今日の臨床サポート 今日の臨床サポート

著者: 高村 浩 公立置賜総合病院 眼科

監修: 沖波聡 倉敷中央病院眼科

著者校正/監修レビュー済:2021/11/24
患者向け説明資料

改訂のポイント:
  1. 定期レビューを行い、IgG4関連疾患や甲状腺眼症などについて加筆修正を行った。

概要・推奨   

  1. 眼窩腫瘍のなかではリンパ増殖性疾患の頻度が高い。
  1. 眼窩血管腫の診断にはdynamic MRIが有用である(推奨度2)
  1. 眼窩に限局した悪性リンパ腫に対する放射線治療は低悪性度リンパ腫に対しては30Gy、中~高悪性度リンパ腫に対しては40Gyが適当である(推奨度1)
アカウントをお持ちの方はログイン
  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご
  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契
  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となり
  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必

病態・疫学・診察 

疫学情報・病態・注意事項  
  1. 眼窩内に異常を来す疾患には、腫瘍性疾患、感染を含む炎症性疾患( 眼窩炎症疾患 )、血管異常、外傷( 眼外傷 )、甲状腺眼症( 甲状腺機能亢進症 、 眼球突出 )、先天性疾患( 眼球突出 )など多くのものがある。
  1. 眼窩原発の腫瘍のなかで、良性腫瘍で多いものは血管腫や涙腺多形腺腫など、悪性腫瘍で多いものは悪性リンパ腫である。
  1. 続発性(浸潤性)の眼窩腫瘍で多いものは、副鼻腔の嚢胞性疾患や扁平上皮癌である。転移性眼窩腫瘍では、悪性リンパ腫や肺癌、乳癌などの転移が多い。
  1. 血管異常には、内頚動脈海綿静脈洞瘻や眼窩静脈瘤、海綿静脈洞血栓症( 脳静脈血栓症 、 動静脈奇形 )などがある。
  1. 眼窩内異常を来す疾患では、 眼球突出 、眼球運動制限、複視、眼球偏位、視力低下などが生じる。血管異常では、間欠性眼球突出や球結膜の血管怒張などがみられる。炎症・感染性疾患では、疼痛、眼瞼や結膜の発赤、腫脹などを来す。甲状腺眼症では眼瞼後退などの眼瞼異常を伴う。眼窩壁骨折などの外傷では眼球が陥凹することもある。
 
 
  1. 眼窩腫瘍のなかではリンパ増殖性疾患の頻度が高い(M)
  1. 日本で報告された眼窩腫瘍症例のメタ解析研究がある[1]。全1,483例のうち、原発性眼窩腫瘍が47%、続発性眼窩腫瘍が30%、炎症性疾患が22%であった。原発性眼窩腫瘍のなかで最も多いのが悪性リンパ腫(12%)で、次が多形腺腫(7%)であった。転移性眼窩腫瘍のなかで多いのは肺癌、乳癌、甲状腺癌だった。全体で最も多かったのは炎症性偽腫瘍(18%)だった。
  1. 悪性リンパ腫と炎症性偽腫瘍を合わせてリンパ増殖性疾患と呼称するが、それが眼窩腫瘍のなかで大きな割合を占めていた。
問診・診察のポイント  
 
  1. 問診では以下の項目について詳細な情報を得る。
    ①発症時期(罹病期間)、②進行速度(急激か緩徐か)、③眼球運動時痛など疼痛の有無、④複視や視力低下など視機能の変化、⑤頭痛や鼻づまりなど眼窩周辺部位の症状の有無、⑥副鼻腔疾患、脳疾患、甲状腺機能異常や悪性腫瘍などの全身疾患の既往歴、⑦外傷の有無
 
 
  1. 診察では視力や眼圧測定に加えて以下の項目の有無について観察する。
    ①眼窩縁や眼瞼皮下での腫瘤の触知、② 眼球突出 (ヘルテル眼球突出計)、③眼球運動制限・眼球偏位・複視(ヘスチャート)、④眼瞼の発赤・腫脹、結膜充血、⑤球結膜血管怒張、眼窩部での血管雑音(聴診器)、⑥視神経乳頭の腫脹あるいは萎縮や網・脈絡膜皺襞、網膜血管走行異常、⑦頚部リンパ節などの腫大
 
検査
  1. 画像検査:

これより先の閲覧には個人契約のトライアルまたはお申込みが必要です。

最新のエビデンスに基づいた二次文献データベース「今日の臨床サポート」。
常時アップデートされており、最新のエビデンスを各分野のエキスパートが豊富な図表や処方・検査例を交えて分かりやすく解説。日常臨床で遭遇するほぼ全ての症状・疾患から薬剤・検査情報まで瞬時に検索可能です。

まずは15日間無料トライアル
本サイトの知的財産権は全てエルゼビアまたはコンテンツのライセンサーに帰属します。私的利用及び別途規定されている場合を除き、本サイトの利用はいかなる許諾を与えるものでもありません。 本サイト、そのコンテンツ、製品およびサービスのご利用は、お客様ご自身の責任において行ってください。本サイトの利用に基づくいかなる損害についても、エルゼビアは一切の責任及び賠償義務を負いません。 また、本サイトの利用を以て、本サイト利用者は、本サイトの利用に基づき第三者に生じるいかなる損害についても、エルゼビアを免責することに合意したことになります。  本サイトを利用される医学・医療提供者は、独自の臨床的判断を行使するべきです。本サイト利用者の判断においてリスクを正当なものとして受け入れる用意がない限り、コンテンツにおいて提案されている検査または処置がなされるべきではありません。 医学の急速な進歩に鑑み、エルゼビアは、本サイト利用者が診断方法および投与量について、独自に検証を行うことを推奨いたします。

文献 

Keigo Shikishima, Kazushige Kawai, Kenji Kitahara
Pathological evaluation of orbital tumours in Japan: analysis of a large case series and 1379 cases reported in the Japanese literature.
Clin Experiment Ophthalmol. 2006 Apr;34(3):239-44. doi: 10.1111/j.1442-9071.2006.01192.x.
Abstract/Text PURPOSE: To review epidemiological features of orbital tumours in Japan.
METHODS: Retrospective, observational case series and systematic review. A total of 104 patients with orbital tumours collected at the authors' institution during 1983-2002 were assessed. In addition, 1379 cases from a large series of orbital tumours diagnosed by histopathological analysis that were previously published in Japanese ophthalmological journals from 1980 to 2004 were analysed.
RESULTS: After combining the current data with the previously published cases, there were a total of 1483 lesions with 47% classified as primary, 30% as secondary and 22% as inflammatory. The most common primary tumour was malignant lymphoma (12%) followed by pleomorphic adenoma (7%). Carcinomas from the lung, breast and thyroid were found to predominate among orbital metastases. Inflammatory pseudotumour had the highest lesion frequency (18%).
CONCLUSIONS: Malignant lymphoma by far was found to have the most dramatic increases within the recent reported series. Pleomorphic adenomas in Japan were found to be much more common compared with that reported for American and European studies.

PMID 16671904
Steven Yeh, Rod Foroozan
Orbital apex syndrome.
Curr Opin Ophthalmol. 2004 Dec;15(6):490-8.
Abstract/Text PURPOSE OF REVIEW: Visual loss from optic neuropathy and ophthalmoplegia involving multiple cranial nerves are the hallmarks of an orbital apex syndrome. Historically, the terms superior orbital fissure, orbital apex, and cavernous sinus have been used to define the anatomic locations of a disease process. However, the diagnostic evaluation and management is similar for each of these entities. The authors reviewed the literature on the diagnosis and evaluation of disorders involving the orbital apex.
RECENT FINDINGS: High-resolution MRI is the preferred modality for evaluating most lesions involving the orbital apex. CT is a useful tool in the setting of trauma, to evaluate bone involvement, or when MRI is contraindicated. Although laboratory studies may be useful adjuncts in the diagnostic evaluation of lesions involving the orbital apex, surgical biopsy is often required for definitive diagnosis.
SUMMARY: Orbital apex syndromes may result from a variety of inflammatory, infectious, neoplastic, iatrogenic/traumatic, and vascular conditions. A detailed history with review of systems is important in narrowing the differential diagnosis. Management is directed at the underlying cause and may be guided by surgical biopsy. Corticosteroids may be useful if an inflammatory etiology is suspected, but should be used with caution.

PMID 15523194
Taku Sugiyama, Satoshi Kuroda, Naoki Nakayama, Kiyohiro Houkin
[Invasive paranasal sinus fungal infection developing orbital apex syndrome and causing internal carotid artery infiltration: reports of 3 cases].
No Shinkei Geka. 2011 Feb;39(2):155-61.
Abstract/Text Fungal infection is an increasing problem in patients treated with intensive chemotherapy or immunosuppressive therapy. Of these, invasive aspergillosis or mucormycosis on rare occasions, infiltrate the internal carotid artery (ICA) by contiguous spread from an infected paranasal sinus. Here, we report three cases of ICA infiltration secondary to the fungal infections of the paranasal sinus. All cases developed orbital apex syndrome when the lesion spread to the orbital apex or cavernous sinus, and aggressive progression led to ICA invasion causing life-threatening cerebral infarction or hemorrhage. In this case report, we describe the aggressive clinical course of the fungal ICA invasion from the paranasal sinus, and then discuss the clinical feature, diagnostic methods, and current treatment of intracranial fungal infection.

PMID 21321374
Yoshino Ueki, Toshinari Kazuta, Eri Naitou, Masaharu Hayashi, Kozue Tanaka, Toshio Mizutani, Shunsaku Hirai
[A case of CNS aspergillosis developing orbital apex syndrome and causing mycotic aneurysm and the subsequent cerebral infarction].
Rinsho Shinkeigaku. 2002 Aug;42(8):761-5.
Abstract/Text A 79-year-old woman, with no immune deficit, had presented progressive visual disturbance, diplopia and ptosis of her left eye over 2 weeks. T1-weighted MR images with gadolinium showed a heterogeneously enhanced lesion extending from the left orbital apex along the optic nerve to the cavernous sinus. Although we could not detect fungus by a transsphenoidal biopsy, we suspected fungal infection because of high level of galactomanan antigen in serum. Despite antifungal chemotherapy, her symptoms did not improve. CT image on day 40 showed an aneurysm in the left internal carotid artery, on day 43 cerebral infarction in the left internal carotid artery distribution and on day 45 she died. Autopsy disclosed that aspergillus hyphae invaded the left sphenoid sinus, cavernous sinus and wall of the aneurysm. In this case, fungal infection in the frontal skull base including orbital apex caused mycotic aneurysm in the intracavernous portion of the left internal carotid artery. Skull base aspergillosis presenting orbital apex syndrome is itself rare and in addition, the occurrence of cerebral infarction in the mycotic aneurysm has hardly been reported. We should have cerebrovascular disease in mind as a complication of CNS aspergillosis.

PMID 12701224
N Fernández, E Murias, P Vega, A Sainz, A Meilán
[Angioplasty confirmation of the spontaneous resolution of two low-flow carotid-cavernous fistulas].
Neurologia. 2010 Jun;25(5):333-6.
Abstract/Text
PMID 20643045
Isabella T Phan, William F Hoyt, Timothy J McCulley, Thomas N Hwang
Blindness from orbital varices: case report.
Orbit. 2009;28(5):303-5. doi: 10.3109/01676830903043843.
Abstract/Text A 41-year-old woman presented with a 21-year history of a left orbital mass. She reported 3 distinct episodes of sudden proptosis, periorbital bruising, pain, nausea and vomiting with resulting stepwise deterioration in her vision. Her symptoms resolved spontaneously over several days, with the exception of loss in vision, which persisted. Examination was notable for ipsilateral enophthalmos in primary gaze. With Valsalva she developed proptosis. Magnetic resonance imaging (MRI) demonstrated a left orbital apex malformation consistent with a varix. She had no light perception on the left with end-stage optic atrophy. This case illustrates the severity of visual loss that can occur with orbital varices.

PMID 19874125
Tsugio Amemiya, Hirohiko Hayashida, Yoshinori Dake
Metastatic orbital tumors in Japan: a review of the literature.
Ophthalmic Epidemiol. 2002 Feb;9(1):35-47.
Abstract/Text A review of reports on metastatic orbital tumors published from 1903 to 1998 in Japan revealed 128 patients, 74 males, 52 females and 2 whose sex was not recorded. The average age was 44.8 years, but varied depending on the primary tumor. Since 1980, metastatic orbital tumors have increased in Japan, especially those from the lung, liver and adrenal gland, while metastasis from the stomach has decreased slightly. Metastasis from the breast is still common. Most metastatic orbital tumors were from the lung, followed, in order, by breast, liver, adrenal gland and stomach. Males had four times as many metastatic orbital tumors from lung cancer than did females; only females had metastases from breast cancer; almost 90% of metastases from hepatoma were in males; metastasis from renal carcinoma was 2-3 times more common in males than in females. Metastasis from the liver and stomach is seen more frequently in Japan than in the United States and Europe. Ocular signs due to orbital metastases from hepatoma, neuroblastoma and gastric cancer were apt to appear earlier than the signs of the primary lesion. Metastases to the orbit were frequently bilateral in patients with neuroblastoma and malignant lymphoma. Specific ocular signs such as ecchymosis and conjunctival hemorrhages were seen in orbital metastasis from neuroblastoma and seminoma, while ocular pain was characteristic of malignant lymphoma. Orbital metastasis was very rare in patients with carcinoma of the uterus, ovaries, bladder, pancreas, colon or rectum in both Japan, the United States and Europe.

PMID 11815894
Atsuo Tanaka, Futoshi Mihara, Takashi Yoshiura, Osamu Togao, Yasuo Kuwabara, Yoshihiro Natori, Tomio Sasaki, Hiroshi Honda
Differentiation of cavernous hemangioma from schwannoma of the orbit: a dynamic MRI study.
AJR Am J Roentgenol. 2004 Dec;183(6):1799-804. doi: 10.2214/ajr.183.6.01831799.
Abstract/Text OBJECTIVE: The purpose of this study was to determine the capability of dynamic contrast MRI to differentiate hemangioma from schwannoma of the orbit.
MATERIALS AND METHODS: Sixteen patients (three males and 13 females; mean age, 39 +/- 17.3 [SD] years; age range, 10-71 years) with unilateral orbital tumors, including eight cavernous hemangiomas and eight schwannomas, were examined. In addition to conventional MRI, we performed a dynamic contrast study (fast spin-echo sequence, 20-sec interval) after bolus administration of the contrast material (gadopentetate dimeglumine, 0.1 mmol/kg). We evaluated the features of the contrast enhancement spread pattern and the tumors' time-intensity curves.
RESULTS: In the early phase, all the hemangiomas started the enhancement from one point or portion, although all the schwannomas started the enhancement from a wide area. The difference in the contrast-enhancement spread pattern features between the two types of tumors was statistically significant (p < 0.0001). The gradient of the time-intensity curve did not show a significant difference.
CONCLUSION: Hemangioma and schwannoma of the orbit can be differentiated by the contrast-enhancement spread pattern on dynamic MRI.

PMID 15547232
Alon Kahana, Mark J Lucarelli, Allison M Grayev, Jeremy J Van Buren, Cat N Burkat, Lindell R Gentry
Noninvasive dynamic magnetic resonance angiography with Time-Resolved Imaging of Contrast KineticS (TRICKS) in the evaluation of orbital vascular lesions.
Arch Ophthalmol. 2007 Dec;125(12):1635-42. doi: 10.1001/archopht.125.12.1635.
Abstract/Text OBJECTIVE: To evaluate the clinical utility of time-resolved contrast-enhanced magnetic resonance angiography (MRA) in the evaluation of vascular orbital tumors.
METHODS: Retrospective medical record review of patients with vascular orbital lesions imaged with Time-Resolved Imaging of Contrast KineticS (TRICKS; GE Healthcare [Chalfont St Giles, England]) MRA, a noninvasive dynamic imaging modality.
RESULTS: Five patients with orbital vascular tumors were evaluated using TRICKS MRA. These included 1 patient with a cavernous hemangioma, 2 patients with orbital varices, 1 patient with an orbitocutaneous arteriovenous malformation, and 1 patient who had a solitary fibrous tumor with features of a hemangiopericytoma. In 2 patients, diagnoses were altered as a result of TRICKS MRA. In addition, a young patient with a large orbitocutaneous arteriovenous malformation involving the ophthalmic artery was followed perioperatively and noninvasively using TRICKS MRA, which produced exquisite images and added substantial value in the care of these patients.
CONCLUSION: Dynamic MRA in the form of TRICKS is a newly available imaging modality with great potential for improving the evaluation and management of patients with complex orbital tumors.

PMID 18071114
Jayant Sastri Goda, Lisa W Le, Normand J Lapperriere, Barbara-Ann Millar, David Payne, Mary K Gospodarowicz, Woodrow Wells, David C Hodgson, Alexander Sun, Rand Simpson, Richard W Tsang
Localized orbital mucosa-associated lymphoma tissue lymphoma managed with primary radiation therapy: efficacy and toxicity.
Int J Radiat Oncol Biol Phys. 2011 Nov 15;81(4):e659-66. doi: 10.1016/j.ijrobp.2011.03.050. Epub 2011 Jun 2.
Abstract/Text PURPOSE: To evaluate the clinical outcomes and late effects of radiation therapy (RT) in localized primary orbital mucosa-associated lymphoma tissue (MALT) lymphoma (POML).
METHODS AND MATERIALS: From 1989 to 2007, 89 patients with Stage IE POML received RT. The median age was 56 years old. Sites involved conjunctiva (59 patients [66%]), lacrimal gland (20 patients [23%]), and soft tissue (10 patients [11%]). Megavoltage beam(s) was used in 91%, electrons in 7%, and orthovoltage in 2% of cases. The dose given was 25 Gy in 97% and 30 Gy in 3% of patients. Lens shielding was possible in 57% of patients.
RESULTS: The median follow-up was 5.9 years. Complete response or unconfirmed complete response was seen in 88 patients (99%). Relapse occurred in 22 patients (25%). First relapse sites were local (2 patients [9%]), in the contralateral orbit (5 patients [23%]), and distant (15 patients [68%]). The 7-year overall survival (OS), cause-specific survival (CSS), relapse-free survival (RFS), and local control (LC) rates were 91%, 96%, 64%, and 97%, respectively. Radiation-related late sequelae were documented in 40 patients (45%). Cataracts were observed in 22 patients (Grade 1 in 2 patients; Grade 3 in 20 patients). The incidence of Grade 3 cataract at 7 years was 25%. Other late sequelae (n = 28) were dry eye(s) (22 patients [Grade 1 in 14 patients; Grade 2 in 2 patients; Grade 3 in 2 patients; n/s in 4 patients), keratitis (3 patients), macular degeneration/cystoid edema (2 patients), and vitreous detachment (1 patient). Five patients developed Grade 3 noncataract late effects. Lens shielding reduced the incidence of Grade 3 cataract and all Grade ≥2 late sequelae. Seventeen patients (16 with cataracts) underwent surgery; 23 patients were treated conservatively. The outcome for managing late effects was generally successful, with 30 patients completely improved, and 9 patients with persisting late sequelae (10%).
CONCLUSIONS: POML responds favorably to moderate doses of RT but results in significant late morbidity. The majority of late effects were successfully managed. Lens shielding reduced the risk of cataracts and other late sequelae.

Copyright © 2011 Elsevier Inc. All rights reserved.
PMID 21640514
Sudershan Bhatia, Arnold C Paulino, John M Buatti, Nina A Mayr, B-Chen Wen
Curative radiotherapy for primary orbital lymphoma.
Int J Radiat Oncol Biol Phys. 2002 Nov 1;54(3):818-23.
Abstract/Text PURPOSE: To review our institutional experience with primary orbital lymphoma and determine the prognostic factors for survival, local control, and distant metastases. In addition, we also analyzed the risk factors for complications in the radiotherapeutic management of this tumor.
METHODS AND MATERIALS: Between 1973 and 1998, 47 patients (29 women [62%] and 18 men [38%], median age 69 years, range 32-89) with Stage IAE orbital lymphoma were treated with curative intent at one department. Five had bilateral orbital involvement. The tumor was located in the eyelid and extraocular muscles in 23 (44%), conjunctiva in 17 (33%), and lacrimal apparatus in 12 (23%). The histologic features according to the World Heath Organization classification of lymphoid neoplasms was follicular lymphoma in 25, extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type in 8, diffuse large B-cell lymphoma in 12, mantle cell lymphoma in 6, and peripheral T-cell lymphoma in 1. For the purposes of comparison with the existing literature on orbital lymphomas, the grading system according to the Working Formulation was also recorded. The histologic grade was low in 33 (63%), intermediate in 18 (35%), and high in 1 (2%). All patients were treated with primary radiotherapy alone. The median dose for low-grade tumors was 3000 cGy (range 2000-4020); the median dose for intermediate and high-grade tumors was 4000 cGy (range 3000-5100). A lens-sparing approach was used in 19 patients (37%). Late complications for the lens and cornea were scored according to the subjective, objective, management, and analytic (SOMA) scale of the Late Effects of Normal Tissue (LENT) scoring system. The median follow-up was 55 months (range 6-232).
RESULTS: The local control rate was 100% in the 52 orbits treated. The 5-year overall survival and relapse-free survival rate was 73.6% and 65.5%, respectively. Tumor grade and location did not predict for overall survival or relapse-free survival. Seven patients (15%) developed distant recurrence (brain 2, extremity 2, mediastinum 1, liver 1, and retroperitoneum 1). One patient (2%) developed cervical node metastasis. The 5- and 10-year cataract-free survival rate was 56.7% and 32.9%, respectively. Of the 12 lens complications, 8 were LENT Grade 1 and 4 were Grade 3 toxicity. Only male gender predicted for an increased risk of cataract formation. Radiotherapy dose and technique did not predict for cataract formation; however, none of the patients who underwent the lens-sparing technique developed Grade 3 lens toxicity or required surgical correction. Of the nine corneal events, two were Grade 1, four Grade 2, and three were Grade 3 toxicity. Ten dry eyes were recorded; all were mild, and no patient had severe dry eye syndrome. Neovascular glaucoma was seen in 1 patient. No injury to the retina or optic nerve was reported.
CONCLUSION: Radiotherapy alone is a highly effective modality in the curative management of primary orbital lymphoma. Most complications were minimal and did not require medical or surgical intervention. Although the use of the lens-sparing technique did not influence the incidence of cataractogenesis, we continue to recommend this approach whenever possible, because our experience indicates a higher grade of toxicity occurs and a higher incidence of corrective surgery is needed in patients treated without lens protection.

PMID 12377334
Jia-sheng Yu, Ting Lei, Jin-cao Chen, Yue He, Jian Chen, Ling Li
Diagnosis and endovascular treatment of spontaneous direct carotid-cavernous fistula.
Chin Med J (Engl). 2008 Aug 20;121(16):1558-62.
Abstract/Text BACKGROUND: Spontaneous direct carotid-cavernous fistula (CCF) is relatively rare and few reports have been found in the literature. The aim of this paper was to report the clinical characteristics, imaging findings and curative effect of endovascular treatment for patients with spontaneous direct CCF.
METHODS: We retrospectively analyzed the clinical data of nine patients with spontaneous direct CCF admitted between May 2003 and November 2007 and the outcomesof endovascular treatment. Sudden neuro-ophthalmological symptoms were the most common clinical presentation at diagnosis (n=8). No patients had a history of head trauma. Cerebral digital subtraction angiography (DSA) was performed on all cases under local anesthesia and seven cases received endovascular treatment.
RESULTS: In eight patients, internal carotid angiograms recorded during the early arterial phase revealed aneurysms located in the cavernous sinuses, and in one patient, a dilated internal carotid artery (ICA) was seen. Among the nine cases, seven received endovascular treatment via a transarterial approach and complete occlusion of the fistula was obtained with no technique-related complications, one died suddenly before treatment and one gave up treatment. A detachable balloon was used as the embolic material in two cases, a detachable balloon and detachable coil as the embolic material in two cases, balloon-assisted coil embolization in two cases and covered stents were successfully placed in the parent vessel to exclude the aneurysm and fistula from circulation in one case. During a follow-up period of 3-48 months, all treated patients remained asymptomatic except for one patient who suffered from ipsilateral decreased vision.
CONCLUSIONS: Most spontaneous direct CCF may be caused by a ruptured intracavernous aneurysm with direct shunting into the cavernous sinus. Endovascular treatment seems to be a safe and effective method for treating spontaneous direct CCF.

PMID 18982868
Qi Tiewei, Alhothi Ali, Guo Shaolei, Liang Feng, Shi Zhongsong, Li Xuesong, Huang Zhengsong
Carotid cavernous fistulas treated by endovascular covered stent grafts with follow-up results.
Br J Neurosurg. 2010 Aug;24(4):435-40. doi: 10.3109/02688697.2010.487127.
Abstract/Text Historically, carotid cavernous fistulas (CCFs) are usually treated with detachable balloons and coils. While coronary covered stent grafts have been sporadically used in the intracranial vasculature, only a few such cases have been reported in the literature. We present our experience of seven patients with eight CCFs, treated by the placement of covered stents, and provide their clinical and angiographic follow-up results. From 28 cases of CCF treated at our department between November 2000 and January 2007, a total of eight post-traumatic CCF were treated between May 2005 and January 2007 by positioning a Jostent coronary stent graft (Abbott Vascular, Redwood City, CA) in the intracranial ICA. These patients had periodic follow-up examinations (at 12-30 months) with 5 of them receiving a follow-up angiographic examination (at 6-9 months). Immediate post-procedural angiograms demonstrated total exclusion in five CCFs, residual filling in three CCFs, and preserved ICA patency in all CCFs. Symptoms related to the CCF regressed within 1-15 days after the covered stent placement. There was no mortality and no immediate post-procedural morbidity related to the CCF covered stent treatment; however, one patient died of acute myocardial infarction in 10 days following treatment. Six patients showed complete clinical recovery during their clinical follow-up. The angiographic follow-up (mean, 8 months) in five patients with six CCFs revealed regressed residual filling, stable occlusion of all CCFs, and an ICA patency (one case of asymptomatic ICA occlusion) rate of 83.3%. Covered stents are a promising therapeutic alternative for patients with CCF in whom fistulas cannot be successfully occluded with detachable balloons or coils. Our results and the reported results showed that a covered stent could occlude the fistula and preserve the ICA without hemodynamic changes or negative clinical effects. Covered stent grafts could soon become a first-line therapy as experience with this device increases, materials continue to improve, and more data are accumulated.

PMID 20515263
E Archondakis, G Pero, L Valvassori, E Boccardi, G Scialfa
Angiographic follow-up of traumatic carotid cavernous fistulas treated with endovascular stent graft placement.
AJNR Am J Neuroradiol. 2007 Feb;28(2):342-7.
Abstract/Text BACKGROUND AND PURPOSE: Endovascular treatment (EVT) of carotid cavernous fistulas (CCFs) is based on various techniques, mainly those using detachable balloons. Coronary covered stent grafts have been sporadically used in the intracranial arteries and only 2 traumatic CCFs have been reported in the literature; moreover, there is poor information about the long-term follow-up. We present 8 cases of CCFs treated by the placement of a covered stent, 5 of which have a 1-year clinical and angiographic follow-up.
METHODS: Eight patients with posttraumatic CCF were treated by positioning a covered stent in the intracranial internal carotid artery (ICA) to occlude the fistula. They received periodic clinical and angiographic follow-up to evaluate the patency and the stability of clinical results.
RESULTS: In all cases, the symptoms related to the CCF regressed after treatment and did not recur in the follow-up. Two patients presented residual filling of the CCF at the end of the procedure. The angiographic follow-up revealed in 6 patients of 7 a good patency of the ICA; in 1 patient, there was an ICA asymptomatic occlusion. One patient required transvenous coil occlusion of the cavernous sinus.
CONCLUSION: When standard treatments fail, covered stent grafts can be used as a valid alternative in the treatment of CCFs, but more data are needed, especially in the long-term follow-up.

PMID 17297009
Steven M Couch, James A Garrity, J Douglas Cameron, Harry J Cloft
Embolization of orbital varices with N-butyl cyanoacrylate as an aid in surgical excision: results of 4 cases with histopathologic examination.
Am J Ophthalmol. 2009 Oct;148(4):614-618.e1. doi: 10.1016/j.ajo.2009.04.024. Epub 2009 Jul 9.
Abstract/Text PURPOSE: To report the results of intervention with percutaneously injected n-butyl cyanoacrylate (NBCA) to embolize orbital varices followed by surgical resection.
DESIGN: Retrospective case series.
METHODS: Four patients with symptomatic orbital varices were treated with percutaneous injection of NBCA to embolize the varicosity before surgical resection. Intervention was indicated because of progressive orbital pain attributed to orbital varices. Three of the 4 described cases were associated with severe episodic proptosis. The vision was not affected by the orbital varix in any of the cases before intervention. Radiographic guidance was used during injection of the NBCA. Surgical resection was undertaken via orbitotomy immediately after embolization. The resected tissue was submitted for histopathologic evaluation.
RESULTS: Follow-up after surgery ranged from 7 to 19 months. All of the patients experienced relief of orbital pain. All patients noted transient binocular diplopia in extremes of gaze after the procedure, which resolved spontaneously. No patients had diplopia in primary gaze. No patient lost vision as a result of the procedure. There was no difficulty with procedure-related hemostasis in any of the cases.
CONCLUSIONS: Percutaneously injected NBCA seems to be useful and safe as an aid in visualization and hemorrhage prevention during surgical resection of symptomatic orbital varices.

PMID 19541289
Desheng Xu, Dong Liu, Zhiyuan Zhang, Yipei Zhang, Guoxiang Song
Gamma knife radiosurgery for primary orbital varices: a preliminary report.
Br J Ophthalmol. 2011 Sep;95(9):1264-7. doi: 10.1136/bjo.2009.170001. Epub 2010 Oct 22.
Abstract/Text AIM: The aim of this retrospective study is to evaluate the authors' experience using gamma knife radiosurgery in the management of primary orbital varices.
METHODS: Fourteen patients, six males and eight females, with ages ranging from 7 to 56 years of age, were treated with gamma knife radiosurgery from April 2001 to June 2005 for primary orbital varices. The median prescription peripheral dose was 16 Gy, ranging from 15 to 20 Gy, and the median maximum dose was 32 Gy, ranging from 30 to 40 Gy. The median volume of the lesion at radiosurgery was 4.5 ml (range 1.9 ml to 9.0 ml). The mean dose to optic nerve was below 10 Gy. A mean of 10 isocentres (range 8 to 13) were used for treating these lesions.
RESULTS: At a median follow-up period of 36 months (range 18 to 66 months), recurrent exophthalmos and diplopia resolved in 10 patients. Two of the remaining four patients showed evidence of decrease in distensibility, while the other two remain unchanged. The median time of their presenting symptoms resolving was 7 months (range 3 to 18 months). One patient lost her sight 18 months after gamma knife radiosurgery. No patient had intraorbital haemorrhage after treatment.
CONCLUSION: Gamma knife radiosurgery provides effective long-term relief of symptoms in selected patients with primary orbital varices.

PMID 20971792
Mathew Bujak, Edward Margolin, Andrew Thompson, Jonathan D Trobe
Spontaneous resolution of two dural carotid-cavernous fistulas presenting with optic neuropathy and marked congestive ophthalmopathy.
J Neuroophthalmol. 2010 Sep;30(3):222-7. doi: 10.1097/WNO.0b013e3181ceb483.
Abstract/Text Two patients with dural carotid-cavernous fistulas (CCFs) presented with optic neuropathy and marked congestive ophthalmopathy, including 1 patient with a narrowed anterior chamber angle due to choroidal effusions. Endovascular intervention was planned but deferred for logistic reasons. While the patients awaited the procedures, the clinical features markedly improved, and time-resolved imaging of contrast kinetics (TRICKS) MRA was consistent with closure of the CCFs. These patients serve as a reminder that spontaneous resolution may occur in dural CCFs even when presenting clinical features are florid and vision appears to be threatened. In fact, a rapid worsening of clinical manifestations may be a sign that a dural CCF is about to close spontaneously.

PMID 20498622
Mazhar Ishaq, Muhammad Aamir Arain, Saadullah Ahmed, Muhammad Khizar Niazi, Muhammad Dawood Khan, Zamir Iqbal
Spontaneous resolution of direct carotid cavernous fistula.
J Coll Physicians Surg Pak. 2010 Sep;20(9):622-4. doi: 04.2010/JCPSP.622624.
Abstract/Text Proptosis due to carotid cavernous fistula is rare sequelae of head injury. We report a case of post-traumatic, direct high flow carotid cavernous fistula that resolved spontaneously 06 weeks after carotid angiography. It however, resulted in loss of vision due to delay in early treatment. In the cases of post-traumatic proptosis, carotid cavernous fistula should be kept in mind.

PMID 20810059
Satoshi Nakamura, Takakazu Kawamata, Tomonori Kobayashi, Tomokatsu Hori
Clival inflammation with cavernous sinus thrombophlebitis and orbital subperiosteal abscess--case report.
Neurol Med Chir (Tokyo). 2010;50(2):168-71.
Abstract/Text A 64-year-old woman presented with a very rare case of three infectious lesions, cavernous sinus thrombophlebitis, clival inflammation, and orbital subperiosteal abscess (SPA), manifesting as abducens palsy. An isolated non-specific mass in upper clivus was initially suspected to be derived from paranasal sinusitis. The clival lesion was approached by an endonasal transsphenoidal route and diagnosed as inflammation. However, progressive enlargement of an orbital mass was recognized, with eyelid erythema and swelling. Magnetic resonance imaging showed massive paranasal sinusitis and an intra-orbital mass, which was proved to be an orbital SPA by open surgery. Cavernous sinus thrombophlebitis might have been caused by primary paranasal sinusitis, and the origin of orbital cellulitis was suspected to be cavernous sinusitis based on the preoperative radiological findings. These unusual lesions should be kept in mind as one of the differential diagnoses.

PMID 20185888
Chisho Hoshino, Noriyuki Satoh, Shinichi Sugawara, Chizuko Kuriyama, Akio Kikuchi, Masahiro Ohta
Septic cavernous sinus thrombosis complicated by narrowing of the internal carotid artery, subarachnoid abscess and multiple pulmonary septic emboli.
Intern Med. 2007;46(6):317-23. Epub 2007 Mar 15.
Abstract/Text A 56-year-old woman was admitted because of a high fever, right ptosis, chemosis, proptosis and ocular muscle palsy. Cranial MRI revealed a cavernous sinus thrombosis and a subarachnoid abscess. Carotid angio-gram demonstrated marked stenosis as well as aneurismal formation of the right internal carotid artery at the intracavernous portion. Chest radiograph showed bilateral multiple pulmonary nodules, some of which contained a cavity. Blood culture was positive for Streptococcus constellatus. She was diagnosed with septic cavernous sinus thrombosis complicated by narrowing of the internal carotid artery, subarachnoid abscess and multiple pulmonary septic emboli. She recovered with partial ocular sequelae as a result of seven weeks of intravenous antimicrobial therapy.

PMID 17380002
M J DiNubile
Septic thrombosis of the cavernous sinuses.
Arch Neurol. 1988 May;45(5):567-72.
Abstract/Text Cavernous sinus thrombosis may occur as a complication of infectious and noninfectious processes. Septic thrombosis of the cavernous sinuses most commonly follows infections of the middle third of the face due to Staphylococcus aureus. Other antecedent sites of infection include paranasal (usually sphenoid) sinusitis, dental abscess and, less often, otitis media. Fever is a nearly constant finding, but headache may not be prominent. Periorbital edema, chemosis, proptosis, and limitation of extraocular movements (especially lateral gaze) develop in almost all recognized cases. Involvement of the opposite eye frequently appears within two days following the onset of unilateral signs. Although computed tomography may be helpful, magnetic resonance imaging is probably the diagnostic procedure of choice. Treatment includes appropriate antibiotics and, oftentimes, surgical drainage of the primary focus of infection. Less than half of the patients recover completely; the mortality rate is approximately 30%.

PMID 3282499
薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、渡邉裕次、井ノ口岳洋、梅田将光および日本医科大学多摩永山病院 副薬剤部長 林太祐による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、 著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※同効薬・小児・妊娠および授乳中の注意事項等は、海外の情報も掲載しており、日本の医療事情に適応しない場合があります。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適応の査定において保険適応及び保険適応外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適応の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
高村 浩 : 特に申告事項無し[2024年]
監修:沖波聡 : 特に申告事項無し[2024年]

ページ上部に戻る

眼窩内異常

戻る