Pediatric Surgery: Meconium Ileus

Meconium Ileus

Definition

  • Meconium Ileus: Small Bowel Obstruction from Thick Meconium
  • Meconium Plug Syndrome: Colonic Obstruction from Thick Meconium

Pathogenesis

  • Thickened Meconium Accumulates & Obstructs
    • Causes 10-25% of Intestinal Obstructions in Neonates
  • Causes:
    • Pancreatic Exocrine Enzyme Deficiency
    • Secretion of Highly Viscus Mucus
  • Associated with Cystic Fibrosis
    • Meconium Ileus is the Presenting Feature in 20.8% of Cystic Fibrosis Patients
    • Family Hx of Cystic Fibrosis is Present in 10-40% of Patients with In-Utero Diagnosis Meconium Ileus
    • May Have Pancreatic Enzyme Deficits
    • Diagnose CF by Sweat Chloride Test

Presentation

  • Abdominal Distention (May Be Present at Birth – Other Causes are Delayed Due to Need for Air Accumulation)
  • Vomiting
  • Failure to Pass Meconium
  • Complicated Disease:
    • Intestinal Atresia
    • Volvulus
    • Perforation
    • Meconium Peritonitis
    • Meconium Pseudocyst – Perforation with Meconium Accumulation Over Weeks-Months Creating a Calcified Cystic Wall
    • Bowel Necrosis
  • Meconium Aspiration is The Most Common Indication for ECMO in Peds

Diagnosis

  • Diagnosis: Combination of History, Physical & Radiology
  • AXR Findings:
    • Dilated Proximal Bowel & Distal Microcolon
    • No Air-Fluid Levels – Meconium Too Thick for Air to Layer Above
    • “Soap Bubble” or “Ground Glass” Appearance – From Swallowed Air Mixed with Meconium
  • Contrast Enema (Gastrografin) is Diagnostic & Therapeutic

Treatment

  • Simple Disease (Initial Treatment): Gastrografin Enema
    • Also Include NG Decompression, IV Antibiotics & Aggressive Fluid Resuscitation
    • Also Consider N-Acetylcysteine Through NG Tube from Above
    • May Repeat After 8-12 Hours if Evacuation is Incomplete
  • Complicated Disease or Simple Disease That Fails Enema: Enterotomy with Irrigation & Meconium Evacuation
    • May Consider an Enterostomy (Best Procedure is Not Yet Determined)
    • Procedures:
      • Mikulicz Double-Barreled Enterostomy
      • Bishop-Koop (Distal Chimney) Enterostomy
      • Santulli (Proximal Chimney) Enterostomy
      • Tube Enterostomy
      • T-Tube Enterostomy
      • Appendectomy with Cecostomy Through the Appendiceal Stump
      • Resection with Primary Anastomosis
      • Resection with Enterostomy

Meconium Ileus 1

Meconium Removed from Intestine 1

Bowel Distention without Air-Fluid Levels 1

Contrast Enema with Multiple Filling Defects in (A) Normal-Sized Colon and (B) Microcolon 1

References

  1. Kim HY, Kim SH, Cho YH, Byun SY, Han YM, Kim AY. Meconium-related ileus in very low birth weight and extremely low birth weight infants: immediate and one-year postoperative outcomes. Ann Surg Treat Res. 2015 Sep;89(3):151-7. (License: CC BY-NC-4.0)