Ординатура / Офтальмология / Английские материалы / Surgical Atlas of Orbital Diseases_Mallajosyula_2009

REFERENCES

1.Barrow D, Spector R, Braun I, et al. Classification and treatment of spontaneous carotidcavernous sinus fistulas. J Neurosurgery 1985; 62: 248-56.

2.Debrun GB, Lacour P, Fox AJ, et al. Traumatic carotid-cavernous fistulas: etiology, clinical presentation, diagnosis, treatment, results. Semin Intervent Radiol 1987; 4:242-8.

3.Higashida RT, Halbach VV, Tsai FY, et al. Interventional neurovascular treatment of traumatic carotid and vertebral artery lesions. Results in 234 cases. AJR 1986; 153: 577-82.

4.Halbach VV, Hieshima GB, Higashida RT, et al. Carotidcavernous fistulae; Indications for urgent treatment. AJR 1987; 149: 587-93.

5.de Keizer R. Carotid-cavernous and orbital arteriovenous fistulas: ocular features, diagnostic and hemodynamic considerations in relation to visual impairment and morbidity. Orbit 2003; 22: 121-42.

Some orbital lesions are known to have other ocular features and also associated with other lesions elsewhere in the body. It is always essential to have a knowledge of these conditions, so that we can investigate accordingly and detect the disease in a very early stage and sometimes in asymptomatic stage,and help the patient. If needed we should refer them to the respective specialists to prevent complications and ensure overall care of the patient.

In this chapter an attempt is made to list ocular and systemic associations of common orbital disorders presenting with proptosis. Some others like leukemia, lymphomas are dealt with separately elsewhere in this book.

Capillary Hemangiomas

(a)Ocular features: Strawberry nevus of the lids, proptosis, ptosis, astigmatism, amblyopia, optic atrophy and exposure keratitis.

(b)Systemic associations:

•Kasabach-Merritt syndrome (multiple capillary hemangiomas + thrombocytopenia)

•Mafucci syndrome (multiple capillary hemangiomas + multiple enchondromatosis)

•Phaces syndrome (posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta, eye abnormalities and sternum abnormalities).1,2

Neurofibromatosis

(a)Ocular features: Eyelid neurofibromas, prominent corneal nerves, glaucoma, Lisch nodules, congenital ectropion uveae, iris mamillations, choroidal naevi, retinal astrocytomas, optic nerve gliomas, optic nerve meningiomas and spheno-orbital encephalocele with pulsating proptosis.

(b)Systemic associations: Chiasmal tumors, hypopituitarism, spinal and gastrointestinal neurofibromas, pheochromocytoma, juvenile xanthogranuloma, capillary hemangioma, Wilms’ tumor, rhabdomyosarcoma, scoliosis, macrocephaly, aqueductal stenosis and seizures.3,4

Diagnostic Criteria for NF-1

NF 1 is diagnosed if 2 or more of the following group of seven conditions are met

•Six or more caif-au-lait spots > 5 mm in diameter in prepubescents or > 15 mm in postpubescents

•Two or more neurofibromas or one plexiform neurofibroma

•Axillary or inguinal freckling

•Optic N glioma

•Two or more Lisch’s nodules

•Sphenoid bone dysplasia or thinning of long bone cortex, with or without pseudoarthrosis

•First degree relative with NF-1.

[Source: NIH Consensus development conference. Arch Neurol. 1988;45:575-580]

Neurofibroma type 2 Features

Meningioma, glioma, schwannoma, vestibular schwannoma, posterior subcapsular lenticular opacities, hamartoma of retina and retinal pigment epithelium.

NF2: Criteria for Diagnosis

Presence of any one of the following features: Bilateral vestibular schwannoma

First degree relative with NF2 plus unilateral vestibular schwannoma < 30 years.

First degree relative with NF2 plus any 2 of the following:

•Meningioma

•Glioma

•Schwannoma

•Juvenile posterior subcapsular lenticular opacities

•Juvenile cortical cataract.

[Source: National Institute of Health Consensus Development Conference Neurofibromatosis: Conference Statement. Arch Neurol 1988,45:,575-78.]

Diagnostic evaluation for NF2: MRI for neurological and neuro-otologic evaluation is mandatory, since nearly 90% of NF2 exhibit bilateral vestibular schwannomas.10

Thyroid Orbitopathy (Grave’s disease)

(a)Ocular features: Lid retraction, chemosis, proptosis, superior limbic Keratoconjunctivitis, keratoconjunctivitis sicca, diplopia, optic neuropathy and choroidal folds.

(b)Systemic associations: Thyroid acropachy, plummer nails, tremors, fatigue, tachycardia, atrial fibrillation, pretibial myxoedema, alopecia, vitiligo, high output failure and myasthenia gravis.

Craniofacial Dysostosis

Crouzon Syndrome

(a)Ocular features: Proptosis, hypertelorism, V pattern exotropia, hypertropia, optic atrophy, exposure keratitis, megalocornea, glaucoma, colobomas, aniridia and blue sclera.

(b)Systemic associations: Wide cranium, midfacial hypoplasia, parrot-beak nose, frog facies,

Ocular and Systemic Associations of Proptosis 367

mandibular prognathism and acanthosis nigricans.5,6

Apert Syndrome

(a)Ocular features: Shallow orbits, proptosis, hypertelorism, exotropia, antimongoloid slant, exposure keratitis, optic atrophy, keratoconus, congenital glaucoma and ectopia lentis.

(b)Systemic associations: Oxycephaly, midfacial hypoplasia, low set ears, high arched palate, cleft palate, syndactyly, mental handicap and anomalies of the heart, lungs and kidney.5,6

Encephalocele

(a)Ocular features: Pulsatile proptosis, dystopia, medial canthal swelling, microphthalmos, colobomas and morning glory syndrome.

(b)Systemic associations: Neurofibromatosis, hypertelorism, broad nasal bridge and cleft palate.7

Wegener’s Granulomatosis

(a)Ocular features: Nasolacrimal duct obstruction, dacryocystitis, scleritis, peripheral ulcerative keratitis, nonspecific orbital inflammatory disease and occlusive retinal periarteritis.

(b)Systemic associations: Necrotizing granulomas of upper respiratory tract, necrotizing glomerulonephritis, perforation of nasal septum, saddle shaped nasal deformity, nasal-paranasal fistulae, vasulitis of the spleen and adrenals, polyneuritis and meningioencephalitis.7

Wyburn-Mason Syndrome

(a)Ocular features: Arterio-venous malformations of the conjunctiva, lids and retina, orbital A-V malformations causing proptosis and bruit, vitreous hemorrhage and neovascular glaucoma.

(b)Systemic associations: A-V malformations of the CNS causing headaches and seizures, cranial nerve palsies, motor and sensory deficits.7

Langerhans Cell Histiocytosis

(a)Ocular features: Proptosis, ptosis, periorbital swelling, localized pain.

(b)Systemic associations: Osteolytic lesions of skull, ribs and long bones, diabetic insipidus and soft tissue lesions of liver and spleen.8

368 Surgical Atlas of Orbital Diseases

Hurler’s Syndrome

(a)Ocular features: Corneal clouding, papilloedema, shallow orbits causing proptosis.

(b)Systemic associations: Stiff joints, coarse face, chest deformities, dwarfism, hepatosplenomegaly and deafness.9

Nonspecific Orbital Inflammation Syndrome

•Crohn's disease,

•Systemic lupus erythematosus

•Rheumatoid arthritis

•Myasthenia gravis

•Ankylosing spondylitis.

Sclerosing inflammation of the orbit

•Riedel's thyroiditis

•Mediastinal fibrosis

•Sclerosing cholangitis

•Fibrosis of parotid gland, lacrimal gland and lung.

Osteoma

•Gardner's syndrome (Familial polyposis of large bowel, plus osteoma of skull or jaw plus epidermal and subcutaneous cysts)

•Turcot Syndrome (Familial adenomatous polyposis plus CNS gliomas).

Fibrous dysplasia

McCune-Albright syndrome (ployostotic fibrous dysplasia + sexual precocity + cutaneous pigmentation).

Orbital Hamartoma (tuberous sclerosis)

Hemangioblastoma

Von HippelLindau's disease

•Retinal hemangioblastoma

•Cerebellar and spinal hemangioblastoma

•Renal cell carcinoma

•Pheochromocytoma

•Others: Pancreatic tumors, cystadenoma of epididymis.

REFERENCES

1.Haik BG, Karcioglu ZA, Gordon RA, Pechous BP. Capillary hemangioma. Surv Ophthalmol. 1994;38:399-426.

2.Kushner BJ. Hemangiomas. Arch Ophthalmol. 2000; 118:835-36.

3.Beauchamp GR. Neurofibromatosis type 1 in children. Trans Am Ophthalmol soc. 1995;93:445-72.

4.Listernick R, Charrow J, Greenwald MJ, et al. Natural history of optic pathway tumors in children with neurofibromatosis type 1. J Pediatr.1994;125:63-66.

5.Cohen MM. The child with multiple birth defects. 2nd ed.New york:Oxford 1997:178-96.

6.Gorlin RJ, Cohen MM, Levin LS. Syndromes of the head and Neck. 3rd ed. Newyork Oxford; 1990.

7.Kanski JJ. Clinical Ophthalmology:A systematic approach. 6th ed. Butterworth - Heinemann. 2007.

8.Huang F, Arceci R. The histiocytoses of infancy. Semin Perinatol 1999;23:319-31.

9.AAO. Pediatric Ophthalmology and strabismus. Section 6. Ocular findings in inborn errors of metabolism. AAO publication 2006.

10.Arun D Singh, Bertil F Damato,, Jacob Pe'er, A. Linn Murphee, Julian Perry: Clinical Ophthalmic oncology, first edition, 2007 Saunders Elsevier,Philadelphia.

A

3-D reconstruction of orbit 84 Adenoid cystic carcinoma 194

clinical features 194 imaging 195 management 196

pathology and pathogenesis 195 basaloid variant 195 comedocarcinoma variant 195 cribriform (glandular or swiss-

cheese) pattern 195 sclerosing variant 195 tubular (ductal) variant 195

prognosis 196 Angiosarcoma 76

Applied anatomy of orbit 3 cavernous sinus 8 extraocular muscles 9 globe 8

lacrimal system 13 lids 10

nerves of the orbit 14 optic nerve 14

parasympathetic innervation of the orbit 16

sensory innervation of the orbit 14 sympathetic innervation of the

orbit 16 orbital apex 6 orbital osteology 3 periorbita 6

Auscultation 51

B

Basal cell and squamous cell carcinoma 348

B-cell lymphoma 174 Benign tumors of orbit 103

capillary hemangioma 103

cavernous hemangioma 103 hemangiopericytoma 103 lymphangioma 103 meningiomas 104

Bone tumors of orbit 180 case-illustration 181

aneurysmal bone cyst 184 cholesterol granuloma 184 chondroma 183 chondrosarcoma 186 Ewing's sarcoma 186 fibrous dysplasia 181 giant cell lesions 184

Langerhans cell histiocytosis (lCH) 186

mesenchymal chondrosarcoma 186 myeloma 186

ossifying fibroma 182 osteoblastoma 182 osteogenic sarcoma 184

clinical presentation 162 clinico-pathological classification of

primary orbital bone disorders 180 osteoma 180

Bony lesions 62 Bony orbit 60

C

Capillary hemangioma 348 Carotid-cavernous fistula 39 Carotid-cavernous fistulae 356

direct CCF 360 indirect CCF 363

management of CCF 360 pathophysiology 357

clinical features 357 prognosis 363

radiological investigations 359

Cell cycle and the principles of antineoplastic therapy 347

Cephalocele 201

radiological finding 202 treatment 202

Choroidal melanomas 349 Classification of orbital tumors 102

primary 102 hemopoietic 102 lacrimal gland 102 mesenchymal 102 miscellaneous 102 neural 102

secondary 102

direct extension 102 Cystic lesions of the orbit 78

Cysts of the optic nerve sheath 203 Cytology smear 91

D

Dacryocele 205 Decision making 271

apical conal lesions 279 intraconal lesion 273

lesions of superior peripheral space 279 thyroid associated orbitopathy 285

Dermoid and epidermoid cysts 200 investigations 200

MRI 200 treatment 201

Developmental lesions of orbit 98 dermoid and epidermoids 99 fibrous dysplasia 99 hamartoma 99 meningioencephalocoeles 98 neurofibromatosis 98 sphenoid wing dysplasia 98

Diagnosis of orbital tumors 98 oculomotor paresis 98 optic neuropathy 98

pain 98

papillary abnormalities 98 proptosis 98

Duplex Doppler 56

mesenchymal soft tissue tumors 170 striated muscle tumors 170

rhabdomyoma 172 rhabdomyosarcoma 170

Microphthalmos with cyst 202 Mucocele 202

Müller's muscle 46

N

Neurofibroma 75

Neurosurgical approach to proptosis 309 Non-Hodgkin's lymphoma 147 Nonspecific orbital inflammatory

syndrome (NSOIS) 131

O

Ocular and systemic associations of proptosis 366

capillary hemangiomas 366 systemic associations 366 ocular features 366

craniofacial dysostosis 367 apert syndrome 367 crouzon syndrome 367 encephalocele 367 fibrous dysplasia 368 hemangioblastoma 368 Hurler's syndrome 368

Langerhans cell histiocytosis 367 nonspecific orbital inflammation

syndrome 368

orbital hamartoma (tuberous sclerosis) 368

osteoma 368

sclerosing inflammation of the orbit 368

Wegener's granulomatosis 367 Wyburn-Mason syndrome 367

diagnostic criteria for NF-1 366 neurofibroma type 2 367

criteria for diagnosis 367 diagnostic evaluation 367 features 367

neurofibromatosis 366 ocular features 366 systemic associations 366

thyroid orbitopathy 367 Ocular metastasis 352 Optic nerve glioma 353

Optic nerve meningioma 353 Orbital amyloidosis 129 Orbital diseases 97

classification 97 developmental 97 endocrine 97

inflammatory 97 miscellaneous 97 neoplastic 97 traumatic 97 vascular 97

Orbital exenteration 318 complications of exenteration 320 indications 318

management of the exenterated socket 320

myocutaneous flaps 320 patient preparation 318 prosthesis 320

skin flaps 320 skin grafting 320

spontaneous granulation 320 surgical procedure 318 types 319

anterior exenteration 319 lid sparing exenteration 319 radical exenteration 319 total exenteration 319

Orbital fractures 220 anatomy 220 examination 222 floor fractures 233

general operative considerations 229 antibiotics 230

decision repair or not repair 232 timing of surgery 231

imaging 226

implant materials 227

late and secondary fracture repair 239 lateral wall and zygomatico-maxillary fractures 238

Orbital infections 120 demographic profile 120 diagnosis 121

emergency department care 122 etiological causes 121

bacterial infections 121 fungal infections 121 parasitic infections 121 protozoal infections 121

imaging studies 121 risk factors 121

Orbital lymphoma 146 modified Rye's classification of

Hodgkin's lymphoma 147 classic HD 147

nodular lymphocyte-predominant HD 147

revised European American lymphoma classification (REAL classification) 146

leukemias and lymphomas of T-cell origin 146

WHO classification of NHL 146 B-cell neoplasms 146

T-cell neoplasms 146 Orbital lymphoma 352 Orbital prosthesis 327

assemble the prosthesis 331 casting 329

fabrications of ocular prosthesis 331 impression of the orbital defect 328 moulding 330

preparation of the patient 328 sculpting 329

types 327

adhesive retained prosthesis 327 magnetic retained prosthesis 327 partial prosthesis 327

spectacle mounted prosthesis 327 using the desired material 331

Orbital tumors 352

Orbital tumors of neurological origin 162 optic nerve glioma 162

optic nerve meningioma 163

orbital schwannoma (neurilemmoma) and neurofibroma 165

Orbital xanthogranuloma 134 Orbitotomies 288

approaches 289 general principles 288 lateral orbitotomy 290

Stallard-Wright lateral orbitotomy 290 swinging lower lid flap 289 transcarcuncular approach 291 transfrontal orbitotomy 291

complications 291 postoperative management 291

transnasal endoscopic approach and transantral approach 291

P

Parasitic cysts of orbit 207 investigations 208 treatment 208

Patterson's operation 305 Perception of color vision 41 Peripheral surgical space 91 Plaque radiotherapy 346 Pleomorphic adenoma 191

clinical features 191 imaging 192 management 192

pathology and pathogenesis 192 prognosis 192

Pleomorphic adenoma 76 Proptosis 28

axial proptosis 29

Index 371

down and in displacement of the globe 32

down and out proptosis 29 measurement of proptosis 35 upward displacement of globe 35

Pulsations of globe 37

R

Radiation therapy delivery methods 344 Reese-Berke's incision 273 Retinoblastoma 349

treatment 349 chemoreduction 350 cryotherapy 349 photocoagulation 349 subtenon (subconjunctival)

chemotherapy 350 surgery (enucleation) 350

Rhabdomyosarcoma 77, 352

Role of cytology in orbital lesions 85 fine needle aspiration/sampling

technique 85 intraoperative-operative diagnosis by

squash and imprint cytology 85 squash or imprint cytology 85

Rosai Dorfman disease 133

S

Sarcoidosis 130 Sebaceous carcinoma 348

Secondary and metastatic orbital tumors 244

malignant melanoma of eyelid 256 metastatic orbital tumors 263 orbital extension of conjunctival

tumors 258

malignant melanoma of the conjunctiva 259

squamous cell carcinoma of the conjunctiva 258

orbital extension of eyelid tumors 252 orbital extension of intracranial tumors

257

orbital extension of intraocular tumors 244

orbital extension of lacrimal sac tumors 250

orbital extension of medulloepithelioma 246

orbital extension of nasopharyngeal tumors 262

orbital extension of retinoblastoma 244

orbital extension of tumors of the nasal cavity and paranasal sinus 260

V

Val salva 51

Vascular anatomy of the orbit 17 arterial supply 17

outflow 19 paranasal sinuses 20 venous 19

Vascular lesions of orbit 151 malformations 151

cavernous hemangioma 152 lymphangioma 151

orbital varices 152

other congenital malformations 152 shunts 153

angiosarcoma 155 capillary hemangioma 154

carotid-cavernous fistula 153 hemangioblastoma 155 hemangioendothelioma 155 hemangiopericytoma 155 Kaposi's sarcoma 155

new growths 154 Vascular pulsations 39

Von Recklinghausen disease 75

W

Wegener's granulomatosis 132 Whitall's tubercle 12 Wyburn-Mason syndrome 153