Cell Biochem Biophys DOI 10.1007/s12013-014-0347-0
ORIGINAL PAPER
Thoracolumbar Arachnoid Cyst with Horner Syndrome: A Case Report and Review of the Literature Yang Gao • Hui Zhang • Jiancheng Yang • Qiang Fu • Jianfeng Zheng • Ming Shuai • Zhengbo Hu • Wei Tan • Wei Cao • Xiao Yang • Anmin Jin Renshi Zhou • Hai Lu
•
Ó Springer Science+Business Media New York 2014
Abstract Horner syndrome (HS) results from the interruption of sympathic pathway, and the patients have a group of signs including miosis, ptosis, enophthalmos, and anhydrosis. While HS is mainly caused by cervical sympathetic nerve injury such as sympathetic chain tumor, we report here a HS case caused by a thoracolumbar arachnoid cyst. Imageological examination showed the cyst existed in spinal canal from the T11 to L3 level, which was further confirmed by operation. The tumor attacked the lateral margin of intervertebral foramen at certain stages. In MRI scan, no abnormality was found in the patient’s crania, cervical vertebra, thoracic vertebra, or the other parts. After removal of the cyst with operation, the patient’s HS symptoms and weakness of lower limbs were relieved apparently. Although the sympathetic center origins from the cornu laterale medullae spinalis of T1 to L3, there are many reports about HS caused by lumbar anesthesia and epidural anesthesia according to our literature review, and there is no report about HS results from intraspinal spaceoccupying lesion below T11 level. Our finding suggests that when the sympathetic center below the level of T11 emits nerve to dominate abdominal viscera, it can also Yang Gao and Hui Zhang contributed equally as first authors. Y. Gao H. Zhang J. Yang Q. Fu J. Zheng Z. Hu W. Tan W. Cao X. Yang A. Jin H. Lu (&) Orthopaedic Center, Zhujiang Hospital, Southern Medical University, 253 Gongye Dadao Zhong, Guangzhou 510282, People’s Republic of China e-mail: [email protected] M. Shuai R. Zhou (&) Department of Orthopaedic, Yangchun People’s Hospital, 24 Huancheng Nanlu, Yangchun, Guangdong, People’s Republic of China e-mail: [email protected]
control the sweat glands from face to feet, including pupils and eyelids. When physicians encounter patients with HS and one side of the body and abdominal viscera sympathetic syndromes, the pathological changes in lower thoracic vertebra or lumbar vertebra should be taken in consideration. Keywords Horner syndrome Arachnoid cyst Thoracic vertebra Lumbar vertebra
Introduction Horner syndrome, also known as cervical sympathetic paralysis syndrome, is characterized by a classic triad of ipsilateral pupillary miosis, partial eyelid ptosis, and is associated with apparent enophthalmos and vasodilatation [1]. It may also present as a sequela of cervical sympathetic denervation. HS can be caused by a variety of etiologies such as trauma, surgery, and tumor that damage the neck and brain stem sympathetic nerve [2]. HS in the obstetrics setting was first reported by Kepes et al. in 1972 as an uncommon complication of lumbar epidural block [3]. The reported incidence of HS after lumbar labor epidural block ranges from 0.5 to 4 % [4–6]. Arachnoid cysts are fluid-filled duplications or splittings of the arachnoid layer with a content that is similar but not equal to the cerebrospinal fluid. Arachnoid cysts are not actual neurodegenerative disorders. The underlying defect of the texture of the arachnoid layer is probably congenital in nature [7]. It is categorized as congenital and secondary arachnoid cyst. Congenital arachnoid cyst is formed by cerebrospinal fluid surrounded in arachnoid bag structure, which is not connected with subarachnoid space [8]. Arachnoid cyst is a kind of congenital benign brain cysts,
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which is caused by abnormal arachnoid division. Secondary arachnoid cyst forms in the subarachnoid after inflammatory diseases, trauma, or tumor. The diseases usually have no symptoms but a large volume of cyst can compress the brain tissue and skull, and causes general neural symptoms and skull progressive change [9].
gait of the patient was normal. The physiological curve of the spine, the superficial of skin, and the muscular tension were normal. The pathology reflects had not been induced.
Case Report A 44-year-old Cantonese male was hospitalized due to absence of sweating of his right side body and pain on back with lower extremity weakness for 2 years. The patient presented a vague back pain with no obvious cause 2 years ago. The pain had no significant relationship with fatigue, and it aggravated at night accompanied by lower extremity weakness and lack of sweating of the right side body. The patient did not take any medications in the past 2 years. The above symptoms gradually worsened with the emergence of abdominal discomfort. On physical examination, the right eye of the patient showed miosis, ptosis, and enophthalmos. His right hemiface and limb were dry with decreased sweat secretion. The
Fig. 2 CT scan shows the spinal canal from T12 to L1 becomes larger, especially on the right side
123
Fig. 1 DR shows no significant abnormality in the spine
Cell Biochem Biophys
The spinous process was not sensitive to percussion or tenderness. The muscle force of the legs and upper limbs were grade IV and V, respectively. X-ray showed there was no significant abnormality in the spine (Fig. 1). CT scan showed the spinal canal from T12 to L1 enlarged, especially on the right side (Fig. 2). MRI further delineates this finding, showing an epidural cystic occupancy was benign at the level from T11 to L3 and the degeneration of the disci intervertebrales between L5 and S1 (Fig. 3). No abnormality was seen on the MRI of the cranial and cervical spine. After operation resection of the lesion, the pathological finding showed the mass was an arachnoid cyst (Fig. 4). Under the tracheal intubation anesthesia, the cyst was excised through removal of the whole T11-L13 vertebral plate in the posterior longitudinal approach. Then the pedicle screw internal fixation was performed (Fig. 5). During surgery, a 2 9 4 9 12 cm3 cystic structure in spinal canal behind the dural sac was found, partially adhering to dura mater spinalis and attacking the edge of T11–L2 intervertebral foramen. The cyst was full of light yellow transparent liquid and there was abundant blood supplying around the cyst.
Three days after the operation, the patient’s anhydrosis of the right limb and miosis of his right eye were relieved apparently. Ten days later, the symptoms above were in complete remission, and the back pain and lower extremity weakness were also relieved.
Discussion A large number of reports revealed that the main causes of HS are canalis spinalis space-occupying lesions, paraspinal tumors and sympathetic ganglia tumors. The lowest level of canalis spinalis space-occupying lesions we found was T2, which had been reported by Fridman in a case of solitary plasmacytoma of second thoracic vertebra [10]. The tumor of the patient in our case was at the level between T11 and L3, which has not been reported before. Moreover, beside HS symptoms, our patient also had abdominal discomfort and dry skin without sweat on his right side body. It indicates that intraspinal tumors in this case can lead to the whole side body sympathetic damage from head to foot. There is no similar case report yet.
Fig. 3 MRI shows an epidural cystic occupancy at the level from T11 to L3 and the degeneration of the disci intervertebrales between L5 and S1
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Fig. 4 Pathological finding shows the mass is an arachnoid cyst
Some reports indicate that lumbar anesthesia and lumbar epidural anesthesia can cause HS symptoms, especially in those who have epidural anesthesia for obstetric procedures. The diffusion range of anesthesia can be very wide, while the range of intraspinal cyst is limited. What we found further confirmed that the damage of spinal nerve in lower thoracic vertebrae or lumbar vertebra could cause sympathetic symptoms in cephalofacial. The findings brought new subject to the anatomy of sympathetic nerve studies to understand whether the nerve fibers from lower thoracolumbar sympathetic nerve center can affect the function of the cervical sympathetic ganglia through sympathetic ganglia or sympathetic chain. Related anatomy findings have not been reported. In conclusion, the nerves that arise from the sympathetic nerve center below the T11 level can participate in controlling the function of the whole one-side body from head to foot, including eyelids, pupils, internal organs, skin sweat gland, and abdominal organs. There may be some nerve fibers that connect the thoracolumbar sympathetic nerve with the cervical region. When a patient has sympathetic symptoms, the extent of the lesion can exist from cervical part to the third lumbar. In this case, the patient’s acratia in the lower extremities (the myodynamia of the upper limbs were normal) and abdomen discomfort were more useful to conform the disease location. Acknowledgments This work was supported by National Natural Science Foundation of China (Grant Nos. 81372869 and 81172555 to Y.G., Z.H., A.J., H.Z., and H.L.) and National Natural Science Fundation of Guangdong Province (Grant No. S2013010016052 to R.Z. and H.L.).
References
Fig. 5 The cyst was excised and the pedicle screw internal fixation was performed
We did the MRI of the head, the cervical vertebra, the thoracic vertebrae, and the lumbar vertebrae before operation and did not find any spinal or the paraspinal sympathetic chain damages except the analis spinalis spaceoccupying lesion from T11 to L3. After tumor resection, the patient’s HS symptoms and the sympathetic ganglia damage of one-side limbs were completely relieved. This also indicates the symptoms were caused by the compression of the cyst.
123
1. Pirouzian, A., Holz, H. A., Ip, K. C., & Sudesh, R. (2010). Acquired infantile Horner syndrome and spontaneous internal carotid artery dissection: A case report and review of literature. Journal of AAPOS : The Official Publication of the American Association for Pediatric Ophthalmology and Strabismus/American Association for Pediatric Ophthalmology and Strabismus, 14, 172–174. 2. Syed, M. I., Baring, D., Addidle, M., Murray, C., & Adams, C. (2007). Lemierre syndrome: Two cases and a review. The Laryngoscope, 117, 1605–1610. 3. Kepes, E. R., Stark, D., Pantuck, E., et al. (1972). Horner’s syndrome following caudal anesthesia. New York State Journal of Medicine, 72, 946–947. 4. Clayton, K. C. (1983). The incidence of Horner’s syndrome during lumbar extradural for elective Caesarean section and provision of analgesia during labour. Anaesthesia, 38, 583–585. 5. Mohan, J., & Potter, J. M. (1975). Pupillary constriction and ptosis following caudal epidural analgesia. Anaesthesia, 30, 769–773. 6. Mohan, J., Lloyd, J. W., & Potter, J. M. (1973). Pupillary constriction following extradural analgesia. Injury, 5, 151–152. 7. Westermaier, T., Schweitzer, T., & Ernestus, R. I. (2012). Arachnoid cysts. Advances in Experimental Medicine and Biology, 724, 37–50.
Cell Biochem Biophys 8. Inagaki, T. (2011). Congenital anomalies in the central nervous system (12) arachnoid cyst. No shinkei geka. Neurological Surgery, 39, 1105–1111. 9. Greenberg, M. S., & Arredondo, N. (2006). Handbook of neurosurgery (6th ed.). Lakeland, FL, New York: Greenberg Graphics; Thieme Medical Publishers.
10. Fridman, A. (2004). Reversible Horner syndrome caused by solitary plasmacytoma of second thoracic vertebrae. Leukaemia & Lymphoma, 45(12), 2531–2532.
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ORIGINAL PAPER
Thoracolumbar Arachnoid Cyst with Horner Syndrome: A Case Report and Review of the Literature Yang Gao • Hui Zhang • Jiancheng Yang • Qiang Fu • Jianfeng Zheng • Ming Shuai • Zhengbo Hu • Wei Tan • Wei Cao • Xiao Yang • Anmin Jin Renshi Zhou • Hai Lu
•
Ó Springer Science+Business Media New York 2014
Abstract Horner syndrome (HS) results from the interruption of sympathic pathway, and the patients have a group of signs including miosis, ptosis, enophthalmos, and anhydrosis. While HS is mainly caused by cervical sympathetic nerve injury such as sympathetic chain tumor, we report here a HS case caused by a thoracolumbar arachnoid cyst. Imageological examination showed the cyst existed in spinal canal from the T11 to L3 level, which was further confirmed by operation. The tumor attacked the lateral margin of intervertebral foramen at certain stages. In MRI scan, no abnormality was found in the patient’s crania, cervical vertebra, thoracic vertebra, or the other parts. After removal of the cyst with operation, the patient’s HS symptoms and weakness of lower limbs were relieved apparently. Although the sympathetic center origins from the cornu laterale medullae spinalis of T1 to L3, there are many reports about HS caused by lumbar anesthesia and epidural anesthesia according to our literature review, and there is no report about HS results from intraspinal spaceoccupying lesion below T11 level. Our finding suggests that when the sympathetic center below the level of T11 emits nerve to dominate abdominal viscera, it can also Yang Gao and Hui Zhang contributed equally as first authors. Y. Gao H. Zhang J. Yang Q. Fu J. Zheng Z. Hu W. Tan W. Cao X. Yang A. Jin H. Lu (&) Orthopaedic Center, Zhujiang Hospital, Southern Medical University, 253 Gongye Dadao Zhong, Guangzhou 510282, People’s Republic of China e-mail: [email protected] M. Shuai R. Zhou (&) Department of Orthopaedic, Yangchun People’s Hospital, 24 Huancheng Nanlu, Yangchun, Guangdong, People’s Republic of China e-mail: [email protected]
control the sweat glands from face to feet, including pupils and eyelids. When physicians encounter patients with HS and one side of the body and abdominal viscera sympathetic syndromes, the pathological changes in lower thoracic vertebra or lumbar vertebra should be taken in consideration. Keywords Horner syndrome Arachnoid cyst Thoracic vertebra Lumbar vertebra
Introduction Horner syndrome, also known as cervical sympathetic paralysis syndrome, is characterized by a classic triad of ipsilateral pupillary miosis, partial eyelid ptosis, and is associated with apparent enophthalmos and vasodilatation [1]. It may also present as a sequela of cervical sympathetic denervation. HS can be caused by a variety of etiologies such as trauma, surgery, and tumor that damage the neck and brain stem sympathetic nerve [2]. HS in the obstetrics setting was first reported by Kepes et al. in 1972 as an uncommon complication of lumbar epidural block [3]. The reported incidence of HS after lumbar labor epidural block ranges from 0.5 to 4 % [4–6]. Arachnoid cysts are fluid-filled duplications or splittings of the arachnoid layer with a content that is similar but not equal to the cerebrospinal fluid. Arachnoid cysts are not actual neurodegenerative disorders. The underlying defect of the texture of the arachnoid layer is probably congenital in nature [7]. It is categorized as congenital and secondary arachnoid cyst. Congenital arachnoid cyst is formed by cerebrospinal fluid surrounded in arachnoid bag structure, which is not connected with subarachnoid space [8]. Arachnoid cyst is a kind of congenital benign brain cysts,
123
Cell Biochem Biophys
which is caused by abnormal arachnoid division. Secondary arachnoid cyst forms in the subarachnoid after inflammatory diseases, trauma, or tumor. The diseases usually have no symptoms but a large volume of cyst can compress the brain tissue and skull, and causes general neural symptoms and skull progressive change [9].
gait of the patient was normal. The physiological curve of the spine, the superficial of skin, and the muscular tension were normal. The pathology reflects had not been induced.
Case Report A 44-year-old Cantonese male was hospitalized due to absence of sweating of his right side body and pain on back with lower extremity weakness for 2 years. The patient presented a vague back pain with no obvious cause 2 years ago. The pain had no significant relationship with fatigue, and it aggravated at night accompanied by lower extremity weakness and lack of sweating of the right side body. The patient did not take any medications in the past 2 years. The above symptoms gradually worsened with the emergence of abdominal discomfort. On physical examination, the right eye of the patient showed miosis, ptosis, and enophthalmos. His right hemiface and limb were dry with decreased sweat secretion. The
Fig. 2 CT scan shows the spinal canal from T12 to L1 becomes larger, especially on the right side
123
Fig. 1 DR shows no significant abnormality in the spine
Cell Biochem Biophys
The spinous process was not sensitive to percussion or tenderness. The muscle force of the legs and upper limbs were grade IV and V, respectively. X-ray showed there was no significant abnormality in the spine (Fig. 1). CT scan showed the spinal canal from T12 to L1 enlarged, especially on the right side (Fig. 2). MRI further delineates this finding, showing an epidural cystic occupancy was benign at the level from T11 to L3 and the degeneration of the disci intervertebrales between L5 and S1 (Fig. 3). No abnormality was seen on the MRI of the cranial and cervical spine. After operation resection of the lesion, the pathological finding showed the mass was an arachnoid cyst (Fig. 4). Under the tracheal intubation anesthesia, the cyst was excised through removal of the whole T11-L13 vertebral plate in the posterior longitudinal approach. Then the pedicle screw internal fixation was performed (Fig. 5). During surgery, a 2 9 4 9 12 cm3 cystic structure in spinal canal behind the dural sac was found, partially adhering to dura mater spinalis and attacking the edge of T11–L2 intervertebral foramen. The cyst was full of light yellow transparent liquid and there was abundant blood supplying around the cyst.
Three days after the operation, the patient’s anhydrosis of the right limb and miosis of his right eye were relieved apparently. Ten days later, the symptoms above were in complete remission, and the back pain and lower extremity weakness were also relieved.
Discussion A large number of reports revealed that the main causes of HS are canalis spinalis space-occupying lesions, paraspinal tumors and sympathetic ganglia tumors. The lowest level of canalis spinalis space-occupying lesions we found was T2, which had been reported by Fridman in a case of solitary plasmacytoma of second thoracic vertebra [10]. The tumor of the patient in our case was at the level between T11 and L3, which has not been reported before. Moreover, beside HS symptoms, our patient also had abdominal discomfort and dry skin without sweat on his right side body. It indicates that intraspinal tumors in this case can lead to the whole side body sympathetic damage from head to foot. There is no similar case report yet.
Fig. 3 MRI shows an epidural cystic occupancy at the level from T11 to L3 and the degeneration of the disci intervertebrales between L5 and S1
123
Cell Biochem Biophys
Fig. 4 Pathological finding shows the mass is an arachnoid cyst
Some reports indicate that lumbar anesthesia and lumbar epidural anesthesia can cause HS symptoms, especially in those who have epidural anesthesia for obstetric procedures. The diffusion range of anesthesia can be very wide, while the range of intraspinal cyst is limited. What we found further confirmed that the damage of spinal nerve in lower thoracic vertebrae or lumbar vertebra could cause sympathetic symptoms in cephalofacial. The findings brought new subject to the anatomy of sympathetic nerve studies to understand whether the nerve fibers from lower thoracolumbar sympathetic nerve center can affect the function of the cervical sympathetic ganglia through sympathetic ganglia or sympathetic chain. Related anatomy findings have not been reported. In conclusion, the nerves that arise from the sympathetic nerve center below the T11 level can participate in controlling the function of the whole one-side body from head to foot, including eyelids, pupils, internal organs, skin sweat gland, and abdominal organs. There may be some nerve fibers that connect the thoracolumbar sympathetic nerve with the cervical region. When a patient has sympathetic symptoms, the extent of the lesion can exist from cervical part to the third lumbar. In this case, the patient’s acratia in the lower extremities (the myodynamia of the upper limbs were normal) and abdomen discomfort were more useful to conform the disease location. Acknowledgments This work was supported by National Natural Science Foundation of China (Grant Nos. 81372869 and 81172555 to Y.G., Z.H., A.J., H.Z., and H.L.) and National Natural Science Fundation of Guangdong Province (Grant No. S2013010016052 to R.Z. and H.L.).
References
Fig. 5 The cyst was excised and the pedicle screw internal fixation was performed
We did the MRI of the head, the cervical vertebra, the thoracic vertebrae, and the lumbar vertebrae before operation and did not find any spinal or the paraspinal sympathetic chain damages except the analis spinalis spaceoccupying lesion from T11 to L3. After tumor resection, the patient’s HS symptoms and the sympathetic ganglia damage of one-side limbs were completely relieved. This also indicates the symptoms were caused by the compression of the cyst.
123
1. Pirouzian, A., Holz, H. A., Ip, K. C., & Sudesh, R. (2010). Acquired infantile Horner syndrome and spontaneous internal carotid artery dissection: A case report and review of literature. Journal of AAPOS : The Official Publication of the American Association for Pediatric Ophthalmology and Strabismus/American Association for Pediatric Ophthalmology and Strabismus, 14, 172–174. 2. Syed, M. I., Baring, D., Addidle, M., Murray, C., & Adams, C. (2007). Lemierre syndrome: Two cases and a review. The Laryngoscope, 117, 1605–1610. 3. Kepes, E. R., Stark, D., Pantuck, E., et al. (1972). Horner’s syndrome following caudal anesthesia. New York State Journal of Medicine, 72, 946–947. 4. Clayton, K. C. (1983). The incidence of Horner’s syndrome during lumbar extradural for elective Caesarean section and provision of analgesia during labour. Anaesthesia, 38, 583–585. 5. Mohan, J., & Potter, J. M. (1975). Pupillary constriction and ptosis following caudal epidural analgesia. Anaesthesia, 30, 769–773. 6. Mohan, J., Lloyd, J. W., & Potter, J. M. (1973). Pupillary constriction following extradural analgesia. Injury, 5, 151–152. 7. Westermaier, T., Schweitzer, T., & Ernestus, R. I. (2012). Arachnoid cysts. Advances in Experimental Medicine and Biology, 724, 37–50.
Cell Biochem Biophys 8. Inagaki, T. (2011). Congenital anomalies in the central nervous system (12) arachnoid cyst. No shinkei geka. Neurological Surgery, 39, 1105–1111. 9. Greenberg, M. S., & Arredondo, N. (2006). Handbook of neurosurgery (6th ed.). Lakeland, FL, New York: Greenberg Graphics; Thieme Medical Publishers.
10. Fridman, A. (2004). Reversible Horner syndrome caused by solitary plasmacytoma of second thoracic vertebrae. Leukaemia & Lymphoma, 45(12), 2531–2532.
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