- •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
184 CHAPTER 13 Disorders of the lacrimal system
Dacryoadenitis
ETIOLOGY AND CLINICAL PRESENTATION
Dacryoadenitis is an inflammatory event affecting the lacrimal gland. It is usually an acute process, with sudden enlargement of the lacrimal gland. Often, there is overlying eyelid edema and erythema. The eyelid has a classic S-shaped appearance, owing to the inferior displacement of the lateral upper eyelid.
DIAGNOSIS
Acute dacryoadenitis is often infectious or inflammatory. Viral etiologies, such as mumps virus, herpes zoster virus, and Epstein–Barr virus17 are much more common than bacterial etiologies. Idiopathic orbital inflammation (or orbital pseudotumor) can present as acute lacrimal gland swelling, pain, and edema. In children, this condition is often bilateral, and will include constitutional symptoms such as fever, headache, and emesis.18 Neuroimaging can be useful in differentiating between entities. Lacrimal gland biopsy may be necessary to establish a certain diagnosis prior to treatment.
MANAGEMENT/TREATMENT
Viral dacryoadenitis is treated with comfort measures such as warm compresses and antiinflammatory medication. Idiopathic orbital inflammation is treated with oral steroids, and has been shown to be very sensitive to this treatment. In cases recalcitrant to steroid treatment, biopsy may be necessary to rule out other potential causes. A rapidly-developing malignancy, such as rhabdomyosarcoma, must also be considered should the treatment fail to obviate the symptoms.
CHAPTER 14
185
The eyelids
Srinivas Iyengar, MD
•Introduction
•Anophthalmia/ microphthalmia
•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
186 CHAPTER 14 The eyelids
Introduction
The eyelids are important accessory structures to the eye and can directly impact on vision. They are derived from surface ectoderm and fused together until the sixth month of gestation. With the exception of certain rare conditions such as ankyloblepharon, separation usually occurs before birth. Eyelid movement, or blinking, plays a key role in supporting the anterior ocular surface, and subsequent development of useful vision. In children, even benign appearing eyelid maldevelopment or pathology can lead to loss of vision by causing amblyopia.
Two different cranial nerves are responsible for eyelid opening and closing. The levator palpebrae superioris is responsible for eyelid opening and is innervated by the superior division of the third cranial nerve. The orbicularis oculi is served by the temporal branch of the facial nerve and closes the eyelid. Muller’s muscle, located posterior to the levator muscle, is sympathetically innervated and can affect eyelid position in cases of Horner’s syndrome or thyroid eye disease. The tarsal plates, made of firm connective tissue, provide structure to the eyelid margins and contain glands that contribute to the tear film. The upper eyelid tarsus is approximately 10 mm in height, while the lower eyelid tarsus is 5 mm in vertical height.
The eyelids are served by a delicate anastomotic arterial network from both the internal and external carotid circulations. Two clinically important arcades are the marginal and peripheral arcades of the upper eyelid. The marginal arcade is a superficial, horizontal arterial supply that lies just 2 mm above the eyelid margin. The peripheral arcade, while also horizontal, lies deeper, and just superior to the
234
234 Anophthalmia.
tarsal plate. It serves as a surgical landmark between the levator and Muller’s muscles. The eyelid crease is created by the attachment of strands of the underlying orbicularis oculi muscle to the skin and can show varying positions in cases of ptosis.1
Anophthalmia/ microphthalmia
Just as eyelid structure is important to ocular development, normal eye development is similarly critical to eyelid development. Anophthalmos is a rare condition where the optical vesicle fails to form and there is no globe in the orbit (234). More common is microphthalmos, which is the development of a small but disorganized globe. In both cases, eye socket growth is impaired and the eyelids may show shortened horizontal palpebral fissures. In many cases proper growth can be stimulated by the placement of expanding conformers. Surgical correction may involve expanding the orbital volume with dermis fat grafts or synthetic material.
Cryptophthalmos and ankyloblepharon
Cryptophthalmos is a rare condition where there is complete failure of the development of eyelid folds. This is nearly always accompanied by abnormal development of the anterior segment of the eye. Ankyloblepharon is failure of the eyelids to separate. Ankyloblepharon fili forme adnatum occurs when fine bands of tissue connect the upper and lower eyelids. The treatment involves surgical division and possible lid margin repair.
Coloboma of the eyelid
This cleftlike deformity may vary from a small indentation or notch of the free margin of the eyelid to a large defect involving almost the entire lid (235). If the gap is extensive, ulceration and corneal damage may result from exposure. Early surgical correction of the lid defect is recommended in such cases. Other deformities frequently associated with eyelid colobomas
Blepharoptosis 187
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globe; they often occur in a position |
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colobomas may also be associated with extensive |
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facial malformation, as in mandibulofacial |
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dysostosis (Franceschetti or Treacher–Collins |
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syndrome). Upper eyelid colobomas are |
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syndrome. |
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235 Eyelid coloboma. |
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Blepharoptosis
DEFINITION/OVERVIEW AND ETIOLOGY
In blepharoptosis, the upper eyelid droops below its normal level. Congenital ptosis is usually a result of a localized dystrophy of the levator muscle in which the striated muscle fibers are replaced with fibrous tissue. The condition may be unilateral or bilateral and can be familial, transmitted as an autosomal dominant (AD) trait.
CLINICAL PRESENTATION AND DIAGNOSIS
Parents often comment that the eye looks smaller because of the drooping eyelid. The lid crease is decreased or absent where the levator muscle would normally insert below the skin surface (236). Because the levator is replaced by fibrous tissue, the lid does not move downward fully in downgaze (lid lag). If the ptosis is severe, affected children often attempt to raise the lid by lifting their brow or adapting a chin-up head posture to maintain binocular vision. Marcus–Gunn jaw-winking ptosis accounts for 5% of ptosis in children. In this syndrome, an abnormal synkinesis exists between the 5th and 3rd cranial nerves; this causes the eyelid to elevate with movement of the jaw. The wink is produced by chewing or sucking and may be more noticeable than the ptosis itself.
Although ptosis in children is often an isolated finding, it may occur in association with other ocular or systemic disorders. Systemic disorders include myasthenia gravis, muscular dystrophy, and botulism. Ocular disorders include mechanical ptosis secondary to lid tumors, blepharophimosis syndrome, congenital fibrosis syndrome, combined levator/superior rectus maldevelopment, and
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236 Blepharoptosis with absent lid crease.
congenital or acquired third nerve palsy. A small degree of ptosis is seen in Horner’s syndrome. A complete ophthalmic and systemic examination is therefore important in the evaluation of a child with ptosis.
Amblyopia may occur in children with ptosis. The amblyopia may be secondary to the lid’s covering the visual axis (deprivation) or induced astigmatism (anisometropia). When amblyopia occurs, it should generally be treated before correcting the ptosis.
MANAGEMENT/TREATMENT
Treatment of ptosis in a child is indicated for elimination of an abnormal head posture, improvement in the visual field, prevention of amblyopia, and restoration of a normal eyelid appearance. The timing of surgery depends on the degree of ptosis, its cosmetic and functional severity, the presence or absence of compensatory posturing, the wishes of the parents, and the discretion of the surgeon. Surgical treatment is determined by the amount of levator function that is present. In cases of congenital ptosis, levator function is often
