Severe kyphoscoliosis after primary Echinococcus granulosus infection of the spine

Abstract

A primary Echinococcus granulosus infection of the spine involving the vertebrae T8 and T9 of a 6-year-old child was treated elsewhere by thoracotomy, partial corporectomy, multiple laminectomies and uninstrumented fusion. Owing to inappropriate stabilization, severe deformity developed secondary to these surgeries. X-rays, CT and MRI scans of the spine revealed a severe thoracic kyphoscoliosis of more than 100° (Fig. 1) and recurrence of Echinococcus granulosus infection. The intraspinal cyst formation was located between the stretched dural sac and the vertebral bodies of the kyphotic apex causing significant compression of the cord (Figs. 2,3,4). A progressive neurologic deficit was reported by the patient. At the time of referral, the patient was wheelchair bound and unable to walk by herself (Frankel Grade C). Standard antiinfectious therapy of Echinococcus granulosus requires a minimum treatment period of 3 months. This should be done before any surgical intervention because in case of a rupture of an active cyst, the delivered lipoprotein antigens of the parasite may cause a potentially lethal anaphylactic shock. Owing to the critical neurological status, we decided to perform surgery without full length preoperative antiinfectious therapy. Surgical treatment consisted in posterior vertebral column resection technique with an extensive bilateral costotransversectomy over three levels, re-decompression with cyst excision around the apex and multilevel corporectomy of the apex of the deformity. Stabilisation and correction of the spinal deformity were done by insertion of a vertebral body replacement cage anteriorly and posterior shortening by compression and by a multisegmental pedicle screw construct. After the surgery, antihelminthic therapy was continued. The patients neurological deficits resolved quickly: 4 weeks after surgery, the patient had Frankel Grade D and was ambulatory without any assistance. After an 18-month follow-up, the patient is free of recurrence of infection and free of neurologically deficits (Frankel E). This case demonstrates that inappropriate treatment-partial resection of the cyst, inappropriate anterior stabilization and posterior multilevel laminectomies without posterior stabilization-may lead to severe progressive kyphoscoliotic deformity and recurrence of infection, both leading to significant neurological injury presenting as a very difficult to treat pathology.

Spinal Cystic Echinococcosis – A Systematic Analysis and Review of the Literature: Part 2. Treatment, Follow-up and Outcome

“…The location and the extent of the lesion(s) determine the surgical approach and the necessary surgical procedure (table 2). After resection of affected bone, a wide spectrum of techniques has been employed to stabilize the vertebral column (including bone grafts [17], [25]–[43], dorsal stabilisation by pedicle screw systems [9], [26], [27], [37], [44]–[48], Harrington- [9], [27], [36], [39], [49]–[51] and Luque- [9], [45], [50], [51] rods, vertebral body replacement by titanium cage implantation [44], [52], [53], plating [25], [35], [52], [54], bone cement [26], [55], [56]). However, in the absence of evidence no recommendations regarding the use of a specific technique can be given besides possibly bone grafting.…”

“…Among the reviewed cases with vertebral bone involvement we found an overall recurrence rate of 48%, but the stage of disease among the cases varied widely and data on follow-up was mostly scarce (table 1). The available literature states a recurrence rate of 30–100% [18], [24], [28], [38], [44], [57], [58] and in advanced cases interventions might generally be considered palliative with recurrence inevitable [3], [38]. However, remarkable cases with extensive vertebral CE, multiple palliative operations and prolonged survival over many years and even decades have been described [59]–[61].…”

Intradural extramedullary primary hydatid cyst of the spine: a case report and review of literature

“…Hydatid disease is caused by the parasitic tapeworm Echinococcus ( E. granulosus , and less commonly E. multilocularis ) [1]. Hydatidosis caused by Echinococcus granulosus is an endemic parasitic disease in Mediterranean areas, such as North Africa, Spain, Greece, Turkey, Portugal, Middle East, Australia, New Zealand, South America, Baltic areas, the Philippines, Northern China and the Indian sub-continent [2]. Although the disease is rare in Western Europe and North America [3], but has a worldwide distribution [4] and given the ease of modern travel Echinococcus is a worldwide problem [5].…”

Answer to the Letter to the Editor of F.-A. Dauchy et al. concerning manuscript “Intradural extramedullary primary hydatid cyst of the spine: a case report and review of literature”. Eur Spine J, doi:10.1007/s00586-012-2373-1 by I. Lotfinia, S. Sayyahmelli, A. Mahdkhah, M. Shoja M (2012)

“…The cysts consist of an outer fibrous layer and a cestode-derived inner germinal membrane containing scolices [9]. The innermost germinal layer produces hydatid fluid and may include numerous embryonal scolices termed ''hydatid sand'' [10]. Scolices, the infectious embryonic tapeworms, develop from an outpouching of the germinal layer known as the brood capsule.…”

“…In ruptured cysts, the rate of recurrence will be higher and irrigation with normal saline as well as medical therapy should be performed. Totally, the rate of recurrence is dramatically high, and in different reports the intraoperative seeding is commonly leading to recurrence rates up to 18-100 % [7,10,[12][13][14][15]. Recurrences occur 2-28 months (mean 25.2 months) after the initial operation [16,17].…”