Capillary Hemangioma of the Spinal Cord: Case Report and Systematic Review of Literature.

BACKGROUND: Capillary hemangiomas are rare vascular lesions that rarely affect the central nervous system. When they present within the spinal canal, they are typically confined intradurally, with intramedullary extension rare. We present a rare case of spinal intramedullary capillary hemangioma, with a systematic review of the literature. METHODS: Medical records and imaging data were retrospectively reviewed using the health record software EPIC (Verona, Wisconsin, USA) and the radiology management software system RIS/PACS (Radiology Information System/Picture Archiving and Communication System; QREADS). The report was written in accordance with the CARE (case reports) guidelines. We also performed a systematic review of the literature on all cases of intramedullary spinal capillary hemangiomas in accordance with PRISMA (preferred reporting items for systematic reviews and meta-analyses) guidelines. RESULTS: We report a case of a 54-year-old man who presented with progressive paraplegia and sensory deficits in the lower extremities. Spinal magnetic resonance imaging showed an intramedullary enhancing lesion centered at T11 with associated spinal cord compression. He underwent thoracic laminectomy and gross total resection of the lesion without complications and subsequent improvement on his neurological examination. Histological examination showed findings consistent with a capillary hemangioma. The literature review also documented 21 studies with a combined total of 38 cases of intramedullary spinal capillary hemangioma. CONCLUSIONS: Purely intramedullary capillary hemangiomas are unusual spinal lesions with only a few cases reported in the literature. These should be considered in the differential diagnosis of intramedullary tumors. Surgical management remains the first line of treatment for symptomatic patients.

Intramedullary spinal capillary hemangioma with secondary neurulation defect in children.

Intramedullary spinal capillary hemangioma is a rare occurrence in pediatric patients, and only limited cases have been reported. This study presents the first two cases of spinal capillary hemangioma co-present with retained medullary cord and one case of spinal capillary hemangioma with lumbosacral lipomatous malformation. Previous literature on ten patients with this pathology was reviewed. We speculated pathogenesis, imaging features, and histopathologic findings of the disease.

Documented growth of an intracranial capillary hemangioma: A case report.

Intracranial capillary hemangiomas in adults are rare, and diagnosis can be challenging. Hemangiomas, in general (and particularly in the skin), are more often noted in the pediatric population. Due to the lack of imaging undertaken in the presymptomatic phase, the literature provides few clues on the rate of growth of these unusual tumors. Therefore, we report a case of a 64-year-old man with a medical history of Lyme disease who presented with exhaustion and confusion. Imaging demonstrated an intra-axial lesion with vascularity in the posterior right temporal lobe, raising the possibility of a glioma. Imaging two years prior revealed a very small lesion in the same location. The patient underwent a craniectomy, total resection of the lesion was completed, and his symptoms of confusion resolved. Biopsy revealed a capillary hemangioma composed of small vascular channels lined by endothelial cells and pericytes without smooth muscle. Features of glioma, vascular neoplasms or neuroborreliosis (cerebral Lyme disease) were not identified. Our case documents the growth over two years of a rare intracranial capillary hemangioma in an older adult male.

Spontaneous hemorrhage of spinal epidural capillary hemangioma resulting in hyperacute neurologic deficit: A case report.

INTRODUCTION: Spinal epidural capillary hemangioma is a very rare variety of tumors, usually with a predilection for the thoracic spine. CASE PRESENTATION: A 16-year-old female complained of hyperacute neurologic deficit progressed within hour, which presented by acute paraplegia, and loss of all sensations from her lower limbs up to her breasts. Neurologic exam revealed paralysis of lower limbs (0/5 on both legs) with a flaccid tone, absence of reflexes, weakness of the trunk with sensory level T4, bilateral flexion of plantar reflexes, and loss of sphincters' controls. Emergent magnetic resonance imaging showed a dumbbell-shaped epidural mass in the posterior aspect of the spinal canal at the T1-T2 level, measuring approximately 1.1 × 4.5 × 1.5 cm in size. The lesion was isointense on T1-weighted, hyperintense on T2-weighted, and a little enhancement after gadolinium administration. The surgery was obtained nearly 16 hours after paralysis, which eradicated the lesion with good hemostasis. Histological examination showed a well-organized vascular tissue that haphazardly arranged and confirmed the diagnosis of capillary hemangioma. Neurological improvement was quickly observed within days after surgery and further complete recovery was achieved 2 months after discharge. CONCLUSION: We report an extremely rare case of spinal epidural capillary hemangioma, where acute spontaneous hemorrhage in the lesion resulted in the hyperacute neurologic deficit within an hour. Since these are benign lesions, the immediate surgical intervention results in a very favorable prognosis and is considered the treatment of choice. Also, this case highlighted and rose the question of a better neurologic improvement in younger age patients with spinal cord injury.

Capillary Haemangioma of Gingiva - A Rare Lesion.

Capillary Haemangioma is a benign vascular tumour characterized by proliferation of blood vessels with a very striking similarity to pyogenic granuloma with a predilection to occur on the gingiva. Though the head and neck are the most common region of occurrence, but is considered to be rare occurring on the gingiva, intra-orally. The lesion clinically mimicked pyogenic granuloma but was histopathologically diagnosed as capillary haemangioma. It was surgically excised and followed up for 6 months without any recurrence.

[Deforming endonasal mass during pregnancy]. / Deformierende endonasale Raumforderung während der Schwangerschaft.

This article presents the case of a 33-year-old woman who consulted the authors' ENT clinic in the 39th week of pregnancy with recurrent epistaxis. A livid endonasal mass was found on the left side, subtotally displacing the nose and leading to deformation of the external nose. External biopsy provided no indications of malignancy. Postpartum CT of the paranasal sinuses revealed a mass destroying the cartilaginous nasal septum. Endoscopic resection of the finding was performed with preservation of the clinically sound nasal septal cartilage. Histopathological examination revealed a capillary hemangioma, which was classified as granuloma gravidarum due to its occurrence during pregnancy.

Distribution of problematic localized facial infantile haemangiomas and their response to propranolol: a retrospective cohort study.

BACKGROUND: The distribution and response to propranolol of problematic facial infantile haemangiomas (IHs) has rarely been described in the literature. AIM: To map problematic facial IHs and observe their response to propranolol. METHODS: Eligible patients were categorized according to focal location and cohorts corresponding to these (buccal, medial, zygomatic, lateral and multiregional) were created. The primary efficacy variable was regression score ranging from 1 to 4, calculated using results of colour Doppler ultrasonography. RESULTS: In total, 104 patients met the inclusion criteria. There were 32 (30·8%) IHs located in the buccal area, 12 (11·5%) in the medial area, 49 (47·1%) in the lateral area and 1 (1·0%) in the zygomatic area, with 10 (9·6%) IH cases having multiregional lesions. We found that the distribution pattern of most IHs matched the surface projection of the trunk of the external carotid and the facial arteries. Further analysis showed that the median regression score in the buccal and medial groups were significantly lower than those in the lateral and multiregional groups. CONCLUSION: Treatment of buccal and medial haemangiomas tends to be more challenging and their distribution pattern mainly reflects the direction of the facial vessels.

Clinical Relevance of Cherry Angiomas. / Relevancia clínica de los angiomas en cereza.

Cherry angiomas are the most common vascular tumors of the skin. They are particularly prevalent in the general population and become more common with age. Although an association with cancer was suggested at the end of the 19th century, when these tumors were first described, it could not be demonstrated. For many decades, therefore, cherry angiomas were considered to have no clinical relevance other than their association with age. A number of studies in recent years, however, have shown a link between cherry angiomas and exposure to various toxic substances and medications, benign and malignant diseases, and immunosuppression, rekindling interest in these lesions and providing clues for a better understanding of their etiology, pathophysiology, and clinical relevance.

Spinal epidural capillary hemangioma: case report and review of the literature.

BACKGROUND: Almost all of the epidural hemangiomas reported are cavernous hemangiomas. Purely extradural spinal capillary hemangiomas are very rare. Capillary hemangiomas are hamartomatous malformations that result from proliferations of vascular endothelial cells. Only ten cases have been reported in the English literature, treated with surgical excision. CASE DESCRIPTION: A case of a dorsal extradural spinal capillary hemangioma is described. A total surgical removal has been performed after spinal angiography and embolization. Complete surgical removal should always be the goal in these lesions. Embolization did not show to reduce bleeding during the surgical procedure in this case.

Immunohistochemistry for FOSB and FOS is a Useful Ancillary Tool in the Diagnosis of Epithelioid Hemangioma but There are Pitfalls in Interpretation Including Expression in Other Vascular Lesions.

Introduction. Epithelioid hemangioma is a benign vascular neoplasm associated with FOS and/or FOSB protein overexpression detected by immunohistochemistry (IHC). Methods. The aim of our study was to determine the co-expression or independent IHC expression of FOS and FOSB in a cohort of epithelioid hemangiomas. We also included two cohorts of other vascular lesions: papillary endothelial hyperplasia and lobular capillary hemangioma / pyogenic granuloma. Results. We identified 50 cases of epithelioid hemangioma, 84% of which were cutaneous and the remaining involved other anatomic locations. Over two thirds of all cases expressed FOSB (68%; 34/50) while FOS immunoreactivity was identified in 46% of all cases. Co-expression of FOSB and FOS occurred in 37% of cases while 76% of all cases stained for at least one of the antibodies. Fifty-eight percent (n = 14/24) and 33% (8/24) of all cases of papillary endothelial hyperplasia expressed FOS and FOSB, respectively. Thirty-two per cent of lobular capillary hemangiomas (n = 8/25) were positive for either FOS or FOSB. Conclusion. In summary, we present the largest cohort of epithelioid hemangiomas assessed with both FOS and FOSB and demonstrated that the use of both antibodies increases the detection rate of these proliferations by 10%. Nonetheless, the use of thresholds may not be appropriate, as only a subset of lesional endothelial cells label with FOS/FOSB. Over half of all cases of papillary endothelial hyperplasia and a third of lobular capillary hemangiomas also displayed immunoreactivity with FOS and/or FOSB.

Intradural extramedullary capillary hemangioma with intramedullary component: A case series.

RATIONALE: Capillary hemangioma in the spinal cord is an exceedingly rare entity that is liable to be misdiagnosed. To summarize the clinical presentation, radiological characteristics, and pathological features of capillary hemangioma in the spinal cord and to share our experience for the diagnosis and treatment of intradural extramedullary capillary hemangioma. PATIENT CONCERNS: Three patients underwent surgical treatment at our hospital between January 2020 and August 2020. All patients were male[median age at presentation: 57 years (range: 56-60)]. Two patients were experiencing pain and numbness in the lower back, and 1 patient was experiencing numbness and weakness in the left lower limb. The duration of symptoms ranged from 1 to 5 months. DIAGNOSIS: All patients were diagnosed with spinal cord capillary hemangioma after treatment. All lesions were in an intradural extramedullary location and involved spinal cord components. Two patients had lesions in thoracic segments (T8, Th9-10), and 1 patient had a lesion in lumbar segment (L1). INTERVENTIONS: All patients underwent microscopic laminectomy and complete resection of the extramedullary and intramedullary components of the spinal cord capillary hemangiomas. OUTCOMES: Postoperatively, all patients experienced transient numbness and pain in the lower limbs, which gradually decreased over time. None of the patients experienced bleeding, severe numbness or pain, or recurrence of symptoms at 3-month follow-up. CONCLUSION: Intradural extramedullary capillary hemangioma has unique morphological characteristics. Gross-total resection of the extramedullary and intramedullary components of spinal cord capillary hemangioma is recommended for patients with symptoms of spinal cord compression. Careful preoperative imaging and intraoperative decision-making may help avoid residual lesions or reoperation.

Resection of an Intradural Extramedullary Capillary Hemangioma in the Lumbar Spine.

BACKGROUND AND IMPORTANCE: Capillary hemangiomas are space-occupying lesions that rarely affect the central nervous system. When they present within the spinal canal, they can cause insidious symptoms and threaten neurological function. In this study, we present a case of an intradural extramedullary capillary hemangioma of the lumbar spine, discuss our management strategy, and review the current literature. For the first time for this diagnosis, we also provide an operative video. CLINICAL PRESENTATION: The patient is a previously healthy 40-year-old man who presented with complaints of progressive low back and leg pain, numbness, and intermittent subjective urinary incontinence. MRI revealed a discrete, homogenously enhancing intradural extramedullary lesion at L4. This lesion was resected by performing an L4 laminoplasty, which entails en bloc removal of the L4 lamina and then securing it back into place once the intradural resection and dural closure are completed. Histological analysis revealed a diagnosis of capillary hemangioma. The patient had full resolution of his symptoms postoperatively. DISCUSSION: Definitive management of spinal capillary hemangiomas involves gross total resection and can be accomplished with laminoplasty. Because these benign tumors can be adherent to adjacent structures, intraoperative neuromonitoring is helpful adjunct to preserve neurological function for a good outcome. CONCLUSION: Capillary hemangiomas rarely affect the spine but should be considered on the list of differential diagnoses of intradural lesions.

[Hemangiomas of the optic nerve head]. / Gemangiomy diska zritel'nogo nerva.

The article presents five cases of various forms of hemangiomas of the optic nerve head (ONH) and juxtapapillary retina that were monitored and analyzed using ocular visualization methods. The obtained data allowed a conclusion that capillary hemangioma of the ONH features clearly-seen boundaries of the lesion both on ophthalmoscopy and optical coherence tomography (OCT) images; on the other hand, juxtapapillary granuloma can be characterized by blurred outlines on ophthalmoscopy and a gradual transition of the altered retina on OCT images with secondary changes seen in the macular area. When comorbid, capillary hemangioma of the ONH and retina feature a combination of these visualization signs.

Identification of a Novel EIF2AK Variant and Genetics-Assisted Approach to Diagnosis of Pulmonary Capillary Hemangiomatosis.

Pulmonary capillary hemangiomatosis (PCH) is an uncommon type of pulmonary vascular disease characterized by capillary proliferation and very poor prognosis owing to misdiagnosis and lack of effective therapeutic options. Mutations in the eukaryotic translation initiation factor 2α kinase 4 (EIF2AK4) gene have been reported in pulmonary veno-occlusive disease and PCH. In this report, we present a patient whose diagnosis of PCH was delayed by 2 ½ years despite prior surgical lung biopsy and clinical and laboratory findings suggestive of pulmonary hypertension. Genotyping revealed a novel likely pathogenic variant in the EIF2AK4 gene. Review of surgical lung biopsy performed 2 ½ years prior confirmed PCH histology along with constrictive bronchiolitis.

Endoscopic Transcanal and Transmastoid Laser-Assisted Resection of Middle Ear Capillary Hemangioma.

OBJECTIVE: To present a rare case of a middle ear capillary hemangioma in an adult. PATIENT: A 31-year-old woman with a 6-month history of left ear fullness, pressure, tinnitus, and progressive hearing loss. INTERVENTION: Endoscopic laser-assisted resection. MAIN OUTCOME MEASURES: Clinical, radiographic, and histopathological findings of a capillary hemangioma. RESULTS: Otoscopy revealed an erythematous and slightly pulsating multilobulated middle ear retrotympanic mass. Her audiogram demonstrated a left-sided mixed hearing loss with air-conduction thresholds in the severe-to-profound range. Computed tomography (CT) imaging was significant for total opacification of the left middle ear and mastoid air cells. She underwent a combined endoscopic transcanal and transmastoid excision of the mass with ossicular chain reconstruction. A KTP laser was used to ablate and shrink down the periphery of the lesion. Pathology of the specimen was consistent with a capillary hemangioma. The patient's pulsatile tinnitus and spontaneous vertigo resolved postoperatively. CONCLUSIONS: Capillary hemangiomas are an uncommon cause of vascular middle ear lesions in adults and typically present with symptoms of aural fullness, pulsatile tinnitus, conductive hearing loss, otalgia, and vertigo. Surgery resection provides definitive treatment and the use of laser ablation techniques can allow for hemostasis and excellent visualization.

Anatomic Distribution of Cherry Angiomas in the General Population.

BACKGROUND: Cherry angiomas are common benign vascular skin lesions of unknown aetiology, found largely on the trunk. However, their exact anatomic distribution besides their truncal predisposition, and how they manifest in the general population, has not been characterised. METHODS: Three-dimensional (3D) total body imaging was obtained from 163 adult participants of a general population cohort study in Brisbane, Australia. Demographic, phenotypic, and sun behaviour characteristics were collected using a standard questionnaire along with history of melanoma and keratinocyte cancers. Cherry angiomas were identified using an automated classification algorithm with a sensitivity of 87% and a specificity of 99%, developed specifically for this study population. RESULTS: The 3D total body images of 163 participants were analysed. Participants had a median age of 57 years and 61% were male. On average, males had more angiomas than females (median of 16 vs. 12) and the number and size of cherry angiomas increased with age. In addition to male sex and age, an increase in angiomas was associated with Caucasian ancestry other than British/Irish only, fair skin colour opposed to medium/olive, having green/hazel eyes compared to blue/grey, and personal history of melanoma. The most common site for cherry angiomas was the front trunk, followed by the back. Interestingly, although males had more angiomas overall, females had more angiomas on the legs. CONCLUSION: Describing the distribution of cherry angiomas by body site is an important step towards further understanding of the aetiology of angiomas. While personal history of melanoma is associated with an increased number of cherry angiomas, whether this association is prognostic, co-occurs with development of melanoma, or is merely fortuitous requires further investigation.

Infantile hemangioma. Part 2: Management.

The majority of infantile hemangiomas (IH) can be managed conservatively, but for those requiring active treatment, management has been revolutionized in the last decade by the discovery of propranolol. Patients that may require active intervention should receive specialist review, ideally before 5 weeks of age to mitigate the risk of sequelae. Propranolol can commence for most infants in the outpatient setting and the most frequently employed dosing regimen is 1 mg/kg twice daily. In the future, ß-blockers with a more-selective mechanism of action, such as atenolol, show some promise. In recalcitrant lesions, systemic corticosteroids or sirolimus may be considered. For small, superficial IHs, topical timolol maleate or pulsed dye laser may be considered. Where the IH involutes with cutaneous sequelae, a range of interventions have been reported, including surgery, laser, and embolization. IHs have a well-described clinical trajectory and are readily diagnosed and managed via telemedicine. Algorithms have been constructed to stratify those patients who can be managed remotely from those who warrant in-person review during the COVID-19 pandemic.

Efficacy and Safety of Topical Timolol for the Treatment of Infantile Hemangioma in the Early Proliferative Stage: A Randomized Clinical Trial.

Importance: Treatment of infantile hemangioma (IH) with topical timolol in the first 2 months of life (early proliferative phase) may prevent further growth and the need for treatment with oral propranolol. To our knowledge, no studies have determined whether beginning early treatment with timolol for IH is better than in other proliferative stages. Objective: To evaluate the efficacy and safety of timolol maleate solution, 0.5%, for the early treatment of IH in infants younger than 60 days. Design, Setting, and Participants: This multicenter, randomized, double-blind, placebo-controlled, phase 2a pilot clinical trial included patients aged 10 to 60 days with focal or segmental hemangiomas (superficial, deep, mixed, or minimal/arrested growth). Patients were randomly assigned to treatment with topical timolol maleate solution, 0.5%, or placebo twice daily for 24 weeks. Changes in lesion size (volume, thickness) and color were evaluated from photographs taken at 2, 4, 8, 12, 24, and 36 weeks. Vital signs and adverse effects were recorded at each visit. The study was carried out from November 2015 to January 2017, and data analyses were completed in September 2019. Main Outcomes and Measures: The primary outcome of complete or nearly complete IH resolution and the secondary outcomes of changes in lesion thickness, volume, and color were evaluated by a blinded investigator. Results: Of the 69 patients recruited, the mean (SD) age was 48.4 (10.6) days; 55 (80%) were female; and 51 (74%), 11 (16%), 6 (9%), and 1 (1%) had superficial, mixed, abortive, or deep IHs, respectively. The IHs were localized, segmental, or indeterminate in 60 (87%), 7 (10%), and 2 (3%) patients, respectively. The IHs were located on the head and/or neck (n = 23 [33%]) or other body sites (n = 46 [67%]). The study was completed by 26 of 33 (79%) patients receiving timolol and 31 of 36 (86%) receiving placebo. There were no significant differences between timolol and placebo for complete or nearly complete IH resolution at 24 weeks (n = 11 [42%] vs n = 11 [36%]; P = .37). The odds ratio of complete or almost complete response vs no response at week 24 was 1.33 (95% CI, 0.45-3.89). There were no between-group differences in IH size (volume, thickness). An improvement in color was observed at week 4 in the timolol group, and timolol was well tolerated with no systemic adverse effects. Conclusions and Relevance: In this randomized clinical trial, results demonstrated that topical timolol is well tolerated for the treatment of early proliferative IH but provides limited benefit in lesion resolution when given during the early proliferative stage. Trial Registration: EudraCT Identifier: 2013-005199-17.