1991, The British Journal of Radiology, 64, 465-466 Case reports
Spinal intradural hydatid cyst in a child By Okan Akhan MD, Alp Dincer MD, l$il Saatci MD, Nadir Gulekon MD and Aytekin Besim MD Department of Radiology, Hacettepe University School of Medicine, Ankara, Turkey 06100 (Received July 1990 and in revised form August 1990) Keywords: Echinococcosis, Paraplegia, Intradural cyst
Hydatid disease is an infestation by the larval stages of Echinococcus granulosus. In man, the liver and lungs are the organs most frequently involved. In the spinal column, hydatid disease usually begins in the vertebral body. Extension into the spinal canal results in spinal cord compression. Intradural hydatid cyst is extremely rare compared with the other types of spinal hydatid lesions (Rayport et al, 1964; Ley & Marti, 1970; Pamir et al, 1984). Case report A 6-year-old boy was admitted to hospital because of progressive paraplegia, which had started 2 months previously. He was said to have lost bladder and bowel control in the last few weeks. Physical examination revealed marked reduction in the motor power of the lower limbs. There was bilateral clonus, a positive Babinski's sign and hyperactivity of the deep tendon reflexes at the lower extremities. The chest radiograph showed multiple pulmonary opacities which were shown on thoracic sonography to be cystic in nature, compatible with hydatid cysts. Abdominal ultrasound revealed multiple anechoic cysts with double contours which were suggestive of hydatid disease. A myelogram demonstrated an intradural, extramedullary mass between the levels T9 to T i l ; and an immediate subsequent computed tomography (CT) scan of the region revealed an intradural cystic lesion. Immunologic studies were positive for Echinococcus granulosus. The erythrocyte sedimentation rate was 80 mm/h and the complete blood count showed an eosinophilia of 8%. On the basis of these radiographic, sonographic and immunological findings a diagnosis of hydatidosis was made, and treatment with Mebendazole (50 mg/Kg orally) was begun. In order to relieve the spinal cord compression, laminectomy was performed between the levels of T9 and LI. The dura mater was distended between T10 and Tl 1, where the spinal cord was compressed and displaced to the left by a cyst with firm adhesions to the dura mater. The dimensions of the cyst were 3 x 2 x 1 cm. The dura mater and the cyst were excised. The histopathologic findings confirm the diagnosis. Discussion
Hydatid disease due to Echinococcus granulosus are found in bone in 0.5% to 2% of all cases. Involvement of the vertebral column occurs in about one-half of the osseous cases because of porto-vertebral shunting (Rayport et al, 1964; Apt et al, 1976). Spinal hydatid disease may present with paraplegia or nerve root compression. The spinal hydatid lesions causing para-
Address correspondence to: Dr Alp Dincer, Hacettepe Tip Fakultesi Radyoloji Anabilim Dah, Ankara, Turkey 06100. Vol. 64, No. 761
Figure 1. Lateral and both oblique views obtained by iohexol myelography show a rounded intradural mass between the levels of T9-T11 which has compressed the spinal cord to the left. There are no osseous changes.
plegia are classified into five major groups (Braithwaite & Lees, 1981). 1. 2. 3. 4. 5.
Primary intramedullary cyst Intradural extramedullary hydatid cyst Extradural intraspinal hydatid cyst Hydatid disease of the vertebra Para vertebral hydatid disease
In 1970, Ley and Marti reported an intramedullary hydatid cyst, but, to our knowledge, there are no other references to such cysts. Primary extradural intraspinal hydatid cysts have been reported before, but usually they are considered to arise from an undetected bony focus (Rayport et al, 1964; Pamir et al, 1984). Paraplegia in hydatid disease is usually due to paravertebral hydatid disease or hydatid disease of the vertebra which are not easily differentiated. Primary intradural hydatid cysts are rare and are almost always single. Multiple intradural cysts may develop secondary to rupture and dissemination from an intracranial cyst (Rayport et al, 1964). Being usually located in the dorsal region, primary intradural cysts may lead to rapid spinal cord compression. However, the prognosis is excellent after operation and recurrence is rare. In 1967, Acquaviva et al reviewed eight cases of extramedullary subarachnoidal hydatid cysts, Carrea 465
Case reports and Murphy reported another case in 1964, and there have been several reports of intradural hydatid cysts since 1967 (Karvounis, 1977; Bettaieb et al, 1978; Sharma et al, 1981; Pamir et al, 1984). Our case is of interest because this patient is the youngest of all reported cases. Furthermore, our report is the first CT demonstration of an intradural hydatid cyst. References ACQUAVIVA,
R., TAMIC, P. M.,
KERDOUDI,
H.,
BRU, P.,
LEVY-LEBAR, J. P., OURADON, J., THERENOT, C, BOUFLET-LAHLOU, S. & FRAN^OIS-BENSIMON, S. S., 1967.
L'hydatidose de moelle et du rachis. observations. J. Med. Maroc, 3,2S9-330.
Etude
de 21
APT, W. L., FIERRO, J. L., CALDERON, C , PEREZ, C. & MUJICA,
P., 1976. Vertebral hydatid disease. Journal of Neurosurgery, 44, 72-76. BETTAIEB, A., KHALDI, T., BEN RHOUMA, T. & TOUIBI, S., 1978.
L'echinococcose vertebro-medullarie. 205-210.
Neurochirurgie, 24,
BRAITHWAITE, P. A. & LEES, R. F., 1981. Vertebral hydatid
disease: radiological assessment. Radiology, 140, 763-766. CARREA, R. & MURPHY, G., 1964. Primary hydatid cyst of the spinal cord. Ada Neurologica Latino Americana, 10, 308-312. KARVOUNIS, P. C , SINGOUNAS, E. G. & TSAPROUNIS, G., 1977.
Intradural spinal echinococcus simulating lumbar disc protrusion. Neurochirurgia, 20, 3-60. LEY, A. & MARTI, A., 1970. Intramedullary hydatid cyst. Journal of Neurosurgery, 33, 457-459. PAMIR, M. N., AKALAN, N., OZGEN, T. & ERBENGI, A., 1984.
Spinal hydatid cysts. Surgical Neurology, 21, 53-57. RAYPORT, M., WISOFF, H. S. & ZAIMAN, H., 1964. Vertebral
echinococcosis. Journal of Neurosurgery, 21, 647-659. SHARMA, A., KASHYAP, V., ABRAHAM, J. & KURIAN, S., 1981.
Intradural hydatid cyst. Surgical Neurology, 16, 235-237.
Figure 2. The computed myelograms reveal an intradural unilocular cystic lesion having a CT density between 25 and 50 H. Daughter cysts can not be seen within the cyst.
466
The British Journal of Radiology, May 1991
Spinal intradural hydatid cyst in a child By Okan Akhan MD, Alp Dincer MD, l$il Saatci MD, Nadir Gulekon MD and Aytekin Besim MD Department of Radiology, Hacettepe University School of Medicine, Ankara, Turkey 06100 (Received July 1990 and in revised form August 1990) Keywords: Echinococcosis, Paraplegia, Intradural cyst
Hydatid disease is an infestation by the larval stages of Echinococcus granulosus. In man, the liver and lungs are the organs most frequently involved. In the spinal column, hydatid disease usually begins in the vertebral body. Extension into the spinal canal results in spinal cord compression. Intradural hydatid cyst is extremely rare compared with the other types of spinal hydatid lesions (Rayport et al, 1964; Ley & Marti, 1970; Pamir et al, 1984). Case report A 6-year-old boy was admitted to hospital because of progressive paraplegia, which had started 2 months previously. He was said to have lost bladder and bowel control in the last few weeks. Physical examination revealed marked reduction in the motor power of the lower limbs. There was bilateral clonus, a positive Babinski's sign and hyperactivity of the deep tendon reflexes at the lower extremities. The chest radiograph showed multiple pulmonary opacities which were shown on thoracic sonography to be cystic in nature, compatible with hydatid cysts. Abdominal ultrasound revealed multiple anechoic cysts with double contours which were suggestive of hydatid disease. A myelogram demonstrated an intradural, extramedullary mass between the levels T9 to T i l ; and an immediate subsequent computed tomography (CT) scan of the region revealed an intradural cystic lesion. Immunologic studies were positive for Echinococcus granulosus. The erythrocyte sedimentation rate was 80 mm/h and the complete blood count showed an eosinophilia of 8%. On the basis of these radiographic, sonographic and immunological findings a diagnosis of hydatidosis was made, and treatment with Mebendazole (50 mg/Kg orally) was begun. In order to relieve the spinal cord compression, laminectomy was performed between the levels of T9 and LI. The dura mater was distended between T10 and Tl 1, where the spinal cord was compressed and displaced to the left by a cyst with firm adhesions to the dura mater. The dimensions of the cyst were 3 x 2 x 1 cm. The dura mater and the cyst were excised. The histopathologic findings confirm the diagnosis. Discussion
Hydatid disease due to Echinococcus granulosus are found in bone in 0.5% to 2% of all cases. Involvement of the vertebral column occurs in about one-half of the osseous cases because of porto-vertebral shunting (Rayport et al, 1964; Apt et al, 1976). Spinal hydatid disease may present with paraplegia or nerve root compression. The spinal hydatid lesions causing para-
Address correspondence to: Dr Alp Dincer, Hacettepe Tip Fakultesi Radyoloji Anabilim Dah, Ankara, Turkey 06100. Vol. 64, No. 761
Figure 1. Lateral and both oblique views obtained by iohexol myelography show a rounded intradural mass between the levels of T9-T11 which has compressed the spinal cord to the left. There are no osseous changes.
plegia are classified into five major groups (Braithwaite & Lees, 1981). 1. 2. 3. 4. 5.
Primary intramedullary cyst Intradural extramedullary hydatid cyst Extradural intraspinal hydatid cyst Hydatid disease of the vertebra Para vertebral hydatid disease
In 1970, Ley and Marti reported an intramedullary hydatid cyst, but, to our knowledge, there are no other references to such cysts. Primary extradural intraspinal hydatid cysts have been reported before, but usually they are considered to arise from an undetected bony focus (Rayport et al, 1964; Pamir et al, 1984). Paraplegia in hydatid disease is usually due to paravertebral hydatid disease or hydatid disease of the vertebra which are not easily differentiated. Primary intradural hydatid cysts are rare and are almost always single. Multiple intradural cysts may develop secondary to rupture and dissemination from an intracranial cyst (Rayport et al, 1964). Being usually located in the dorsal region, primary intradural cysts may lead to rapid spinal cord compression. However, the prognosis is excellent after operation and recurrence is rare. In 1967, Acquaviva et al reviewed eight cases of extramedullary subarachnoidal hydatid cysts, Carrea 465
Case reports and Murphy reported another case in 1964, and there have been several reports of intradural hydatid cysts since 1967 (Karvounis, 1977; Bettaieb et al, 1978; Sharma et al, 1981; Pamir et al, 1984). Our case is of interest because this patient is the youngest of all reported cases. Furthermore, our report is the first CT demonstration of an intradural hydatid cyst. References ACQUAVIVA,
R., TAMIC, P. M.,
KERDOUDI,
H.,
BRU, P.,
LEVY-LEBAR, J. P., OURADON, J., THERENOT, C, BOUFLET-LAHLOU, S. & FRAN^OIS-BENSIMON, S. S., 1967.
L'hydatidose de moelle et du rachis. observations. J. Med. Maroc, 3,2S9-330.
Etude
de 21
APT, W. L., FIERRO, J. L., CALDERON, C , PEREZ, C. & MUJICA,
P., 1976. Vertebral hydatid disease. Journal of Neurosurgery, 44, 72-76. BETTAIEB, A., KHALDI, T., BEN RHOUMA, T. & TOUIBI, S., 1978.
L'echinococcose vertebro-medullarie. 205-210.
Neurochirurgie, 24,
BRAITHWAITE, P. A. & LEES, R. F., 1981. Vertebral hydatid
disease: radiological assessment. Radiology, 140, 763-766. CARREA, R. & MURPHY, G., 1964. Primary hydatid cyst of the spinal cord. Ada Neurologica Latino Americana, 10, 308-312. KARVOUNIS, P. C , SINGOUNAS, E. G. & TSAPROUNIS, G., 1977.
Intradural spinal echinococcus simulating lumbar disc protrusion. Neurochirurgia, 20, 3-60. LEY, A. & MARTI, A., 1970. Intramedullary hydatid cyst. Journal of Neurosurgery, 33, 457-459. PAMIR, M. N., AKALAN, N., OZGEN, T. & ERBENGI, A., 1984.
Spinal hydatid cysts. Surgical Neurology, 21, 53-57. RAYPORT, M., WISOFF, H. S. & ZAIMAN, H., 1964. Vertebral
echinococcosis. Journal of Neurosurgery, 21, 647-659. SHARMA, A., KASHYAP, V., ABRAHAM, J. & KURIAN, S., 1981.
Intradural hydatid cyst. Surgical Neurology, 16, 235-237.
Figure 2. The computed myelograms reveal an intradural unilocular cystic lesion having a CT density between 25 and 50 H. Daughter cysts can not be seen within the cyst.
466
The British Journal of Radiology, May 1991
