Lacrimal Gland Agenesis (Congenital Alacrima)

What it is

The lacrimal gland sits under the outer part of the upper eyelid and produces most of the watery layer of tears, especially reflex tears (from irritation) and emotional tears (from crying). Small accessory glands in the eyelid lining make a low baseline flow. In lacrimal gland agenesis, the main gland never forms during fetal development, so only the small baseline flow remains.

Note that newborns often have few tears for the first weeks of life; emotional tears usually appear by 1 to 3 months. A baby who still cries without tears after this age deserves an eye check.

The broader term alacrima means absent or reduced tears from any cause. Agenesis is one cause; others include nerve problems and syndromes in which the gland is present but does not work.

Causes and associated conditions

Most genetic forms are autosomal dominant (ALSG, LADD) or autosomal recessive (triple A, familial dysautonomia), so genetic counseling is useful for families.

Symptoms

• crying without tears
• red, irritated eyes, frequent blinking or eye rubbing
• sensitivity to light and squinting
• sticky or stringy mucus, because there is too little water to dilute it
• blurred vision from a rough corneal surface
• in syndromic forms: dry mouth, dental decay, hearing loss, ear, finger or thumb differences, or problems swallowing

Some children have surprisingly mild symptoms, because the accessory glands provide enough moisture for daily needs.

Diagnosis

1. Eye examination with a slit lamp and fluorescein dye, which highlights dry spots and damage on the cornea.
2. Schirmer test: a strip of filter paper placed inside the lower lid measures tear production over 5 minutes; very low values support alacrima.
3. Imaging: MRI or CT of the eye sockets shows whether the lacrimal gland is absent or present.
4. Further tests if a syndrome is suspected: hearing test, dental assessment, swallowing studies, cortisol and ACTH blood tests for triple A syndrome, and genetic testing.

Treatment

There is no way to grow a missing gland, so treatment protects the eye surface for life.

• Preservative-free artificial tears used often through the day, with thicker gels or ointment at night.
• Punctal plugs or cautery of the tear drainage openings to keep the tears that are made on the eye.
• Humidifiers, moisture-retaining glasses or goggles, and avoiding fans, smoke and dry air.
• Autologous serum drops or prescription anti-inflammatory drops in some cases.
• Treatment of corneal damage: antibiotic drops for infection, and bandage contact lenses or amniotic membrane for persistent defects.
• Surgery for severe disease: narrowing the eyelid opening (tarsorrhaphy), or transplanting a minor salivary gland or part of a submandibular gland to the eye socket to provide moisture.

Children with salivary gland aplasia also need intensive dental prevention, such as fluoride varnish, and those with triple A syndrome need adrenal hormone replacement and treatment for achalasia.

Complications

• dry eye disease and chronic irritation
• corneal abrasions and infection, including corneal ulcers
• corneal scarring, which can blur vision
• in young children, amblyopia (lazy eye) if one eye's vision is reduced

Outlook

With regular lubrication and eye checkups, most children with lacrimal gland agenesis keep useful, often normal, vision. The condition is lifelong, so routines for eye care need to continue into adulthood. The outlook for syndromic forms also depends on the other features of the syndrome.

Frequently asked questions

When should a baby start producing tears?

Many newborns cry without tears for the first few weeks. Emotional tears usually develop between 1 and 3 months of age. If a baby over 3 months never produces tears when crying, or the eyes look red and irritated, an eye doctor should check for alacrima.

Is lacrimal gland agenesis genetic?

Sometimes. It can occur on its own without a clear cause, or run in families as part of conditions such as aplasia of the lacrimal and salivary glands or LADD syndrome, linked to the FGF10, FGFR2 and FGFR3 genes. Genetic testing and counseling can clarify the risk for siblings and future children.

Can lacrimal gland agenesis be cured?

No, the missing gland cannot be replaced by medicine. However, lubricating drops, gels, punctal plugs and, in severe cases, surgery keep the eye surface healthy. Most children keep good vision with consistent care.

What is the difference between alacrima and dry eye?

Alacrima means little or no tear production, often from birth. Dry eye is a broader condition with many causes, such as aging, screen use or Sjögren's syndrome, where tears are reduced or evaporate too fast. Alacrima causes a severe, lifelong form of dry eye.