• Users Online: 205
  • Home
  • Print this page
  • Email this page
Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 


 
 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 9  |  Issue : 2  |  Page : 134-138

A rare case to primary spinal hydatid cyst presenting with neurodeficit


Department of Orthopaedics, MGMMC and MY Hospital, Indore, Madhya Pradesh, India

Date of Web Publication14-Dec-2017

Correspondence Address:
Dr. Saurabh Jain
A-2, Sch 71, Sector C, Footi Khothi Square, Ring Road, Indore - 452 009, Madhya Pradesh
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jotr.jotr_31_16

Rights and Permissions
  Abstract 


Hydatid disease, a parasitic infection caused by Echinococcus granulosus predominantly occur in liver and lungs. Skeletal involvement is uncommon and occurs in only 0.2 to 1 % of all cases. Primary spinal hydatidosis of spine is very rare. We report such a rare case of primary spinal hydatid cyst presenting with neurodeficit, which was treated surgically with excision, decompression and fixation, with aim to create the awareness among surgeon about this rare clinical presentation and pitfalls in diagnosis and management and suggest them to include it in differential diagnosis of the atypical spinal presentation. Hydatid disease necessitates high clinical suspicion, careful neuroradiological evaluation, aggressive surgical intervention, adjuvant chemotherapy and strict follow up to detect recurrence early.

Keywords: Bone cyst, echinocossis, hydatid cyst, hydatidosis, spinal cyst


How to cite this article:
Jain S, Verma A, Rohra S. A rare case to primary spinal hydatid cyst presenting with neurodeficit. J Orthop Traumatol Rehabil 2017;9:134-8

How to cite this URL:
Jain S, Verma A, Rohra S. A rare case to primary spinal hydatid cyst presenting with neurodeficit. J Orthop Traumatol Rehabil [serial online] 2017 [cited 2021 Feb 25];9:134-8. Available from: https://www.jotr.in/text.asp?2017/9/2/134/220766




  Introduction Top


Hydatid disease, a parasitic infection caused by Echinococcus granulosus predominantly, occurs in the liver and lungs. Skeletal involvement is uncommon and occurs in only 0.2%–1% of all cases.[1],[2] Half of these are spinal involvement, caused through direct extension from the abdominal, pulmonary, or pelvic infection. Primary spinal hydatidosis of the spine is very rare.[3],[4] We report such a rare case of primary spinal hydatid cyst presenting with neurodeficit, which was treated surgically with excision, decompression, and fixation, with the aim to create the awareness among surgeon about this rare clinical presentation and pitfalls in diagnosis and management and suggest them to include it in differential diagnosis of the atypical spinal presentation.


  Case Report Top


A 60-year-old female presented with complaints of gradually increasing, dull backache and difficulty in walking for 4 months. She was homemaker of a farmer and was involved in cattle rearing and animal care. On detailed history, the pain was insidious, diffuse over mid-back area which radiated to the left lower limbs. The intensity got aggravated from moderate to severe over the past 2 months, which was not relieved by analgesics. The difficulty in walking had progressed from inability to walk without support to bedridden over the past 1 month. She had urinary and fecal incontinence. There was no history of cough, fever, weight loss, night sweats, or any other constitutive symptoms. Personal and family history was not significant.

On examination, there was moderate tenderness in the middorsal region mainly over D8–D9 level. The lower dorsal spine showed mildly increased kyphotic deformity. Neurological examination showed spastic paraparesis with typical upper motor sign include increased tone in the lower limbs, exaggerated knee and ankle reflexes, absent abdominal and cremasteric reflexes, and extensor plantar response, bilaterally. The power was Grade 2 in both the lower limbs. There were hypoesthesia and numbness from the umbilicus to both lower limbs. General, respiratory, and abdominal examinations did not reveal any abnormality.

Her routine hematological examination was all within normal limits except for anemia and persistently high serum creatinine and urea. Lower abdominal ultrasonography of the patient revealed bilaterally shrunken kidneys with medullary echogenicity denoting stage V chronic kidney diseases with glomerular filtration rate of 19.4 ml/h. The abdominal sonography was normal. Serological tests and acid-fast bacilli were also negative.

Radiological examination of the dorsal spine showed paraspinal fusiform swelling bilaterally extending from D6 to D11, along with the right hemicollapse of D9 vertebra. D9–D10 disc space was reduced mildly with erosion of adjacent end plates [Figure 1]. Chest X-ray revealed clear lung fields with no significant abnormality. Magnetic resonance imaging (MRI) of the dorsal spine showed two rounds to oval cysts measuring 10 cm × 9 cm × 5 cm, in the right pre- and para-vertebral region along with erosion of the right half of D8 and D9. The main cyst contained multiple, round grape bunch-like daughter cysts within, which were hypointense in T1 and hyperintense in T2 sequences. The underlying cord was severely compressed and displaced to the right and posterior with faint myelopathic changes [Figure 2]. On the basis of MRI finding, a diagnosis of spinal hydatidosis was made. Computer tomography (CT) scan of the chest and abdomen was done to search primary involvement of the lungs or liver, which showed no abnormality in the abdominal cavity or chest parenchyma, pleura, or any lymphadenopathy. CT confirmed 9 cm × 5 cm long two well-circumscribed hypoattenuating cystic lesions seen in the posterior mediastinum in the pre- and right para-vertebral space with multiple daughter cysts within along with erosion and destruction of D9 vertebra [Figure 3].
Figure 1: Anteroposterior (a) and lateral (b) lower dorsal spine radiographic views of the patient showing paraspinal fusiform swelling from D6 to D11, along with the right hemicollapse of D9 and reduced D9–D10 intervertebral disc space

Click here to view
Figure 2: Sagittal T2 (a) and T1 (b) with coronal T2 (c) and transverse (d and e) magnetic resonance images dorsolumbar spine of the patient showing two oval cysts measuring 10 cm × 9 cm × 5 cm contained multiple round grape bunch-like daughter cysts, in the right pre- and para-vertebral region along with erosion of the right half of D8 and D9. The cysts are hypointense in T1 and hyperintense in T2 sequences

Click here to view
Figure 3: Coronal (a) and transverse (b and c) computer tomographic views of patient showing the normal liver and chest with long two well-circumscribed hypoattenuating cystic lesions in the posterior mediastinum with multiple daughter cysts with erosion of D9 vertebra

Click here to view


After neuroimaging confirmation of spinal hydatidosis, the patient was planned for surgical excision of cyst, decompression, and fixation. As the patient had stage V nephropathy, adequate optimization of patient was done preoperatively. In the left lateral position, the spine was transpleural approached anteriorly, after removal of the ninth rib. The cyst was identified and cetrimide was injected into the cyst before opening the cyst. The thin-walled cyst had eroded the D9 vertebra and contained multiple round to oval, white shiny pearl-like soft daughter cysts inside the parent cyst. Due care was taken to prevent rupture while removing the daughter cysts, so as to prevent risk of spread, recurrence, or anaphylaxis. No adverse reaction occurred during surgery. The surrounding surgical field was packed with mops to prevent local spillage. After excision of all cysts paravertebrally and adequate canal decompression along with D9 corpectomy, the spinal canal was inspected which did not showed any cyst. Finally, fixation of the spine was done with cage filled with rib graft and anterior screw fixation [Figure 4]. Histopathological examination of the excised cyst confirmed hydatidosis.
Figure 4: (a-d) Intraoperative photos of the patients right paraspinal cyst which contained multiple grape-like daughter cyst, which was excised with due care to present spillage followed by spinal fixation. Postoperative anteroposterior (e) and lateral (f) lower dorsal spine radiographs of the patient after removal of cyst showing fixation with cage and screw

Click here to view


Postoperatively, patient was given albendazole for 3 months and was gradually mobilized with the help of brace. The patient at 6 months follow-up showed neurologically recovery with able to walk unsupported with power in the lower limbs of 4 and no spasticity. She gained good bladder control. The MRI at 6 months confirmed no residual cyst, no recurrence, and adequate decompression of the spine [Figure 5].
Figure 5: Postoperative 6 months follow-up magnetic resonance imaging transverse section no recurrence and adequate decompression

Click here to view



  Discussion Top


Hydatid disease is a rare parasitic infection, caused by larval stage of tapeworm Echinococcus granulosus, mostly seen in sheep-raising areas.[1],[2] Our patient was also involved in cattle caring job. It occurs in all age groups and equally in both sexes.[5] The liver and lungs are involved in more than 90% of cases; but virtually, any body part may be affected. Skeletal involvement is seen in only 0.2%–1% of cases, of which spinal involvement is most common, involved in approximately 45% of cases.[3],[4],[5] Spinal hydatidosis can be primary or secondary from extraspinal hydatidosis through protovertebral shunts or direct extension. It remains controversial as to which region is more commonly involved, but due to higher number of vertebrae, thoracic localization is most frequently reported in the literature.[5],[6]

There are no specific pathognomonic signs or symptoms of spinal hydatidosis.[7] The cyst is usually missed or misdiagnosed in early stage, as it remains asymptomatic for years. Clinical manifestations occur when cyst grows enormous in size to cause significant compression or mechanical instability due to bony destruction or pathological fracture.[8] Thus, it may cause radiculopathy, myelopathy, sphincter disturbances, paresthesia, paraparesis, or paraplegia and/or deformity.[3],[5],[9] Long-term hydatidosis may lead to persistent pain, secondary infections leading to sinuses and spinocutaneous fistulas, localized and remote recurrences, significant persistent neurologic deficits, and spinal instability.[10] Spinal hydatidosis is associated with a high degree of disability, morbidity, and mortality, high as much to 50%, and the prognosis has been compared to that of malignancies (”le cancer blanc”).[5]

Braithwaite and Lees classified of spinal hydatid lesions causing paraplegia as (a) primary intramedullary cyst, (b) intradural extramedullary cyst, (c) extradural intraspinal cyst, (d) hydatid disease of the vertebra, and (e) paravertebral hydatid disease.[11] Our patient had hydatid disease of vertebra along with paravertebral involvement. Vertebral hydatidosis as such does not form pericysts, sequestrum, or abscess and is slow growing. Either paraspinal, vertebral, or inside the canal, the cyst can spread anterior, posterior, or laterally following the line of least resistance.[10],[12]

The differential diagnosis of spinal hydatidosis is diverse, including tuberculosis (Pott's disease), pyogenic infection (osteomyelitis),  Brucellosis More Details, fibrous dysplasia, simple solitary or aneurysmal bone cysts, malignancy (e.g., multiple myeloma, giant-cell tumors, chondrosarcoma, neuroblastoma, etc.), or spinal metastasis.[5],[13],[14]

Serological tests help to diagnosis the hydatidosis.[14] Serological test was negative in our case mainly because the cyst was old and intact. Imaging should be considered for early and accurate diagnosis. The X-ray and CT scan appearances are nonspecific, showing multicystic, or irregular destruction of vertebral bodies and appendages with arcuate lines of calcification in the “capsule” wall and sparing of intervertebral discs.[10],[13],[14],[15] Contrary to the literature, we found decreased disc space between D8 and D9 and hence suspected it to be a tubercular lesion which is endemic in our region. We suspect this decrease disc space may be due to chronicity of the lesion and large cyst in our case, which had eroded the D8, D9 and hence also the involved disc between them. The combination of CT and ultrasonography (USG) is helpful in searching primary lesion in the lung or liver.[9],[16] We have done CT and USG, which ruled out other visceral involvement, and hence, we could diagnosed it as primary hydatidosis of the spine, preoperatively, which was confirmed on histopathological examination postoperatively.

MRI is the most helpful technique for diagnosing hydatid cyst disease, which shows characteristic multilocular, spherical cystic, eccentric, expansive lesion with thin regular wall without septation. The formation of internal daughter cysts forms a grape bunch-like appearance. The T1-weighted image signal of the parent cyst may be isointense or slightly lower signal than its contents, similar to muscle and daughter cyst is similar to water. Both the parent and the daughter cysts show a high signal in the T2W image sequence.[10],[13],[14],[15],[16],[17] Comparing with CT, MRI is more reliable as it shows additionally the relationship of the lesion to the surrounding organs and involvement of neural structures. Recent use of diffusion-weighted MRI has been shown to help differentiate it from other lesions.[18] Further, the effectiveness of medical therapy and recurrence may be evaluated with follow-up CT and MRI, which is indicated in patient even having normal neurologic examinations at follow-up.

As patients usually present at an advanced stage, the treatment is difficult and recurrence is common. The initial treatment of choice is combination of antihelmintic treatment with surgical excision to achieve neural decompression and establish diagnosis.[19] The type of surgical procedures, extent of resection, and decision whether to perform spinal stabilization depends on the site and extent of the disease and bone involvement and destruction causing spinal instability.[20] Since in our case, the cyst was huge, placed paraspinally anteriorly which had eroded bodies of D8–D9, we planned the surgery anteriorly with the aim to remove all cysts, remove dead bone, corpectomy of D9, decompress the canal and stabilize the spine anteriorly. Enough care should be taken during the surgery to prevent rupture of the cysts and spillage of fluid, which can cause anaphylaxis or subsequent recurrence.[10],[21]

Albendazole is the preferred antihelmintic agent used both presurgical and postsurgical which, reduces risk of recurrence and/or facilitates surgery by reducing intracystic pressure, but its efficacy and duration of treatment are both controversial.[22],[23] Although uncommon, the suggested treatment for hydatid cyst can be aspiration and reperfusion of cyst by albendazole.[8],[13],[19]


  Conclusion Top


Due to the rarity of its presentation, unless the clinician includes spinal hydatid disease as part of the differential list of atypical spinal presentations and considers performing neuroimaging and this potentially curable disease is likely to be missed. Hydatid disease necessitates high clinical suspicion, careful neuroradiological evaluation, aggressive surgical intervention, and adjuvant chemotherapy. Careful dissection and irrigation around the cyst are also essential to avoid intraoperative cyst rupture, which leads to recurrence. Despite optimal surgical and medical therapy, outcome is poor with significant long-term morbidity and mortality and recurrences requiring reoperations are higher; hence, strict follow-up and regular MRI scans are needed to detect recurrence early. Further in spite of significant advances, many aspects of vertebral hydatidosis remain poorly understood such as predilection of the spine, route of spread, variations in pathophysiology and clinical manifestations, diagnosis, management, and recurrences. Hence, reporting of these rare case reports, registry-based or multicenter studies are needed on the topic.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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.  Back to cited text no. 1
    
2.
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.  Back to cited text no. 2
    
3.
Govender TS, Aslam M, Parbhoo A, Coor P. Hydatid disease of the spine: A longer followup after surgical treatment. Clin Orthop Relat Res 2000;378:143-7.  Back to cited text no. 3
    
4.
Jena A, Tripathi RP, Jain AK. Primary spinal echinococcosis causing paraplegia: Case report with MR and pathologic correlation. AJNR Am J Neuroradiol 1991;12:560.  Back to cited text no. 4
    
5.
Neumayr A, Tamarozzi F, Goblirsch S, Blum J, Brunetti E. Spinal cystic echinococcosis – A systematic analysis and review of the literature: Part 1. Epidemiology and anatomy. PLoS Negl Trop Dis 2013;7:e2450.  Back to cited text no. 5
    
6.
Ashraf A, Kirmani AR, Bhat AR, Sarmast AH. A rare case of recurrent primary spinal echinococcosis. Asian J Neurosurg 2013;8:206-8.  Back to cited text no. 6
[PUBMED]  [Full text]  
7.
Berk C, Ciftçi E, Erdogan A. MRI in primary intraspinal extradural hydatid disease: Case report. Neuroradiology 1998;40:390-2.  Back to cited text no. 7
    
8.
Abbassioun K, Amirjamshidi A. Diagnosis and management of hydatid cyst of the central nervous system: Part 2: Hydatid cysts of the skull, orbit, and spine. Neurosurg Q 2001;11:10-6.  Back to cited text no. 8
    
9.
Turgut M. Hydatid disease of the spine: A survey study from Turkey. Infection 1997;25:221-6.  Back to cited text no. 9
    
10.
Prabhakar MM, Acharya AJ, Modi DR, Jadav B. Spinal hydatid disease: A case series. J Spinal Cord Med 2005;28:426-31.  Back to cited text no. 10
    
11.
Braithwaite PA, Lees RF. Vertebral hydatid disease: Radiological assessment. Radiology 1981;140:763-6.  Back to cited text no. 11
    
12.
Beggs I. The radiology of hydatid disease. AJR Am J Roentgenol 1985;145:639-48.  Back to cited text no. 12
    
13.
Song X, Liu D, Wen H. Diagnostic pitfalls of spinal echinococcosis. J Spinal Disord Tech 2007;20:180-5.  Back to cited text no. 13
    
14.
Song XH, Ding LW, Wen H. Bone hydatid disease. Postgrad Med J 2007;83:536-42.  Back to cited text no. 14
    
15.
Markakis P, Markaki S, Prevedorou D, Bouropoulou V. Echinococcosis of bone: Clinico-laboratory findings and differential diagnostic problems. Arch Anat Cytol Pathol 1990;38:92-4.  Back to cited text no. 15
    
16.
Turgut AT, Altin L, Topçu S, Kiliçoglu B, Aliinok T, Kaptanoglu E, et al. Unusual imaging characteristics of complicated hydatid disease. Eur J Radiol 2007;63:84-93.  Back to cited text no. 16
    
17.
Wang J, Chen H. Vertebral hydatid disease: MRI diagnosis. Chin J Med Imaging 2002;10:101-2.  Back to cited text no. 17
    
18.
Doganay S, Kantarci M. Role of conventional and diffusion-weighted magnetic resonance imaging of spinal treatment protocol for hydatid disease. J Spinal Cord Med 2009;32:574-7.  Back to cited text no. 18
    
19.
Islekel S, Ersahin Y, Zileli M, Oktar N, Oner K, Ovül I, et al. Spinal hydatid disease. Spinal Cord 1998;36:166-70.  Back to cited text no. 19
    
20.
Pamir MN, Ozduman K, Elmaci I. Spinal hydatid disease. Spinal Cord 2002;40:153-60.  Back to cited text no. 20
    
21.
Normally HC, Aacro SI, Follien PH. Vertebral hydatid cyst infection (Echinococcus granulosus) a case report. Eur Spine J 1988;7:158-61.  Back to cited text no. 21
    
22.
García-Vicuña R, Carvajal I, Ortiz-García A, López-Robledillo JC, Laffón A, Sabando P. Primary solitary Echinococcosis in cervical spine. Postsurgical successful outcome after long-term albendazole treatment. Spine (Phila Pa 1976) 2000;25:520-3.  Back to cited text no. 22
    
23.
Ozdemir HM, Ogün TC, Tasbas B. A lasting solution is hard to achieve in primary hydatid disease of the spine: Long-term results and an overview. Spine (Phila Pa 1976) 2004;29:932-7.  Back to cited text no. 23
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


This article has been cited by
1 An Intraoperative Surprise! A Rare Case Report of Primary Craniovertebral Junction Hydatid Disease Mimicking a Bony Tumor
Mariswamappa Kiran,Chandrachari Komal Prasad,Bhatjiwale Mrudul,Kumar Shishir
World Neurosurgery. 2020;
[Pubmed] | [DOI]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Introduction
Case Report
Discussion
Conclusion
References
Article Figures

 Article Access Statistics
    Viewed940    
    Printed22    
    Emailed0    
    PDF Downloaded112    
    Comments [Add]    
    Cited by others 1    

Recommend this journal