Ptosis

Definition

Ptosis (also known as blepharoptosis) is drooping of the upper eyelid.  It can be the result from both congenital and acquired abnormalities of the muscles that serve to raise the eyelid.

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Basic Anatomy

Some of the various different muscles that surround the palpebral fissures include the orbicularis oculi, the levator palpebrae superiors, and the Müller's muscle. 

  1. 1. The orbicularis oculi muscle surrounds the circumference of the eye and serves to close both the upper and lower eyelid.  It is innervated by the seventh cranial nerve (facial). 
  2. 2. The levator palpebrae superiors muscle helps to raise the upper eyelid.  It is innervated by the third cranial nerve (oculomotor).
  3. 3. The Müller's muscle is an accessory smooth muscle that serves to help elevate the upper eyelid.  It is innervated by the sympathetic nervous system.

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History and Physical Examination:

Obtaining a good history can help reveal the etiology and lead to a correct diagnosis.  It is important to investigate the timing, duration, and progression of the ptosis as well as associated symptoms.  Asking the patient or patient’s family members to show photographs of the patient prior to development of symptoms can help the clinician appreciate the degree of ptosis as well.

Some questions you may want to ask include:

  1. When did you first notice that your eyelid start to droop?  Alternatively, has it been present since birth?
  2. Are both eyes drooping or just one?
  3. Did you notice that your eyelids have been progressively drooping (or feeling heavier) or did this process seem to happen immediately?
  4. Do you notice that the degree of ptosis changes during different times of the day? Or under different settings?
  5. Have you experienced any recent trauma?
  6. Do you have blurry vision?  Vision problems?
  7. Do you have a history of eye surgery?
  8. Do any other members of your family experience similar symptoms?
  9. Any other symptoms that you notice besides the ptosis?

On examination, the clinician should pay attention to how the head is postured and eyebrow is positioned.  Some patients may adapt a “chin up” position and raise their eyebrows in an attempt to maintain vision.  Also take notice of eyelid position, pupil size and reactivity, and extraocular movements.   The clinician should also perform a detailed neurologic examination.

 

Etiology

Congenital

  • Congenital ptosis is usually unilateral and does not progress.  It is associated with absence or diminution of the levator palpebrae superiors muscle.  Other ophthalmologic findings may include amblyopia and strabismus.
  • Congenital orbital fibrosis is an autosomal dominantly inherited condition that produces fibrosis of the extraocular muscles that consequently limits eye movements and has associated ptosis.  Addtionally, blepharophimosis  (another autosomal dominantly inherited condition) is associated with severe bilateral ptosis.
  • Congenital Horner's syndrome due to birth trauma can display ptosis of the affected eye.
  • Marcus Gunn jaw winking: abnormal innervations of the levator muscle by the mandibular branch of the trigeminal nerve causes eyelid retraction when the patient contracts the pterygoid muscle during the acts of sucking or jaw movement.
  • Infantile myasthenia gravis, Surge Weber, von Recklinghausen syndrome, and fetal alcohol syndrome can all generate ptosis.

Mechanical

  • Mechanical ptosis can result from excessive weight on the upper lid.  Sources include infections, inflammation, and eyelid tumors.

Neurologic

  • Any disease that affects the neurologic system (including the brainstem, cranial nerves, neuromuscular junction, muscle, peripheral nerves, etc.) can create ptosis.
  • Third cranial nerve (oculomotor) palsy will result in ptosis, opthalmoplegia and diplopia.  Lesions anywhere on the path between the oculomotor nucleus in the midbrain and the extraocular muscles within the orbit can cause third nerve palsy.
  • Horner’s syndrome is any dysfunction to the sympathetic nervous system that results in ipsilateral ptosis, mitosis, and anhidrosis.  In children, Horner's syndrome can lead to heterochromia (a difference in eye color between the two eyes.)
  • Neuromuscular junction disorders such as botulism and myasthenia gravis often produce ptosis. 
  • Myasthenia gravis is an autoimmune disorder in which antibodies are directed against postganglionic acetylcholine receptors in the neuromuscular junction of voluntary muscles. Isolated ocular findings present initially in approximately half of the cases. Unilateral or bilateral ptosis can develop with/without diplopia along with other ocular motor dysfunctions. Infantile myasthenia gravis occurs when antibodies are transferred to the neonate by the mother with myasthenia gravis.
  • Ptosis can be the result of infectious botulism.
  • Myogenic are muscle disorders, such as mitochondrial myopathy and moronic dystrophy, can present with ptosis.

 

Pseudoptosis

  • Pseudoptosis is when the eyelid falsely appears to be ptotic but the primary pathology may not involve the lid.
  • Some conditions that can display pseudoptosis include an enophthalmic eye (lack of posterior support), hypotropic eye (droop disappears when affected patient fixates on an object with the eye), and lid retraction of one eye (ptosis seems to come into view in the contralateral eye).

 

Management

Managing ptosis can include nonsurgical and/or surgical therapy depending on the underlying pathology.

Nonsurgical therapy

  • In cases where the ptosis can be attributed to myasthenia gravis, medical therapy is considered the gold standard.

Surgical therapy

  • Surgery is indicated mainly in patients who have an obscured visual field due to ptosis. Obscured vision in infants may lead to amblyopia; thus, making surgery the treatment of choice.
  • Surgery can also be employed for cosmetic reasons.  
  • Surgical approaches to the improvement of ptosis include Müller muscle resection, levator muscle resection or advancement, and frontals suspension.  The surgical method chosen is individually determined.

 

References

  1. Fosterer J. Ptosis: causes, presentation, and management. Aesthetic Plast Surg 2003; 27:193.
  2. Toyka KV. Ptosis in myasthenia gravis: extended fatigue and recovery bedside test. Neurology 2006; 67:1524.
  3. Johnson CC. Congenital ptosis. In: Principles and Practice of Ophthamology, Jakobiec FA, Albert DM. (Eds), WB Saunders Co, Philadelphia 1994. p.2839.
  4. Ahmadi AJ, Sires BS. Ptosis in infants and children. Int Ophthalmol Clin 2002; 42:15.
  5. Kersten RC, de Conciliis C, Kulwin DR. Acquired ptosis in the young and middle-aged adult population. Ophthalmology 1995; 102:924.

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