Intussusception (medical disorder)

An intussusception is a medical condition in which a part oI the intestine has invaginated into
another section oI intestine, similar to the way in which the parts oI a collapsible telescope slide
into one another.
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This can oIten result in an obstruction. The part that prolapses into the other
is called the intussusceptum, and the part that receives it is called the intussuscipiens.
Symptoms
arly symptoms can include nausea, vomiting (sometimes bile stained (green color)), pulling
legs to the chest area, and intermittent moderate to severe cramping abdominal pain. Pain is
intermittent not because the intussusception temporarily resolves, but because the intussuscepted
bowel segment transiently stops contracting. Later signs include rectal bleeding, oIten with "red
currant jelly" stool (stool mixed with blood and mucus), and lethargy. Physical examination may
reveal a "sausage-shaped" mass Ielt upon palpation oI the abdomen.
In children or those too young to communicate their symptoms verbally, they may cry, draw
their knees up to their chest or experience dyspnea (diIIicult or painIul breathing) with
paroxysms oI pain.
Fever is not a symptom oI intussusception. However, intussusception can cause a loop oI bowel
to become necrotic. This leads to perIoration and sepsis, which causes Iever.
[edit] Diagnosis
Intussusception is oIten suspected based on history and physical exam, including observation oI
Dance's sign. Per rectal examination is particularly helpIul in children as part oI the
intussusceptum may be Ielt by the Iinger. A deIinite diagnosis oIten requires conIirmation by
diagnostic imaging modalities. Ultrasound is today considered the imaging modality oI choice
Ior diagnosis and exclusion oI intussusception due to its high accuracy and lack oI radiation. A
target-like mass, usually around 3 cm in diameter, conIirms the diagnosis. An x-ray oI the
abdomen may be indicated Ior evaluation oI intestinal obstruction or the presence oI Iree
intraperitoneal gas; the latter Iinding would imply that bowel perIoration has already occurred. In
some institutions, air enema is used Ior diagnosis as the same procedure can be used Ior
treatment.
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[edit] Differential diagnosis
Intussusception has three main diIIerential diagnoses. These are acute gastroenteritis, Henoch
Schnlein purpura, and rectal prolapse. Abdominal pain, vomiting, and stool with mucus and
blood are present in acute gastroenteritis, but diarrhea is the leading symptom. Rectal prolapse
can be diIIerentiated by projecting mucosa that can be Ielt in continuity with the perianal skin,
whereas in intussusception the Iinger may pass indeIinitely into the depth oI sulcus. Henoch
Schnlein purpura presents the characteristic rash.
[edit] Treatment
The condition is not usually immediately liIe-threatening. The intussusception can be treated
with either a barium or water-soluble contrast enema or an air-contrast enema, which both
conIirms the diagnosis oI intussusception, and in most cases successIully reduces it. The success
rate is over 80. However, approximately 510 oI these recur within 24 hours.
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II it cannot be reduced by an enema or iI the intestine is damaged, then a surgical reduction is
necessary. In a surgical reduction, the abdomen is opened and the part that has telescoped in is
squeezed out (rather than pulled out) manually by the surgeon or iI the surgeon is unable to
successIully reduce it or the bowel is damaged, the aIIected section will be resected. More oIten,
the intussusception can be reduced by laparoscopy, whereby the segments oI intestine are pulled
apart by Iorceps.
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[edit] Prognosis
Intussusception may become a medical emergency iI not treated early, as it will eventually cause
death iI not reduced. In developing countries where medical hospitals are not easily accessible,
especially when the occurrence oI intussusception is complicated with other problems, death
becomes almost inevitable. When intussusception or any other severe medical problem is
suspected, the person must be taken to a hospital immediately.
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The outlook Ior intussusception is excellent when treated quickly, but when untreated it can lead
to death within 25 days. Fast treatment is a necessity, because the longer the intestine segment
is prolapsed the longer it goes without bloodIlow, and the less eIIective a non-surgical reduction
will be. Prolonged intussusception also increases the likelihood oI bowel ischemia and necrosis,
requiring surgical resection.
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ln1uSSuSCL1lCn

ackground
Intussusception is a process in which a segment oI intestine invaginates into the adjoining
intestinal lumen, causing bowel obstruction. A common cause oI abdominal pain in children,
intussusception is suggested readily in pediatric practice based on a classic triad oI signs and
symptoms: vomiting, abdominal pain, and passage oI blood per rectum. (See History and
Physical xamination.)
Intussusception presents in 2 variants: idiopathic intussusception, which usually starts at the
ileocolic junction and aIIects inIants and toddlers, and enteroenteral intussusception
(jejunojejunal, jejunoileal, ileoileal), which occurs in older children. The latter is associated with
special medical situations (eg, Henoch-Schnlein purpura |HSP|, cystic Iibrosis, hematologic
dyscrasias) or may be secondary to a lead point and occasionally occur in the postoperative
period. Intussusception is demonstrated in the images below. (See tiology and Pathophysiology.)
Abdomlnal radlograph shows small bowel dllaLaLlon and pauclLy of gas ln Lhe
rlghL lower and upper quadranLs Alr conLrasL enema shows
lnLussuscepLlon ln Lhe cecum 8arlum enema shows lnLussuscepLlon ln
Lhe descendlng colon C1 scan reveals Lhe classlc ylngyang slgn of an
lnLussuscepLum lnslde an lnLussusclplens Abdomlnal ulLrasonography
reveals Lhe classlc LargeL slgn of an lnLussuscepLum lnslde an lnLussusclplens
Laparoscoplc vlew of a [e[uno[e[unal lnLussuscepLlon
o to Pediatric Intussusception Surgery Ior complete inIormation on this topic.
Patient education
ducate parents and caregivers oI a patient treated with nonoperative reduction with regard to the
risks and signs and symptoms oI recurrence so that the initiation oI care is not delayed.
For patient education inIormation, see the Digestive Disorders Center, as well as Abdominal
Pain in Children, Colic, Rectal Bleeding, and Barium nema.

tiology and Patbopbysiology
The pathogenesis oI idiopathic intussusception is not well established. It is believed to be
secondary to an imbalance in the longitudinal Iorces along the intestinal wall. In enteroenteral
intussusception, this imbalance can be caused by a mass acting as a lead point or by a
disorganized pattern oI peristalsis (eg, an ileus in the postoperative period).
As a result oI imbalance in the Iorces oI the intestinal wall, an area oI the intestine invaginates
into the lumen oI adjacent bowel. The invaginating portion oI the intestine (ie, the
intussusceptum) completely 'telescopes into the receiving portion oI the intestine (ie, the
intussuscipiens). This process continues and more proximal areas Iollow, allowing the
intussusceptum to proceed along the lumen oI the intussuscipiens.
II the mesentery oI the intussusceptum is lax and the progression is rapid, the intussusceptum can
proceed to the distal colon or sigmoid and even prolapse out the anus. The mesentery oI the
intussusceptum is invaginated with the intestine, leading to the classic pathophysiologic process
oI any bowel obstruction.
arly in this process, lymphatic return is impeded; then, with increased pressure within the wall
oI the intussusceptum, venous drainage is impaired. II the obstructive process continues, the
pressure reaches a point at which arterial inIlow is inhibited, and inIarction ensues. The intestinal
mucosa is extremely sensitive to ischemia because it is Iarthest away Irom the arterial supply.
Ischemic mucosa sloughs oII, leading to the heme-positive stools and subsequently to the classic
"currant jelly stool" (a mixture oI sloughed mucosa, blood, and mucus). II untreated, transmural
gangrene and perIoration oI the leading edge oI the intussusceptum occur.
ead points
In approximately 2-12 oI children with intussusception, a surgical lead point is Iound.
Occurrence oI surgical lead points increases with age and indicates that the probability oI
nonoperative reduction is highly unlikely. xamples oI lead points are as Iollows:
O ,eckel dlverLlculum
1

O Lnlarged mesenLerlc lymph node
O 8enlgn or mallgnanL Lumors of Lhe mesenLery or of Lhe lnLesLlne lncludlng lymphoma polyps
ganglloneuroma
2
and hamarLomas assoclaLed wlLh euLz!eghers syndrome
O ,esenLerlc or dupllcaLlon cysLs
O Submucosal hemaLomas whlch can occur ln paLlenLs wlLh PS and coagulaLlon dyscraslas
O LcLoplc pancreaLlc and gasLrlc resLs
O lnverLed appendlceal sLumps
O SuLures and sLaples along an anasLomosls
O lnLesLlnal hemaLomas secondary Lo abdomlnal Lrauma
O orelgn body
O Pemangloma
O aposl sarcoma
3

O osLLransplanLaLlon lymphoprollferaLlve dlsorder (1Lu)
4

enocb-Scbnlein purpura
Children with HSP oIten present with abdominal pain secondary to vasculitis in the mesenteric,
pancreatic, and intestinal circulation. II pain precedes cutaneous maniIestations, diIIerentiating
HSP Irom appendicitis, gastroenteritis, intussusception, or other causes oI abdominal pain is
diIIicult.
Occasionally, children with HSP develop submucosal hematomas, which can act as lead points
and cause small bowel intussusception. lucidating the cause oI the pain is essential in any child
in whom HSP is suspected.
Since the intussusception associated with HSP is usually enteroenteral (small bowel to small
bowel), these patients require surgery rather than an enema.
During the initial investigation, obtain supine and upright plain radiographs oI the abdomen to
identiIy the small bowel obstruction associated with intussusception. II radiographic Iindings are
normal, assume the patient with HSP has mesenteric vasculitis and treat with steroids.
emopbilia and otber coagulation disorders
Patients with hemophilia and other bleeding disorders may develop intestinal submucosal
hematomas, leading to intussusception. DiIIerential diagnosis includes retroperitoneal
hemorrhage in addition to other usual causes oI abdominal pain. Radiographs oI the abdomen
should reveal a pattern oI small bowel obstruction iI intussusception is present. In the absence oI
intussusception, treatment is supportive with correction oI coagulopathy.
Postoperative intussusceptions
Intussusception is a rare postoperative complication, occurring in 0.08-0.5 oI laparotomies. It
can take place independently oI the site oI the operation. The likely mechanism is due to a
diIIerence in activity between segments oI the intestine recovering Irom an ileus, which produces
the intussusception.
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Intussusception is suggested in any postoperative patient who has a sudden
onset oI a small bowel obstruction aIter a period oI ileus, usually within the Iirst 2 weeks aIter
surgery. Intestinal obstruction secondary to adhesions usually occurs more than 2 weeks aIter the
operation. The treatment is prompt operative reduction.
Indwelling catbeters
Very rarely, indwelling jejunal catheters can lead to intussusception by acting as a lead point,
which is especially true iI the tip oI the catheter has been manipulated or cut so that its surIace is
not smooth. The clinical picture is that oI a small bowel obstruction. Diagnosis can be Iacilitated
by injecting contrast proximal to the catheter and then through the tip oI the catheter. Surgery is
required to remove the tip oI the catheter and to reduce the intussusception.
Cystic fibrosis
Intussusception occurs in approximately 1 oI patients with cystic Iibrosis. Intussusception is
assumed to be precipitated by the thick, inspissated stool material that adheres to the mucosa and
acts as a lead point. OIten, the course is indolent and chronic. DiIIerential diagnosis includes
distal intestinal obstruction syndrome and appendicitis. The majority oI these patients require
operative reduction.
Utber causes
lectrolyte derangements associated with various medical conditions can produce aberrant
intestinal motility, leading to enteroenteral intussusception.
xperimental studies in animals showed that abnormal intestinal release oI nitric oxide, an
inhibitory neurotransmitter, caused relaxation oI the ileocecal valve, predisposing to ileocecal
intussusception.
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Other studies have demonstrated that certain antibiotics cause ileal lymphoid
hyperplasia and intestinal dysmotility, with resultant intussusception.
Other theories have implicated a viral etiology; however, no theory has proven to be reliable. A
seasonal variation in the incidence oI intussusception that corresponds to the peaks in Irequency
oI gastroenteritis (spring and summer) and respiratory illnesses (midwinter) has been described
but has not been universally corroborated.
An association was Iound between the administration oI a rotavirus vaccine (RotaShield) and the
development oI intussusception.
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RotaShield has since been removed Irom the market. These
patients were younger than usual Ior idiopathic intussusception and were more likely to require
operative reduction. It was hypothesized that the vaccine caused reactive lymphoid hyperplasia,
which acted as a lead point.
(In February 2006, a new rotavirus vaccine |RotaTeq| was approved by the US Food and Drug
Administration |FDA|. RotaTeq did not show an increased risk Ior intussusception compared
with placebo in clinical trials.
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A study that involved more than 63,000 patients who received
Rotarix or placebo at ages 2 and 4 months reported a decreased risk Ior intussusception in those
patients receiving Rotarix.
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)
Familial occurrence oI intussusception has been reported in a Iew cases. Intussusception in
dizygotic twins has also been described; however, these reports are extremely rare.
Idiopatbic
In most inIants and toddlers with intussusception, the etiology is unclear. This group is believed
to have idiopathic intussusception. One theory to explain the possible etiology oI idiopathic
intussusception is that it occurs because oI an enlarged Peyer patch; this hypothesis is derived
Irom 3 observations: (1) oIten, the illness is preceded by an upper respiratory inIection, (2) the
ileocolic region has the highest concentration oI lymph nodes in the mesentery, and (3) enlarged
lymph nodes are oIten observed in patients who require surgery. Whether the enlarged Peyer
patch is a reaction to the intussusception or a cause oI it is unclear.


pidemiology
A wide geographic variation in incidence oI intussusception among countries and cities within
countries makes determining a true prevalence oI the disease diIIicult. Studies on the absolute
prevalence oI intussusception in the United States are not available. Its estimated incidence is
approximately 1 case per 2000 live births. In reat Britain, incidence varies Irom 1.6-4 cases per
1000 live births.
Overall, the male-to-Iemale ratio is approximately 3:1. With advancing age, gender diIIerence
becomes marked; in patients older than 4 years, the male-to-Iemale ratio is 8:1.
Two thirds oI children with intussusception are younger than 1 year; most commonly,
intussusception occurs in inIants aged 5-10 months. Intussusception is the most common cause
oI intestinal obstruction in patients aged 5 months to 3 years.
Intussusception can account Ior as many as 25 oI abdominal surgical emergencies in children
younger than 5 years, exceeding the incidence oI appendicitis. Although extremely rare,
intussusception has been reported in the neonatal period.
Prognosis
The prognosis in patients with intussusception is excellent iI the condition is diagnosed and
treated early; otherwise, severe complications and death may occur.
The recurrence rate oI intussusception aIter nonoperative reduction is usually less than 10 but
has been reported to be as high as 15.
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Most intussusceptions recur within 72 hours oI the
initial event; however, recurrences have been reported as long as 36 months later. More than 1
recurrence suggests the presence oI a lead point. A recurrence is usually heralded by the onset oI
the same symptoms as appeared during the initial event. Provide similar treatment Ior a
recurrence unless the suggestion oI a lead point is very strong (in which case, surgical
exploration should be contemplated).
The recurrence rates aIter air enema and barium enema are 4 and 10, respectively.
Recurrences respond to nonoperative reduction in almost 95 oI cases.
Complications associated with intussusception, which rarely occur when the diagnosis is prompt,
include the Iollowing:
O erforaLlon durlng nonoperaLlve reducLlon
O Jound lnfecLlon
O lnLernal hernlas and adheslons causlng lnLesLlnal obsLrucLlon
O Sepsls from undeLecLed perlLonlLls (ma[or compllcaLlon from a mlssed dlagnosls)
O lnLesLlnal hemorrhage
O necrosls and bowel perforaLlon
O ecurrence
With early diagnosis, appropriate Iluid resuscitation, and therapy, the mortality rate Irom
intussusception in children is less than 1. II leIt untreated, this condition is uniIormly Iatal in 2-
5 days.
revlous
roceed Lo Cllnlcal resenLaLlon



istory
The constellation oI signs and symptoms oI intussusception represents one oI the most classic
presentations oI any pediatric illness; however, the classic triad oI vomiting, abdominal pain, and
passage oI blood per rectum occurs in only one third oI patients. The patient is usually an inIant
who presents with vomiting, abdominal pain, passage oI blood and mucus, lethargy, and a
palpable abdominal mass. These symptoms are oIten preceded by an upper respiratory inIection.
In rare circumstances, the parents report 1 or more previous attacks oI abdominal pain within 10
days to 6 months prior to the current episode. These patients are more likely to have a surgical
lead point causing recurrent attacks oI intussusception with spontaneous reduction.
Pain in intussusception is colicky, severe, and intermittent. The parents or caregivers describe the
child as drawing the legs up to the abdomen and kicking the legs in the air. In between attacks,
the child appears calm and relieved.
Initially, vomiting is nonbilious and reIlexive, but when the intestinal obstruction occurs,
vomiting becomes bilious. Any child with bilious vomiting is assumed to have a condition that
must be treated surgically until proven otherwise.
Parents also report the passage oI stools that look like currant jelly. This is a mixture oI mucus,
sloughed mucosa, and shed blood. Diarrhea can also be an early sign oI intussusception.
Lethargy is a relatively common presenting symptom with intussusception. The reason lethargy
occurs is unknown, because lethargy has not been described with other Iorms oI intestinal
obstruction. Lethargy can be the sole presenting symptom, which makes the diagnosis
challenging. Patients are Iound to have an intestinal process late, aIter initiation oI a septic
workup.
In a prospective observational study, Weihmiller et al evaluated several clinical criteria to risk-
stratiIy children with possible intussusception. This study identiIied that age older than 5 months,
male sex, and lethargy were 3 important clinical predictors oI intussusception.
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Pbysical amination
Upon physical examination, the patient is usually chubby and in good health. Intussusception is
uncommon in children who are malnourished. The child is Iound to have periods oI lethargy
alternating with crying spells, and this cycle repeats every 15-30 minutes. The inIant can be pale,
diaphoretic, and hypotensive iI shock has occurred.
The hallmark physical Iindings in intussusception are a right hypochondrium sausage-shaped
mass and emptiness in the right lower quadrant (Dance sign). This mass is hard to detect and is
best palpated between spasms oI colic, when the inIant is quiet. Abdominal distention Irequently
is Iound iI obstruction is complete.
II intestinal gangrene and inIarction have occurred, peritonitis can be suggested on the basis oI
rigidity and involuntary guarding.
arly in the disease process, occult blood in the stools is the Iirst sign oI impaired mucosal blood
supply. Later on, Irank hematochezia and the classic currant jelly stools appear. Fever and
leukocytosis are late signs and can indicate transmural gangrene and inIarction.
Patients with intussusception oIten have no classic signs and symptoms, which can lead to an
unIortunate delay in diagnosis and disastrous consequences.
Maintaining a high index oI suspicion Ior intussusception is essential when evaluating a child
younger than 5 years who presents with abdominal pain or when evaluating a child with HSP or
hematologic dyscrasias.