Gastroenterology

Managing Achalasia, Nutrition, and Alacrima

At a Glance

Managing Triple A syndrome involves treating achalasia to support swallowing and nutrition, monitoring growth and reflux after procedures, and using lifelong eye lubrication and regular ophthalmology visits to protect the cornea from alacrima.

While adrenal management is a critical part of Triple A syndrome, managing the other two “As”—achalasia and alacrima—is essential for maintaining quality of life, proper nutrition, and eye health ^[1]. Because these symptoms can be progressive, they require ongoing attention from a team of specialists including gastroenterologists and ophthalmologists ^[1].

Managing Achalasia (Swallowing Difficulty)

In Triple A syndrome, the lower part of the esophagus fails to relax, creating a physical “bottleneck” that prevents food from entering the stomach ^[2]. This can lead to vomiting, chest pain, unintended weight loss, and the risk of aspiration (inhaling food or liquid into the lungs) ^[3]^[4]. There are three main surgical and procedural ways to address this, and the choice depends on age, anatomy, and local expertise:

Heller Myotomy: This is a surgical procedure where the tight muscles at the end of the esophagus are cut to allow food to pass ^[5]. In children, this is often performed alongside a fundoplication—a procedure that wraps part of the stomach around the esophagus to help prevent acid reflux after the surgery ^[6]^[7].
POEM (Peroral Endoscopic Myotomy): This is a newer, less invasive procedure where the surgeon uses an endoscope (a flexible tube passed through the mouth) to cut the esophageal muscles without external incisions ^[8]^[9]. Small studies in rare-disease cohorts have shown POEM can improve swallowing symptoms in Triple A patients, though the evidence base is small ^[9]^[8].
Pneumatic Dilation: A specialized balloon is inflated inside the esophagus to stretch the tight muscle ^[10]. While this can provide relief, the effects in children with Triple A syndrome are often temporary, and many patients eventually require a more permanent surgical solution ^[11]^[5].

Safety note: Any procedure represents physical stress. Coordinate a perioperative steroid plan with endocrinology and anesthesia.

Nutrition and Growth

Achalasia can have a profound impact on a child’s growth and an adult’s nutrition. When food cannot reach the stomach, patients may experience malnutrition or “failure to thrive” ^[3].

Nutritional Monitoring: Regular weight and height checks are vital to ensure proper growth ^[12]. Seek immediate care for signs of aspiration, like coughing after eating or breathing difficulty.
Rehabilitation: In cases of severe weight loss, doctors may recommend specialized nutritional support before or after surgery ^[13].
Post-Procedure Watch: Even after successful treatment, some patients develop reflux esophagitis (inflammation from stomach acid moving upward) ^[8]. In one small study, over 60% of Triple A patients who underwent POEM developed objective signs of reflux on testing, which often requires management with acid-reducing medications ^[8]^[14].

Protecting the Eyes (Alacrima)

Because the eyes do not produce enough tears, they are at constant risk for dryness, irritation, and damage to the cornea (the clear front surface of the eye) ^[15]^[16]. Corneal damage can progress even if the eyes do not feel particularly dry.

Lubrication: The first line of defense is the frequent use of preservative-free artificial tears during the day and thick lubricating ointments at night ^[16]^[17].
Corneal Protection: If standard drops aren’t enough, doctors may prescribe moisture chamber goggles to keep humidity around the eyes or punctal plugs, which are tiny devices inserted into the tear ducts to keep what little natural moisture is present on the surface of the eye for longer ^[17]^[18]. Plugs may be ineffective if tear production is extremely low.
Advanced Treatments: For persistent or severe cases, specialized prescription medications like topical cyclosporine may be used to reduce inflammation ^[19].

Regular eye exams are necessary to check for “staining” or thinning of the cornea ^[20]^[19]. Urgent Eye Warning Signs: Seek prompt ophthalmic assessment for severe eye pain, marked redness, light sensitivity, discharge, or any vision change. Treatment is lifelong and focuses on preventing discomfort and long-term scarring ^[15].

Common questions in this guide

What treatments can relieve achalasia in Triple A syndrome?

Treatment options include Heller myotomy, POEM, a procedure performed through the mouth, and pneumatic dilation with a balloon. The best choice depends on the patient's age, anatomy, and local expertise; children may have a fundoplication with Heller myotomy to reduce reflux. Any procedure should include a coordinated steroid plan with endocrinology and anesthesia.

Is POEM or Heller myotomy better for a child with Triple A syndrome?

Neither procedure is best for every patient. POEM has improved swallowing in small studies of people with Triple A syndrome, while Heller myotomy is an established surgical option and may be paired with fundoplication in children. The treating team should weigh age, anatomy, reflux risk, and experience.

How can achalasia affect nutrition and growth?

When food cannot pass into the stomach normally, a child may lose weight, become malnourished, or grow poorly. Regular weight and height checks help the care team track growth, and specialized nutritional support may be recommended around treatment. Coughing after eating or breathing difficulty can signal aspiration and needs prompt medical attention.

What reflux problems can happen after POEM?

Reflux esophagitis can occur after treatment, and a small study found objective signs of reflux in more than 60% of Triple A patients after POEM. Follow-up testing may be needed, and acid-reducing medicine can help manage reflux when prescribed. Ask the treating team how reflux will be monitored.

How is alacrima treated to protect the eyes?

Use preservative-free artificial tears frequently during the day and a thick lubricating ointment at night as directed. If these are not enough, an ophthalmologist may recommend moisture-chamber goggles, punctal plugs, or topical cyclosporine. Regular exams are important because corneal damage can progress even when the eyes do not feel very dry.

When does alacrima require urgent eye care?

Seek prompt care from an eye specialist for severe eye pain, marked redness, light sensitivity, discharge, or any change in vision. These signs can indicate injury or inflammation of the cornea, the clear front surface of the eye. Alacrima treatment is lifelong and aims to prevent discomfort and scarring.

What swallowing or eating signs should a caregiver watch for?

Watch for food feeling stuck, avoidance of foods such as meat or bread, coughing at night or after eating, bad breath, vomiting, or breathing difficulty. These may indicate that food or acid is remaining in the esophagus or that aspiration is occurring. Contact the care team promptly if breathing problems or signs of aspiration occur.

Get notified when new evidence is published on Triple A syndrome.

We monitor PubMed for new peer-reviewed studies on this topic and email a short summary when something meaningful changes.

References (20)

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This page explains achalasia, nutrition, and alacrima care in Triple A syndrome for informational purposes only and does not constitute medical advice. Discuss procedures, steroid planning, nutrition, and eye protection with the patient's gastroenterology, endocrinology, and ophthalmology team.