Cavernous hemangioma of the retina

This paper presents an example of a rare clinical entity, a cavernous hemangioma of the retina, with emphasis on the difficulty in differential diagnosis. Relationships with systemic disorders are emphasized. Clinico-pathological schematic drawings accompany photographs to emphasize the importance of differentiating cavernous hemangiomas from retinal telangiectasia, capillary hemangioma and racemose hemangioma. Management of the cavernous hemangioma is routine follow-up examinations and assurance that there are no systemic abnormalities.     

Intra-lacrimal gland cavernous hemangioma

Orbital cavernous hemangioma usually has a typical clinical and imagery pattern. We present a patient with an enlarged lacrimal gland due to an intra-gland cavernous hemangioma.     

Orbital cavernous hemangioma associated with juxtapapillary exophytic retinal capillary hemangioma.

A 32-year-old man presented with coexisting orbital hemangioma and juxtapapillary exophytic capillary hemangioma. Fluorescein and indocyanine green angiography revealed exophytic juxtapapillary retinal capillary hemangioma. The orbital cavernous hemangioma was surgically removed, and the retinal capillary hemangioma was observed without treatment. Subretinal hemorrhage gradually absorbed and clinical symptoms improved. Juxtapapillary exophytic capillary hemangioma can cause subretinal bleeding secondary to external pressure from an adjacent orbital cavernous hemangioma. Coexistence of both tumors suggests a common etiology.     

眼眶海绵状血管瘤的MRI诊断

目的 分析眼眶海绵状血管瘤的MRI表现,以进一步提高该病诊断的准确性.方法 对23例经手术病理证实眼眶海绵状血管瘤的患者,均行MRI平扫及动态增强扫描.结果 23例眼眶海绵状血管瘤T1WI呈等或稍低信号,T2WI、FLAIR呈高信号,DWI呈等或稍高信号.21例动态增强扫描呈"渐进性强化".结论 "渐进性强化"是诊断眼眶海绵状血管瘤的特异性征象,MRI对眼眶海绵状血管瘤的定位和定性诊断具有很高价值.     

Tc-99m labeled red blood cells scintigraphy: a diagnostic method for orbital cavernous hemangioma.

PURPOSE: To evaluate Tc-99m labeled red blood cells scintigraphy (Tc-99m RBC scintigraphy) as a diagnostic method for orbital cavernous hemangioma. PATIENTS AND METHODS: Retrospective analysis of the medical records of all patients who were diagnosed as suffering from orbital cavernous hemangioma over the last 16 years. RESULTS: Medical records of 12 patients with orbital cavernous hemangioma were identified. In all twelve patients, the tentative diagnosis of orbital cavernous hemangioma was made by CT scan. In all of them, the diagnosis was established by Tc-99m RBC scintigraphy, demonstrating a typical picture of "perfusion blood- pool mismatch" (This is normal radionuclide angiography, followed by a variable radionuclide uptake during the early blood-pool study and an intensive radionuclide uptake during the delayed blood-pool study). The tumor was surgically removed in all 12 patients. This diagnostic method was found to be reliable as no single case was recorded in which the preoperative diagnosis was not confirmed pathologically after surgical removal of the tumor. CONCLUSIONS: Tc-99m RBC scintigraphy is a reliable method for diagnosing orbital cavernous hemangioma. It should be included in our arsenal of diagnostic techniques, particularly for cases which are otherwise difficult to diagnose.     

Unusual preseptal location of an orbital cavernous hemangioma in African patient

Cavernous hemangioma is the most common primary vascular orbital tumor in adults. Generally the benign tumor arises within the muscle cone, revealed by proptosis with choroidal folds, and more rarely develops in other orbital localizations. We report the case of a 42-year-old female African patient who had a cavernous hemangioma in an unusual anterior localization. Clinically, a small superficial tumor was palpated in the right superior nasal orbital angle. After complete surgical excision, the histopathological study established the diagnosis of cavernous hemangioma     

A splice-site mutation in CCM1/KRIT1 is associated with retinal and cerebral cavernous hemangioma

PURPOSE: To report a case of a unilateral retinal cavernous hemangioma associated with a novel splice-site mutation in CCM1/KRIT1. METHODS: An 11-year-old girl was noted to have an asymptomatic retinal cavernous hemangioma in the left eye. CCM1/KRIT1 was screened for mutations. RESULTS: Genetic evaluation of CCM1/KRIT1 revealed a single guanine-to-cytosine transversion in the invariant splice acceptor consensus sequence of intron 8 (c.1146-1G-->C), which is predicted to result in abnormal protein splicing. CONCLUSIONS: Mutations in CCM1/KRIT1 may be found in asymptomatic patients with retinal cavernous hemangioma.     

99mTc标记红细胞放射核素显像在眼眶肿瘤鉴别诊断中的应用

目的 对眼眶肿瘤患者进行99mTc-RBC放射核素显像,根据显像结果对眼眶海绵状血管瘤、血管性疾病和非血管性肿瘤进行诊断和鉴别诊断.方法 经B型超声、CT或MRI等影像学方法证实为眼眶肿瘤的75例患者进行99mTc-RBC放射核素显像,其中男32例,女43例,平均年龄44.7岁.据99mTc-RBC放射核素显像方式不同,做出海绵状血管瘤、血管性疾病、供血丰富肿瘤或其他实体性肿瘤的诊断.所有患者均行手术或活检后经病理组织学诊断.结果 31例海绵状血管瘤显像为血池相开始出现放射性异常浓集影,且随时间推移,1h后放射性浓集影逐渐增强,至4h时晚期延迟相放射核素明显增强;1例海绵状血管瘤各个时相均未见异常放射性浓集影.血管畸形显示为血池相即出现异常的放射性浓集影,30min后逐渐增强,至4h减弱或消失.供血丰富肿瘤显示灌注曲线升高,血池相异常放射性浓集影轻度增强,至延迟相消失或无变化.结论 99mTc-RBC显像可作为诊断和鉴别诊断眼眶海绵状血管瘤、血管性疾病、供血丰富肿瘤及其他实体性肿瘤的一种新方法,为临床选择治疗方法提供依据.     

Cavernous hemangioma of the retina. Genetic and central nervous system involvement

A 1967 report described retinal "telangiectasia" in a brother and sister. The brother also had concurrent immune deficiency with hypogammaglobulinemia. Several reports have since been published on similar retinal vascular lesions, which have been termed "cavernous hemangioma of the retina." In retrospect, it is likely that this sibling pair also had cavernous hemangioma of the retina and brain, and that the hypogammaglobulinemia was an independent problem. Our report provides a 19-year follow-up on three generations of this family. The affected generations support the diagnosis of a phakomatosis having autosomal dominant inheritance. Cavernous hemangioma of the retina is usually a stable disease, but there is a potential risk of intracranial hemorrhage from cavernous hemangioma of the brain. Family members, with and without fundus findings, should therefore undergo computed tomography or magnetic resonance imaging of the brain, because neurosurgical prophylaxis or treatment may be indicated.     

Cavernous hemangioma of the retina, cutaneous angiomas, and intracranial vascular lesion by computed tomography and nuclear magnetic resonance imaging

In a 32-year-old man with a right-sided retinal cavernous hemangioma and cutaneous angiomas, computed tomography and nuclear magnetic resonance imaging confirmed the presence of cerebrovascular lesions. This supports the inclusion of cavernous hemangioma of the retina in the established group of neuro-oculo-cutaneous phacomatoses.     

应用99mTc标记红细胞放射核素显像诊断眼眶海绵状血管瘤

目的 评价99mTc-RBC放射核素显像做为一种新的诊断工具,根据肿瘤核素显像情况,对眼眶海绵状血管瘤进行诊断和鉴别诊断.方法 经B型超声、CT或MRI证实为眼眶肿瘤的患者68例进行99mTc-RBC放射核素显像,其中男30例,女38例,平均年龄37.8岁.如果血池相开始出现放射性异常浓集影,且随时间推移,1h后放射性浓集影逐渐增强,至4h时晚期延迟相放射核素明显增强者可判断为海绵状血管瘤.所有患者均行手术或活检后经病理组织学诊断.结果 24例海绵状血管瘤显像为血池相开始出现放射性异常浓集影,且随时间推移,1h后放射性浓集影逐渐增强,至4h时晚期延迟相放射核素明显增强;1例海绵状血管瘤各个时相均未见异常放射性浓集影.43例其他类型肿瘤显像方式与海绵状血管瘤有显著不同.结论 99mTc-RBC显像可作为诊断和鉴别诊断眼眶海绵状血管瘤的一种新方法,可提供术前组织学诊断,从而选择合适的手术入路.     

眼眶海绵状血管瘤与眼眶神经鞘瘤影像学鉴别诊断的研究

目的 研究眼眶海绵状血管瘤与眼眶神经鞘瘤影像学表现特点,探讨最适合鉴别区分二者的影像学检查方法.方法 总结经手术后、组织病理学证实的眼眶海绵状血管瘤25例与眼眶神经鞘瘤24例的B超、CDFI、CT、MRI,归纳各种检查方法所表现出来的影像学特点,对两种肿瘤进行鉴别诊断,以病理学诊断为标准,影像学诊断与之一致的为诊断符合;诊断符合率比较采用X2检验.总结出最适合鉴别这两种肿瘤的影像学检查方法.结果 眼眶海绵状血管瘤的诊断上,MRI(平扫+增强)扫描诊断符合率为100%,CT(平扫+增强)为93%,CDFI为87%,B超为80%.眼眶神经鞘瘤的诊断上,MRI(平扫+增强)诊断符合率为91%,CT(平扫+增强)为47%,CDFI为53%,B超为46%.结论 MRI(平扫+增强)扫描是有效鉴别此二种肿瘤的一种检查方法.     

Management of Optic Neuropathy from an Apical Orbital-Cavernous Sinus Hemangioma with Radiotherapy

We document a case of reversal of compressive optic neuropathy caused by a cavernous sinus-orbital cavernous hemangioma following treatment by conformal stereotactic radiotherapy.     

Acute recurrence of orbital cavernous hemangioma in a young man: a case report

A 29-year-old man presented with a history of prominent left eye of 6 months' duration. He also reported fluctuating blurred vision since 15 days ago. On examination, proptosis of 3 mm was noted in the left eye. Computed tomography (CT scan) of the orbits showed a well-circumscribed, hyperdense, intraconal mass lesion in left orbit, located in the inferotemporal quadrant. Orbitotomy was performed, and the tumor was delivered with an intact capsule. The clinical diagnosis of cavernous hemangioma was confirmed on histopathologic examination. The presence of intact capsule was confirmed grossly and with histopathology. The patient returned 6 weeks after surgery with recurrent proptosis. Repeated CT scan showed a recurrent intraconal mass of similar characteristics as in the primary presentation. A repeated orbitotomy was done, and the tumor was removed intact. Histopathology of the recurrent tumor confirmed the diagnosis of cavernous hemangioma. We report a rare case of orbital cavernous hemangioma with short-term recurrence (6 weeks).     

眼眶内海绵状血管瘤手术进路的临床分析

目的 探讨海绵状血管瘤手术进路的适应证选择、手术方法及技巧。方法 回顾性分析2002年7月～2003年3月武警总医院眼眶病研究所手术治疗的海绵状血管瘤20例。其中10例采用结膜入路，4例采用外侧开眶术，6例采用前路开眶术。结果 除1例结膜入路因粘连重改外侧开眶，1例因大出血加前路切开放血引流，其余均顺利取出瘤体，无明显并发症。结论 安全、有效地摘除海绵状血管瘤选择手术进路很重要。关键在于术前对肿瘤性质、位置、粘连程度的正确判断和手术操作的技巧。     

Management of optic neuropathy from an apical orbital-cavernous sinus hemangioma with radiotherapy

We document a case of reversal of compressive optic neuropathy caused by a cavernous sinus-orbital cavernous hemangioma following treatment by conformal stereotactic radiotherapy.     

Cavernous hemangiomas of the orbit

Patients with cavernous hemangioma of the orbit seen at the Wills Eye Hospital for the 10-year period, 1972-1981 were reviewed in a retrospective manner. This benign vascular tumor most typically presented as unilateral painless proptosis in a middle-aged woman. The duration of symptoms was between 6 months and 2 years. CT scanning demonstrated a well-circumscribed, homogeneous tumor without bony erosion. Ultrasonography typically showed good sound transmission with moderate internal reflectivity. It is not possible to distinguish clinically a benign cavernous hemangioma from the rare and potentially lethal hemangiopericytoma. Since recurrence and metastasis of hemangiopericytoma may be related to incomplete excision, fastidious dissection and removal of all tumors thought to be cavernous hemangioma before surgery is advised for fear the final pathology may indicate hemangiopericytoma.     

Primary intraosseous cavernous hemangioma of the orbit

PURPOSE: Primary orbital intraosseous hemangioma is a rare, benign neoplasm presenting most frequently in patients in their fourth or fifth decade of life. We describe an elderly patient affected by this tumor. METHODS: Case report. RESULTS: A 75-year-old man presented with a slowly growing, bony mass in the left orbital rim inferolaterally. He had a history of nephrectomy because of a renal carcinoma. Computed tomography showed a bony lesion with internal radiating trabeculations. A biopsy was performed. Histopathologically, the tumor was an intraosseous cavernous hemangioma. CONCLUSION: Primary intraosseous cavernous hemangioma of the orbit may infrequently affect elderly patients. One indication for surgical removal of these tumors in the absence of visual disturbances is to rule out metastatic disease in patients with a history of malignancy.     

质子磁共振波谱在眼眶恤管瘤的应用研究

目的　探讨1H MRS在海绵状血管瘤、静脉性血管瘤和血管肉瘤中的应用价值。方法　全部病例均行MRI检查、术后病理证实,1H MRS检查采用1.5T,定点分辨波谱序列(PRESS序列)对眼眶内肿瘤和同侧额部正常脑组织进行定位,测N 乙酰天门冬氨酸(NAA)、胆碱(Cho)、肌酸(Cr)、乙酸盐(Ace)和乳酸(Lac)波。结果　海绵状血管瘤与静脉性血管瘤、血管肉瘤各代谢物曲线下面积在MRS表现上比较有显著差异(P< 0. 05),海绵状血管瘤组: Cho下降、Cr 上升、Lac下降、Ace下降;静脉性血管瘤和血管肉瘤组:Cho上升、Cr下降、Lac上升、Ace上升。结论　1H MRS检查可很好的运用于血管瘤的临床诊断上,并为血管瘤的影像诊断提供一个定量分析的方法。     

Macular syndrome in choroidal cavernous hemangioma: the role of laser treatment

On the basis of a series of case with natural history of choroidal cavernous hemangioma (21 cases with follow-up of up to 13 years) the authors suggest a classification of macular syndromes (types I, II and III) in choroidal cavernous hemangioma. They also suggest that partial barrage of the tumor can be sufficient to arrest or prevent further V.A. impairment.