- •Contents
- •Preface
- •Abbreviations
- •Introduction
- •Orbit and external eye
- •Extraocular muscles
- •Anterior segment
- •Posterior segment
- •Introduction
- •Ocular examination
- •The ‘red reflex’
- •Assessment of vision and visual acuity
- •Cover test for strabismus
- •Light reflex testing
- •Color vision testing
- •Assessment of stereoacuity
- •Ophthalmoscopy
- •Vision screening
- •Photoscreening
- •Autorefraction
- •Visual evoked potential
- •Strabismus
- •Comitant strabismus
- •Congenital esotropia
- •Accommodative esotropia
- •Congenital exotropia
- •Intermittent exotropia
- •Incomitant strabismus
- •Third cranial nerve palsy
- •Fourth nerve palsy
- •Sixth nerve palsy
- •Strabismus syndromes
- •Duane’s syndrome
- •Brown’s syndrome
- •Monocular elevation deficiency (MED)
- •Möbius syndrome
- •Introduction
- •Conjunctivitis
- •Bacterial conjunctivitis
- •Viral conjunctivitis
- •Herpes conjunctivitis
- •Giant papillary conjunctivitis
- •Allergic conjunctivitis
- •Vernal keratoconjunctivitis
- •Phlyctenular keratoconjunctivitis (phlyctenulosis)
- •Ophthalmia neonatorum
- •Introduction
- •Congenital corneal opacity
- •Embryology
- •Peters anomaly
- •Sclerocornea
- •Congenital dermoid
- •Birth trauma
- •Congenital hereditary endothelial dystrophy
- •Congenital hereditary stromal dystrophy
- •Posterior polymorphous membrane dystrophy
- •Metabolic diseases
- •Mucopolysaccharidosis
- •Hurler’s syndrome (MPS I-H)
- •Scheie’s syndrome (MPS I-S)
- •Hunter’s syndrome (MPS II)
- •Sly’s syndrome (MPS VII)
- •Mucolipidosis
- •Sialidosis (ML I)
- •I-Cell disease (ML II)
- •Pseudo-Hurler dystrophy (ML III)
- •Miscellaneous metabolic diseases
- •Fabry’s disease
- •Cystinosis
- •Tyrosinemia
- •Infectious diseases
- •Herpes simplex virus (HSV)
- •Congenital syphilis
- •Rubella
- •Introduction
- •Structural lens abnormalities
- •Aphakia
- •Spherophakia (microspherophakia)
- •Coloboma
- •Subluxation (ectopia lentis)
- •Lenticonus
- •Persistant fetal vasculature
- •Cataracts
- •Nuclear cataracts
- •Lamellar cataracts
- •Anterior polar cataracts
- •Posterior polar cataracts
- •Sutural cataracts
- •Anterior subcapsular cataracts
- •Posterior subcapsular cataracts
- •Cerulean (blue-dot) cataracts
- •Complete cataracts
- •Etiology of cataracts
- •Genetic and metabolic diseases
- •Trauma
- •Medication and toxicity
- •Maternal infection
- •Diagnosis of cataracts
- •Management/treatment of cataracts
- •Visual significance
- •Surgery
- •Aphakia
- •Pseudophakia
- •Amblyopia
- •Cataract prognosis
- •Introduction
- •Diagnosis of pediatric glaucoma
- •Ocular examination
- •Differential diagnosis of pediatric glaucoma
- •Primary infantile glaucoma
- •Juvenile open-angle glaucoma
- •Primary pediatric glaucoma associated with systemic disease
- •Lowe’s syndrome
- •Sturge–Weber syndrome
- •Neurofibromatosis
- •Axenfeld–Rieger syndrome
- •Aniridia
- •Peters anomaly
- •Secondary childhood glaucoma
- •Trauma
- •Neoplasia
- •Glaucoma following pediatric cataract surgery
- •Other causes of secondary glaucoma in children
- •Treatment of pediatric glaucoma
- •Drug treatment
- •Surgical management
- •Summary
- •Introduction
- •Coats’ disease
- •Leber’s congenital amaurosis
- •X-linked congenital stationary night blindness
- •Achromatopsia
- •Stargardt disease
- •Best’s disease
- •Persistent fetal vasculature
- •X-linked juvenile retinoschisis
- •Albinism
- •Retinal dystrophies with systemic disorders (ciliopathies)
- •Introduction
- •Common clinical features
- •Classification
- •Anterior uveitis
- •Juvenile idiopathic arthritis
- •Juvenile spondyloarthropathies
- •Sarcoidosis
- •Herpetic iridocyclitis
- •Intermediate uveitis
- •Posterior uveitis
- •Toxoplasmosis
- •Toxocariasis
- •Vogt–Koyanagi–Harada syndrome
- •Sympathetic ophthalmia
- •Masquerade syndromes
- •Retinoblastoma
- •Leukemia
- •Introduction
- •Optic nerve hypoplasia
- •Morning glory disc anomaly
- •Optic disc coloboma
- •Peripapillary staphyloma
- •Congenital tilted disc
- •Optic pit
- •Myelinated retinal nerve fibers
- •Papilledema
- •Pseudopapilledema
- •Optic disc drusen
- •Introduction
- •Dacryocele
- •Nasolacrimal duct obstruction
- •Lacrimal sac fistula
- •Decreased tear production
- •Dacryoadenitis
- •Introduction
- •Cryptophthalmos and ankyloblepharon
- •Coloboma of the eyelid
- •Blepharoptosis
- •Epicanthal folds and euryblepharon
- •Lagophthalmos
- •Lid retraction
- •Ectropion, entropion, and epiblepharon
- •Blepharospasm
- •Blepharitis
- •Hordeolum
- •Chalazion
- •Tumors of the eyelid
- •Preseptal and orbital cellulitis
- •Herpes simplex, molluscum contagiosum, and verruca vulgaris
- •Allergic conjunctivitis
- •Trauma
- •Summary
- •Introduction
- •Cystinosis
- •Marfan’s syndrome
- •Homocystinuria
- •Wilson’s disease
- •Fabry disease
- •Osteogenesis imperfecta
- •The mucopolysaccharidoses
- •Sickle cell disease
- •Albinism
- •Congenital rubella
- •Introduction
- •Genetics
- •Malignant potential
- •Formes frustes
- •Neurofibromatosis (von Recklinghausen’s syndrome)
- •Retinocerebellar hemangioblastomatosis (von Hippel–Lindau syndrome)
- •Racemose hemangiomatosis (Wyburn-Mason syndrome)
- •Encephalofacial cavernous hemangiomatosis (Sturge–Weber syndrome)
- •Oculoneurocutaneous cavernous hemangiomatosis
- •Organoid nevus syndrome
- •Introduction
- •Cortical visual impairment
- •Migraine headache
- •Spasmus nutans
- •Opsoclonus
- •Horner’s syndrome
- •Congenital ocular motor apraxia
- •Myasthenia gravis
- •Introduction
- •Eyelid and conjunctiva
- •Intraocular tumors
- •Orbital tumors
- •Diagnostic approaches
- •Eyelid and conjunctiva
- •Intraocular tumors
- •Orbital tumors
- •Therapeutic approaches
- •Eyelid and conjunctiva
- •Intraocular tumors
- •Orbital tumors
- •Eyelid tumors
- •Capillary hemangioma
- •Facial nevus flammeus
- •Kaposi’s sarcoma
- •Basal cell carcinoma
- •Melanocytic nevus
- •Neurofibroma
- •Neurilemoma (schwannoma)
- •Conjunctival tumors
- •Introduction
- •Choristomatous conjunctival tumors
- •Epithelial conjunctival tumors
- •Melanocytic conjunctival tumors
- •Vascular conjunctival tumors
- •Xanthomatous conjunctival tumors
- •Lymphoid/leukemic conjunctival tumors
- •Non-neoplastic lesions that simulate conjunctival tumors
- •Conclusions
- •Intraocular tumors
- •Retinoblastoma
- •Retinal capillary hemangioma
- •Retinal cavernous hemangioma
- •Retinal racemose hemangioma
- •Astrocytic hamartoma of the retina
- •Melanocytoma of the optic nerve
- •Intraocular medulloepithelioma
- •Choroidal hemangioma
- •Choroidal osteoma
- •Uveal nevus
- •Uveal melanoma
- •Congenital hypertrophy of retinal pigment epithelium
- •Leukemia
- •Orbital tumors
- •Dermoid cyst
- •Teratoma
- •Capillary hemangioma
- •Lymphangioma
- •Juvenile pilocytic astrocytoma
- •Rhabdomyosarcoma
- •Granulocytic sarcoma (‘chloroma’)
- •Lymphoma
- •Langerhan’s cell histiocytosis
- •Metastatic neuroblastoma
- •Introduction
- •Eyelid
- •Open globe
- •Ocular surface injury
- •Intraocular trauma
- •Iridodialysis
- •Cataract
- •Retina
- •Optic nerve injury
- •Orbital fracture
- •Other orbital injury
- •Child abuse
- •Shaking injury
- •Index
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Abbreviations |
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AAP American Academy of Pediatrics ACE angiotensin-converting enzyme AD autosomal dominant
ADHD attention deficit hyperactivity disorder AHP anomalous head position
AIDS acquired immune deficiency syndrome AKC atopic keratoconjun ctivitis
AOM acute otitis media
A-RS Axenfeld–Rieger syndrome AR autosomal recessive
AV arteriovenous
CA-MRSA community aquired methicillinresistant Staphylococcus aureus
CBS cystathionine beta synthase
CHED congenital hereditary endothelial dystrophy
CHRP congenital hypertrophy of the retinal pigment epithelium
CHSD congenital hereditary stromal dystrophy
CIN conjunctival intraepithelial neoplasia CNS central nervous system
CSNB congenital stationary night blindness CT computed tomography
CVI cortical visual impairment DNA deoxyribonucleic acid
DVD dissociated vertical deviation ECF-A eosinophil chemotactic factor of
anaphylaxis
EGA estimated gestational age
ELISA enzyme-linked immunosorbent assay EOG electro-oculography
ERG electroretinography ESRD end-stage renal disease
FNAB fine-needle aspiration biopsy GABA gamma aminobutyric acid GAG glycosaminoglycan
GPC giant papillary conjunctivitis GPI glycosylphosphatidyl inositol
HA-MRSA hospital acquired methicillinresistant Staphylococcus aureus
HBID hereditary benign intraepithelial dyskeratosis
HIV human immunodeficiency virus HSV herpes simplex virus
HZV herpes zoster virus
ICE iridocorneal endothelial (syndrome) ICP intracranial pressure
ICRB International Classification of Retinoblastoma
IGF-1 insulin-like growth factor 1 IOL intraocular lens
IOOA inferior oblique muscle overaction IOP intraocular pressure
IUGR intrauterine growth retardation JIA juvenile idiopathic arthritis
JRA juvenile rheumatoid arthritis LCA Leber’s congenital amaurosis MED monocular elevation deficiency
MIC minimum inhibitory concentration ML mucolipidosis
MMP matrix metalloproteinase MPS mucopolysaccharidosis MRI magnetic resonance imaging
MRNF myelinated retinal nerve fiber MRSA methicillin-resistant Staphylococcus
aureus
MSSA methicillin-sensitive Staphylococcus aureus
MTHFR methylene tetrahydrofolate reductase NF neurofibromatosis
NSAID nonsteroidal anti-inflammatory drug OCT optical coherence tomography
OI osteogenesis imperfecta
ONCCH oculoneurocutaneous cavernous hemangiomatosis
ONCS oculoneurocutaneous syndromes ONS organoid nevus syndrome
PAC perennial allergic conjunctivitis PAM primary acquired melanosis PCG primary congenital glaucoma PEVP pattern-evoked potential PFV persistant fetal vasculature
PHPV persistent hyperplastic primary vitreous PMMA polymethylmethacrylate
PPD purified protein derivative
PPMD posterior polymorphous membrane dystrophy
PSR proliferative retinopathy ROP retinopathy of prematurity RP retinitis pigmentosa
RPE retinal pigment epithelium SAC seasonal allergic conjunctivitis SLE systemic lupus erythematosus
SLRP small leucine-rich proteoglycan SW Sturge–Weber syndrome TORCHS toxoplasmosis, rubella,
cytomegalovirus, herpes simplex, and syphilis
TRIC trachoma inclusion conjunctivitis agent
10ABBREVIATIONS
TSC tuberous sclerosis complex UBM ultrasound biomicroscopy
VDRL Venereal disease Research Laboratory VECP visually evoked cortical potential VEGF vascular endothelial growth factor VEP visual evoked potential
VHL von Hippel–Lindau syndrome VKC vernal keratoconjunctivitis
VKH Vogt–Koyanagi–Harada syndrome VMA vanillylmandelic acid
VMD2 vitelliform macular dystrophy type 2 WHO World Health Organization
WM Wyburn-Mason syndrome XLRS X-linked juvenile retinoschisis
CHAPTER 1
11
Functional anatomy
Kammi Gunton, MD
•Introduction
•Orbit and external eye
•Extraocular muscles
•Anterior segment
•Posterior segment
12 CHAPTER 1 Functional anatomy
Introduction
This chapter reviews the basic anatomy of the eye, with emphasis on any differences in the pediatric eye. In addition, attention is directed to the functional relevance of the anatomy. The areas covered will include the orbit and external eye, extraocular muscles, anterior segment, and posterior segment.
Orbit and external eye
Each orbit is a pear-shaped bony cavity that tapers posteriorly to form the optic canal. Its volume is approximately 30 mL and it measures approximately 40 mm in an adult.1 The presence of the globe or an implant is required to continue the bony expansion of the orbit in childhood. The bony orbit is composed of four walls: the roof (frontal bone and lesser wing of
the sphenoid), the lateral wall (zygomatic bone and greater wing of the sphenoid), the floor (maxillary, zygomatic, and the palatine bones), and the medial wall (ethmoid, lacrimal, maxillary, and sphenoid bones) (1). The thinnest walls of the orbit are the lamina papyracea in the ethmoid bone and the posterior—medial portion of the maxillary bone in the floor. With blunt trauma, these bones easily break allowing for decompression of the globe rather than rupture.
The eyelids provide the external covering for the globe. They contain a dense, fibrous tissue called the tarsus that provides the rigidity of the lids. The orbicularis oculi muscle innervated by the facial nerve allows eyelid closure. The levator palpebrae supplied by cranial nerve III, along with Mueller’s muscle innervated by the sympathetic system, opens the eyelids. The levator palpebrae inserts on the anterior surface of the tarsal plate, making the eyelid crease in
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Lesser wing of sphenoid |
Optic canal |
Supraorbital notch |
Superciliary ridge |
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Greater wing of |
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Trochlear fossa |
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sphenoid |
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Posterior ethmoidal |
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Fossa for lacrimal |
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foramen |
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gland |
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Anterior ethmoidal |
Superior orbital |
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foramen |
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fissure |
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Body of the sphenoid |
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Frontal process of |
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bone |
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the frontal bone |
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Nasal bone |
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Orbital tubercle of |
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zygomatic bone |
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Frontal process of |
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Zygomaticofacial |
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maxilla |
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foramen |
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Zygomatic bone |
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Ethmoid bone |
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Inferior orbital |
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Lacrimal bone |
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fissure |
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Infraorbital groove |
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Anterior lacrimal crest |
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Zygomaticomaxillary |
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Fossa for lacrimal sac |
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Infraorbital foramen |
Orbital process of |
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suture |
Orbital plate of maxilla |
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palatine bone |
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1 Bony orbit.(Reproduced with permission from Catalano RA,Nelson LB (1994). Pediatric Ophthalmology: ATextAtlas. Appleton & Lange,Norwalk.)
Orbit and external eye 13
the upper lid. Congenital fibrosis of the levator palpebrae results in congenital ptosis. Meibomian glands are located in the eyelid and produce the oily layer in the tear film. Blockage of these openings results in formation of a chalazion. Finally, the orbital septum is connective tissue that forms a barrier between the anterior orbital structures such as the skin, and the deeper orbital structures. The septum attaches to the orbital rim, the levator aponeurosis, and the lower lid retractors. Penetration of the septum by infection differentiates preseptal cellulitis (anterior to the septum) from orbital cellulitis.
The lacrimal system is responsible for maintaining the moisture of the external eye. Tears play a vital role in the health and protection of the cornea and conjunctiva. The tear film consists of three layers: an outer lipid layer, a middle aqueous layer, and an inner mucus layer. The meibomian glands secrete the
oily layer as previous discussed. The lacrimal gland and the accessory lacrimal glands secrete the middle aqueous layer. The lacrimal gland is located in the superotemporal quadrant of the orbit in the lacrimal gland fossa of the frontal bone.2 The gland is divided into two parts by the aponeurosis of the levator palpebrae muscle: a larger orbital portion and a palpebral portion. The secretory ducts of the lacrimal gland empty into the superior cul-de-sac approximately 5 mm above the tarsal border. All ducts pass through the palpebral lobe. Damage to the palpebral portion will significantly impact on the secretory function. The facial nerve supplies the lacrimal gland. In addition, the accessory lacrimal glands of Krause and Wolfring are located within the superior cul-de-sac (2).
The drainage system for the tears begins with the eyelids pumping the tears towards the puncta which are small outpouchings located 6 mm from the medial angle of the eyelids
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lacrimal glands and |
Superior |
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secretory glands. |
Lacrimal gland |
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conjunctival fornix |
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Bulbar |
Glands of Krause |
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(4–35, Average 23) |
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conjunctiva |
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Glands of Wolfring |
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Glands of Manz |
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Crypts of Henle |
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Tarsal |
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conjunctiva |
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Meibomian glands (30) |
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Glands of Manz |
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Meibomian glands (25) |
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Crypts of Henle |
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Inferior |
Gland of Krause |
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conjunctival fornix |
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