3. Incidence and pathogenesis
• 9-33% of all intestinal obstruction.
• 1:25000 newborns.
• 3rd case of neonatal small bowel obstruction.
• prenatal detection.
• association with congenital anomalies
4. Genuine associations
• Cystic fibrosis and meconium ileus.
• Meconium ileus and the premature infants.
• Meconium with lower carbs, high proteins, and
albumin.
• CFTR gene, approx 1000 mutations.
10. Hyperosmolar Enema
• Aims for hydration and softening of meconium mass.
• Careful resuscitation, hydration, appropriate electrolytes
repletion and maintenance of normothermia.
• Must be performed under fluoroscopic control.
• slow infusion to the rectum. Meconium pellets usually will
pass in the next 24-48 hours.
• Warm saline enemas with 1% N-acetylcysteine is of help
11. Hyperosmolar Enema
• after successful evacuation, 5 ml of 10% N-
acetylcysteine is given through NGT Q6 hours.
• Consider pancreatic enzymes if suspecting CF.
• Success rate range 63-83%
12. Operative Management
• Indications:
• Inadequate meconium evacuation or
complications of contrast enema.
• failure of hyperosmolar enema to promote
passage of meconium in 24-48 hours.
• if associated with intestinal atresia
13. Principles
• Manual evacuation.
• Entrotomy, intraoperative
saline irrigation,
mechanical separation of
pellets from bowel wall.
• 2% or 4% N-
acetylcysteine or 50%
hyperosmolar solution
instillation can be helpful.
14. Principles
• Resection of the extremely dilated proximal
segment (in order to avoid size discrepancy)
anastomosis.
• Complication is leak.
• The other option; resection, with creation of
stoma.
15. Techniques
• Mikulicz Double barrel enterostomy.
• Advantages:
• Less operative time.
• Risk of leak is avoided.
• Solubilizing agents can then be administered
in distal and proximal loops.
• Disadvantages:
• Potential high stoma output and fluid
losses.
• long resections can result in short bowel
• the need for another surgery to restore
continuity in later time.
16. Techniques
• Distal chimney enterostomy (Bishop-
Koop procedure )
• resection with anastomosis between the
end of the proximal segment and the side
of the distal segment of bowel
approximately 4 cm from the opening of
the distal segment
• The distal end is brought out as the
ileostomy
• This technique allows for normal
gastrointestinal transit while providing a
means for management of the distal
obstruction through the ileostomy should it
occur
17.
18. Techniques
• Proximal chimney enterostomy
(Santulli)
• Distal limb end is anastomosed to
the side of proximal limb
• enhanced proximal irrigation and
decompression.
• no need for evacuation at the time of
surgery
• risk of high output stoma, and
consequent electrolyte disturbance
and dehydration.
19. Techniques
• Primary resection and anastomosis
• resection of the obstructed segment.
• irrigation of remaining pellets in the
distal bowel.
• ileocolic anastomosis.
• disadvantages:
• Anastomosis complications.
• resection of additional bowel
(terminal ilium containing pellets with
the dilated segment of ileum)
20. Post-operative management
• Resuscitation, maintenance fluids.
• Replacement of fluid losses ( through NGT or
ileostomy)
• 2 or 4% N-acetylcystein via NGT or ileostomy.
• Workup of Cystic fibrosis.
• Keeping the placed stoma in mind for future
closure.
21. Nutrition Support
• As bowel function resumes, oral feeding can start with
breast milk or infant formula.
• Supplemental pancreatic enzymes and vitamin.
• those with long post-surgical course might require
enteral feeds or TPN.
• watch for TPN associated cholestasis.
• Glutamine-enriched formulas to the distal bowel maybe
tried to enhance bowel growth
22. References
• Pediatric Surgery Diagnosis and Management,
Prem Puri, Michael Holwarth
• Ashcraft’s pediatric surgery, 5th edition.
• Pediatric Surgery (Springer Surgery Atlas Series).
• Operative pediatric suregry, Lewis Spitz, Arnold
G Coran. 7th edition
Simple: terminal ileum is filled with firm concretions, small in diameter, and mold around the inspissated lumps of meconium.
proximal dilated bowel. distally, disused colon (microcolon)
Complicated: usually present dramatically, immediately after birth. some will develop symptoms within 24 hours of birth.
Signs of meconium peritonitis ( Abd distension, tenderness, abdominal wall erythema), clinical evidence of sepsis. intraperitoneal calcifications on abdominal X-rays.
complications include: perforation, meconium peritonitis, volvolus and atresia
meconium ileus is one of the most common causes of intestinal obstruction in the newborn.
Meconium ileus is always associated with CF.
can be the earliest clinical manifestation of CF ( 16% of patients with CF will present as meconium ileus)
16% of CF patients will present with meconium ileus, and 80-90% of meconium ileum cases have CF.
Meconium ileus is the earliest clinical manifestation of cystic fibrosis (CF) and occurs in 8–10% of patients with CF at birth
CF: autosomal recessive, 4-5%, occurs 1:3000 live births yearly.
Mutations in the Cystic fibrosis transmembrane regulator encoding for chloride channel, results in reduced clearance of secretions.
soap-bubble (the “Singleton’s sign”) or ground-glass appearance is due to a dense meconium mixed with air, typical of the distal ileum: this picture is usually located in the mid- abdomen or in the right iliac fossa
Stomas placed in the course of surgical management should be closed as soon as possible (4 to 6 weeks) to help avoid prolonged problems with fluid, electrolyte, nutritional losses, and choles- tatic jaundice.