Management of Congenital Cataracts

The treatment and care for congenital cataracts necessitates a comprehensive, multidisciplinary approach, tailored to each child’s unique circumstances and the underlying cause of the cataracts.

Aims of Management

The overarching goals of managing congenital cataracts are:

• Lens Opacity Removal & Vision Restoration: To surgically remove the clouded lens and restore clear vision in the affected eye(s), allowing for proper visual input.

• Cognitive Development: To facilitate the child’s attainment of typical cognitive development milestones, which can be affected by visual impairment.

• Sensory Awareness: To ensure the child recognizes and responds appropriately to their sensory needs, especially visual input.

• Injury Prevention: To minimize the risk of accidental injury due to impaired vision and coordination.

• Anxiety Reduction: To lessen anxiety and distress in both the child and family related to the condition and its management, providing reassurance and support.

• Education for Adaptive Living: To provide thorough education and training to the child and family on strategies to cope with altered visual abilities and maximize functional independence.

Generally, early intervention is crucial, especially for younger children, to mitigate the risk of amblyopia (lazy eye).

For optimal visual development in infants, it is generally recommended that:

• Visually significant unilateral congenital cataracts be detected and surgically removed before 6 weeks of age.

• Visually significant bilateral congenital cataracts should ideally be removed before 10 weeks of age.

However, some congenital cataracts are too small to impede vision. In such cases:

• No immediate surgery or treatment is necessary.

• If the cataracts are superficial and minor, an ophthalmologist will monitor them throughout the patient’s life to detect any changes or progression.

• Patients with small, non-visually significant congenital cataracts may, in many cases, develop visually significant cataracts later in adulthood, often decades later.

Congenital cataracts are a significant cause of visual impairment in infancy, but also one of the most treatable, with an estimated prevalence of 1 to 6 cases per 10,000 live births.

Nursing Assessment:

• Recent Medication Review: Inquire about the patient’s recent medication intake, as anticoagulant therapy may need to be temporarily discontinued before surgery to minimize the risk of bleeding behind the eye (retrobulbar hemorrhage).

• Preoperative Testing Review: Check for completion and results of standard preoperative tests, which may be ordered based on the patient’s overall health and medical history. These tests can include:

  • Complete blood count (CBC)

  • Electrocardiogram (ECG)

  • Urinalysis

• Vital Signs Monitoring: Confirm stable vital signs (temperature, pulse, respiration, blood pressure) prior to surgery, ensuring the patient is medically fit for the procedure.

• Visual Acuity Test Results: Review the results of visual acuity tests, such as Snellen’s chart (for older children), to establish a baseline and understand the extent of visual impairment.

• Patient Medical History: Thoroughly assess the patient’s medical history to identify any pre-existing conditions or factors that may influence preoperative testing requirements or surgical planning.

Nursing Diagnoses:

Based on the assessment, relevant nursing diagnoses may include:

• Disturbed Visual Sensory Perception: Related to altered sensory reception or changes in sensory organ status, as evidenced by the patient using hands to explore or locate objects in their environment.

• Risk for Trauma: Related to compromised vision and reduced hand-eye coordination, increasing the likelihood of accidental injuries.

• Anxiety: Related to the potential for permanent vision loss or dependence, as evidenced by patient restlessness and expressed concerns about vision.

Medical Management: Pharmacologic Therapy:

• Pupil Dilating Drops: Administered both before and after surgery to:

  • Dilate the pupil (mydriasis), facilitating surgical access to the lens.

  • Reduce postoperative inflammation and discomfort.

• Antibiotic Medications: Administered prophylactically to:

  • Prevent postoperative infection, a potential complication of eye surgery.

  • Minimize inflammation within the eye following surgery.

• Intravenous (IV) Sedation: Sedation may be used preoperatively to:

  • Reduce patient anxiety and apprehension before surgery.

  • Minimize discomfort associated with preoperative procedures, especially in young children.

Surgical Intervention:

• Surgery as Primary Treatment: Surgery is the fundamental and most effective treatment for congenital cataracts that are visually significant.

• Pediatric Ophthalmologist Expertise: The surgical procedure is typically performed by pediatric ophthalmologists, who have specialized training and experience in cataract surgery in children.

• Cataract Removal Procedure: During surgery:

  • A small surgical incision is carefully made in the eye.

  • The cloudy lens (cataract) is meticulously removed from the eye.

• Additional Procedures (Possible): In certain cases, depending on the specific needs of the child and the surgical approach, additional procedures may be required, such as:

  • Intraocular lens (IOL) implantation at the time of cataract removal.

  • Capsulotomy (creating an opening in the lens capsule) to improve visual axis clarity.

Lens Replacement Options:

Various lens replacement options are available after cataract removal, each with advantages and considerations:

• Phacoemulsification: This common technique involves:

  • Removing a portion of the anterior lens capsule (anterior capsulotomy).

  • Using ultrasound energy to emulsify and aspirate the lens nucleus and cortex (the cloudy part of the cataract).

  • Leaving the posterior lens capsule and zonular support structures intact.

  • Lens replacement options following phacoemulsification include:

    • Aphakic glasses: Thick eyeglasses to correct vision without a lens.

    • Contact lenses: Custom-fitted contact lenses for visual correction.

    • Intraocular lens (IOL) implants: Artificial lenses surgically implanted in the eye to restore focusing power.

• Extracapsular Cataract Extraction (ECCE): A surgical technique where:

  • The anterior lens capsule is removed.

  • The lens nucleus and cortex are extracted from the eye.

  • The posterior lens capsule is intentionally left intact, providing support for potential IOL implantation.

• Intracapsular Cataract Extraction (ICCE): An older technique, less commonly used now, where:

  • The entire lens, including its intact capsule, is surgically removed.

Timing of Surgical Intervention:

• Individualized Timing: The optimal timing for cataract surgery is determined based on several factors, ensuring the best possible visual outcomes for the child. These factors include:

  • The child’s age at diagnosis.

  • The severity of the cataract and its impact on vision.

  • The presence of any other associated eye conditions or health issues.

• Early Surgery Recommendation: Generally, early surgical intervention is recommended for visually significant congenital cataracts to:

  • Prevent visual deprivation amblyopia (lazy eye), which can develop if vision is impaired early in life.

  • Promote normal visual development during critical periods of visual pathway maturation in infancy.

• Delayed Surgery (Selective Cases): In certain specific situations, surgical intervention may be intentionally delayed, for example:

  • When there are other underlying medical conditions that need to be stabilized or addressed first before undergoing eye surgery.

Correction of Refractive Errors:

• Post-Surgery Refractive Changes: Following cataract surgery, children frequently develop refractive errors, as the eye’s natural lens has been removed. Common refractive errors include:

  • Nearsightedness (myopia)

  • Farsightedness (hyperopia)

• Corrective Measures: To optimize visual acuity after surgery, corrective measures are necessary, such as:

  • Prescription eyeglasses, with lenses tailored to correct the refractive error.

  • Contact lenses, offering a wider field of vision and potentially better visual acuity in some cases, particularly for younger children or unilateral cataracts.

Amblyopia Management:

• Amblyopia Risk: Amblyopia, also known as “lazy eye,” is a common and significant complication associated with congenital cataracts.

• Mechanism of Amblyopia: Amblyopia develops when the brain favors one eye over the other due to early visual deprivation in the eye with the cataract. The visual pathways for the weaker eye do not develop properly.

• Amblyopia Treatment: Treatment strategies are essential to encourage visual development in the weaker eye and may include:

  • Eye Patching: Patching the stronger, better-seeing eye to force the child to use the weaker, amblyopic eye, stimulating its visual pathway development.

  • Blurring the Stronger Eye: Using eye drops or special lenses to temporarily blur vision in the stronger eye, achieving the same effect as patching by encouraging use of the weaker eye.

Regular Follow-up and Monitoring:

• Long-Term Care is Essential: Children with congenital cataracts require lifelong regular follow-up and ongoing monitoring by a pediatric ophthalmologist to ensure optimal visual outcomes and detect any complications.

• Regular Eye Examinations: Scheduled eye examinations are crucial for:

  • Assessing visual acuity at regular intervals to track visual development.

  • Monitoring for any potential postoperative complications, such as glaucoma, retinal detachment, or lens reproliferation.

  • Adjusting treatment plans as needed, including changes in glasses prescriptions, contact lens fitting, or amblyopia therapy adjustments, to optimize vision throughout childhood and adolescence.