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Ординатура / Офтальмология / Английские материалы / Oxford American Handbook of Ophthalmology_Tsai, Denniston, Murray_2011

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606 CHAPTER 18 Pediatric ophthalmology

Pediatric examination

The assessment of children (see Tables 18.4 and 18.5) requires a flexible and often undignified approach. The goal is to keep everyone—patient, parents, and extended family—on the same side. Without this it is very difficult to achieve an adequate clinical assessment and impossible to institute treatment.

The awake child

Attempt to entertain the child during history taking (e.g., with a toy) and turn the examination into a game. Explain what you are about to do (e.g., with drops) and why.

Examine opportunistically and be patient. Surprisingly, young children may be happy to be examined at the slit lamp (standing, kneeling on the chair, or sitting on caregiver’s knee). If this is impossible, consider a portable slit lamp for the anterior segment, the indirect ophthalmoscope for the fundus, and the direct ophthalmoscope for higher magnification of the macula and disc. Applanation tonometry and gonioscopy may only be possible by examination under anesthesia (EUA).

Keeping the child happy usually keeps the adults happy. Good communication is essential.

The anesthetized child (EUA)

An EUA may be indicated if detailed examination is impossible with the child awake. It may be performed when the child is being anesthetized for a different procedure, thus coordinated care with other specialists involved with the child is essential. The anesthesiologist should have appropriate experience with pediatric anesthesia.

The presence of the speculum may affect IOP and refraction. It is thus recommended that tonometry (Tonopen or Perkins) and retinoscopy be performed early in the examination and before insertion of the speculum.

Examine the anterior segment with the portable slit lamp, the operating microscope, and gonioscopy lens. Examine the posterior segment with the direct and indirect ophthalmoscope. Consider A- and B-scan ultrasonography.

 

Table 18.4

Visual milestones

 

 

 

 

 

6 weeks

Can fix and follow a light source, face or large, colorful toy,

 

 

smiling responsively

 

3 months

Fixation is central, steady and maintained, can follow a slow

 

 

target, and converge

 

6 months

Reaches out accurately for toys

 

2 years

Picture matching

 

3 years

Letter matching of single letters (e.g., Sheridan Gardiner)

 

5 years

Snellen chart by matching or naming

 

 

 

 

 

 

PEDIATRIC EXAMINATION

607

 

 

 

 

 

Table 18.5 A systematic approach to examining children

 

 

 

 

 

 

 

 

Visual symptoms

History of poor visual behavior for their age,

 

 

 

 

strabismus, nystagmus, head nodding, red eye, epiphora,

 

 

 

 

photophobia, asymmetry of pupils, corneas, globes, or

 

 

 

 

red reflexes

 

 

 

POH

Previous or current eye disease; refractive error

 

 

 

PMH

Obstetric and perinatal history; developmental history

 

 

 

Systemic conditions

Any other systemic (especially CNS) abnormalities

 

 

 

SH

Family support

 

 

 

FH

Family history of strabismus/other visual problems

 

 

 

Drug history

Drugs

 

 

 

Allergy history

Allergies

 

 

 

Visual acuity

Select test according to age (p. 9); when quantitative

 

 

 

 

testing is not possible, grade ability to fix gaze and follow

 

 

 

 

(i.e., is it central, steady, and maintained?)

 

 

 

Visual function

Check for RAPD, binocularity, stereopsis, suppression

 

 

 

 

and retinal correspondence (pp. 9–10)

 

 

 

Cover test

Near/distance/prism cover test

 

 

 

Motility

Ductions, versions, convergence, saccades, doll’s eye

 

 

 

 

movements

 

 

 

Accommodation

 

 

 

 

BSV

Level of BSV

 

 

 

Fixation

Fixation behavior, visuscope

 

 

 

Refraction

Cycloplegic refraction

 

 

 

Orbit

Proptosis, inflammation, masses

 

 

 

Lids

Lid crease, additional skin folds, puncta

 

 

 

Conjunctiva

Inflammation, adhesions

 

 

 

Cornea

Diameter, thickness, opacity, staining

 

 

 

AC

Flare, cells

 

 

 

Gonioscopy

(may require EUA) Angle, dysgenesis

 

 

 

Iris

Coloboma, anisocoria, polycoria, corectopia

 

 

 

Lens

Lens opacity, shape, position

 

 

 

Tonometry

Applanation (may require EUA); digital

 

 

 

 

 

 

Vitreous

Hyaloid remnants, inflammation, empty

 

 

 

Optic disc

Size, cup, congenital anomaly, edema

 

 

 

Fundus

Macula, vessels, retina (e.g., tumors, inflammation,

 

 

 

 

dystrophies, exudation)

 

 

 

Systemic review

For dysmorphic features (including face, ears, teeth, hair)

 

 

 

 

or any other systemic abnormalities

 

 

 

 

 

 

 

608 CHAPTER 18 Pediatric ophthalmology

The child who does not see

Worldwide, there are over 1.5 million children who are blind or severely visually impaired. Major causes include inherited abnormalities (e.g., cataracts, glaucoma, retinal dystrophies), intrauterine insults (e.g., infection) and acquired problems (e.g., retinopathy of prematurity, trauma).

The ophthalmologist’s primary aim—the best possible vision for the child—must be seen in the context of the child’s overall health, quality of life, and family support. Likewise, the ophthalmologist’s contribution should be seen in the context of the multidisciplinary team, which may include pediatricians, optometrists, orthoptists, primary care physicians, specialist nurses, social workers, and teachers. The challenge to provide the best possible care for the child (and family) will depend on the following factors.

Disability

Is the visual impairment the only problem, or are there associated disabilities? These may range from mild developmental delay (e.g., motor, speech, social) to profound neurological or systemic abnormalities. In some severe diseases, life expectancy may also be considerably reduced.

Such children require the full benefit of the multidisciplinary team, usually coordinated by the pediatrician.

Treatment

What treatment might be possible now or in the future? Be realistic about what is and what is not currently possible. Ensure best visual potential with refraction, visual aids, and other supportive measures.

When more invasive treatment is indicated, ensure that the parents are fully aware of the risks, realistic outcome, and the extent of care that they will need to give in the perioperative period (e.g., drops, contact lens, frequent clinic visits).

Equipment

What equipment will help the child function best at home and at school? Reading may require Braille (it is important to start early) or large-print books (usually beneficial if reading vision is worse than N10).

Normal-sized print may be read by closed-circuit television (CCTV) magnification or by a scanner attached to a computer that has a magnified display facility or has optical character recognition with a speech synthesizer. The ease of use of standard computer systems has been revolutionized since accessibility options became a standard feature of computer operating systems (e.g., Windows®).

Schooling

Will the child manage best in a specialist school (for the blind or partially sighted) or in a mainstream school (with specialist teacher support)? This is usually determined by the level of visual impairment, any associated disabilities, and the availability of resources locally.

THE CHILD WHO DOES NOT SEE 609

Resources

How much assistance (practical and financial) is the family and/or social services able to provide? Social workers should ensure that parents are receiving appropriate financial benefits. Community-based pediatricians may be invaluable in coordinating local resources. Nonprofit, governmental organizations often provide help, including advice and emotional support for the parents.

Social

Is the disability accepted by the family and community? The diagnosis may stretch family relationships to a breaking point. Siblings may become jealous of the extra attention the child needs.

In some communities, blindness is regarded as a stigma. This may adversely affect family dynamics and hinder the child’s wider social interactions.

Implications

Are other family members or future siblings at risk of developing the disease, or of being carriers? Initial knowledge of related genetic disease may produce strong emotions; counseling requires time, patience, and often multiple consultations. The parents may feel guilty about passing on an inherited disease to their child.

Prognosis

Is the visual impairment stationary or progressive? Parents may want to know the probable impact on navigation, education, work, and driving. Whenever possible, balance the negative (what they won’t be able to do) with the positive (what they will be able to do). Stress that medical knowledge is limited and that such prognoses are a best guess.

610 CHAPTER 18 Pediatric ophthalmology

Child abuse

The physician has a legal duty of care toward any child he or she sees. This means that if there is any concern or suspicion of possible abuse, it is the physician’s responsibility to act in the child’s best interest.

Concern might relate to injuries that are inconsistent with the mobility of the child or with the reported mechanism, histories that are inconsistent with each other or evolve with time, or an unusual relationship between the caregiver and child. Appropriate action may include discussion with a senior ophthalmologist, referral for a pediatric opinion, direct referral to social services, or consultation with the child’s teacher. It is not acceptable to ignore concerns or to assume someone else will act.

On occasion, the ophthalmologist may be asked to examine a child as part of child protective services investigations. This should be performed by the most senior ophthalmologist available in the care pediatric pateints. It is important to complete as full an examination as possible and for it to be carefully documented.

Photographs may be helpful: if a digital system is used, an unmodified printout should be made at the time and signed by two witnesses. If a report is required, this should be phrased in terms comprehensible to an educated lay public and include the examiner’s full name, qualifications, and the situation in which he or she saw the child.

Retinal hemorrhages and shaken baby syndrome

Shaken baby syndrome (SBS)

Retinal hemorrhages in the absence of bony injury or external eye injury may arise from severe shaking of young children (shaken baby syndrome). They are not diagnostic of abuse and must be taken in the context of the whole patient.

Alternative mechanisms

Additional consideration for other putative mechanisms of retinal and intracranial hemorrhage include the following:

Normal handling (e.g., vigorous play): it is highly unlikely that the forces required to produce retinal hemorrhage in a child <2 years of age would be generated by a reasonable person during the course of vigorous (even rough) play.

Short-distance falls: in a child with retinal hemorrhages and subdural hemorrhages who has not sustained a high-velocity injury and in whom other recognized causes of such hemorrhages have been excluded, child abuse is the most likely explanation; rarely, accidental trauma may give rise to a similar picture.

High cervical injuries: cervical injuries alone do not result in retinal bleeding, unless combined with circulatory collapse.

Hypoxia: acute hypoxia from transient apnea has not been shown to result in the SBS picture, unless combined with circulatory collapse.

Intracranial bleeding: Terson syndrome (retinal hemorrhages secondary to intracranial bleeding) is rare in children and any hemorrhages tend to be concentrated around the optic disc.

COMMON CLINICAL PRESENTATIONS: VISION AND MOVEMENT 611

Common clinical presentations: vision and movement

The following discussion outline common reasons for parents to seek ophthalmic advice. The underlying diseases range from the innocuous to the blinding and/or fatal. A complete ophthalmic (and usually systemic) examination should be performed in all cases.

Tables 18.6, 18.7, and 18.8 indicate the main causes of these clinical presentations, their key features, and/or a cross-reference to further information.

The child who does not see

Unilateral visual loss may not be noticed by parents until picked up at screening or during investigation for an associated abnormality (usually strabismus). Bilateral visual loss will be apparent in the child’s visual behavior. In addition, children who have bilateral poor vision from an early age often have nystagmus or roving eye movements, although this does not occur in patients with retrochiasmal lesions.

Examination: orthoptic, refractive, ophthalmic, neurological ± systemic (as indicated) (Table 18.6).

Abnormal eye alignment

Strabismus is common, affecting around 2% of children. While many cases are detected by parents, significant deviations may be missed because of their size, intermittent nature, or compensatory head posture. Conversely, a number of factors may give the appearance of strabismus in a perfectly orthophoric child—pseudostrabismus.

Examination: refractive, ophthalmic, neurological ± systemic (as indicated) (Table 18.7).

Abnormal eye movements

Abnormal supplementary eye movements may occur as an isolated phenomenon or secondary to ocular or systemic disease (usually CNS pathology). They may be broadly divided into nystagmus or saccadic abnormalities.

Examination: refractive, ophthalmic, neurological ± systemic (as indicated) (Table 18.8).

612 CHAPTER 18 Pediatric ophthalmology

Table 18.6 Poor vision: outline of causes

General

Specific

Refractive

Myopia, hypermetropia, astigmatism

Cornea

Opacity, edema, abnormal curvature, or size

Lens

Cataract, subluxation, lenticonus

Vitreous

Persistent fetal vasculature, inflammation, hemorrhage

Retina

Coloboma, ROP, detachment, dysplasia, dystrophy, albinism

Macula

Hypoplasia, dystrophy, edema, inflammation, scarring,

 

traction

Optic nerve

Inherited optic atrophy, compression, infiltration,

 

inflammation

CNS

Hypoxia, inflammation, hydrocephalus, compression,

 

delayed visual maturation

Other

Amblyopia, delayed visual maturation, functional

 

 

Table 18.7 Abnormal ocular alignment: outline of etiologies and key features

Strabismus (p. 580)

Intermittent or manifest misalignment of eyes that

 

may be horizontal, vertical, or torsional

Pseudostrabismus

Consider epicanthal folds, asymmetry of face, globes

 

(proptosis/ enophthalmos) or pupils, abnormal

 

interpupillary distance or abnormal angle kappa

 

 

Table 18.8 Abnormal eye movements: outline of etiologies and key features

 

 

Nystagmus (p. 557)

Slow movement away from fixation corrected by

 

 

 

fast movement (jerk nystagmus) or another slow

 

 

 

movement (pendular nystagmus)

 

 

Saccadic abnormalities

Fast movement away from fixation, corrected by fast

 

 

 

(p. 561)

movement immediately (oscillation e.g., opsoclonus,

 

 

 

ocular flutter) or after delay (intrusion)

 

 

 

 

COMMON CLINICAL PRESENTATIONS 613

Common clinical presentations: red eye, watery eye, and photophobia

Red (Table 18.9) or watery eyes (Table 18.10) are among the most common ocular presentations in primary care. Often these are relatively benign conditions, many of which may be successfully treated by general practitioners.

However in the presence of atypical features (particularly visual symptoms), more serious diagnoses should be considered. The presence of photophobia is usually an indication of more severe ocular pathology (Table 18.11).

Examination: ophthalmic ± refractive, neurological, systemic (as indicated).

Red eye(s)

Table 18.9 Red eye: etiologies and key features

Normal VA

 

 

 

Conjunctivitis (infective,

Gritty, often itchy, discharge, diffuse superficial

 

allergic, chemical)

injection, ± lid papillae/follicles

 

Foreign body

FB sensation, FB visible or in fornix/subtarsal, local

 

 

injection, linear corneal abrasions (if subtarsal FB)

 

Episcleritis

Mild local pain, sectoral superficial injection

 

 

(constricted by phenylephrine)

 

Scleritis

Severe pain, deep often diffuse injection;

 

 

complications may lead to dVA

 

Vascular malformation

Abnormal conjunctival blood vessels, usually chronic,

 

 

± systemic vascular abnormalities

 

dVA

 

 

 

Corneal abrasion/

Photophobia, watery eye, sectoral/circumlimbal

 

erosion

injection, epithelial defect

 

Keratitis

Photophobia, watery eye, circumlimbal injection,

 

 

corneal infiltrate ± epithelial defect ± AC activity

 

Glaucoma (acute iIOP)

Photophobia, watery eye, corneal edema, iIOP 9

 

 

anterior segment/angle abnormalities

 

Anterior uveitis (acute)

Photophobia, watery eye, keratic precipitates, AC

 

 

activity, ± posterior synechiae

 

Endophthalmitis

Pain, floaters, watery eye, diffuse deep injection,

 

 

 

inflammation (vitreous > AC), chorioretinitis,

 

 

decreased vision (most common)

 

 

 

 

 

614 CHAPTER 18 Pediatric ophthalmology

Photophobia

Table 18.10 Watery eye: etiologies and key features

Increased tears

 

Blepharitis (posterior)

Chronic gritty, irritable eyes, poor tear film quality, ±

 

meibomitis

Conjunctivitis (infective,

Gritty, often itchy, discharge may be sticky, diffuse

allergic, chemical)

superficial injection, ± lid papillae/follicles

Foreign body

FB sensation, FB visible or in fornix/subtarsal, local

 

injection, corneal lacerations (if subtarsal FB)

Corneal abrasion/

Photophobia, sectoral/circumlimbal injection, epithelial

erosion

defect

Keratitis

Photophobia, sectoral/circumlimbal injection, corneal

 

infiltrate ± epithelial defect ± AC activity

Glaucoma (acute iIOP)

Photophobia, injection, corneal edema, iIOP ±

 

anterior segment/angle abnormalities

Anterior uveitis

Photophobia, circumlimbal injection, keratic

 

precipitates, AC activity, ± posterior synechiae

Decreased drainage

 

Nasolacrimal duct

Chronic watery eye (may have sticky discharge)

obstruction

without other ocular signs ± lacrimal sac swelling

 

 

Watery eyes

Table 18.11 Photophobia: etiologies and key features

Anterior segment disease

 

 

Corneal abrasion/

Watery eye, sectoral/circumlimbal injection, epithelial

 

 

erosion

defect

 

 

Keratitis

Watery eye, circumlimbal injection, corneal infiltrate ±

 

 

 

epithelial defect ± AC activity

 

 

Anterior uveitis

Watery eye, circumlimbal injection, keratic precipitates,

 

 

(acute)

AC activity, ± posterior synechiae

 

 

Glaucoma (acute

Watery eye, injection, corneal edema, iIOP ± anterior

 

 

iIOP)

segment/angle abnormalities

 

 

Inadequate iris

Complete/partial absence of tissue (e.g., aniridia,

 

 

sphincter

coloboma), mydriasis or hypopigmentation (albinism)

 

 

Posterior segment disease

 

 

 

Endophthalmitis

Decreased vision, pain, floaters, watering, diffuse deep

 

 

 

injection, inflammation (vitreous > AC), chorioretinitis

 

 

Retinal dystrophies

Cone deficiencies (achromatopsia, blue cone

 

 

 

monochromatism) or later-onset dystrophies

 

 

CNS disease

 

 

 

Meningitis/

Fever, headache, neck stiffness, altered mental state,

 

 

encephalitis

neurological dysfunction, normal ocular examination

 

 

 

 

COMMON CLINICAL PRESENTATIONS 615

Common clinical presentations: proptosis and globe size

Abnormalities of the whole globe are usually congenital and represent developmental abnormalities. Abnormal protrusion of the eye (proptosis) usually represents an acquired, progressive disease.

Proptosis

Abnormal protrusion of the eye (proptosis) is uncommon, but usually signifies severe orbital pathology (Table 18.12). Acute onset in an ill child may represent orbital cellulitis, an ophthalmic emergency. Orbital tumors (Table 18.13) usually present with more gradual proptosis, although rhabdomyosarcoma is well known to present acutely, mimicking orbital cellulitis.

Table 18.12 Proptosis: etiologies and key features

Infection

 

 

 

Orbital cellulitis

Febrile, illness, with acute pain, lid swelling,

 

 

restricted eye movements, ± dVA

 

Inflammation

 

 

 

Idiopathic orbital

Acute pain, lid swelling, conjunctival injection 9

 

inflammatory disease

intraocular inflammation and dVA; diffuse orbital

 

 

disease vs. localized (e.g., myositis or dacroadenitis)

 

Thyroid eye disease

Pain, conjunctival injection, lid retraction, restrictive

 

 

myopathy, dVA; usually older children

 

Vasculitis

Usually present acutely and are ill (e.g., Wegener’s

 

 

granulomatosis, PAN)

 

Tumors

 

 

 

Acquired (e.g.,

Proptosis ± pain, dVA, abnormal eye

 

rhabdomyosarcoma)

movements; usually gradual onset but some (e.g.,

 

 

rhabdomyosarcoma) may present acutely

 

Congenital (e.g., dermoid

Superficial lesions present early as a round lump,

 

cysts)

deep lesions may cause pain and gradual proptosis

 

Vascular anomalies

 

 

 

Congenital orbital varices

Intermittent proptosis exaggerated by Valsalva

 

 

maneuver or forward posture

 

Carotid–cavernous fistula

Arterialized conjunctival vessels, chemosis, ±

 

 

bruit; usu. traumatic in children; orbital bruit on

 

Bony anomalies

auscultation

 

 

 

 

 

 

 

 

Sphenoid dysplasia

Pulsatile proptosis, encephalocele, associated with

 

 

neurofibromatosis-1

 

Craniosynostosis

Premature fusion of sutures resulting in

 

 

characteristic skull abnormalities

 

Other

 

 

 

Pseudoproptosis

Consider ipsilateral large globe or lid retraction,

 

 

contralateral enophthalmos or ptosis, facial

 

 

asymmetry, shallow orbits