Orbital Cavernous Hemangioma Research Articles

Biological distinctions between juvenile nasopharyngeal angiofibroma and vascular malformation: An immunohistochemical study

The exact nature of juvenile nasopharyngeal angiofibroma (JNA) is still in dispute. In recent years, the main controversy of its nature has focused on hemangioma and vascular malformation. In this study, the immunolocalization of vascular endothelial growth factor (VEGF), VEGF receptor-1/fms-like tyrosine kinase-1 (VEGFR-1/Flt-1), VEGF receptor-2/fetal liver kinase-1 (VEGFR-2/Flk-1), proliferating cell nuclear antigen (PCNA), and CD34 was investigated in 28 cases of JNA and 20 cases of orbital cavernous hemangiomas (OCH). The immunostaining levels of VEGF, Flt-1, and Flk-1 were higher and more frequent in vascular endothelial cells of JNA than those of OCH ( p < 0.05). The average microvessel density (MVD) marked by CD34 in JNA was (49.3 ± 9.1)/HPF (high power field), which was higher than OCH (29.1 ± 6.7)/HPF ( p < 0.05). Immunoreactivity of PCNA was localized in both endothelial and stromal cell components of JNA, but was predominantly seen in the stromal cells. However, no PCNA immunoreactivity was identified in any of the stromal and endothelial cells in cases of OCH. The immunostaining levels of CD34, VEGF, Flt-1, Flk-1, and PCNA in JNA were higher than those in OCH. These data support the view that JNA has biological characteristics of an angiogenic histogenetic tumor. In the future, anti-angiogenic therapy may represent a novel treatment strategy for JNA.

Evaluation of MR imaging findings differentiating cavernous haemangiomas from schwannomas in the orbit

ObjectiveIt is important to distinguish between orbital cavernous haemangioma and schwannoma because the treatments of choice for the two tumours are different. The aim was to evaluate MR imaging findings distinguishing the two tumours.MethodsMagnetic resonance imaging including T1- and T2-weighted imaging and contrast-enhanced MR imaging was performed in 43 patients with cavernous haemangiomas and 16 patients with schwannomas confirmed by pathology. Location, configuration, margins, signal intensity, homogeneity and enhancement pattern of the tumour were retrospectively evaluated.ResultsThere was a significant difference between cavernous haemangiomas and schwannomas regarding the location, configuration and margins of the mass, signal intensity and homogeneity on T1- and T2-weighted imaging, the spread pattern of contrast enhancement, the enhancement pattern and the type of time–intensity curve (P < 0.05). Markedly homogeneous hyperintensity signal on T2-weighted imaging and the spread pattern of the contrast enhancement favoured cavernous haemangioma rather than schwannoma (P < 0.01).ConclusionCavernous haemangiomas and schwannomas have different MR imaging features that could be helpful in the differentiation between the tumours. The spread pattern of the contrast enhancement on dynamic contrast-enhanced MR imaging is the most reliable finding distinguishing cavernous haemangiomas from schwannomas.

Orbital Cavernous Hemangiomas: Ultrasound and Magnetic Resonance Imaging Evaluation

Cavernous hemangioma is the most common intraorbital lesion in adults. The aim of our study was to evaluate the magnetic resonance imaging (MRI) and ultrasound (US) characteristics of cavernous hemangioma and their role in the differential diagnosis of orbital tumors. Eight patients with orbital cavernous hemangiomas, five women and three men with a mean age of 48 years were examined in a period of six years. All patients underwent MRI examination and four patients were also evaluated by US. In all cases MRI depicted a well-defined intraconal tumor. The lesions were homogeneous, isointense to muscle on T1-weighted sequence and hyperintense to muscle on T2-weighted sequence in six patients. In one patient the mass was isointense on T1WI with heterogeneous signal intensity on T2WI and in one patient the lesion had heterogeneous signal intensity on both T1- and T2-weighted sequences. After intravenous contrast medium administration, the tumors showed initial inhomogeneous enhancement with progressive accumulation of contrast material on delayed images in seven patients and initial homogeneous enhancement in one patient. On ultrasonography, the orbital masses appeared slightly hyperechoic, heterogeneous with small areas of slow blood flow. The analysis of imaging characteristics of a well-defined intraconal lesion in an adult patient with painless progressive proptosis can be highly suggestive of the diagnosis of cavernous hemangioma.

The Supraorbital Approach to a Large Retrobulbar Cavernous Hemangioma of the Orbit-One Case Report

Purpose: To report a complete excision of a large superior orbital retrobulbar cavernous hemangioma using supraorbital craniotomy through the suprabrow approach combined with superior orbital wall removal. Case summary: A 31-year-old woman presented to our clinic with left exophthalmos beginning one month earlier. Visual acuities and intraocular pressures of both eyes were normal and there was no diplopia and no limitation in ocular movement. On exophthalmometry, the right eye was 15 mm and the left eye was 18 mm. CT and MRI demonstrated a superior orbital retrobulbar mass of 2.02.62.0 cm in size, and the mass displaced the superior rectus and levator palpebrae muscles. After six months, a neurosurgeon attempted a classic supraorbital craniotomy but failed. Fourteen months later, the authors completely excised the tumor through superior orbital wall removal. The tumor was confirmed as a cavernous hemangioma after histopathologic study. Conclusions: The authors succeeded in complete excision of a superior orbital retrobulbar cavernous hemangioma using supraorbital craniotomy through a suprabrow approach combined with superior orbital wall removal.

Surgical Outcomes of Transconjunctival Anterior Orbitotomy for Intraconal Orbital Cavernous Hemangioma

PurposeTo describe surgical outcomes for transconjunctival anterior orbitotomy for intraconal cavernous hemangiomas.MethodsThe medical records of 9 consecutive patients with intraconal cavernous hemangiomas who underwent surgical removal by transconjunctival anterior orbitotomy were retrospectively reviewed. The conjunctiva was incised and retracted with a traction suture. For large tumors, a rectus muscle was temporarily disinserted. Tenon's capsule was separated and the tumor was removed with a cryoprobe or clamp. Surgical outcomes, positions of the tumors, methods of approach, and intra- and post-operative complications were evaluated.ResultsThe mean follow-up period was 33 ± 6.8 months. No bony orbitotomy was used in this technique and the cosmetic results were very satisfactory. All tumors were removed intact. In 4 patients, tumors were extirpated with the aid of a cryoprobe. No patients had residual proptosis or limitation of ocular movement. No signs of recurrence were noted in any cases at 33 months follow-up. No serious or permanent complications were observed during or after the operation.ConclusionsTransconjunctival anterior orbitotomy is an important surgical procedure in the treatment of intraconal cavernous hemangiomas. It can produce an excellent result, even if the posterior border of the tumor abuts the orbital apex.

Multiple cavernous hemangiomas of the orbit: Separate occurrence within a 24-year period

Cavernous hemangioma is a frequent and the most common, primary, benign tumor of the orbit in adults. It is typically single and unilateral, considered not to recur after having been completely excised. Multiple orbital cavernous hemangiomas without signs of hemangiomatosis are rare. Multiple cavernous hemangiomas may recur after a complete excision and may exist with concurrent systemic tumors. Tumor recurrence is supposed to develop from vasculature that is present already in response to a proliferate stimulus. A 39-year-old female with painless proptosis of the right orbit was found to have four orbital tumors. The first orbitotomy was performed in 1984 by excising four cavernous hemangiomas. Six years later, another, the fifth one cavernous hemangioma was totally excised from the same orbit. Nine years after the first operation, reorbitotomy was performed because of positive radiological and clinical signs of de novo tumor in the orbit. The operation did not confirm the tumorous tissue. The fourth orbitotomy was performed 24 years after the first operation and two cavernous hemangiomas were totally excised. This case show the possibility of cavernous hemangioma recurrence after a previously totally excised tumor, separated more than two decades. A very long follow-up of the patients operated for these benign tumor lesions is recommended.

Orbital cavernous hemangioma in an infant with intracranial lesions: a case report.

Cavernous hemangiomas of the orbit are benign vascular malformations, commonly encountered in adults. Although they are infrequent in pediatric population their diagnosis and course are of a great significance, mainly because they can cause visual disturbances such as amblyopia that can ensue, and secondarily due to their cosmetic and psychological effect. Special attention is required in follow up and treatment. Additionally, a systemic evaluation is necessary in order to discover asymptomatic lesions elsewhere in the body carrying a risk of complications. The authors describe the clinical course, diagnosis, therapeutic approach and prognosis of an infant with an orbital cavernous hemangioma accompanying intracranial lesions. A female infant 18 months of age, presented with a mass in the left upper eyelid, causing blepharoptosis. Preoperative magnetic resonance imaging and angiography of the brain and the orbits showed a hemangioma of the left upper eyelid and intracranial lesions to the left temporal fossa and the pons. At the age of 2 years and 8 months she was admitted again due to severe eyelid swelling, intense strong pain, exophthalmos and collateral ophthalmoplegia. Two operations were performed to remove the orbit mass. Histological examination, showed characteristics of cavernous hemangioma. The atypical presentation of cavernous orbital hemangioma with early infantile onset, merits attention.

Orbital Cavernous Hemangioma Presenting With Atypical Appearance -Case Report-

A 25-year-old male presented with unilateral retroorbital dysesthesia persisting for 2 weeks followed by progressive palpebral ptosis without preceding trauma, paranasal sinus surgery, or infectious signs. Neuroophthalmological inspection revealed mildly increased intraocular pressure and exophthalmos on the affected side, without conjunctival chemosis, restriction of the extraocular movements, double vision, or visual impairment. These symptoms did not vary with posture, straining, and Valsalva maneuver. Neuroimaging showed an irregularly-shaped orbital mass without enhancement mainly situated in the medial compartment of the orbit and encasing the optic nerve. Bruit was not audible and abnormal vasculatures were not identified in and around the affected orbit. Transcranial surgical exploration through the surgical window formed by the superior and medial rectus muscles revealed a purplish elastic-soft mass, heterogeneous in consistency and tightly adhering to the surrounding tissue, so cryoprobe-assisted radical tumor resection could not be completed without jeopardizing the optic nerve. The mass was subtotally resected piecemeal without postoperative visual impairment. The histological diagnosis was cavernous hemangioma. Orbital cavernous hemangiomas may present with an atypical appearance which confuses pretreatment diagnosis and makes surgical extirpation more hazardous. Conservative surgery should be indicated for poorly demarcated orbital cavernous hemangiomas considering the usual benign clinical course and postoperative sequelae.

Orbital cavernous hemangioma in an infant with intracranial lesions: a case report

IntroductionCavernous hemangiomas of the orbit are benign vascular malformations, commonly encountered in adults. Although they are infrequent in pediatric population their diagnosis and course are of a great significance, mainly because they can cause visual disturbances such as amblyopia that can ensue, and secondarily due to their cosmetic and psychological effect. Special attention is required in follow up and treatment. Additionally, a systemic evaluation is necessary in order to discover asymptomatic lesions elsewhere in the body carrying a risk of complications.Case presentationThe authors describe the clinical course, diagnosis, therapeutic approach and prognosis of an infant with an orbital cavernous hemangioma accompanying intracranial lesions. A female infant 18 months of age, presented with a mass in the left upper eyelid, causing blepharoptosis. Preoperative magnetic resonance imaging and angiography of the brain and the orbits showed a hemangioma of the left upper eyelid and intracranial lesions to the left temporal fossa and the pons. At the age of 2 years and 8 months she was admitted again due to severe eyelid swelling, intense strong pain, exophthalmos and collateral ophthalmoplegia. Two operations were performed to remove the orbit mass. Histological examination, showed characteristics of cavernous hemangioma.ConclusionThe atypical presentation of cavernous orbital hemangioma with early infantile onset, merits attention.

Transconjunctival orbitotomy for orbital cavernous hemangiomas

Background: Orbital cavernous hemangiomas, particularly those within the retrobulbar space, are approached by neurosurgeons as well as by ophthalmic surgeons. Several surgical techniques have been applied over the past decades. Less traumatic approaches in this functionally and cosmetically important region are desirable. We describe a transconjunctival approach in 39 patients with orbital cavernous hemangiomas. Methods: Thirty-nine patients with orbital cavernous hemangiomas were treated with transconjunctival orbitotomy. The clinical and radiologic characteristics of orbital cavernous hemangiomas were analyzed in the course of their appropriate treatment. Results: In all patients, cavernous hemangiomas were just behind the globe or were compressing it. Computed tomography and magnetic resonance imaging showed orbital cavernous hemangiomas as round, well-defined intraconal masses. In 37 cases, the tumors were removed intact, and only 1 patient suffered permanent vision loss as a result of intraoperative hemorrhage. In the other 2 cases, the tumor fragments were incompletely removed. Visual acuity increased in 6 of the 9 cases with primary visual impairment. Thirty-two patients with primary proptosis were resolved completely. Six patients with visual field defect were also improved. Interpretation: Retrobulbar intraconal cavernous hemangiomas can be removed successfully through a transconjunctival approach. However, if the tumor is close to, or touching, the orbital apex, adhering tightly to retrobulbar tissue, or combined with large draining veins, operation through other approaches must be considered.

Cavernous hemangioma with rapidly developing proptosis

To report an unusual case of cavernous hemangioma with rapidly developing proptosis. Case report. The clinical features, imaging findings and orbital surgery were presented. A 54-year-old man presented with rapidly developing proptosis and a mass in the lower eyelid of his left eye for 8 h. The left eye had 5 mm of proptosis. Color Doppler ultrasonography and computed tomography disclosed a well-defined soft tissue mass in the left anterior orbit, which extended along the floor of the orbit and sized 1.7 x 2.2 cm without bone destruction. An anterior orbitotomy was performed. At surgery a red-purple, well-defined and cone-shaped mass and a hematoma, which was found on the lateral part of the mass, were removed. The histopathological evaluation of the mass revealed cavernous hemangioma. Patient with a rapidly developing proptosis might have the possibility of orbital cavernous hemangioma.

Resection of an Orbital Rim Intraosseous Cavernous Hemangioma and Reconstruction by Chin Graft and Resorbable Fixation Plate

Intraosseous hemangiomas are uncommon in the orbital rim and pose difficulties for removal and reconstruction. Here we report an excellent cosmetic and functional outcome following en bloc resection and reconstruction, using a corticocancellous bone graft from the chin and LactoSorb perforated tray plate. The lesion treated is characterized by both computed axial tomography and histology, while the postsurgical bony contour also is revealed by CT.

The operation of orbital cavernous hemangioma

目的 探讨眼眶海绵状血管瘤的诊断及手术方法.方法 将20例(20眼)眼眶海绵状血管瘤的临床资料、彩色多普勒、超声、CT、MRT的影像表现及手术状况进行回顾性分析研究.结果 20例(20眼)术后矫正视力无明显变化,眼球突出明显改善,眼球运动良好,术后病理证实为海绵状血管瘤.结论 海绵状血管瘤具有特征性的影像学改变.联合影像学特征进行诊断及鉴别诊断并采取灵活的手术方式,为肿物的彻底清除及视力保护具有重要意义。

Growth Potential of Orbital Cavernous Hemangioma Suggested by Vascular Endothelial Growth Factor and its Receptor Flk-1

Orbital cavernous hemangiomas (CHs) manifest as slowly developing symptoms indicative of slow growth. The present study investigated the involvement of angiogenic factors and their receptors in the growth of orbital CHs. Surgical specimens of orbital CHs were obtained from nine patients. Formalin-fixed, paraffin-embedded specimens were stained immunohistochemically using antibodies against Ki-67, CD31, alpha-smooth muscle actin (alpha-SMA), vascular endothelial growth factor (VEGF), basic fibroblast growth factor (bFGF), and VEGF receptors (flt-1 and flk-1). CD31 was expressed in the single layer of endothelial cells lining the vascular cavity. The thick vascular walls were positive for alpha-SMA, indicating that the vascular walls were smooth muscle cells. Ki-67 antigen immunostaining was mostly positive in the vascular walls and the staining index ranged from 0% to 6.8% (mean +/- standard deviation, 2.7 +/- 1.9%). VEGF and bFGF immunostaining were positive in all specimens. Flt-1 immunostaining was negative in all specimens, but flk-1 immunostaining was positive in both endothelial cells and smooth muscle cells. These results suggest that both VEGF and its receptor flk-1 are important in the growth of orbital CH.

Orbital cavernous hemangioma of childhood

Ocular and orbital tumors, both benign and malignant, occur relatively frequently in infants and children. Benign masses are much more common than malignant in the orbital region. However, childhood tumors show great variability and it is difficult to differentiate benign from malignant lesions. Cavernous hemangioma is the most common benign neoplasm of the orbit in adults. We report a case of orbital cavernous hemangioma in a four-year-old girl presenting as unilateral painless proptosis.

Acute Progressive Multiple Cavernous Hemangiomas of Orbit in a Child—A Case Report

Orbital cavernous hemangioma, a slowly progressive tumor of middle aged women is usually unilateral and solitary. We report a case of rapidly progressive multiple cavernous hemangiomas of the orbit in a young male patient.

Removal of cavernous hemangiomas in the muscle funnel by transconjunctival approach

目的 探讨经结膜入路摘除肌肉锥体内海绵状血管瘤的适应证选择、手术方法及技巧.方法回顾性分析2003年1月～2004年12月我院经结膜入路摘除肌肉锥体内海绵状血管瘤21例.结果经结膜入路均可顺利摘除肌锥内海绵状血管瘤,无明显并发症.结论通过术前准确定性,正确定位,判断粘连程度,来选择结膜入路可以安全而简便地摘除肌锥内海绵状血管瘤.

Diagnosis and surgical management of 209 cases of orbital cavernous hemangioma

To study the clinical features, preoperative diagnosis and surgical choice of orbital cavernous hemangioma. Records of all cavernous hemangioma patients (209 cases) who were seen in Eye Hospital, Zhongshan Ophthalmic Center, Sun Yat-sen University from January 1, 1986 to December 31, 2000 were reviewed. Among 209 cases, 123 patients were females and 86 were males. The left orbit was affected in 123 cases and the right orbit in 86 cases. No case was bilaterally involved. The mean age was 39.2 years old, ranging from 5 to 68 years. A painless, gradually progressive proptosis and visual disturbance were the main clinical signs. Ninety-five percent (199/209) of them could be accurately diagnosed preoperatively based on ultrasound scan and CT/MRI examination. The tumors were removed successfully by the surgical procedure of standard lateral orbitotomy in 36% (75/209) of patients and anterior orbitotomy in 64% cases (134/209). Nearly all patients with cavernous hemangioma could be correctly diagnosed by preoperative image studying. The simple surgical procedure of anterior orbitotomy can be successfully used in about two third of cases with less surgical complication.

Orbital cavernous hemangioma with bone erosion

To report a rare computed tomography (CT) feature in one case of orbital cavernous hemangioma (OCH). Case report. The clinical features, CT findings, and pathological examination are presented. A 43-year-old Chinese man developed slowly progressive proptosis and decreased vision in his left eye over 13 years. The best-corrected visual acuity was 20/400 OS. Fundus examination showed moderate disc pallor in the left eye. CT scan revealed a 3.7 x 3.5-cm homogeneous soft tissue mass occupying nearly the whole retrobulbar space. There was focal bone erosion of both the deep lateral orbital wall and deep superior wall. At surgery, a 3.5 x 3.0 x 3.3-cm purplish, well-defined, round mass was removed intact without complication. The histopathologic examination proved it to be OCH. One year later, the visual acuity recovered to 20/15. Patients with OCH may show bone erosion on CT scan, so the presence of bone defect on CT scan should consider the possibility of OCH.

Rapid Development of Amputation Neuroma Following Excision of an Orbital Cavernous Hemangioma

A 48-year-old man with left proptosis and signs of optic nerve compression underwent medial transconjunctival orbitotomy for an excisional biopsy of a posteromedial orbital cavernous hemangioma. The left eye once again became proptotic and painful only 5 weeks later because of a more anteriorly growing orbital mass, which was homogeneously hyperintense relative to orbital fat on both T1- and T2-weighted MRI studies. The tumor proved to be an amputation neuroma after total excision. The latter tumor is presumed to originate most probably from the transected branch of the oculomotor nerve innervating the medial rectus muscle, which remained paralytic after the first operation.