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Giant bipolar intramedullary nondysraphic spinal cord lipoma: A case report
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NEUCHI-951; No. of Pages 3
Neurochirurgie xxx (2018) xxx–xxx
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Clinical case
Giant bipolar intramedullary nondysraphic spinal cord lipoma: A case report R. Kouadria ∗ , A. Si Ali , Y. Aidouni , W. Maaz , C. Mhamdi Department of neurosurgery and radiology, faculty of Medicine, UDL Sidi Bel Abbes, Algeria
a r t i c l e
i n f o
Article history: Received 10 August 2017 Received in revised form 19 June 2018 Accepted 22 August 2018 Available online xxx Keywords: Spinal cord Lipoma Nondysraphic
a b s t r a c t Introduction. – Intramedullary spinal cord lipomas (IMSCL) are a rare entity, accounting for less than 1% of spinal tumors. Most cases are associated with lumbosacral dysraphism and occur predominantly in the pediatric population. Material and method. – We report the case of an isolated bipolar lipoma, which was very large in the cervicodorsal region and small in the conus medularis. The patient was a 9-years-old male who had muscle weakness for approximately 6 months. MRI showed a hyperintense lesion on T1, which was less intense on T2, without contrast enhancement at the cervicodorsal location and the conus medularis. At his admission, the patient experienced severe decompensation with flaccid tetraplegia and abdominal respiration. He immediately underwent decompression and partial resection of the cervical lesion. The anatomical pathology evaluation confirmed the diagnosis of a lipoma. Discussion/conclusion. – The possibility of quality resection of intramedullary spinal cord lipomas is controversial due to the lack of a cleavage plane with the anterior tracts. Any attempt at total resection would expose the patient to the risk of neurological worsening which is often irreversible. In our case, the surgical indication was self-imposed, the main objective of which was decompression. © 2018 Published by Elsevier Masson SAS.
1. Introduction Intramedullary spinal cord lipomas (IMSCL) are rare benign tumors, accounting for less than 1% of spinal tumors. The most common are spinal dysraphic lipomas. The lack of dysraphism makes IMSCL a rare entity and its diagnosis more difficult. MRI, especially the fat saturation sequences, is very suggestive but may pose a problem of differential diagnosis with dermoid cysts. Surgery is the treatment of choice if there is a real motor deficit and signs of progression on imaging; however the option of total excision remains very controversial because of the morbidity risk. We report the case of giant bipolar cervicodorsal and conus medularis lipoma without dysraphism. The diagnosis and therapeutic features are discussed in the light of a review of the literature.
2. Observation The 9-year-old child (B.Y.), who was attending school, consulted for repeated nuchal stiffness with appearance of paresthesia in both upper limbs. The progression led to muscular weakness of the four
∗ Corresponding author. E-mail address: [email protected] (R. Kouadria).
limbs over 6 months, with recent loss of walking autonomy and urinary retention. At admission, the child was conscious. A neurological examination found severe spastic tetraparesis with McCormick grade of 4. Spinal cord MRI revealed an extensive intramedullary lesion from C4 to T8, with a hyperintense signal on T1-weighted sequences and less intense signal on T2-weighted sequences, without enhancement. This lesion was attenuated on fat saturation with cervico-bulbar edema. It widened the spinal cord and erased the epidural space. A second lesion with identical imaging features was located in the conus medularis (Fig. 1). The diagnosis of a dermoid cyst was retained. One day after admission, the patient experienced severe neurological worsening with flaccid tetraplegia and paradoxical breathing. We operated the child immediately. The surgery consisted of decompression and mass reduction through cervical myelotomy extending from C3 to C7. The lesion was yellowish with small vessels, friable, hemorrhagic, and difficult to grasp, but relatively easy to dissect in contact with the posterior fasciculus with a clear plane. This plane disappeared progressively towards the anterior tract. The excision was deliberately partial (Fig. 2). In the immediate postoperative course, there was a transient improvement of the respiratory disorders. One day after surgery,
https://doi.org/10.1016/j.neuchi.2018.08.005 0028-3770/© 2018 Published by Elsevier Masson SAS.
Please cite this article in press as: Kouadria R, et al. Giant bipolar intramedullary nondysraphic spinal cord lipoma: A case report. Neurochirurgie (2018), https://doi.org/10.1016/j.neuchi.2018.08.005
G Model NEUCHI-951; No. of Pages 3 2
ARTICLE IN PRESS R. Kouadria et al. / Neurochirurgie xxx (2018) xxx–xxx
Fig. 1. MRI. A sagittal T1W image demonstrates a homogenous well-defined hyperintense intradural cervico-dorsal spinal cord lesion (A). A T2W image showing a hyperintense lesion of the conus medularis, but the mass lesion is less intense compared to the T1W images (B). The signal intensity dropped dramatically in fat saturation sequence (C). An axila T1W image demonstrating the intramedullary location of the lesion (D).
Fig. 2. MRI A T1 T2 weighed sagittal MRI, showing a partial resection.
the patient had respiratory distress which required him to be transferred to the intensive care unit for intubation and ventilation. Pulmonary X-ray and CT scanning revealed left lung atelectasis due to community-acquired pneumonia on the main branch. The patient’s recovery was favorable with disappearance of the respiratory disorders and a slight improvement in motor strength. After 4 months of neurological and respiratory rehabilitation, the child regained full use of both upper limbs with the possibility of using a wheelchair. On the pulmonary level, the atelectasis was completely resolved, which allowed us to bring in the patient for a second surgical procedure for the excision of the conus medularis lesion. The excision was partial through a T12-L1 laminectomy and myelotomy. This lipoma had the same appearance as the first lipoma excised. The postoperative course was marked by an improvement in lower limb tonus with persistence of bladder retention.
In the two locations, histopathology confirmed the diagnosis of lipoma (Fig. 3).
3. Discussion IMSCLs are rare and often associated with dysraphism. They represent less than 1% of adult spinal tumors; however they are more common in the pediatric population with a rate reaching 7% [1,2,3]. Ninety percent of the spinal lipomas are located in the lumbosacral region with dysraphism visible since birth [4]. In contrast, nondysraphic lipomas like our case are completely intradural and have no cutaneous and subcutaneous marks [5]. The clinical features are not specific; however progressive neurological decline such as spastic paresis has often been reported [6]. Also, other less frequent signs such as growth retardation,
Please cite this article in press as: Kouadria R, et al. Giant bipolar intramedullary nondysraphic spinal cord lipoma: A case report. Neurochirurgie (2018), https://doi.org/10.1016/j.neuchi.2018.08.005
G Model NEUCHI-951; No. of Pages 3
ARTICLE IN PRESS R. Kouadria et al. / Neurochirurgie xxx (2018) xxx–xxx
3
In our case, the surgery was dictated by the acute development of paradoxical breathing, which was presumably secondary to C4C5 cervical medullary involvement, hence the choice of performing cervical decompression first. As in our case, Sanli reported that only 50% of cervical lipomas are improved and that attempts at total resection have led to significant morbidity [11]. 4. Conclusion
Fig. 3. Color HE × 250: histology of a lipocytic lipoma made of large fat cells, optically contiguous, polyhydric and regular.
respiratory difficulty, sensory disturbances, gait disorders and scoliosis have been described [7]. MRI is the exam of choice to highlight the IMSCL whose signal is very suggestive on T1-weighted images and also in fat suppression sequences. Since dysraphism is absent, this appearance could lead to confusion with dermoid cysts, like in our case. The lack of experience in the imaging of nondysraphic lipomas is due to their rarity. For this reason, nondysraphic lipomas are often considered as dermoid cysts. IMSCL fat is metabolically similar to body fat. For this reason, and because of the known surgical risk, some authors advocate weight loss and a strict diet in paucisymptomatic and/or stable patients [8]. IMSCL surgery, in addition to the histological diagnosis, must preserve neurological function, provide adequate spinal cord decompression and maintain spinal stability. Prophylactic surgery for asymptomatic patients is not recommended in IMSCL because of its slow progression. However, as in our case, early surgery could have avoided the neurological deterioration which is often irreversible. The main goal is decompression through partial debulking [9]. An attempt at large and aggressive excision could have disastrous neurological consequences due to the absence of a cleavage plane, especially with the motor tracts. Ammerman reported that the two patients who underwent total resection developed postoperative paraplegia. However, those who underwent simple decompression with partial resection had improvement or stabilization of their deficits [10].
Contrary to spinal dysraphic lipomas which are apparent since birth, non-dysraphic intramedullary lipomas are rare, and often have been described as isolated cases. In addition to decompressive laminectomy, debulking of the lipoma is performed systematically to achieve the best long-term neurological results. Aggressive total excision is not recommended because of the absence of an obvious cleavage plane with the spinal cord tissue, especially the anterior tract. However patients with IMSCL who present with significant neurological deficits have a very compromised functional prognosis and generally do not have any improvement with surgical resection. After decompressive surgery, long-term follow-up is necessary and reoperation is indicated when there are signs of neurological and/or volumetric worsening. Disclosure of interest The authors declare that they have no competing interest. References [1] Lee M, Rezai AR, Abbott R, Coelho DH, Epstein FJ. Intramedullary spinal cord lipomas. Neurosurgery 1995;82:394–400. [2] El Mostarchid B, Ali A, Maftah M, Mansouri A, et al. Non-dysraphic intramedullary spinal cord lipoma. A case report. Joint Bone Spine 2002;69:511–4. [3] Endo T, Yoshida Y, Shirane R, Yoshimoto T. Tethered spinal cord with double lipomas. J Neurosurg 95 2001;277. [4] Muraszko K. Spinal dysraphism in the adult and pediatric populations. In: Grossman RG, editor. Principles of neurosurgery. 2nd ed Philadelphia: Lippincott- Raven; 1999. p. 59–75. [5] Blount JP, Elton S. Spinal lipomas. Neurosurg Focus 2001;10:e3. [6] Klekamp J, Fusco M, Samii M. Thoracic intradural extramedullary lipomas. Report of three cases and review of the literature. Acta Neurochir 2001;143:767–73. [7] Naim-ur-Rahman N, Shahat AH, et al. Intramedullary lipoma of the cervicodorsal spinal cord with intracranial extension: case report. Surg Neurol 2006;65:486–9. [8] Akyuz M, Goksu E, Tuncer R. Spontaneous decrease in the size of a residual thoracic intradural lipoma. Br J Neurosurg 2005;19:53–5. [9] Kabir SM, Thompson D, Rezajooi K, Casey AT. Non-dysraphic intradural spinal cord lipoma: case series, literature review and guidelines for management. Acta Neurochir 2010;152:1139–44. [10] Ammerman BJ, Henry JM, De Girolami U, Earle KM. Intradural lipomas of the spinal cord: a clinico-pathological correlation. J Neurosurg 1976;44:331–6. [11] Sanli AM, Türko˘glu E, Kahveci R, Sekerci Z. Intradural lipoma of the cervicothoracic spinal cord with intracranial extension. Childs Nerv Syst 2010;26:847–52.
Please cite this article in press as: Kouadria R, et al. Giant bipolar intramedullary nondysraphic spinal cord lipoma: A case report. Neurochirurgie (2018), https://doi.org/10.1016/j.neuchi.2018.08.005
ARTICLE IN PRESS
NEUCHI-951; No. of Pages 3
Neurochirurgie xxx (2018) xxx–xxx
Disponible en ligne sur
ScienceDirect www.sciencedirect.com
Clinical case
Giant bipolar intramedullary nondysraphic spinal cord lipoma: A case report R. Kouadria ∗ , A. Si Ali , Y. Aidouni , W. Maaz , C. Mhamdi Department of neurosurgery and radiology, faculty of Medicine, UDL Sidi Bel Abbes, Algeria
a r t i c l e
i n f o
Article history: Received 10 August 2017 Received in revised form 19 June 2018 Accepted 22 August 2018 Available online xxx Keywords: Spinal cord Lipoma Nondysraphic
a b s t r a c t Introduction. – Intramedullary spinal cord lipomas (IMSCL) are a rare entity, accounting for less than 1% of spinal tumors. Most cases are associated with lumbosacral dysraphism and occur predominantly in the pediatric population. Material and method. – We report the case of an isolated bipolar lipoma, which was very large in the cervicodorsal region and small in the conus medularis. The patient was a 9-years-old male who had muscle weakness for approximately 6 months. MRI showed a hyperintense lesion on T1, which was less intense on T2, without contrast enhancement at the cervicodorsal location and the conus medularis. At his admission, the patient experienced severe decompensation with flaccid tetraplegia and abdominal respiration. He immediately underwent decompression and partial resection of the cervical lesion. The anatomical pathology evaluation confirmed the diagnosis of a lipoma. Discussion/conclusion. – The possibility of quality resection of intramedullary spinal cord lipomas is controversial due to the lack of a cleavage plane with the anterior tracts. Any attempt at total resection would expose the patient to the risk of neurological worsening which is often irreversible. In our case, the surgical indication was self-imposed, the main objective of which was decompression. © 2018 Published by Elsevier Masson SAS.
1. Introduction Intramedullary spinal cord lipomas (IMSCL) are rare benign tumors, accounting for less than 1% of spinal tumors. The most common are spinal dysraphic lipomas. The lack of dysraphism makes IMSCL a rare entity and its diagnosis more difficult. MRI, especially the fat saturation sequences, is very suggestive but may pose a problem of differential diagnosis with dermoid cysts. Surgery is the treatment of choice if there is a real motor deficit and signs of progression on imaging; however the option of total excision remains very controversial because of the morbidity risk. We report the case of giant bipolar cervicodorsal and conus medularis lipoma without dysraphism. The diagnosis and therapeutic features are discussed in the light of a review of the literature.
2. Observation The 9-year-old child (B.Y.), who was attending school, consulted for repeated nuchal stiffness with appearance of paresthesia in both upper limbs. The progression led to muscular weakness of the four
∗ Corresponding author. E-mail address: [email protected] (R. Kouadria).
limbs over 6 months, with recent loss of walking autonomy and urinary retention. At admission, the child was conscious. A neurological examination found severe spastic tetraparesis with McCormick grade of 4. Spinal cord MRI revealed an extensive intramedullary lesion from C4 to T8, with a hyperintense signal on T1-weighted sequences and less intense signal on T2-weighted sequences, without enhancement. This lesion was attenuated on fat saturation with cervico-bulbar edema. It widened the spinal cord and erased the epidural space. A second lesion with identical imaging features was located in the conus medularis (Fig. 1). The diagnosis of a dermoid cyst was retained. One day after admission, the patient experienced severe neurological worsening with flaccid tetraplegia and paradoxical breathing. We operated the child immediately. The surgery consisted of decompression and mass reduction through cervical myelotomy extending from C3 to C7. The lesion was yellowish with small vessels, friable, hemorrhagic, and difficult to grasp, but relatively easy to dissect in contact with the posterior fasciculus with a clear plane. This plane disappeared progressively towards the anterior tract. The excision was deliberately partial (Fig. 2). In the immediate postoperative course, there was a transient improvement of the respiratory disorders. One day after surgery,
https://doi.org/10.1016/j.neuchi.2018.08.005 0028-3770/© 2018 Published by Elsevier Masson SAS.
Please cite this article in press as: Kouadria R, et al. Giant bipolar intramedullary nondysraphic spinal cord lipoma: A case report. Neurochirurgie (2018), https://doi.org/10.1016/j.neuchi.2018.08.005
G Model NEUCHI-951; No. of Pages 3 2
ARTICLE IN PRESS R. Kouadria et al. / Neurochirurgie xxx (2018) xxx–xxx
Fig. 1. MRI. A sagittal T1W image demonstrates a homogenous well-defined hyperintense intradural cervico-dorsal spinal cord lesion (A). A T2W image showing a hyperintense lesion of the conus medularis, but the mass lesion is less intense compared to the T1W images (B). The signal intensity dropped dramatically in fat saturation sequence (C). An axila T1W image demonstrating the intramedullary location of the lesion (D).
Fig. 2. MRI A T1 T2 weighed sagittal MRI, showing a partial resection.
the patient had respiratory distress which required him to be transferred to the intensive care unit for intubation and ventilation. Pulmonary X-ray and CT scanning revealed left lung atelectasis due to community-acquired pneumonia on the main branch. The patient’s recovery was favorable with disappearance of the respiratory disorders and a slight improvement in motor strength. After 4 months of neurological and respiratory rehabilitation, the child regained full use of both upper limbs with the possibility of using a wheelchair. On the pulmonary level, the atelectasis was completely resolved, which allowed us to bring in the patient for a second surgical procedure for the excision of the conus medularis lesion. The excision was partial through a T12-L1 laminectomy and myelotomy. This lipoma had the same appearance as the first lipoma excised. The postoperative course was marked by an improvement in lower limb tonus with persistence of bladder retention.
In the two locations, histopathology confirmed the diagnosis of lipoma (Fig. 3).
3. Discussion IMSCLs are rare and often associated with dysraphism. They represent less than 1% of adult spinal tumors; however they are more common in the pediatric population with a rate reaching 7% [1,2,3]. Ninety percent of the spinal lipomas are located in the lumbosacral region with dysraphism visible since birth [4]. In contrast, nondysraphic lipomas like our case are completely intradural and have no cutaneous and subcutaneous marks [5]. The clinical features are not specific; however progressive neurological decline such as spastic paresis has often been reported [6]. Also, other less frequent signs such as growth retardation,
Please cite this article in press as: Kouadria R, et al. Giant bipolar intramedullary nondysraphic spinal cord lipoma: A case report. Neurochirurgie (2018), https://doi.org/10.1016/j.neuchi.2018.08.005
G Model NEUCHI-951; No. of Pages 3
ARTICLE IN PRESS R. Kouadria et al. / Neurochirurgie xxx (2018) xxx–xxx
3
In our case, the surgery was dictated by the acute development of paradoxical breathing, which was presumably secondary to C4C5 cervical medullary involvement, hence the choice of performing cervical decompression first. As in our case, Sanli reported that only 50% of cervical lipomas are improved and that attempts at total resection have led to significant morbidity [11]. 4. Conclusion
Fig. 3. Color HE × 250: histology of a lipocytic lipoma made of large fat cells, optically contiguous, polyhydric and regular.
respiratory difficulty, sensory disturbances, gait disorders and scoliosis have been described [7]. MRI is the exam of choice to highlight the IMSCL whose signal is very suggestive on T1-weighted images and also in fat suppression sequences. Since dysraphism is absent, this appearance could lead to confusion with dermoid cysts, like in our case. The lack of experience in the imaging of nondysraphic lipomas is due to their rarity. For this reason, nondysraphic lipomas are often considered as dermoid cysts. IMSCL fat is metabolically similar to body fat. For this reason, and because of the known surgical risk, some authors advocate weight loss and a strict diet in paucisymptomatic and/or stable patients [8]. IMSCL surgery, in addition to the histological diagnosis, must preserve neurological function, provide adequate spinal cord decompression and maintain spinal stability. Prophylactic surgery for asymptomatic patients is not recommended in IMSCL because of its slow progression. However, as in our case, early surgery could have avoided the neurological deterioration which is often irreversible. The main goal is decompression through partial debulking [9]. An attempt at large and aggressive excision could have disastrous neurological consequences due to the absence of a cleavage plane, especially with the motor tracts. Ammerman reported that the two patients who underwent total resection developed postoperative paraplegia. However, those who underwent simple decompression with partial resection had improvement or stabilization of their deficits [10].
Contrary to spinal dysraphic lipomas which are apparent since birth, non-dysraphic intramedullary lipomas are rare, and often have been described as isolated cases. In addition to decompressive laminectomy, debulking of the lipoma is performed systematically to achieve the best long-term neurological results. Aggressive total excision is not recommended because of the absence of an obvious cleavage plane with the spinal cord tissue, especially the anterior tract. However patients with IMSCL who present with significant neurological deficits have a very compromised functional prognosis and generally do not have any improvement with surgical resection. After decompressive surgery, long-term follow-up is necessary and reoperation is indicated when there are signs of neurological and/or volumetric worsening. Disclosure of interest The authors declare that they have no competing interest. References [1] Lee M, Rezai AR, Abbott R, Coelho DH, Epstein FJ. Intramedullary spinal cord lipomas. Neurosurgery 1995;82:394–400. [2] El Mostarchid B, Ali A, Maftah M, Mansouri A, et al. Non-dysraphic intramedullary spinal cord lipoma. A case report. Joint Bone Spine 2002;69:511–4. [3] Endo T, Yoshida Y, Shirane R, Yoshimoto T. Tethered spinal cord with double lipomas. J Neurosurg 95 2001;277. [4] Muraszko K. Spinal dysraphism in the adult and pediatric populations. In: Grossman RG, editor. Principles of neurosurgery. 2nd ed Philadelphia: Lippincott- Raven; 1999. p. 59–75. [5] Blount JP, Elton S. Spinal lipomas. Neurosurg Focus 2001;10:e3. [6] Klekamp J, Fusco M, Samii M. Thoracic intradural extramedullary lipomas. Report of three cases and review of the literature. Acta Neurochir 2001;143:767–73. [7] Naim-ur-Rahman N, Shahat AH, et al. Intramedullary lipoma of the cervicodorsal spinal cord with intracranial extension: case report. Surg Neurol 2006;65:486–9. [8] Akyuz M, Goksu E, Tuncer R. Spontaneous decrease in the size of a residual thoracic intradural lipoma. Br J Neurosurg 2005;19:53–5. [9] Kabir SM, Thompson D, Rezajooi K, Casey AT. Non-dysraphic intradural spinal cord lipoma: case series, literature review and guidelines for management. Acta Neurochir 2010;152:1139–44. [10] Ammerman BJ, Henry JM, De Girolami U, Earle KM. Intradural lipomas of the spinal cord: a clinico-pathological correlation. J Neurosurg 1976;44:331–6. [11] Sanli AM, Türko˘glu E, Kahveci R, Sekerci Z. Intradural lipoma of the cervicothoracic spinal cord with intracranial extension. Childs Nerv Syst 2010;26:847–52.
Please cite this article in press as: Kouadria R, et al. Giant bipolar intramedullary nondysraphic spinal cord lipoma: A case report. Neurochirurgie (2018), https://doi.org/10.1016/j.neuchi.2018.08.005