What is the management approach for Triple A (Adrenal Insufficiency, Achalasia, Alacrima) syndrome without rupture?

Management of Triple A Syndrome (Allgrove Syndrome) Without Rupture

The management of Triple A syndrome requires a multidisciplinary approach addressing all three cardinal manifestations: adrenal insufficiency with glucocorticoid and mineralocorticoid replacement, achalasia with endoscopic or surgical intervention, and alacrima with artificial tears. 1, 2

Adrenal Insufficiency Management

Glucocorticoid Replacement

• Hydrocortisone is the preferred glucocorticoid replacement:
  • Standard dosage: 10-30 mg daily in divided doses 3
  • Typical regimen: Higher morning dose (⅔ of total) and smaller afternoon dose (⅓ of total)
  • Alternative: Prednisone 5-10 mg daily can be used 3

Mineralocorticoid Replacement

• Fludrocortisone (standard therapy):
  • Initial dose: 0.1 mg daily 4
  • Dose range: 0.1 mg three times weekly to 0.2 mg daily 4
  • Reduce to 0.05 mg daily if transient hypertension develops 4

Stress Dosing Protocol

• Minor illness (fever <38°C): Double oral glucocorticoid dose 3
• Moderate illness (fever >38°C, vomiting, diarrhea): Triple oral dose or use parenteral hydrocortisone 3
• Severe illness/surgery: 100 mg hydrocortisone IV/IM before procedure, then every 6 hours 3
• Adrenal crisis: Immediate 100 mg hydrocortisone IV bolus, followed by continuous infusion of 200 mg/24h or 50 mg every 6 hours, plus IV isotonic saline 3

Achalasia Management

Diagnosis

• High-resolution manometry (HRM) to determine achalasia subtype 5
• Timed barium esophagram to assess esophageal emptying
• Endoscopy to rule out pseudoachalasia (malignancy)

Treatment Options

1. Per-Oral Endoscopic Myotomy (POEM):

   • First-line for Type III achalasia 5
   • Comparable to laparoscopic Heller myotomy for all achalasia subtypes 5
   • Should be performed by experienced physicians in high-volume centers (20-40 procedures needed for competence) 5
   • Post-POEM patients require management for reflux (PPI therapy and/or surveillance endoscopy) 5

2. Laparoscopic Heller Myotomy (LHM):

   • Highly efficacious for Type I and II achalasia 5
   • Consider for patients with advanced esophageal dilation, sigmoidization, epiphrenic diverticulum, or hiatal hernia 5

3. Pneumatic Dilation (PD):

   • Effective for Type I and II achalasia with less morbidity and cost than surgery 5
   • May require repeat dilations over time 5

Alacrima Management

• Artificial tears for regular lubrication
• Moisture chamber glasses in severe cases
• Regular ophthalmological follow-up to prevent corneal damage

Neurological Manifestations

• Regular neurological assessment for early detection of:
  • Autonomic dysfunction (most common neurological manifestation) 2
  • Peripheral neuropathy
  • Amyotrophy
  • Optic atrophy

Follow-Up and Monitoring

• Regular adrenal function testing, especially in children with AAAS gene mutations 6
• Endocrine monitoring: Electrolytes, blood pressure, weight
• Gastrointestinal follow-up: Symptom assessment, nutritional status
• Ophthalmologic evaluation: Corneal integrity, tear production (Schirmer's test)
• Neurological assessment: Peripheral nerve function, autonomic function

Patient Education

• Medical alert bracelet/card identifying adrenal insufficiency 3
• Education on stress dosing and emergency injectable hydrocortisone use 3
• Recognition of early warning signs of adrenal crisis: fever, chills, sweats, fatigue, nausea, vomiting, abdominal pain, and hypotension 3

Special Considerations

• Early genetic testing recommended when alacrima and at least one more symptom are present 6, 7
• Patients may present with late-onset neurological findings without the classic triad 8
• Regular follow-up of adrenal function is necessary in all children with AAAS gene mutations to prevent adrenal crisis 6