Eyelid ptosis: causes, symptoms, diagnosis, treatment

Ptosis of the upper eyelid (syn. blepharoptosis) is an abnormally low position of the upper eyelid, which can be congenital or acquired.

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Classification of eyelid ptosis

1. Neurogenic
   • oculomotor nerve palsy
   • Horner syndrome
   • Marcus Gunn syndrome
   • oculomotor nerve aplasia syndrome
2. Myogenic
   • myasthenia gravis
   • muscular dystrophy
   • ophthalmoplegic myopathy
   • simple congenital
   • blepharophimosis syndrome
3. Aponeurotic
   • involutional
   • postoperative
4. Mechanical
   • dermatochalasis
   • tumors
   • edema
   • anterior orbital lesions
   • scarring

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Inevitable ptosis of the eyelid

The inevitable ptosis of the eyelid is caused by a violation of the innervation of the third pair of cranial nerves and paralysis of the n. oenlosympathetic nerve.

Cranial nerve aplasia syndrome III

The syndrome of aplasia of the third pair of cranial nerves can be congenital or acquired due to paresis of the oculomotor nerve, the latter cause being more common.

Symptoms of III cranial nerve aplasia syndrome

Pathological movements of the upper eyelid accompanying movements of the eyeball.

Treatment of III cranial nerve aplasia syndrome

Resection of the levator tendon and suspension to the eyebrow.

Myogenic ptosis of the eyelid

Myogenic ptosis of the eyelid occurs due to myopathy of the levator of the eyelid or deterioration of neuromuscular transmission (neuromyopathy). Acquired myogenic ptosis occurs in myasthenia gravis, myotonic dystrophy and ocular myopathies.

Aponeurotic ptosis

Aponeurotic ptosis is caused by a delamination, avulsion of the tendon or stretching of the levator aponeurosis, which limits the transmission of force from the normal levator muscle to the upper eyelid. This pathology is most often based on age-related degenerative changes.

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Symptoms of aponeurotic ptosis of the eyelid

• 1. Usually bilateral ptosis of varying severity with good levator function.
  
  
  2. High fold of the upper eyelid (12 mm or more) because the posterior attachment of the aponeurosis to the tarsal cartilage is disrupted, while the anterior attachment to the skin remains intact and pulls the skin fold upward.
  3. In severe cases, the superior fold of the eyelid may be absent, the eyelid above the tarsal plate is thinned, and the superior groove is deepened.

Treatment of aponeurotic ptosis of the eyelid includes levator resection, reflexion, or reconstruction of the anterior levator aponeurosis.

Mechanical ptosis of the eyelid

Mechanical ptosis occurs when the upper eyelid is not moving properly. Causes include dermatochalasis, large eyelid tumors such as neurofibromas, scarring, severe eyelid edema, and anterior orbital lesions.

Causes of mechanical ptosis of the eyelid

Dermatochalasis

Dermatochalasis is a common, usually bilateral condition, occurring mainly in elderly patients and characterized by "excess" skin of the upper eyelid, sometimes combined with a herniation of the cellular tissue through a weakened orbital septum. Saccular sagging of the eyelid skin with atrophic folds is observed.

Treatment in severe cases involves removing the “excess” skin (blepharoplasty).

Blepharochalasis

Blepharochalasis is a rare disorder caused by recurring, painless, firm swelling of the upper eyelids that usually resolves spontaneously within a few days. The disorder begins during puberty with swelling that decreases in frequency over the years. In severe cases, the skin of the upper eyelid becomes stretched, sagging, and thins like tissue paper. In other cases, weakening of the orbital septum leads to herniation of the cellular tissue.

Atonic eyelid syndrome

Atonic ("flapping") eyelid syndrome is a rare, unilateral or bilateral disorder that often goes undiagnosed. This disorder occurs in very obese people who snore and have sleep apnea.

Symptoms of atonic ("flapping") eyelid

• Soft and flaccid upper eyelids.
• Eversion of the eyelids during sleep leads to damage to the exposed tarsal conjunctiva and chronic papillary conjunctivitis.

Treatment of flapping eyelid in mild cases involves using an eye protective ointment or an eyelid patch at night. In severe cases, horizontal eyelid shortening is required.

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Principles of surgical treatment of mechanical ptosis

Fasanella-Servat technique

• Indications: Moderate ptosis with levator function of at least 10 mm. Used in most cases of Horner syndrome and moderate congenital ptosis.
• Technique: The superior edge of the tarsal cartilage is excised together with the inferior edge of the Müller muscle and the overlying conjunctiva.

Levator resection

• Indications: Ptosis of varying degrees with levator function of at least 5 mm. The volume of resection depends on the levator function and the severity of ptosis.
• Technique: Shortening of the levator via an anterior (skin) or posterior (conjunctiva) approach.

Frontalis Suspension

Indications

• Marked ptosis (>4 mm) with very poor levator function (<4 mm).
• Marcus Gunn syndrome.
• Aberrant regeneration of the oculomotor nerve.
• Blepharophimosis syndrome.
• Complete paresis of the oculomotor nerve.
• Unsatisfactory result of previous levator resection.

Technique: Suspension of the tarsal cartilage to the frontalis muscle with a ligature made of the patient's own fascia lata or a non-absorbable synthetic material such as proline or silicone.

Restoration of aponeurosis

1. Indications: Aponeurotic ptosis with high levator function.
2. Technique: Relocation and suturing of the intact aponeurosis to the tarsal cartilage through an anterior or posterior approach.

Congenital ptosis of the eyelid

Congenital ptosis of the eyelid is a disease with an autosomal dominant type of inheritance, in which isolated dystrophy of the muscle that raises the upper eyelid (myogenic) develops, or there is aplasia of the nucleus of the oculomotor nerve (neurogenic). A distinction is made between congenital ptosis with normal function of the superior rectus muscle of the eye (the most common type of congenital ptosis) and ptosis with weakness of this muscle. Ptosis is often unilateral, but can manifest itself in both eyes. With partial ptosis, the child lifts the eyelids using the frontal muscles and throws back the head (the "stargazer" pose). The superior palpebral groove is usually weakly expressed or absent. When looking straight ahead, the upper eyelid is drooping, and when looking down, it is located higher than the opposite one.

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Symptoms of congenital ptosis

1. Unilateral or bilateral ptosis of varying severity.
2. Absence of the superior palpebral fold and decreased levator function.
3. When looking down, the ptotic eyelid is located higher than the healthy one due to insufficient relaxation of the levator muscle; with acquired ptosis, the affected eyelid is located at the level of or below the healthy one.

Treatment of congenital ptosis

Treatment should be carried out in preschool age after all necessary diagnostic procedures have been carried out. However, in severe cases, it is recommended to start treatment at an earlier age to prevent amblyopia. In most cases, resection of the levator is required.

Palpebromandibular syndrome (Gunn's syndrome) is a rare congenital, usually unilateral ptosis associated with synkinetic retraction of the drooping upper eyelid upon stimulation of the pterygoid muscle on the side of ptosis. Involuntary raising of the drooping upper eyelid occurs during chewing, opening the mouth or yawning, and abduction of the lower jaw to the side opposite to ptosis may also be accompanied by retraction of the upper eyelid. In this syndrome, the muscle that raises the upper eyelid receives innervation from the motor branches of the trigeminal nerve. Pathological synkinesis of this type is caused by lesions of the brainstem, often complicated by amblyopia or strabismus.

Marcus Gunn Syndrome

Marcus Gunn syndrome (palpebromandibular) is found in approximately 5% of cases of congenital ptosis, in most cases it is unilateral. Although the etiology of the disease is unclear, pathological innervation of the levator palpebrae by the motor branch of the trigeminal nerve is assumed.

Symptoms of Marcus Gunn Syndrome

1. Retraction of the drooping eyelid with irritation of the ipsilateral pterygoid muscle during chewing, opening the mouth, or separating the jaw in the direction opposite to ptosis.
2. Less common stimulations include jaw thrusting, smiling, swallowing, and clenching of teeth.
3. Marcus Gunn syndrome does not go away with age, but patients are able to mask it.

Treatment of Marcus Gunn Syndrome

It is necessary to decide whether the syndrome and the associated ptosis are a significant functional or cosmetic defect. Although surgical treatment does not always achieve satisfactory results, the following methods are used.

1. Unilateral levator resection in moderate cases with levator function of 5 mm or greater.
2. Unilateral separation and resection of the levator tendon with insilateral suspension to the eyebrow (frontalis muscle) in more severe cases.
3. Bilateral division and resection of the levator tendon with insilateral suspension to the eyebrow (frontalis muscle) to achieve a symmetrical result.

Blepharophimosis

Blepharophimosis is a rare developmental anomaly caused by shortening and narrowing of the palpebral sulcus, bilateral ptosis, with an autosomal dominant type of inheritance. It is characterized by weak function of the muscle that lifts the upper eyelid, epicanthus, and eversion of the lower eyelid.

Symptoms of Blepharophimosis

1. Symmetrical ptosis of varying severity with insufficiency of the levator function.
2. Shortening of the palpebral fissure in the horizontal direction.
3. Telecanthus and inverted epicanthus.
4. Lateral ectropion of the lower eyelids.
5. Poorly developed nasal bridge and hypoplasia of the superior orbital rim.

Treatment of blepharophimosis

Treatment of blepharophimosis includes initial correction of epicanthus and telecanthus, after a few months bilateral frontal fixation is performed. It is also important to treat amblyopia, which can occur in approximately 50% of cases.

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Acquired ptosis of the eyelid

Acquired ptosis of the eyelid is observed much more often than congenital. Depending on the origin, neurogenic, myogenic, aponeurotic and mechanical acquired ptosis are distinguished.

Neurogenic ptosis of the eyelid with paralysis of the oculomotor nerve is usually unilateral and complete, most often caused by diabetic neuropathy and intracranial aneurysms, tumors, injuries and inflammation. With complete paralysis of the oculomotor nerve, pathology of the extraocular muscles and clinical manifestations of internal ophthalmoplegia are determined: loss of accommodation and pupillary reflexes, mydriasis. Thus, an aneurysm of the internal carotid artery inside the cavernous sinus can lead to complete external ophthalmoplegia with anesthesia of the innervation area of the eye and the infraorbital branch of the trigeminal nerve.

Eyelid ptosis can be induced for protective purposes in the treatment of corneal ulcers that do not heal due to the non-closing palpebral fissure in lagophthalmos. The effect of chemical denervation of the muscle raising the upper eyelid with botulinum toxin is temporary (about 3 months), and is usually sufficient to stop the corneal process. This method of treatment is an alternative to blepharorrhea (eyelid suturing).

Eyelid ptosis in Horner's syndrome (usually acquired, but can also be congenital) is caused by a disturbance of the sympathetic innervation of the smooth muscle of Müller. This syndrome is characterized by some narrowing of the palpebral fissure due to drooping of the upper eyelid by 1-2 mm and a slight lifting of the lower eyelid, miosis, and impaired sweating on the corresponding half of the face or eyelids.

Myogenic ptosis of the eyelid occurs with myasthenia, is often bilateral, and may be asymmetrical. The severity of ptosis varies from day to day, it is provoked by exertion and may be combined with double vision. The endorphin test temporarily eliminates muscle weakness, corrects ptosis, and confirms the diagnosis of myasthenia.

Aponeurotic ptosis is a very common type of age-related ptosis; it is characterized by the fact that the tendon of the muscle that raises the upper eyelid partially detaches from the tarsal (cartilage-like) plate. Aponeurotic ptosis can be post-traumatic; it is believed that in a large number of cases, post-operative ptosis has this mechanism of development.

Mechanical ptosis of the eyelid occurs with horizontal shortening of the eyelid due to tumor or cicatricial origin, as well as in the absence of the eyeball.

In preschool children, ptosis leads to persistent vision loss. Early surgical treatment of severe ptosis can prevent the development of amblyopia. In case of poor mobility of the upper eyelid (0-5 mm), it is advisable to suspend it to the frontal muscle. In case of moderate eyelid excursion (6-10 mm), ptosis is corrected by resection of the muscle that raises the upper eyelid. In case of a combination of congenital ptosis and dysfunction of the superior rectus muscle, resection of the levator tendon is performed in a larger volume. High eyelid excursion (more than 10 mm) allows resection (duplication) of the levator aponeurosis or Müller muscle.

Treatment of acquired pathology depends on the etiology and extent of ptosis, as well as on the mobility of the eyelid. A large number of methods have been proposed, but the principles of treatment remain unchanged. Neurogenic ptosis in adults requires early conservative treatment. In all other cases, surgical treatment is advisable.

If the eyelid is drooping by 1-3 mm and has good mobility, a transconjunctival resection of the Müller muscle is performed.

In cases of moderate ptosis (3-4 mm) and good or satisfactory eyelid mobility, surgery on the muscle that lifts the upper eyelid is indicated (tendon plasty, refixation, resection or duplication).

With minimal eyelid mobility, it is suspended from the frontal muscle, which ensures mechanical lifting of the eyelid when raising the eyebrow. The cosmetic and functional results of this operation are worse than the effect of interventions on the upper eyelid levators, but this category of patients has no alternative to suspension.

For mechanical lifting of the eyelid, it is possible to use special bows fixed to the frames of glasses, or use special contact lenses. Usually, these devices are poorly tolerated, so they are very rarely used.

With good eyelid mobility, the effect of surgical treatment is high and stable.

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Clinical features of ptosis

Congenital and acquired ptosis differ in the patient's age when the pathology appeared and the duration of its course. In doubtful cases, old photographs of the patient may be useful. It is also important to learn about possible manifestations of systemic diseases, such as associated diplopia, differences in the degree of ptosis during the day or against the background of fatigue.

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Pseudoptosis

The following pathologies can be mistaken for ptosis.

• Insufficient support of the eyelids by the eyeball due to a decrease in the volume of the orbital contents (artificial eye, microphthalmos, enophthalmos, phthisis of the eyeball).
• Contralateral eyelid retraction is detected by comparing the levels of the upper eyelids, taking into account that the upper eyelid normally covers the cornea by 2 mm.
• Ipsilateral hypotrophy, in which the upper eyelid droops downwards, following the eyeball. Pseudoptosis disappears if the patient fixes his gaze with the hypotrophic eye while the healthy eye is closed.
• Eyebrow ptosis due to "excess" skin above the eyebrow or due to facial nerve paralysis, which can be detected by lifting the eyebrow with your hand.
• Dermatochalasis, in which "excess" skin of the upper eyelids causes the formation of normal or pseudoptosis.

Measurements

• Distance edge of the eyelid - reflex. This is the distance between the upper edge of the eyelid and the corneal reflection of the beam of a pen-flashlight, which the patient is looking at.
• The height of the palpebral fissure is the distance between the upper and lower edges of the eyelid, measured in the meridian passing through the pupil. The edge of the upper eyelid is usually located approximately 2 mm below the superior limbus, the lower eyelid - 1 mm or less above the inferior limbus. In men, the height is less (7-10 mm) than in women (8-12 mm). Unilateral ptosis is assessed by the difference in height with the co-lateral side. Ptosis is classified as mild (up to 2 mm), moderate (3 mm), and severe (4 mm or more).
• Levator function (upper eyelid excursion). Measured by holding the patient's eyebrow with the thumb while the patient looks down to exclude the action of the frontal muscle. Then the patient looks up as much as possible, the eyelid excursion is measured with a ruler. Normal function is 15 mm or more, good is 12-14 mm, sufficient is 5-11 mm and insufficient is 4 mm or less.
• The superior palpebral groove is the vertical distance between the edge of the eyelid and the lid crease when looking downwards. In women, it is approximately 10 mm. In men, it is 8 mm. The absence of a fold in a patient with congenital ptosis is an indirect sign of levator insufficiency, while a high fold indicates a defect in the aponeurosis. The skin fold serves as a marker for the initial incision.
• Pretarsal distance is the distance between the edge of the eyelid and the fold of skin when fixating a distant object.

Associative features

1. Increased innervation may affect the levator on the side of ptosis, especially when looking up. Combined increased innervation of the contralateral intact levator results in upward traction of the eyelid. It is necessary to lift the eyelid affected by ptosis with a finger and observe the descent of the intact eyelid. In this case, the patient should be warned that surgical correction of ptosis may stimulate drooping of the contralateral eyelid.
2. The fatigue test is performed for 30 seconds without the patient blinking. Progressive drooping of one or both eyelids or inability to direct the gaze downwards are pathognomonic signs of myasthenia. In myasthenic ptosis, a deviation of the upper eyelid on saccades from downward gaze to straight ahead gaze (Cogan twitching symptom) or a "jump" when looking to the side is detected.
3. Impaired ocular motility (especially superior rectus dysfunction) should be evaluated in patients with congenital ptosis. Correction of ipsilateral hypotrophy may improve ptosis.
4. Palpebromandibular syndrome is detected if the patient makes chewing movements or drops the jaw to the side.
5. The Bell phenomenon is examined by holding the patient's open eyelids with the hands; when attempting to close the eyes, an upward movement of the eyeball is observed. If the phenomenon is not expressed, there is a risk of postoperative exposure keratopathies, especially after large levator resections or suspension techniques.