3. intestinal obstructionintestinal obstruction
in the neonate is one of the most common diagnosesin the neonate is one of the most common diagnoses
requiring admission to the neonatal intensive care unit,requiring admission to the neonatal intensive care unit,
accounting for as many as one third of all admissions.accounting for as many as one third of all admissions.
Failure to pass meconium within the first 24 to 48Failure to pass meconium within the first 24 to 48
hours of life, feeding intolerance, abdominal distention,hours of life, feeding intolerance, abdominal distention,
and bilious emesis are hallmarks of intestinaland bilious emesis are hallmarks of intestinal
obstruction in the newborn period and evoke aobstruction in the newborn period and evoke a
differential diagnosis of obstruction based ondifferential diagnosis of obstruction based on
anatomic, metabolic, and functional considerations.anatomic, metabolic, and functional considerations.
Strictly speaking, the termStrictly speaking, the term meconium diseasemeconium disease refers torefers to
meconium ileus and meconium plug syndrome.meconium ileus and meconium plug syndrome.
4. MECONIUM ILEUSMECONIUM ILEUS
Meconium ileusMeconium ileus is one of the most common causes ofis one of the most common causes of
intestinal obstruction in the newborn, accounting forintestinal obstruction in the newborn, accounting for
9% to 33% of neonatal intestinal obstructions.9% to 33% of neonatal intestinal obstructions.
It is characterized by extremely viscid, protein-rich,It is characterized by extremely viscid, protein-rich,
inspissated meconium causing an intraluminal obturator typeinspissated meconium causing an intraluminal obturator type
obstruction of the distal ileum.obstruction of the distal ileum.
It is the earliest clinical manifestation of cystic fibrosis (CF),It is the earliest clinical manifestation of cystic fibrosis (CF),
occurring in approximately 16% of patients with that disease.occurring in approximately 16% of patients with that disease.
Although meconium ileus may occur with rare conditionsAlthough meconium ileus may occur with rare conditions
such as pancreatic aplasia and total colonic aganglionosissuch as pancreatic aplasia and total colonic aganglionosis
5. Due to abnormalities of exocrine mucus secretion andDue to abnormalities of exocrine mucus secretion and
pancreatic enzyme deficiency, the meconium inpancreatic enzyme deficiency, the meconium in
meconium ileus differs from normal meconium in that:meconium ileus differs from normal meconium in that:
â–ş it has less water contentit has less water content
â–ş lower sucrase and lactase levelslower sucrase and lactase levels
â–ş increased albumin,increased albumin,
â–ş decreased pancreatic enzymesdecreased pancreatic enzymes
â–ş concentrations of sodium, potassium, magnesium,concentrations of sodium, potassium, magnesium,
â–ş And heavy metals in meconium are reduced in CFAnd heavy metals in meconium are reduced in CF
patients.patients.
6. Cystic FibrosisCystic Fibrosis
is the most common potentially lethal genetic defect affectingis the most common potentially lethal genetic defect affecting
whites, with approximately 1200 infants with CF born eachwhites, with approximately 1200 infants with CF born each
year.year.
Approximately 30,000 children and young adults live with CF inApproximately 30,000 children and young adults live with CF in
the United States. It is an inherited autosomal recessivethe United States. It is an inherited autosomal recessive
disease with a 4% to 5% carrier rate and occurs in 1:3000 todisease with a 4% to 5% carrier rate and occurs in 1:3000 to
1:2000 live births yearly.1:2000 live births yearly.
The incidences of CF in non white populations are 1 in 15,000The incidences of CF in non white populations are 1 in 15,000
Africo-American births (much lower in native Africans), 1 inAfrico-American births (much lower in native Africans), 1 in
31,000 in Asian-American births, 1 in 10,500 Native American31,000 in Asian-American births, 1 in 10,500 Native American
Aleut (Eskimo) births, and 1:13,500 in Hispanic-white births.Aleut (Eskimo) births, and 1:13,500 in Hispanic-white births.
7. Gastrointestinal PathophysiologyGastrointestinal Pathophysiology
Cystic fibrosis is characterized by mucoviscidosis ofCystic fibrosis is characterized by mucoviscidosis of
exocrine secretions throughout the body resultingexocrine secretions throughout the body resulting
from abnormal transport of chloride ions across thefrom abnormal transport of chloride ions across the
apical membranes of epithelial cells.apical membranes of epithelial cells.
The clinical result is chronic obstruction and infection of theThe clinical result is chronic obstruction and infection of the
respiratory tract, insufficiency of the exocrine pancreas,respiratory tract, insufficiency of the exocrine pancreas,
and elevated sweat chloride levels.and elevated sweat chloride levels.
Other variants can have minimal other manifestations, suchOther variants can have minimal other manifestations, such
as patients with chronic sinusitis or men with congenitalas patients with chronic sinusitis or men with congenital
bilateral absence of the vas deferens.bilateral absence of the vas deferens.
8. Abnormal intestinal motility may also contribute to theAbnormal intestinal motility may also contribute to the
development of meconium ileus.development of meconium ileus.
Some patients with CF have prolonged small intestinalSome patients with CF have prolonged small intestinal
transit times.transit times.
Non-CF diseases associated with abnormal gut motility,Non-CF diseases associated with abnormal gut motility,
such as Hirschsprung’s disease and chronic intestinalsuch as Hirschsprung’s disease and chronic intestinal
pseudo-obstruction, have been associated with meconiumpseudo-obstruction, have been associated with meconium
ileus-like disease, signifying that decreased peristalsis mayileus-like disease, signifying that decreased peristalsis may
allow for increased reabsorption of water and theallow for increased reabsorption of water and the
development of abnormal meconium.development of abnormal meconium.
9. Prenatal Diagnosis and ScreeningPrenatal Diagnosis and Screening
The antenatal diagnosis of meconium ileus can be made inThe antenatal diagnosis of meconium ileus can be made in
two different groups, a high-risk group and a low-risk group.two different groups, a high-risk group and a low-risk group.
In the low-risk group the diagnosis is suspected when theIn the low-risk group the diagnosis is suspected when the
sonographic appearances of meconium ileus are found onsonographic appearances of meconium ileus are found on
routine prenatal ultrasonography when there is no previousroutine prenatal ultrasonography when there is no previous
family history of CF.family history of CF.
All pregnancies subsequent to the birth of a CF affected childAll pregnancies subsequent to the birth of a CF affected child
are considered high risk, and parents of a child with CF areare considered high risk, and parents of a child with CF are
considered to be obligate carriers of a CF mutation.considered to be obligate carriers of a CF mutation.
Pediatric surgeons are often asked to provide prenatalPediatric surgeons are often asked to provide prenatal
consultations to parents of fetuses suspected ofconsultations to parents of fetuses suspected of
bowel obstruction. Meconium ileus must be consideredbowel obstruction. Meconium ileus must be considered
in the differential diagnosis, particularly in the high riskin the differential diagnosis, particularly in the high risk
fetus.fetus.
10. Ultrasound EvaluationUltrasound Evaluation
Sonographic characteristics associated withSonographic characteristics associated with
meconium ileus include a hyper echoic, intra-meconium ileus include a hyper echoic, intra-
abdominal mass (inspissated meconium) dilatedabdominal mass (inspissated meconium) dilated
bowel and non visualization of the gallbladder.bowel and non visualization of the gallbladder.
Normal fetal meconium, when visualized in theNormal fetal meconium, when visualized in the
second and third trimesters, is usually hypo echoicsecond and third trimesters, is usually hypo echoic
or iso echoic to adjacent abdominal structures.or iso echoic to adjacent abdominal structures.
The sensitivity of intra-abdominal echogenicThe sensitivity of intra-abdominal echogenic
masses in the detection of meconium ileus ismasses in the detection of meconium ileus is
reported to be between 30% and 70%.reported to be between 30% and 70%.
11.
12. Clinical PresentationClinical Presentation
Meconium ileusMeconium ileus is categorized as either simple oris categorized as either simple or
complicated.complicated.
Thickened meconium begins to form in utero.Thickened meconium begins to form in utero.
As it obstructs the mid-ileum, proximal small bowelAs it obstructs the mid-ileum, proximal small bowel
dilatation and bowel wall thickening occur.dilatation and bowel wall thickening occur.
Approximately one half of these neonates presentApproximately one half of these neonates present
with a simple uncomplicated obstruction.with a simple uncomplicated obstruction.
The remaining patients present with complications ofThe remaining patients present with complications of
meconium ileus, including volvulus, gangrene,meconium ileus, including volvulus, gangrene,
atresia, and perforation, which may result inatresia, and perforation, which may result in
meconium peritonitis and giant cysticmeconium peritonitis and giant cystic
meconium peritonitis.meconium peritonitis.
13. Simple Meconium IleusSimple Meconium Ileus
In simple meconium ileus, the terminal ileum is filled with firmIn simple meconium ileus, the terminal ileum is filled with firm
concretions, is small in diameter, and molds around the inspissatedconcretions, is small in diameter, and molds around the inspissated
lumps of meconium.lumps of meconium.
Proximally, the ileum becomes dilated and filled with thick stickyProximally, the ileum becomes dilated and filled with thick sticky
meconium with gas and fluid found within the small bowel proximalmeconium with gas and fluid found within the small bowel proximal
to this area.to this area.
Newborns with uncomplicated meconium ileus can appear healthyNewborns with uncomplicated meconium ileus can appear healthy
immediately after birth.immediately after birth.
However, within 1 to 2 days, they develop abdominal distention andHowever, within 1 to 2 days, they develop abdominal distention and
bilious vomiting, Normal meconium will not be passed.bilious vomiting, Normal meconium will not be passed.
Dilated loops of bowel become visible on examination and have aDilated loops of bowel become visible on examination and have a
“doughy” character that indents on palpation.“doughy” character that indents on palpation.
The rectum and anus are often narrow, a finding that may beThe rectum and anus are often narrow, a finding that may be
misinterpreted as anal stenosis. The presentation of the patientmisinterpreted as anal stenosis. The presentation of the patient
with meconium ileus is similar to that of many types of neonatalwith meconium ileus is similar to that of many types of neonatal
small bowel obstructions. Therefore, the clinician shouldsmall bowel obstructions. Therefore, the clinician should
simultaneously consider and evaluate for malrotation, smallsimultaneously consider and evaluate for malrotation, small
intestinal atresia, colonic atresia, and meconium plug syndrome.intestinal atresia, colonic atresia, and meconium plug syndrome.
14. Complicated Meconium IleusComplicated Meconium Ileus
Complicated meconium ileus may present dramaticallyComplicated meconium ileus may present dramatically
and immediately after birth. In others, symptomsand immediately after birth. In others, symptoms
will develop within 24 hours of birth. Signs of peritonitiswill develop within 24 hours of birth. Signs of peritonitis
(abdominal distention, tenderness, abdominal(abdominal distention, tenderness, abdominal
wall erythema) as well as clinical evidence of sepsis,wall erythema) as well as clinical evidence of sepsis,
may be present on the initial neonatal examinationmay be present on the initial neonatal examination
as a result of in utero perforation or bowel compromise.as a result of in utero perforation or bowel compromise.
Abdominal distention can be so severe as toAbdominal distention can be so severe as to
cause immediate respiratory distress. A palpable masscause immediate respiratory distress. A palpable mass
suggests pseudo cyst formation, which results from in uterosuggests pseudo cyst formation, which results from in utero
bowel perforation. Often the neonate is in extremis andbowel perforation. Often the neonate is in extremis and
needs urgent resuscitation and surgicalneeds urgent resuscitation and surgical
exploration.exploration.
15. Radiographic FeaturesRadiographic Features
Uncomplicated meconiumUncomplicated meconium ileus is characterized by apatternileus is characterized by apattern
of unevenly dilated loops of bowel with the variable presenceof unevenly dilated loops of bowel with the variable presence
of air-fluid levels on abdominal radiography.of air-fluid levels on abdominal radiography.
The absence of air-fluid levels is due to the increased viscosityThe absence of air-fluid levels is due to the increased viscosity
of the meconium not allowing an air interface with the fluid.of the meconium not allowing an air interface with the fluid.
As swallowed air mixes with the tenacious meconium, bubblesAs swallowed air mixes with the tenacious meconium, bubbles
of gas develop.of gas develop.
This ground-glass appearance depends on the viscosity of theThis ground-glass appearance depends on the viscosity of the
meconium and is not a constant feature. Although each ofmeconium and is not a constant feature. Although each of
these features alone is not diagnostic of meconium ileus,these features alone is not diagnostic of meconium ileus,
collectively with a family history of CF, they strongly suggest thecollectively with a family history of CF, they strongly suggest the
diagnosis. Radiographic findings.diagnosis. Radiographic findings.
inin complicated meconiumcomplicated meconium ileus vary with the complication.ileus vary with the complication.
16. Figure. This abdominalFigure. This abdominal
radiograph in a neonateradiograph in a neonate
with meconiumwith meconium
ileus shows the typical ground-ileus shows the typical ground-
glass appearance in theglass appearance in the
right lower abdomen. Alsoright lower abdomen. Also
note the different-sizednote the different-sized
loops of distended smallloops of distended small
bowel.bowel.
17. Figure . ClassicFigure . Classic
radiographic findings ofradiographic findings of
meconium ileusmeconium ileus
are seen on this retrogradeare seen on this retrograde
contrast study. First, acontrast study. First, a
“micro colon of“micro colon of
disuse” is seen. The colondisuse” is seen. The colon
is extremely small andis extremely small and
unused. Second,unused. Second,
inspissated pellets (fillinginspissated pellets (filling
defects) of meconiumdefects) of meconium
are seen in theare seen in the
more proximal small bowel.more proximal small bowel.
Third, note there is aThird, note there is a
small bowelsmall bowel
obstruction as the contrastobstruction as the contrast
material has notmaterial has not
reached the markedlyreached the markedly
dilated loops of smalldilated loops of small
bowel.bowel.
18. Diagnostic TestingDiagnostic Testing
The diagnosis of CF is established with a sweat testThe diagnosis of CF is established with a sweat test
at several weeks of life.at several weeks of life.
A concentration of 60 mmol/L in 100 mg of sweat is diagnosticA concentration of 60 mmol/L in 100 mg of sweat is diagnostic
of CF with 40 to 60 mmol/L being intermediateof CF with 40 to 60 mmol/L being intermediate
(but more likely to be diagnostic in infants) and less than(but more likely to be diagnostic in infants) and less than
40 mmol/L being normal.40 mmol/L being normal.
To ensure an adequate sweat sample, the test is typicallyTo ensure an adequate sweat sample, the test is typically
performed at several weeks of life.performed at several weeks of life.
Neonatal CF screening programs using the Guthrie bloodNeonatal CF screening programs using the Guthrie blood
spot test for raised concentrations of immune reactivespot test for raised concentrations of immune reactive
trypsinogen are available in many countries but musttrypsinogen are available in many countries but must
be confirmed in a two-stage approach incorporatingbe confirmed in a two-stage approach incorporating
CFTRCFTR mutation analysis.mutation analysis.
19. TreatmentTreatment
Non operative Management of Simple Meconium IleusNon operative Management of Simple Meconium Ileus
Treatment of meconium ileus has evolved over theTreatment of meconium ileus has evolved over the
past 55 years. Neonates should initially be managedpast 55 years. Neonates should initially be managed
as having a newborn intestinal obstruction. Volumeas having a newborn intestinal obstruction. Volume
resuscitation, gastric decompression, and mechanicalresuscitation, gastric decompression, and mechanical
respiratory support should be provided as necessary.respiratory support should be provided as necessary.
Correction of any coagulation disorders and empiricalCorrection of any coagulation disorders and empirical
broad-spectrum antibiotic coverage complete this initialbroad-spectrum antibiotic coverage complete this initial
management. When meconium ileus is suspectedmanagement. When meconium ileus is suspected
or diagnosed, a diagnostic enema or hyperosmolaror diagnosed, a diagnostic enema or hyperosmolar
enema is performed initially to exclude other causesenema is performed initially to exclude other causes
of neonatal intestinal obstruction. Many newbornsof neonatal intestinal obstruction. Many newborns
with meconium ileus can be managed non operativelywith meconium ileus can be managed non operatively
with hyperosmolar enema washouts.with hyperosmolar enema washouts.
20. Operative ManagementOperative Management
Simple Meconium IleusSimple Meconium Ileus
The indications for operative management of simple meconiumThe indications for operative management of simple meconium
ileus are inadequate meconium evacuation or a complicationileus are inadequate meconium evacuation or a complication
of the contrast enema (e.g. perforation).of the contrast enema (e.g. perforation).
Failure of non operative treatment with hyperosmolar enemasFailure of non operative treatment with hyperosmolar enemas
may result from the technical inability to advance the enemamay result from the technical inability to advance the enema
column for a sufficient distance into the ileum or from ancolumn for a sufficient distance into the ileum or from an
unsuspected, associated intestinal atresia. If the enema failsunsuspected, associated intestinal atresia. If the enema fails
to promote passage of meconium within 24 to 48 hours, orto promote passage of meconium within 24 to 48 hours, or
two attempts at washout are unsuccessful, an operativetwo attempts at washout are unsuccessful, an operative
approach is indicated.approach is indicated.
21. Figure. AtFigure. At
operation, theoperation, the
meconium in ameconium in a
neonate withneonate with
cystic fibrosiscystic fibrosis
is very thickis very thick
and tenacious.and tenacious.
22. Complicated Meconium IleusComplicated Meconium Ileus
An operation is almost always indicated in cases ofAn operation is almost always indicated in cases of
complicated meconium ileus.complicated meconium ileus.
One exception is a rare in-utero spontaneouslyOne exception is a rare in-utero spontaneously
sealed perforation with intact intestinal continuitysealed perforation with intact intestinal continuity
and extra-luminal intra- peritoneal calcifiedand extra-luminal intra- peritoneal calcified
meconium.meconium.
Surgical management includes early diagnosis,Surgical management includes early diagnosis,
debridement of necrotic material, pseudo cystdebridement of necrotic material, pseudo cyst
resection, diverting stoma (s),resection, diverting stoma (s),
antibiotics, and meticulous postoperative care.antibiotics, and meticulous postoperative care.
Creation of an ostomy is the fastest and safestCreation of an ostomy is the fastest and safest
course, alleviating concern over bowelcourse, alleviating concern over bowel
discrepancy, anastomotic breakdown, and returndiscrepancy, anastomotic breakdown, and return
of bowel activity. Decortications of the cyst wall isof bowel activity. Decortications of the cyst wall is
recommended, if possible.recommended, if possible.
23. Postoperative ManagementPostoperative Management
The initial postoperative management involves ongoingThe initial postoperative management involves ongoing
resuscitation. Maintenance fluids and replacement ofresuscitation. Maintenance fluids and replacement of
insensible fluid losses, as well as gastrointestinal lossesinsensible fluid losses, as well as gastrointestinal losses
(nasogastric suction and ileostomy) must be carefully(nasogastric suction and ileostomy) must be carefully
monitored.monitored.
Instillation of 2% or 4%Instillation of 2% or 4% NN- acetylcysteine via a- acetylcysteine via a
nasogastric tube or ileostomy will help solubilize residualnasogastric tube or ileostomy will help solubilize residual
meconium.meconium.
In the patient with fetal or neonatal bowel obstruction, CFIn the patient with fetal or neonatal bowel obstruction, CF
must be suspected, and diagnostic tests should bemust be suspected, and diagnostic tests should be
performed as soon as possible. Stomas placed in theperformed as soon as possible. Stomas placed in the
course of surgical management should be closed ascourse of surgical management should be closed as
soon as possible (4 to 6 weeks) to help avoid prolongedsoon as possible (4 to 6 weeks) to help avoid prolonged
problems with fluid, electrolyte, nutritional losses, andproblems with fluid, electrolyte, nutritional losses, and
cholestatic jaundice.cholestatic jaundice.
24. PrognosisPrognosis
Improved survival in infants with meconium ileus canImproved survival in infants with meconium ileus can
be attributed to many factors. Because ofbe attributed to many factors. Because of
advances in prenatal diagnosis, pulmonary andadvances in prenatal diagnosis, pulmonary and
neonatal intensive care, nutrition, antibiotics,neonatal intensive care, nutrition, antibiotics,
anesthesia, operative management, and ananesthesia, operative management, and an
improved understanding of the pathophysiologyimproved understanding of the pathophysiology
and treatment of the complications of CF, theand treatment of the complications of CF, the
prognosis for infants with both complicated andprognosis for infants with both complicated and
simple meconium ileus has improved dramatically.simple meconium ileus has improved dramatically.
Survival rates of 85% to 100% have been reportedSurvival rates of 85% to 100% have been reported
in uncomplicated meconium ileus and up to 93% inin uncomplicated meconium ileus and up to 93% in
complicated cases.complicated cases.
25. MECONIUM PLUG SYNDROMEMECONIUM PLUG SYNDROME
Meconium plug syndrome, a cause of newborn colonicMeconium plug syndrome, a cause of newborn colonic
obstruction, was first described in 1956.It was hypothesizedobstruction, was first described in 1956.It was hypothesized
that either colonic motility or the character of the meconiumthat either colonic motility or the character of the meconium
was altered, thereby preventing normal passage andwas altered, thereby preventing normal passage and
decompression of the colon in the newborn period.decompression of the colon in the newborn period.
Under normal conditions, the terminal 2 cm of neonatalUnder normal conditions, the terminal 2 cm of neonatal
meconium is firm in texture, forming a whitish cap.meconium is firm in texture, forming a whitish cap.
Most newborns pass this cap of meconium before, during, orMost newborns pass this cap of meconium before, during, or
shortly after delivery.shortly after delivery.
One in 500 newborns will have a longer, more tenaciousOne in 500 newborns will have a longer, more tenacious
obstructive plug. Failure to pass this plug resultsobstructive plug. Failure to pass this plug results
in meconium plug syndrome and the termin meconium plug syndrome and the term plugged-upplugged-up
babiesbabies was coinedwas coined
26. The presentation of meconium plug syndrome isThe presentation of meconium plug syndrome is
similar to that of meconium ileus. Signs include failuresimilar to that of meconium ileus. Signs include failure
to pass meconium, bilious vomiting, and abdominalto pass meconium, bilious vomiting, and abdominal
distention with an obstructive pattern on plaindistention with an obstructive pattern on plain
abdominal radiographs. Often, the meconium plugabdominal radiographs. Often, the meconium plug
may become dislodged after digital stimulation of themay become dislodged after digital stimulation of the
anus and rectum. Fortunately, colon function is generallyanus and rectum. Fortunately, colon function is generally
preserved and returns to normal after passage ofpreserved and returns to normal after passage of
the plug. Ultimately, most of these infants are foundthe plug. Ultimately, most of these infants are found
to be healthy.to be healthy.
27. Pathologic causes of meconium plug syndromePathologic causes of meconium plug syndrome
include:include:
CF, small left colon syndrome, and Hirschsprung’sCF, small left colon syndrome, and Hirschsprung’s
disease.Less common causes include congenitaldisease.Less common causes include congenital
hypothyroidism, maternal narcotic addiction, andhypothyroidism, maternal narcotic addiction, and
neuronal intestinal dysplasia. Affected newborns shouldneuronal intestinal dysplasia. Affected newborns should
undergo contrast enemas, which may be therapeutic asundergo contrast enemas, which may be therapeutic as
well as diagnostic. After resolution, a sweat test should bewell as diagnostic. After resolution, a sweat test should be
performed to evaluate for CF and a thyroid-stimulatingperformed to evaluate for CF and a thyroid-stimulating
hormone level should be checked. All patients withhormone level should be checked. All patients with
slow passage of meconium require close observationslow passage of meconium require close observation
of their stool pattern. Historically, a rectal biopsy hasof their stool pattern. Historically, a rectal biopsy has
been recommended to rule out Hirschsprung’s diseasebeen recommended to rule out Hirschsprung’s disease
28. A recent report documents a 13% incidenceA recent report documents a 13% incidence
of Hirschsprung’s disease in a neonate with meconiumof Hirschsprung’s disease in a neonate with meconium
plug syndrome. Therefore, a suction biopsy could beplug syndrome. Therefore, a suction biopsy could be
reserved for those neonates with an abnormal stoolingreserved for those neonates with an abnormal stooling
patternpattern