Ординатура / Офтальмология / Английские материалы / Oxford American Handbook of Ophthalmology_Tsai, Denniston, Murray_2011
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616 CHAPTER 18 Pediatric ophthalmology
Table 18.13 Orbital tumors of childhood (selected)
Congenital |
Examples |
Choristoma |
Dermoid cysts |
Acquired |
|
Optic nerve |
Glioma |
Vascular |
Capillary hemangioma, lymphangioma |
Infiltrative |
Myeloid leukemia, histiocytosis |
Other |
Rhabdomyosarcoma, teratoma |
Metastases |
Neuroblastoma, nephroblastoma, Ewing’s sarcoma |
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Abnormal eye size
Abnormalities of globe size usually result from abnormalities of development, although it may arise secondary to ocular disease (e.g., buphthalmos in glaucoma) (Table 18.14). While severe forms may be obvious from simple observation, milder isolated aberrations of size may only be evident as an axial refractive error.
Table 18.14 Abnormal eye size: causes and key features
Abnormally large eye
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Axial myopia |
Mild (physiological) to severe and progressive |
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(pathological) ilength; ± other ocular abnormalities |
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Buphthalmos |
Diffusely large eye (with megalocornea) associated with |
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glaucoma |
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Megalophthalmos |
Diffusely large eye (with megalocornea) without |
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glaucoma; ± other ocular abnormalities |
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Pseudo-large eye |
Consider proptosis or abnormally small contralateral eye |
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Abnormally small eye |
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Microphthalmos |
Diffusely small eye (axial length 2 SD < normal) ± ocular |
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or systemic anomalies |
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Nanophthalmos |
Small eye with microcornea, normal-sized lens, and |
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abnormally thick sclera |
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Phthisis bulbi |
Acquired shrinkage of the eye due to chronic ocular |
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disease |
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Pseudo-small eye |
Consider ipsilateral ptosis or enophthalmos, or |
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abnormally large contralateral eye |
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COMMON CLINICAL PRESENTATIONS 617
Common clinical presentations: cloudy cornea and leukocoria
Opacification of the cornea, lens, or posterior structures is usually associated with poor vision and may indicate serious, even life-threatening, pathology.
Cloudy cornea
Corneal opacities may be focal (either central or peripheral) or diffuse in nature (Table 18.15). They may be an isolated finding, associated with other ocular abnormalities, or part of an inherited syndrome. They may be congenital, acquired at birth, or develop during childhood.
Leukocoria
All patients with leukocoria (Table 18.16) must be urgently assessed for the possibility of retinoblastoma. Congenital cataracts are generally easily identified. Other conditions may be less readily differentiated from retinoblastoma, most commonly persistent fetal vasculature syndrome, Coats’ disease, toxocara infection, and ROP.
Table 18.15 Corneal opacities: etiologies and key features
Diffuse |
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Birth trauma |
Forceps injury may induce ruptures in Descemet’s |
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membrane (usually unilateral with vertical break) |
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Keratitis (infective, |
Photophobia, watery eye, circumlimbal injection, |
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allergic, exposure) |
corneal infiltrate ± epithelial defect ± AC activity |
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Corneal dystrophies |
Clinical pattern varies but may be evident from birth |
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(e.g., congenital hereditary endothelial dysfunction) |
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Metabolic |
Bilateral corneal clouding with systemic abnormalities |
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in some mucopolysaccharidoses or mucolipidoses |
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Central |
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Peter’s anomaly |
Congenital, usually bilateral central opacities 9 |
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adhesions to iris/lens (posterior ulcer of von Hippel) |
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Peripheral |
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Sclerocornea |
Bilateral (often asymmetric), peripheral opacification |
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with vascularization ± other corneal/angle anomalies |
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Limbal dermoid |
Solid white mass that may involve peripheral cornea; |
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rarely bilateral and 360*around the limbus |
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Posterior embryotoxon |
Peripheral opacity due to anteriorly displaced |
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Schwalbe’s line ± other angle/ocular abnormalities |
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618 CHAPTER 18 Pediatric ophthalmology
Table 18.16 Leukocoria: etiologies and key features
Lens |
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Cataract |
Lens opacity: stationary or progressive; isolated, |
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or associated with other ocular or systemic |
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abnormalities |
Vitreous |
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Persistent fetal |
Variable persistence of fetal vasculature/hyaloid |
vasculature |
remnants; often microphthalmic; usually unilateral |
syndrome |
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Inflammatory cyclitic |
Fibrous membrane behind the lens arising from |
membrane |
the ciliary body due to chronic intraocular |
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inflammation |
Retina |
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Retinoblastoma |
Retinal mass of endophytic, exophytic, or |
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infiltrating type; tumor may spread to anterior |
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segment, orbit. This is life threatening if untreated! |
Coloboma |
Developmental defect resulting in variably sized |
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defect involving optic disc, choroid, and retina |
Coats’ disease |
Retinal telangiectasia with exudation, lexudative |
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retinal detachment in severe cases |
Retinopathy of |
Early cessation of peripheral retinal vascularization |
prematurity (ROP) |
due to prematurity causes fibrovascular |
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proliferation |
Familial exudative |
Early cessation of peripheral retinal vascularization |
vitreoretinopathy |
due to inherited defect causes ROP-like picture in |
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full-term infant |
Incontinentia |
Abnormal peripheral retinal vascularization due |
pigmenti |
to inherited defect causes ROP-like picture in girls |
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(lethal in boys) |
Retinal dysplasia |
Gray vascularized mass from extensive gliosis (e.g., |
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Norries disease, Patau syndrome) |
Infection |
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Toxocara |
Unilateral granuloma or endophthalmitis |
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620 CHAPTER 18 Pediatric ophthalmology
Table 18.17 Clinical features of congenital toxoplasmosis
Ocular |
Retinochoroiditis (more commonly bilateral and affecting the |
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macula than in acquired disease), cataract, microphthalmos, |
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strabismus |
Systemic |
Hydrocephalus, intracranial calcification, hepatosplenomegaly |
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Table 18.18 Clinical features of congenital syphilis
Early disease (<2 years of age)
Ocular |
Chorioretinitis and retinal vasculitis (results in characteristic |
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salt-and-pepper fundus) |
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Conjunctivitis |
Systemic |
Mucocutaneous rash; periostitis and osteochondritis |
Late disease (>2 years of age) |
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Ocular |
Interstitial keratitis (usually presents at 5–20 years of age) |
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Optic atrophy |
Systemic |
Saddle nose, frontal bossing, saber shins, Hutchinson’s teeth, |
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scoliosis, hard palate perforation |
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Table 18.19 Clinical features of congenital rubella
Ocular |
Nuclear cataract, microphthalmos, glaucoma (congenital or |
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infantile), corneal clouding, retinitis |
Systemic |
Congenital heart disease, sensorineural deafness, anemia, |
(early/late) |
thrombocytopenia, bone abnormalities, hepatitis, CNS |
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abnormalities (e.g., encephalitis) |
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Table 18.20 Clinical features of congenital CMV
Ocular |
Retinitis (focal) |
Systemic |
IUGR, microcephaly, hydrocephalus, intracranial calcification, |
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hepatosplenomegaly, thrombocytopenia |
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Table 18.21 Clinical features of congenital HSV
Ocular |
Chorioretinitis |
Systemic |
Microcephaly, intracranial calcification |
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OPHTHALMIA NEONATORUM 621
Ophthalmia neonatorum
Ophthalmia neonatorum is defined as a conjunctivitis occurring within the first month of life. Organisms are commonly acquired from the birth canal. The main risk factor is therefore the presence of sexually transmitted disease in the mother.
Ophthalmia neonatorum affects up to 12% of neonates in the Western world and up to 23% in developing countries. It is potentially sight threatening and may cause systemic complications. In some countries (including the United States), it is a reportable disease (within 12 hours).
Gonococcal neonatal conjunctivitis
Clinical features
•Hyperacute (within 1–3 days of birth), with severe purulent discharge, lid edema, chemosis, ± pseudomembrane, ± keratitis.
Investigation
•Prewet swab or conjunctival scrapings: immediate Gram stain (gramnegative diplococci), culture (chocolate agar), and sensitivities.
Treatment
•Ceftriaxone 50 mg/kg IV 1x/day 1 week; frequent saline irrigation of discharge until eliminated.
•After appropriate counseling, refer mother (with partner) to urogenital physician.
Chlamydial neonatal conjunctivitis
This is the most common cause of neonatal conjunctivitis. A papillary rather than follicular reaction is seen from delayed development of palpebral lymphoid tissue.
Clinical features
•Subacute onset (4–28 days after birth), mucopurulent discharge, papillae, ± preseptal cellulitis.
•Systemic (uncommon): rhinitis, otitis, pneumonitis.
Investigation
•Prewet swabs are usually for immunofluorescent staining, but cell culture, PCR, and ELISA may be used.
•Conjunctival scrapings: Giemsa stain.
Treatment
•Erythromycin 25 mg/kg 2x/day for 2 weeks.
•After appropriate counseling, refer mother (with partner) to urogenital physician.
Other bacterial neonatal conjunctivitis
Other bacterial causes include Staphylococcus aureus, Streptococcus pneumoniae (which require topical antibiotics only), and Haemophilus and Pseudomonas (which requires additional systemic antibiotics to prevent systemic complications).
Clinical features
•Subacute onset (4–28 days after birth), purulent discharge, lid edema, chemosis, ± keratitis (Pseudomonas)
622 CHAPTER 18 Pediatric ophthalmology
Investigation
• Prewet swab or conjunctival scrapings: Gram stain, culture, sensitivities.
Treatment
•Gram-positive organisms: topical (e.g., erythromycin ointment 4x/day); adjust according to sensitivities.
•Gram-negative organisms: topical (e.g., tobramycin ointment 4x/day); adjust according to sensitivities.
HSV neonatal conjunctivitis
Although viral causes of neonatal conjunctivitis are uncommon, they may cause serious ocular morbidity and systemic disease.
Clinical features
•Acute onset (1–14 days), vesicular lid lesions, mucoid discharge ± keratitis (e.g., microdendrities), anterior uveitis, cataract, retinitis, optic neuritis (rare).
•Systemic (uncommon but may be fatal): jaundice, hepatosplenomegaly, pneumonitis, meningoencephalitis, disseminated intravascular coagulopathy (DIC).
Investigation
•Swab or conjunctival scrapings transported in viral culture medium; PCR.
•Newborns with ocular HSV infection must be evaluated for systemic infection. There should be a very low threshold for hospital admission and systemic antiviral treatment.
Treatment
•Acyclovir ointment 5x/day for 1week ± acyclovir IV 10 mg/kg 3x/day for 10 days.
Chemical conjunctivitis
Silver nitrate drops are commonly used in some parts of the world as a protective measure against ophthalmia neonatorum (Table 18.22). While effective against gonococcal disease, they are of limited use against other bacteria and are of no use against Chlamydia or viruses. In most neonates the drops cause red, watery eyes 12–48 hours after instillation.
Conjunctivitis in the older child
Children are commonly affected by infective and allergic conjunctivitis. In the older child, it behaves in a more similar manner to adult disease: viral (p. 142), bacterial (p. 140), chlamydial (p. 144), and allergic (p. 146).
Table 18.22 Timing of onset of ophthalmia neonatorum by etiology
Chemical |
<2 days |
Gonococcal |
1–3 days |
Other bacteria |
2–5 days |
HSV |
1–14 days |
Chlamydia |
4–28 days |
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ORBITAL AND PRESEPTAL CELLULITIS 623
Orbital and preseptal cellulitis
Orbital cellulitis may cause blindness and even death. It requires emergency assessment, imaging, and treatment under the joint care of an ophthalmologist, ENT specialist, and pediatrician. Part of the ophthalmologist’s role is to assist in differentiating orbital cellulitis from the more limited preseptal cellulitis.
Orbital cellulitis
Infective organisms include Streptococcus pneumoniae, Staphylococcus aureus, Streptococcus pyogenes, and Haemophilus influenza (previously common in younger children, but less likely if Hib vaccinated).
Risk factors
•Sinus disease: ethmoidal sinusitis (common), maxillary sinusitis.
•Infection of other adjacent structures: preseptal or facial infection, dacrocystitis, dental abscess.
•Trauma: septal perforation.
•Surgical: orbital, lacrimal, and vitreoretinal surgery.
Clinical features
•Fever, malaise, painful, swollen orbit.
•Inflamed lids (swollen, red, tender, warm), proptosis, painful restricted eye movements ± optic nerve dysfunction (dVA, dcolor vision, RAPD).
•Complications: optic nerve compromise is the most important; also exposure keratopathy, iIOP, CRAO, CRVO.
•Systemic: meningitis, cerebral abscess, cavernous sinus thrombosis, orbital or periorbital abscess.
Investigation
•Temperature.
•CBC, blood culture.
•CT (dedicated CT for orbit and sinuses; possibly brain): diffuse orbital infiltrate with fat stranding, proptosis ± sinus opacity, orbital abscess.
Treatment
•Admit for intravenous antibiotics (e.g., either floxacillin 25 mg/kg 4x/day or cefuroxime 50 mg/kg 4x/day with metronidazole 7.5 mg/kg 3x/day).
•ENT specialist to assess for sinus drainage (required in up to 50%).
Preseptal cellulitis
Preseptal infection is much more common than orbital cellulitis. The main causative organisms are once again staphylococci and streptococci.
This is generally a less severe disease, at least in adults and older children (see Table 18.23). In younger children in whom the orbital septum is not fully developed, there is a high risk of progression, thus the disease should be treated similarly to orbital cellulitis.
624 CHAPTER 18 Pediatric ophthalmology
Clinical features
•Fever, malaise, painful, swollen lid/periorbita.
•Inflamed lids but no proptosis, normal eye movements, normal optic nerve function.
Investigation
Investigation is not usually necessary unless there is concern about possible orbital or sinus involvement (Table 18.24).
Treatment
•Admit young or ill children; otherwise daily observation is sufficient until disease resolution.
•Treat with oral antibiotics (e.g., floxacillin and metronidazole).
Table 18.23 Differentiating features of orbital vs preseptal cellulitis
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Orbital |
Preseptal |
Proptosis |
Present |
Absent |
Ocular motility |
Painful + restricted |
Normal |
VA |
d(in severe cases) |
Normal |
Color vision |
d(in severe cases) |
Normal |
RAPD |
Present (in severe cases) |
Normal |
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Table 18.24 Development of paranasal sinuses
Sinus |
Onset of development |
Onset of adult configuration |
Maxillary |
In utero |
Late childhood (12 years) |
Sphenoidal |
In utero |
Puberty |
Ethmoidal |
In utero |
Puberty |
Frontal |
Postnatal |
Adulthood |
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