Intramedullary spinal cord cavernous malformations in the pediatric population

Abstract

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.

Spinal Cord Cavernous Malformation: A Case Report

“…Adjacent intramedullary bleeding is also common and is typically eccentric and bidirectional. 7 We should note that up to 34% of patients have associated venous malformations, and calcification occurs less frequently than in cerebral cavernous malformations. 8 The differential diagnosis might emerge with a thrombosed arteriovenous malformation (AVM), telangiectasia, or hemangioblastoma.…”

“…Gadolinium injection afford the distinction between intramedullary hemorrhagic tumors and cavernomas. 7 The therapy is mostly surgical, and the indication arises in the presence of a patient with a neurological disability and the ideal time for surgical intervention in these patients remains controversial while asymptomatic cases require medical treatment with clinical and radiological monitoring. Usually, it's postponed in cases of abrupt paraplegia to prevent spinal cord aggression by the surgical act, as was the situation with our patient.…”

“…Gadolinium injection afford the distinction between intramedullary hemorrhagic tumors and cavernomas. 7 …”

Intramedullary Spinal Cystic Lesions Mimicking Cavernoma with Spontaneous Myelum Hemorrhage in Children: A Case Report

“…The main cornerstone of the diagnostic modality is still MRI, which is able to reveal the detailed condition and features of the mass and surrounding tissue. Adjuvant studies such as angiography are conditional [5]. However, the use of advanced MRI techniques such as MRA/MRV can greatly assist the characterization of intramedullary lesions, especially for the suspected vascular lesion as in this patient [14].…”

“…Intramedullary spinal cavernous malformations make up only 1% of all intramedullary spinal lesions in the pediatric population [5]. Children most typically present with acute neurological deterioration characterized by the acute onset of severe motor deficits due to either an acute macro hemorrhage forming a spaceoccupying lesion, possibly accompanied by edema of the spinal cord, or a worsening of preexisting symptoms as the result of recurrent hemorrhage, however, repetitive intralesional microhemorrhages can lead to a more slowly progressive decline in neurological function [6], [7].…”