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Ptosis

Ptosis is the drooping of 1 or both eyelids.May be minimal or compromise visual axis.

Principal Causes of Ptosis

1. Defectivedevelopment of the levator palpebrae muscle
2. Trauma
3. Neurologic disorders
   1. Oculomotornerve palsy
   2. Ophthalmoplegic migraine
   3. Horner syndrome
4. Neuromuscular junction disorders
   1. Myastheniagravis
5. Muscle disorders
6. Neoplasm
7. Marcus Gunn phenomenon

Clinical Features and Diagnosis

Defective Development of Levator Palpebrae Muscle

Most commoncause of congenital ptosis is defective development of levator palpebraemuscle, whose function is to raise the lid.

Usually unilateral but may be bilateral.

Occurrence is usually sporadic, butit can be transmitted as autosomal-dominant trait or in conjunctionwith extraocular muscle disorders.

Trauma

Contusion or eyelid laceration may produceptosis. Sometimes CT or MRI is necessary to define nature of injury.

Neurologic Disorders

Oculomotor Nerve Palsy

Injury tooculomotor nerve may produce ptosis, mydriasis, and strabismus.Eye usually appears "down and out," with deficitsin elevation and adduction.

Common causes are head trauma, meningitis,encephalitis, and intracranial tumors.

Ophthalmoplegic Migraine

Association of severe unilateral periorbitalheadaches with oculomotor nerve palsy is known as ophthalmoplegicmigraine. Headache may last a few hours, but ophthalmoplegia maypersist for days or weeks.

Horner Syndrome

Consistsof unilateral ptosis (1–2 mm), miosis, and anhidrosis.It is due to sympathetic denervation and may be congenital or acquired.

Congenital cases are presumably dueto birth trauma, especially injury of lower roots of brachial plexus.

Acquired causes include injuries (trauma,or cardiovascular or spine surgery) and tumors (neuroblastoma) thataffect superior cervical ganglion or sympathetic trunks around carotidartery.

If 1 pupil dilates and the other staysthe same in dim light, testing of pupils with a drop of 4% cocainecan confirm diagnosis. The small pupil stays the same size aftercocaine instillation in Horner syndrome, and further investigationmay be necessary to determine the specific cause. Dilation of bothpupils indicates presence of physiologic anisocoria (unequal pupils).

Neuromuscular Junction Disorders

Myasthenia Gravis

Myastheniagravis, an autoimmune disease, may present with unilateral or bilateral ptosisthat worsens with fatigue. Ptosis may be only sign of this disorderin infancy.

Although Tensilon test may be diagnostic,it can be equivocal or negative.

Anti–acetylcholine receptorantibody titers also can be measured.

Another test that can be used to determinewhether ptosis is due to myasthenia is the ice test. Small bag ofcrushed ice is held over eyelid for 2 mins. If ptosis recedes, childhas myasthenia gravis. Effect probably occurs through combinationof cooling and eyelid rest leading to decreased activity of acetylcholinesterase,although cooling effects seem to be predominant (Golnik et al.,1999). See Chap. 33, Hypotoniaand Weakness.

Muscle Disorders

Ptosis and external ophthalmoplegia may occurwith myotubular myopathy and mitochondrial myopathy (see Chap. 33, Hypotonia and Weakness).

Neoplasm

Tumors ofeyelid (e.g., hemangioma, plexiform neuroma, and rhabdomyosarcoma) mayproduce ptosis.

Hemangioma can usually be recognizedby cutaneous elements.

Characteristic café au laitskin pigmentation provides clue for neurofibromatosis.

Histologic diagnosis is necessary forall tumors except perhaps for hemangioma.

Marcus Gunn Phenomenon

Is the decrease or disappearance of ptosiswith opening of mouth. Usually unilateral and more commonly involvesleft eye. The reason for its occurrence is that some fibers fromcranial nerve V innervate levator muscle, which should normallybe innervated by cranial nerve III.

Diagnostic Approach

Completehistory and physical exam should be performed, including eye exam.Presence of ptosis in neonates and involvement of 1 or both eyesprovide useful diagnostic clues.

Congenital ptosis may be due to defectivedevelopment of levator muscle, birth trauma to levator muscle orcervical sympathetic chain (Horner syndrome), congenital myopathy,or myasthenia gravis. With muscle weakness, myasthenia gravis andcongenital myopathy should be suspected. Ice test or testing withneostigmine or edrophonium may be diagnostic for myasthenia gravis.Muscle biopsy is necessary when considering diagnosis of congenitalmyopathy. Pharmacologic testing with cocaine drops can be used toconfirm diagnosis of Horner syndrome.

Trauma is most common cause of acquiredptosis, and surgical trauma is most common cause of acquired Hornersyndrome. Any mass lesion of eye producing ptosis should be investigatedfor possible malignancy.

Any child with persistent congenitalor acquired ptosis should be referred for ophthalmologic consultationto determine severity and possible treatment, so that amblyopiacan be prevented.

References

1. Aicardi J. Disease of the nervous systemin childhood, 2nd ed. London: Mac Keith Press, 1998.
2. Beard C. A new classification of blepharoptosis. IntOphthalmol Clin 1989;29:214–216.
3. Callahan MA, Beard C. Beard's ptosis, 4thed. Birmingham, AL: Aesculapius, 1990.
4. Catalano RA, Nelson LB. Pediatric ophthalmology: atext atlas. Norwalk, CT: Appleton & Lange, 1994.
5. Dubowitz V. Muscle disorders in childhood, 2nd ed.London: WB Saunders, 1995.
6. Golnik KC, et al. An ice test for the diagnosis ofmyasthenia gravis. Ophthalmology 1999;106:1282–1286.
7. Nelson LB, ed. Harley's pediatric ophthalmology,4th ed. Philadelphia: WB Saunders, 1998.
8. Online Mendelian Inheritance in Man (OMIM). McKusick-NathansInstitute for Genetic Medicine, Johns Hopkins University (Baltimore,MD) and National Center for Biotechnology Information, NationalLibrary of Medicine (Bethesda, MD), 2000. World Wide Web URL: http://www.ncbi.nlm.nih.gov/omim.
9. Reynolds D, Collin R. Lids. In: Taylor D, ed. Paediatricophthalmology, 2nd ed. Oxford: Blackwell Science, 1997:214–236.
10. Rudolph AM, ed. Rudolph's pediatrics, 20thed. Stamford, CT: Appleton & Lange, 1996.

Book Source Details

• Book Title: The Diagnostic Approach to Symptoms and Signs in Pediatrics
• Author(s): Paul S. Bellet
• Year of Publication: 2006
• Copyright Details: The Diagnostic Approach to Symptoms and Signs in Pediatrics, Copyright © 2006 Lippincott Williams & Wilkins.

Other Book Chapters Related to Eyelid swelling

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Copyright Details: The Diagnostic Approach to Symptoms and Signs in Pediatrics, Copyright © 2008 Williams & Wilkins.

More About Causes of Eyelid swelling

• Back to symptom: Eyelid swelling: Introduction (review 100 causes)
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More About This Book: Title: The Diagnostic Approach to Symptoms and Signs in Pediatrics Authors: Paul S. Bellet Publisher: Lippincott Williams & Wilkins Copyright: 2006 ISBN: 0-78172-899-1

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