treatment for visceral hydatid cysts.5 Patients often refuse hemipelvectomy because of the potential for functional loss. Thus, different surgical methods for individual reconstruction have been developed.6 We report here our experience in treating nine patients with osseous hydatid disease of the pelvis.
We treated nine patients with hydatid disease involving the pelvis between 1995 and 2011 (Table 1). The patients were three men and six women, with a mean age of 32.0 years (range 14–52 years). Plain radiography, computed tomography (CT), and magnetic resonance imaging (MRI) were used in all of the patients to establish a diagnosis and to assess the status of their visceral organs. Laboratory testing for echinococcosis was performed using eight immunodiagnosis tests, with ELISA and the gold-label method (dot immunogold filtration assay, DIGFA). These tests can detect four types of antigens, namely Echinococcus granulosus crude hydatid cyst fluid antigen (EgCF), E. granulosus protoscolex antigen extract (EgP), hydatid cyst fluid native antigen B (EgB), and Echinococcus multilocularis metacestode laminated layer extract (Em2). The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level were also determined for the nine patients. Three patients presented with infection of the ilium, one presented with infection of the acetabulum, one presented with infections of the ilium and sacrum, another presented with infections of the ilium and sacroiliac joint, two presented with infections of the ilium and acetabulum, and one presented with infections of the hemipelvis and greater trochanter. The majority of the patients presented with pain in the hip or gluteal region, and one patient (patient 7) presented with sciatica. Four patients (patients 6–9) presented with restricted hip motion.
§ This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial-No Derivative Works License, which permits non-commercial use, distribution, and reproduction in any medium, provided the original author and source are credited. * Corresponding author. Tel.: +86 15199189693; fax: +86 991 4366792. E-mail address: [email protected] (X. Song).
1201-9712/$36.00 – see front matter ß 2013 The Authors. Published by Elsevier Ltd on behalf of International Society for Infectious Diseases. All rights reserved. http://dx.doi.org/10.1016/j.ijid.2013.09.010
Q. Liang et al. / International Journal of Infectious Diseases 18 (2014) 57–61
58 Table 1 Characteristics of the patients Patient
Age, years/sex
Location
Treatment
1
29/M
Ilium, ischium, pubis, hip, and greater trochanter
2 3
26/F 24/F
Ilium Ilium and acetabulum
4 5
45/F 31/M
Acetabulum Ilium
6 7
52/M 14/F
Ilium Ilium and sacrum
8 9
35/F 32/F
Ilium and acetabulum Ilium and sacroiliac joint
One debridement, one hemipelvis replantation after liquid nitrogen devitalization with femoral prosthesis replacement, chemotherapy Four debridements and chemotherapy Two debridements and one bone cement filling, chemotherapy Internal fixation with bone cement filling Two debridements with radiotherapy, chemotherapy Numerous debridements Numerous debridements, one screws and rods fixation, chemotherapy Five debridements and chemotherapy Three debridements with radiotherapy, chemotherapy
Status of liver and lung
Outcome
7
Uninfected
Symptom-free
9 5
Hepatic infestation Uninfected
Symptom-free Symptom-free
2 6
Uninfected Uninfected
Symptom-free Symptom-free
7 19
Hepatic infestation Uninfected
7 4
Hepatic infestation Uninfected
Hip pain Walking difficulty, productive sinuses Hip pain Walking difficulty
Follow-up, years
F, female; M, male.
Five patients underwent simple debridement (patients 2, 5, 6, 8, and 9) because of the extent of disease involvement and the destruction of soft tissue barriers prior to admission to our hospital. Patient 4 underwent reconstruction with two plates fixed to the lateral parts of the iliac ala and three cortical bone screws fixed to the bone defects along the long axis combined with bone cement filling. Two patients (patients 3 and 4) received bone cement filling after local excision. Patient 1 was diagnosed with hydatid disease after the first debridement in 2005. Imaging studies performed in 2009 revealed multiple cystic images of the right pelvis, including the ilium, acetabulum, ischium, pubis, and greater trochanter; the sacroiliac articulation remained intact. Wide resection was carried out on the involved muscles, and a hemipelvectomy was performed on the lateral parts of the sacroiliac articulation and symphysis pubis combined with femoral osteotomy over the lesser trochanter. The resected hemipelvis was immersed in liquid nitrogen for 20 min. Detected lesions were excised completely after thawing. Four plates and screws were used to stabilize the hemipelvis. A femoral prosthesis was carefully inserted into the acetabulum, which was filled with bone cement (Figure 1). Patient 7 was diagnosed with sacrum echinococcosis disease when she was 14 years old. Numerous debridements were performed on the patient because of the frequent recurrence of the disease. We used a pedicle screw-rod system on the patient in 2004 to reconstruct bone defects and stabilize the pelvic ring (Figure 2). The patients were administered postoperative chemotherapy with albendazole (10 mg/kg/day) for 6–12 months with close observation of the liver enzymes. Two patients (patients 5 and 9) received radiotherapy (Dose of total = 6900 cGy/23 f/30 days). 3. Results All patients underwent MRI scanning, and five out of the nine cases were identified as having hydatid disease. The liver was involved in three patients (Table 1). Nine patients (100%) were positive in the eight immunodiagnosis tests. The average ESR was 18 mm/h (range 3–48 mm/h), and the average CRP was 5.8 mg/l (range 2.3–61.4 mg/l). The final diagnosis was confirmed by histopathological examination of the resected tissue. Resection margins were wide in one patient (patient 1), marginal in two patients (patients 3 and 4), and intralesional in the remaining patients. The mean follow-up was 7.3 years (range 2–19 years). Five patients (patients 1–5) were symptom-free, had no recurrence, and were able to walk unaided at the latest followup; the remaining patients suffered from hip pain, limitation of
movement, or productive sinuses. Five patients required several debridement procedures in combination with chemotherapy or radiotherapy. One of these five patients (patient 7) developed chronic lesions with sinus formation in the lumbosacral region 4 years after reconstructive surgery with a pedicle screw-rod system; she received another debridement with removal of the internal fixator in 2011 (Figure 2). A hemipelvectomy was proposed for patients 6 and 8, but both refused and instead opted for repeated debridement procedures. Radiography of patient 1 in January 2013 showed that the femoral prosthesis and pelvis were in good alignment and osteotomy sites were healed, with no sign of recurrence for 3 years (Figure 1). None of the patients died during the follow-up period. 4. Discussion The progression of hydatid disease is slow, and the symptoms of pelvic localization usually only become evident in adulthood in the form of pain, swelling, claudication, sinus formation, vague abdominal pains, or pressure symptoms involving the adjacent organs.2,5–7 Similar to our observations, the literature reports that sciatica is the first clinical symptom that appears when the pelvic hydatid cyst compresses the lumbosacral nerve plexus.8 It must be emphasized that there are no specific symptoms for pelvic hydatid disease; the mode of presentation is determined by the size of the cyst and any attendant complications. Diagnosis of hydatid disease from pelvic localization by radiological detection may be difficult, and symptoms are often nonspecific. Whilst serological tests are important for the differential diagnosis, their reliability is not 100%.3 The eight immunodiagnosis tests include ELISA and DIGFA. The overall sensitivity of DIGFA is 80.7% for human cystic echinococcosis and 92.9% for human alveolar echinococcosis. The highest specificity was 93.4% with EgB extract for cystic echinococcosis, and 90.3% with Em2 antigen for alveolar echinococcosis when cystic echinococcosis versus alveolar echinococcosis cross-reactivity was excluded. The major advantages of this method include its rapidity, convenience, and ability to provide an initial diagnosis. Differentiation between cystic echinococcosis and alveolar echinococcosis is also possible in approximately 80% of all cases in either the clinical or community setting.9 Blood testing for inflammatory markers has been observed to lack specificity. A definitive diagnosis may be obtained only by histopathological examination of the resected tissue. The only curative treatment for osseous hydatidosis is surgery.5 The treatment of patients by different surgical options has been reported in the literature, including simple drainage or
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Figure 1. A 33-year-old male patient with hydatid disease of the right hemipelvis and ipsilateral greater trochanter. (a) Radiograph of the pelvis in 2009 showing a multilocular lytic lesion involving the right pelvis and greater trochanter. (b) MRI scan showing a shift in signals from the inferior part of the right iliac region to the acetabulum and a lobulated cyst in the soft tissue around the right hip region, as well as the iliac fossa, iliacus and internal obturator muscle. (c) Appearance of the resected hemipelvis and the liquid nitrogen treatment. (d) Most of the scolexes aspirated from lesions in the bone before liquid nitrogen treatment could not be stained for direct microscopic examination (40, eosin stain). By contrast, all of the scolexes obtained after liquid nitrogen treatment were stained (200, eosin stain). (e) Radiograph performed 3 years after surgery showing the femoral prosthesis and pelvis in good alignment.
debridement, complete excision, total hip replacement, large bone grafts, femoropubic arthrodesis, megaprosthetic replacement, massive arthroplasty, osteosynthesis, and hemipelvectomy.10–12 Simple drainage or debridement is suggested as a treatment option that is readily accepted by many patients; however, incomplete removal results in early recurrence and dissemination. The basic technique involves exposure of the lesion and protection of the adjacent normal tissue with gauze soaked in 20% NaCl solution, combined with burnishing of the interior wall of the residual cavity using a high-speed burr. Dexamethasone (10– 20 mg) is injected during the operation to prevent allergic shock. The hydatid peel is removed by aspiration, and the compressed normal tissue or ‘capsule’ wall is cleaned with alcoholic phenyl and soaked in 20% hypertonic NaCl solution for 10 min. Reduction of contamination is achieved by carefully performing curettage and using operative instruments separately. Patient 6 in our study was misdiagnosed with tuberculosis in 2006 and received the first debridement without the protection of adjacent tissues. She has so far undergone eight debridements, and involvement of the sacroiliac articulation was observed at her last follow-up. Patient 1 was initially diagnosed with hip arthritis for right hip pain but was confirmed to have cystic echinococcosis by pathological evaluation after surgical debridement. Imaging studies performed after 4 years revealed an important osseous change, with multiple cystic images in the right pelvis and greater trochanter. Thus, basic techniques must be followed when the diagnosis of echinococcosis cannot be ruled out before operation. Bone cement filling has been proven to be an effective method for reducing the recurrence rate of cysts because the elevation in temperature in the polymerizing cement has a necrotizing effect that may kill daughter cysts.13 No recurrence in the ilium region
was observed in patients who had been treated with bone cement, and their recoveries appeared excellent. Several surgical methods for reconstruction are available. Patient 7 in our study underwent reconstruction with a pedicle screw-rod system. This method is a simple reconstructive procedure that does not require lengthy preoperative customization. However, it cannot guarantee complete cutting of the edge around the sacroiliac joints, and surgery sometimes results in disease recurrence or worse conditions. Martinez et al.6 reported that a hip replacement should probably not be attempted when the destruction of the hip joint is extensive. Neelapala et al.14 proposed that subtotal excision of the cyst along with joint replacement is an acceptable treatment option and that loose components can be successfully revised by this method to achieve good clinical outcomes. Wirbel et al.12 showed that megaprosthetic replacement for hydatid disease results in restricted but acceptable limb function. Kapoor et al.15 reported the formation of bone defects after debridement of the ilium, along with filling of the proximal femur with bone cement and augmentation of the femur using an intra-medullary nail. Eradication of the parasite was not the goal of this surgical treatment; instead, the procedure aimed to promote maintenance of limb function. Gdoura et al.11 observed a case of pelvic bone hydatidosis that required broad surgical resection and reconstruction by femoropubic and sacral arthrodesis; however, the fixation fractured 7 months after surgery. Some complications observed appear to be associated with the large bone defects that occur after radical resection. Thus, we formulated an unusual combination procedure for patient 1, involving replantation of the resected hemipelvis after devitalization and replacement of the femoral prosthesis. We believe that such a combination can provide a desirable bone stock and retain
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Figure 2. A 37-year-old female patient with hydatid diseases of the ilium and sacrum. (a) CT of the pelvis showing an important osseous change of the sacrum in 1999. (b) Radiograph performed 7 years after reconstructive surgery using a pedicle screw-rod system. (c) MRI scan performed in 2012 showing recurrent lesions around the sacrum and pelvic cavity. (d) Appearance of the lumbosacral area in 2012 showing a surgical scar with sinus formation.
sufficient limb function without redundant procedures. The sacroiliac articulation was left intact, and invasion by parasitic formation in the surrounding soft tissues was not extensive. Considering the barriers presented by the pelvic muscles, iliectomy of the healthy bone may give hope of complete surgical eradication. The advantages of liquid nitrogen treatment include simplicity, desirable bone stock, sufficient biomechanical strength, maintenance of osteoinductive and osteoconductive properties, and absence of harmful denatured substances.16,17 Hildreth et al.18 demonstrated the destruction of E. multilocularis eggs by the application of liquid nitrogen for 15 min via in vitro and in vivo experiments. Colli and Williams19 found that eggs from E. granulosus remained infectious for 24 h when stored at 50 8C, but were not infectious when stored at 70 8C. Utilization of liquid nitrogen as a cryogenic agent to sterilize Echinococcus cysts in the bone has yet to be reported. Bone hydatid cysts are different from visceral hydatid cysts; the former lack a fibrous capsule and manifest osteolytic bone destruction, thereby allowing the easier penetration of liquid nitrogen to devitalize the parasite in the damaged bone. In our study, we compared the results of eosin staining before and after treatment with liquid nitrogen by microscopy and found that Echinococcus cysts in the bone may be devitalized by the application of liquid nitrogen for 20 min (Figure 1). The prognosis for pelvic bone hydatidosis depends on whether or not the coxofemoral and sacroiliac articulations are affected. The prognosis is usually poor when the disease is widespread in the basin; surgical eradication becomes uncertain in these cases and extensive mutilation must be accepted.5 Siwach et al.10 reported a case of extensive hydatidosis of the femur and pelvis bone, in which the patient died of sepsis and extensive bedsores 1 month after a hindquarter amputation. A hemipelvectomy was recommended for patients 6 and 8 in our study, but both refused this option because of the potential for functional loss. Therapeutic dilemmas can arise in cases of extended disease, with many muscles or muscle layers in different sites of the body communicating through fistulas.14 One patient in our study received reconstruction with a pedicle screw-rod system, but developed chronic sinus formation after 4 years that did not cease
until the internal fixation had been removed. Therefore, internal fixation should not be used when lesions are hard to resect completely. Most surgeons concur that treatment of osseous hydatidosis better resembles oncologic therapy than the usual surgical treatment of visceral hydatid cysts.5 The wide resection of the involved bone together with the surrounding soft tissue is advocated because incomplete resection confers a great risk of recurrence.20 We believe that the Enneking system for staging malignant musculoskeletal tumors is also instructively significant for pelvic bone hydatidosis because the pelvic regions (including types I, II, III, and IV) indicated for disease onset and malignant tumor progression are similar to those of echinococcosis. However, pelvic bone hydatidosis cannot be fully equated with pelvic malignant tumors because of the following: (a) The invasiveness of malignant tumors is much higher than that of hydatid disease. For example, Mankin et al.21 reported that the mean survival rate of patients with high-grade malignant pelvic tumors is approximately 50%, whereas hydatid disease is rarely life-threatening even with multiple-organ involvement. Masse et al.22 believed that, even without complete eradication, the slow extension of hydatid disease allows a hypothetically wide margin for surgical excision. (b) The age of onset of hydatid disease is generally low and most cases occur in the 15–35 years range; most malignant tumors develop after 45 years of age. As such, most of the patients in this study opted for debridement or reconstruction instead of hemipelvic resection even when the destruction was extensive. A custom-made hemipelvic prosthesis has previously proven to be an effective treatment for malignant tumors, however it shows a relatively short service life in hydatidosis compared with devitalization and replantation. Although surgery is the most effective treatment for hydatidosis, the combination of preoperative albendazole therapy, surgery, and postoperative albendazole therapy has been proven to be an effective regimen. Medical treatment aims at reducing the size of the cysts and sterilizing their content before and after the surgery to allow the treatment of small cysts that are virtually undetectable;23 drug therapy is generally not used as the primary treatment except in cases where the patient is not fit for surgery.
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Radiotherapy is used as an adjunct to surgery and appears to be a good alternative for patients with contraindications to medication. In conclusion, pelvic bone hydatidosis is a rare disease even in endemic countries. Surgery of the pelvis is difficult and requires meticulous technical preparation. Early diagnosis helps in the eradication and salvage of the bone. The surgeon should be aware that incomplete resection exposes the patient to a greater risk of recurrence than a complete one. We also advocate that treatment of the patient be done during the early stages of the disease. Total exeresis and individual reconstruction is necessary when the osseous and soft tissue involvement is not extensive. Internal fixation should not be used when surgical eradication is uncertain. Conflict of interest: No conflict of interest to declare. References 1. Sapkas GS, Stathakopoulos DP, Babis GC, Tsarouchas JK. Hydatid disease of bones and joints. 8 cases followed for 4–16 years. Acta Orthop Scand 1998;69:89–94. 2. Terek MC, Ayan C, Ulukus M, Zekioglu O, Ozkinay E, Erhan Y. Primary pelvic hydatid cyst. Arch Gynecol Obstet 2000;264:93–6. 3. Song XH, Ding LW, Wen H. Bone hydatid disease. Postgrad Med J 2007;83:536– 42. 4. Duran H, Ferrandez L, Gomez-Castresana F, Lopez-Duran L, Mata P, Brandau D, et al. Osseous hydatidosis. J Bone Joint Surg Am 1978;60:685–90. 5. Zlitni M, Ezzaouia K, Lebib H, Karray M, Kooli M, Mestiri M. Hydatid cyst of bone: diagnosis and treatment. World J Surg 2001;25:75–82. 6. Martinez AA, Herrera A, Cuenca J, Herrero L. Hydatidosis of the pelvis and hip. Int Orthop 2001;25:302–4. 7. Seenu V, Misra MC, Tiwari SC, Jain R, Chandrashekhar C. Primary pelvic hydatid cyst presenting with obstructive uropathy and renal failure. Postgrad Med J 1994;70:930–2. 8. Sanal HT, Kocaoglu M, Bulakbasi N, Yildirim D. Pelvic hydatid disease: CT and MRI findings causing sciatica. Korean J Radiol 2007;8:548–51.
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9. Feng XH, Wen H, Zhang ZX, Chen XH, Ma XD, Zhang JP, et al. Dot immunogold filtration assay (DIGFA) with multiple native antigens for rapid serodiagnosis of human cystic and alveolar echinococcosis. Acta Trop 2010;113:114–20. 10. Siwach R, Singh R, Kadian VK, Singh Z, Jain M, Madan H, et al. Extensive hydatidosis of the femur and pelvis with pathological fracture: a case report. Int J Infect Dis 2009;13:e480–2. 11. Gdoura F, Trigui M, Zribi W, Ellouze Z, Bouzidi R, Ayedi K, et al. Pelvic bone hydatidosis. J Orthop Traumatol Surg Res 2010;96:85–9. 12. Wirbel RJ, Schulte M, Maier B, Mutschler WE. Mega-prosthetic replacement of the pelvis. Function in 17 cases. Acta Orthop Scand 1999;70:348–52. 13. Yildiz Y, Bayrakci K, Altay M, Saglik Y. The use of polymethylmethacrylate in the management of hydatid disease of bone. J Bone Joint Surg Br 2001;83: 1005–8. 14. Neelapala VS, Chandrasekar CR, Grimer RJ. Revision hip replacement for recurrent hydatid disease of the pelvis: a case report and review of the literature. J Orthop Surg Res 2010;5:17. 15. Kapoor SK, Kataria H, Patra SR, Bharadwaj M, Vijay V, Kapoor S. Multi-organ hydatidosis with extensive involvement of the hemi-pelvis and ipsilateral femur. Parasitol Int 2013;62:82–5. 16. Tanzawa Y, Tsuchiya H, Shirai T, Hayashi K, Yo Z, Tomita K. Histological examination of frozen autograft treated by liquid nitrogen removed after implantation. J Orthop Sci 2009;14:761–8. 17. Tsuchiya H, Nishida H, Srisawat P, Shirai T, Hayashi K, Takeuchi A, et al. Pedicle frozen autograft reconstruction in malignant bone tumors. J Orthop Sci 2010;15:340–9. 18. Hildreth MB, Blunt DS, Oaks JA. Lethal effects of freezing Echinococcus multilocularis eggs at ultralow temperatures. J Parasitol 2004;90:841–4. 19. Colli CW, Williams JF. Influence of temperature on the infectivity of eggs of Echinococcus granulosus in laboratory rodents. J Parasitol 1972;58:422–6. 20. Herrera A, Martinez AA. Extraspinal bone hydatidosis. J Bone Joint Surg Am 2003;85:1790–4. 21. Mankin HJ, Hornicek FJ, Temple HT. Malignant tumors of the pelvis: an outcome study. Clin Orthop Relat Res 2004;425:212–7. 22. Masse A, Parola PG, Brach del Prever EM, Gallinaro P. Hydatidosis of the pelvis: a case report with a 25-year follow-up. Arch Orthop Trauma Surg 2004;124:203– 5. 23. Merkle EM, Schulte M, Vogel T, Tomczak R, Reiber A, Kern P, et al. Musculoskeletal involvement in cystic echinococcosis report of eight cases and review of the literature. AJR Am J Roentgenol 1997;168:1531–4.
![[Rare cause of symptomatic lumbosciatica: Pelvic bone hydatidosis].](/assets/img/hold.png)
