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JEJUNOILEAL ATRESIA AND STENOSIS

Etiology
Jejunoileal atresia occurs in approximately 1 in 5,000 live births. It occurs equally in
males and females, and about one in three infants is premature.56 Although the majority of
cases are thought to occur sporadically, familial cases of intestinal atresias have been
described.57 It is generally accepted that jejunoileal atresia occurs as a result of an
intrauterine ischemic insult to the midgut, affecting single or multiple segments of the
already developed intestine.13,58–61 Intrauterine vascular disruption can lead to ischemic
necrosis of the bowel with subsequent resorp- tion of the affected segment or segments
(Fig. 30-9).

The hypothesis that most cases of jejunoileal atresia occur secondary to vascular
disruption during fetal life is derived from experimental as well as clinical evidence.
Isolated mesenteric vascular insults and interference with the segmental blood supply to
the small intestine were created in fetal dogs, and resulted in different degrees and
patterns of intraluminal obstruction, reproducing the spectrum of stenosis and atresia
found in humans.62–64 Moreover, the presence of bile, lanugo hair, and squa- mous
epithelial cells from swallowed amniotic uid distal to an atresia suggests that the atresia
occurs subsequent to some event, but that at some time in gestation the intestinal lumen
was patent, thus allowing passage of these contents. Additionally, atresias seen in
association with other intrauterine vascular insults such as fetal intussusception, midgut
volvulus, thromboembolic occlusions, transmesenteric internal hernias, and
End arteries

Vessel thrombosed
Relative ischemia

Impaired function

Critical ischemia Irreversible changes Disintegration Atretic segment

FIGURE 30-9 ■ The proposed mechanism of vascular compro- mise and subsequent development of
jejunoileal atresias is depicted.
incarceration or snaring of bowel in an omphalocele or gastroschisis have contributed to
wide acceptance of this hypothesis.58,64–68

The presence of associated extra-abdominal organ abnormalities in jejunoileal atresia is


low (<10%) due to its occurrence later in fetal life and the localized nature of the vascular
insult.69 Rarely, jejunoileal atresia has been found in patients with Hirschsprung disease,
cystic bro- sis, malrotation, Down syndrome, anorectal and vertebral anomalies, neural
tube defects, congenital heart disease, and other GI atresias.56,69,70 Methylene blue,
previously used for amniocentesis in twin pregnancies, has been implicated in causing
small bowel atresia.71

Although jejunoileal atresias are usually not heredi- tary, there is a well-documented
autosomal recessive pattern of inheritance of multiple atresias.72 In these cases, intestinal
rotation was normal, mesenteric defects were never observed, and lanugo hairs and
squamous cells were not identi ed distal to the most proximal atresia. All these ndings
suggest an early intrauterine event. Survival is poor in these infants, even with success-
ful bowel resection.

No correlations have been found between jejunoileal atresia and parental or maternal
disease. However, the use of maternal vasoconstrictive medications, as well as maternal
cigarette smoking in the rst trimester of preg- nancy, has been shown to increase the risk
of small bowel atresia.73 Chromosomal abnormalities are seen in less than 1% of the
patients with jejunoileal atresia.74

30 Duodenal and Intestinal Atresia and Stenosis 419

Pathology
The Grosfeld classi cation system separates these defects into four groups, with an
additional consideration for type III(b) (Fig. 30-10).4 This classi cation has signi cant
prognostic and therapeutic value as it emphasizes the importance of associated loss of
intestinal length, abnor- mal collateral intestinal blood supply, and concomitant atresia or
stenosis.75 Regarding classi cation, the most proximal atresia determines whether the
atresia is classi- ed as jejunal or ileal atresia. Multiple atresias are found in up to 30% of
patients.56,76

Stenosis

Stenosis is de ned as a localized narrowing of the intes- tinal lumen without disruption in
the intestinal wall or a defect in the mesentery (see Fig. 30-10A). At the stenotic site, a
short, narrow, somewhat rigid segment of intestine with a small lumen is found. Often the
muscularis is irregular and the submucosa is thickened. Stenosis may also take the form
of a type I atresia with a fenestrated web. Patients with jejunoileal stenosis usually have a
normal length of small intestine.

Stenosis

Type I

Type II
D

Type III (a)

Type III (b)

Type IV

FIGURE 30-10 ■ The classi cation system for jejunoileal atresia and stenosis is seen.

420 SECTION IV Abdomen Type I Atresia

In type I jejunoileal atresia, the intestinal obstruction occurs secondary to a membrane or


web formed by both mucosa and submucosa, while the muscularis and serosa remain
intact (see Figs 30-10B and 30-11). On gross inspection, the bowel and its mesentery
appear to be in continuity. However, the proximal bowel is dilated while the distal bowel
is collapsed. With the increased intralu- minal pressure in the proximal bowel, bulging of
the web into the distal intestine can create a windsock effect. As with stenosis, there is no
foreshortening of the bowel in type I atresias.

Type II Atresia

The clinical ndings of a type II atresia are a dilated, blind-ending proximal bowel loop
connected by a brous cord to the collapsed distal bowel with an intact
FIGURE 30-11 ■ In this infant with abdominal distention and evi- dence of a congenital bowel obstruction, a
type I jejunal atresia was found. Note the mesentery is intact and the small, distal jejunum is connected to the
very dilated, proximal jejunum.

mesentery (see Fig. 30-10C). Increased intraluminal pressure in the dilated and
hypertrophied proximal bowel may lead to focal proximal small bowel ischemia. The
distal collapsed bowel commences as a blind end, which sometimes assumes a bulbous
appearance owing to the remains of an intussusception. Again, the total small bowel
length is usually normal.
Type III(a) Atresia

In type III(a) atresia, the proximal bowel ends blindly with no brous connecting cord to
the distal intestine. A V-shaped mesenteric defect of varying size is present between the
two ends of intestine (see Figs 30-10D and 30-12). The dilated, blind-ending proximal
bowel is often aperistaltic and frequently undergoes torsion or becomes overdistended,
with subsequent necrosis and perforation occurring as a secondary event.77 In this sce-
nario, the total length of the small bowel is variable (but usually less than normal), owing
to intrauterine resorp- tion of the affected bowel.

Type III(b) Atresia

Type III(b) atresia (apple-peel, Christmas tree, or Maypole deformity) consists of a


proximal jejunal atresia, absence of the superior mesenteric artery beyond the origin of
the middle colic branch, agenesis of the dorsal mesentery, a signi cant loss of intestinal
length, and a large mesenteric defect (see Fig. 30-10E). The decom- pressed distal small
bowel lies free in the abdomen and assumes a helical con guration around a single
perfusing vessel arising from the ileocolic or right colic arcades (Fig. 30-13).
Occasionally, additional type I or type II atresias are found distal to the initial atresia.
Also, the vascularity of the distal bowel is often impaired. This type of atresia has been
found in families with a pattern sug- gestive of an autosomal recessive mode of
inheritance. It also has been encountered in siblings with identical lesions and in twins.77–
80

The occurrence of intestinal atresia in other siblings, the association of multiple atresias
(15%), and the dis- cordance in a set of apparently monozygotic twins may point to more
complex genetic transmission with an
FIGURE30-12 ■ This baby was suspected of having a proximal jejunal atresia based on symptoms of bilious
emesis and the abdominal lm. (A, B) At operation, this type III(a) jejunal atresia was found. Note the V-shaped
mesenteric defect between the dilated proximal atretic bowel and the distal decompressed bowel. Due to the
size discrepancy between the two ends of the intestine, the proximal dilated bowel was resected at the arrow
and an end-to-side anastomosis was performed. (C) The operation was performed through a slightly extended
umbilical incision. The baby recovered nicely and no complications developed.

30 Duodenal and Intestinal Atresia and Stenosis 421


FIGURE30-13 ■ (A) The operative ndings in neonate with a type III(b) intestinal atresia are seen. Note the
classic ‘apple-core’ or ‘Christmas tree’ deformity as well as the wide mesenteric gap between the proximal
dilated bowel and distal decompressed ileum. Also, the colon and distal small bowel are perfused through a
single artery (arrow) running through the mesentery of the distal bowel. (B) This operation was performed
through a small umbilical incision. This baby recovered nicely and has not developed any complications.

overall recurrence rate of 18%.78,81,82 Infants with this anomaly are often premature, and
up to 50% may have malrotation. Short bowel syndrome is present in nearly 75% of
cases. Accordingly, there is increased morbidity (63%) and mortality (54%) in this
population.78,83 Type III(b) atresias are most likely the result of a proximal superior
mesenteric arterial occlusion with extensive infarction of the proximal segment of the
midgut. Also, it can develop from a midgut volvulus.58,84 Primary failure of development
of the distal superior mesenteric artery has also been suggested as an etiologic factor.
However, this is unlikely because meconium is usually found in the bowel distal to the
atresia. This nding indicates that the atresia develops after bile secretion begins, which
occurs around week 12 of intrauterine life. The superior mesenteric artery develops much
earlier than 12 weeks.85

Type IV atresia

Multiple-segment atresias or a combination of types I to III are classi ed as type IV (see


Fig. 30-10F). Twenty to 35% of infants affected with jejunoileal atresia present with
multiple atresias.56,76 The majority of cases of multiple-segment atresias are sporadic with
no other family history of intestinal abnormalities. They are likely a result of multiple
vascular insults to the mesentery, intrauterine in ammatory processes, or a malformation
of the GI tract occurring during embryonic develop- ment.76,86 Embolic material from a
nonviable fetus to a living monochorionic twin through placental vascular connections
could also account for single or multiple intestinal atresias.87 Associated defects,
particularly abnor- malities of the central nervous system, have been noted in
approximately 25% of nonfamilial multiple intestinal atresia patients.76 Multiple atresias
have also been seen in association with severe immunode ciency.23

A familial form of multiple intestinal atresia (FMIA) involving the stomach, duodenum,
and both the small

and large bowel has been described.72,88 It is associated with prematurity and shortened
bowel length. To date, it has been uniformly fatal. It is associated with type I and II
atresias, with type II predominating. An autosomal recessive mode of transmission has
been suggested for this familial condition because it is unlikely that an iso- lated prenatal
vascular accident is responsible for such extensive involvement of the GI tract. In
addition, infants affected with this familial form are found to have long segments of
completely occluded small or large intestine without a recognizable lumen.88–90 Another
pathogno- monic feature seen in FMIA is the sieve-like appearance of the intestine on
histologic examination where multiple lumina are surrounded by epithelial cells and
muscularis mucosa.88

Pathophysiology
The vascular and subsequent ischemic insult not only causes morphologic abnormalities
but also adversely in uences the structure and subsequent function of the remaining
proximal and distal bowel.58,59,91 The blind- ended proximal bowel is dilated and
hypertrophied with histologically normal villi, but without effective peristaltic activity. A
de ciency of mucosal enzymes and muscular adenosine triphosphatase has also been
found.92 At the level of the atresia, the ganglia of the enteric nervous system are atrophic
with minimal acetylcho- linesterase activity. These changes are most likely sec- ondary to
local ischemia. Obstruction alone can elicit similar, but less severe, morphologic and
functional abnormalities.92

Experimental studies showing that the intestinal atresia results from ischemic necrosis of
the intestine also imply that there is a precarious blood supply to the proxi- mally dilated
bowel. This has been con rmed with post- mortem injection of barium sulfate into the
mesenteric vessels.58,59,85 However, it has also been postulated that the

422 SECTION IV Abdomen

intestine is not ischemic at birth, but rather becomes so only with swallowing air.
Distention and increased intra- luminal pressure or torsion can then occur. The good
results obtained with tapering procedures without resec- tion of the bulbous portion
would support the contention that the blood and nerve supply to the bowel adjacent to the
atresia is normal.58 However, this ischemic insult may interfere with mucosal and neural
function. Defective peristalsis is commonly noticed in the atretic area, thus supporting
resection of the dilated bulbous proximal end for better function.93 Because the proximal
end of the distal atretic bowel has been subjected to a similar insult, a small portion of it
should be resected at the time of operative correction as well.

Clinical Manifestations
Prompt recognition of intestinal obstruction in the neonate is paramount due to the
possibility of midgut volvulus or an internal hernia with subsequent ischemia. Although
prenatal ultrasound is more reliable at detect- ing duodenal atresia, in recent years it has
become useful in diagnosing jejunoileal atresia as well. The ultrasound ndings include
dilated loops of bowel and polyhydram- nios, which may not be present early in gestation
or only with very distal obstructions. A fetus with these abnormal ndings should elicit a
search for familial GI abnormali- ties as well as referral for prenatal evaluation. The vast
majority of patients with jejunoileal atresia will not be diagnosed prenatally.

In neonates with atresia or stenosis, the presenting symptoms are consistent with bowel
obstruction, includ- ing bilious emesis and abdominal distention. Although

FIGURE30-14 ■ (A) The abdominal radiograph in this neonate shows several proximally dilated intestinal
loops consistent with jejunal atresia. (B) A type III(a) distal atresia was found at operation.

the meconium may appear normal, it is more common to see gray plugs of mucus passed
via the rectum. Occasion- ally, if the distal bowel in type III(b) atresia is ischemic, blood
may be passed through the rectum.

Intestinal stenosis is more likely to create diagnostic dif culty when compared to
intestinal atresia. Intermit- tent partial obstruction or malabsorption may improve without
treatment. Clinical investigations can initially be normal. However, these babies usually
develop failure to thrive and ultimately progress to complete intestinal obstruction and
require exploration.

Diagnosis
The diagnosis of jejunoileal atresia can usually be made by radiographic examination of
the abdomen using swal- lowed air as contrast. Swallowed air reaches the proximal bowel
by one hour and the distal small bowel by three hours in a normal vigorous infant in
whom its passage is blocked, but this pattern may be delayed in premature or sick infants
with poor sucking.94,95 Jejunal atresia patients can have a few gas- lled and uid- lled loops
of small bowel, but the remainder of the abdomen is gasless (Fig. 30-14). When the
atresia is associated with cystic brosis, fewer air- uid levels are evident, and the typical
ground- glass appearance of inspissated meconium is seen. A limited-contrast study may
be useful if intestinal stenosis is suspected.

As haustral markings are rarely seen in neonates, distal ileal atresia may be dif cult to
differentiate from colonic atresia (Fig. 30-15). A contrast enema will reveal an unused
appearance to the colon. Reliance on intraopera- tive injection of saline into the large
bowel to con rm
FIGURE30-15 ■ The diagnosis of colonic atresia can be dif cult on the plain abdominal radiograph. This
radiograph shows mul- tiple dilated intestinal loops and appears similar to the ndings shown in Figure 30-14.
At operation, the infant was found to have atresia of the transverse colon (see Fig. 30-16).

distal bowel patency may fail to identify an associated colonic or rectal atresia.96,97 If the
small bowel atresia occurred late in gestation, the bowel distal to the atresia may have a
more normal caliber. Occasionally, air and meconium can accumulate proximal to an
atresia, mim- icking the radiologic appearance of meconium ileus. Additionally, total
colonic aganglionosis may be dif cult to differentiate from small bowel atresia.

Ten per cent of babies with jejunoileal atresia present with meconium peritonitis.4 The
intestinal perforation usually occurs proximal to the obstruction, near the bulbous blind
end. The radiologic appearance of a meco- nium pseudocyst containing a large air/ uid
level is related to the late intrauterine bowel perforation. Intra- luminal calci cation of
meconium or intramural calci ca- tion in the form of diffuse punctate or rounded
aggregations have been reported with intestinal stenosis or atresia.98 Meconium calci
cation in patients with hereditary FMIA produces a ‘string of pearls,’ which is
pathognomonic of this condition.72,88

The clinical and radiologic picture of jejunoileal ste- nosis is determined by the level and
degree of stenosis, and the diagnosis may be delayed for years. Morphologic and
functional changes in the proximal obstructed intes- tine vary depending on the degree of
obstruction.

Differential Diagnosis
Diseases that mimic jejunoileal atresia include colonic atresia, midgut volvulus,
meconium ileus, duplication cysts, internal hernias, ileus due to sepsis, birth trauma,

30 Duodenal and Intestinal Atresia and Stenosis 423

maternal medications, prematurity, and hypothy- roidism.4,99,100 Special investigations


including an upper GI contrast study, contrast enema, rectal biopsy, and a delta F508
gene deletion assay or sweat test to exclude associated cystic brosis may be needed.99,101

Management
Delay in diagnosis may lead to impairment of intestinal viability (50%), frank necrosis
and perforation (10–20%), uid and electrolyte abnormalities, and sepsis. Preopera- tive
management should include gastric decompression and uid resuscitation to correct
electrolyte abnormali- ties and hypovolemia. Antibiotics should be initiated if there is
suspicion for perforation or infection.

Surgical Considerations
The operative management of intestinal atresias is based on the location of the lesion,
anatomic ndings, associated conditions noted at operation, and the length of the
remaining instestine.56 Resection of the dilated and hypertrophied proximal bowel (see
Fig. 30-12B), with primary end-to-end anastomosis with or without tapering of the
proximal bowel, is the most common technique.4, 77,100

As recently as the 1950s, the surgical mortality for newborns with intestinal atresia was
80% to 90%.56,99 This high mortality rate was mostly related to late pres- entation and
dysmotility of the proximal dilated bowel which led to complications related to chronic
obstruction and inanition. Fortunately, the current survival rate is greater than 90%.56 The
understanding that the proximal bowel is dysfunctional, improvements in the anastomotic
technique and suture material, and the development of total parenteral nutrition (TPN) are
the primary reasons for this signi cantly improved survival in recent years. Currently,
only infants with severe associated congenital abnormalities or short bowel syndrome
should not have a good prognosis.

Operative Considerations
The repair of small intestinal atresia can be undertaken via several approaches. One
option is to evaluate using a laparoscopic approach, with subsequent resection and
anastomosis performed in an extra-corporeal fashion. Although this approach seems
attractive, it can be dif - cult to identify the atresia due to the markedly dilated small
intestine and the small working space of the neonate’s abdominal cavity. To overcome
these limita- tions, we explore the abdomen through the umbilicus. With this technique,
the umbilical skin is incised and the fascia is opened vertically in the midline to the extent
allowed by the umbilical skin incision. The small intes- tine can be exteriorized relatively
easy through the umbilical incision (see Figs 30-12C and 30-13B). In a retrospective
report, a circumumbilical incision for neo- natal surgery was found to as effective as the
transverse abdominal incision with less morbidity and better cos- metic results.102 The
traditional transverse supra- or infraumbilical incision is also appropriate. Regardless of

424 SECTION IV Abdomen

the approach, access to the entire intestine and peritoneal cavity is necessary. Careful
inspection of the entire bowel is performed and the site and type of obstruction should be
noted as well as any other abnormalities. In addition, the length of bowel should be
assessed. The most distal limb of the atretic bowel can then be cannulated with a red
rubber catheter and irrigated with warm saline to evaluate for distal obstruction.
Continuity of the colon can be established preoperatively by a contrast enema or with a
prepositioned transrectal catheter placed prior to prepping.103 Failure to adequately
evaluate for distal obstruction or stenosis can lead to postoperative compli- cations,
including an anastomotic leak. If present, malro- tation should be corrected with a Ladd
procedure. Because the length of functional bowel has important prognostic signi cance,
and determines the most appro- priate method of repair, the length of functional bowel
should be carefully measured along the antimesenteric border and documented in the
operative report.

Delayed intestinal function in the blind proximal atretic segment as well as functional
obstruction after performance of a side-to-side anastomosis without resec- tion of the
dilated proximal atretic bowel have been described.100 Therefore, if the length of
functional bowel is adequate, the bulbous hypertrophied proximal bowel should be
resected to approximately normal caliber bowel. Ultimately, the goal is to restore bowel
continuity while maintaining both intestinal function and length. Intestinal imbrication
has also been shown to be an effec- tive method to reduce the caliber of the dilated bowel
while maintaining mucosal absorptive surface.104 Regard- ing the distal segment, a short
length (4–5 cm) of bowel is obliquely resected, leaving the mesenteric side longer than
the antimesenteric aspect. An incision along the antimesenteric border to create a ‘ sh
mouth’ may be needed to create an adequate distal enterotomy for the anastomosis.

Although there are multiple techniques for the anas- tomosis, we generally perform a
one-layer modi cation of the end-to-back technique using 5-0 or 6-0 sutures. Once the
anastomosis is completed, the suture line is tested for leaks, and reinforcing sutures are
placed as needed. The mesenteric defect is repaired with careful attention to avoid
rotation or kinking of the anastomosis, or injury to the blood supply. A temporary
enterostomy should be performed if there is a question of bowel viabil- ity.56 However,
neither decompressive gastrostomy nor transanastomotic stents are usually needed.105,106

Similar techniques are used for stenosis and jejunoileal membranes. Procedures such as
transverse enteroplasty, excision of the membrane, and bypassing techniques are not
recommended primarily because they fail to remove the abnormal segments of bowel,
and may produce blind- loop syndromes.

Prognostic Factors
The normal small bowel length in term neonates is approximately 250 cm. In preterm
infants, it ranges from 160–240 cm. With the development of TPN, special enteral diets
and pharmacologic management of short

gut syndrome, previous estimates that a small bowel length of 100 cm or more is
necessary to sustain oral intake and survival may no longer be applicable. Preser- vation
of bowel length at the expense of a poorly func- tioning anastomosis should be avoided.

If proximal resection will lead to signi cant, or unac- ceptable bowel loss, tapering or
plication of the dilated bowel is a useful technique.104,107 Tapering enteroplasty as far
proximal as the second portion of the duodenum can be accomplished by resecting an
antimesenteric strip of the dilated proximal bowel.108 During tapering duodeno-
jejunoplasty, particularly with type III atresias, the duo- denum is de-rotated, thus
allowing a direct caudal descent from the stomach which decreases the risk for obstruc-
tion. Additionally, the mesentery should be maximally opened, while meticulously
protecting the small bowel blood supply. During this process, the cecum can be
mobilized to the left which results in a broader mesentery and also allows the
anastomosis to lie in manner that will help avoid kinking.109 The tapering can be safely
per- formed up to 35 cm.107 The tapered bowel may then be anastomosed to the distal
bowel or exteriorized as a stoma.

A primary anastomosis may be contraindicated in cases of peritonitis, volvulus with


vascular compromise, meco- nium ileus, or type III(b) atresia.110,111 Under these cir-
cumstances, exteriorization of both ends of the atresia may be needed.

Intestinal atresia encountered in a baby with gastro- schisis may be single or multiple,
and may be located in either the small or large bowel. In a series from our institution,
12.6% of 199 patients with gastroschisis had an associated atresia.112 The most common
location for the atresia was jejunoileal and most were type III(a). Our current
management algorithm for patients with gastro- schisis and atresia is to rst assess the
extent of reactive change (peel) on the intestine. If there is minimal peel, primary
anastomosis may be an option. This is rare and should be considered only in the most
optimal situations. In nearly all instances, the atresia should be left undis- turbed at the
initial operation. After fascial closure is accomplished, management should include
gastric decompression and TPN support with subsequent atresia repair four to six weeks
later.

With type III(b) atresia, restricting bands along the free edge of the distal coiled and
narrow mesentery should be divided to optimize the blood supply. The bowel should be
returned to the abdomen with careful inspection of the mesentery to prevent torsing the
single marginal artery and vein. In cases of questionable intes- tinal viability, improved
long-term results have been achieved with resection and tapering of the dilated proxi- mal
bowel with limited resection of the distal bowel.113,114

Bowel-length conservation methods, such as multiple anastomoses for multiple atresias,


may result in increased morbidity. A silicone (Silastic) catheter stent can be used with
multiple primary anastomoses and serves as a conduit for radiologic evidence of
anastomotic integrity, luminal patency, and enteral feeding.115 If multiple atresias are
grouped closely together and there is adequate bowel length, a single resection and
anastomosis can be performed.

No attempt at any bowel lengthening procedures should be entertained at the initial


operation. However, such procedures may ultimately obviate the need for pro- longed
TPN in patients with short gut syndrome.

Postoperative Care
Parenteral nutrition is mandatory and should begin as soon as possible, and should
continue until the infant is tolerating full enteral feeds.

Enteral feedings can be initiated when the gastric aspi- rate is clear, output is minimal,
and the infant is stooling. At our institution, enteral feeding is usually started through a
feeding tube at a rate of 20 mL/kg/day of breast milk or formula in a continuous fashion.
The feeds are increased by 20–30 mL/kg/day. Oral intake is started when the baby is
alert, able to suck, and tolerating at least 8 mL of tube feeds per hour.

Transient GI dysfunction is frequently observed in infants with jejunal and ileal atresia,
and its etiology is multifactorial.4,116 Lactose intolerance, malabsorption (owing to stasis
with bacterial overgrowth), and diarrhea may be signi cant in infants who have undergone
repair of type III(b) atresia, or in those with short bowel syn- drome after surgery for
multiple atresias. Regular moni- toring for clinical signs of intestinal overload or
intolerance is required. Water-loss stools, increasing fre- quency of stooling,
hematochezia, fecal-reducing sub- stances, or a decreased stool pH warrant biochemical
evaluation of the stool for disaccharide or monosaccha- ride intolerance.117 Unintentional
injury to the mucosa can be caused by sugars, high-osmolarity feeds, oral med- ications,
and bacterial or viral infections. Pharmacologic control of altered GI function may hasten
adaptation. Loperamide hydrochloride decreases intestinal peristaltic activity and
cholestyramine is effective in binding bile salts.117,118 Cholestyramine should not be given
unless water-loss stools are evident. Vitamin B12 and folic acid should be given regularly
to the patient without a termi- nal ileum to prevent megaloblastic anemia.

Functional outcome ultimately depends on the follow- ing factors: (1) the location of the
atresia (the ileum adapts to a greater degree than the jejunum); (2) the maturity of the
intestine (the small intestine in a prema- ture infant still has time for maturation and
growth); and (3) the length of the small intestine, which can be dif cult to determine
accurately after birth.119 The ileocecal valve is critically important as it allows for more
rapid intestinal adaptation when the residual small bowel length is short.

COLONIC ATRESIA
Colonic atresia (CA) is a rare cause of intestinal obstruc- tion and comprises 1.8–15% of
all GI atresias.120,121 The reported incidence of CA varies greatly from 1:5000 to 1 :
60,000 live births.122–125 The accepted incidence is approximately 1 in 20,000 live births.
Although it is most commonly reported as an isolated anomaly, approxi- mately one-third
of babies have associated congenital lesions.123,124,126 There are various classi cations of
CA, but the one most commonly used divides CA into three

30 Duodenal and Intestinal Atresia and Stenosis 425

types. Type I consists of mucosal atresia with an intact bowel wall and mesentery. In type
II, the atretic ends are separated by a brous cord. In type III, the atretic ends are separated
by a V-shaped mesenteric gap (Fig. 30-16). Type III lesions are the most commonly
occurring lesions overall, while types I and II are seen more commonly distal to the
splenic exure.122,127

The rate of associated anomalies with CA is much smaller when compared to other
atresias. CAs have been found in approximately 2.5% of neonates with gastro- schisis.112
There are less than 25 published cases of CA and Hirschsprung disease (HD).126 Complex
urologic abnormalities, multiple small intestinal atresias, an un xed mesentery, and
skeletal anomalies have also been reported with CA.122,127–129 Similar to small bowel
atresias, a vascular insult to the colon continues to be the accepted etiology for all types
of CA.130,131

The characteristic clinical features of CA are abdomi- nal distention, bilious emesis, and
failure to pass meco- nium. On plain radiographs, air- uid levels are usually appreciated
as well as dilated intestinal loops of large bowel often associated with a ‘ground-glass’
appearance of meconium mixed with air. Occasionally, the dilation can be so massive
that it mimics pneumoperitoneum (Fig. 30-17). The diagnosis is made with a contrast
enema showing a small diameter distal colon that comes to an abrupt halt at the level of
the obstruction (Fig. 30-18).

The diagnosis of CA is an indication for urgent opera- tive management as the risk for
perforation is higher than is seen in jejunoileal atresias. The operative approach depends
on the clinical status of the patient, the level of
FIGURE 30-16 ■ A type III colonic atresia was found at operation in this infant with intestinal obstruction.
Note the cecum and appendix and the very dilated right colon. Also, note the extremely small distal colon
(arrow). A colostomy was per- formed as the initial procedure in this infant.
426 SECTION IV

Abdomen
A

FIGURE 30-17 ■ (A) Abdominal radiograph of colonic atresia showing huge air- lled proxi- mal colon
mimicking a pneumoperitoneum. (B) Right colonic atresia with rectal stenosis.
FIGURE 30-18 ■ The contrast enema on the right in a patient with a distal intestinal obstruction (left) shows a
small colon and failure of the contrast to move proximally past the mid-transverse colon.

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