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J Spinal Cord Med. 2005; 28(5): 426–431.
PMCID: PMC1808269

Spinal Hydatid Disease: A Case Series

Mukund M Prabhakar, MS (Ortho), Apurv J Acharya, MS (Ortho), Dhaval R Modi, MS (Ortho), and Bhavin Jadav, MS (Ortho)

Abstract

Background:

Over the past 10 years, 4 cases of spinal hydatid disease (3 men, 1 woman) were diagnosed and treated at our institution, with an average follow-up of 4 years. Hydatid disease of the spine is a rare condition with a poor prognosis that presents diagnostic and therapeutic challenges.

Methods:

The patients were evaluated clinically, using the latest imaging modalities available in our institution. Decompressive surgeries were performed and the diagnosis was confirmed by histopathologic examination. All patients received long-term antihelminthic therapy with 400 mg of albendazole 3 times daily for 1 year.

Results:

After surgery, all patients improved; however, over time, recurrence and residual disease were observed. Two patients had complete neurologic recovery at follow-up at 2 to 3 years, although there were radiographic signs of recurrence. The other 2 patients did not achieve complete neurologic recovery despite anterior decompression; they developed recurrent disease and the neurologic status deteriorated to spastic paraplegia. All patients refused further surgeries for recurrences and 2 patients died of complications of paraplegia.

Conclusion:

Diagnosis was challenging, eradication was difficult, and hydatid disease recurred in all 4 patients. In our experience, morbidity and mortality were high and prognosis was poor.

Keywords: Infection, Hydatid disease, Echinococcus granulosus, Tuberculosis, Spine, Paraplegia, Albendazole, Scolicide

INTRODUCTION

Hydatid disease in humans is caused by the cystic (larval) stage of the tapeworm Echinococcus granulosus, which is endemic to the temperate climate. Canines are the primary host. The life cycle of E granulosus may also involve sheep, cattle, goats, and humans. This infection is transmitted orally via eggs shed in the feces of infected animals. Hydatid disease is not uncommon in the Saurashtra and Kutch areas of Gujarat state, where cattle rearing is a common occupation. Primary hydatidosis is common in the liver, spleen, and lungs (13). Theoretically, it can occur at any site except teeth, hair, and nails (4). Musculo-skeletal involvement is secondary and uncommon, with an incidence of less than 2.5%. It affects the pelvis and sacrum, metaphyses of the long bones, skull, spine, and ribs in decreasing order of incidence. Spinal involvement is rare, with an incidence of less than 1% (58).

Over a period of 10 years (January 1993 through December 2003), 4 cases of spinal hydatid disease with neurologic deficit were diagnosed and treated at our institution (a public hospital) and were observed for an average follow-up period of 4 years (range = 2–6 years). The aim of this retrospective study is to share our experiences of the pitfalls and challenges in the diagnosis and management of spinal hydatid disease and to provide a perspective through review of the literature.

Clinical Presentation

During the last 10 years, we diagnosed and treated 4 patients with spinal hydatid disease. The series included 3 men and 1 woman (mean age = 40 years ± SD 7.07; range = 35–50 years; Table 1). They presented with persistent backache not responsive to usual conservative treatment and gradually increasing neurologic deficit of the lower limbs. Two patients presented with spastic paraplegia; 1 with impairment equal to American Spinal Injury Association (ASIA) grade C, and 1 patient, who had had surgery before being seen at our institution, presented with neurologic deterioration and cauda equina syndrome. Risk for tapeworm infection is high among people with close animal contact. We could not confirm close contact with animals in all cases, but all of the patients came from the rural areas of Saurashtra and Kutch, where the typical triad of shepherd community, cattle, and dogs is found.

Table 1
Clinical Presentation

Diagnostic Workup

All of the patients were investigated with routine hematologic investigations including blood counts, plain radiographs of the spine in anteroposterior and lateral views, and underwent ultrasonographic examination of the abdomen to rule out visceral hydatid disease. All of the patients presented with neurologic complications. Two patients underwent conventional myelography, 1 patient underwent a computerized tomography (CT) scan and magnetic resonance imaging (MRI) and the fourth patient was investigated with a postmyelography CT scan and an MRI scan (Table 2).

Table 2
Management

Surgical Intervention

All patients underwent surgery to excise the cysts and had a laminectomy performed through the posterior approach for neurologic decompression at the level of spinal involvement. Due care was taken to prevent rupture while removing the cysts, which contain hundreds, even thousands, of protoscolicies, each of which can form a new hydatid cyst. The surrounding surgical field was packed with mops to prevent local spillage. However, scolicidal solutions, such as hypertonic saline and cetrimide, were not used during surgery for fear of chemical damage to the cord.

Follow-up

In all patients, histopathologic confirmation of the diagnosis was obtained and antihelminthic therapy with 400 mg of albendazole 3 times daily was prescribed for 1 year. The patients were followed every 6 weeks initially, in the form of neurologic reassessment and imaging investigations. Two patients who developed symptomatic recurrence were treated by repeat surgery for thorough decompression and fusion with iliac crest bone grafts through the anterior approach. They were rehabilitated at our spinal care center, including postoperative care and gradual mobilization with spinal braces.

Laboratory Findings

Laboratory Data

Hematologic studies were inconclusive. Two patients were subjected to biopsy initially, but inconclusive reports were obtained. The histopathologic diagnosis in these 2 patients was made by biopsy after imaging methods indicated the possibility of hydatid disease of the spine. Final histopathologic confirmation of hydatid disease was obtained in all 4 patients. Because tuberculosis is the most common nontraumatic cause of spinal destruction with neurologic involvement in India, 2 of the patients were treated empirically with antituberculous treatment. Subsequent neurologic deterioration occurred, which led to the correct diagnosis in these 2 patients.

Imaging Findings

Plain radiographs showed multiple, well-defined, osteolytic expansile cavitatory areas without periosteal reaction or sclerosis. On plain radiographs, involvement of adjacent vertebral bodies, posterior elements, and contiguous ribs were noted. Ultrasonography of the abdomen did not reveal any abdominal organ involvement in any of the patients at the time of first presentation. Myelography (2 patients) showed the characteristic brush-border appearance, suggestive of extradural lesion, with complete blockage in 2 patients with dorsal spine involvement. CT scan (2 patients) showed multiple cystic, osteolytic expansile lesions in vertebral bodies and posterior rib ends without enhancement of the lesion or the margins on intravenous (IV) contrast study. Postmyelogram CT scan (1 patient) demonstrated cord compression (Figure 1).

Figure 1
Sagittal view, T2-weighted MRI scan of the thoracic spine showing multi-level involvement by numerous hydatid cysts with spinal intrusion and penetration of surrounding soft tissue. The cyst fluid appears iso-intense with the cerebrospinal fluid in both ...

MRI (2 patients) showed multiple cystic fluid-filled lesions with thin walls and irregular branching resembling a grape bunch at multiple levels on axial, sagittal, and coronal images of the spine, with contiguous involvement of posterior rib ends (Figures 2 and and3).3). The sacrum was found to be involved in contiguity in the patient with the lumbar spine involvement. Paravertebral muscles had multiple spherical large cystic lesions with daughter cysts inside. On MR myelogram, continuous soft tissue mass with intraspinal extension and multiple level cord compression with combination of extradural and intradural lesions were seen (Figure 2).

Figure 2
(A) and (B) Transverse cut of CT myelogram showing lumbar spine involvement with multiple circular cavities in the body and displacement of the cord caused by soft tissue invasion of the spinal canal by the cysts.
Figure 3
Transverse view, T2-weighted MRI scan showing multiple echinococcal cysts in the body and appendages of the vertebra, with intrusion of the hydatid cysts into the spinal canal.

Surgical Findings

At the time of surgery, multiple pearly shiny grape-like cysts were seen bulging from the spinal canal with multi-level intraspinal and extraspinal extensions and paraspinal muscle involvement, which required extensive decompression and debridement through wide laminectomy (Figure 4).

Figure 4
Perioperative photograph of the fourth patient during posterior decompression of the cord through laminectomy, showing multiple grape-like hydatid cysts bulging through the spinal canal in the center.

Two patients underwent a second surgery in the form of anterior decompression, debridement, and fusion with tricortical iliac autogenous bone grafting. Preoperatively, destruction of the vertebral body trabeculae and multiple small cysts were observed.

All patients had delayed incision healing at an average of 1 month, with serous fluid discharge from the wound that showed no growth on routine bacterial culture methods.

Follow-up

Initial follow-up results were good, with immediate neurologic recovery, but the results deteriorated constantly with time. The imaging studies at 1-year follow-up invariably showed residual or recurrent cysts. The 2 patients with the thoracic spine involvement had worsening neurologic status, thus, underwent a second surgery. At later follow-up at 2 to 3 years, they again had deteriorated to paraplegia, but refused further surgical intervention and succumbed to the disease and the complications of paraplegia.

The patients with lumbosacral disease developed recurrence, but because there was no neurologic worsening, they refused surgery and were lost to follow-up after 2 to 3 years.

DISCUSSION

Hydatid disease usually affects the soft tissues first and bones are involved later. Out of our 4 patients, none had detectable primary lesions on ultrasonography of the abdomen. In bone, hydatid growth continues in an outward direction in surrounding cancellous bone, with destruction by pressure necrosis and resorption leading to exogenous cyst formation. Thus, pericyst does not form in the bone hydatid (9). There is no pus formation or sequestration of bone.

The neurologic complications are the result of invasive intradural and extradural growth of the cysts causing direct compression. Destruction of the bones causes mechanical instability and secondary neurologic damage. This mixed picture poses a confusing picture for diagnosis and treatment planning (8,10,11). These 4 patients were referred to our center because of incorrect diagnosis and inappropriate treatment. Because of lack of clinical suspicion of hydatid disease, imaging investigations were inadequate and biopsy reports inconclusive.

On plain radiographs, multiple cystic lesions in multiple contiguous vertebral bodies and appendages are seen, usually without vertebral collapse and with sparing of intervertebral disks. Involvement of contiguous ribs and paravertebral masses are suggestive of hydatid disease (12). There is absence of the classical paradiscal sclerosis and sequestration that is suggestive of tuberculous infection.

CT scan with IV contrast does not show any enhancement. Myelogram demonstrates multi-level cord compression providing precise anatomic localization of the lesions (13). Postmyelogram CT scan is useful when MRI is not available.

MRI shows soft tissue masses in paraspinal muscles, which are spherical thin-walled, fluid-filled parent cysts. The formation of internal daughter cysts forms a grape-bunch–like appearance. Similarly, continuous intraspinal extension appears as vertebral and rib lesions, representing intradural and extradural multiple cysts compressing the cord (Figures 2 and and33).

MRI is superior to CT for demonstrating neural involvement (1416). All of these studies helped establish a preoperative diagnosis in our cases. The diagnosis can be definitely confirmed histopathologically after decompressive surgeries by observing the allergic-type tissue reaction in the surrounding tissue and demonstration of the cyst wall histology and scolices.

Efficacy of albendazole for primary bony hydatid involvement is questionable; postoperative albendazole therapy seems only to retard recurrence (8,17,18). All of our patients received 400 mg of albendazole 3 times daily for 1 year, but recurrence rate was still 100%.

The aim of surgery is removal of all of the cysts early in the course of the disease (8). Usually, posterior spinal decompression through laminectomy and debridement of paravertebral lesions is the initial surgery (Figure 4) (5,8), but complete clearance is difficult because of invasive diffuse spread within the bone and canal (18). Often, spillage of fluid caused by cyst rupture leads to subsequent recurrence (7,18). According to the guidelines for excisional surgery, the surgical area needs to be irrigated with hypertonic saline (8). We avoided this irrigation to prevent chemical damage to the spinal cord; we took enough care not to rupture the cysts; and we attempted the intact removal of all of the visible cysts by wide laminectomy. Ideally, these lesions should be treated by radical operation with circumferential approach and extensive removal of all cysts and affected bone and soft tissues (8,1820).

Follow-Up and Prognosis

Our patients had significant neurologic improvement over 12 weeks and 2 patients had normal neurologic status at the last follow-up of 2 to 3 years. MRI is the best method for detecting any residual or recurrent lesions (21), therefore, the 2 patients who had normal neurologic examinations at follow-up also underwent MRI. Despite evidence of recurrence, the patients opted for no further surgical treatment.

The 2 patients with thoracic spine involvement had partial improvement initially, but deteriorated over 1 year to complete paraplegia again. Because MRI or CT scan was not available at that time, myelography was done, which showed recurrence with severe cord compression at the original levels. A transthoracic approach was used for anterior spinal decompression, and thorough debridement and fusion with iliac crest bone grafts was done. On later follow-up, fusion was successful and a partial neurologic improvement was observed, which deteriorated again to complete paraplegia at 5 to 6 years and to the death of both patients from complications.

In patients with spinal hydatid disease, neurologic deterioration requires reintervention. Despite multiple spinal surgical interventions, partial or complete neurologic deficit may persist (6).

Long-term hydatidosis leads to persistent pain, secondary infections leading to sinuses and spinocutaneous fistulas, localized and remote recurrences, significant persistent neurologic deficits, and spinal instability. With its high morbidity and mortality and poor prognosis, hydatid disease behaves like locally malignant lesions (10,19,22).

CONCLUSION

Hydatid disease is not uncommon in rural areas of Gujarat state. It is acquired in childhood, and usually presents in adulthood. Musculoskeletal involvement is a less common presentation, of which, spinal involvement is infrequent. The infection may be misdiagnosed initially as tuberculosis of the spine, which delays proper diagnosis and intervention. In addition to the many difficulties in the diagnosis, the management of the disease is even more challenging because of a high recurrence rate, requiring extensive and repeated spinal surgeries with high rates of complications and significant long-term morbidity and mortality. Results are seldom satisfactory and prognosis is usually poor. Control of conditions favorable to tapeworm transmission is key to preventing hydatidosis in humans.

Acknowledgments

We thank the following people for their help in the preparation of this article: Dr Tejas Thakker, MS (Ortho), assistant professor, Department of Orthopedics, B. J. Medical College and Civil Hospital, Ahmedabad; Dr Bhavesh Jesalpura, MS (Ortho); and Dr Dhaval Bhatiya, MS (Ortho).

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