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CONGENITAL ABNORMALITIES OF THE EYE- CONGENITAL PTOSIS (Medical Mangement,…
CONGENITAL ABNORMALITIES OF THE EYE- CONGENITAL PTOSIS
Definition
This is a dropping of eyelid which is present at birth and which may be due to congenital third nerve palsy
Medical Mangement
Mild cases of congenital ptosis can be observed if there are no amblyopia, strabismus, or abnormal head posture.
Depending on the severity of the congenital ptosis, patients should be monitored every 3-12 months for signs of amblyopia due to congenital ptosis.
External photographs can be helpful in monitoring patients and their head posture. If a child with previous chin-up head posture stops this behavior, amblyopia should be suspected.
To prevent amblyopia, children with severe ptosis should have their lid taped up for several hours of the waking day while awaiting surgical repair. Children with ptosis should also be checked for astigmatism.
Pathophysiology
The eyelids are elevated by the contraction of the levator palpebrae superioris. In most cases of congenital ptosis, a droopy eyelid results from a localized myogenic dysgenesis. Rather than normal muscle fibers, fibrous and adipose tissues are present in the muscle belly, diminishing the ability of the levator to contract and relax. Therefore, the condition is commonly called congenital myogenic ptosis.
Causes
Blepharophimosis syndrome: This condition consists of short palpebral fissures, congenital ptosis, epicanthus inversus, and telecanthus. The condition is autosomal dominant and may be associated with forkhead box L2 gene defects and, occasionally, ovarian dysfunction. [5]
Third cranial nerve palsy: Signs of aberrant regeneration such as lid gaze synkinesis may be present. The pupil may be enlarged or paradoxically small and nonreactive.
Horner syndrome: Ipsilateral findings of mild ptosis, miosis, and anhidrosis characterize this syndrome. The ipsilateral lower eyelid may be elevated. Also, because of the lack of sympathetic innervation to the iris melanocyte development, a difference in the iris color between the eyes may result (called heterochromia).
Marcus Gunn jaw-winking syndrome: The motor nerve to the external pterygoid muscle is misdirected to the ipsilateral levator muscle. Lid elevation occurs with mastication or with movement of the jaw to the opposite side.
Birth trauma
Diagnostic Studies
Physical Examination
Visual acuity, refractive error, and cycloplegic refraction should be recorded. In infants, the surgeon should make sure that the baby can fixate and follow objects with each eye individually.
The patient should be evaluated for strabismus (misalignment) and undergo a dilated fundus examination.
Serial external photographs of the eyes and the face may be included in the patient's record for documentation.
Corneal sensitivity should be tested if possible. This may be a difficult test in young pediatric patients.
An exophthalmometer can be used to assess relative proptosis or enophthalmos of each eye. In pseudoptosis, a proptosis of the contralateral eye gives the false impression that the normal upper eyelid is droopy.
Labarotory Studies
check serum acetylcholine receptor antibody levels.
Imaging Studies
MRI, CT of the orbit and brain
Surgical Mangement
Levator muscle resection
This procedure is the shortening of the levator-aponeurosis complex through a lid-crease incision. The skin incision is hidden either in the existing lid fold or in a new lid fold created to match that of the contralateral eyelid.
Frontalis suspension procedure
This procedure is designed to augment the patient's lid elevation through brow elevation. Frontalis suspension procedures produce lagophthalmos in most cases. Some surgeons may perform a bilateral suspension procedure for severe unilateral congenital ptosis to obtain symmetry.
Risk Factors
eyelid tumors, cysts, or swelling
Horner's syndrome
muscular problems
nerve damage in the eye muscles
neurological conditions
Signs and Symptoms
The main symptom of ptosis is the droopy eyelid itself.
Nursing Care Plan
