Ординатура / Офтальмология / Английские материалы / Handbook of Pediatric Neuro-Ophthalmology_Wright, Spiegel, Thompson_2006
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g.Allow the parents to cry or to express shock, grief, anger, or any other emotion they feel.
h.If you are too busy to spend sufficient time, arrange for another appointment so that parents can have adequate time to ask questions.
i.Do not try to ameliorate grief by saying such things as “It could be worse.”
j.Try to give appropriate referrals. It helps both the family and the physician to be able to do something.
k.If possible, do not request payment from a family in shock. A staff member can contact the family at a later date.
l.Teach your staff about all these points. Insist on professional kindness in your office.
Acknowledgment. I thank Marilyn Horn, L.C.S.W., for all her hard work with the original subject matter.
APPENDIX
Parents need referrals. Contacting resources is something concrete parents can do for their child, and for many individuals, taking action also relieves anxiety. Resources are different in each state. We have prepared a list that gives you a place to start. Parents can use this list to find out what other resources may exist in their community.
Cancer
American Cancer Society 46 First St. NE
Atlanta, GA 30308 800/ACS-2345 404/320-3333
Candlelighters Childhood Cancer Foundation 1312 18th St. NW, Suite 300
Washington, DC 20036 800/366-2223 202/659-5136
(See also Visual Impairments for Retinoblastoma resources)
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Cerebral Palsy
Canadian Cerebral Palsy Association 800 Wellington St., Suite 612 Ottawa, Ontario
Canada K1R 6K7 800/267-6572 (in Canada) 613/235-2144
United Cerebral Palsy Association 7 Penn Plaza, Suite 804
New York, NY 10001 800/USA-1UCP 212/268-6655
CHARGE Syndrome
CHARGE Accounts c/o Quota Club 2004 Parkade Blvd.
Columbia, MO 65202 314/442-7604
Chronic Illness
N.O.R.D.
National Organization for Rare Disorders P.O. Box 8923
New Fairfield, CT 06812 http://www.rarediseases.org
Magic Foundation
(Optic Nerve Hypoplasia) 1327 N. Harlem Ave. Oak Park, IL 60302 709/383-0808
http://www.magicfoundation.org
Parents of Chronically Ill Children 1527 Maryland St.
Springfield, IL 62702 217/522-6810
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Deaf/Blind
John Tracy Clinic 806 West Adams Blvd
Los Angeles, CA 90007 800/522-4582
Hydrocephalus
Hydrocephalus Association 2040 Polk St., Box 342
San Francisco, CA 94109 415/776-4713
Hydrocephalus Support Group c/o Kathy McGowan
6059 Mission Rd., #106 San Diego, CA 92108 619/282-1070
National Hydrocephalus Foundation 22427 S. River Rd.
Joliet, IL 60436 815/467-6548
Lawrence Moon Bardet Biedl Syndrome
Lawrence Moon Bardet Biedl Syndrome Network 18 Strawberry Hill
Windsor, CT 06095 203/688-7880
Marfan Syndrome
National Marfan Foundation 382 Main St.
Port Washington, NY 10050 516/883-8712
Mental Retardation
Association for Retarded Citizens of the U.S. 500 E. Border St., Suite 300
Arlington, TX 76010 817/261-6003
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Neurofibromatosis
National Neurofibromatosis Foundation 141 Fifth Ave., Suite 7-S
New York, NY 10010 800/323-7938 212/460-8980
Visual Impairments
American Foundation for the Blind 15 West 16th St.
New York, NY 10011 800/AF-BLIND (232-5463) 212/620-2043
American Printing House for the Blind 1839 Frankfort Ave.
P.O. Box 6085
Louisville, KY 40206-0085 502/895-2405
Association for Macular Diseases 210 East 64th St.
New York, NY 10021 212/655-3007
The Institute for Families of Blind Children P.O. Box 54700
Mailstop #111
Los Angeles, CA 90054-0700 323/669-4649
National Association for the Visually Impaired P.O. Box 317
Watertown, MA 02272-0317 800/562-6265
Fax: 617/972-7444
(Some areas have a state organization as well; NAPVI can direct the parent)
National Organization for Albinism and Hypopigmentation (NOAH)
155 Locust St., Suite 1816 Philadelphia, PA 19102 800/473-2310
215/545-2322
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Parents and Cataract Kids (PACK) c/o Geraldine Miller
P.O. Box 73 Southeastern, PA 19399 215/352-0719
Retinoblastoma International 4650 Sunset Blvd., M.S. 88 Los Angeles, CA 90027 323/669-2299 www.retinoblastoma.net
New England Retinoblastoma Support Group
603 Fourth Range Road
Pembroke, NH 03275
General Resources
The Family Resource Coalition 230 N. Michigan Avenue
Suite 1625, Dept. W Chicago, IL 60601
(Identification of parent support groups all over the country)
Reaching Out: A Directory of National Organizations Related to Maternal and Child Health
38th and R Streets, NW
Washington, DC 20057 202/625-8400
Team of Advocates for Special Kids 100 W. Cerritos Ave.
Anaheim, CA 92805 714/533-8275
Other National Toll-Free Numbers:
American Council of the Blind 800/424-8666
Better Hearing Institute 800/424-8576
Epilepsy Information Line 800/332-1000
Cystic Fibrosis Foundation 800/344-4823
Downs Syndrome 800/221-4602
Easter Seal Society 800/221-6827
Health Information Clearinghouse 800/336-4797
Spina Bifida 800/621-3141
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Fragile X Foundation 800/835-2246 American Kidney Fund 800/835-8018
National Information Center for Orphan Drugs and Rare Disease 800/336-4797
Sickle Cell Association 800/421-8453
Retinitis Pigmentosa (RP) Association International 800/ 344-4877
Local School Districts or State Departments of Special Education
Search on the Internet for most current information.
5
Ocular Motility Disorders
Mitra Maybodi, Richard W. Hertle, and
Brian N. Bachynski
Normal individuals and most patients with common concomitant childhood strabismus have full ocular rotations (versions and ductions). This chapter is devoted to some of the more frequently encountered childhood disorders of the central and peripheral nervous systems, neuromuscular junction, and extraocular muscles that appear clinically to have incomitant
ocular misalignments.
Analysis of ocular alignment, versions, ductions, forced ductions, and generated force allows the examiner to sort the causes of these limited eye movements into three general categories:
(1) neuromuscular dysfunction, (2) restriction of the globe by orbital tissues, and (3) combined neuromuscular dysfunction and restriction (Fig. 5-1). Diagnosis in children is especially challenging because it is rarely possible to clinically test the force generated by extraocular muscle action. A general anesthetic is routinely required to perform forced ductions. It may therefore be necessary to base diagnostic and therapeutic decisions on incomplete clinical information, and the clinician must rely on familiarity with the epidemiologic and clinical characteristics of each disorder.
DISORDERS OF THE CENTRAL AND PERIPHERAL NERVOUS SYSTEMS
Eye movement disorders arising from disturbance of the normal neurophysiology may be classified as supra-nuclear, internuclear, or infranuclear.
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FIGURE 5-1. Clinical evaluation of range of eye movements. Versions and cover test measurements allow the examiner to decide whether the eye movements are normal (no limitation) or limited. Forced duction testing is used to differentiate a restriction (positive resistance to movement of the globe) from a “paresis” (no resistance to movement of the globe).
Supranuclear Eye Movements
Cranial nerves III, IV, and VI serve together with the extraocular muscles as a final mechanism that executes all eye movements. Supranuclear pathways initiate, control, and coordinate various types of eye movements. Several types of eye movements are briefly mentioned here (Table 5-1), but a detailed and lucid synthesis of current concepts of the neural control of eye movements can be found in many other sources.288
PHYSIOLOGY AND CLINICAL ASSESSMENT
The vestibular apparatus drives reflex eye movements, which allow us to keep images of the world steady on the retinas as we move our heads during various activities. The eyes move in the opposite direction to the movement of the head so that they remain in a steady position in space. The semicircular canals are the end organs that provide the innervation to the vestibular
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nuclei, which in turn drive cranial nerves III, IV, and VI to compensate for rotations of the head. In contrast, the otoliths respond to linear accelerations of the head and to gravity when the head is tilted. You can easily test the effectiveness of input from the semicircular canals by testing the vestibulo-ocular reflex (VOR). First, hold your head still and observe an object such as your index finger as you move it side to side at about 1 to 3 cycles/s. The image is a blur. However, if you hold your finger steady and rotate your head from side to side at the same frequency, you are able to maintain a clear image.
Several forms of saccades, fast eye movements, can be clinically observed. Voluntary saccades may be predictive, in anticipation of a target appearing in a specific location; command-generated, in response to a command such as “look to the right”; memory-guided; or antisaccades, in which a reflexive saccade to an abruptly appearing peripheral target is suppressed and, instead, a voluntary saccade is generated in the equidistant but opposite direction. Involuntary saccades consist of the fast phase of nystagmus due to vestibular and optokinetic stimuli; spontaneous saccades, providing repetitive scanning of the environment, although also occurring in the dark and in severely visually impaired children; and reflex saccades, occurring involuntarily in response to new visual, auditory, olfactory, or tactile cues, suppressable by antisaccades.83
TABLE 5-1. Types of Eye Movements.
Type of eye |
|
|
|
movement |
Function |
Stimulus |
Clinical tests |
Vestibular |
Maintain steady |
Head rotation |
Fixate on object while |
|
fixation during |
|
moving head; calorics |
|
head rotation |
|
|
Saccades |
Rapid refixation |
Eccentric retinal |
Voluntary movement |
|
to eccentric |
image |
between two objects; |
|
stimuli |
|
fast phases of OKN or |
|
|
|
vestibular nystagmus |
Smooth |
Keep moving |
Retinal image slip |
Voluntarily follow a |
pursuit |
object on fovea |
|
moving target; OKN |
|
|
|
slow phases |
Vergence |
Disconjugate, slow |
Binasal or |
Fusional amplitudes; near |
|
movement to |
bitemporal |
point of convergence |
|
maintain |
disparity; |
|
|
binocular vision |
retinal blur |
|
|
|
|
|
OKN, optokinetic nystagmus.
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The pathway of saccades originates in the visual cortex and projects through the anterior limb of the internal capsule, through the diencephalon. It then divides into dorsal and ventral pathways, the dorsal limb going to the superior colliculi, and the ventral limb (which contains the ocular motor pathways for horizontal and vertical eye movements) to the pons and midbrain. The superior colliculus acts as an important relay for some of these projections (Fig. 5-2).
The neurons responsible for generating the burst, or discharge, for saccades are classified as excitatory burst neurons (EBN); inhibitory burst neurons (IBN) function to silence activ-
FIGURE 5-2. The superior colliculi are a pair of ovoid masses composed of alternating layers of gray and white matter; they are centers for visual reflexes and ocular movements, and their connections to other structures in the brain and spinal cord are varied and complex. Some of these other structures include the retina, visual and nonvisual cerebral cortex, inferior colliculus, paramedian pontine reticular formation, thalamus, basal ganglia, and spinal cord ventral gray horn. The fibers of the medial longitudinal fasciculus form a fringe on its ventrolateral side: 1, superior (cranial) colliculus; 2, brachium of superior (cranial) colliculus; 3, medial geniculate nucleus; 4, brachium of inferior (caudal) colliculus; 5, central gray substance; 6, cerebral aqueduct; 7, visceral nucleus of oculomotor nerve (Edinger–Westphal nucleus); 8, nucleus of oculomotor nerve; 9, medial lemniscus; 10, central tegmental tract; 11, medial longitudinal fasciculus; 12, red nucleus; 13, fibers of oculomotor nerve; 14, substantia nigra; 15, basis pedunculi.