Orbital Diseases

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Disorders of the Orbit （ Orbital diseases ） 　　眼眶疾病

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Orbital anatomy 眼眶解剖

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1. Bony orbit 骨眶  Each person have two orbits in the frontead  Each orbit seven bones: frontal zygomatic (malar) maxillary, palatine lacrimal, ethmoid sphenoid

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1. Bony orbit Each orbit: four walls  Roof of the orbit  Lateral wall  Medial wall  Floor of the orbit Orbital apex

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2. Intraorbital contents 眶内容  globe and optic nerve  extraocular muscles  vessels and nerves  lacrimal gland  fat and fascia

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 orbital septum, fascia and periorbita

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3. Intraorbital spaces 眶内间隙  Subperiorbital surgical space  extraconal surgical space  intraconal surgical space  episcleral surgical space

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Orbital diseases 　　眼眶疾病

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Introduction

 Orbital disorders are uncommon but their diagnosis and treatment are challenging  The incidence and prevalence of orbital diseases vary with geographic location ， age, gender and race.  Orbital disorders have close relations with brain, nose and sinuses , endocrine, et al.

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Classifications

 Congenital malformations 先天性疾病 and genetic disorders 遗传性疾病  Inflammations 炎症  orbital tumors 肿瘤  orbital vascular malformations 血管畸形  orbital trauma 外伤  thyroid associated ophthalmopathy 甲状腺相关眼病

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     

Symptoms and Signs

Clinical manifestations 临床表现 proptosis, exophthalmos 眼球突出 enophthalmos 眼球凹陷 pain 疼痛 Diplopia and movement disorder 复视 Palpation (mass ) 触诊 Pulsation without or with bruit 搏动与杂音 Periorbital changes 眶周改变

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Diagnosis

Diagnosis of orbital disorder includes:  Detailed histroy 病史 onset, course, and duration of symptoms signs, prior disease, systemic disease, injury, family history, therapy  physical examination 体格检查  Imaging examination 影像检查  Laboratory examination 实验室检查  pathology exam 病理检查

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Treatment

1. Medical treatment 药物治疗 2. Surgical treatment 手术治疗 3. Radiotherapy

放射治疗

4. Chemotherapy

化

疗

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First clinical college of ZZU

一、 Orbital inflammations 1. orbital cellulitis 眶蜂窝织炎 2. orbital inflammatory pseudotumor 眼眶炎性假瘤

3. thyroid associated ophthalmopathy （ TAO ） 甲状腺相关眼病

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一、 Orbital inflammations 1. orbital cellulitis 眶蜂窝织炎

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orbital cellulitis

【 Etiology 】 bacterial infection in intraorbital and periorbital soft tissues  most common in baby, children teenage  infectious sources ： from sinusitis, periorbititis, skin infection orbital injury and post-operative distant focus: otitis, pneumonia metastatic disorders: septicemia, influenza

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orbitall cellulitis

Two types • Preseptal cellulitis infection of eyelid and periorbital tissues anterior to orbital septum • Orbital cellulitis acute infection of orbital soft tissues posterior to orbital septum

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orbital cellulitis

【 Clinical findings 】      

palpebral redness and swelling eyelid tightness and solid, pain conjunctival congestion conjunctival edema (chemosis) proptosis restriction of eye movement and diplopia  Decreased vision and pupillary abnormality

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orbital cellulitis

【 Imaging exam 】 CT can show the extent and size of disorder involvemen

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Imaging exam • B-scan can find the abscess

orbital cellulitis

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orbital cellulitis

【 Treatment 】  antibiotics: high dose iv broad-spectrum  corticosteroid: in some cases  surgical drainage: if abscess

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orbital cellulitis

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一、 Orbital inflammations 2. orbital inflammatory pseudotumor 眼眶炎性假瘤

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【 Etiology 】

pseudotumor remains unknown

 Conception: idiopathic tumor-like inflammation in orbital tissure  involved tissues form inflammatory mass  it looks just like a tumor but it’s not neoplasm so we call it pseudotumor

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【 histology 】

pseudotumor

inflammatory cells

diffuse or localized infiltration and fibrosis in orbital tissue 

lymphocytes

 fibroblast  plasma  histocyte

pseudotumor

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【 clinical types 】 according to involved tissues and locations  dacryoadenitis  myositis

泪腺炎 眼外肌炎

 periscleritis 巩膜周围炎  perioptic nerve inflammation 视神经周围炎 

superior orbital fissure and cavernous sinus ( Tolosa-Hunt syndrome or painful ophthalmoplegia )

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myositis 眼 外 肌 炎

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dacryoadenitis 泪腺炎型 perioptic nerve inflammation and myositis 视神经周围炎 和眼外肌炎

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pseudotumor

【 Clinical findings 】  Acute 、 subacute onset with orbital pain  proptosis  eyelid erythema and swelling  Conjunctival congestion and chemosis  restricted eye movement and diplopia  impaired vision if optic nerve involved  Inflammatory mass may be touched

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pseudotumor

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pseudotumor

【 Clinical features 】  Usually, unilateral or bilateral  onset ： acute, subacute, chronical  recurrent

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【 Imaging exam 】 CT can show • diffuse enlargement of lacrimal gland • thickened muscles • proptosis

pseudotumor

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【 Imaging exam 】 • B-scan low echo mass with much blood flow

pseudotumor

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pseudotumor

【 differential diagnosis 】 inflammation from malignant tumor by biopsy • frozen-section • permanent-section

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pseudotumor

【 Treatment 】  medical treatment corticosteroid broad-spectrum antibiotics cyclophosphamide  radiotherapy 20~25 Gery

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一、 Orbital inflammations 3. thyroid associated ophthalmopathy （ TAO ） 甲状腺相关眼病

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TAO

【 Etiology 】 Remain unknown • Autoimmune disease –chronic inflammation • most common age: between 25~50y • thyroid functions hyperthyroid hypothyroid euthyroid

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TAO

【 histology 】 diffuse infiltration of inflammatory cells, such as lymphocytes, plasma, fibroblast, histocyte in extraocular muscles, orbital fat, lacrimal gland, perisclera, perioptic nerve

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【 clinical findings 】 • Eyelid retraction and eyelid lag 90% • Proptosis 60% • Eyelid redness and edema • Conjunctival congestion and edema

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【 Clinical findings 】 • Restricted muscle motility with strabismus and diplopia in 40%  infammation, enlargement and fibrosis of muscle

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【 Imaging exam 】 CT Scan • Enlargement of multiple extraocular muscles • lacrimal gland swelling • Enlarged retrobulbar fat space • proptosis

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TAO

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【 Complications 】 Corneal exposure corneal ulceration Pannus compressive optic neuropathy

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【 diagnosis 】 according to three features and thyroid function • eyelid retraction and lag • proptosis • enlargement of multiple extraocular muscles

TAO

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TAO

【 Treatment 】  corneal protection: eye solution or ointment moisture chambers or patching  corticosteroid dose 80~100mg , daily  radiotherapy: 15~20 Gery  surgery : if terrible proptosis, corneal ulcer suture eyelid, orbital decompression

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First clinical college of ZZU

First clinical college of ZZU

First clinical college of ZZU

First clinical college of ZZU

二、 Orbital tumors 1. Dermoid and epidermoid 皮样囊肿和表皮样囊肿 2. Cavernous hemangioma 海绵状血管瘤 3. Rhabdomyosarcoma 横纹肌肉瘤

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二、 Orbital tumors

1. Dermoid and epidermoid 皮样囊肿和表皮样囊肿

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Dermoid and epidermoid

【 Etiology 】 developmental malformation • benign choristoma in the orbit from surface ectoderm not true neoplasms • Onset ages: from born to adult

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【 Etiology 】  forming: dermoid element left in bone suture in the course of embryonic development  usually located in superior temporal quadrant or superior rim of the orbit

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【 pathological classifications 】 • dermoid cyst: keratinizing epidermis with dermal appenedages, hair follicles, sebaceous glands in the cyst wall keratin, hair, oily fluid, teeth in the cyst • epidermic cyst: only epidermis without dermal appendages

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【 symptoms and signs 】 • proptosis -- slow growth • high orbital pressure • high retrobulbar resistance

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【 Imaging exam 】 CT mass characters:  clear border  thick wall  low density area  bony defect shape in round, or irr

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【 Imaging exam 】 B-scan and doppler:  Cystic mass  no blood flood within mass

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【 Imaging exam 】 MRI  Water T1WI low, T2WI high signal  Lipid-like both T1WI, T2WI high signals

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First clinical college of ZZU

【 Treatment 】  surgical extraction: remove the cystic wall, thoroughly very important any epidermis left -- recurrent

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二、 Orbital tumor 2. Cavernous hemangioma 海绵状血管瘤

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Cavernous hemangioma

【 Etiology 】 unknown the most common benign, well-encapsulated orbital neoplasm in adult • its section: spongy • middle-aged women and men commonly afflicted

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Cavernous hemangioma

【 Clinical findings 】  proptosis: slowly progressive 【 Complication 】  if tumor press the globe and optic nerve retinal striate, hyperopia increased IOP, blured vision

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【 Imaging exam 】 tumors mostly in muscle cone CT characters: • high density mass • round shape • clear border

Cavernous hemangioma

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【 Imaging exam 】 tumors mostly in the space of muscle cone • B- scan: clear border, high uniform echoes • CDFI: no blood flow within the mass

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【 Treatment 】

 surgical extraction usually  anterior orbitotomy  lateral orbitotomy

Cavernous hemangioma

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二、 Orbital tumor

3. Rhabdomyosarcoma 横纹肌肉瘤

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Rhabdomyosarcoma

【 Etiology 】  unknown

 most common primary malignant orbit tumor in childhood  onset age: < 7~8 years  characteristic: rapid growth

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Rhabdomyosarcoma

【 typical clinical findings 】 • unilateral proptosis: sudden onset, rapid evolution • eyelid swelling and rednees, chemosis • pain and tear • palpation: mass in orbit, or eyelid • other exam: regional lymph nodes

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Rhabdomyosarcoma

【 Imaging exam 】 • CT: high density mass in orbit with and without bone destruction • CDFI: low echo mass rich blood flow

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First clinical college of ZZU

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Rhabdomyosarcoma

【 pathology exam 】 • fast frozen section • light microscope section • electron microscope

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Rhabdomyosarcoma

【 Treatment 】 before 1965 exenteration  Surgery: local extraction+radiotherapy  radiotherapy: 4500~6000cGy  chemotherapy:  local extraction + radiotherapy + cheth 5 year survive rate over 90%

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First clinical college of ZZU

三、 Orbital Trauma  Penetrating orbital injury and intraorbital foreign body  Orbital fractures  Orbital hemorrhage  Traumatic optic neuropathy

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intraorbital foreign body

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intraorbital foreign body

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Blowout fracture

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Orbital Hemorrhage

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Traumatic Optic Neuropathy

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 the classifications of orbital diseases ?  the clinic findings of the thyroid related orbitopathy ?  the common orbital tumors ?  the common orbital trauma ?