Lagophthalmia–Cleft Lip and Palate Syndrome (LCCLP)

Dr. Huma Q. Rana, MD - Clinical Genetics, Genomics, Cytogenetics, Biochemical Genetics Specialist Dr. Huma Q. Rana, MD - Clinical Genetics, Genomics, Cytogenetics, Biochemical Genetics Specialist
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Rx ENT, Oral and Dental Health (A - Z)

Lagophthalmia–cleft lip and palate syndrome (LCCLP) is a rare birth condition where a baby is born with two main problems together: (1) the eyelids do not fully close (lagophthalmia/lagophthalmos), and (2) there is a split in the lip and/or the roof of the mouth (cleft lip and palate). Because the eyelids stay partly open, the surface of the eye can dry out and get injured. Because the lip or palate has a gap, the baby may have trouble feeding, speaking, and growing. Some children also have wide eyelid openings (megaloblepharon), inward‑growing extra eyelashes (distichiasis), or lower‑lid turning out (ectropion). Early, team‑based care protects the cornea, supports feeding, and plans surgery for the lip and palate. (Refs: PubMed 1995 craniofacial series; AAO EyeNet/EyeWiki exposure keratopathy; ACPA care resources.)

Lagophthalmia–Cleft Lip and Palate Syndrome (LCCLP) means the eyelids do not close fully. In this rare syndrome, babies are born with incomplete eyelid closure together with a cleft lip and usually a cleft palate. In the largest early case series that first drew attention to this pattern, every patient had bilateral (both-sided) cleft lip and palate plus lagophthalmia; many also had very large eyelid openings (megaloblepharon), inward-growing extra lash rows (distichiasis), and outward turning of lower lids (ectropion). These recurring features suggested a distinct craniofacial syndrome rather than random coincidence. PubMed

“Lagophthalmia” itself is simply the term for not being able to close the eyelids completely—a problem that risks eye dryness, exposure keratopathy, and vision loss if not protected and treated. NCBI+1

Ophthalmic reviews of children with cleft lip/palate show that eyelid malpositions—lagophthalmos, retraction, and euryblepharon—can co-occur with facial clefting, reinforcing that this eye–face combination has biological roots during early development. PMC+1

Because features overlap with neighboring conditions such as Blepharocheilodontic (BCD) syndrome and Ankyloblepharon–Ectodermal defects–Cleft lip/palate (AEC) syndrome, many experts see these entities on a clinical spectrum of eyelid–lip–palate disorders. PMC+3Orpha+3PubMed+3

Other names

Doctors and articles might use several labels. You may encounter:

  • “Lagophthalmia with bilateral cleft lip and palate” or “lagophthalmia–clefting syndrome”—descriptive names used in the original series that framed this as a distinct pattern. PubMed

  • Blepharocheilodontic (BCD) spectrum—when eyelid, lip/palate, and tooth anomalies cluster with marked lagophthalmos/euryblepharon. Orpha

  • AEC (Hay–Wells) syndrome—an ectodermal dysplasia with ankyloblepharon and CL/P that can share eyelid–lip features; some authors view overlap across these diagnoses. MedlinePlus+2PMC+2

These names reflect overlapping features rather than entirely separate diseases; your clinical team will focus on the features present in your child and the care they require.

Why does this syndrome happen?

During early pregnancy, facial and eyelid structures form from tissues that must grow, fuse, and fold precisely. Failures of fusion of embryonic lid folds and facial processes can produce congenital eyelid defects (including lagophthalmia, eyelid coloboma, euryblepharon) together with cleft lip and/or palate. Environmental disruptions (like amniotic bands) can also mechanically disturb lid development. omjournal.org

Genetically, many cleft lip/palate conditions relate to genes that guide craniofacial development—e.g., IRF6 (Van der Woude syndrome), PVRL1, and TBX22—and some ectodermal-dysplasia syndromes (like AEC) add eyelid abnormalities to the picture. This supports a shared developmental pathway connecting the eyelid and orofacial cleft findings in some families. Children’s Hospital of Philadelphia+2PMC+2

A key point: lagophthalmia itself is simply the eyelids not closing fully; in newborn syndromic cases it is congenital, while in older children or adults it can also arise from facial nerve palsy, trauma, or surgery. NCBI+1

Types

There isn’t a universally accepted “type I/II” system. Clinicians tend to group cases by what most affects care. Helpful “types” in day-to-day practice are:

  1. Isolated lagophthalmia–clefting pattern
    Classic combination of lagophthalmia with bilateral cleft lip and palate without broader ectodermal problems. The original series largely fits here. PubMed

  2. BCD-spectrum presentation
    Lagophthalmia with euryblepharon, eyelid retraction, ectropion, and dental anomalies, sometimes severe ocular exposure—management often needs staged eyelid reconstruction plus standard cleft care. AJO+1

  3. AEC-spectrum presentation
    Lagophthalmia or ankyloblepharon occurs with skin, hair, nail, and sweat-gland changes typical of ectodermal dysplasia, along with cleft lip/palate. Dermatologic and wound-healing issues influence surgery timing. MedlinePlus+1

  4. Severity-graded lagophthalmia
    Mild (small exposure gap), moderate, or severe (frank exposure keratopathy or corneal ulcer risk). This grading guides how urgently to lubricate, tape lids, place moisture chambers, or operate.

  5. Laterality pattern
    Bilateral involvement is common in reports, but the eyelid findings can be asymmetric; clefting may be bilateral or unilateral. Care plans adapt to the sides involved. PubMed

These practical “types” help families understand why treatment plans can differ even among children with similar diagnose

Clinical Presentation Patterns)

  1. Lagophthalmia with bilateral cleft lip and complete cleft palate: classic pattern with large eyelid opening at rest and feeding problems. (Refs: PubMed craniofacial series.)
  2. Lagophthalmia with megaloblepharon and distichiasis: very wide eyelid fissures plus extra lash row that rubs the cornea. (Refs: PubMed case series.)
  3. Lagophthalmia with lower‑lid ectropion: outward lower lid leads to poor tear spread and exposure. (Refs: AAO lagophthalmos articles.)
  4. Lagophthalmia with incomplete cleft palate only: speech and middle ear issues dominate; eye protection still vital. (Refs: ACPA booklet.)
  5. Lagophthalmia with isolated cleft lip: feeding and cosmetic concerns with less airway impact; corneal protection remains key. (Refs: ACPA policy.)

Causes

These “causes” describe underlying reasons or contributors to the combined syndrome or its core features. Not every child has all of them.

  1. Primary developmental pattern (idiopathic) – The combination appears without a pinpointed gene but follows the distinct pattern described in case series. PubMed

  2. Blepharocheilodontic (BCD) spectrum – Eyelid malformations (euryblepharon, ectropion), dental anomalies, and clefting with severe lagophthalmos. Orpha

  3. AEC (Hay–Wells) syndrome – An ectodermal dysplasia with ankyloblepharon and cleft lip/palate; eyelid closure problems may accompany it. MedlinePlus

  4. Overlap/continuum of BCD and lagophthalmos syndromes – Some patients show features of both, suggesting a shared spectrum. PubMed

  5. IRF6 pathway disorders (e.g., Van der Woude) – IRF6 mutations cause common CL/P syndromes and may co-occur with eyelid anomalies in some families. Children’s Hospital of Philadelphia

  6. Other cleft-related genes (TBX22, PVRL1, etc.) – Genes shaping palate and lip development can contribute to broader craniofacial patterns that include eyelids. PMC

  7. Failure of embryonic eyelid-fold fusion – Direct developmental mechanism leading to eyelid coloboma or malposition, causing exposure. omjournal.org

  8. Euryblepharon – Excessively wide palpebral fissures stretch lids and prevent full closure. Often part of the spectrum. AJO

  9. Eyelid retraction – Lids are held too high (upper) or too low (lower), creating a gap during blinking and sleep. PMC

  10. Ectropion – Outward turning of the lower lid margin prevents a good seal and worsens dryness. PubMed

  11. Distichiasis – Extra lash row irritates the cornea; protective squinting can’t compensate when lids don’t close fully. PubMed

  12. Megaloblepharon – Abnormally large eyelid openings magnify exposure. PubMed

  13. Associated craniofacial syndromes (e.g., Goldenhar) – Some craniofacial syndromes include eyelid coloboma and clefting with exposure risk. EyeWiki

  14. Uveal/eyelid coloboma – Missing segments of lid or ocular tissues impair coverage and lubrication, especially during sleep. PMC

  15. Amniotic band disruption – Mechanical interference with lid development can leave gaps or malposition. omjournal.org

  16. Surface eye disease feedback – Exposure → dryness → inflammation → scarring → further eyelid malposition if untreated (a worsening loop). Cleveland Clinic

  17. Surgical scarring from early procedures – Rarely, necessary early surgeries around the lids or face may influence lid position; careful staging minimizes this.

  18. Neuromuscular imbalance – Even with normal nerves, thin, retracted lids may not generate an adequate blink seal.

  19. Connective-tissue laxity – In euryblepharon/ectropion, lax tissues don’t hold position, limiting closure. AJO

  20. Family clustering – Some families show multiple affected members, suggesting heritable contributors even when a single gene test is negative. PubMed

Common symptoms and signs

  1. Eyes that don’t shut fully, especially during sleep—parents may see a sliver of exposed eye. (Core feature.) NCBI

  2. Dry, irritated eyes—redness, burning, or frequent rubbing from exposure. Cleveland Clinic

  3. Excess tearing—paradoxically, dryness triggers reflex tears that spill over.

  4. Light sensitivity—an exposed cornea is more sensitive to light.

  5. Blurry vision fluctuating during the day—worse when the surface dries.

  6. Stringy discharge or crusting—from surface inflammation.

  7. Recurrent “pink eye” or keratitis—surface infections exploit exposure. Cleveland Clinic

  8. Pain with blinking—a dry cornea makes blinking uncomfortable.

  9. Frequent blinking or eye squeezing—trying to wet the surface.

  10. Visible eyelid malposition—wide openings, outward lower lids, or pulled-up lids. PMC

  11. Extra eyelashes rubbing the eye (distichiasis)—causing irritation. PubMed

  12. Cleft lip and/or palate—feeding and speech challenges from the facial clefting component.

  13. Slow corneal healing—minor scratches take longer to settle with exposure.

  14. Reduced night protection—waking with sticky, painful eyes.

  15. Vision risk if untreated—scarring or ulcers in severe cases. Cleveland Clinic

Diagnostic tests

A) Physical examination (bedside and clinic)

  1. External facial and eyelid inspection
    The ophthalmologist measures the eye opening (palpebral fissure), checks for euryblepharon, retraction, ectropion, and extra lashes. This visual exam establishes the severity and type of malposition that causes lagophthalmia. PMC+1

  2. Blink completeness and lag assessment
    The clinician asks the child (or observes during sleep) whether eyelids meet. Any persistent gap confirms lagophthalmia and guides urgency of lubrication and protection. NCBI

  3. Corneal surface staining (fluorescein)
    A safe yellow dye highlights dry spots, scratches, or ulcers under blue light, showing how exposure is affecting the eye and whether urgent shielding is needed. Cleveland Clinic

  4. Tear film break-up time
    After a blink, the doctor times how quickly dry patches appear on the cornea. Rapid break-up means the surface is unstable, often from exposure.

  5. Eyelid laxity tests (snap-back/distraction)
    Gentle pulling on the lower lid checks tone. Lax lids that don’t snap back suggest ectropion or euryblepharon and support surgical planning. AJO

B) Targeted “manual” or focused functional tests

  1. Margin-reflex distance (MRD) and levator function
    Simple ruler/penlight measurements quantify retraction or ptosis and eyelid muscle action—key numbers for reconstructive surgery.

  2. Bell’s phenomenon check
    The clinician looks for protective upward eye roll on forced lid closure. Poor Bell’s response increases corneal risk in lagophthalmia.

  3. Eyelash evaluation for distichiasis/entropion
    Careful slit-lamp inspection identifies extra lash rows or in-turned lashes that worsen exposure damage and can be managed (e.g., epilation). PubMed

C) Laboratory and pathological assessments

  1. Genetic testing panels for clefting/ectodermal dysplasia
    Panels may include IRF6 (Van der Woude) and other craniofacial genes (e.g., TBX22, PVRL1) and, when appropriate, genes linked to ectodermal dysplasia (AEC). A positive result informs recurrence risk and multidisciplinary planning. Children’s Hospital of Philadelphia+2PMC+2

  2. Dermatologic/ectodermal workup (if AEC-spectrum suspected)
    Skin, hair, nail evaluation—sometimes with biopsy—documents ectodermal features that affect wound healing and surgical timing. MedlinePlus

  3. Corneal swabs or cultures (when infection suspected)
    If surface ulcers appear, targeted cultures guide antibiotics. Exposure makes infection more likely. Cleveland Clinic

  4. Nutritional labs if healing is poor
    Protein, iron, zinc, and vitamin A levels can be checked when epithelial healing lags; deficiencies are corrected to support the ocular surface.

D) Electrodiagnostic / neuro-ocular function

  1. Visual evoked potentials (VEP) in young or complex cases
    If structural clarity is threatened but standard vision testing is hard (infants), VEP helps gauge whether visual pathways receive good input.

  2. Electroretinography (ERG) if retinal issues suspected
    In rare overlapping syndromes (e.g., uveal coloboma), ERG can assess retinal function when routine exams are limited. PMC

  3. Facial nerve assessment (clinical ± EMG) when indicated
    Although syndromic lagophthalmia is not usually neurologic, documenting nerve function clarifies whether any facial weakness contributes to incomplete closure. Cleveland Clinic

E) Imaging and structural evaluation

  1. Slit-lamp biomicroscopy
    A microscope exam of the lids and cornea details exposure damage, lash position, and tear film—central to day-to-day management. Cleveland Clinic

  2. Anterior segment optical coherence tomography (AS-OCT)
    Cross-section images measure corneal thickness and epithelial integrity, tracking healing after lubrication or surgery.

  3. Ocular surface topography/keratography
    Maps corneal curvature and tear film stability; helps predict visual quality and astigmatism from exposure.

  4. Craniofacial imaging (3D CT/low-dose CT) for surgical planning
    Used by craniofacial teams to coordinate lip/palate repair and, when needed, bony work that can secondarily influence eyelid position. (E.g., in broader craniofacial syndromes.) EyeWiki

  5. Photography and video blink analysis
    Standardized photos and short videos document severity over time, help families see progress, and aid decisions about surgery timing.

Non‑Pharmacological Treatments (Therapies and Others)

  1. Frequent Preservative‑Free Artificial Tears (Daytime Lubrication)
    Description: Non‑medicated tear drops used many times per day keep the eye surface moist. In lagophthalmia, the eye dries quickly because the lids don’t close fully. Using preservative‑free tears every 1–2 hours during waking time lowers friction, burning, and risk of scratches. Families learn hand hygiene and drop technique.
    Purpose: Prevent exposure keratopathy (surface injury) and pain.
    Mechanism: Replaces missing tear volume; improves the tear film layer so the cornea stays smooth and wet, reducing micro‑abrasions and inflammatory triggers. (Refs: AAO EyeNet; EyeWiki exposure keratopathy.)
  2. Nighttime Lubricating Ointment and Lid Taping
    Description: A sterile petrolatum‑based ointment is applied at bedtime to coat the cornea, and the eyelids may be gently taped closed with hypoallergenic tape. Parents are taught clean technique and how to remove tape in the morning.
    Purpose: Protect the eye during sleep when natural blink is absent.
    Mechanism: Ointment slows evaporation; taping reduces the exposed area, so the cornea stays hydrated overnight. (Refs: AAO EyeNet; EyeWiki.)
  3. Moisture Chamber or Eye Shield at Night
    Description: A clear shield (e.g., swimming‑goggle style) creates a humid pocket over the eye while sleeping.
    Purpose: Prevents nocturnal drying and recurrent erosions.
    Mechanism: Traps moisture; reduces airflow over the cornea; lowers evaporative loss. (Refs: EyeWiki exposure keratopathy.)
  4. Daytime Protective Eyewear (Wraparound Glasses)
    Description: Wraparound frames reduce wind and dust. Prescription lenses can be added.
    Purpose: Shield the cornea from drafts and particles that worsen dryness or cause abrasions.
    Mechanism: Physical barrier deflects airflow and foreign bodies; stabilizes tear film. (Refs: EyeWiki.)
  5. Bandage Contact Lens (Specialist‑Supervised)
    Description: A soft, high‑oxygen contact lens is placed by an eye specialist to cover the cornea temporarily. Strict follow‑up is essential to avoid infection.
    Purpose: Promote epithelial healing and comfort during severe exposure or erosion.
    Mechanism: Acts like a smooth, protective cover; reduces friction from blinking and lashes. (Refs: EyeWiki; AAO articles.)
  6. Punctal Occlusion (Temporary Plugs)
    Description: Tiny plugs are inserted into tear drains by the ophthalmologist so tears stay longer on the eye.
    Purpose: Increase natural lubrication time.
    Mechanism: Slows tear outflow; raises tear volume and residence time on the cornea. (Refs: AAO.)
  7. Tarsorrhaphy (Temporary/Adjustable Suture Closure)
    Description: A minor procedure partially closes the eyelids with sutures to narrow the opening. It may be temporary in infants to protect the cornea until definitive lid surgery.
    Purpose: Immediate exposure reduction.
    Mechanism: Smaller palpebral fissure lowers evaporation and improves blink coverage. (Refs: AAO EyeNet.)
  8. Definitive Eyelid Reconstruction (e.g., Gold/Platinum Weight, Canthoplasty)
    Description: Surgical techniques add weight to the upper lid or tighten the corners of the eye to help closure.
    Purpose: Restore near‑normal blink and protect the cornea long‑term.
    Mechanism: Gravity assist and tendon tightening improve dynamic closure and tear spread. (Refs: AAO lagophthalmos management.)
  9. Lash Epilation or Cryotherapy for Distichiasis
    Description: Problematic extra lashes are plucked or permanently removed by cryotherapy/electrolysis if they rub the cornea.
    Purpose: Prevent recurrent corneal scratches.
    Mechanism: Removes mechanical abrasion source; allows healing. (Refs: PubMed case series; AAO.)
  10. Feeding Support: Specialty Bottles and Positioning
    Description: Cleft‑adapted nipples and bottles with one‑way valves help babies get milk without strong suction. Upright positioning reduces choking and nasal regurgitation.
    Purpose: Improve nutrition, weight gain, and safety.
    Mechanism: Bypasses suction deficit from cleft; steady milk flow matches weak seal. (Refs: ACPA booklet; AAPD/ACPA policy.)
  11. Early Speech‑Language Therapy
    Description: Therapists guide parent‑child exercises to develop sounds and reduce nasal escape after palate repair.
    Purpose: Support clear speech and social development.
    Mechanism: Trains correct placement and airflow; strengthens oral motor patterns. (Refs: ACPA booklet.)
  12. Ear and Hearing Care (Tympanostomy When Indicated)
    Description: Regular hearing checks; ENT evaluation for fluid buildup; tubes placed if needed.
    Purpose: Prevent speech delay due to conductive hearing loss.
    Mechanism: Ventilates middle ear; reduces infections and effusion. (Refs: ACPA policy.)
  13. Dental and Orthodontic Care
    Description: Early dental hygiene teaching; orthodontic expansion or alveolar support as the face grows.
    Purpose: Healthy teeth and proper bite for feeding and speech.
    Mechanism: Controls tooth position and arch form; prevents caries and crowding. (Refs: AAPD/ACPA policy.)
  14. Nasal Saline and Humidification
    Description: Gentle saline drops or sprays and home humidifiers keep nasal passages moist, especially after surgery.
    Purpose: Ease breathing and reduce crusts.
    Mechanism: Hydrates mucosa; helps cilia move mucus. (Refs: ACPA booklet.)
  15. Nutritional Counseling and Growth Monitoring
    Description: Dietitians track calories and teach high‑calorie options when feeding is slow.
    Purpose: Prevent malnutrition and support healing around surgeries.
    Mechanism: Ensures adequate protein, iron, zinc, and vitamins for growth and tissue repair. (Refs: ACPA booklet.)
  16. Family Education on Eye Safety and Red‑Flag Symptoms
    Description: Parents learn to spot eye pain, redness, light sensitivity, or discharge and to seek urgent care.
    Purpose: Rapid treatment of corneal problems.
    Mechanism: Early recognition prevents ulcers and scars. (Refs: AAO/EyeWiki.)
  17. Multidisciplinary Cleft Team Follow‑up
    Description: Coordinated care with ophthalmology, plastic surgery, ENT, pediatrics, dentistry, speech, nutrition, genetics, and psychology.
    Purpose: Seamless, age‑based care pathway from birth through adolescence.
    Mechanism: Team protocols schedule the right help at the right time. (Refs: ACPA policy.)
  18. Scar and Skin Care After Lip Repair
    Description: Sun protection, gentle massage, silicone gel sheeting when recommended.
    Purpose: Better cosmetic result and flexible scar.
    Mechanism: Silicone normalizes hydration and collagen alignment. (Refs: surgical best practices.)
  19. Psychosocial Support and Parent Peer Groups
    Description: Counseling and support groups reduce anxiety and improve adherence.
    Purpose: Build resilience for child and family.
    Mechanism: Education and coping skills buffer stress; improves clinic follow‑through. (Refs: ACPA booklet.)
  20. Sleep and Airway Assessment
    Description: Check for snoring, apnea, or airway obstruction related to anatomy; refer to sleep study if needed.
    Purpose: Protect brain and growth from poor sleep and hypoxia.
    Mechanism: Identifies treatable airway issues; supports oxygenation and development. (Refs: cleft team guidelines.)

Drug Treatments

  1. Lifitegrast 5% Ophthalmic (Xiidra®)
    Class: LFA‑1 antagonist; anti‑inflammatory for dry eye.
    Dosage/Time: 1 drop in each eye twice daily about 12 hours apart (per label).
    Purpose: Reduces eye surface inflammation to stabilize tears in exposure‑related dryness.
    Mechanism: Blocks LFA‑1/ICAM‑1 interaction on T‑cells, decreasing inflammatory signaling on the ocular surface.
    Side Effects: Temporary irritation, dysgeusia (unusual taste), reduced visual acuity, headaches; remove contact lenses before use.
    Description: In lagophthalmia, the cornea dries and becomes inflamed. Lifitegrast can calm this inflammation and improve symptoms alongside frequent lubrication. It does not replace physical protection at night but may cut burning and stinging during the day. Use only with ophthalmologist approval in children; safety data in pediatrics are limited; avoid touching the dropper tip. (FDA label data: dosing, indication, adverse events.)
  2. Cyclosporine 0.05% Ophthalmic Emulsion (Restasis®)
    Class: Topical calcineurin inhibitor; tear‑stimulant in inflammatory dry eye.
    Dosage/Time: 1 drop twice daily in each eye ~12 hours apart.
    Purpose: Increase natural tear production when reduced by ocular surface inflammation.
    Mechanism: Inhibits T‑cell activation and cytokine release in lacrimal glands and conjunctiva.
    Side Effects: Burning/stinging on instillation, foreign body sensation, eye pain; rare hypersensitivity.
    Description: For exposure‑driven dryness, cyclosporine helps glands make more tears over weeks to months. It is used together with tears/ointment and eyelid protection. Pediatric use is at specialist discretion. Do not use contact lenses during dosing; single‑use vials help sterility. (FDA label data.)
  3. Moxifloxacin 0.5% Ophthalmic (Vigamox®)
    Class: Topical fluoroquinolone antibiotic.
    Dosage/Time: 1 drop three times daily for 7 days for bacterial conjunctivitis; dosing for corneal infection is specialist‑directed.
    Purpose: Treat suspected bacterial keratitis or conjunctivitis when exposure has led to infection.
    Mechanism: Inhibits bacterial DNA gyrase/topoisomerase IV.
    Side Effects: Eye irritation, dryness; rare hypersensitivity. Avoid contact lenses with active infection.
    Description: If the exposed cornea becomes infected, moxifloxacin provides broad coverage with good corneal penetration. It is used urgently and under close follow‑up to protect vision. (FDA label data.)
  4. Erythromycin Ophthalmic Ointment
    Class: Macrolide antibiotic ointment.
    Dosage/Time: Applied as a ~1 cm ribbon to eyelid margin up to 4–6 times daily for infection prophylaxis/blepharitis as directed.
    Purpose: Nighttime antibiotic lubrication when infection risk is high.
    Mechanism: Inhibits bacterial protein synthesis (50S ribosomal subunit).
    Side Effects: Blurred vision after application, irritation; hypersensitivity is rare.
    Description: Many clinicians start with an antibiotic ointment at bedtime in high‑risk exposure to both lubricate and prevent bacterial overgrowth on the drying surface. (FDA communications/label data.)
  5. Acetaminophen (Paracetamol) Oral/IV
    Class: Analgesic/antipyretic.
    Dosage/Time: Weight‑based pediatric dosing; given every 4–6 hours as directed; IV formulations used peri‑operatively.
    Purpose: Pain and fever control after surgeries (lip/palate repair; ear tubes).
    Mechanism: Central COX inhibition and serotonergic pathways reduce pain and fever.
    Side Effects: Overdose can cause liver injury; always follow weight‑based dosing.
    Description: Gentle pain control supports feeding and healing. Caregivers receive precise dosing charts from the cleft team. (FDA label data.)
  6. Ibuprofen Pediatric Suspension
    Class: NSAID analgesic/anti‑inflammatory.
    Dosage/Time: Weight‑based every 6–8 hours with food as directed; avoid in dehydration/renal disease.
    Purpose: Post‑operative pain and inflammation reduction when appropriate.
    Mechanism: Reversible COX‑1/COX‑2 inhibition reduces prostaglandins.
    Side Effects: Gastric upset, rare bleeding or kidney issues; avoid with other NSAIDs.
    Description: Used short‑term after surgeries if permitted by the surgeon, alternating with acetaminophen for comfort. (FDA label data.)
  7. Amoxicillin/Clavulanate (Augmentin®)
    Class: Beta‑lactam antibiotic + beta‑lactamase inhibitor.
    Dosage/Time: Weight‑based dosing every 8–12 hours; dose depends on infection site/severity.
    Purpose: Treat postoperative or ENT infections (e.g., otitis media, sinusitis) when indicated.
    Mechanism: Inhibits bacterial cell wall synthesis; clavulanate blocks beta‑lactamases.
    Side Effects: Diarrhea, rash, candidiasis; allergic reactions in penicillin‑allergic patients.
    Description: Not used routinely, but prescribed when bacterial infection is confirmed or strongly suspected. (FDA label data.)
  8. Preservative‑Free Carboxymethylcellulose/Hypromellose Tears
    Class: Ocular lubricants (OTC).
    Dosage/Time: 1–2 drops as often as needed; hourly in severe exposure per physician plan.
    Purpose: Core daytime lubrication.
    Mechanism: Viscous polymers bind water and spread evenly over the cornea to reduce friction.
    Side Effects: Temporary blur or stickiness.
    Description: First‑line in all ages; preservative‑free single‑use vials lower irritation in frequent use. (Clinical practice references.)
  9. Petrolatum‑Mineral Oil Ophthalmic Ointment (OTC)
    Class: Lubricating ointment.
    Dosage/Time: Nightly; more often if exposed.
    Purpose: Overnight corneal protection.
    Mechanism: Occlusive layer reduces evaporation and shear.
    Side Effects: Temporary blur; rare sensitivity.
    Description: Standard of care for nocturnal lagophthalmos. (AAO/EyeWiki.)
  10. Topical Steroid (Short Course, Specialist‑Directed)
    Class: Anti‑inflammatory.
    Dosage/Time: Very short, low‑potency courses with close follow‑up.
    Purpose: Calm acute surface inflammation not controlled by lubricants.
    Mechanism: Suppresses inflammatory cytokines; reduces redness and pain.
    Side Effects: Elevated intraocular pressure, infection risk, cataract with prolonged use—specialist supervision only. (AAO.)
  11. Topical Antihistamine/Mast‑Cell Stabilizers
    Class: Antiallergy drops.
    Dosage/Time: Once or twice daily per product.
    Purpose: Reduce itch/rub cycle that worsens exposure injury.
    Mechanism: Blocks histamine; stabilizes mast cells to prevent mediator release.
    Side Effects: Mild sting, dry eye. (Labeling.)
  12. Topical Antibiotic Ointments (e.g., Bacitracin/Polymyxin)
    Class: Broad topical antimicrobials.
    Use: For lid margin hygiene or superficial infections as directed.
    Risks: Contact dermatitis; use short course. (Labeling.)
  13. Nasal Saline Sprays/Drops
    Class: Isotonic saline (OTC).
    Purpose: Post‑op comfort and crust control after cleft surgeries. (ACPA booklet.)
  14. Vitamin D and Iron if Deficient
    Class: Nutrient replacement.
    Purpose: Correct documented deficiencies that affect growth and healing. (Pediatric nutrition standards.)
  15. Chlorhexidine Mouth Rinses (Older Children, if Indicated)
    Class: Antiseptic.
    Use: Short courses for oral hygiene after dental work; avoid in small children who can’t spit. (Dental guidelines.)
  16. Analgesic Local Anesthetics (Clinic Use)
    Class: Topical ocular anesthetics for procedures only.
    Note: Not for home use due to corneal toxicity risk. (AAO.)
  17. ENT Ear Drops (Antibiotic ± Steroid) When Indicated
    Use: For otorrhea with tubes under ENT guidance. (ENT guidelines.)
  18. Prophylactic Antibiotics (Surgical Protocols)
    Use: As per surgeon’s protocol at time of repair. (Surgical guidelines.)
  19. Fluoride Varnish (Dental Prevention)
    Use: Applied in clinic to prevent caries. (AAPD.)
  20. Zinc or Multivitamin if Diet Inadequate (Clinician‑Directed)
    Use: Corrects poor intake; do not exceed age‑appropriate doses. (Pediatric nutrition.)

Important: Many products are off‑label in infants/children with this rare syndrome. Always follow a pediatric ophthalmologist/cleft team plan.

Dietary Molecular Supplements

  1. Omega‑3 (EPA/DHA): 1–3% of calories or age‑appropriate dosing may support tear film lipid layer and reduce ocular surface inflammation; mechanism via resolvins/protectins that calm cytokines. Can reduce meibomian dysfunction dryness. Watch for fish allergy and bleeding risk with high doses. (Clinical reviews.)
  2. Vitamin A (Within RDA): Supports corneal epithelial health and mucin production. Excess causes toxicity; use only if deficient or as part of multivitamin under supervision. (Pediatrics.)
  3. Vitamin D: Deficiency is common and linked to impaired immune function and bone growth; replete to normal range for surgical recovery and general health. (Guidelines.)
  4. Vitamin C: Cofactor for collagen synthesis; may aid wound healing after repairs; use within dietary ranges. (Surgical nutrition.)
  5. Zinc: Cofactor in epithelial repair and immune function; correct deficiency to aid wound healing. (Nutrition texts.)
  6. L‑Carnitine: Mitochondrial fatty acid transport; sometimes used for fatigue/feeding issues; evidence limited in this context. (Nutrition references.)
  7. Probiotics (Lactobacillus/Bifidobacterium): May improve antibiotic‑associated diarrhea during necessary courses; choose pediatric strains/doses. (Pediatric gastro guidance.)
  8. Selenium (Within RDA): Antioxidant enzyme component; avoid excess. (Nutrition.)
  9. Curcumin (Food‑based/Turmeric): Anti‑inflammatory effects via NF‑κB pathways; culinary use preferred; supplement only with clinician approval to avoid interactions. (Supplements research.)
  10. Hyaluronic Acid (Oral/Topical in Tears): In tears, it increases corneal surface wetting; oral forms have limited ocular data. (Ocular surface studies.)

Drugs for Immunity/Regenerative/Stem‑Cell–Related Support

  1. Topical Autologous Serum Tears (AST): 20–50% serum prepared from the patient’s blood, used as drops for severe surface disease. Dose: Several times daily. Function/Mechanism: Delivers growth factors (EGF, NGF) and vitamins similar to natural tears; promotes epithelial healing. (Ocular surface literature.)
  2. Platelet‑Rich Plasma (PRP) Eye Drops: Dose: Specialist‑prepared; multiple daily drops. Function: Concentrated growth factors support regeneration. Mechanism: PDGF/VEGF/TGF‑β enhance epithelial repair. (Emerging evidence.)
  3. Amniotic Membrane Graft (Biologic): Dose: Single placement on cornea. Function: Biological bandage. Mechanism: Anti‑inflammatory matrix rich in growth factors speeds re‑epithelialization. (Corneal surgery literature.)
  4. Topical Cyclosporine (Immunomodulator): Dose: Twice daily. Function: Restores tear production by down‑regulating T‑cell–mediated inflammation. Mechanism: Calcineurin inhibition reduces cytokines from the lacrimal unit. (FDA label.)
  5. Topical Lifitegrast (Immunomodulator): Dose: Twice daily. Function: Reduces surface inflammation to enable healing. Mechanism: Blocks LFA‑1/ICAM‑1 binding to suppress T‑cell activation. (FDA label.)
  6. Biologic/Tissue Adhesives in Lid Surgery: Dose: Single intraoperative use. Function: Aid secure lid reconstruction and reduce inflammation. Mechanism: Scaffold/sealant supports tissue integration. (Surgical literature.)

Surgeries (Procedures and Why They’re Done)

  1. Cleft Lip Repair (Cheiloplasty): Usually in early months. Why: Close the lip gap to improve feeding, seal, and appearance; sets foundation for later dental and speech outcomes. (ACPA timeline.)
  2. Cleft Palate Repair (Palatoplasty): Often between 6–12 months. Why: Separate nose and mouth for speech development, swallowing, and ear health; reduces nasal regurgitation. (Management reviews.)
  3. Eyelid Reconstruction/Tarsorrhaphy: Early partial closure or definitive lid tightening/weights. Why: Protect cornea from constant exposure and prevent scarring/vision loss. (AAO.)
  4. ENT Ear Tubes (Myringotomy with Tympanostomy): Why: Ventilate middle ear to stop fluid buildup and improve hearing during speech‑critical years. (ACPA policy.)
  5. Secondary Revisions (Alveolar Bone Grafting, Rhinoplasty, Speech Surgery): Why: Improve bite, facial balance, and velopharyngeal function as the child grows. (Cleft team protocols.)

10 Preventions (Everyday Steps)

  1. Keep eyes lubricated day and night exactly as taught.
  2. Use shields or tape at night to prevent drying.
  3. Avoid rubbing eyes; trim nails; use mittens in infants.
  4. Wear wraparound glasses outdoors.
  5. Follow feeding strategies and growth checks.
  6. Keep up with hearing tests and ENT visits.
  7. Brush teeth with fluoride paste and see pediatric dentist early.
  8. Use sun protection on scars.
  9. Stay current with vaccines and routine pediatric care.
  10. Attend all cleft‑team follow‑ups and call early for eye redness/pain/light sensitivity.

When to See Doctors (Urgent and Routine)

  • Urgent eye care now: eye pain, light sensitivity, redness, discharge, sudden blur, or child keeping eye closed. These can mean corneal abrasion or infection and need same‑day ophthalmology.
  • Routine: frequent early visits with pediatric ophthalmology to adjust lubrication plan and consider plugs/lid procedures; scheduled reviews with plastic surgery/ENT/speech/dentistry/nutrition.
  • Post‑op: any bleeding, fever, poor feeding, breathing problems, or wound issues after lip/palate surgery. (AAO/ACPA guidance.)

10 What to Eat and What to Avoid (Plain Tips)

  • Eat: breast milk or formula using cleft‑adapted bottle systems; later, soft high‑protein foods (eggs, fish, yogurt), fruits/vegetables, iron‑rich meats/legumes, and healthy fats (olive oil, avocado) to meet calories for growth.
  • Avoid (or delay): hard sharp foods that can injure the palate repair early on; sugary snacks/drinks that cause cavities; very spicy/salty foods if they irritate; choking hazards; and herbal supplements without team approval.
  • Hydration: offer small frequent liquids to keep mucosa moist. (ACPA nutrition tips.)

15 Frequently Asked Questions (Simple English)

  1. Is LCCLP the same as AEC syndrome? No. They share clefting and eyelid problems, but AEC has skin, hair, and nail issues and different genetics. (AEC references.)
  2. Can my baby’s eyes be protected without surgery? Yes—tears, ointment, taping, shields, and sometimes plugs. Surgery is for persistent exposure. (AAO.)
  3. Will my child see normally? Many do well if the cornea is protected early; untreated exposure can scar the cornea. (AAO/EyeWiki.)
  4. When is lip and palate surgery done? Lip in early infancy; palate often 6–12 months, adjusted by the team. (Cleft management reviews.)
  5. Why are ear tubes common? Cleft palate affects the eustachian tube. Tubes prevent hearing loss from fluid. (ACPA.)
  6. Do special bottles really help? Yes, they reduce the need for suction so babies gain weight better. (ACPA booklet.)
  7. Are eye antibiotics always needed? No—only when infection is suspected; otherwise lubrication is the main treatment. (AAO.)
  8. Are cyclosporine or lifitegrast safe for kids? Pediatric use is specialist‑decided; labels focus on adults. Benefits must outweigh risks. (FDA labels.)
  9. Will my child need glasses? Possibly; exposure and lid anatomy can affect the surface and focusing. (Ophthalmology practice.)
  10. Can eyelashes scratch the eye? Yes, extra lashes (distichiasis) may rub the cornea; removal may be needed. (Case series/AAO.)
  11. Is there a genetic test? For isolated LCCLP, clear genes are not well established; AEC and other syndromes have known genes. A genetics consult is useful. (MedlinePlus/Orphanet.)
  12. How long do we use ointment at night? As long as exposure persists; many use it nightly for months to years. (AAO.)
  13. Do supplements cure the condition? No. Supplements may support nutrition and healing but do not replace surgeries or eye protection. (Pediatrics.)
  14. What are danger signs after surgery? Fever, bleeding, breathing trouble, dehydration, or wound opening—call the surgeon. (Post‑op guidance.)
  15. Will my child need more surgeries later? Often yes—growth changes the face; revisions improve function and appearance. (Cleft team protocols.)

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