Echinococcal cysts involving the spine: A rare cause of progressive neurological deterioration

Clinical Cases 25.06.2024
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 62 years, male
Authors: Tjaša Kitanovski 1, Peter Kompare 2, Karlo Pintarić 1
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AI Report

Clinical History

A 62-year-old male, with a history of recurrent vertebral echinococcal cysts, presented to the Emergency Department with a 14-day decline in lower limb function, gait instability, and bowel and bladder incontinence. Lumbar spine tenderness and neurological deficits in both lower limbs were noted during the examination, with no reported trauma history.

Imaging Findings

X-ray of the thoracolumbar spine revealed signs of multiple spinal surgeries with inserted surgical materials, yet no apparent cause for acute deterioration (Figures 1a and 1b). MRI revealed an extensive multicystic mass extending from Th12 to L2 with prominent cystic changes in the posterior elements of the L2 vertebra, which significantly indented the dural sac dorsally (Figures 2a and 2b). Over the course of one year, progressive cystic changes in the posterior part of the L2 vertebra led to the development of severe spinal stenosis, resulting in the complete displacement of cerebrospinal fluid and compression of the spinal cord at the level of the L2 vertebra (Figure 3).

Discussion

Human echinococcosis (hydatid disease) is a zoonotic ailment resulting from tapeworm parasites transmitted by domestic dogs, particularly prevalent in the Mediterranean region [1].

Hydatid disease is primarily hepatic; spinal involvement is rare, constituting 0.2–1% of cases [2]. The spinal disease typically extends beyond the vertebral bodies, manifesting as an extraosseous soft tissue cystic mass that affects the paravertebral soft tissues, intervertebral discs, spinal canal with its spinal cord, and posterior spinal elements [3]. Based on the anatomical location, Braithwaite and Lees classified spinal echinococcosis into five types: intramedullary (type 1), intradural, extramedullary (type 2), extradural, intraspinal (type 3), vertebral (type 4), and paravertebral (type 5); however, multiple types can co-exist [3].

Diagnosing hydatid disease requires a comprehensive approach, considering clinical symptoms and radiological imaging for accurate detection [1]. Symptoms may manifest as back pain, limb weakness, radiculopathy, myelopathy, or pathological fractures [3,4].

Plain radiographs and CT scans are effective for visualising bony destruction, while MRI is the preferred diagnostic modality, due to its high sensitivity in detecting cysts and providing detailed anatomical insights [2,3,5].

Key MRI findings of spinal disease include multiple fluid-filled cystic lesions with thin-walled septations, resembling a cluster of grapes, with large, spherical cystic lesions within paravertebral muscles. These cysts typically manifest as hypointense on T1-weighted images and hyperintense on T2-weighted images, exhibiting subtle peripheral enhancement on post-contrast imaging. Multilocular cysts may indicate daughter cysts, which grow slowly, so the surrounding bone appears capsular and distensible with smooth and often sclerotic circumscription. These MRI findings are crucial for differentiating echinococcus from other pathologies, as biopsy or cyst aspiration is discouraged due to the high risk of disease spread and/or anaphylactic reaction. When diagnosis remains uncertain, serology can be employed to strengthen diagnostic accuracy. Our patient’s medical history and MRI findings strongly supported the progression of previous hydatid disease, further reinforced by positive serology for echinococcus. Subsequently, the diagnosis was confirmed through histopathological examination of the excised tissue [2,3,5].

Current treatment involves surgical excision for neural decompression and cyst removal, often combined with antihelminthic drugs. Despite available treatments, spinal echinococcosis has a high recurrence rate, attributed to cyst infiltrative nature and high intraoperative risk of rupture, emphasising the importance of early, pre-operative recognition and long-term surveillance, preferably with MRI [3,5,6].

Take Home Message

In endemic regions, clinicians should consider spinal echinococcosis in patients with back pain and neurological symptoms, especially when MRI reveals cystic lesions.

All patient data have been completely anonymised throughout the entire manuscript and related files.

Differential Diagnosis List

Solitary bone cyst
Aneurysmal bone cyst
Arachnoid cyst
Pott’s disease
Osteomyelitis
Giant cell tumour
Chondrosarcoma
Neurofibromatosis
Spinal compression due to progression of hydatid cyst disease
Tubercular spondylodiscitis

Final Diagnosis

Spinal compression due to progression of hydatid cyst disease

Figures

X-rays of the thoracolumbar spine

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Lateral x-ray of the thoracolumbar spine shows a state post-L1 corpectomy, with a vertebral body replacement spacer at the L1 level, lateral vertebral body fixation at the levels of Th12–L2 supplemented by laminar hook fixation of a longer segment of the thoracolumbar spine.
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AP x-ray of the thoracolumbar spine shows a state post-L1 corpectomy, with a vertebral body replacement spacer at the L1 leve

PD FSE MRI of the lumbosacral spine

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Sagittal Proton Density Fast Spin-Echo (PD FSE) MRI of the lumbosacral spine showing an extensive multicystic mass extending from Th12 to L2 vertebra with newly developed marked spinal stenosis at the L2 level (white arrow).
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Axial PD FSE MRI of the lumbosacral spine at the L2 vertebra showing displacement of cerebrospinal fluid due to hydatid cysts compression of the dural sac (white arrow), causing neurological symptoms.

PD FSE MRI scans illustrating the disease progression

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Sagittal PD FSE MRI scans of the lumbosacral illustrating the disease progression over one year (yellow arrow). Based on the previous MRI examination, there is a progression involving cystic masses in the posterior elements of the L2 vertebral bodies, which now significantly indent the dural sac dorsally. Cerebrospinal fluid is entirely displaced, and the spinal cord measures 3–4mm in the anteroposterior diameter, indicating severe newly developed spinal stenosis at the L2 level. The mass also involves the