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MECONIUM ILEUS
AND MECONIUM PERITONITIS
WATYEKERE SAMSON CLAUS
Meconium ileus
• Meconium: The earliest stool of mammalian infant. It’s a sterile mixture of
materials ingested during the time the infant spends in the uterus; epithelial
cells, mucus, amniotic fluid, bile and water.
• Meconium ileus: Is a cause of mechanical intestinal obstruction from
thickened (inspissated) meconium in the terminal ileum. Foetus fails to pass
meconium 24hrs after birth.
• It is the first manifestation of cystic fibrosis(CF) and occurs in approximately
20% of patients diagnosed with CF. It is a rare phenomenon in patients who
do not have this condition.
• Cystic fibrosis is an autosomal recessive disease involving bronchioles,
exocrine pancreas and sweat gland. (exocrine pancreatic insufficiency)
Cont…….
• It can present in two forms;
Simple MI
Complex MI
In simple MI, viscid meconium physically obstructs the terminal ileum and the
small intestine proximal to the obstruction, then becomes dilated with additional
meconium , gas and fluid.
In complex MI the meconium-distended segments of ileum can give way to
complications like prenatal volvulus, ischemic necrosis, intestinal atresia or
perforation.
Pathogenesis
• Infants with cystic fibrosis have characteristic pancreatic enzyme deficiencies
and abnormal chloride secretion in the intestine that result in the production of
viscous, water-poor meconium.
• This phenotype is explained by the presence of mutations in the cystic
fibrosis transmembrane conductance regulator(CFTR) gene. Meconium
ileus occurs when this thick, highly viscous dark green meconium becomes
impacted in the ileum and leads to high-grade intestinal obstruction.
• There is hypertrophy and dilatation of the proximal ileum. The distal ileum and
colon are contracted and narrow having greyish meconium pellets. Meconium
gets calcified very rapidly. Gangrene, perforation, volvulus can occur in 50%
of the cases
CLINICAL FEATURES
• When ileum is blocked, superiorly in small intestines, it enlarges & cause
abdominal distention
• Failure to pass stool in first 24hrs of birth.
• Abdominal pain.
• Bilious vomiting.
• Respiratory symptoms- cough, wheezing.
• High salt in sweat. (NaCl >90mmol/L)
Diagnosis/ investigations.
1. Clinical symptoms;- family hx of cystic fibrosis is sufficient to make a
diagnosis of meconium ileus.
2. Vomitus of a patient who does not contain trypsin, when poured on an
exposed X-ray film will not digest the gelatin of the film whereas vomitus of
patient with normal pancreas does.
3. Radiology
Plain Abdominal X-Ray
• Dilated bowel loops appearance without air-fluid levels is typical.
• Normal meconium is invisible on X-Ray. ”Soap bubble” or ‘’ground glass’’
appearance of small air bubbles n meconium (Neuhauser sign)
• Calcified meconium flecks enclosing peritoneal surfaces and the scrotum
are encountered in meconium peritonitis due to perforation.
7
Diagnosis cont’d
Prenatal ultrasonography
• Antenatal US may reveal the presence of intra-abdominal or scrotal
calcifications or distended bowel loops
Contrast Enema ( diagonostic & therapeutic)
• The diagnosis of meconium ileus is confirmed by a contrast enema,
which typically demonstrates a microcolon.
Barium enema
• Terminal ileum obstruction with distal “micro colon” can be precisely
looked for.
4. Sweat testing (pilocarpine ) and Genetic testing
• For diagnosis of cystic fibrosis when suspected.
• Histology- distended globlet cells in intestinal mucosa
9
Treatment
Initial mgt.
Conservative management in simple, uncomplicated, stable neonates.
• NPO, IV Fluids(N/S &D5%), NG tube for decompression.
• Dilute water-soluble contrast or N-acetylcystein, is infused transanally via
catheter under fluoroscopic control into the dilated portion of the
ileum.(facilitates absorption hence risks electrolyte imbalance)
• Dissolution through enema using gastrograffin or Acetyl choline (2-4%)
peranal (Foley’s catheter) or wash (Ryle’s tube 10ml 6 hourly)
• Urinary catheter for hourly urine monitoring.
• I.V antibiotics; e.g. ampicillin, genta, cefuroxime, metro).
• Laboratory evaluation of CBC, serum electrolytes, urea, creatinine.
• Mechanical respiratory support and monitor vitals.
• AFTER, ASSESS & PLAN IFOPERATIVE/Surgical OR NON OPERATIVE MGT.
10
Surgery
Surgical management involves EX-Lap in complicated cases.
• A double barrel ileostomy
• Bishop- Koop operation(very sick neonates)
• Santulli operation
• Resection and anastomosis (adequately fit child)
Bishop Koop operation
Santulli operation
Complications
• Volvulus - sigmoid.
• Bowel infarction
• Bowel atresia
• Bowel perforation
• Meconium peritonitis
14
Differential diagnosis
• Hirschsprung’s disease
• Meconium plug syndrome
• Anorectal malformations
• Malrotation
• Atresia
• Sepsis (NEC)
• Electrolyte imbalance
15
Associated syndrome
Meconium plug syndrome
Also reported in CF. In this condition transient obstruction of the
distal colon occurs.
• Manifests in the first 2 days after birth.
• Also known as functional colonic obstruction.
• A tenacious mass of mucus prevents the meconium from passing
out.
• Symptoms include abdominal distension and vomiting.
• MPS has a reduced risk of intestinal perforation.
• In barium enema, MPS shows a normal or dilated colon.
16
Prognosis
• MI is curable, once treated adequately. There’s no
relapse.
• Survival at 1 year is 90% in patients with simple
meconium ileus.
• Further prognosis depends on the probable presence
of underlying systemic disease such as Cystic Fibrosis.
• Tx then concentrates on enzyme replacement therapy
and control of recurrent pulmonary infections.
• Future prospects include Gene transfer therapy.
17
Meconium peritonitis
• Is consequent upon a fetal intestinal perforation. The baby is born
with a firm, distended, discoloured abdomen and signs of
obstruction.
• Meconium escapes into peritoneum leading to peritonitis.
• Infants present with a distended abdomen, vomiting.
• A prenatal U/S scan shows calcifications in the peritoneum.
• An abdominal x-ray shows dilated intestinal loops and areas of
calcification.
18
Clinical manifestations
• Baby is born with a firm, distended abdomen
• Discolored abdomen
• Signs of intestinal obstruction i.e.; abdominal tenderness, fever , shock.
Treatment
• Occasionally, the perforation resolves spontaneously before
birth, but most neonates with meconium peritonitis will need
surgery.
References
• Bailey and love short practice of surgery
• Schwartz’s principles of surgery,10th edition
• SRB manual of surgery

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Meconium ileus and peritonitis.pptx

  • 1. MECONIUM ILEUS AND MECONIUM PERITONITIS WATYEKERE SAMSON CLAUS
  • 2. Meconium ileus • Meconium: The earliest stool of mammalian infant. It’s a sterile mixture of materials ingested during the time the infant spends in the uterus; epithelial cells, mucus, amniotic fluid, bile and water. • Meconium ileus: Is a cause of mechanical intestinal obstruction from thickened (inspissated) meconium in the terminal ileum. Foetus fails to pass meconium 24hrs after birth. • It is the first manifestation of cystic fibrosis(CF) and occurs in approximately 20% of patients diagnosed with CF. It is a rare phenomenon in patients who do not have this condition. • Cystic fibrosis is an autosomal recessive disease involving bronchioles, exocrine pancreas and sweat gland. (exocrine pancreatic insufficiency)
  • 3.
  • 4. Cont……. • It can present in two forms; Simple MI Complex MI In simple MI, viscid meconium physically obstructs the terminal ileum and the small intestine proximal to the obstruction, then becomes dilated with additional meconium , gas and fluid. In complex MI the meconium-distended segments of ileum can give way to complications like prenatal volvulus, ischemic necrosis, intestinal atresia or perforation.
  • 5. Pathogenesis • Infants with cystic fibrosis have characteristic pancreatic enzyme deficiencies and abnormal chloride secretion in the intestine that result in the production of viscous, water-poor meconium. • This phenotype is explained by the presence of mutations in the cystic fibrosis transmembrane conductance regulator(CFTR) gene. Meconium ileus occurs when this thick, highly viscous dark green meconium becomes impacted in the ileum and leads to high-grade intestinal obstruction. • There is hypertrophy and dilatation of the proximal ileum. The distal ileum and colon are contracted and narrow having greyish meconium pellets. Meconium gets calcified very rapidly. Gangrene, perforation, volvulus can occur in 50% of the cases
  • 6. CLINICAL FEATURES • When ileum is blocked, superiorly in small intestines, it enlarges & cause abdominal distention • Failure to pass stool in first 24hrs of birth. • Abdominal pain. • Bilious vomiting. • Respiratory symptoms- cough, wheezing. • High salt in sweat. (NaCl >90mmol/L)
  • 7. Diagnosis/ investigations. 1. Clinical symptoms;- family hx of cystic fibrosis is sufficient to make a diagnosis of meconium ileus. 2. Vomitus of a patient who does not contain trypsin, when poured on an exposed X-ray film will not digest the gelatin of the film whereas vomitus of patient with normal pancreas does. 3. Radiology Plain Abdominal X-Ray • Dilated bowel loops appearance without air-fluid levels is typical. • Normal meconium is invisible on X-Ray. ”Soap bubble” or ‘’ground glass’’ appearance of small air bubbles n meconium (Neuhauser sign) • Calcified meconium flecks enclosing peritoneal surfaces and the scrotum are encountered in meconium peritonitis due to perforation. 7
  • 8.
  • 9. Diagnosis cont’d Prenatal ultrasonography • Antenatal US may reveal the presence of intra-abdominal or scrotal calcifications or distended bowel loops Contrast Enema ( diagonostic & therapeutic) • The diagnosis of meconium ileus is confirmed by a contrast enema, which typically demonstrates a microcolon. Barium enema • Terminal ileum obstruction with distal “micro colon” can be precisely looked for. 4. Sweat testing (pilocarpine ) and Genetic testing • For diagnosis of cystic fibrosis when suspected. • Histology- distended globlet cells in intestinal mucosa 9
  • 10. Treatment Initial mgt. Conservative management in simple, uncomplicated, stable neonates. • NPO, IV Fluids(N/S &D5%), NG tube for decompression. • Dilute water-soluble contrast or N-acetylcystein, is infused transanally via catheter under fluoroscopic control into the dilated portion of the ileum.(facilitates absorption hence risks electrolyte imbalance) • Dissolution through enema using gastrograffin or Acetyl choline (2-4%) peranal (Foley’s catheter) or wash (Ryle’s tube 10ml 6 hourly) • Urinary catheter for hourly urine monitoring. • I.V antibiotics; e.g. ampicillin, genta, cefuroxime, metro). • Laboratory evaluation of CBC, serum electrolytes, urea, creatinine. • Mechanical respiratory support and monitor vitals. • AFTER, ASSESS & PLAN IFOPERATIVE/Surgical OR NON OPERATIVE MGT. 10
  • 11. Surgery Surgical management involves EX-Lap in complicated cases. • A double barrel ileostomy • Bishop- Koop operation(very sick neonates) • Santulli operation • Resection and anastomosis (adequately fit child)
  • 14. Complications • Volvulus - sigmoid. • Bowel infarction • Bowel atresia • Bowel perforation • Meconium peritonitis 14
  • 15. Differential diagnosis • Hirschsprung’s disease • Meconium plug syndrome • Anorectal malformations • Malrotation • Atresia • Sepsis (NEC) • Electrolyte imbalance 15
  • 16. Associated syndrome Meconium plug syndrome Also reported in CF. In this condition transient obstruction of the distal colon occurs. • Manifests in the first 2 days after birth. • Also known as functional colonic obstruction. • A tenacious mass of mucus prevents the meconium from passing out. • Symptoms include abdominal distension and vomiting. • MPS has a reduced risk of intestinal perforation. • In barium enema, MPS shows a normal or dilated colon. 16
  • 17. Prognosis • MI is curable, once treated adequately. There’s no relapse. • Survival at 1 year is 90% in patients with simple meconium ileus. • Further prognosis depends on the probable presence of underlying systemic disease such as Cystic Fibrosis. • Tx then concentrates on enzyme replacement therapy and control of recurrent pulmonary infections. • Future prospects include Gene transfer therapy. 17
  • 18. Meconium peritonitis • Is consequent upon a fetal intestinal perforation. The baby is born with a firm, distended, discoloured abdomen and signs of obstruction. • Meconium escapes into peritoneum leading to peritonitis. • Infants present with a distended abdomen, vomiting. • A prenatal U/S scan shows calcifications in the peritoneum. • An abdominal x-ray shows dilated intestinal loops and areas of calcification. 18
  • 19. Clinical manifestations • Baby is born with a firm, distended abdomen • Discolored abdomen • Signs of intestinal obstruction i.e.; abdominal tenderness, fever , shock.
  • 20. Treatment • Occasionally, the perforation resolves spontaneously before birth, but most neonates with meconium peritonitis will need surgery.
  • 21.
  • 22. References • Bailey and love short practice of surgery • Schwartz’s principles of surgery,10th edition • SRB manual of surgery