Intestinal Obstruction in Neonatal/Pediatric Surgery: by C.A. Hajivassiliou

Download as pdf or txt
Download as pdf or txt
You are on page 1of 13

Intestinal Obstruction in Neonatal/Pediatric Surgery

By C.A. Hajivassiliou
Glasgow, Scotland, UK

Intestinal obstruction in the newborn infant and older child increase in associated morbidity and mortality, and ap-
may be due to a variety of conditions, including atresia and propriate surgical treatment becomes more hazardous.
stenosis, annular pancreas, malrotation, duplication cyst,
meconium ileus, meconium plug syndrome and neonatal
Early diagnosis depends largely on the prompt detection
small left colon syndrome, Hirschsprung’s disease, neopla- of obstructive manifestations by the clinician and the
sia, trauma, and other rarer causes. The mode of presenta- subsequent accurate interpretation of radiographic find-
tion can be acute or more chronic with systemic upset due ings and other investigations, leading to definitive treat-
to shock. Neonates, more so than older children, with un- ment (usually surgical), which should always be pre-
recognized intestinal obstruction deteriorate rapidly, show
an increase of associated morbidity and mortality and ap-
ceded by appropriate resuscitation/preparation of the
propriate surgical treatment becomes more hazardous. infant/child. Conservative management has a much
Early diagnosis depends largely on the prompt detection of lower chance of success in acute cases and cannot be
obstructive manifestations by the clinician and the subse- advocated generally.
quent accurate interpretation of radiographic findings and Detailed history, careful clinical examination, and
other investigations, leading to definitive treatment, which
should always be preceded by appropriate resuscitation/
early instigation of general resuscitation measures (cor-
preparation of the infant/child. Management of intestinal rection of hypovolemia, oxygen administration, intrave-
obstruction will almost always be surgical, apart from some nous antibiotics, gastric decompression) and baseline
notable exceptions and all are discussed in more detail. With radiological1 and laboratory investigations (biochemis-
the advent of pediatric and neonatal intensive care and try, hematology, blood crosshatch) are considered at
multidisciplinary care, the morbidity and mortality of cases
of intestinal obstruction reported in current series is gener-
least as important as detailed “second line” investiga-
ally extremely low and mainly determined by the coexist- tions, which will eventually direct definitive treatment.
ence of other major congenital anomalies (eg, cardiac), de- Management of intestinal obstruction will almost al-
lays in diagnosis and treatment or coexisting medical ways be surgical, apart from some notable exceptions
conditions. Newer treatments and future developments (including among others some cases of postoperative
may reduce the residual mortality in such cases as ultra-
short-bowel syndrome.
adhesion obstruction, meconium ileus, neonatal narcotiz-
© 2003 Elsevier Inc. All rights reserved. ing enterocolitis [NNEC], colonic volvulus, neoplasia—
notably lymphoma, inflammatory bowel disease), dis-
cussed in more detail in subsequent sections of this
I NTESTINAL OBSTRUCTION IN the newborn in-
fant and older child may be due to a variety of
conditions, including atresia and stenosis, annular pan-
article.
GASTROINTESTINAL TRACT OBSTRUCTION
creas, malrotation, duplication cyst, meconium ileus, ANATOMY/PATHOGENESIS/ETIOLOGY/TREATMENT
meconium plug syndrome and neonatal small left colon There are many ways to classify intestinal obstruction,
syndrome, Hirschsprung’s disease, neoplasia, trauma, perhaps the most widely used being the classical way,
and other rarer causes.1 describing the origin of the obstruction: extrinsic, mural,
This section will review and revisit the different com- or intraluminal. Another mnemonic, which attempts to
mon and uncommon causes of intestinal obstruction in classify according to etiology appears in Fig 1.
neonates and children and summarize their treatment and Obstructions at each anatomical region will be de-
other salient features. Other sections will focus appro- scribed in more detail from stomach to the anorectum.
priately on other issues, eg, investigations, etc.
Lesions of the Stomach
PRESENTATION/GENERAL MANAGEMENT OF Pyloric stenosis (Fig 2) is the commonest cause of
INTESTINAL OBSTRUCTION gastric outlet obstruction. The repeated vomiting leads to
The mode of presentation can be acute, with obvious
features of obstruction (vomiting, which will invariably From the Royal Hospital for Sick Children and University Depart-
become bile-stained); pain (usually colicky), with or ment of Surgical Paediatrics, University of Glasgow, Glasgow, Scot-
without features of peritonism and perforation; and se- land, UK.
vere systemic upset due to shock. The presentation can Address reprint requests to C.A. Hajivassiliou, Royal Hospital for
Sick Children and University Department of Surgical Paediatrics,
also be more subtle and chronic in cases of incomplete or
University of Glasgow, Yorkhill, Glasgow G3 8SJ, Scotland, UK.
recurring/resolving bouts of obstruction. © 2003 Elsevier Inc. All rights reserved.
Neonates, more so than older children, with unrecog- 1055-8586/03/1204-0005$30.00/0
nized intestinal obstruction deteriorate rapidly, show an doi:10.1053/j.sempedsurg.2003.08.005

Seminars in Pediatric Surgery, Vol 12, No 4 (November), 2003: pp 241-253 241


242 C.A. HAJIVASSILIOU

Duodenal Atresia4
Duodenal obstruction is most commonly due to com-
plete atresia of the postampullary segment of the duode-
num and presents with bile-stained vomiting and a clas-
sic “double-bubble” appearance of the plain abdominal
x-ray. It commonly coexists with an annular pancreas
due to the associated embryological defect. Treatment is
by duodeno-duodenostomy (Figs 3 and 4), either linear
or diamond-shaped, and—although classically described,
a transanastomotic tube is no longer advocated as it
delays recovery and increases the time to full feeding.5 A
duodenal web (which may confuse the diagnosis with
that of proximal jejunal atresia due to a “windsock ef-
fect”) is much rarer and is treated by duodenotomy,
excision of the web, and duodenoplasty.

Jejunoileal Atresia
Intestinal atresia (Fig 5) is classified into 5 types: Type
Fig 1. Mnemonic for classifying causes of intestinal obstruction
(or indeed most other conditions).
1 denotes an obstructing membrane and Type 2 a cord
anomaly at the site of the atresia. Type 3A involves
complete separation of the 2 ends by a mesenteric defect
and Type 3B is described as the apple-peel deformity.
dehydration and frequently hyponatremic, hypochlore- Type 4 describes multiple intestinal atresias. Treatment
mic, hyperkalemic metabolic alkalosis. Rarely, in the is, whenever possible, by primary anastomosis with a
latest stages of the disease, paradoxical aciduria ensues general principle being the preservation of as much
in an attempt to conserve potassium at the expense of bowel length as possible.
hydrogen excretion by the kidney. These need to be Type 3A or “apple-peel” atresia presents a special
corrected prior to surgery, which involves the classical case, where the bowel is very abnormal, shortened, and
Ramstedt’s pyloromyotomy with virtually no mortality revolves round and entirely supplied by the ileocolic
or recurrence. artery (Fig 6). Radiographic evidence of high small-
Other rarer causes include pyloric web or atresia,
infective2 or syndromic conditions, eg the junctional
epidermolysis bullosa-pyloric atresia syndrome, which is
recognized as a distinct autosomal recessive entity. Af-
fected infants present with skin fragility and inability to
feed due to intestinal obstruction. Despite successful
surgical repair of the anatomical defect, the outcome is
poor owing to poor feeding, malabsorption, failure to
thrive, and sepsis, with death occurring before age 11
months. The poor prognosis of this condition must be
considered when decisions are made regarding surgical
correction and, although attempting surgical correction
may be warranted in individual circumstances, withhold-
ing surgical intervention and providing palliative support
may be an acceptable alternative.3

Intestinal Atresia and Stenosis


Atresia is the most common cause of congenital intes-
tinal obstruction and accounts for approximately one Fig 2. Contrast meal showing pyloric stenosis. Note the “shoul-
third of cases of obstruction in the newborn with inci- dering” of duodenal mucosa towards the pylorus (arrow), which puts
dence of approximately 1:2700 births. The mortality of it in peril during surgical incision of the pyloric muscle. Test feed and,
in difficult cases, ultrasound scan of the pylorus should have a virtu-
the condition was 90% prior to 1950, but this declined ally 100% diagnostic accuracy making contrast examination unnec-
sharply and remains at approximately 10% (Table 1). essary.
INTESTINAL OBSTRUCTION IN NEONATAL/PEDIATRIC SURGERY 243

Table 1. Review of Cause, Intervention, and Outcome in 227 Neonates with Intestinal Atresia73
Long-term Survival
Level of Obstruction Associated (Morbidity and Mortality
(227 neonates) Anomalies/Features Surgical Management Operative Mortality Due to)

Duodenal (n ⫽ 138) Prematurity, 46% Duodeno-duodenostomy, 86% 4% 86% (cardiac anomalies)


Polyhydramnios, 33% Duodenotomy and web
excision, 7%
Down syndrome, 24% Duodeno-jejunostomy, 5%
Annular pancreas, 33%
Malrotation, 28%
Jejunoileal (n ⫽ 128) Intrauterine volvulus, 27% Resection with (70%) or 0.8% 84% (ultrashort-bowel
without (30%) syndrome)
Gastroschisis, 16% primary anastomosis 76%
Meconium ileus, 12% Temporary stoma without
resection, 20%
Web excision, 4%
Bianchi procedure, 1 case
Colonic (n ⫽ 21) Temporary stoma and delayed 0% 100%
anastomosis, 86%
Resection and primary
anastomosis, 14%
Multiple (n ⫽ 10)

bowel obstruction and a malrotated microcolon on pre- Fifty-seven cases of apple-peel jejunal atresia have
operative roentgenogram with contrast enema should been reported in the English literature. Patients with this
suggest this diagnosis. It has been suggested that this anomaly have a high incidence of prematurity (70%),
disorder is transmitted by either an autosomal recessive malrotation (54%), short-gut syndrome (74%), multiple
gene in some cases or that there may be a more complex atresias (15%), complications (63%), and mortality
spectrum of genetic transmission in others.6 (54%). Mortality has decreased from 63% to 47% since
1970.6

Fig 3. Duodeno-duodenostomy for duodenal atresia (arrowhead: Fig 4. Duodeno-duodenostomy suture line completed. No transa-
proximal duodenotomy, arrow: distal duodenotomy). nastomotic tube is used.5
244 C.A. HAJIVASSILIOU

Fig 5. Ileal atresia. Note the grossly distended blind ending prox-
imal loop (arrowhead) and the collapsed distal end containing meco-
nium (arrow).

Colonic Atresia
Colonic atresia is rare. Treatment is generally by seg-
mental resection with or without primary anastomosis, Fig 7. Prolapsed obstructing vitellointestinal tract remnant
through umbilical defect.
but because of the association of colonic atresia with
Hirschsprung’s disease, a careful examination of the
resected specimen to rule out Hirschsprung’s disease is doses of pancreatic supplements. The treatment princi-
recommended. Performing a rectal biopsy must be con- ples are the same as those for colonic atresia.
sidered for patients who initially were treated for colonic
atresia and who have a slow return of normal gut func- Omphalomesenteric Tract Remnants
tion. All colonic atresias in a reported series were diag- Omphalomesenteric duct malformations comprise a
nosed neonatally; however, there was mean delay of 15.4 wide spectrum of anatomic structures and associated
months (range, 1 month to 5 years) in diagnosing asso- symptoms (or no symptoms). They may range from a
ciated Hirschsprung’s disease.7 completely patent omphalomesenteric duct at the umbi-
Colonic Stenosis licus to a variety of lesser remnants, including the diver-
ticulum of Meckel. Symptoms may involve intestinal
Colonic stenosis may be congenital8 or acquired.9 Ac- fistulas at the umbilicus, intussusception/prolapse of il-
quired colonic stricture in older patients has been re- eum at the umbilicus (Fig 7), intestinal obstruction from
ported in association with cystic fibrosis (cystic fibrosis a variety of causes, melena and anemia, abdominal pain
colonopathy10) and may be caused by excessively high and inflammation. Symptoms occur most frequently dur-
ing childhood years (especially in the first 2 years of
life).11

Duplications
Intestinal duplication may occur at any level of the
gastrointestinal tract from mouth, esophagus, stomach,12
ileum,13 colon, cecum14 to rectum.15 They may contain
heterotopic tissue (eg, pancreas13) and may rarely be
associated with intraspinal communications (residual
neuroenteric canal-split notochord syndrome) causing
high morbidity and mortality.
Duplications may be fusiform, tubular, or cystic and
usually present with obstruction or bleeding. Treatment
is by complete excision and primary anastomosis where
possible. Where the cyst cannot be safely excised (usu-
ally because of the sharing of a common wall with the
Fig 6. “Apple peel” atresias and intestinal ischemia. The patient normal bowel segment, or because complete excision
did not survive. may endanger the bile ducts) every attempt should be
INTESTINAL OBSTRUCTION IN NEONATAL/PEDIATRIC SURGERY 245

(Figs 9 and 10). A high index of suspicion in the neonate


with vomiting, rapid diagnosis, and appropriate operative
therapy results in a predictable favorable outcome for
children with malrotation with or without volvulus.25
Volvulus Without Malrotation
A literature review indicates a significant relation of
intrauterine midgut volvulus without malrotation to pre-
term birth with low-birthweight infants, usually present-
ing in the immediate postnatal period. The speed of
presentation and intervention can ensure a favorable out-
come.26
Volvulus may also occur round an intraabdominal
fibrotic or omental band (Fig 11) or present as an isolated
entity with axial rotation around a Meckel’s diverticu-
lum.27
Colonic Volvulus
Colonic volvulus can also occur due to the idiosyn-
crasies of the peritoneal fixations/or nonfixation of seg-
ments of the colon and may involve the cecum,28 trans-
Fig 8. Upper gastrointestinal meal and follow-through of a 4-day-
old infant with bile-stained vomiting showing malrotation with vol- verse colon,29,30 splenic flexure,31 or sigmoid.32,33 It is
vulus. Note the duodenojejunal (DJ) flexure (arrow) does not cross usually preceded by recurrent low-grade symptoms and
the midline and lies at a level below the pylorus. Contrast then flows commonly associated with intestinal dysmotility and
into the proximal jejunum in a “corkscrew” manner.
constipation.31 Presentation is usually acute, with asso-
ciated systemic symptoms and shock.
made to completely excise its mucosal lining as dupli-
Cecal Volvulus
cation cysts are associated with late malignant transfor-
mation.16-21 Resection and primary end-to-end ileocolic anastomo-
sis is advocated in the presence of gangrene or perfora-
Malrotation
Malrotation of the intestinal tract is the result of a
well-defined aberrant sequence of embryological
events. Because the consequences of malrotation as-
sociated with a midgut volvulus may be so cata-
strophic, an understanding of the anatomy, diagnostic
criteria, and appropriate therapy for this putative
emergency illness is imperative. More than half of the
patients present during the first month of life, and
virtually all have bile-stained vomiting. However,
symptoms may first become manifest in teenagers and
adults, often with disastrous outcomes.22 Most inves-
tigators, therefore, recommend surgical correction of
all cases of malrotation.23,24
Upper gastrointestinal contrast series (Fig 8) is the
preferred diagnostic study, being both sensitive (18/19,
95%) and accurate (18/21, 86%).25 A Ladd procedure is
the preferred treatment, which typically includes eviscer-
ation and inspection of the mesenteric root; derotation of
a midgut volvulus (which has always been reported to
occur in a clockwise direction); lysis of Ladd’s bands
with Kocherization of the duodenum along the right Fig 9. Operative findings in patient from Fig 8, showing the
complete volvulus around the foregut mesentery carrying the mobile
abdominal gutter, with or without appendectomy; and caecum and appendix (arrow) to the left upper quadrant (small
placement of the cecum into the left side of the abdomen bowel at the top of the picture and large bowel at the bottom).
246 C.A. HAJIVASSILIOU

Splenic Flexure Volvulus


Resection and primary end-to-end anastomosis is also
the treatment of choice in this condition.31

Sigmoid Volvulus
Sigmoid volvulus, although not uncommon in the
adult population,33 is an exceptionally rare and poten-
tially life-threatening condition in the pediatric age
group. In a review of 63 cases of sigmoid volvulus in
children since 1940 (median age was 7 years), two dis-
tinct presentations (acute and recurrent) were identified.
Abdominal symptoms dominated the clinical picture.
Barium enemas either confirmed or were highly sugges-
tive of sigmoid volvulus,32,33 however the classic roent-
genographic omega sign of volvulus was present on plain
films in only 29% of the cases.33 Reduction by barium
enema was successful in 77% (10 of 13) of the attempts.
Forty-nine patients underwent operative treatment, with
sigmoidectomy (with or without primary anastomosis)
being the most common. The overall mortality rate was
6%, operative mortality was 8.1%, and neonatal mortal-
Fig 10. Ladd’s procedure completed. After full kocherization of ity was 14%. It was associated with Hirschsprung’s
the duodenum, the jejunum and small intestine are placed in the disease in 17% of cases (11 of 63 patients).32
right-hand side of the abdomen and the large intestine on the lower Sigmoid resection is the definitive treatment for chil-
left. The appendix is generally left behind, with clear notes and
instructions that it does not lie in the right iliac fossa. dren as well as adults, but nonoperative decompression
to allow for elective resection could be attempted in
patients who have no evidence of peritonitis.33

tion of the cecum. In noncomplicated cases of cecal Meconium Disease of Infancy


volvulus, detorsion, and cecopexy is also an option be- Meconium ileus. Intestinal and pancreatic dysfunc-
cause of low mortality, morbidity, and recurrence rates.28 tion, which in most cases results from the autosomal
recessive disease cystic fibrosis, can result in intestinal
Transverse Colon Volvulus
obstruction due to meconium ileus (Fig 12), which is the
Resection and primary end-to-end anastomosis is the result of the accumulation of sticky and inspissated in-
treatment of choice in this condition,29,30,34 as operative traluminal meconium. Both nonoperative and operative
or colonoscopic management by detorsion34 can be as- therapies can be effective in relieving this small-bowel
sociated with recurrence. Transverse colon appears to be obstruction. In the past, although less so today, a suc-
the only part of the bowel reported to volve in a coun- cessful nonoperative treatment was associated with a
terclockwise direction. more favorable outcome. Once the meconium ileus is

Fig 11. Inflamed Meckel’s diverticulum (white arrow)


with omental band adhesion (black arrow) acting as a pivot,
round which small intestine volved causing closed loop
obstruction. Treatment was by segmental resection and
primary anastomosis.
INTESTINAL OBSTRUCTION IN NEONATAL/PEDIATRIC SURGERY 247

relieved by rectal stimulation (eg, glycerin suppository,


rectal washouts, or contrast enema).

Milk Curd Syndrome


Intestinal obstruction caused by the inspissation of
formula feeds should be considered in any infant pre-
senting with distal small-bowel obstruction. There are
typical radiographic and ultrasonographic changes that
suggest the diagnosis. Relief of the obstruction is often
possible without surgery.40

Intussusception
Intussusception is the invagination of one portion of
the intestine into another. It is the most common form of
intestinal obstruction in infants and accounts for about
Fig 12. Plain abdominal radiograph of a neonate with intestinal 700 hospital admissions each year in England and
obstruction due to meconium ileus. Note the grossly distended in- Wales.41 It rarely occurs out with the first 5 years of life
testinal loops and bowel wall edema.
and is classically associated with intense intermittent
abdominal pain, vomiting, bloody mucoid diarrhea, and
a palpable abdominal mass. Etiologies of childhood in-
relieved successfully, and the diagnosis of cystic fibrosis tussusception differ depending on age at presentation: in
is established, the treatment for the intestinal manifesta- younger infants (⬍18 months of age) the cause appears
tions of the disease focuses on nutritional supplementa- to be an inflamed enlarged intestinal lymph tissue patch
tion, and pancreatic enzyme replacement. Simulta- acting as the lead point, whereas in older children the
neously, the treatment of the life-threatening pulmonary lead point may be, amongst others,42 a hemangioma or
disease focuses on mucous retention and chronic infec- other mural neoplasm or a Meckel’s diverticulum. Plain
tion in the lungs.35 abdominal x-ray may or may not be diagnostic. Im-
Gastrointestinal manifestations of cystic fibrosis in- proved results of treatment have followed recent techno-
clude, in addition to neonatal meconium ileus, distal logical developments, which include ultrasonographic
intestinal obstruction syndrome, constipation and ac- imaging and pneumatic reduction techniques. Contrast
quired megacolon, rectal prolapse, and rarely pancreati- or, preferably, air enema can be both diagnostic and
tis. If the intestinal malabsorption is well controlled with therapeutic. Most intussusceptions can be reduced suc-
an effective pancreatic enzyme preparation, distal intes- cessfully without the need for operation but close coop-
tinal obstruction syndrome, constipation, and rectal pro- eration between surgeon and radiologist is essential. In
lapse are infrequent. Persisting gastrointestinal symp- cases of prolonged symptoms, evidence of small-intesti-
toms should be investigated thoroughly to exclude other
disorders not directly related to the cystic fibrosis.36,37
Survival at 1 year was 92% in patients with uncom-
plicated meconium ileus and 89% for those with com-
plicated meconium ileus. The therapy of choice for un-
complicated meconium ileus is nonoperative hyperosmolar
(Gastrograffin or Omnipaque) enema (Fig 13), with en-
terotomy and irrigation reserved for enema failures.
Complicated cases require exploration and, in the ab-
sence of giant cystic meconium peritonitis, are usually
amenable to bowel resection and primary anastomosis.38
Injection of a solution of n-acetyl cysteine in the inspis-
sated meconium helps to loosen it and facilitate its re-
moval from the bowel lumen.
Meconium plug syndrome. Meconium plug syn-
drome or meconium obstruction in very low birth weight
Fig 13. PA view of the above patient after contrast enema. Note
(VLBW) infants does not appear to be associated with microcolon (arrowhead), reflux of contrast into distended bowel
cystic fibrosis or Hirschsprung’s disease.39 It is usually loops and outlines of meconium plugs in lumen (arrow).
248 C.A. HAJIVASSILIOU

The mainstay of surgical treatment is resection with


enterostomy, although resection and primary anastomo-
sis is useful in selected cases. In addition, some neonates
may benefit from peritoneal drainage, second-look pro-
cedures, or proximal diversion.47

Hirschsprung’s Disease
Timely passage of the first stool is a hallmark of the
well-being of the newborn infant. Failure of a full-term
newborn to pass meconium in the first 24 hours may
signal intestinal obstruction. Lower intestinal obstruction
may be associated with disorders such as Hirschsprung’s
disease, anorectal malformations, meconium plug syn-
Fig 14. Operative findings in a case of severe NNEC, showing drome, small left colon syndrome, hypoganglionosis,
bowel gangrene and ischemia. Rubber shod clamp at level of re- neuronal intestinal dysplasia, and megacystis-microco-
sected perforated intestinal segment.
lon-intestinal hypoperistalsis syndrome.
Hirschsprung’s disease is a relatively common cause
of intestinal obstruction in the newborn. It is character-
nal obstruction or peritonitis surgical treatment may be ized by an absence of ganglion cells in the distal bowel
required.43 beginning at the internal sphincter and extending proxi-
It is exceedingly important to emphasize that these mally for varying distances.48 It may be considered to be
children can be extremely ill at presentation, with few a “neurocristopathy”—an abnormality of migration of
typical manifestations of the gravity of their clinical neural crest cells and is associated with many other
condition until they reach a point of decompensation and conditions, notably sensorineural deafness and Down
rapid deterioration. The immediate management, there- syndrome (Fig 15).
fore, should center on the full and effective resuscitation Radiologic studies and rectal biopsy are usually re-
of the child prior to any radiological or operative manip- quired to make the diagnosis.49 The mainstay of treat-
ulation. Mortality and morbidity rates from the condition ment initially involves the performance of bowel wash-
have progressively declined in recent decades but avoid- outs (Fig 15) to ensure bowel remains empty and
able deaths still occur.41 decompressed (not effective in total colonic disease)
until the infant begins to thrive and is able to tolerate
Internal Hernia definitive treatment. The performance of colostomy and
subsequent Duchamel procedure has now been replaced
An acute intestinal obstruction with strangulation in
by 1-stage endorectal pull-through techniques and, more
the absence of an external hernia and with no history of
recently, laparoscopically assisted pull-through proce-
previous surgical procedures must suggest the possibility
dures. It is important to remove the affected bowel com-
of an internal hernia,44,45 especially if the patient has a
pletely and this is facilitated by intraoperative availabil-
history of chronic intermittent abdominal distress and a
palpable abdominal mass is found on examination.44

Neonatal Necrotizing Enterocolitis


NNEC is one of the most common gastrointestinal
emergencies observed in neonatal intensive care units.
Despite extensive research efforts, the etiology and
pathogenesis of necrotizing enterocolitis remain unclear.
Unfortunately, the most sensitive and specific tests detect
only advanced disease and perforation (Fig 14).
Intestinal stenosis or stricture occurs in approximately
one third of medically treated infants surviving the acute
phase of NNEC. Identification of these lesions by the use
of routine contrast enemas has been advocated as a
means of decreasing potential morbidity from delayed
diagnosis, especially those infants not residing near pe- Fig 15. Bowel washout in a patient with Hirschsprung’s disease
diatric surgical facilities.46 and Down syndrome.
INTESTINAL OBSTRUCTION IN NEONATAL/PEDIATRIC SURGERY 249

Fig 16. The characteristic appearance of a transitional zone in


Hirschsprung’s disease.

ity of frozen-section biopsy reporting to ensure that the


transition zone is totally removed (Fig 16).

Anorectal Atresia Spectrum


Anorectal atresia (Figs 17 and 18) may present with
Fig 18. Anoscrotal fistula in a male infant.
intestinal obstruction, but most often presents with fail-
ure to pass meconium and an easily recognizable ana-
tomical aberration of the perineum. Rectal atresia and
stenosis are rare and peculiar anorectal malformations Inflammatory Bowel Disease
for which many and varied surgical procedures have Crohn’s disease is a chronic, transmural inflammatory
been described.50 The mainstay of treatment involves disease of the intestinal tract most frequently involving
large-bowel decompression by a defunctioning dismem- the terminal ileum and colon. It is a disorder of undeter-
bered sigmoid colostomy (allowing sufficient length of mined etiology that shares many clinical aspects with
distal colon) to allow full anatomical investigation/eval- chronic ulcerative colitis. Intestinal obstruction can be
uation followed by an anorectal pull-through procedure. caused by stricture formation or involvement of bowel
loops in an inflammatory mass. Surgical treatment of
Trauma
Crohn’s disease continues to be generally limited to the
The most commonly reported intestinal injury from treatment of the complications of the disease. The sur-
seat belts or other blunt abdominal trauma in children is gical principle generally adhered to, is to remove only
perforation. A rarely reported late sequel following this enough intestine to relieve the complication but to main-
type of injury is posttraumatic intestinal stricture.51 tain as much intestinal length as possible. Because of this
requirement, strictureplasty has become a worthwhile
surgical adjunct, especially when dealing with multiple
minimally inflamed fibrotic strictures in the small intes-
tine.52

Tropical Infections: Ascariasis


Ascaris lumbricoides, the most frequent human intes-
tinal nematode, is the causative agent of ascariasis, with
an estimated worldwide prevalence of over 1 billion
cases. Although characterized with low morbidity and
mortality rates, the global prevalence of ascariasis still
results in approximately 20,000 deaths annually, primar-
ily as a consequence of intestinal obstruction. In humans,
transmission usually occurs by hand-to-mouth route by
way of contaminated agricultural products and food, or
Fig 17. Anoperineal fistula in a female infant. from dirty hands.
250 C.A. HAJIVASSILIOU

Other Rare Causes of Intestinal Obstruction


Ingested foreign bodies. Typical patterns of symp-
toms of intermittent obstruction resembling the adult
gallstone ileus syndrome can be the presenting features
of foreign body ingestion (peanut, phytobezoar,56 or
pica). Therapeutic approach includes conservative mea-
sures, endoscopic or operative removal of foreign body.
Pica is a serious health risk for mentally handicapped
patients: 75% of cases required surgical intervention,
with a 30% complication rate and 11% death rate.57
Mesenteric lymphatic cyst. Cystic mesenteric lym-
phatic malformations are uncommon abdominal masses,
which can cause abdominal signs and symptoms for a
wide variety of reasons (Figs 19 and 20). Also known as
mesenteric, omental, or retroperitoneal cysts, they can
present in a variety of ways, including intestinal obstruc-
tion, volvulus,58 nonspecific abdominal pain, intracystic
hemorrhage, or as an asymptomatic abdominal mass.
Abdominal ultrasound scan provides a definitive diagno-
sis in most suspected cases. Complete resection is pos-
sible in most patients, except those with extensive retro-
Fig 19. Contrast follow-through in an infant with abdominal peritoneal involvement. Recurrence is unusual when
distention and feed intolerance showing collapsed and elongated complete resection is accomplished.59
bowel loop in right flank, with impression of mesenteric filling de- Solitary intestinal fibromatosis. Solitary intestinal fi-
fect.
bromatosis is a very rare condition, which may present
with intestinal obstruction. It appears to be a condition of
infancy and carries a very good prognosis after segmen-
Plain abdominal x-ray may suggest the diagnosis, but tal resection.60
contrast radiography and ultrasonography, and some- Eosinophilic enteritis. Eosinophilic enteritis or gas-
times endoscopic retrograde cholangiopancreatography, troenteritis is a rare disease characterized by tissue eo-
may be required. Ultrasonography can detect worms in sinophilia, which can affect different layers of the bowel
the biliary tract, pancreas, and intestine and is a useful wall. It can affect any area of gastrointestinal tract from
noninvasive technique for diagnosis and follow-up of the esophagus to the rectum, although the stomach and
such patients. small intestine are the sites most frequently affected.61 It
Although generally asymptomatic, heavy infestation may present as childhood gastrointestinal obstructive
(more than 60 organisms) may cause partial or complete
obstruction of biliary or intestinal tracts. The organism
load is about 10-fold higher in fatal cases.53 Infestation
can be complicated by intussusception, perforation, and
gangrene of the bowel; acute appendicitis; and appendic-
ular perforation.54 Anthelminthic chemotherapy is re-
quired to eradicate the parasites and prevent potentially
serious complications. Mebendazole, albendazole, pyr-
antel, and levamisole are the most widely used agents to
treat ascariasis and can be delivered to communities in
endemic regions, serving as an affordable and cost-ef-
fective strategy to reduce the prevalence and morbidity
in these regions.54,55
Surgical treatment involves enterotomy and removal
of the parasites or treatment of other complications, eg,
intestinal gangrene. Endoscopic retrograde cholangio- Fig 20. Operative picture of above, demonstrating a mesenteric
lymphangioma containing chyle. Definitive treatment was by com-
pancreatography can be used to extract worms from the plete excision of the cyst with segmental bowel resection and pri-
biliary and pancreatic ducts when indicated. mary anastomosis.
INTESTINAL OBSTRUCTION IN NEONATAL/PEDIATRIC SURGERY 251

disease with involvement of the colon but sparing of the obstruction and evidence of gross intestinal dilatation on
stomach.62 Symptoms can be controlled with steroid abdominal x-ray, but the gastrointestinal tract is fully
therapy. patent. It is a very difficult clinical condition to treat and
Benign intestinal tumors. Intestinal tumors are rare both medical (eg, prokinetic agents) and surgical meth-
in children. However, an intestinal tumor can cause ob- ods (laparotomy, adhesion lysis, enteropexy, temporary
struction and would usually be treatable by segmental placement of long intestinal tube) are generally ineffec-
resection and primary anastomosis. An inflammatory tive. The diagnosis is usually made after a negative
myofibroblastic tumor of the jejunum is such an entity. laparotomy and is an important one to make in order to
Histological characterization is essential, as potential re- avoid further unnecessary explorations.
currence would necessitate long-term follow-up.63 AIDS. The pathologic changes in the gastrointestinal
Malignant intestinal tumors. Malignant intestinal tu- tract of children with AIDS are variable, clinically sig-
mors are also, thankfully, very rare in children. The most nificant, and reflect multisystemic disease processes, in-
common tumor is intestinal lymphoma, which is very volving any combination of inflammation, changes in the
responsive to chemotherapy. Primary intestinal leiomy- lymphoid tissue, miscellaneous lesions, AIDS associated
osarcomas are other rare tumors in children. Visceral arteriopathy, and tumors. Cytomegalovirus infection of
metastases from these neoplasms are atypical and, with the gastrointestinal tract can be associated with ulcer-
complete excision, recorded long-term prognosis may be ation, bleeding, perforations, and intestinal obstruction
favorable, unlike their counterpart in the adult popula- and carries a high morbidity and mortality. The remain-
tion.64 ing infections are generally not life-threatening. Both
Adenocarcinoma of the colon has also been reported lymphomas and smooth muscle tumor in children with
in a 10-year-old boy presenting with history of lower AIDS can cause intestinal obstruction and are related to
gastrointestinal bleeding for 1 year and acute intestinal Epstein-Barr virus infection. The smooth muscle tumors
obstruction.65 Although short-term follow up was favor- are frequently malignant and multiple.68
able after radical colectomy, these tumors are usually of Investigations. Imaging plays a major role in most
poor prognosis. neonatal/pediatric gastrointestinal emergencies. The role
The abdominal cocoon. This is a rare cause of intes- may vary from helping to establish a diagnosis, to the
tinal obstruction most often found in adolescent girls evaluation of associated abnormalities, to surgical plan-
from tropical and subtropical countries. It is character- ning, or to therapy for some conditions like meconium
ized by a thick fibrotic sac covering the small bowel ileus or meconium plug syndrome. Plain film of the
partially or completely, the etiology of which is un- abdomen is often helpful in determining the level of
known. A correct diagnosis is not often made preopera- obstruction1 and usually dictates, together with clinical
tively; however, following simple surgical release of the symptoms, the choice of further investigations (with ultra-
entrapped bowel, these patients usually do well.66 sound, computed tomography scan, and magnetic reso-
Chronic intestinal pseudo-obstruction.67 This condi- nance imaging playing roles in more complex cases69,70).
tion may present with symptoms and signs of intestinal Other investigative modalities may be required to evaluate

Fig 21. Plain x-ray and intravenous contrast-enhanced computed tomograph showing bowel ischemia, in a case of late presentation of
incarcerated diaphragmatic hernia.
252 C.A. HAJIVASSILIOU

complex cases with concomitant congenital and chromo- Future therapies for patients with gastrointestinal dis-
somal anomalies.71 ease secondary to cystic fibrosis include lung transplan-
In cases of suspected small-bowel obstruction, com- tation, pharmacologic manipulation of the epithelial cell
puted tomography may confirm the diagnosis and dem- abnormality, and gene transfer therapy.35
onstrate the cause of obstruction, preventing a delay in When, by the nature of the intestinal disorder (eg,
surgical treatment. In addition, it may have a role in multiple jejunoileal atresias, NNEC), the patient suffers
differentiating simple from strangulated small-bowel ob- from ultrashort-bowel syndrome (⬍40 cm) requiring
struction72 (Fig 21). long-term total parenteral nutrition, which can be com-
Detailed review of the investigation of the vomiting plicated by liver disease, the morbidity and mortality can
child will be presented in another article. be prohibitive. Use of growth factors to enhance adap-
tation, probiotics and selective substrates (glutamine,
CONCLUSIONS/THE FUTURE raftulose), and advances in small-bowel transplantation
With the advent of pediatric and neonatal intensive may improve long-term outcomes.73
care and multidisciplinary care the morbidity and mor- Finally, the exciting developments in laparoscopic sur-
tality of cases of intestinal obstruction reported in current gical technique and refinements in the instrumentation
series is generally extremely low and mainly determined involved (the first report of a successful diamond-shaped
by the coexistence of other major congenital anomalies duodeno-duodenostomy for duodenal atresia has been
(eg, cardiac), delays in diagnosis and treatment or coex- reported by Bax74) are further complementing the treat-
isting medical conditions73 (Table 1). ment modalities for intestinal obstruction.

REFERENCES
1. De Backer AI, De Schepper AM, Deprettere A, Van Reempts P, 15. Rajah S, Ramanujam TM, Anas SR, et al: Duplication of the
Vaneerdeweg W: Radiographic manifestations of intestinal obstruction rectum: report of four cases and review of the literature. Pediatr Surg
in the newborn. JBR-BTR 82:159-166, 1999 Int 13:373-376, 1998
2. Moon A, Spivak W, Brandt LJ: Cryptosporidium-induced gastric 16. Michael D, Cohen CR, Northover JM: Adenocarcinoma within
obstruction in a child with congenital HIV infection: case report and a rectal duplication cyst: case report and literature review. Ann R Coll
review of the literature. J Pediatr Gastroenterol Nutr 28:108-111, 1999 Surg Engl 81:205-206, 1999
3. Dank JP, Kim S, Parisi MA, et al: Outcome after surgical repair 17. Lee MY, Jensen E, Kwak S, Larson RA: Metastatic adenocar-
of junctional epidermolysis bullosa-pyloric atresia syndrome: a report cinoma arising in a congenital foregut cyst of the esophagus: a case
of 3 cases and review of the literature. Arch Dermatol 135:1243-1247, report with review of the literature. Am J Clin Oncol 21:64-66, 1998
1999 18. Otter MI, Marks CG, Cook MG: An unusual presentation of
4. Nixon HH: Duodenal atresia. Br J Hosp Med 41:134, 138, 140, intestinal duplication with a literature review. Dig Dis Sci 41:627-629,
1989 1996
5. Upadhyay V, Sakalkale R, Parashar K, et al: Duodenal atresia: a 19. Luciani G, Mingoli A, Modini C, Marzano M, Civitelli S,
comparison of three modes of treatment. Eur J Pediatr Surg 6:75-77, Iascone C: [Duplication of the gastrointestinal tract: a report of a rare
1996 case of mediastinal cyst]. G Chir 16:55-57, 1995
6. Seashore JH, Collins FS, Markowitz RI, Seashore MR: Familial 20. Johnson JA III, Poole GV: Ileal duplications in adults. Presen-
apple peel jejunal atresia: surgical, genetic, and radiographic aspects. tation and treatment. Arch Surg 129:659-661, 1994
Pediatrics 80:540-544, 1987 21. Schickedanz H, Clausner A [Occurrence of cancers in duplica-
7. Kim PC, Superina RA, Ein S: Colonic atresia combined with tions of the digestive tract]. Z Kinderchir 45:304 –307, 1990
Hirschsprung’s disease: a diagnostic and therapeutic challenge. J Pe- 22. Pelucio M, Haywood Y: Midgut volvulus: an unusual case of
diatr Surg 30:1216-1217, 1995 adolescent abdominal pain. Am J Emerg Med 12:167-171, 1994
8. Abu-Judeh HH, Methratta S, Ybasco A, Garrow E, Ali S: Con- 23. Powell DM, Othersen HB, Smith CD: Malrotation of the intes-
genital colonic stenosis. South Med J 94:344-346, 2001 tines in children: the effect of age on presentation and therapy. J Pediatr
9. Akamine M, Araki Y, Chijiiwa Y, Shimizu S, Shimura H, Nawata Surg 24:777-780, 1989
H: A case of Meckel’s diverticulum complicated by stenosis of the 24. Filston HC, Kirks DR: Malrotation—the ubiquitous anomaly.
colon. Am J Gastroenterol 92:2114-2116, 1997 J Pediatr Surg 16:614-620, 1981
10. Smyth RL: Fibrosing colonopathy in cystic fibrosis. Arch Dis 25. Torres AM, Ziegler MM: Malrotation of the intestine. World
Child 74:464-468, 1996 J Surg 17:326-331, 1993
11. Moore TC: Omphalomesenteric duct malformations. Semin Pe- 26. Di Maggio G, De Felice C, Messina M, Biagini G, Tota G,
diatr Surg 5:116-123, 1996 Bracci R: Intrauterine volvulus without malrotation in a very low-birth-
12. Bartels RJ: Duplication of the stomach. Case report and review weight preterm infant. Eur J Pediatr Surg 7:364-366, 1997
of the literature. Am Surg 33:747-752, 1967 27. Moore GP, Burkle FM Jr: Isolated axial volvulus of a Meckel’s
13. Sato T, Oyamada M, Chiba H, et al: Ileal duplication cyst diverticulum. Am J Emerg Med 6:137-142, 1988
associated with heterotopic pancreas: report of a case and literature 28. Gupta S, Gupta SK: Acute caecal volvulus: report of 22 cases
review. Acta Pathol Jpn 43:597-602, 1993 and review of literature. Ital J Gastroenterol 25:380-384, 1993
14. Oudshoorn JH, Heij HA: Intestinal obstruction caused by dupli- 29. Mercado-Deane MG, Burton EM, Howell CG: Transverse colon
cation of the caecum. Eur J Pediatr 155:338-340, 1996 volvulus in pediatric patients. Pediatr Radiol 25:111-112, 1995
INTESTINAL OBSTRUCTION IN NEONATAL/PEDIATRIC SURGERY 253

30. Asabe K, Ushijima H, Bepu R, Shirakusa T: A case of transverse 54. Khuroo MS: Ascariasis. Gastroenterol Clin North Am 25:553-
colon volvulus in a child and a review of the literature in Japan. 577, 1996
J Pediatr Surg 37:1626-1628, 2002 55. St Georgiev V: Pharmacotherapy of ascariasis. Expert Opin
31. Hajivassiliou CA, Farrow G, Harvey J: Splenic flexure volvulus Pharmacother 2:223-239, 2001
presenting as proximal small intestinal obstruction. Aust N Z J Surg 56. Mares AJ, Finaly R, Mordechai J, Motovic A: “Pantaloon”
69:318-319, 1999 phytobezoar: an unusual cause of intestinal obstruction associated with
32. Salas S, Angel CA, Salas N, Murillo C, Swischuk L: Sigmoid Meckel’s diverticulum. Isr J Med Sci 29:683-685, 1993
volvulus in children and adolescents. J Am Coll Surg 190:717-723, 57. Decker CJ: Pica in the mentally handicapped: a 15-year surgical
2000 perspective. Can J Surg 36:551-554, 1993
33. Smith SD, Golladay ES, Wagner C, Seibert JJ: Sigmoid volvu- 58. Traubici J, Daneman A, Wales P, Gibbs D, Fecteau A, Kim P:
lus in childhood. South Med J 83:778-781, 1990 Mesenteric lymphatic malformation associated with small-bowel vol-
34. Houshian S, Sorensen JS, Jensen KE: Volvulus of the transverse vulus—two cases and a review of the literature. Pediatr Radiol 32:362-
colon in children. J Pediatr Surg 33:1399-1401, 1988 365, 2002
35. Ziegler MM: Meconium ileus. Curr Probl Surg 31:731-777, 59. Lin JI, Fisher J, Caty MG: Newborn intraabdominal cystic
1994 lymphatic malformations. Semin Pediatr Surg 9:141-145, 2000
36. Littlewood JM: Cystic fibrosis: gastrointestinal complications. 60. Choo KL, Borzi PA, Mortimore RJ: Neonatal intestinal obstruc-
Br Med Bull 48:847-859, 1992 tion from solitary intestinal fibromatosis. Pediatr Surg Int 17:467-469,
37. Eggermont E, De Boeck K: Small-intestinal abnormalities in 2001
cystic fibrosis patients. Eur J Pediatr 150:824-828, 1991 61. Karande T, Oak SN, Trivedi A, Karmarkar S, Kulkarni B,
38. Rescorla FJ, Grosfeld JL: Contemporary management of meco- Kalgutkar A: Proximal jejunal obstruction due to eosinophilic gastro-
nium ileus. World J Surg 17:318-325, 1993 enteritis. J Postgrad Med 42:121-123, 1996
39. Dimmitt RA, Moss RL: Meconium diseases in infants with very 62. Zora JA, O’Connell EJ, Sachs MI, Hoffman AD: Eosinophilic
low birth weight. Semin Pediatr Surg 9:79-83, 2000
gastroenteritis: a case report and review of the literature. Ann Allergy
40. Konvolinka CW, Frederick J: Milk curd syndrome in neonates.
53:45-47, 1984
J Pediatr Surg 24:497-498, 1989
63. Demirkan NC, Akalin T, Yilmaz F, et al: Inflammatory myofi-
41. Stringer MD, Pablot SM, Brereton RJ: Paediatric intussuscep-
broblastic tumor of small bowel wall in childhood: report of a case and
tion. Br J Surg 79:867-876, 1992
a review of the literature. Pathol Int 51:47-49, 2001
42. Pollack CV Jr, Pender ES: Unusual cases of intussusception.
64. Kennedy AP Jr, Cameron B, Dorion RP, McGill C: Pediatric
J Emerg Med 9:347-355, 1991
intestinal leiomyosarcomas: case report and review of the literature.
43. DiFiore JW: Intussusception. Semin Pediatr Surg 8:214-220,
J Pediatr Surg 32:1234-1236, 1997
1999
65. Shah RS, Pikale HS, Birmole BJ, Kulkarni BK, Borwankar SS:
44. Janin Y, Stone AM, Wise L: Mesenteric hernia. Surg Gynecol
Adenocarcinoma of the colon in a child. J Postgrad Med 38:81-83,
Obstet 150:747-754, 1980
1992
45. Lough JO, Estrada RL, Wiglesworth FW: Internal hernia into
Treves’ Field Pouch: report of two cases and review of literature. 66. Kumar M, Deb M, Parshad R: Abdominal cocoon: report of a
J Pediatr Surg 4:198-207, 1969 case. Surg Today 30:950-953, 2000
46. Hartman GE, Drugas GT, Shochat SJ: Post-necrotizing entero- 67. Faulk DL, Anuras S, Christensen J: Chronic intestinal
colitis strictures presenting with sepsis or perforation: risk of clinical pseudoobstruction. Gastroenterology 74:922-931, 1978
observation. J Pediatr Surg 23:562-566, 1988 68. Kahn E: Gastrointestinal manifestations in pediatric AIDS. Pe-
47. Rescorla FJ: Surgical management of pediatric necrotizing en- diatr Pathol Lab Med 17:171-208, 1997
terocolitis. Curr Opin Pediatr 7:335-341, 1995 69. McAlister WH, Kronemer KA: Emergency gastrointestinal ra-
48. Puri P: Hirschsprung’s disease: clinical and experimental obser- diology of the newborn. Radiol Clin North Am 34:819-844, 1996
vations. World J Surg 17:374-384, 1993 70. Hernanz-Schulman M: Imaging of neonatal gastrointestinal ob-
49. Loening-Baucke V, Kimura K: Failure to pass meconium: di- struction. Radiol Clin North Am 37:1163-1186, 2000
agnosing neonatal intestinal obstruction. Am Fam Physician 60:2050- 71. Pameijer CR, Hubbard AM, Coleman B, Flake AW: Combined
2043, 1999 pure esophageal atresia, duodenal atresia, biliary atresia, and pancreatic
50. Miraj AM, Brereton RJ, Huskisson L: Rectal atresia and stenosis ductal atresia: prenatal diagnostic features and review of the literature.
[comment]. J Pediatr Surg 30:1546 –1550, 1995 J Pediatr Surg 35:745-747, 2000
51. Lynch JM, Albanese CT, Meza MP, Wiener ES: Intestinal 72. Ramseyer L, Abernethy EA III, McCune EA, Steffen HL: The
stricture following seat belt injury in children. J Pediatr Surg 31:1354- role of CT in the diagnosis of small bowel obstruction: a case and
1357, 1996 literature review. J Okla State Med Assoc 91:103-106, 1998
52. Telander RL: Surgical management of Crohn’s disease in chil- 73. Dalla Vecchia LK, Grosfeld JL, West KW, Rescorla FJ, Scherer
dren. Curr Opin Pediatr 7:328-334, 1995 LR, Engum SA: Intestinal atresia and stenosis: a 25-year experience
53. de Silva NR, Guyatt HL, Bundy DA: Worm burden in intestinal with 277 cases. Arch Surg 133:490-496, 1998
obstruction caused by Ascaris lumbricoides. Trop Med Int Health 74. Bax NM, Ure BM, van der Zee DC, van Tuijl I: Laparoscopic
2:189-190, 1997 duodenoduodenostomy for duodenal atresia. Surg Endosc 15:217, 2001

You might also like