Pediatrics / Strabismus

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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Jun 4, 2016 | Posted by in OPHTHALMOLOGY | Comments Off on Pediatrics / Strabismus

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