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anesthetic to minimize the physiologic insult to the infant.

In 1969 a novel therapy applied a transrectal irrigation and


solubilization of the desiccated and tenacious meconium with
the use of the hyperosmolar diatrizoate (Gastrografin) enema,
permitting treatment of uncomplicated meconium ileus with-
out the need for any operative intervention, a success that has
been sustained for more than two thirds of patients.16 By the
early 1970s these advances along with an improved
management of the electrolyte, nutritional, and pulmonary
abnormalities of CF had improved patient survival to almost
75%.17,18
In the past 2 decades we have seen continued progress.
First, the genetic lesion of a mutation in the CF transmem-
brane regulator (CFTR) gene was defined as the causal lesion
of CF,19,20 and it is now recognized that patients with meco-
nium ileus likely represent a distinct phenotype with earlier
presentation and worse pulmonary function.21,22 Second,
the diagnostic criteria remain largely the same clinically, with
definitive diagnosis achieved by a combination of biochemical
and now genetic testing. Interestingly, when enema irrigation
is not successful in managing meconium ileus and relieving its
bowel obstruction, the preferred operative intervention is that
described originally in 1948, namely, enterotomy followed by
CHAPTER 83 irrigation, typically using meconium-solubilizing agents.
These strategies, coupled with aggressive attention to the
pulmonary and nutritional status of the patient, have seen a

Meconium Ileus
continued improvement of patient survival now approaching
95% to 100%.17,23,24

Moritz M. Ziegler Pathophysiology


------------------------------------------------------------------------------------------------------------------------------------------------

GENETICS
Cystic fibrosis is an autosomal recessive disease that occurs
History among whites with a heterozygote frequency of 1 in 29 births.
------------------------------------------------------------------------------------------------------------------------------------------------
It is rare in blacks (1/17,000 live births), and it is almost
Meconium ileus was first described in 1905 when Landsteiner unheard of in Asians (1/90,000 live births) and Africans.
linked the observation of meconium obstructing the small The 5% to 6% carrier rate of the abnormal gene in whites
bowel with pathologic changes in the pancreas, which he attrib- results in a CF incidence of 1 per 1150 to 2500 live births.
uted to a putative enzyme deficiency. Subsequent authors rec- An offspring of two heterozygous parents has a 1 in 4 chance
ognized a similar association of a thickened and tenacious of developing the disease.25 Meconium ileus, potentially
meconium with a mechanical pancreatic duct obstruction or unique to CF, represents a disorder of neonates characterized
stenosis.1–4 The term cystic fibrosis of the pancreas was first by intestinal obstruction secondary to the intraluminal accu-
coined in 1936 by Fanconi to describe the association of chronic mulation of inspissated and desiccated meconium. Meconium
pulmonary disease of infancy with pancreatic insufficiency.5 ileus is the earliest clinical manifestation of CF because such
However, it was Anderson who described, in 1938, the relation- patients are typically diagnosed and treated in the immediate
ship of the histologic likeness of pancreatic abnormalities in neonatal period.26 Meconium ileus has been reported to be
both meconium ileus and cystic fibrosis (CF) and described the presenting feature of 20.8% of the CF population in the
meconium ileus as an early and more severe manifestation of United States. Importantly for in-utero diagnosis, a family his-
the overall lung and pancreatic insufficiency.6,7 Subsequent au- tory of CF has been found in 10% to 40% of new patients with
thors defined the inspissated nature of meconium, linking the meconium ileus. In the presence of an index case of meco-
finding to both pancreatic insufficiency, as well as to abnormal nium ileus complicating CF, reports have suggested that an
mucus secreted by the intestine of patients with CF.8–13 incidence of subsequent meconium ileus in the presence of
With progress in the science of genetics, CF is now recog- CF varies from 30% to 40%.
nized as the most common potentially lethal autosomal reces- In 1989 the genetic mutation that codes for the cell mem-
sive disease in the Caucasian population. Meconium ileus brane protein termed the CF transmembrane regulator was
itself carried an almost uniformly fatal prognosis into the late identified by Francis Collins as that locus associated with
1940s,13–15 when a series of interventions were devised that the diagnosis of CF.27,28 This locus was identified on the long
by design either eliminated or bypassed the obstructing arm of chromosome 7, band q31.29 The protein was identified
inspissated intraluminal meconium, minimizing the operative as a cyclic adenosine monophosphate–induced chloride
intestinal manipulation and even the need for a general channel that regulates ion flow across the apical surface of

1073

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1074 PART VII ABDOMEN

epithelial cells.30 One of more than 400 mutations of the CFTR cellular lumen.26,35 The highly concentrated material then
that have been identified produces abnormal electrolyte becomes toxic to those cells laden with the product.
content along the external apical environment of epithelial Soon after the CFTR gene was located on chromosome 7 by
membranes. Tubular structures lined by such affected epi- restriction fragment length polymorphism analysis, it was rec-
thelia will be characterized by desiccation and reduced clear- ognized that CFTR was itself a membrane chloride channel
ance of their secretions, and the affected systems include and that it further regulated chloride conductance through
epithelial cells of respiratory, gastrointestinal, biliary, pan- other channels.36 This may be the mechanism that forms
creatic, and reproductive systems. The clinical correlate of the basis for the increased levels of sodium and chloride in
this pathophysiology has included pancreatic insufficiency the eccrine sweat gland secretions that characterize patients
(90%), meconium ileus (10% to 20%), diabetes mellitus with CF. Here a defect in electrolyte resorption of sodium
(20%), obstructive biliary disease (15% to 20%), and azoo- and water by ductular cells and the impermeability of these
spermia (nearly 100%). The exact mechanism of the epithelial cells to chloride ions result in an excretory product high in
glandular abnormality and the elaboration of a hyperviscous both sodium and chloride. This theory may be supported
mucin remain unsolved. Certainly, the tenacious meconium by the known characteristics of the CFTR membrane protein,
protein and water content and its increased viscosity have which as a chloride channel, functions to regulate salt and
all been described. water transport onto epithelial surfaces. Thus a disturbed
The DF508 mutation is the most common of many CFTR function alters exocrine secretion electrolyte content and
gene locations, occurring as a homozygous pair in almost may lead to desiccation and reduced clearance of such
50% of CF patients, whereas another 25% to 30% of patients secretions.
will have one copy of this mutation. Such homozygous Meconium itself has a variable composition, as seen from
individuals nearly always phenotypically express pancreatic samples from varying intestinal locations of normal infants
exocrine insufficiency, and they also present with a higher at varying gestational ages that are contrasted to meconium
incidence of meconium ileus. A similar higher frequency of isolated from patients with CF with and without meconium
meconium ileus is also seen in DF508-expressing patients ileus.37 The meconium concentrations of sodium, potassium,
who also express G542X. However, the disease etiology is less and magnesium are almost twice as high in samples from nor-
clear when it is recognized that not all such genotypic patients mal children or well siblings than in samples from children
will have meconium ileus. The association of obstructive lung with CF with or without meconium ileus. Similarly, the
disease with meconium ileus suggests other CFTR modifier enzyme-catalyzing heavy metals are also at higher levels in
genes or even environmental factors that may play a role in this meconium from control and well siblings than from children
disorder. Furthermore, large patient consortia have failed to with CF with or without meconium ileus. Samples from
identify predictors of the complications of CF including an patients with meconium ileus also differ from normal meco-
incidence for meconium ileus. nium by containing greater amounts of protein nitrogen and
lesser amounts of carbohydrate. Analysis of the protein con-
tent of abnormal meconium results in a further identification
PATHOGENESIS
of abnormal mucoproteins. These products are two-thirds
The defect of CF is an exocrine-eccrine gland dysfunction, par- protein and one-third carbohydrate, a composition similar
ticularly of the mucus-secreting and sweat glands.31 Pancreatic to duodenal fluid from patients with CF. The origin of these
achylia was thought to be the mechanism responsible for proteins is uncertain, but they may arise from either glandular
meconium ileus because of early reports of meconium- secretions or swallowed amniotic fluid in utero. Because most
induced bowel obstruction in patients with congenital stenosis of this protein is albumin, it is speculated that the intraluminal
of the pancreatic ducts. However, data that began accumu- proteins are of plasma origin. They react with mucopolysac-
lating in the 1940s suggested that lesions in the pancreas of charides, and, in the absence of degrading enzymes, they
patients with CF were variable and were most profound in impart a highly viscid rubbery character to the involved secre-
children older than 1 year of age. In contrast, the preponder- tions. The viscosity of these mucoproteins could be further
ance of intestinal mucosal gland abnormalities in the subset of influenced by the surrounding electrolyte environment.
patients with CF and meconium ileus was more striking, sug- Finally, this increased albumin content of meconium from
gesting that these glandular lesions were the more likely origin patients with CF has led to the development of a meconium
of the tenacious material that causes the intraluminal obstruc- screening test for the diagnosis of the disease.
tion in patients with meconium ileus.32 Additional pathologic In summary, two simultaneous pathogenetic events in
data have been interpreted to suggest that intestinal glandular meconium ileus appear to begin in utero and result in an intra-
disease plays the dominant role and pancreatic disease a luminal accumulation of a highly viscid and tenacious meco-
secondary role in the pathogenesis of meconium ileus.33,34 nium: the developments of pancreatic exocrine enzyme
The glandular abnormality accounting for or producing deficiency and the secretion of hyperviscous mucus by path-
these changes is less certain. Various explanations include ologically abnormal intestinal glands. The thickened meco-
the glandular secretion of hyperpermeable mucus mediated nium accumulates and begins in utero to obstruct the
by calcium that influences the physicochemical properties intestine lumen. This accumulation accounts for the mecha-
of mucus to be viscid and permeable to the loss of water, fur- nism of the complications of meconium ileus (i.e., twist of a
ther concentrating intraductal secretions. This glandular- heavy loop with perforation, peritonitis, and cyst or atresia)
obstructing mucus would lead to the pathologic lesions noted and the pattern of abdominal distention and obstruction seen
earlier. A second hypothesis is that an impairment of fluid in the neonate. The proximal ileum dilates, and its wall
movement to and from the extravascular space through secre- thickens as it becomes filled with the tenacious and tarry
tory cells exists that may prevent the normal dilution of the meconium. Concomitantly, the narrowed distal small bowel

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CHAPTER 83 MECONIUM ILEUS 1075

and, at times, the colon contains beaded or “boxcar” concre-


tions of gray-white, putty-like inspissated meconium. The
more distal colon is small or unused, a microcolon.

Clinical Features
------------------------------------------------------------------------------------------------------------------------------------------------

SPECTRUM OF THE DISORDER


Meconium ileus presents in the neonate as uncomplicated or
simple or as complicated. In the older child or young adult the
presentation is as a meconium ileus equivalent or distal ileal
obstruction syndrome.
Uncomplicated meconium ileus typically presents immedi-
ately at birth with the recognition of abdominal distention, a
unique feature of inspissated meconium filling and obstruct-
ing the distal small bowel. This problem may first have been
heralded by an in utero genetic diagnosis of CF and an ultra- FIGURE 83-1 Newborn with abdominal distention at birth. The family
sound suggestive of intestinal obstruction with echogenic had a history of cystic fibrosis, and this newborn had a cytogenic diagnosis
of cystic fibrosis in utero. On fetal ultrasound examination there was
bowel. In addition to the distention, intestinal obstruction is evidence of a hyperechoic bowel wall compatible with a diagnosis of
heralded by bilious vomiting and failure to stool. meconium ileus.
In contrast, complicated meconium ileus will present either
in utero or postnatally with evidence of bowel obstruction does not follow. In the presence of an in-utero perforation with
complicated by evidence of previous intestinal perforation meconium peritonitis and “cyst” formation, a palpable abdo-
and/or necrosis: Crescents or speckles of intra-abdominal calci- minal mass, discoloration of the abdominal wall, and signs
fication may be present, or on clinical assessment there may be of peritoneal irritation are often observed. Physical evidence
evidence of peritonitis including an erythematous or edematous of hypovolemia may rapidly develop in infants with peritoni-
abdominal wall and/or demonstrable abdominal tenderness.38 tis. On passage of a nasogastric tube, a quantity of bile-stained
gastric fluid usually exceeds 20 mL.

CLINICAL HISTORY
RADIOLOGIC STUDY
A family history of CF is present in 10% to 33% of patients
with meconium ileus.39 This history, coupled with in utero In utero, meconium ileus bowel may be distended but an
amniocentesis with restriction fragment length polymorphism echogenic bowel wall in the third trimester may be diagnostic
analysis, permits the accurate diagnosis of the fetus afflicted as well.40–42 After delivery, uncomplicated meconium ileus is
with CF. Coupling this information with the results of serial characterized by a typical plain obstruction radiographic
in utero sonography permits the accurate predication of which series of the abdomen (Fig. 83-2).43,44 In addition to the
infants are at risk for the development of the intestinal supine and erect films appearing remarkably similar, the char-
obstruction of meconium ileus (about 20% of the CF popula- acteristic findings include (1) great disparity in the size of the
tion) with or without a complicating meconium cyst. Maternal intestinal loops because of the configuration of different seg-
polyhydramnios may be a feature of in utero meconium ileus, ments of the bowel; (2) no or few air-fluid levels on the erect
a finding putatively resulting from the high-grade intestinal film because swallowed air cannot layer above the thickened
obstruction. Such excess maternal fluid accumulation occurs inspissated meconium; and (3) a granular, “soap bubble,” or
in the mothers of almost 20% of newborns with meconium “ground-glass” appearance seen frequently in the right half
ileus and at a frequency still higher for the patient with a of the abdomen, a finding that requires swallowed air bubbles
complicated meconium ileus. Although in utero growth retar- to intermix within the sticky meconium (Fig. 83-3).45 Each of
dation is common, prematurity and other associated anomalies these features alone is not exclusively diagnostic of meconium
are rare. ileus and may be seen with other causes of intestinal obstruc-
tion. Collectively, however, they strongly suggest meconium
ileus. Plain radiography done for differential diagnosis will
PHYSICAL EXAMINATION
include any cause of a distal small bowel obstruction includ-
Neonates with meconium ileus are often born with abdominal ing ileal atresia, Hirschsprung disease, or the meconium plug
distention (Fig. 83-1). In fact, meconium ileus is the only syndrome. A confirming study that may support the plain
variety of neonatal intestinal obstruction that produces ab- radiographic diagnosis of meconium ileus is the contrast
dominal distention at birth before the neonate swallows air. enema. A contrast enema (whether with barium, Gastrografin,
Visible peristaltic waves and palpable, doughy bowel loops or any water-soluble contrast agent) will outline a normally
are often present. Finger pressure over a firm loop of bowel positioned colon of appropriate length but of small caliber
may hold the indentation, the so-called putty sign. In simple (see Fig. 83-3).46 It will be empty or will contain pellets of
or uncomplicated meconium ileus, no findings of peritoneal inspissated meconium. The colon will be the typical “unused”
irritation are present. The findings of a rectal examination colon or “microcolon.” If reflux of contrast agent into the
are unremarkable, but characteristically on withdrawal of terminal ileum occurs, it will outline pellets of inspissated
the examining finger a spontaneous expulsion of meconium meconium.44,47 If the contrast agent refluxes more proximally

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1076 PART VII ABDOMEN

into the ileum, the transition into dilated loops of small bowel
will be encountered. Failure to reflux contrast medium into
the proximal dilated small bowel will neither prove the diag-
nosis of meconium ileus nor determine the exact level of the
intestinal obstruction; and with this failure of the contrast me-
dium to reflux into the dilated segment, operative intervention
for diagnosis and treatment becomes necessary.

Laboratory Testing
The definitive study to confirm the diagnosis of CF is the sweat
test. With the use of the pilocarpine iontophoresis method,
sweat is collected from the infant’s forearm, leg, or back; the
amount is quantified, and the concentration of sodium and
chloride in the sample is measured.48,49 The minimum
amount of sweat to be collected is 100 mg, and a measured
concentration of sweat chloride in excess of 60 mEq/L is diag-
nostic of CF. The adequacy of the size of the sweat sample is
the factor that usually precludes application of this test to the
newborn, despite reports to the contrary. A few individuals
have been identified with elevated sweat chloride levels but
with no other features of CF. These problems, plus a poten-
tially elevated sweat sodium and chloride level in normal new-
borns, may make it necessary to defer application of the sweat
chloride test until the neonate reaches several weeks of age.
Genetic testing for CF can be done by analyzing cellular
DNA for CFTR, thus establishing the carrier status of parents
of a putative child with CF presenting with features of meco-
FIGURE 83-2 An abdominal plain upright radiograph is compatible with nium ileus. However, because of the minimum number of
the diagnosis of meconium ileus. There are dilated small bowel loops of
disparate size, few air-fluid levels, and a “ground-glass” or “soap-bubble”
mutations tested by these commercial analyses, negative
appearance in the right lower quadrant. (From Rescorla FJ, Grosfeld JL: results become less meaningful. If a family has known CFTR
Contemporary management of meconium ileus. World J Surg 1993;17:381.) mutations, then amniocentesis with fetal DNA restriction

A B
FIGURE 83-3 A, Abdominal plain radiograph is compatible with the diagnosis of meconium ileus. There are distended bowel loops of disparate size, few
air-fluid levels, and a “soap bubble” right lower quadrant appearance. B, Same patient as in A is studied further with a contrast enema. A microcolon or
unused colon is demonstrated, and contrast agent refluxes into dilated, more proximal small bowel where inspissated intraluminal pellets of meconium are
seen. These findings strongly suggest the diagnosis of meconium ileus.

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CHAPTER 83 MECONIUM ILEUS 1077

fragment length polymorphism analysis may predict a fetal CF


diagnosis.
The pathophysiologic alteration of an increased albumin
concentration in meconium may prove useful as a diagnostic
screening tool for meconium ileus.50,51 This test, which uses
a tetrabromophenolethylester blue indicator, detects meco-
nium albumin concentrations in excess of 20 mg/g of stool;
however, a persisting incidence of false-positive results was
seen from such factors as prematurity, melena, hematochezia,
and intrauterine infection. A false-negative rate has been
reported as well. This simple and relatively inexpensive tool
is straightforward and rapid, and the colorimetric indicator
is easy to interpret.23,52 Meconium from normal neonates
has an albumin concentration of less than 5 mg/g of stool,
whereas meconium from neonates with CF has values at times
in excess of 80 mg/g.53,54
Stool trypsin and chymotrypsin analysis has historically
been a popular screening test for meconium ileus.53–55 A tryp-
sin level less than 80 mg/g of stool, coupled with operative
findings, supports the diagnosis of meconium ileus. Some
centers have advocated for population CF screening using a
measurement of immunoreactive trypsinogen in blood.
Operative tissue specimens that aid in the diagnosis of CF
include intestinal (rectal) or appendiceal pathognomonic FIGURE 83-4 A contrast enema is done in a newborn with a distal bowel
obstruction evident on plain radiography. A microcolon is found leading to
changes that include goblet cell hyperplasia and the accumu- a small-caliber distal ileum, and contrast agent does not reflux into the di-
lation of secretions within the crypts or within the lumen.56 lated intestine. This is most compatible with the diagnosis of a distal intes-
If operation is done for putative meconium ileus and an tinal atresia. (From Rescorla FJ, Grosfeld JL: Contemporary management of
appendicostomy is used as a bowel intraluminal irrigation site, meconium ileus. World J Surg 1993;17:381.)
appendectomy may be warranted to obtain such a diagnostic
pathologic specimen.

DIFFERENTIAL DIAGNOSIS
The combination of family history, physical examination, and
radiologic evaluation in the neonate are the three criteria of high
sensitivity that permit an accurate clinical diagnosis of meco-
nium ileus. The addition of linkage analysis on maternal amnio-
centesis samples or a sweat test confirms the diagnosis of CF.
A definitive diagnosis depends on the confirmation of the cause
of intestinal obstruction. Meconium ileus accounts for 10% to
25% of cases in large series of patients with neonatal intestinal
obstruction. Therefore the major differential diagnosis lies with
the variety of causes of neonatal intestinal obstruction including
ileal atresia, Hirschsprung disease, neonatal small left colon,
and the meconium plug syndrome.57
Ileal atresia is usually suggested by a distal bowel obstruc-
tion pattern on plain radiographs with the associated presence FIGURE 83-5 Operative findings of a case of meconium ileus compli-
of air-fluid levels.58,59 If a microcolon is demonstrated on cated by an acquired ileal atresia. The small bowel is distended proximal
contrast enema, the contrast agent will not reflux into the to the atresia, and there are inspissated pellets of meconium in the distal
ileum and colon.
proximal dilated “atretic” bowel as it might in both meconium
ileus and Hirschsprung disease (Fig. 83-4). Once ileal atresia
is suspected, the final confirmation of the diagnosis requires or the histopathologic findings of aganglionosis seen on rectal
operative exploration. Meconium ileus can be associated with biopsy. The radiographic features of colonic Hirschsprung dis-
ileal atresia, and at operation the findings characteristic of the ease likely will include a transition zone; in patients with total
sticky meconium of meconium ileus should raise this possibil- colonic Hirschsprung disease, a reflux of contrast medium
ity (Fig. 83-5). Hirschsprung disease, especially total colonic into the terminal ileum will typically not demonstrate the fill-
aganglionosis, as well as extended small bowel aganglionosis, ing defects of meconium ileus but, rather, will show air-fluid
may also mimic meconium ileus.60–62 In fact, nearly all cases levels and a proximally dilated bowel.
of total intestinal aganglionosis were initially misdiagnosed as Neonatal small left colon syndrome may also require the
meconium ileus.63 A definitive diagnosis of Hirschsprung differentiation from meconium ileus.64–66 This abnormality
disease may be suggested both radiographically and by anor- is confined to the left colon, appears as a funnel-shaped taper-
ectal manometrics, but ultimately it depends on the histo- ing on contrast enema, and is often associated with a diagno-
chemical findings of increased acetylcholinesterase content sis of maternal diabetes, hyperthyroidism, drug abuse, or

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1078 PART VII ABDOMEN

eclampsia (Fig. 83-6). Rectal biopsy is required to exclude documented. Definitive exclusion of CF clinically requires a
Hirschsprung disease. Other features of meconium ileus are sweat chloride analysis, DNA analysis, or both.
usually not present.
Meconium plug syndrome is usually confirmed by a con-
trast enema radiograph with the finding of “plugs” or “casts” Nonoperative Management
of meconium in the sigmoid or descending colon (see ------------------------------------------------------------------------------------------------------------------------------------------------

Fig. 83-6).67–70 Such plugs will often spontaneously pass after Nonoperative management of meconium ileus depends on
withdrawal of the enema catheter and expulsion of the enema. the dissolution of the inspissated intraluminal meconium in
There is a significant association of meconium plug syndrome an otherwise patent and uncompromised ileocolon.47,78,80
with other gastrointestinal anomalies, and up to 14% of Although various solubilizing agents historically had been
neonates with CF will be seen to have meconium plug syn- administered by mouth, intraoperatively, or by rectum, the
drome.71 The pathogenesis of the meconium plug is poorly mainstay of meconium ileus treatment remained an operative
understood but may relate to a bowel hypomotility. Meconium procedure.80 In 1969 several additional reports suggested that
plug syndrome has also been associated with prematurity, solvents were effective and the value of nonoperative appli-
hypotonia, hypermagnesemia, respiratory distress, sepsis, cation of such solvents was suggested both clinically and
hypothyroidism, diabetes, and Hirschsprung disease.72,73 experimentally.81 Noblett reported the successful use of a
These associations suggest that patients with meconium plug hypertonic contrast enema in four neonates with uncompli-
syndrome should be studied with both a sweat test to exclude cated meconium ileus. She described the need to fulfill the
CF and a rectal biopsy to exclude Hirschsprung disease after flowing criteria before applying such therapy: (1) an initial
symptoms of obstruction have been relieved.74,75 diagnostic contrast enema should exclude other causes of
Meconium ileus may occur in the absence of CF in term or distal intestinal obstruction; (2) the complications of vol-
preterm infants with pancreatic or intestinal insufficiency vulus, atresia, perforation, or peritonitis must be excluded;
from a variety of causes.39,76,77 These intraluminal obstruc- (3) the enema must be performed with careful fluoroscopic
tions result from the accumulation of an inspissated sticky control; (4) intravenous antibiotics should be administered;
meconium in the terminal ileum or right colon, but the meco- (5) the patient should be attended by a pediatric surgeon
nium is neither tarlike nor resistant to conventional enema during the procedure; (6) the patient should have a full
irrigation that usually proves to be therapeutic. Whether fluid resuscitation with fluids given aggressively (one to
intestinal secretion insufficiency or a pancreatic achylia is eti- three times maintenance) during the procedure; and (7) the
ologic is variable, but rarely has pancreatic insufficiency been patient should be prepared for imminent operation should

A B
FIGURE 83-6 The differential diagnosis of meconium ileus includes several entities characterized by left colon pathology. A, A contrast enema in a new-
born presenting with a plain radiographic pattern of a distal bowel obstruction depicts tapering of the colon below the splenic flexure and an intraluminal
filling defect in the rectosigmoid colon. These findings would suggest Hirschsprung disease, neonatal small left colon syndrome, or meconium plug
syndrome. B, After the removal of the enema catheter, the patient spontaneously passed a “plug” of meconium and the obstruction was relieved. A sweat
test and a rectal biopsy may be indicated to exclude the diagnoses of cystic fibrosis and Hirschsprung disease, respectively.

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CHAPTER 83 MECONIUM ILEUS 1079

complications develop.82,83 Since that report, a Gastrografin The success of nonoperative treatment is variable.88,89 The
enema has been the standard of nonoperative treatment. initial report of Noblett suggested that up to two thirds of
Meglumine diatrizoate is a hyperosmolar, water-soluble, radi- patients were successfully treated by this technique. Advan-
opaque solution containing 0.1% polysorbate 80 (Tween 80), tages of the nonoperative therapy include a reduction in pul-
a solubilizing or wetting agent, and 37% organically bound monary morbidity and a reduced length of hospital stay.
iodine. The meglumine is a 76% aqueous solution of sodium- Disadvantages of therapy include a delay in operative inter-
methyl-glucamine salt of n1n1-diacetyl-3,5-diamino-2,4,6- vention for those unsuccessfully treated by the enema, the risk
triiodobenzoic acid, and this hypertonic solution has an osmo- of immediate and delayed intestinal injury or perforation, and
lality of 1900 mOsm/L, a property that draws fluid into the the induction of hypovolemia. Bowel injury leading to a
intestinal lumen and aids in the release of the inspissated potential perforation may be a product of repeated enemas,
meconium. After administration, both a transient osmotic diar- injudicious inflation of an enema catheter balloon, or a direct
rhea and a putative osmotic diuresis occur, factors that empha- mucosal injury induced by the enema agent.90–94 The mech-
size the importance of aggressive fluid resuscitation. In addition, anism of such an injury may be related to bowel distention or
the product is radiopaque, which enables a safe fluoroscopi- to the polysorbate 80 content. The latter injury may be pre-
cally monitored administration. Polysorbate 80 (Tween 80) is vented by using a solubilizing enema agent containing 1%
a nonionic surface-active emulsifier that may not only better to 2% polysorbate 80 with isotonic Gastrografin diluted with
define radiographically the bowel mucosal pattern but also water to a final osmolality of 320 to 340 mOsm/L or by using
facilitate the passage of the hypertonic Gastrografin between an alternate isotonic contrast agent.
the mucosa and the adherent meconium at the site of obstruc-
tion. Polysorbate 80 as a 10% solution has been administered
intraoperatively by way of an enterostomy to liquefy meco- Operative Management
nium. Other hypertonic water-soluble agents (e.g., 40% sodium ------------------------------------------------------------------------------------------------------------------------------------------------

diatrizoate [40% Hypaque]) not containing polysorbate 80 Multiple indications for operative intervention in the manage-
are also effective in relieving the obstruction, and they may ment of meconium ileus exist.95–97 One third to one half of
prevent the adverse influence of Gastrografin on colonic patients undergoing operation represent cases of simple or
mucosa.79,84 uncomplicated meconium ileus that have failed to respond
The technique of solubilizing enema treatment of meco- to nonoperative treatment with enema solubilizing agents.
nium ileus continues to use the aforementioned guidelines The remaining one half to two thirds of patients have com-
of Noblett. After fluid resuscitation and nasogastric decom- plications of meconium ileus, which include intestinal atre-
pression have been performed and after physical examination sia, volvulus, perforation, meconium cyst formation with
assessment and plain abdominal radiographs have excluded peritonitis, intestinal gangrene, or combinations of these
the diagnosis of peritonitis or perforation, the diagnostic con- events. In the management of simple meconium ileus, the
trast enema with barium or water-soluble agent is adminis- goal of operation is the relief of intraluminal ileocolonic
tered. When the preliminary diagnostic study has been obstruction by either the evacuation of the adherent intra-
completed, an enema-tip nonballoon catheter is inserted into luminal meconium or by resection of the portion of bowel
the anorectum and the buttocks are taped together around the filled with inspissated material.98
catheter. With fluoroscopic guidance and an initial solution of
50% Gastrografin in water, the contrast agent is slowly
SIMPLE MECONIUM ILEUS
injected by a catheter-tipped syringe. When contrast medium
traverses the colon and reaches the dilated meconium- A variety of operative procedures are available for the manage-
impacted ileum, the study is terminated and the infant is ment of patients with meconium ileus (Figs. 83-7 and 83-8).98
returned to a bed for monitoring, fluid administration (two The initial patient survivors underwent enterotomies with
times maintenance), and normalization of body temperature. irrigation coupled with a limited resection. This operative tech-
Spontaneous passage of the inspissated meconium per rectum nique is the one most commonly in use today. Irrigating solutions
should follow. An abdominal radiograph should be repeated may include warmed saline, a 50% diatrizoate solution, a 1%
in 8 to 12 hours to determine whether the obstruction has solution of pancreatic enzymes (Viokase; A.H. Robbins Co.,
been relieved. If instead the evacuation is incomplete and Richmond, Va.), hydrogen peroxide, and, most commonly, either
obstruction persists, the enema may be repeated with the a 2% or a 4% solution of N-acetylcysteine (Mucomyst; Apo-
same concentration of Gastrografin. If either no evacuation thecon, Princeton, N.J.).85,99 More concentrated solutions of
occurs after a successfully refluxing enema or if contrast N-acetylcysteine or Gastrografin or the use of hydrogen pero-
medium cannot be refluxed into dilated bowel, then this xide and its attendant risk of air embolism may produce greater
technique should be abandoned and operative intervention risk than benefit. After solubilization by the irrigant, injected
planned. Similarly, signs of worsening obstruction, clinical through an enterotomy catheter, the meconium is gently milked
distention, greater distention of loops on radiograph, or signs distally into the colon or evacuated through the enterotomy (see
of peritonitis resulting from a possible perforation are also Fig. 83-7). The enterotomy and the abdomen may then be
indications for operative intervention. Noblett suggests that closed, an enterostomy may be created, or the site can be con-
after a successful enema, 5 mL of a 10% N-acetylcysteine trolled by insertion of a T tube.100–103 This last treatment has
solution should be administered every 6 hours through a been designed to be located at the junction of proximal distended
nasogastric tube to liquefy upper gastrointestinal secretions.85 ileum with distal (more collapsed) ileum where intraluminal
Furthermore, when formula feedings are begun, supplemen- balls of inspissated meconium are found. Leaving this tube in
tal pancreatic enzymes must be administered with each place and attaching the enterotomy site against the anterior
feeding.86,87 abdominal wall ensures a controlled fistula, as well as a route

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1080 PART VII ABDOMEN

of gastrointestinal access for the instillation of pancreatic enzyme and enterostomy closure. An alternative technique is appendec-
solutions beginning on the first postoperative day. By the seventh tomy with appendicostomy, with meconium evacuation or irri-
to fourteenth postoperative day, the irrigant should pass freely gation through this route.104 A temporary indwelling tube
into the colon, the obstruction should be relieved, and thereafter cecostomy may alternatively be left in place. For such an irrigant
the catheter can be removed. This avoids the need for reoperation technique to be successful, the bowel must be handled gently, not
overdistended, and not excessively massaged or “milked” in an
effort to evacuate the inspissated meconium.
An alternative operation to enterotomy-irrigation is place-
ment of a temporary obstruction-relieving stoma with or with-
out an associated partial resection. Gross initially advocated
placement of the Mikulicz double-barreled enterostomy,
which could be performed quickly and which did not require
intraoperative meconium evacuation (see Fig. 83-8). The
exteriorized bowel loop could be opened after the abdominal
incision has been closed, thereby minimizing intraperitoneal
contamination. After the obstruction is relieved and the infant
has recovered, a spur-crushing Mikulicz clamp can be applied
externally at the stoma to complete a side-to-side anastomosis.
It may not be necessary to return to the operating room to
close the stoma because after the clamp-induced anastomosis,
the residual enterocutaneous fistula may spontaneously close.
An alternative to such an “extraabdominal resection” and
delayed stomal closure was the primary resection and anasto-
mosis recommended a decade later by Swenson and Noblett.
FIGURE 83-7 The most commonly preferred contemporary operative After meconium had been evacuated, a primary intraperito-
management of meconium ileus is shown. An enterotomy is made in the neal anastomosis could be performed, or the infant could
dilated small bowel segment just proximal to the site of inspissated distal be allowed to recover more fully, after which the stoma could
meconium, and an irrigating catheter is inserted. After instillation of solubiliz- be closed in delayed fashion by an end-to-end anastomosis.
ing agent, the catheter may be removed and the enterotomy closed; instead,
the catheter may be left in place or replaced with a T tube for continued post- An alternative operation is resection coupled with a distal
operative solubilizing agent instillation. (From Rescorla FJ, Grosfeld JL: chimney enterostomy, the so-called Bishop-Koop procedure
Contemporary management of meconium ileus. World J Surg 1993;17:381.) (see Fig. 83-8).105 This technique was developed with the

A B

C D

FIGURE 83-8 The operative options for simple meconium ileus. A, The Mikulicz resection and enterostomy. B, The Bishop-Koop resection and enterostomy.
C, The Santulli enterostomy. D, Tube enterostomy. (From Rescorla FJ, Grosfeld JL: Contemporary management of meconium ileus. World J Surg 1993;17:381.)

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CHAPTER 83 MECONIUM ILEUS 1081

following criteria: (1) limit intraoperative bowel trauma in


the neonatal period; (2) resect the disparately enlarged ileal loop
filled with inspissated thickened meconium; (3) create an appro-
priately sized end of proximal to side of distal ileum anastomosis
close to the abdominal wall exiting the distal ileum, to serve as a
functionally decompressing “proximal stoma” while distal ob-
struction persisted; (4) provide access for insertion of a catheter
into the distal bowel containing inspissated meconium pellets so
that solubilizing pancreatic enzymes could be instilled postop-
eratively; and (5) permit an eventual enterostomy closure by
bedside ligation of the “chimney stoma,” putatively avoiding
the risks of an additional anesthetic in a child with known CF.
Intraoperatively, an 8-Fr rubber catheter is passed through the
ostomy chimney into the distal ileum. Within 12 to 24 hours af-
ter the operation, catheter irrigations are begun with a pancreatic
enzyme solution (1 teaspoon Viokase per 1 oz water) repeated
every 4 to 6 hours until the distal intraluminal obstruction is re-
lieved, at which time the catheter is removed. After an initial large
volume of enterostomy output, the ostomy drainage will
diminish as the more distal obstruction is relieved. The transco-
lonic passage of stool will follow. Thereafter, the output from the
stoma may cease altogether. Eventually the chimney may be trea-
ted by one of two techniques. At the bedside the “stoma” may be
ligated. If the result of this noninvasive technique is a persis-
tent enterocutaneous fistula, then a formal intraperitoneal or
extraperitoneal stomal closure can be performed with the
patient under a general anesthetic. This latter procedure is FIGURE 83-9 A plain abdominal radiograph compatible with the diagno-
necessary in approximately 75% of patients treated by this sis of complicated meconium ileus. The large, central, air-filled mass
technique.17 suggests intestinal perforation and giant meconium cyst formation. If the
Santulli described a proximal chimney enterostomy, an perforation has been of longer standing, intraperitoneal calcification may
also be present. (From Rescorla FJ, Grosfeld JL: Contemporary management
operation that in essence is the reverse of the resection of meconium ileus. World J Surg 1993;17:381.)
coupled with a distal chimney enterostomy (see Fig. 83-8).
The distal ileal end is anastomosed end-to-side to the proximal
ileum at a level corresponding to an immediate subfascial “pseudocyst” fibrous wall forms around an accumulation of me-
plane, and the proximal ileum is exited as an end enterostomy. conium, and spared bowel loops are peripheral to this cyst (see
With this stoma arrangement, irrigation and decompression of Fig. 83-9). Adhesive meconium peritonitis follows meconium
the proximal ileum is enhanced. As with the Bishop-Koop contamination of the peritoneal cavity for days to weeks before
procedure, an intraoperative catheter passed through the delivery. Dense and vascular adhesions make operative relief of
stoma is positioned into the distal ileum for the postoperative the adhesive intestinal obstruction difficult. Scattered calcifi-
instillation of solubilizing agent. Because a high-output func- cations may be present. When intestinal perforation occurs only
tional end enterostomy has been created, it is necessary to close a few days before delivery, an abdomen filled with meconium
such a stoma early to avoid the complications induced by ascites results and calcification is absent. The fourth variant of
excessive fluid and electrolyte losses. meconium peritonitis is bacterially infected ascites, which
occurs when colonized intestinal organisms penetrate from
COMPLICATED MECONIUM ILEUS the perforated intestine into the peritoneal cavity.
At operation, it is mandatory to assess residual intestinal
The treatment of complicated meconium ileus almost always length, and it is critical to use conservative resection. An effec-
requires an operation,106 an exception being the rare in utero tive armamentarium of operative options is necessary to opti-
perforation that has left a telltale remnant of extraluminal mize treatment of meconium peritonitis, volvulus, atresia, or
intraperitoneal calcified meconium, a spontaneously sealed the ischemic intestine that may be encountered. Only the
perforation, and no interruption in intestinal continuity.105 presence of a diffuse bacterial peritonitis precludes a primary
Another finding for the latter process is calcified meconium anastomosis. The Bishop-Koop, Mikulicz, and Santulli proce-
seen in a patent processus vaginalis during a hernia operation dures each have application in the management of patients
or by abdominal radiograph later in life. In contrast, operative with complicated meconium ileus.
indications include persisting intestinal obstruction, an en-
larging abdominal mass, and signs of peritonitis, which may
include abdominal wall edema and discoloration, tenderness Postoperative Management
on physical examination, and clinical and laboratory signs of ------------------------------------------------------------------------------------------------------------------------------------------------

ongoing sepsis (Fig. 83-9). After the operative management of meconium ileus, care must
Meconium peritonitis may be seen as one of several varieties. be centered on support of the infant’s general physiology, as well
A meconium pseudocyst is a result of meconium accumulating as on the evacuation of any residual proximal or distal gastro-
in the peritoneal cavity for weeks to months. A calcified intestinal inspissated intraluminal meconium. Generally, the

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1082 PART VII ABDOMEN

latter can be treated by instillation of 2% or 4% acetylcysteine symptoms are heralded by crampy abdominal pain, disten-
(Mucomyst) delivered through a nasogastric tube, which will tion, and a palpable right lower quadrant mass; and a high-
solubilize the residual meconium. When gut patency is verified grade obstruction is associated with obstipation, distention,
clinically or by plain film radiograph, enteral nutrition may and vomiting. It is important to consider a broad differential
begin with an elemental formula such as Pregestimil (Mead- diagnosis that may include constipation, intussusception, and
Johnson, Evansville, Ind.). When enteral feedings begin, sup- even appendicitis; and the plain abdominal radiograph sup-
plemental pancreatic enzymes must also be started (Viokase, plemented with contrast body imaging should prove diagnos-
Pancrease [McNeil Pharmaceuticals, Spring Hill, Pa.] and tic. Once the diagnosis is established, the preferred treatment
Cotazym-S [Organon Inc., West Orange, N.J.] are alternatives). is solubilizing agents such as acetylcysteine and Gastrografin,
If postoperative ileus is prolonged or if short-bowel syndrome is given both orally and per rectum. Operative intervention is
a product of the operative treatment, then total parenteral nu- rarely required. Adjustment of oral enzyme therapy supple-
trition becomes an important early and potentially prolonged mented with better hydration and the use of stool softeners
form of postoperative nutritive therapy. Early enteral or paren- may be used to prevent recurrence. Both histamine H2
teral nutrition therapy must include maintenance of salt and blockers and proton pump inhibitors may prove efficacious
mineral balance; appropriate vitamin supplementation includ- by increasing intestinal pH, and prokinetic agents have also
ing vitamin K; and aggressive management of any associated been used.
short-bowel syndrome deficiencies. The association of the Appendiceal luminal obstruction that produces signs and
inherent malabsorption of CF coupled with the potential of symptoms compatible with both acute and “chronic appendi-
an extreme short-bowel syndrome resulting from complicated citis” may also occur.111 The diagnosis of appendiceal pathol-
meconium ileus makes for an extremely difficult management ogy depends on both clinical assessment and the potential use
combination.107,108 of contrast medium-enhanced computed tomography. These
Short-term postoperative parenteral antibiotics are used for complications and the most common gastrointestinal compli-
the care of either simple or complicated meconium ileus, but cation, malabsorption, are improved with appropriate enzyme
directed and more prolonged antibiotics may be continued replacement therapy. Malabsorption of carbohydrates, pro-
to minimize potential pulmonary complications. Vigorous tein, and fat may all occur, a problem worsened both by the
pulmonary physiotherapy is initiated early after operation extent of peritoneal inflammation and the magnitude of the
and is continued indefinitely. Teaching parents to perform short-bowel syndrome. The latter may be functional or ana-
such pulmonary physiotherapy that includes postural drain- tomic, an anatomy foreshortened because of atresia or a resec-
age is a mandatory component of predischarge planning. tion. The malabsorption is worsened by a disaccharidase
In those patients with an enterostomy, teaching parents appro- deficiency and by inspissated biliary secretions that produce
priate stomal care is also important. Only in those circum- both jaundice and impaired fat absorption. This same patho-
stances in which a Bishop-Koop ileostomy or a Mikulicz physiology may account for the occurrence of gallstones in
ileostomy was created would bedside ligation of the chimney about one fourth of patients with CF, as well as the incidence
or application of a Mikulicz clamp, respectively, be considered of symptomatic chronic calculous cholecystitis or biliary dys-
before hospital discharge. kinesia, which also characterizes this population. The nutrient
Perhaps the most important feature of postoperative care is malabsorption takes on greater significance if a concomitant
to secure the diagnosis of CF as the cause of the patient’s me- increased energy need occurs in the presence of recurrent
conium ileus. A series of reports exist in which neonatal intra- and chronic pulmonary infection. The application of total par-
luminal inspissated meconium is the cause of small bowel enteral nutrition may prove beneficial to supplement enteral
obstruction and the same management scheme as outlined nutrients and to provide gut rest, as well as appropriate calo-
earlier is applied. However, postoperatively these patients ries during periods of prolonged postoperative ileus, espe-
are not found to have abnormal sweat electrolytes. Such cases cially if anastomotic healing or closure of enterocutaneous
have been carefully distinguished from the colonic meconium fistulas is required. Furthermore, evidence suggests that the
plug syndrome because the plugging has been confined to malnutrition of meconium ileus is significantly different or
the small bowel. This condition has been reported in prema- even worse than that seen in age-matched peers who have
ture infants, in siblings without CF, in patients with partial CF without meconium ileus.
pancreatic aplasia, and in patients with pancreatic ductal Intussusception occurs in approximately 1% of older CF
stenosis.109 These findings remain an important distinction if patients, likely secondary to inspissated intraluminal stool
the alternative diagnosis is meconium ileus associated with CF. serving as the lead point. The site of pathology is typically ileo-
colic, but small bowel and large bowel intussusception may
also occur. Typically, the diagnosis is difficult to establish
Complications
------------------------------------------------------------------------------------------------------------------------------------------------ and distinguish from other right lower quadrant CF pathology,
and operation with reduction and/or resection becomes the
GASTROINTESTINAL
usual therapeutic outcome.
Many gastrointestinal complications of meconium ileus Rectal prolapse may be the first clinical presentation of a
exist and include an increased incidence of intussusception child subsequently proven by sweat test to have CF. Up to
and rectal prolapse. However, in the child and adolescent one third to one fifth of children with CF develop prolapse
with CF, the most common gastrointestinal problem is distal typically between the ages of 1 and 3 years of age. The
intestinal obstruction termed meconium ileus equivalent or preferred treatment after the diagnosis of CF is established
distal intestinal obstruction syndrome.110 A partial bowel ob- is oral enzyme therapy, and rarely will more aggressive trans-
struction by intraluminal material may be a product of steator- anal rectal submucosal sclerotherapy or operative rectopexy
rhea or noncompliance with oral enzyme therapy. Signs and be indicated.

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CHAPTER 83 MECONIUM ILEUS 1083

Colonic strictures86,87,90,94 presumptively secondary to Cumulative survival


120%
large-dose oral enzyme therapy have been reported in CF chil-
dren, half of whom previously have had meconium ileus. Most 100%
commonly such disease is localized to the right colon, but seg-
mental colitis and even pancolonic disease has been reported. 80%
The local signs of obstruction and pain must be distinguished
from the meconium ileus–equivalent patient, and a contrast 60%
enema is typically diagnostic. Regulation of enzyme therapy
may be tried, but most commonly resection is indicated for 40%
intractable strictures.
20%
PULMONARY
0%
0 5 10 15 20 25
The early morbidity and mortality of meconium ileus predom- Age (years)
inantly has a pulmonary origin, the products of which include 1960s 1970s 1980s
bacterial sepsis and bronchopneumonia. Some authors sug-
FIGURE 83-10 Long-term survival rates of patients with meconium ileus
gest that beyond 6 months after the treatment of meconium depicted by decade. A statistically significant improvement in survival is
ileus, the prognosis and morbidity assume that of a patient seen when 1960s, 1970s, and 1980s survival data are compared (P <
with CF without meconium ileus. However, other studies 0.0001). (From Del Pin CA, Czyrko C, Ziegler MM, et al: Management and
suggest that children with meconium ileus have worse lung survival of meconium ileus: A 30-year review. Ann Surg 1992;215:179-185.)
function and more obstructive lung disease between ages
8 and 12 years than those with CF but without meconium operatively, survival rates at 5 and 10 years for the two catego-
ileus. These changes occur with relatively reduced lung vol- ries improved steadily from the 1960s (30% and 10%, respec-
umes. When Pseudomonas, coliforms, or other specific colo- tively) through the 1970s (80% and 70%, respectively) and
nizing or pathogenic organisms are identified, the early and the 1980s (100% at 5-year follow-up).27,116–118 In the past,
aggressive use of aminoglycosides and semisynthetic penicil- nonoperative treatment had contributed to an improved sur-
lins has been shown to improve survival and outcome, even if vival rate. But in the past decade the survival rate for both op-
used in a prophylactic manner. Finally, the use of aggressive erative and nonoperative treatment was 100%. The significant
physical therapy and postural drainage may further minimize improvement in operative survival has come since the 1960s
pulmonary complications. when the 6-month survival rate was only 33%.119 The oper-
ative 6-month survival rate had improved to 60% before
INGUINOSCROTAL DISEASE 1979 and to 100% between 1979 and 1989.120 No significant
overall differences in outcome were observed with regard to
Children with CF have been reported to have an increased patient gender, whether complication of meconium ileus
incidence of inguinal hernias and hydroceles, as well as an was present, or with regard to the type of operation performed
increased incidence of cryptorchidism.112,113 Two operative (ileostomy, resection with primary anastomosis, resection and
findings at the time of inguinal hernia repair may suggest Mikulicz ileostomy, and Bishop-Koop enterostomy). Addi-
the underlying diagnosis of CF, namely, the presence of calci- tionally, the long-term survival rates (measured at 6 years)
fied meconium in the hernia sac and the absence of a vas of patients treated with the Bishop-Koop procedure (62%
deferens.114 Both findings in the absence of a previous diag- survival) did not differ from those of patients with alternate
nosis of CF suggest the need for a diagnostic sweat test. In operations. Furthermore, all deaths in patients older than 6
addition, infertile males without a vas deferens may actually months of age were cardiopulmonary or pneumonitic deaths
be asymptomatic heterozygotes carrying the CF gene. related to the underlying CF and not to complications of
operation. Interestingly, both simple and complicated cases
of meconium ileus had 72% 10-year survival rates.121,122
Results of Treatment Death occurs from multiple causes, which include intra-
------------------------------------------------------------------------------------------------------------------------------------------------
peritoneal sepsis from unrecognized leakage, pulmonary
The outcome of the treatment of patients with meconium sepsis and bronchopneumonia, or short-bowel syndrome
ileus, whether the condition is complicated or simple, has with complicating liver failure.123–125
steadily improved during the past 3 decades, the most recently
reported survival rates approaching 100% (Fig. 83-10).115,17 The complete reference list is available online at www.
In a series of patients treated both nonoperatively and expertconsult.com.

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