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PERIORBITAL & ORBITAL CELLULITIS
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ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES
The fascia of the eyelids joins with the fibrous orbital septum to isolate the orbit from the lids.
The orbital septum helps decrease the risk of an eyelid infection extending into the orbit.
Infections arising anterior to the orbital septum are termed preseptal.
Orbital cellulitis denotes infection posterior to the orbital septum and may cause serious complications, such as a cavernous sinus thrombosis, cerebral abscess, meningitis, septicemia, and optic neuropathy.
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Preseptal (periorbital) cellulitis usually arises from a local exogenous source such as an abrasion of the eyelid, from other infections (hordeolum, dacryocystitis), or from infected varicella or insect bite lesions. S aureus and S pyogenes are the most common pathogens cultured from these sources.
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Orbital cellulitis almost always arises from paranasal sinus infection (most commonly ethmoid sinusitis) because the walls of three sinuses make up portions of the orbital walls, and infection can breach these walls or extend by way of a richly anastomosing venous system. The pathogenic agents are those of acute or chronic sinusitis—respiratory flora and anaerobes, In addition to the normal culprits of preseptal cellulitis. Streptococcus anginosis is an emerging cause of orbital cellulitis and often causes a more serious infection with increased frequency of intracranial or spinal abscesses that may require neurosurgical intervention.
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Rhino-orbital-cerebral mucormycosis is an uncommon but devastating infection that can lead to blindness and death in children with immunosuppression and poorly-controlled diabetes.
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Children with preseptal cellulitis present with erythematous and edematous eyelids, pain, and mild fever. The vision, eye movements, and eye itself are normal. Decreased vision, restricted eye movements, and an APD suggest orbital cellulitis.
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Orbital cellulitis presents with orbital signs, which are proptosis, eye movement restriction, decreased vision, and APD. The eye often appears red and chemotic. CT or MRI is required to establish the extent of the infection within the orbit and sinuses.
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Differential Diagnosis
++
Severe conjunctivitis can cause (often bilateral) eyelid swelling and redness that can mimic preseptal/orbital cellulitis. Other confusing diseases are primary or metastatic neoplasm of the orbit, orbital pseudotumor (idiopathic orbital inflammation), and orbital foreign body with secondary infection.
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Preseptal cellulitis can progress to orbital cellulitis. Orbital cellulitis can result in permanent vision loss due to compressive optic neuropathy. Proptosis can cause corneal exposure, dryness, and scarring. Cavernous sinus thrombosis, intracranial extension, blindness, and death can result from severe orbital cellulitis.
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Initial therapy for preseptal and orbital cellulitis infection is with broad-spectrum systemic antibiotics, which may be later narrowed based on culture findings and clinical improvement. Treatment may require surgical drainage for subperiosteal abscess in addition to drainage of infected sinuses, which is a crucial part of the therapy. Surgery is indicated for decreased visual acuity attributable to orbital cellulitis, failure to improve clinically after 24–48 hours of IV antibiotics, or demonstration of worsened abscess on repeat CT. Patients with proptosis, eye movement restriction, elevated eye pressure, and age greater than 9 years are significantly more likely to require surgical intervention.
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Most patients do well with timely treatment.
+
Davies
BW, Smith
JM, Hink
EM, Durairaj
VD: C-Reactive protein as a marker for initiating steroid treatment in children with orbital cellulitis. Ophthal Plast Reconstru Surg 2014 Nov 12;
[PubMed: 25393908]
. [Epub ahead of print]
+
Smith
JM, Bratton
EM, DeWitt
P, Davies
BW, Hink
EM, Durairaj
VD. Predicting the need for surgical intervention in pediatric orbital cellulitis. Am J Ophthalmol Aug 2014;158(2):387–394
[PubMed: 24794092]
.
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CRANIOFACIAL ANOMALIES
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ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES
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Ocular abnormalities associated with craniofacial abnormalities involving the orbits include visual impairment, proptosis, corneal exposure, hypertelorism (widely spaced orbits), strabismus, amblyopia, lid coloboma, papilledema, refractive errors, and optic atrophy.
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Orbital and ocular abnormalities associated with craniofacial anomalies often require a multispecialty approach. Management may require orbital and strabismus surgery. Ophthalmologists also treat amblyopia, refractive errors, and corneal exposure when present.
+
Taub
PJ, Lampert
JA: Pediatric craniofacial surgery: a review for the multidisciplinary team. Cleft Palate Craniofac J 2011;48(6):670–683
[PubMed: 21740182]
.
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ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES
The most common benign tumor of infancy is capillary hemangioma, arising in the first few weeks of life and exhibiting a characteristic sequence of growth and spontaneous involution (Figure 16–25).
The most common primary malignant tumor of the orbit is rhabdomyosarcoma.
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Capillary hemangiomas may be located superficially in the lid or deep in the orbit and can cause ptosis (see Figure 16–25), refractive errors, and amblyopia. Deeper lesions may cause proptosis.
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Orbital dermoid cysts are benign congenital orbital choristomas that vary in size and are usually found temporally at the brow and orbital rim or supranasally. These lesions are firm, well encapsulated, and mobile. Rupture of the cyst causes a severe inflammatory reaction.
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Lymphangioma occurring in the orbit is typically poorly encapsulated, increases in size with upper respiratory infection, and is susceptible to hemorrhage. Other benign tumors of the orbit are varix, plexiform neurofibroma, teratoma, and tumors arising from bone, connective tissue, and neural tissue.
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Orbital rhabdomyosarcoma (see Chapter 31) grows rapidly and displaces the globe. The average age at onset is 6–7 years. The tumor is often initially mistaken for orbital swelling due to insignificant trauma or can mimic orbital cellulitis.
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Tumors metastatic to the orbit also occur; neuroblastoma is most common. The patient may exhibit proptosis, orbital ecchymosis (raccoon eyes), Horner syndrome, or opsoclonus (dancing eyes). Ewing sarcoma, leukemia, Burkitt lymphoma, and Langerhans cell histiocytosis may involve the orbit.
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Examination of vision, eye movements, eyelids, and orbits often reveals amblyopia, eyelid malposition, strabismus, and proptosis. Neuroimaging with CT or MRI is required to delineate the location and size of orbital tumors.
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Differential Diagnosis
++
Orbital pseudotumor (idiopathic orbital inflammation) and orbital cellulitis.
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Most capillary hemangiomas do not require treatment. Topical and systemic β-blockers have shown success in treating capillary hemangiomas. Treatment is indicated if the lesion is large enough to cause amblyopia. Induced astigmatism or amblyopia (or both) are treated with glasses and patching, respectively. Treatment of orbital dermoids is by excision.
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Rhabdomyosarcoma is treated with chemotherapy combined with surgery, radiation, or both. With expeditious diagnosis and proper treatment, the survival rate of patients with orbital rhabdomyosarcoma confined to the orbit approaches 90%.
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Treatment of metastatic disease requires management by an oncologist and may require chemotherapy and radiation therapy.
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Prognosis depends on the underlying disease.
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ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES
Nystagmus is an involuntary rhythmic oscillation of eyes. It may be unilateral or bilateral, more pronounced in one eye, or be gaze-dependent.
Nystagmus can be caused by poor vision. Reduced visual acuity can also occur from nystagmus due to excessive motion of images on the retina.
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Nystagmus can be grouped into infantile nystagmus, which usually appears in the first 3–6 months of life, and acquired nystagmus, which appears later in life. Infantile nystagmus can be idiopathic, associated with retinal diseases (ie, foveal hypoplasia), due to low vision (ie, optic nerve hypoplasia), or due to visual deprivation early in life (ie, cataracts). Foveal hypoplasia can be isolated or be associated with aniridia, albinism, and achromatopsia.
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Nystagmus can also occur with normal ocular structures and seemingly normal CNS development. Infantile nystagmus syndrome, also known as congenital motor nystagmus, is an ocular disorder of unknown etiology that presents at birth or early infancy. Latent nystagmus occurs when one eye is occluded. This type of nystagmus occurs in patients with strabismus.
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Acquired nystagmus can result from neurologic disorders, vestibular dysfunction, and drug toxicity.
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Spasmus nutans, in which a rapid, shimmering, disconjugate nystagmus occurs with head bobbing and torticollis, is a benign disorder that resolves with time. Clinically, nystagmus of spasmus nutans is indistinguishable from nystagmus due to optic pathway glioma. MRI brain/orbits with and without contrast is necessary to determine if the cause of the nystagmus is due to a CNS disease. An electroretinogram may be required to rule out retinal pathology as the cause of nystagmus if neuroimaging is normal.
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When characterizing nystagmus, the examiner can note the laterality (unilateral, bilateral, asymmetric), plane (horizontal, vertical, torsional), conjugacy, amplitude and frequency of nystagmus.
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Nystagmus due to low vision or foveal hypoplasia may exhibit a slow “roving” nystagmus. Infantile nystagmus syndrome is characterized by horizontal, uniplanar nystagmus that increases in intensity with fixation and decreases in intensity with convergence and at a null point in gaze. Children may have an abnormal head posture in order to hold their gaze at the null point. They may have crossing (esotropia) due to nystagmus blockage syndrome since nystagmus is dampened by convergence. See-saw nystagmus is associated with a suprasellar mass (ie, craniopharyngioma).
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Children with infantile nystagmus do not complain of oscillopsia (perception of oscillations in vision). Neurologic disease should be suspected in acquired nystagmus and also when nystagmus is unilateral or asymmetrical.
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Differential Diagnosis
++
Opsoclonus (chaotic bilateral eye movements), voluntary eye movements (cannot be prolonged).
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Therapy is directed at managing the underlying ocular or CNS disease. An ophthalmologist can optimize vision by correcting significant refractive errors and strabismus. The range of vision varies depending on the cause of the nystagmus. Some patients may benefit from extraocular muscle surgery and contact lenses.
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Most affected individuals have subnormal vision, but spasmus nutans usually improves with time.
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ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES
Amblyopia is a unilateral or bilateral reduction in vision due to strabismus, refractive errors, and/or visual deprivation.
Amblyopia can occur only during the critical period of visual development in the first decade of life when the visual nervous system is plastic.
Approximately 3% of the population is amblyopic.
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Amblyopia is classified according to its cause. Strabismic amblyopia can occur in the nondominant eye of a strabismic child. Refractive amblyopia can occur in both eyes if significant refractive errors are untreated (ametropic or refractive amblyopia). Another type of refractive amblyopia can occur in the eye with the worse refractive error when imbalance is present between the eyes (anisometropic amblyopia). Deprivation amblyopia occurs when dense cataracts or complete ptosis prevents formation of a formed retinal image. Of the three types of amblyopia, the deprivation form of amblyopia results in the worst vision.
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Normal binocular development leads to progressive refinement of monocular visual acuity, stereoacuity (3D vision), and fusion of images from both eyes. Individuals with amblyopia may not only have decreased visual acuity but also decreased contrast sensitivity, stereoacuity, and fixation stability, and increased vulnerability to “crowding,” difficulty identifying shapes surrounded by visual “clutter.”
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Vision screening and referral to an eye care professional if amblyopia is suspected.
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Screening for amblyopia should be a component of periodic well-child examinations. The single best screening technique to discover amblyopia is obtaining visual acuity in each eye. In preverbal children unable to respond to visual acuity assessment, amblyogenic factors are sought, including strabismus, media opacities, unequal Brückner reflexes (pupillary red reflexes), and a family history suggestive of strabismus, amblyopia, or ocular disease occurring in childhood (see section Ophthalmic Examination).
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The earlier treatment is begun, the better will be the chance of improving visual acuity. Treatment is usually discontinued after age 9 years. Amblyogenic factors such as refractive errors are addressed. Because of the extreme sensitivity of the visual nervous system in infants, congenital cataracts and media opacities must be diagnosed and treated within the first few weeks of life. Visual rehabilitation and amblyopia treatment must then be started to foster visual development.
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After eradicating amblyogenic factors, the mainstay of treatment is patching the sound eye, which causes the visual nervous system to process input from the amblyopic eye and in that way permits the development of useful vision. Other treatment modalities include “fogging” the sound eye with cycloplegic drops (atropine), lenses, and filters.
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Prognosis depends on the compliance with treatment but is usually good.
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ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES
Strabismus is misalignment of the eyes.
Its prevalence in childhood is about 2%–3%.
Strabismus is categorized by the direction of the deviation (esotropia, exotropia, hypertropia, hypotropia) and its frequency (constant or intermittent).
Strabismus may cause or be due to amblyopia.
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Esotropia (Crossed Eyes)
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ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES
Pseudoesotropia can result from prominent epicanthal folds and wide nose bridges that give the appearance of crossed eyes when they are actually straight.
Esotropia is deviation of the eyes toward the nose and may involve one or both eyes.
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Primary infantile esotropia (also known as congenital esotropia) has its onset in the first year of life. The deviation of the eyes toward the nose is large and obvious. The most frequent type of acquired esotropia is the accommodative type (Figure 16–26). Onset is usually between ages 2 and 5 years. The deviation is variable in magnitude and constancy and is often accompanied by amblyopia. Refractive accommodative esotropia is associated with a high hyperopic refraction. In another type, the deviation is worse with near than with distant vision (high AC/A accommodative esotropia). This type of esodeviation is usually associated with lower refractive errors.
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Esotropia can be associated with certain syndromes. In Möbius syndrome (congenital facial diplegia), a sixth nerve palsy causing esotropia is associated with palsies of the 7th and 12th cranial nerves and limb deformities. Duane syndrome can affect the medial or lateral rectus muscles (or both). It may be an isolated defect or may be associated with a multitude of systemic defects (eg, Goldenhar syndrome). Duane syndrome is often misdiagnosed as a sixth (abducens) nerve palsy. The left eye is involved more commonly, but both eyes can be involved. Girls are affected more frequently.
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After age 5 years, any esotropia of recent onset should arouse suspicion of CNS disease. Intracranial masses, hydrocephalus, demyelinating diseases, and idiopathic intracranial hypertension are causes of abducens palsy, where patients may present with esotropia and diplopia. Papilledema is often, but not invariably, present with increased ICP.
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Besides the vulnerability of the abducens nerve to increased ICP, it is susceptible to infection and inflammation. Otitis media and Gradenigo syndrome (inflammatory disease of the petrous bone) can cause sixth nerve palsy. Less commonly, migraine and diabetes mellitus are considerations in children with sixth nerve palsy.
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Observation of the reflection of a penlight on the cornea, the corneal light reflex, is an accurate means of determining if the eyes are straight. This is a good way to differentiate true esotropia from pseudostrabismus. If strabismus is present, the corneal light reflex will not be centered in one or both eyes. Observation of eye movements may reveal restriction of eye movements in certain positions of gaze. Children with unilateral paretic or restrictive causes of esotropia may develop face turns toward the affected eye to maintain binocularity. The face turn is an attempt to maintain binocularity away from the field of action of the paretic muscle. Alternate cover testing of the eyes while the child is fixating on a near and/or distant target will reveal refixation movements laterally if the eyes are crossed. Motility, cycloplegic refraction, and a dilated funduscopic examination by an ophthalmologist are necessary to determine the etiology of esotropia. Some children require imaging studies and neurologic consultation.
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Amblyopia and poor stereoacuity/depth perception.
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Surgery is the mainstay of treatment for primary infantile esotropia. Surgery is typically performed between 6 months and 2 years of age in order to obtain optimal results.
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Management of accommodative esotropia includes glasses with or without bifocals, amblyopia treatment, and, in some cases, surgery.
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Underlying neurologic disease should be referred to the appropriate specialists for further management.
++
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Exotropia (Wall-Eyed, Drifting of eyes)
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ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES
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The deviation of the eyes toward the ears most often begins intermittently and occurs after age 2 years (see Figure 16–27). Congenital (infantile) exotropia is extremely rare in an otherwise healthy infant. Early-onset exotropia may occur in infants and children with severe neurologic problems. All children with constant, congenital exotropia require CNS neuroimaging. Referral to an ophthalmologist is indicated.
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Evaluation of the corneal light reflex reveals the penlight’s reflection in the deviated eye is displaced nasally. Cover uncover test or alternate cover test will reveal refixation eye movements inward (toward the nose) when exotropia is present.
++
Convergence insufficiency is a special type of exotropia where the deviation is larger at near compared to distance. They have poor convergence (ability to converge or cross their eyes when looking at a near target). Common symptoms include asthenopia, binocular diplopia, or visual blur with near visual tasks.
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Poor stereoacuity/depth perception. Amblyopia is less commonly seen in exotropia compared to esotropia but can occur in monocular or poorly controlled exotropia.
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Treatment of exotropia includes observation, glasses and/or surgery. Indications for surgery of intermittent exotropia include poor control of deviation, large angle deviation, and worsening stereoacuity. There is strong evidence that orthoptic exercises can improve the symptoms and signs of convergence insufficiency. Classic exercise is pencil push-ups, which can be performed at home or in the office. There are other computer-based exercises that can help reduce the symptoms of convergence insufficiency.
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UNEXPLAINED DECREASED VISION IN INFANTS & CHILDREN
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ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES
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Occult causes of poor vision and blindness in children may be due to hereditary retinal dystrophies such as Leber congenital amaurosis and optic nerve abnormalities, including optic nerve hypoplasia and atrophy.
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Cortical visual impairment, also known as cortical blindness, is manifested as decreased visual attentiveness of varying degree. Cortical visual impairment can occur from generalized cerebral damage or from targeted insult to the occipital lobes from retro-chiasmal and visual association pathway pathology. Perinatal hypoxia, trauma, intracranial hemorrhage, and periventricular leukomalacia are some of the causes of cortical visual impairment.
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Affected infants will have poor eye contact, fail to fixate and follow a visual target, and be unresponsive to visual threat. Wandering or roving eye movements and nystagmus are common. Eye poking is seen in some infants with low vision.
++
Referral to an ophthalmologist is indicated to determine the etiology of the low vision. Diagnostic tests such as an electroretinogram and visual evoked response may be required. Imaging studies of the brain, genetics, and neurology consultations may be useful.
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Differential Diagnosis
++
Delayed visual maturation, vision loss due to an ocular versus neurologic disease.
++
Low-vision aids enhance remaining vision. Devices used include magnifiers for both distance and near vision, closed-circuit television, and large-print reading materials. Vision rehabilitation specialists and support groups can help teach the affected child and their family how to best use these devices. Clinical trials are underway for treatment of Leber congenital amaurosis with gene therapy.
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Generally poor for vision.
+
Hufnagel
RB, Ahmed
ZM, Corrêa
ZM, Sisk
RA: Gene therapy for Leber congenital amaurosis: advances and future directions. Graefes Arch Clin Exp Ophthalmol 2012;250(8):1117–1128
[PubMed: 22644094]
.
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LEARNING DISABILITIES & DYSLEXIA
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ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES
Learning disabilities and dyslexia result in poor reading comprehension and writing.
Children often have vague complaints of ocular fatigue, headaches, and difficulty reading.
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Evaluation of the child with learning disabilities and dyslexia should include ophthalmologic examination to identify any ocular disorders that could cause or contribute to poor school performance. Most children with learning difficulties have no demonstrable problems on ophthalmic examination.
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A multidisciplinary approach is recommended by the AAP, the AAPOS, and the AAO for evaluating and treating children with learning disabilities. According to the joint AAP report, there is no scientific evidence to support the claims that visual training, muscle exercises, ocular pursuit-and-tracking exercises, behavioral/perceptual vision therapy, “training” glasses, prisms, and colored lenses and filters are effective treatments for learning disabilities, dyslexia, and strabismus. There is no evidence that children who participate in vision therapy are more responsive to educational instruction than children who do not participate.
+
Handler
SM, Fierson
WM, Section on Ophthalmology; Council on Children With Disabilities; American Academy of Ophthalmology; American Association for Pediatric Ophthalmology and Strabismus; American Association of Certified Orthoptists: Learning disabilities, dyslexia, and vision. Pediatrics 2011;127(3):e818–e856.
[PubMed: 21357342]
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Rucker
JC, Phillips
PH: Efferent Vision Therapy. J Neuroophthalmol Jan 4, 2017;1–7. doi: 10.1097/WNO.0000000000000480.28059865.
+
Ali
MJ: Pediatric acute dacryocystitis. Ophthal Plast Reconstr Surg Sep-Oct 2015;31(5):341–347. doi: 10.1097/IOP.0000000000000472.25856337.
+
American Academy of Ophthalmology:
www.aao.org. Accessed: November 27, 2017
+
American Association of Pediatric Ophthalmology and Strabismus:
www.aapos.org. Accessed: November 27, 2017
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Hammersmith
KM: Blepharokeratoconjunctivitis in children. Curr Opin Ophthalmol Jul 2015;26(4):301–305
[PubMed: 26058029]
.
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O’Gallagher
M, Bunce
C, Hingorani
M, Larkin
F, Tuft
S, Dahlmann-Noor
A: Topical treatments for blepharokeratoconjunctivitis in children. Cochrane Database Syst Rev Feb 7, 2017;2:CD011965. doi: 10.1002/14651858.CD011965.pub2.28170093.
+
O’Gallagher
M, Banteka
M, Bunce
C, Larkin
F, Tuft
S, Dahlmann-Noor
A: Systemic treatment for blepharokeratoconjunctivitis in children. Cochrane Database Syst Rev May 2016;30(5):CD011750. doi: 10.1002/14651858.CD011750.pub2.27236587.