- Department of Neurosurgery, Desert Regional Medical Center, Palm Springs, CA,
- Kentucky College of Osteopathic Medicine, University of Pikeville, Pikeville, KY,
- College of Osteopathic Medicine, Western University of Health Sciences, Pomona, CA, United States,
- School of Medicine, Universidad Anáhuac Querétaro, Cto. Universidades, Santiago, de Querétaro, Mexico,
- William Beaumont School of Medicine, Oakland University, Rochester, Michigan,
- Grossman School of Medicine, New York University, New York,
- Department of Neurosurgery, University of Colorado, Aurora, Colorado, United States.
Department of Neurosurgery, University of Colorado, Aurora, Colorado, United States.
DOI:10.25259/SNI_494_2020Copyright: © 2020 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Brian Fiani1, Taylor Reardon2, Ryne Jenkins3, Claudia Covarrubias4, Manraj Sekhon5, Marisol Soula6, Michael Kortz7. Intramedullary spinal cord cavernous malformations in the pediatric population. 05-Sep-2020;11:275
How to cite this URL: Brian Fiani1, Taylor Reardon2, Ryne Jenkins3, Claudia Covarrubias4, Manraj Sekhon5, Marisol Soula6, Michael Kortz7. Intramedullary spinal cord cavernous malformations in the pediatric population. 05-Sep-2020;11:275. Available from: https://surgicalneurologyint.com/surgicalint-articles/10242/
Background: Intramedullary spinal cavernous malformations (ISCM) account for just 1% of all intramedullary pediatric spinal cord lesions. Pathologically, they are well-circumscribed vascular malformations that typically appear dark blue or reddish-brown, often coming to the spinal cord surface. With regard to the histopathology findings, ISCMs are comprised sinusoidal vascular spaces lined by a single layer of endothelial cells within a loose connective tissue stroma. As these lesions are often misdiagnosed in the pediatric population, appropriate treatment may be unduly delayed.
Methods: The authors performed an extensive review of the published literature (PubMed) focusing on ISCM in the pediatric age group.
Results: The search yielded 17 articles exclusively pertaining to ISCM affecting the pediatric population.
Conclusion: Here, we reviewed the clinical, radiographic, surgical, and outcome data for the treatment of ISCM in the pediatric age groups. Notably, over 50% of pediatric patients with ISCM experienced an improvement in their neurological status after a mean postoperative follow-up duration of 4 years. Future meta-analyses are needed to highlight the potential presence of ISCM and, thereby, decrease the rate of misdiagnosis of these lesions in the pediatric population presenting with recurrent intramedullary spinal cord hemorrhages.
Keywords: Cavernoma, Cavernous malformation, Spinal cord cavernous malformations, Spinal cord, Vascular malformation
Intramedullary spinal cavernous malformations (ISCM) make up only 1% of all intramedullary spinal lesions in the pediatric population. Children most typically present with acute neurological deterioration characterized by the acute onset of severe motor deficits.[
Unfortunately, the misdiagnosis rate for ISCM is estimated to be as high as 55%, as the clinical presentation is too frequently misinterpreted as consistent with cerebral palsy, syringomyelia, and transverse myelitis.[
Cavernous malformations are well-circumscribed vascular malformations that are dark blue or reddish-brown. They can often be seen coming to the spinal cord surface.[
Spinal cavernous malformations often occur in association with multiple cerebral or systemic lesions; therefore, patients should undergo full neuroaxis and complete-body imaging. There is a high incidence of these lesions reported in the Hispanic population likely due to an autosomal dominant alteration of chromosome 7q.[
Magnetic resonance imaging (MRI) is the diagnostic modality of choice as these spinal lesions tend to be occult on angiographic imaging. On MRI, they are well-circumscribed and multilobulated with heterogeneous intralesional T1and T2 signals reflecting a reticulated core. Their “popcorn-like” appearance is attributed to the presence of blood products in different phases of evolution (e.g., best seen on gradient- echo images).[
Differential diagnoses for ISCM include intramedullary tumors (particularly hemangioblastomas) or arteriovenous malformations; angiography may rule out the latter.[
The annual hemorrhage rate for spinal cavernous malformations in both adult and pediatric patients ranges from 1.4% to 6.8%; the re-hemorrhage rate is 2%. Better outcomes are reported for patients undergoing resection versus conservative management.[
Neurological sequelae of intramedullary cavernous malformations in the pediatric population is highly variable. Ren et al. looked at pediatric patients with ISCMs and categorized symptomatic presentations to include pain, weakness, sensory deficits, and bowel/bladder dysfunctions; deficits ranged from ASIA scale grades A-D on admission.[
Pin-pointing the location of ISCM in the cord (ventrally, dorsally, etc.) is critical to operative planning. Surgical is typically indicated by the presence of unremitting and/or progressive neurological symptoms/signs, or for lesions larger >5 mm in diameter, and/or growing.[
Typically, prone positioning is preferred for a posterior approach to the spine, while supine positioning is preferred for an anterior approach. If fusion is required in the thoracolumbar spine, then hip extension may be used to increase lordosis. A lumbar drain can be placed to prevent cerebrospinal fluid leak and pseudomeningocele formation and is typically left in for 1–2 days postoperatively. The surgical resection should be limited the extent of myelotomy to maximize white matter preservation and postoperative deficits. Further, all surgical approaches to the spine (e.g., anterior, anterolateral, posterior, and posterolateral), including minimally invasive approaches, have their own unique respective risks and complications.
Intraoperative neuromonitoring, utilizing electromyography, electroencephalography, somatosensory evoked potentials, and motor evoked potentials are a critical adjunct to limit postoperative neurological deficits for the patient undergoing ISCM surgery.[
The surgical removal of ISCM in the pediatric population may provide adequate functional outcomes. Although most patients remained unchanged from their preoperative status, over 50% improve over a mean follow-up duration of 4 years.[
Rates of hemorrhage and re-hemorrhage are higher in pediatric patients with ISCMs and, thus, should prompt early surgery and complete lesion resections where feasible. Extensive meta- analyses specific to the pediatric population are needed to examine the relationship between specific neurologic sequelae and neuroradiological findings to ultimately decrease the rate of misdiagnosis and hemorrhage rates.
1. Anson JA, Spetzler RF. Surgical resection of intramedullary spinal cord cavernous malformations. J Neurosurg. 1993. 78: 446-51
2. Badhiwala JH, Farrokhyar F, Alhazzani W, Yarascavitch B, Aref M, Algird A. Surgical outcomes and natural history of intramedullary spinal cord cavernous malformations: A single-center series and meta-analysis of individual patient data: Clinic article. J Neurosurg Spine. 2014. 21: 662-76
3. Clark AJ, Wang DD, Lawton MT. Spinal cavernous malformations. Handb Clin Neurol. 2017. 143: 303-8
4. Das JM, Hoang S, Mesfin FB.editors. Cancer, Intramedullary Spinal Cord Tumors. Treasure Island, FL: Stat Pearls; 2020. p.
5. Deutsch H, Shrivistava R, Epstein F, Jallo GI. Pediatric intramedullary spinal cavernous malformations. Spine (Phila Pa 1976). 2001. 26: E427-31
6. Goyal A, Rinaldo L, Alkhataybeh R, Kerezoudis P, Alvi MA, Flemming KD. Clinical presentation, natural history and outcomes of intramedullary spinal cord cavernous malformations. J Neurol Neurosurg Psychiatry. 2019. 90: 695-703
7. Hegde AN, Mohan S, Lim CC. CNS cavernous haemangioma: “Popcorn” in the brain and spinal cord. Clin Radiol. 2012. 67: 380-8
8. Khalatbari MR, Hamidi M, Moharamzad Y. Pediatric intramedullary cavernous malformation of the conus medullaris: Case report and review of the literature. Childs Nerv Syst. 2011. 27: 507-11
9. Kramer CL. Vascular disorders of the spinal cord. Continuum (Minneap Minn). 2018. 24: 407-26
10. Mikula A, Kalina P, Meissner I, Krauss WE. Spontaneous regression of a symptomatic intramedullary spinal cord lesion. Cureus. 2020. 12: e7271
11. Mitha AP, Turner JD, Spetzler RF. Surgical approaches to intramedullary cavernous malformations of the spinal cord. Neurosurgery. 2011. 68: 317-24
12. Narayan P, Barrow DL. Intramedullary spinal cavernous malformation following spinal irradiation. Case report and review of the literature. J Neurosurg. 2003. 98: 68-72
13. Ogilvy CS, Louis DN, Ojemann RG. Intramedullary cavernous angiomas of the spinal cord: Clinical presentation, pathological features, and surgical management. Neurosurgery. 1992. 31: 219-29
14. Panda A, Diehn FE, Kim DK, Bydon M, Goyal A, Benson JC. Spinal cord cavernous malformations: MRI commonly shows adjacent intramedullary hemorrhage. J Neuroimaging. 2020. p.
15. Ren J, Hong T, Zeng G, He C, Li X, Ma Y. Characteristics and long-term outcome of 20 children with intramedullary spinal cord cavernous malformations. Neurosurgery. 2020. 86: 817-24
16. Riche MC, Modenesi-Freitas J, Djindjian M, Merland JJ. Arteriovenous malformations (AVM) of the spinal cord in children. A review of 38 cases. Neuroradiology. 1982. 22: 171-80
17. Santoro A, Piccirilli M, Brunetto GM, Delfini R, Cantore G. Intramedullary cavernous angioma of the spinal cord in a pediatric patient, with multiple cavernomas, familial occurrence and partial spontaneous regression: Case report and review of the literature. Childs Nerv Syst. 2007. 23: 1319-26
18. Sun I, Pamir MN. Spinal cavernomas: Outcome of surgically treated 10 patients. Front Neurol. 2017. 8: 672
19. Weil AG, Bhatia S. Resection of a ventral intramedullary cervical spinal cord cavernous malformation through an anterior approach. Neurosurg Focus. 2014. 37: Video18