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