The outcome of hydatid disease significantly depends on the location of the lesions. In this context the classification of Braithwaite and Lees provides an initial reference for the expected outcome (Braithwaite and Lees 1981). According to their anatomic location, cysts can be divided into intramedullary, intradural-extramedullary, extradural, spinal (vertebral), and paraspinal lesions extending into spinal structures. Besides these anatomic references, especially the spinal level influences the neurologic outcome. There is a remarkable difference regarding the outcome between cervical, thoracic, lumbar, and sacral manifestations of the spinal hydatid disease, especially in advanced stages when irreversible nerve tissue damage exists.

Surgical decompression and chemotherapy are the principal forms of treatment in pediatric and adolescent cases of spinal hydatidosis. Unfortunately even with extensive surgery, results are far from being curative. The recurrence and mortality rates for intraspinal hydatid disease range up to 50 and 15 %, respectively (Taratuto and Venturiello 1997; Toussaint et al. 2001; Wang et al. 2009). Up to 40 % of patients are reported to have recurrent symptomatology within 2 years after the initial operation (Spies et al. 2008; Takci et al. 2008; Taghipoor and Razmkon 2009). As the recurrence rate is high, adjuvant anthelmintic agents are recommended following surgery. Anthelmintic drugs are strongly recommended in case of recurrence, systemic disease, intraoperative rupture or puncture, poor suitability for surgery, and prophylaxis (Papagelopoulos et al. 1997; Schnepper and Johnson 2004; Romig 2009; Sharma et al. 2011). However, a clearly structured aftercare and follow-up calendar including physical examination, imaging, as well as serologic testing is mandatory. In parallel to the aftercare of soft tissue sarcoma, we recommend restaging every 3 months for the first 2 years. This should include CT scans of the abdomen and MRI of the spine. In the following the intervals can be reduced to twice a year up to year 5 and once a year until year 10 after onset of the disease.

The imaging features of intraspinal hydatid cyst usually comprise multiple spherical lesions with clearly defined borders, containing fluid with similar CSF intensity on both CT and MRIs. Though, due to the need for repetitive scanning on a regular basis, we regard MRI for several reasons as the best imaging technique, since it allows early diagnosis of spinal cord compression and more accurate localization of intra- and paraspinal manifestation if multiplanar images are acquired. On MRI it is possible to detect the exact anatomical location of the lesion and to show the viability of the hydatid cysts. On T2-weighted images, a cystic lesion has a high intensity, and a decrease in signal indicates a necrotic cyst. Thus, a decreased signal in this sequence is a characteristic indicator of a necrotic cyst. Furthermore, the layers of the cyst can only be demonstrated by MRI, and the pericyst may show enhancement after contrast material administration due to its rich vascularization. In particular positron emission tomography scanning in combination with CT scan or MRI might show active lesions at a time when clinical symptoms are absent or unspecific and recurrence of parasitic tissue is not detectable by conventional imaging (Brunetti et al. 2010). Imaging of spinal hydatid disease is discussed in Chap. 12.

Blood tests should include benzimidazole blood levels as well as serological tests (Nell et al. 2011). Determination of benzimidazole blood levels, 4 h after the morning dose, is recommended 1, 4, and 12 weeks after initiating drug therapy. Detection of antibody directed to echinococcal polypeptide antigens has the highest degree of specificity. Enzyme-linked immunosorbent assay (ELISA), indirect hemagglutination, and complement fixation tests are reported to be 80–100 % sensitive and 88–96 % specific in abdominal disease. However, the sensitivity decreases abruptly to 25–56 % in extrahepatic disease which limits their use in the diagnosis or follow-up for primary bone disease. Second-line tests like immunoblotting (antigen 5 precipitation: arc-5 test) may be used for confirmation after ELISA and hemagglutination. Laboratory findings of patients with spinal hydatidosis are discussed in Chap. 10.

Albendazole should be administered orally at a dose rate of 10–15 mg/kg/day divided into two doses (WHO 1996). In practice a daily dose of 800 mg is applicable to adults. Intermittent or cyclic treatment is no longer necessary, since albendazole treatment is well tolerated and it has been reported that a dose rate of 20 mg/kg/day is tolerated for up to 5 years. Alternatively if albendazole is not enough tolerated or available, mebendazole could be administered at a daily dose rate of 40–50 mg/kg/day. Benzimidazoles are generally well tolerated. Adverse reactions include hepatotoxicity, alopecia, gastrointestinal disturbances, and leucopenia (Vuitton 2009). Moreover the only contraindication for benzimidazoles is pregnancy. Albendazole is the most widely used benzimidazole worldwide. Benefits of albendazole are a better bioavailability to achieve higher plasma levels, an easy administration, and an overall better efficacy. However, benzimidazoles in general are only parasitostatic and do not erase E. multilocularis metacestodes.