Pediatrics / Strabismus

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5 Pediatrics / Strabismus

PEDIATRICS

Anatomy
Physiology
Orbital disorders
Craniofacial disorders
Lid disorders
Lacrimal disorders
Conjunctival disorders
Corneal disorders
Iris disorders
Lens disorders
Glaucoma
Uveitis
Metabolic disorders
Retinal disorders
Phakomatoses
Optic nerve disorders
STRABISMUS

Anatomy and physiology
Nystagmus
Ocular alignment
Special forms of strabismus
Strabismus surgery

Pediatrics

Anatomy

At birth, diameter of eye is 66% that of adult eye

Eye enlarges until 2 years of age, then further growth in puberty (Table 5-1)

Table 5-1 Changes in ocular measurements with age

Ocular dimensions Infant (mm) Adult (mm)
Axial length 17 24
Corneal diameter 9.5–10.5 12
Corneal radius of curvature 6.6–7.4 7.4–8.4
Scleral thickness Half of adult  

Infants have variable levels of astigmatism

Majority of children are hyperopic; increases up to 7 years of age, then diminishes

Eye color darkens during first few months of life

Dilator pupillae poorly developed at birth

Fovea matures during first few months of life

Myelinization of optic nerve completed shortly after birth

Physiology

Visual acuity levels (see Strabismus section)

Documented by visual evoked potentials in infancy and preferential looking tests in first months of life
Premature infants reach landmarks later

Decreased vision in infants and children

History

family history, complications in pregnancy, perinatal problems

Examination: visual response (assess each eye independently), pupillary reactions (paradoxical response suggests achromatopsia, congenital stationary night blindness (CSNB), optic nerve hypoplasia), ocular motility (strabismus, nystagmus, torticollis), cycloplegic refraction, fundus examination
Additional tests: optokinetic (OKN) response, forced preferential looking, electroretinogram (ERG), visual evoked response (VEP, VER), ultrasonography, imaging studies
Differential diagnosis of infant with poor vision and normal ocular structures: Leber’s congenital amaurosis, achromatopsia, blue cone monochromatism, CSNB, albinism, optic nerve hypoplasia, optic atrophy, congenital infection (TORCH syndrome), cortical visual impairment (due to extensive occipital lobe damage), delay in visual maturation

Orbital Disorders

Congenital Anomalies

Anophthalmos

Bilateral absence of eye due to failure of primary optic vesicle to form; extremely rare

Findings

hypoplastic orbits

Buphthalmos

Large eye due to increased intraocular pressure in congenital glaucoma

Cryptophthalmos

Failure of differentiation of lid and anterior eye structures

Partial or complete absence of the eyebrow, palpebral fissure, eyelashes, and conjunctiva

Attenuation of the levator, orbicularis, tarsus, and conjunctiva

Often associated with severe ocular defects

Microphthalmos

Small, disorganized eye

Disruption of ocular development occurs after budding of optic vesicle

Usually unilateral

Microphthalmos with Cyst

Due to failure of embryonic fissure to close

Usually blue mass of lower lid

Associated with congenital rubella, congenital toxoplasmosis, maternal vitamin A deficiency, maternal thalidomide ingestion, trisomy 13, trisomy 15, and chromosome 18 deletion

Nanophthalmos

Small eye with normal features

Normal-sized lens and thickened sclera

Associated with hyperopia and angle-closure glaucoma

Increased risk of choroidal effusion during intraocular surgery

Infections

Preseptal Cellulitis

Infection anterior to orbital septum; spares globe

Largest risk factor is recent skin trauma

Most common organism

Staphylococcus aureus

Findings

lid edema, erythema, and pain; may have fever; may progress to orbital cellulitis

Treatment

systemic antibiotics (IV in severe cases, children <5 years old, and failed oral treatment)

Orbital Cellulitis

Infection posterior to orbital septum; involves globe

Most common cause of proptosis in children

Usually secondary to sinusitis (ethmoid sinus is most common)

Associated with subperiosteal abscesses

Etiology

extension of infection from periorbital structures, sinusitis, dacryocystitis, dacryoadenitis, endophthalmitis, dental infections, intracranial infections, trauma or previous surgery, endogenous (bacteremia with septic embolization)

Organisms

S. aureus (most common organism in children), Streptococcus pneumoniae, fungi (Phycomycetes, most aggressive)

Findings

fever, decreased vision, positive RAPD, proptosis, restriction of ocular motility, pain on eye movement, periorbital swelling, chemosis, optic disc swelling

CT scan

diagnosis, localization, and involvement of adjacent structures

Treatment

IV antibiotics, surgical drainage

Complications

subperiosteal abscess, cavernous sinus thrombosis, or intracranial extension causing blindness or death

Benign Lesions

Dermoid Cyst (Choristoma)

Arises from dermal elements (neural crest origin)

Lined by keratinizing epithelium with dermal appendages

Most common orbital mass in childhood

Usually located in superotemporal quadrant near brow, often adjacent to bony suture

Often filled with keratin

Generally do not enlarge after 1 year of age

May induce bony erosion

Rupture can cause intense inflammatory reaction

CT scan

well circumscribed with bony molding

Treatment

complete excision, remove en bloc because contents may cause granulomatous inflammation

Epidermoid Cyst (Choristoma)

Arises from epidermal elements

Lined by epidermis only (no dermal appendages)

Usually filled with keratin

Rupture can lead to an acute inflammatory process

Lipodermoid

Solid tumor usually located beneath the conjunctiva over lateral surface of globe

May appear similar to prolapsed orbital fat, prolapsed lacrimal gland, or lymphoma

Usually no treatment is needed

Difficult to excise completely

Teratoma

Rare, cystic tumor arising from 2 or more germinal layers

Usually composed of ectoderm along with either endoderm or mesoderm (or both)

Can cause dramatic proptosis at birth

Rarely malignant

Capillary Hemangioma

Most common benign tumor of the orbit in children

Often manifests in the first few weeks of life and enlarges over the first 6 to 12 months, with complete regression by age 5–8 years in 80% of cases

Spontaneous involution over the next few years

Predilection for the superior nasal quadrant of the orbit and medial upper eyelid

Female > male (3 : 2)

Diffuse irregular mass of plump endothelial cells and small vascular channels

High-flow lesion

Findings

Strawberry nevus: skin involvement; appears as red, irregularly dimpled, elevated surface; blanches with direct pressure (port wine stain does not blanch)
Orbital location: can present with proptosis; bluish appearance of eyelids and conjunctiva

Pathology

numerous blood-filled channels lined by endothelium; little contribution from larger vessels or stroma; unencapsulated (Figure 5-1)

image

Figure 5-1 Capillary hemangioma demonstrating abnormal proliferation of blood vessels and endothelial cells.

(From Yanoff M, Fine BS: Ocular Pathology, 5th edn, St Louis, Mosby, 2002.)

CT / MRI scan

well-circumscribed lesion

Treatment

required if tumor causing ptosis or astigmatism with resultant anisometropia, strabismus, or amblyopia. Options include observation, intralesional steroid injection, systemic steroids, interferon, laser, radiation, or excision

Complications

Eyelid involvement may cause: ptosis with occlusion amblyopia, astigmatism and anisometropia with refractive amblyopia, strabismus with strabismic amblyopia
Kassabach-Merritt syndrome: consumptive coagulopathy with platelet trapping, resulting in thrombocytopenia and cardiac failure; acute hemorrhage is possible; mortality approximately 30%

High-output congestive heart failure can occur with multiple visceral capillary hemangiomas

Respiratory compromise with subglottic hemangiomas

Lymphangioma

Rare, lymphatic-filled choristoma

Often superonasal

Appears in first decade of life

Involves the eyelids, conjunctiva, and deeper orbital tissues

Lesion waxes and wanes, but does not involute

Findings

acute pain, proptosis (often increasing with upper respiratory infection), bluish hue to overlying lid, may hemorrhage into channels (chocolate cyst)

Pathology

lymph-filled vascular channels lined by endothelium; unencapsulated (Figure 5-2)

image

Figure 5-2 Lymphangioma demonstrating lymph-filled spaces, some with blood.

(From Yanoff M, Fine BS: Ocular Pathology, 5th edn, St Louis, Mosby, 2002.)

CT scan

may show layered blood in a lobular, cystic mass with infiltrative pattern

MRI

infiltrating pattern with indistinct margins

Treatment

observation (spontaneous regression common); rarely surgical evacuation because complete excision difficult and recurrence common

Complications

anisometropia, amblyopia, strabismus

Varix

Most common vascular abnormality

Dilations of preexisting venous channels

Findings

proptosis (increases with crying or straining; can occur when head is in a dependent position), orbital hemorrhage (especially after trauma), motility disturbance (usually restricted upgaze), disc swelling, optic atrophy

Radiograph

phlebolith present in 30% of cases

Treatment

surgery

Indications: cosmetic, severe proptosis, optic nerve compression, pain; surgery is often difficult because of intertwining of normal structures and direct communication with cavernous sinus

Fibrous Dysplasia

Tumor of fibrous connective tissue, cartilage, and bone

Progressive disease of childhood and young adulthood

Monostotic (in young adults) or polyostotic

Orbital involvement

usually monostotic; frontal (most common), sphenoid, and ethmoid bones; causes unilateral proptosis during first 2 decades of life

Polyostotic

multiple bones involved; can cause narrowing of optic canal and lacrimal drainage system

Albright’s syndrome

polyostotic fibrous dysplasia, short stature, premature closure of epiphysis, precocious puberty, and hyperpigmented macules

Findings: diplopia, proptosis, headaches, facial asymmetry, decreased visual acuity (compromised optic canal), hearing loss (compromised external ear canal)
Pathology: normal bone is replaced by immature woven bone and osteoid in a cellular fibrous matrix, stroma of the bone is highly vascularized, no osteoblasts present

Neurofibroma

Hamartoma

18% have neurofibromatosis (NF) type 1

Nearly all adults with NF 1 have neurofibromas

Plexiform neurofibroma

most commonly involves the upper lid; tortuous, fibrous cords infiltrate orbital tissues

Nodular

firm and rubbery consistency

Findings

often involves eyelid or causes proptosis, may cause glaucoma

Pathology

well-circumscribed, nonencapsulated proliferation of Schwann cells, perineural cells, and axons; stains with S-100 (specific for neural crest–derived structures) (Figure 5-3)

image

Figure 5-3 Neurofibroma demonstrating proliferation of Schwann cells.

(From Yanoff M, Fine BS: Ocular Pathology, 5th edn, St Louis, Mosby, 2002.)

Treatment

surgical excision

Optic Nerve Glioma (Grade I Astrocytoma)

Considered pilocytic astrocytoma of the juvenile type

Slow-growing hamartoma derived from interstitial cells, astroglia, and oligodendroglia

Usually does not metastasize

Usually appears during first decade of life

Associated with neurofibromatosis (25–50%)

Findings

unilateral proptosis, loss of vision, strabismus, and papilledema; may develop retinal vascular occlusions, optociliary shunt vessels; orbital tumors may cause chorioretinal folds, optic disc swelling, or atrophy; chiasmal tumors may cause pituitary or hypothalamic dysfunction, and nystagmus and head nodding from compression of 3rd ventricle

Pathology

circumscribed astrocytic tumor from neural crest tissue, glial hypercellularity, Rosenthal fibers, myxomatous, arachnoid hyperplasia; reactive meningothelial hyperplasia may occur, which can lead to a false diagnosis of meningioma

CT scan

fusiform enlargement of ON, ‘RR track’ appearance (pathognomonic), enlargement of optic foramen, bony erosion

Treatment

observation; consider surgery if tumor spreads posteriorly into the chiasm

Prognosis

in adults, tumor is malignant with death occurring in 6–12 months; better prognosis in children or in adults with neurofibromatosis

Idiopathic Orbital Inflammation (Orbital Pseudotumor [IOI])

Idiopathic inflammatory disorder of orbit

Commonly bilateral with episodic recurrence

Findings

decreased vision, diplopia, red eye, headache; acute, painful presentation in children; resembles orbital cellulitis

Other findings

constitutional symptoms in 50%

Treatment

systemic steroids

Graves’ Disease

(Occasionally occurs in adolescents; see Ch. 6, Orbit / Lids / Adnexa.)

Malignant Neoplasms

Rhabdomyosarcoma

Most common primary orbital malignancy of children

Most common soft tissue malignancy of childhood

Most common mesenchymal tumor of orbit

Malignant spindle cell tumor with loose myxomatous matrix

Average age at diagnosis is 8 years old (90% before age 16)

Cell of origin is an undifferentiated, pluripotent cell of the soft tissue; does not originate from the extraocular muscles

Unilateral; tends to involve superonasal portion of orbit

More common in males (5 : 3)

Aggressive local spread through orbital bones; hematogenous spread to lungs and cervical lymph nodes; most common location for metastasis is chest

Findings

rapidly progressive proptosis, reddish discoloration of eyelid; may have ptosis; later develop tortuous retinal veins, choroidal folds, and optic nerve edema

Types

Embryonal: most common (70%), usually occurs in children; tumor appears circumscribed, but often there is microscopic evidence of invasion of nearby structures

PATHOLOGY: elongated spindle cell with central hyperchromatic nucleus, eosinophilic granular cytoplasm; cells arranged in parallel palisading bands; obvious cross-striations are rare (Figure 5-4)

Botryoid: subtype of embryonal; can occur in anterior orbit, embryonal rhabomyosarcoma abutting a mucosal surface, multiple polypoid masses protruding from hollow viscera
Pleomorphic: least common, usually occurs in adults, rare involvement of orbit, most differentiated, best prognosis

PATHOLOGY: dense spindle cell arrangement with enlarged cells containing hyperchromatic nuclei, deeply eosinophilic cytoplasm; may see cross-striations on microscopic examination

Alveolar: poorest prognosis, usually found in inferior orbit, generally arises in extremities (adolescents)

PATHOLOGY: dense cellular proliferations of loosely cohesive small round cells separated by connective tissue septa; lack of cohesiveness may explain metastatic potential

image

Figure 5-4 A, Embryonal type demonstrating primitive rhabdomyoblasts. B, Cross-striations in cytoplasm of some cells.

(From Yanoff M, Fine BS: Ocular Pathology, 5th edn, St Louis, Mosby, 2002.)

CT scan

well-circumscribed orbital mass with possible extension into adjacent orbital bones or sinuses, bony destruction

A-scan ultrasound

orbital mass with medium internal reflectivity

Treatment

urgent biopsy, radiation (100% local control with 6000 cGy; 30% mortality due to metastases), chemotherapy for any microscopic metastases, surgical debulking

Prognosis

with chemotherapy and XRT, 3-year survival = 90%; cure rate close to 100% with localized orbital tumor; 60% if invasion of adjacent structures

Tumors arising in the orbit, bladder, and prostate: 77% disease-free survival at 2 years
Intrathoracic tumors: worst prognosis, 24% disease-free survival at 2 years

Neuroblastoma

Most common metastatic orbital tumor of childhood

Usually originates in the adrenal gland or sympathetic ganglion chain, also mediastinum or neck

40% develop orbital metastases

Average age of presentation with metastatic neuroblastoma to orbit is 2 years old

Spontaneous regression is rare

Findings

sudden proptosis and periorbital ecchymosis (raccoon eyes); may have ipsilateral Horner’s syndrome and opsoclonus (better prognosis)

Paraneoplastic syndrome associated with metastatic neuroblastoma: opsoclonus (saccadomania; random, rapid eye movements in all directions that disappear during sleep)

Pathology

sheets of indiscrete round cells with scant cytoplasm and high mitotic figures, areas of tumor necrosis; can have bony invasion; usually positive for neuronal markers (synaptophysin and neuron-specific enolase) (Figure 5-5)

image

Figure 5-5 Neuroblastoma.

(Case presented by Dr E. Torcynski at the meeting of the Eastern Ophthalmic Society, 1994. From Yanoff M, Fine BS: Ocular Pathology, 5th edn, St Louis, Mosby, 2002.)

CT scan

bony destruction

Treatment

chemotherapy and local XRT

Prognosis

poor if age of onset is over 1 year with metastases to bone; however, metastases to just liver, bone marrow, or spleen can be associated with a survival rate as high as 84%

Accounts for 15% of all childhood cancer deaths

In adults, neuroblastoma usually metastasizes to the uveal tract

Ewing’s Sarcoma

Second most frequent metastatic tumor to the orbit

Primary intermedullary malignancy of bone; originates in long bones of extremities or axial skeleton

Frequently metastasizes to bone and lungs

Occurs in 2nd-3rd decade of life

Findings

acute proptosis, hemorrhage, and inflammation from tumor necrosis

Treatment

chemotherapy

Granulocytic Sarcoma (‘Chloroma’)

Patients with myelogenous leukemia may present with orbital signs before hematologic evidence of leukemia

Findings

proptosis, may have subcutaneous periocular mass

Pathology

infiltration of involved tissues by leukemic cells

Treatment

radiotherapy, chemotherapy

Histiocytosis X

Group of disorders resulting from abnormal proliferation of histiocytes (Langerhans’ cells) that may involve orbit

Spectrum of disease, from isolated bone lesions with excellent prognosis (eosinophilic granuloma) to systemic spread with rapid death (Letterer-Siwe disease)

Children under age 2 with multifocal disease have a poor prognosis (50% survival rate)

Findings

most frequent orbital presentation is lytic defect of orbital roof causing progressive proptosis

Pathology

granulomatous histiocytic infiltrate, Birbeck granules (central, dense core, and thick outer shell), stains positively for S-100 and vimentin

Eosinophilic granuloma

Benign, local, solitary bone lesion

Most likely to involve superior temporal bony orbit in childhood or adolescence

CT SCAN AND X-RAY: sharply demarcated osteolytic lesions
TREATMENT: incision and curettage, intralesional steroids, or radiotherapy

Hand-Schüller-Christian disease

Triad of proptosis, lytic skull defects, and diabetes insipidus
More aggressive
Multifocal bony lesions; frequently involves orbit, usually superolaterally

TREATMENT: systemic steroids and chemotherapy
PATHOLOGY: good

Letterer-Siwe disease

Most severe and malignant
Progressive and fatal
Multisystem involvement
Rarely ocular / orbital involvement

PATHOLOGY: systemic steroids and chemotherapy

Burkitt’s Lymphoma

Primarily affects the maxilla in black children, with secondary invasion of the orbit

Related to Epstein-Barr virus infection

Findings

proptosis and bony destruction

Pathology

malignant B cells, ‘starry sky’ appearance due to histiocytes interspersed among uniform background of lymphocytes

Craniofacial Disorders

Structural development of head and face occur during 4th–8th week of gestation

Ocular motility disturbances occur in 75% of patients with craniofacial disorders

Craniofacial cleft syndromes

1 or more facial fissures fail to close during the 6th–7th week of gestation

Ocular complications

Corneal exposure from proptosis or lid defects
Refractive errors from lid anomalies
Ocular motility disturbances, most commonly exotropia
Papilledema and optic atrophy from increased intracranial pressure (ICP) or fibrous dysplasia involving optic canal
Ocular anomalies from embryogenesis

Syndromes

Treacher-Collins Syndrome (Mandibulofacial Dysostosis)

Hypoplasia of the midface

Associated with dental and ear abnormalities, microtia, micrognathia, hypoplastic malar bones, and low sideburns

Findings

lateral lid defects, absent lateral canthal tendon, absent medial lashes, antimongoloid slant, ectropion, poorly developed puncta and meibomian glands

Goldenhar’s Syndrome (Oculoauriculovertebral Dysplasia)

Abnormalities of the 1st and 2nd brachial arches

Findings

limbal dermoids and dermolipomas; may have lower lid colobomas

Other findings

hypoplastic facial bones, pretragal auricular appendages, vertebral abnormalities; may have fistulas between the mouth and ear

Associated with Duane’s syndrome

Hypertelorism

Findings

increased interpupillary distance due to increased distance between medial orbital walls

Associated with blepharophimosis, frontal meningoceles, encephaloceles, meningoencephaloceles, and fibrous dysplasia

Craniosynostoses

Premature closure of bony sutures; inhibits growth of the cranium perpendicular to the axis of the suture; growth can continue parallel to the suture

Findings

hypertelorism; proptosis can also occur

Plagiocephaly

premature closure of one-half of the coronal sutures

Skull develops normally on one side and is underdeveloped on the other side (resulting in a flattened face)

Other findings

midfacial hypoplasia, proptosis, telecanthus, V-pattern XT, oral and dental abnormalities, and respiratory problems

Crouzon’s Syndrome (Craniofacial Dysostosis) (autosomal dominant [AD] or Sporadic)

Absence of forward development of the cranium and midface

Multiple combinations of suture closure can occur

Findings

proptosis, V-pattern exotropia, nystagmus, hypertelorism; shallow orbits; optic atrophy (in 25–50%; due to narrowing of optic canal, kinking or stretching of optic nerve)

Other findings

mental retardation, hypoplasia of maxilla, parrot’s beak nose, high arched palate, external auditory canal atresia, anodonia

Apert’s Syndrome (AD)

Crouzon’s plus syndactyly

Anterior megalophthalmos

Associated with increased paternal age

Pfeiffer’s Syndrome (AD)

Findings

hypertelorism and pointy head; may have shallow orbits, syndactyly, and short digits

Carpenter’s Syndrome (autosomal recessive [AR])

Severe mental retardation

Involvement of sagittal, lambdoidal, and coronal sutures

Median Facial Cleft Syndrome

Findings

hypertelorism, exotropia

Other findings

medial cleft nose, lip, palate; widow’s peak; cranium bifidum occultum

Waardenburg’s Syndrome (AD)

Findings

lateral displacement of inner canthi / puncta, confluent eyebrows, heterochromia iridis, fundus hypopigmentation

Other findings

sensorineural deafness, white forelock

Hemifacial Microsomia

Findings

upper lid coloboma, strabismus

Other findings

facial asymmetry with microtia, macrostomia, mandibular anomalies, orbital dystopia, ear tags, vertebral anomalies

Hallermann-Streiff Syndrome

Sporadic

Findings

bilateral cataracts, glaucoma, microphakia, microcornea

Other findings

mandibular hypoplasia, beaked nose

Pierre Robin Sequence

Findings

retinal detachment, congenital glaucoma and / or cataracts, high myopia

Other findings

micrognathia, glossoptosis, cleft palate

Associated with Stickler’s syndrome

Fetal Alcohol Syndrome

Findings

short palpebral fissures, telecanthus, epicanthal folds, comitant strabismus, optic disc anomalies; may have high myopia, anterior segment anomalies

Other findings

thin vermilion border of upper lip, variable mental retardation, small birth weight and height, cardiovascular and skeletal abnormalities

Lid Disorders

Ablepharon

Absence of lids

Ankyloblepharon

Partial or complete fusion of lid margins; usually temporal, often bilateral

Often AD

Associated with craniofacial abnormalities

Can be secondary to thermal or chemical burns, inflammation, ocular cicatricial pemphigoid, Stevens-Johnson syndrome

Blepharophimosis

Horizontally and vertically shortened palpebral fissures with poor levator function

Absent lid crease

May be part of autosomal dominant syndrome (chromosome 3q) with ptosis, telecanthus, epicanthus inversus, lower lid ectropion, hypoplasia of nasal bridge and superior orbital rim, anteverted ears, hypertelorism

Treatment

surgical correction requires multiple procedures

Coloboma

Embryologic cleft involving lid margin; unilateral or bilateral; partial or full thickness

Ranges from notch to absence of entire lid

Upper lid

usually medial third, not associated with systemic abnormalities, usually full thickness

Lower lid

usually lateral third, associated with other abnormalities (Treacher-Collins, Goldenhar’s syndromes), usually partial thickness

May have exposure keratitis

Treatment

method of surgical repair depends on size of defect (see section on Lid avulsion)

Congenital Blepharoptosis

Droopy eyelid; 75% unilateral; nonhereditary

Rarely causes amblyopia

Associated with blepharophimosis syndrome (AD)

Myogenic is most common

Etiology

Myogenic:

Dysgenesis of levator muscle
Fibroadipose tissue in muscle belly
Poor levator function, loss of lid crease, eyelid lag, sometimes lagophthalmos

Aponeurotic:

Rare, possibly due to birth trauma
Good eyelid excursion, high or indistinct lid crease

Neurogenic:

Congenital CN 3 palsy
Marcus Gunn jaw-winking (aberrant connections between motor division of CN 5 and levator muscle; jaw movements cause elevation of ptotic lid)

Congenital Horner’s Syndrome

Ptosis, miosis, anhydrosis

May cause amblyopia

Treatment

surgical repair (levator resection), severe cases require frontalis sling

Congenital Ectropion

Eversion of eyelid margin due to vertical shortening of anterior lamella

Etiology

inclusion conjunctivitis, anterior lamella inflammation, Down’s syndrome

Associated with blepharophimosis syndrome

Treatment

usually not required; otherwise, treat as the cicatricial form

Congenital Entropion

Inversion of eyelid margin

Etiology

lid retractor dysgenesis, tarsal plate defects, vertical shortening of posterior lamella

Usually involves lower eyelid

Treatment

may require surgical repair

Congenital Tarsal Kink

Upper eyelid bent back and open with complete fold of tarsal plate

May cause corneal abrasion and exposure keratopathy

Distichiasis

Partial or complete accessory row of eyelashes growing from or posterior to meibomian orifices

Due to improper differentiation of pilosebaceous units

Usually well tolerated, but trichiasis may develop

Treatment

lubrication, cryoepilation, surgical epilation

Epiblepharon

Pretarsal skin and orbicularis override the lid margin, causing a horizontal fold of tissue to push cilia vertically; no entropion

Most common among Asians

Usually occurs in lower lid and resolves spontaneously

Rarely requires surgery (excision of skin and muscle for significant trichiasis)

Epicanthus

Medial canthal vertical skin folds

Due to immature facial bones or redundant skin

Usually bilateral

Produces pseudoesotropia

Types

Epicanthus tarsalis:

Fold most prominent in upper eyelid
Commonly associated with Asian eyelid

Epicanthus inversus:

Fold most prominent in lower eyelid
Associated with blepharophimosis syndrome

Epicanthus palpebralis:

Fold is equally distributed in upper and lower eyelids

Epicanthus supraciliaris:

Fold arises from eyebrow and extends to lacrimal sac

Euryblepharon

Horizontal widening of palpebral fissure due to inferior insertion of lateral canthal tendon

Associated with ectropion of lateral third of lid

Poor lid closure with exposure keratitis

Treatment

lubrication, may require surgical repair with resection of excess lid, lateral canthal tendon repositioning, and vertical eyelid lengthening

Microblepharon

Vertical shortening of lids

May have exposure keratitis

Telecanthus

Widened intercanthal distance due to long medial canthal tendons

Associated with fetal alcohol, Waardenburg’s, and blepharophimosis syndromes

Treatment

surgery with transnasal wiring

Lacrimal Disorders

Developmental Anomalies

Atresia of lacrimal puncta

ranges from thin membrane over punctal site to atresia of canaliculus

Supernumerary puncta

no treatment needed

Lacrimal fistula

usually located inferonasal to the medial canthus

Dacryocystocele

Cystic swelling of lacrimal sac accompanies obstruction of lacrimal drainage system above and below the sac

Presents at birth as bluish swelling inferior and nasal to medial canthus

Infection (dacryocystitis) develops if condition does not resolve spontaneously

Common organisms: Haemophilus influenzae, S. pneumoniae, Staphylococcus, Klebsiella, Pseudomonas

Treatment

digital massage, lacrimal probing, or surgical decompression (for dacryocystitis)

Nasolacrimal Duct Obstruction (NLDO)

Up to 5% of infants have obstruction of the NLD, usually due to membrane covering valve of Hasner

Most open spontaneously within 4–6 weeks of birth; image are bilateral

Findings

tearing, discharge; may develop dacryocystitis or conjunctivitis

Digital pressure over lacrimal sac producing mucoid reflux indicates obstruction of nasolacrimal duct

May have a dacryocystocele (dilated lacrimal sac), or amniotic fluid or mucus trapped in tear sac

Treatment

lacrimal sac massage, topical antibiotic, probing and irrigation by 13 months of age (95% cure rate); consider turbinate infracture and silicone intubation if probing unsuccessful; dacryocystorhinostomy (DCR) after multiple failures

Conjunctival Disorders

Ocular Melanocytosis

Unilateral excessive pigment in uvea, sclera, and episclera

Increase in number of normal melanocytes

More common among Caucasians

Findings

slate grey appearance of sclera, darkened appearance of fundus

When associated with pigmentation of eyelid skin, called nevus of Ota (congenital oculodermal melanocytosis; more common among African Americans and Asians; malignant potential only in Caucasians)

Other findings

heterochromia iridis due to diffuse nevus of uvea; may develop glaucoma (due to melanocytes in trabecular meshwork), rarely uveal, orbital, or meningeal melanoma

Pathology

spindle-shaped pigment cells in subepithelial tissue

Conjunctivitis

Ophthalmia Neonatorum

Conjunctivitis within first month of life

Papillary conjunctivitis (no follicular reaction in neonate due to immaturity of immune system)

Etiology

Chemical: caused by silver nitrate 1% solution (Crede’s prophylaxis); occurs in first 24 hours, lasts 24 to 36 hours (therefore, prophylaxis is now with erythromycin or tetracycline ointment)

FINDINGS: usually bilateral, bulbar injection with clear watery discharge
No treatment necessary

Neisseria gonorrheae: days 1–2; can occur earlier with premature rupture of membranes

FINDINGS: severe purulent discharge, chemosis, eyelid edema; can be hemorrhagic; may develop corneal ulceration or perforation
DIAGNOSIS: Gram’s stain (Gram-negative intracellular diploccocci)
TREATMENT: IV ceftriaxone × 7 days; bacitracin ointment topically
High incidence of Chlamydia coinfection; therefore, also use oral erythromycin syrup and treat mother and sexual partners

Other bacteria: days 4–5; Staphylococci, Streptococci, Haemophilus, Enterococci

TREATMENT: bacitracin, erythromycin, or gentamicin ointment; fortified antibiotics for Pseudomonas

HSV: days 5–14; type 2 in 70%

FINDINGS: serous discharge, conjunctival injection, keratitis; may have vesicular lid lesions
Can have systemic herpetic infection
DIAGNOSIS: conjunctival scrapings with multinucleated giant cells; cultures take 5–7 days
TREATMENT: Zirgan 5×/d or Viroptic q2h × 1 week; acyclovir 10 mg/kg IV tid × 10 days

Chlamydia (neonatal inclusion conjunctivitis): days 5–14; most common infectious cause of neonatal conjunctivitis

FINDINGS: acute, mucopurulent, papillary (no follicles until 3 months of age); may have pseudomembranes
Associated with pneumonitis, otitis, nasopharyngitis, gastritis
DIAGNOSIS: intracytoplasmic inclusions on Giemsa stain
TREATMENT: topical erythromycin, oral erythromycin syrup × 2–3 weeks (125 mg/kg/day qid) to prevent pneumonitis (onset 3–13 weeks later); treat mother and sexual partners with doxycycline 100 mg bid × 1 week (do not use in nursing mothers)
Rule out concomitant Gonococcus infection

DDx

trauma, foreign body, corneal abrasion, congenital glaucoma, NLDO, dacryocystitis

Prophylaxis

tetracycline 1% or erythromycin 0.5% ointment at birth

Other Infections

Pediatric conjunctivitis is usually bacterial (50–80%) vs. adult infectious conjunctivitis, which is usually viral

Age dependent; more common in younger children (<3 years old)

Organisms

H. influenzae (50–65%), S. pneumoniae (20-30%), Moraxella catarrhalis (10%); rarely Streptococcus pyogenes or S. aureus

Findings

purulent discharge (80%), red eye (50%)

Other findings

otitis media (30% in <3 year olds; H. influenzae), preseptal cellulitis (>3 year olds with sinusitis, fever, and elevated WBCs; S. pneumoniae)

Diagnosis

culture refractory cases and neonates

Treatment

topical antibiotics; oral antibiotics (cefixime) for extraocular involvement (otitis media, sinusitis)

Vernal Keratoconjunctivitis

Form of seasonal (warm months), allergic conjunctivitis

Male > female (2 : 1); onset by age 10 years, lasts 2–10 years, usually resolves by puberty

Associated with atopic dermatitis (75%) or family history of atopy (66%)

Limbal vernal

more common among African Americans

Symptoms

intense itching, photophobia, pain

Findings

large upper tarsal papillae (cobblestones), minimal conjunctival hyperemia, Horner-Trantas’ dots (elevated white accumulations of eosinophils at limbus), limbal follicles (gelatinous nodules at limbus), copious ropy mucus; may have pseudomembrane, keratitis, micropannus, shield ulcer (central oval epithelial defect with white fibrin coating); high levels of histamine and IgE in tears

Pathology

chronic papillary hypertrophy, epithelial hypertrophy then atrophy, conjunctiva contains many mast cells, eosinophils, and basophils

Treatment

topical allergy medication and steroids; consider topical ciclosporin (cyclosporine)

Ligneous

Rare, bilateral, pseudomembranous conjunctivitis in children; commonly young girls

Acute onset, chronic course

Etiology

appears to be exaggerated response to tissue injury following infection, surgery, or trauma

Findings

unilateral or bilateral highly vascularized, raised, friable lesion; with continued inflammation, a white, thickened avascular woody (ligneous) mass appears above the neovascular membrane, usually on palpebral conjunctiva; easily removed but bleeds; may recur

Process affects all mucous membranes (mouth, vagina, etc)

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