Single Layer Of Endothelial Cells Research Articles

Lymphatic Vessels in the Inner Ear of Patients With Meniere Disease: A Novel Pathological Finding.

Meniere disease, characterized by intermittent episodes of vertigo, fluctuating sensorineural hearing loss, tinnitus, and aural pressure, is a common cause of vertigo in humans. The pathogenesis of Meniere disease remains unknown. The current study aimed to describe a novel pathological change discovered in the inner ears of patients with Meniere disease who underwent labyrinthectomy. This retrospective case-control study was conducted with 21 patients with MD who underwent labyrinthectomy. A total of 15 patients diagnosed with acoustic neuroma or glomus jugular tumor were review over the same period of time as control. The clinical information of the patients and the pathological features of the membrane are described. The new pathological tissue was a morbid membrane structure sealing the round window, characterized by the formation of lymphatic capillaries. Histochemical and immunofluorescent staining was positive for D2-40, LYVE-1, podoplanin, and PROX1, which are the classical markers of the lymphatic vessels. Transmission electron microscopy revealed that the lymph capillaries lacked a typical basement membrane and that their ends were blind, composed of a single layer of endothelial cells with valval connection structures between adjacent capillary epithelial cells. This is the first report of lymphatic vessels in the human inner ear, and this pathological structure is a completely new discovery. The lymphatic vessels may develop due to inflammation or decompensation of pressure in the inner ear, suggesting that the inner ear can reactively form lymphatic vessels in some inflammation and fluid flow-dependent pathological conditions. The current findings help in improving our understanding of the pathogenesis of Meniere disease.

Fallopian Tube Hemangioma Discovered on Follow-up for Uterine Leiomyoma.

Hemangioma in female reproductive organs, particularly in the fallopian tube (FT), is a sporadic disease. In this report, we describe a case of hidden capillary hemangioma in FT in a 39-year-old woman who suffered from uterine leiomyoma. During the preoperative stage, pelvic sonography, computed tomography, and diagnostic laparoscopy revealed a subserous leiomyomatous nodule located along the posterior wall of the uterus. Despite this, intraoperatively, a benign vascular neoplasm was diagnosed. Histologically, it is characterized by multiple thin-walled vascular spaces lined with a single layer of endothelial cells, in which single mitoses were observed. The diagnosis was then confirmed immunohistochemically by CD31 and CD34 expression in the endothelial cells lining the inner surface of the spaces and the low mitotic activity of the tumor cells. It is virtually impossible to diagnose this asymptomatic neoplasm before and during surgery, which can result in an inadequate number of surgeries. Incorrect interpretation of a benign tumor at a young age can lead to unnecessary radical surgery with a resulting loss of fertility, and an unrevealed malignant process can threaten life.

Intravascular papillary endothelial hyperplasia in the lungs of a wild Korean raccoon dog.

A male Korean raccoon dog of unknown age was rescued and placed at the Daejeon Wildlife Rescue Center, Korea. Physical examination revealed severe emaciation and dehydration, as well as thick crusts and alopecia over most of the body. During medical care, the animal died and was submitted for postmortem examination. Firm, brown-red lesions of various sizes were observed on the surface of the lungs. In cross-sections of the lungs, pulmonary vessels were thickened and dilated, with white irregular papillary luminal projections. Histologically, pulmonary blood vessels were severely hyperplastic, characterized by thickened dilated walls and fibrous papillary projections covered with a single layer of endothelial cells (ECs). Hyperplastic fibrous connective tissue was confirmed by Masson trichrome staining. The ECs expressed CD31. We diagnosed the lesion as intravascular papillary endothelial hyperplasia, a unique non-neoplastic reactive process that has not been reported previously in pulmonary vessels of canids, equids, or felids, to our knowledge.

A large hemangioma of the breast influencing the management of breast cancer

Introduction: Hemangiomas are a rare welldemarcated proliferation of mature blood vessels lined by a single layer of endothelial cells occurring in up to 1.3% of mastectomy specimens. Accurate diagnosis often requires the incorporation of clinical, radiological, and histological features. Case Report: A 61-year-old female presented with a self-detected right breast lesion. Initial work-up revealed a right breast lesion at 12 o’clock with core biopsy confirming intermediate grade ductal carcinoma in situ. The management was complicated by the presence of a large right chest wall congenital cavernous hemangioma. Wide local excision of the breast lesion was performed with no sentinel lymph node biopsy done due to extensive vascular malformation. Further excision of margins was required to achieve surgical clearance. Conclusion: Despite its rarity, clinicians should be aware of the potential occurrence of a hemangioma on the breast and potential implications for the surgical management of breast cancer.

Unilateral vascular hamartomas of the vaginal tunic in a dog.

A 12-y-old, male Dachshund was presented for elective orchiectomy. The testes were of normal size. The left testis had numerous dark-red, blood clot-like foci within the vaginal tunic over the pampiniform plexus, epididymis, and testis. Histologically, the red foci were limited to the vaginal tunic and consisted of disorderly growing, variably sized, thin-walled blood vessels lined by a single layer of endothelial cells without mitoses and supported by a thin layer of pericytes. The blood vessels were distended by erythrocytes without thrombus formation. Endothelial cells had cytoplasmic immunolabeling for CD31; pericytes had strong cytoplasmic immunolabeling for α-smooth muscle actin. Our case of subclinical unilateral vascular hamartomas of the vaginal tunic in a dog has not been reported previously in domestic animals or humans, to our knowledge.

Validity of Customized Branched Tissue Engineered Vascular Graft in a Porcine Model

BackgroundPatient-specific, 3-dimensional printed, tissue engineered vascular grafts (3DTEVGs) are manufactured to optimize hemodynamic performance and to accommodate growth. We evaluate growth outcomes of 3DTEVGs compared with standard grafts for pulmonary artery reconstruction in porcine models. MethodsMagnetic resonance imaging (MRI) with 4-dimensional flow data was acquired in porcine models (n = 8). 3DTEVGs guided in design by computational flow dynamics were implanted (n = 4), with polytetrafluorethylene grafts used as controls (n = 4). Postoperative MRI and histologic features of explanted grafts were evaluated after 10 weeks. ResultsAll pigs survived, with evidence of patent grafts on postoperative MRI. Graft inner diameter changes were 0.47 ± 2.31 mm in 3DTEVGs and −4.61 ± 2.15 mm in controls (P = .018). Mean main pulmonary artery wall shear stress was significantly lower in 3DTEVGs (7.12 ± 4.21 Pa) than in controls (18.15 ± 8.37 Pa; P = .0396). Histologic evaluation of 3DTEVGs showed a single layer of endothelial cells, an organized smooth muscle layer, and collagen deposition with a remaining scaffold area of 21.37% ± 20.46%. ConclusionsOur patient-specific 3DTEVGs demonstrated optimal anatomic fit while maintaining ideal flow dynamics and promoting appropriate neovessel formation.

Thymic cavernous haemangioma in a dog

A 12-year-old male neutered Australian Shepherd Dog was presented to Charlotte Animal Referral & Emergency with a history of a thoracic mass. On physical examination, physiological parameters were within the normal ranges, and a complete haemogram and serum biochemistry profile were unremarkable except for mild thrombocytopenia. A computed tomography scan revealed a 21 × 15 × 12.7 cm thoracic mass encompassing the cranial mediastinum and extending to the right caudal thorax. The mass was surgically removed and histopathological evaluation revealed that it comprised remnants of the thymus and a neoplasm composed of large blood-filled vascular spaces lined by a single layer of endothelial cells with mild anisocytosis and anisokaryosis. The neoplastic cells had diffuse strong immunolabeling for endothelial cell marker CD31. Multifocally, there were large cystic degenerated areas of thymic tissue lined by plump cytokeratin AE1/AE3-positive epithelial cells. Based on these findings, a diagnosis of thymic cavernous haemangioma (CH) was made. Thymic CH is rare in animals, with the only reported case in a cross bred cow. To our knowledge, this is the first case of a thymic CH in a dog.

Cross-linking porcine peritoneum by oxidized konjac glucomannan: a novel method to improve the properties of cardiovascular substitute material

The use of natural polysaccharide crosslinkers for decellularized matrices is an effective approach to prepare cardiovascular substitute materials. In this research, NaIO4 was applied to oxidize konjac glucomannan to prepare the polysaccharide crosslinker oxidized konjac glucomannan (OKGM). The as-prepared crosslinker was then used to stabilize collagen-rich decellularized porcine peritoneum (DPP) to construct a cardiovascular substitute material (OKGM-fixed DPP). The results demonstrated that compared with GA-fixed DPP and GNP-fixed DPP, 3.75% OKGM [1:1.5 (KGM: NaIO4)]-fixed DPP demonstrated suitable mechanical properties, as well as good hemocompatibility, excellent anti-calcification capability, and anti-enzymolysis in vitro. Furthermore, 3.75% OKGM [1:1.5 (KGM: NaIO4)]-fixed DPP was suitable for vascular endothelial cell adhesion and rapid proliferation, and a single layer of endothelial cells was formed on the fifth day of culture. The in vivo experimental results also showed excellent histocompatibility. The current results demonstrted that OKGM was a novel polysaccharide cross-linking reagent for crosslinking natural tissues featured with rich collagen content, and 3.75% OKGM [1:1.5 (KGM: NaIO4)]-fixed DPP was a potential cardiovascular substitute material.Graphical

Cystic angiomatosis in children: clinical experience and review of literature

Cystic angiomatosis is a rare benign disease manifesting as multiple lytic and sclerotic bone lesions, described as the proliferation of vascular and lymphatic channels lined by a single layer of endothelial cells. However, the potential pathogenetic mechanism of the disease still remains unknown. Here, we reported a case of cystic angiomatosis with multifocal bone lesion evaluated by whole exome sequencing. In this presentation, we reported a case of an 11-year-old boy with pain in his chest. Computed tomography (CT) revealed the multiple lytic of the bone in the ribs, clavicle, vertebra thoracalis, skull, mandibula, shoulder blade, etc. The blood test showed ALP to be 393U/L and VEGF to be 287.26 pg/ml. The patient was performed with an open biopsy in the ribs and was diagnosed with cystic angiomatosis. Besides, the whole exome sequencing reported the single-nucleotide substitutions in the coding region of BRIP1, CHEK2, GRM4, and MUC16. Then, the upregulated genes involved CASC15, CENPF, ABCA13, ALK, BLM, and FGFR3. In this article, we report a rare case of cystic angiomatosis in a child with abnormal VEGF and ALP reported by peripheral blood examination. The whole exome sequencing could provide the reference for the potential molecular mechanism in the diagnosis and treatment of cystic angiomatosis.

S3575 Case of Iron Deficiency Anemia Secondary to a Pyogenic Granuloma

Introduction: Pyogenic granulomas (PG) are lesions in the skin or mucosa composed of vasodilative granulation tissue. Although commonly found in the oral cavity, they are rare throughout the rest of the gastrointestinal (GI) tract. Gastrointestinal PGs typically present as insidious microcytic anemia, which can be severe. We present a case of a patient with iron deficiency anemia secondary to pyogenic granulomas. Case Description/Methods: A 75-year-old Caucasian female referred to the hospital for evaluation for coronary artery bypass graft. Medical history was significant for coronary artery disease, microcytic anemia, tobacco use. On workup, she was found to have iron deficiency anemia with labs showing a hemoglobin of 8.5 g/dL and mean corpuscular volume of 78.3 fL. Her baseline hemoglobin was 12 g/dL last known 5 years before. Iron studies showed low iron at 24 ug/dL and low transferrin saturation of 7%. She denied any hematemesis, melena, or hematochezia. Digital rectal exam demonstrated soft, brown stool. Diet at that time was unchanged from baseline. No other sources of non-GI bleed were identified. Further endoscopic work-up was obtained. Colonoscopy and video pill capsule were unmarkable. Esophagogastroduodenoscopy revealed three large, gastric polypoid lesions that were consistent with visual findings of pyogenic granulomas (Figure). Biopsy revealed an entirely fibrin composition and inflammatory cells with granulation tissue and a strip of reactive foveolar epithelium. Patient was treated conservatively with iron supplementation. Discussion: Pyogenic granulomas are a benign polypoid form of capillary hemangiomas. Initially, it was thought that PGs were caused by infection, but they are most likely multifactorial, including mucosal irritation resulting in reactive processes. Endoscopically, these lesions are commonly erythematous, pedunculated polyps. They are typically described as having a smooth, but visibly ulcerated, surface that can be friable or oozing blood. Further, visual aspects of PG include the underlying vasculature causing dark red to fresh sanguine appearance. Histologically, PGs are lobular-patterned hemangiomas with capillary vessels. Similar to this case, granulation tissue and a single layer of endothelial cells may also be present. Conservative management includes supporting blood loss with iron supplementation. If unresponsive, endoscopic management includes resection with a snare polypectomy, endoscopic mucosal resection, band ligation, or heat application.Figure 1.: Semi-pedunculated polyps (arrows) with hyper vascular appearance. No obvious stigmata of bleeding.

Capillary communication: the role of capillaries in sensing the tissue environment, coordinating the microvascular, and controlling blood flow.

Historically, capillaries have been viewed as the microvascular site for flux of nutrients to cells and removal of waste products. Capillaries are the most numerous blood vessel segment within the tissue, whose vascular wall consists of only a single layer of endothelial cells and are situated within microns of each cell of the tissue, all of which optimizes capillaries for the exchange of nutrients between the blood compartment and the interstitial space of tissues. There is, however, a growing body of evidence to support that capillaries play an important role in sensing the tissue environment, coordinating microvascular network responses, and controlling blood flow. Much of our growing understanding of capillaries stems from work in skeletal muscle and more recent work in the brain, where capillaries can be stimulated by products released from cells of the tissue during increased activity and are able to communicate with upstream and downstream vascular segments, enabling capillaries to sense the activity levels of the tissue and send signals to the microvascular network to coordinate the blood flow response. This review will focus on the emerging role that capillaries play in communication between cells of the tissue and the vascular network required to direct blood flow to active cells in skeletal muscle and the brain. We will also highlight the emerging central role that disruptions in capillary communication may play in blood flow dysregulation, pathophysiology, and disease.

28423 Treatment of cutaneous lesions in patient with blue rubber bleb nevus with diode laser

Blue rubber bleb nevus (BRBN) is a rare congenital skin disorder characterized by numerous, diffuse venous malformations of the cutaneous. The treatment of cutaneous lesions is not usually required except for cosmetic purposes while previous methods to remove cutaneous lesions including ND:YAG laser, photocoagulation, sclerotherapy, surgery often could lead to scars. We report a case of BRBN who had a good response to diode laser without adverse effects. A 16-month-old girl presented to our hospital with multiple, discrete, dark blue to violaceous soft compressible nodules on the skin as well as palm and sole. Especially, a large violaceous perianal lesion was about 4 cm in diameter that was at high risk for hemorrhage due to an anal fissure making the children occasionally bleeding while a bowel movement. Most of the lesions presented at birth remained stable in size and number. Abdominal ultrasound has not been discovered any abnormalities. Histopathology image of the cutaneous lesion shows variably sized, irregular and thin-walled vessels lined by a single layer of endothelial cells in the dermis. Immunohistochemical stain demonstrates endothelial cells positive for CD31. The girl has been treated using 810-nm diode laser at the setting 30-35 J/cm2, 30 ms for the cutaneous lesions on her back when the diagnosis of BRBN was established. After four times of treatment, a significant reduction in color and size was achieved on the back cutaneous lesions without adverse effects. In conclusion, this is the case of BRBN showing a good response to diode laser.

Ovarian Cystic Lymphangioma: A Rare Case Report with Review of Literature

Lymphangiomas of the ovary are unusual benign tumours characterised by proliferation of lymphatic channels, composed of cystic spaces lined by a single layer of endothelial cells. We present a case of 61-year-old woman who presented with complaints of pain abdomen. Investigations revealed a large left ovarian multiseptate multiloculated cystic mass extending to pelvis, abdominal cavity and infraumbilical region likely neoplastic. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed suspecting malignancy. Histopathological examination revealed an ovarian lymphangioma.

Intramedullary spinal cord cavernous malformations in the pediatric population.

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.

Mural Cell-Specific Deletion of Cerebral Cavernous Malformation 3 in the Brain Induces Cerebral Cavernous Malformations.

Cerebral cavernous malformations (CCM), consisting of dilated capillary channels formed by a single layer of endothelial cells lacking surrounding mural cells. It is unclear why CCM lesions are primarily confined to brain vasculature, although the 3 CCM-associated genes (CCM1, CCM2, and CCM3) are ubiquitously expressed in all tissues. We aimed to determine the role of CCM gene in brain mural cell in CCM pathogenesis. Approach and Results: SM22α-Cre was used to drive a specific deletion of Ccm3 in mural cells, including pericytes and smooth muscle cells (Ccm3smKO). Ccm3smKO mice developed CCM lesions in the brain with onset at neonatal stages. One-third of Ccm3smKO mice survived upto 6 weeks of age, exhibiting seizures, and severe brain hemorrhage. The early CCM lesions in Ccm3smKO neonates were loosely wrapped by mural cells, and adult Ccm3smKO mice had clustered and enlarged capillary channels (caverns) formed by a single layer of endothelium lacking mural cell coverage. Importantly, CCM lesions throughout the entire brain in Ccm3smKO mice, which more accurately mimicked human disease than the current endothelial cell-specific CCM3 deletion models. Mechanistically, CCM3 loss in brain pericytes dramatically increased paxillin stability and focal adhesion formation, enhancing ITG-β1 (integrin β1) activity and extracellular matrix adhesion but reducing cell migration and endothelial cell-pericyte associations. Moreover, CCM3-wild type, but not a paxillin-binding defective mutant, rescued the phenotypes in CCM3-deficient pericytes. Our data demonstrate for the first time that deletion of a CCM gene in the brain mural cell induces CCM pathogenesis.

Hepatic Hemangioma Treatment Using Microwave Coagulation Therapy—a Systematic Review

Hepatic hemangiomas (HH) are the most common benign liver tumors, with a prevalence ranging from 5 to 7.35% of all benign tumors of the liver. Most lesions occur to the fifth and sixth decades of life more frequently in females (female/male ratio = 5:1). Hepatic hemangiomas are composed of blood-filled cavities lined by a single layer of endothelial cells, fed by the hepatic artery. Hepatic hemangiomas are composed of blood-filled cavities lined by a single layer of endothelial cells, fed by the hepatic artery. The exact etiology of hepatic hemangiomas still is unclear though the genetic predisposition and estrogen in stimulating and/or contraceptive use have been proposed. A majority of hepatic hemangiomas are often asymptomatic and discovered incidentally. It is a slow-growing, so medical/surgical intervention is often not needed. However, the so-called giant cavernous hemangiomas (GCH) (greater than 5 cm in diameter) will usually develop symptoms and complications, which pose a higher risk of rupture that a radical intervention needs to be considered. Nowadays, hepatic hemangiomas therapy included surgical resection, intervention embolization, radio frequency ablation, microwave ablation, and drug therapy. There have been existing heated debates on the treatment strategies of hepatic hemangiomas for a long time because of the researchers lacked the evidence-based medical evidence data to analyze those treatment methods respectively. The present study is a comprehensive review of various treatments specially use of microwave ablation in large and symptomatic hepatic hemangiomas.

PAPILLARY ENDOTHELIAL HYPERPLASIA—A CASE REPORT OF THIS RARE VASCULAR LESION

We will report a case of intravascular papillary endothelial hyperplasia, an unusual blood vessel disease occurring in the oral cavity. A female patient, 54 years of age, showed a bluish asymptomatic nodule in the lower lip, measuring 5 mm in diameter. The clinical diagnostic hypotheses were hemangioma or mucocele. An excisional biopsy was performed. The histopathologic examination demonstrated papillary tufts with a central hyaline core lined by a single layer of endothelial cells, which were positive for CD31 staining. The final diagnosis was papillary endothelial hyperplasia. This is a benign reactive vascular lesion with uncertain etiology. Scientific studies report this lesion as an organized thrombus. The treatment performed was surgical with a good prognosis. The 1-year follow-up period showed no signs of clinical recurrence.

Cervical Intramedullary Cavernoma in Children: Case report and literature review

Case presentation: A 10-year-old male patient was admitted due to posterior cervicalgia, vomiting and progressive generalized weakness. Physical examination showed difficulty in ambulation and tetraparesis. Magnetic resonance imaging (MRI) of the cervical medulla enhanced by the contrast agent, gadolinium, showed a poorly contrasted mass with a hemorrhagic center. We undertook a posterior cervicotomy (C1-C3). Post-operative evolution was satisfactory with complete regression of the deficits 90 days after surgery. Control MRI at intervals of 1 and 4 years confirmed absence of the tumor. Discussion: Cavernomas are vascular malformations, consisting of coarsely dilated vascular channels and coated by a single layer of endothelial cells, devoid of endothelium and myothelium. They represent only 5-12% of all vascular pathologies of the medulla, with only 10% affecting the pediatric population. There are only thirty cases of pediatric intramedullary spinal cavernomas in the literature, with predominance among males (2.1:1). Thoracic and cervical spinal cavernomas consist in 55% and 45% of the cases, respectively. In six cases (20%) cavernomatous lesions were associated with synchronic intracranial cavernoma. Among the reported cases, only one had poor evolution after surgery, whereas six patients persisted with prior symptoms. Conclusion: Spinal intramedullary cavernomas are rare entities, especially in the pediatric population, and are treated with surgery which improves prior neurologic deficits, besides preventing rebleeding

Pathogenesis of atherosclerosis in the tunica intima, media, and adventitia of coronary arteries: An updated review.

Atherosclerosis is a chronic inflammatory disease of arteries and it affects the structure and function of all three layers of the coronary artery wall. Current theories suggest that the dysfunction of endothelial cells is one of the initial steps in the development of atherosclerosis. The view that the tunica intima normally consists of a single layer of endothelial cells attached to the subendothelial layer and internal elastic membrane has been questioned in recent years. The structure of intima changes with age and it becomes multilayered due to migration of smooth muscle cells from the media to intima. At this stage, the migration and proliferation of smooth muscle cells do not cause pathological changes in the intima. The multilayering of intima is classically considered to be an important stage in the development of atherosclerosis, but in fact atherosclerotic plaques develop only focally due to the interplay of various processes that involve the resident and invading inflammatory cells. The tunica media consists of multiple layers of smooth muscle cells that produce the extracellular matrix, and this layer normally does not contain microvessels. During the development of atherosclerosis, the microvessels from the tunica adventitia or from the lumen may penetrate thickened media to provide nutrition and oxygenation. According to some theories, the endothelial dysfunction of these nutritive vessels may significantly contribute to the atherosclerosis of coronary arteries. The adventitia contains fibroblasts, progenitor cells, immune cells, microvessels, and adrenergic nerves. The degree of inflammatory cell infiltration into the adventitia, which can lead to the formation of tertiary lymphoid organs, correlates with the severity of atherosclerotic plaques. Coronary arteries are surrounded by perivascular adipose tissue that also participates in the atherosclerotic process.

Cervical Intramedullary Cavernoma in Children: case report and literature review

Case presentation: A 10-year-old male patient was admitted due to posterior cervicalgia, vomiting and progressive generalizedweakness. Physical examination showed difficulty in ambulation and tetraparesis. Gadolinium-loaded magnetic resonance imaging(MRI) of the cervical medulla, showed a poorly contrasted mass with a hemorrhagic center. We undertook a posterior cervicotomy(C1-C3). Post-operative evolution was satisfactory with complete regression of the deficits 90 days after the surgery. Control MRIat intervals of 1 and 4 years confirmed absence of the tumor. Discussion: Cavernomas are vascular malformations, consisting ofcoarsely dilated vascular channels and coated by a single layer of endothelial cells, devoid of endothelium and myothelium. Theyrepresent only 5-12% of all vascular pathologies of the medulla, with only 10% affecting the pediatric population. There are onlythirty cases of pediatric intramedullary spinal cavernomas in the literature, with predominance among males (2.1:1). Thoracic andcervical spinal cavernomas consist in 55% and 45% of the cases, respectively. In six cases (20%) cavernomatous lesions were associatedwith synchronic intracranial cavernoma. Among the reported cases, only one had poor evolution after surgery, whereas six patientspersisted with prior symptoms. Conclusion: Spinal intramedullary cavernomas are rare entities, especially in the pediatric population,and are treated with surgery which improves prior neurologic deficits, besides preventing rebleeding.