Pediatric Ptosis

Pediatric Ptosis

Ptosis is the abnormal drooping of the upper eyelid that results in narrowing of the palpebral fissure. In children, it can lead to amblyopia, astigmatism, and impaired visual development if not addressed promptly.

Key Points

Affects 1 in 842 births
Can be unilateral or bilateral
Critical period for visual development: first 7-8 years
Early intervention prevents amblyopia
Associated with various systemic conditions

Types of Pediatric Ptosis

1. Congenital Ptosis

Simple Congenital Ptosis: Most common form (90%)
- Dystrophic levator muscle
- Isolated abnormality
- Usually unilateral (70%)
Complex Congenital Ptosis:
- Associated with other ocular or systemic abnormalities
- Part of genetic syndromes

2. Acquired Ptosis

Neurogenic:
- Third nerve palsy
- Horner syndrome
- Myasthenia gravis
Mechanical:
- Lid masses
- Trauma
- Post-surgical
Myogenic:
- Chronic progressive external ophthalmoplegia
- Muscular dystrophies

Clinical Presentation

Common Signs

Drooping of upper eyelid
Chin-up head posture
Frontalis muscle overaction
Asymmetric lid creases
Poor levator function

Associated Features

Marcus Gunn Jaw-Winking:
- Synkinetic movement between eyelid and jaw
- Present in 5% of congenital ptosis
Bell's Phenomenon: Upward rotation of globe on attempted closure
Strabismus: Present in 30% of cases

Complications

Amblyopia (especially in unilateral cases)
Astigmatism
Impaired binocular vision
Psychosocial impact
Neck pain from compensatory head posture

Clinical Evaluation

Essential Measurements

Margin Reflex Distance (MRD):
- MRD1: Distance from upper lid margin to corneal light reflex
- MRD2: Distance from lower lid margin to corneal light reflex
- Normal MRD1: 4-5mm
Levator Function:
- Measured in millimeters
- Poor: 0-4mm
- Fair: 5-7mm
- Good: >8mm
Palpebral Fissure Height
Lid Crease Position

Additional Examinations

Visual acuity assessment
Pupillary examination
Ocular motility
Bell's phenomenon
Corneal sensation
Fatiguability testing

Treatment Approaches

Conservative Management

Observation in mild cases
Amblyopia monitoring and treatment
Regular follow-up

Surgical Management

Timing:
- Early intervention if amblyopia risk
- Usually between 3-5 years
- Emergency surgery if corneal exposure
Surgical Options:
- Frontalis Sling:
  - For poor levator function (<4mm)
  - Materials: Fascia lata, silicone, Gore-Tex
- Levator Resection:
  - For moderate to good levator function
  - Amount based on severity
- Whitnall Sling: For moderate cases
- Mullerectomy: For mild cases

Associated Syndromes and Conditions

Neurogenic Associations

Congenital Fibrosis Syndrome:
- Multiple ptosis variants
- Restricted eye movements
- Autosomal dominant inheritance
Myasthenia Gravis:
- Fluctuating ptosis
- Fatigability
- Response to anticholinesterase

Systemic Syndromes

Blepharophimosis Syndrome:
- Severe bilateral ptosis
- Telecanthus
- Epicanthus inversus
- FOXL2 gene mutation
Mitochondrial Disorders:
- Progressive external ophthalmoplegia
- Kearns-Sayre syndrome
- MELAS syndrome
Other Associated Conditions:
- Turner syndrome
- Marfan syndrome
- Duane retraction syndrome
- Horner syndrome

Further Reading