(Ashcraft,....

Intussusception is one of the most frequent causes of bowel obstruction in infants and
toddlers. Nevertheless, an individual pediatrician may encounter this condition only rarely. It
was first described in 1674 by Paul Barbette of Amsterdam and was defined by Treves in
1899 as the prolapse of one part of the intestine into the lumen of the immediately adjoining
part.1 John Hutchinson reported the first successful operation for intussusception in 1873.2 In
1876, Harold Hirschsprung described hydrostatic reduction, which led to a 23% reduction in
mortality.3 Ravitch popularized the use of contrast enema reduction for intussusception,
which gradually became the accepted initial treatment for pediatric intussusception in stable
patients.
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PENDAHULUAN
Intususepsi merupakan salah satu penyebab obstruksi usus pada bayi dan balita.
Intususepsi pertama kali dijelaskan pada tahun 1674 oleh Paul Barbette dari Amsterdam dan
didefinisikan oleh Treves pada tahun 1899 sebagai prolaps satu bagian usus ke dalam lumen
bagian yang bersebelahan. John Hutchinson melaporkan operasi pertama yang berhasil untuk
intususepsi pada tahun 1873. Pada tahun 1876, Harold Hirschsprung menggambarkan reduksi
hidrostatik, yang menyebabkan penurunan mortalitas 23%. Ravitch memperkenalkan
penggunaan kontras enema untuk intususepsi, yang menjadi pengobatan awal intususepsi
pada pasien stabil.
PATOFISIOLOGI
Intussusception is the acquired invagination of one portion of the intestine into the adjacent
bowel. It is described by the proximal, inner segment of intestine (intussusceptum) first and
the outer distal, receiving portion of intestine (intussuscipiens) last. Eighty to 95 percent of
pediatric intussusceptions are ileocolic. The ileoileal, cecocolic, colocolic, and jejunojejunal
varieties occur with increasing rarity.5 Occasionally, an intussusception may have an
identifiable lesion that serves as a lead point, drawing the intussusceptum into the distal
bowel by peristaltic activity. As the mesentery of the proximal bowel is drawn into the distal
bowel, it is compressed, resulting in venous obstruction and edema of the bowel wall. If
reduction of the intussusception does not occur, arterial insufficiency will ultimately lead to
ischemia and bowel wall necrosis. Although spontaneous reduction undoubtedly occurs, the
natural history of an intussusception is to progress to a fatal outcome as a result of sepsis
unless the condition is recognized and treated appropriately. For many reasons, the morbidity
and mortality rates have decreased dramatically at children’s hospitals in North America
since the mid 1940s.
--
PATOFISIOLOGI
Intususepsi adalah invaginasi yang diperoleh dari satu bagian usus ke usus yang
berdekatan. Hal ini dijelaskan oleh bagian proksimal, bagian dalam usus (intussusceptum)
pertama dan distal luar yang merupakan bagian penerima usus (intussuscipiens). Delapan
hingga 95 persen kasus intususepsi anak adalah ileocolic, sedangkan ileoileal, cecocolic,
colocolic, dan jejunojejunal jarang terjadi. Intususespsi terjadi saat intususeptum masuk ke
dalam usus distal oleh aktivitas peristaltik. Mesenterium usus proksimal tertarik ke usus
distal, kemudian terkompres sehingga terjadi obstruksi vena dan edema dinding usus. Jika
intususepsi terus berlanjut, maka insufisiensi arteri akhirnya akan menyebabkan iskemia dan
nekrosis dinding usus.
Idiopathic (or Primary) Intussusception
The vast majority of cases of intussusception do not have a pathologic lead point and are
classified as primary or idiopathic intussusceptions. In idiopathic intussusception, the lead
point is generally attributed to hypertrophied Peyer’s patches within the ileal wall.5
Intussusception occurs frequently in the wake of an upper respiratory tract infection or an
episode of gastroenteritis, providing an etiology for the enlargement of the lymphoid tissue.
Adenoviruses, and to a much lesser extent rotaviruses, have been implicated in up to 50% of
cases.7,8 Most cases of primary intussusception occur in children between the ages of 6 to 36
months of age when there is a high susceptibility to these viruses. Other contributing
evidence that viruses might play a role in idiopathic intussusception includes the rise in cases
during seasonal respiratory viral illnesses and the documented increase in the incidence of
intussusception associated with previous rotaviral immunization.9 The most recent
immunization, Rotashield, has not been associated with a similar increase in intussusception.
--
Dalam intususepsi idiopatik, titik utama umumnya dikaitkan dengan hipertrofi Peyer’s
patches di dalam dinding ileum. Intususepsi sering terjadi setelah infeksi saluran pernapasan
atas atau episode gastroenteritis, yang menyebabkan terjadinya pembesaran jaringan limfoid.
Adenovirus, dan pada tingkat yang jauh lebih rendah, rotavirus, telah terlibat hingga 50% dari
kasus.7,8 Sebagian besar kasus intususepsi primer terjadi pada anak-anak antara usia 6
hingga 36 bulan ketika ada kerentanan yang tinggi terhadap virus. Penyebab lain yang
berkontribusi bahwa virus mungkin berperan dalam intususepsi idiopatik yaitu peningkatan
kasus selama penyakit virus pernapasan musiman dan peningkatan insiden intususepsi yang
dikaitkan dengan imunisasi rotaviral sebelumnya.

Secondary Intussusception
An intussusception may have an identifiable lesion that serves as a lead point, drawing the
proximal bowel into the distal bowel by peristaltic activity. These anatomic lead points tend
to increase in proportion to age, especially after 2 years of age.5,13,14 The incidence of a
definite anatomic lead point ranges from 1.5% to 12%.6,12,13 The most common pathologic
lead point is a Meckel’s diverticulum followed by polyps and duplications (see Fig. 41-6).
Other benign lead points are the appendix, hemangiomas, carcinoid tumors, foreign bodies,
ectopic pancreas or gastric mucosa, hamartomas from Peutz-Jeghers syndrome (Fig. 39-1),
and lipomas. Malignant causes, which are very rare, include lymphomas, lymphosarcomas,
small bowel tumors, and melanomas.15 The occurrence of malignant lesions increases with
age. Small bowel intussusceptions related to gastrojejunostomy tubes also have been
described.

Various systemic diseases, such as Henoch-Schönlein purpura and cystic fibrosis, may also
be complicated by intussusception. The majority of abdominal complaints in Henoch-
Schönlein purpura are due to vasculitis in the gastrointestinal tract. However, submucosal
hemorrhages within the bowel wall can function as lead points in Henoch-Schönlein purpura
and cause similar abdominal complaints. Patients with cystic fibrosis are prone to
intussusception due to the inspissated secretions and thick fecal matter in the intestinal lumen.
This thick, tenacious stool acts as a lead point to produce repeated intussusceptions, more
typically seen in children aged 9 to 12 years.5 Other rare diseases associated with
intussusception are celiac disease and Clostridium difficile colitis.17

INCIDENCE
Idiopathic intussusception can occur at any age. However, the greatest incidence occurs in
infants between ages 5 and 10 months.18 The incidence of intussusception is highest in the
first and second years of life and is uncommon below 3 months of age and after 3 years of
life. The condition has been described in premature infants and has been postulated as the
cause of small bowel atresia in some cases.19 Most patients are wellnourished, healthy
infants. Approximately two thirds are male.5

Intususepsi idiopatik dapat terjadi pada semua usia. Namun insiden terbanyak terjadi pada
anak usia 5-10 bulan. Insiden tertinggi terjadi pada tahun pertama dan kedua kehidupan dan
jarang terjadi pada anak usia di bawah 3 bulan dan setelah 3 tahun kehidupan. Kondisi ini
telah dideskripsikan pada bayi prematur sebagai penyebab atresia usus kecil dalam beberapa
kasus. Sebagian besar pasien adalah bayi sehat dan gizi baik. Sekitar dua pertiga adalah laki-
laki
CLINICAL PRESENTATION

The classic presentation of intussusception is a young child with intermittent, crampy

abdominal pain associated with “currant jelly” stools and a palpable mass on physical
examination, although this triad is seen in less than a fourth of children.20 The abdominal
pain is sudden in onset in a child who was previously comfortable. The child may stiffen and
pull the legs up to the abdomen. Hyperextension, writhing, and breath holding may be
followed by vomiting. The attack often ceases as suddenly as it started. Between attacks, the
child may appear comfortable or may fall asleep. After some time, the child becomes
lethargic between episodes of pain. The symptoms are associated with anorexia and
dehydration.

Small or normal bowel movements may result initially from the straining as the colon
evacuates distal to the obstruction. As the obstruction worsens, the child will have bilious
emesis and worsening abdominal distention. Later in the course, the stools may be tinged
with blood. The progression of bowel ischemia, sloughing of mucosa, and compression of the
mucous glands within the intussusceptum leads to the evacuation of dark, red mucoid clots or
“currant jelly” stools. The latter is often a late sign. A diagnostic pitfall is to wait for this sign
to occur.

Presentasi klasik intususepsi adalah anak dengan nyeri perut intermittent dan crampy, feses
"currant jelly" dan teraba massa pada pemeriksaan fisik, meskipun triad ini terlihat pada
kurang dari seperempat anak-anak. Anak merasakan nyeri perut mendadak. Anak mungkin
kaku (stifness) dan menarik kaki ke arah perut. Terjadi hiperekstensi, menggeliat, dan
menahan nafas yang dapat diikuti dengan muntah. Serangan itu sering terjadi secara
berulang-ulang. Di antara serangan, anak mungkin tampak nyaman atau tertidur. Setelah
beberapa waktu, anak menjadi letargi di antara episode nyeri. Gejalanya berhubungan dengan
anoreksia dan dehidrasi. Gerakan usus kecil atau normal dapat terjadi awalnya dari
mengejan saat usus besar menguap jauh ke obstruksi. Ketika obstruksi memburuk, anak
akan mengalami bilious emesis dan memburuknya distensi abdomen. Kemudian dalam
perjalanan, di dalam feses dijumpai darah. Usus iskemik, peluruhan mukosa, dan kompresi
kelenjar mukus di dalam intususeptum menyebabkan “currant jelly” stools.
PHYSICAL EXAMINATION
The child’s vital signs are usually normal early in the course of the disease. During painless
intervals, the child might look comfortable and the physical examination will be
unremarkable. Based on the benign clinical appearance, this may lead to an erroneous
diagnosis of constipation or gastroenteritis. However the cramping episodes usually occur
every 15 to 30 minutes. When the pain occurs, the child may be difficult to examine. There
may be audible peristaltic rushes, and a mass might be palpable anywhere in the abdomen or
even visualized if the child is relatively thin (Fig. 39-2). The right lower abdominal quadrant
may appear flat or empty (Dance’s sign) as the intussuscepted mass is pulled up. The mass is
often curved because it is tethered by the blood vessels and mesentery on one side. On rectal
examination, blood-stained mucus or blood may be encountered. The longer the duration of
symptoms, the more likely the probability of identifying gross or occult blood. Palpation of
the intussuscepted mass on bimanual examination is possible but rare.

Prolapse of the intussusceptum through the anus is a grave sign, particularly when the
intussusceptum is ischemic. An ileocolic or colocolic intussusception can progress to the
rectosigmoid and through the anus. Such a patient would undoubtedly exhibit signs of
systemic illness. The greatest danger in a case of prolapse of the intussusceptum is that the
examiner will misdiagnose the condition and attempt to reduce what is thought to be a rectal
prolapse. Careful physical examination of the intussusceptum through the anus is mandatory
to avoid this potentially life-threatening error in diagnosis. This is done by inserting a
lubricated tongue blade along the side of the protruding mass before reduction is attempted. If
the blade can be inserted more than 1 or 2 cm into the anus alongside of the mass, the
diagnosis of intussusception should be considered. Rectal prolapse, while producing
discomfort, is not generally accompanied by vomiting or signs of sepsis.

If the obstructive process worsens and bowel ischemia has occurred, dehydration, fever,
tachycardia, and hypotension can develop in quick succession as a result of bacteremia and
bowel perforation. In the
absence of a rapid diagnosis, fluid resuscitation, and operation, a fatal outcome is likely.
DIAGNOSIS
Laboratory Studies
Although there are no specific laboratory studies that aid with the specific diagnosis of
intussusception, as the process progresses there may be associated electrolyte abnormalities
due to dehydration, anemia, and/or leukocytosis.
Abdominal Radiography
In about half of cases, the diagnosis of intussusception can be suspected on plain flat and
upright abdominal radiographs (Fig. 39-3). Suggestive radiographic abnormalities include an
abdominal mass, abnormal distribution of gas and fecal contents, sparse large bowel gas, and
air-fluid levels in the presence of bowel obstruction. 21 The target sign or coiled spring sign
denotes a cross-sectional appearance of the invaginated mesentery and bowel into the
intussuscipiens, appearing as concentric lucencies on plain film. The meniscus sign is a
crescent-shaped lucency in the colon outlining the distal end of the intussusception. However,
plain films have limited value in confirming the diagnosis and cannot be used as the sole
diagnostic test.22

Ultrasonography
The use of abdominal ultrasound for the evaluation of intussusception was first described in
1977.23 Since then, many institutions have adopted its use as a screening tool because of the
lack of radiation exposure and decreased cost.24 The intussusception is usually discovered in
the right side of the abdomen. A transverse sonographic image of the bowel consists of
alternating rings of low and high echogenicity representing the bowel wall and mesenteric fat
within the intussusceptum. This characteristic finding has been referred to as a “target” or
“doughnut” lesion (Fig.39-4). The “pseudokidney” sign is seen on a longitudinal section and
appears as superimposed hypoechoic and hyperechoic layers (Fig. 39-5). This pattern is
similar to a sandwich and represents the edematous walls of the intussusceptum within the
intussuscipiens. Ultrasonography can also guide the therapeutic reduction of an
intussusception by using a 10% meglumine iothalamate enema in a balanced salt solution or
using sonographically guided pneumatic pressure.24,25 Successful reduction results in a
smaller “donut,” with an echogenic rim representing the edema of the terminal ileum and
ileocecal valve. Equivocal findings using this modality should mandate a conventional
contrast or air enema.26
Computed Tomography and Magnetic Resonance Imaging
Neither computed tomography (CT) nor magnetic resonance imaging (MRI) are routinely
used in the evaluation of a patient with intussusception, although either may reveal possible
pathologic causes for intussusception, such as a malignancy (i.e., lymphoma). The
characteristic finding is a target or doughnut sign (invaginated bowel within the contiguous
bowel loop) (Fig. 39-6). Transient small bowel intussusceptions that are discovered on CT or
MRI are usually not clinically significant.27 These “incidental” intussusceptions involve a
small segment of bowel with no pathologic lead point. Repeat imaging usually demonstrates
resolution of the intussusception. Radiographic or surgical treatment should be based on
clinical findings in symptomatic patients.28 Laparoscopy is an excellent means to evaluate
these patients if surgical intervention is needed.

NONOPERATIVE MANAGEMENT
If the diagnosis of intussusception is suspected, a nasogastric tube may be needed to
decompress the stomach. Bowel rest and intravenous fluid resuscitation should be initiated. A
complete blood cell count and serum electrolytes are obtained. An air or contrast enema is the
study of choice for diagnosis and potential firstline treatment. The complications with
hydrostatic or pneumatic reduction are minimal as long as certain guidelines are followed.
Absolute contraindications to nonoperative reduction are intestinal perforation (free intra
abdominal air), peritonitis, or persistent hypotension. Findings of peritonitis or bowel
perforation warrant surgical exploration.

Hydrostatic Reduction
The methodology for hydrostatic reduction has not changed significantly since its first
description in 1876.3 Although hydrostatic reduction with barium under fluoroscopic
guidance has been the historic method since the mid 1980s,22 most pediatric centers use
water-soluble isotonic contrast because of the potential hazard of barium peritonitis in
patients with intestinal perforation.
A large, lubricated catheter is inserted into the rectum, and a seal is attempted by
firmly taping the buttocks together. Balloon catheters are avoided by most radiologists owing
to the risk of perforation and potential for a closed-loop obstruction. The “rule of threes” is
commonly described, consisting of (1) hydrostatic reduction kept at a height of 3 feet above
the patient and (2) no more than three attempts, with (3) each attempt no more than 3 minutes
each. Under fluoroscopic evaluation, the contrast agent is observed until a concave filling
defect is seen (Fig. 39-7). Occasionally, a curvilinear spiral pattern can be seen as the contrast
medium surrounds the intussusceptum. Constant hydrostatic pressure is continued as long as
reduction is occurring. Additional attempts can be repeated a second or third time.
Hydrostatic reduction is complete when the contrast
medium freely flows through the ileocecal valve into the terminal ileum. Successful reduction
in uncomplicated patients is seen in about 85% of cases and ranges from 42% to 95%.29
There is less success with long-segment intussusceptions,30 patients with symptoms for more
than 24 hours, and those with pathologic lesions. The advantages of nonoperative over
operative reduction are decreased morbidity, cost, and length of hospitalization.

Pneumatic Reduction
Air reduction of intussusception was first described in 1897.31 Pneumatic reduction gained
popularity in the late 1980s, owing to the higher rates of successful reduction reported in
large international series.32 Success rates of reduction reported between 1980 and 1991 using
hydrostatic techniques were 50% to compared with 75% to 94% between 1986 and 1991
using pneumatic reduction.5 Advocates of the air enema believe that the method is quicker
and safer, is less messy, and decreases the exposure time to radiation.32 The procedure is
fluoroscopically monitored as air is insufflated into the rectum (Fig. 39-8). The maximum
safe air pressure is 80 mm Hg for younger infants and 110 to 120 mm Hg for older infants.
Carbon dioxide can be used instead of air because of the advantages of rapid reabsorption and
less abdominal discomfort. Accurate pressure measurements are possible, and reduction rates
are higher than with hydrostatic techniques.33 Potential drawbacks of pneumatic reduction
include the possibility of development of a tension pneumoperitoneum, poor visualization of
lead points, and relatively poor visualization of the intussusception and reduction process,
resulting in false-positive reductions.33-35 Rates of perforation range from 0.4% to 2.5%.36
Several studies have shown improved reduction rates by a second attempt after
waiting between 30 minutes to 24 hours after the initial attempt.37-39 However, the risks of
the increasing radiation burden must be weighed against the risks of emergency surgery and
anesthesia.40 If nonoperative reduction is successful either by hydrostatic or pneumatic
technique, the patient should be admitted for observation and should receive a short period of
bowel rest and intravenous fluids. Any clinical signs of abdominal pain after reduction could
be a sign of ischemic bowel or recurrent intussusception (see later).

OPERATIVE TREATMENT
Open Approach
Surgery is indicated when nonoperative reduction is unsuccessful or incomplete (Fig.
39-9), for signs of peritonitis, for the presence of a pathologic lead point, or for radiographic
evidence of pneumoperitoneum. Preoperative preparation includes administration of broad-
spectrum antibiotics, intravenous fluid resuscitation, and placement of a nasogastric tube for
decompression.
 Open exploration of the abdomen and intestines has been traditionally performed
through a right lower quadrant incision. Moderate serous ascites may be encountered owing
to the obstructive lesion. Usually, the intussusception involves the cecum and terminal ileum,
which can be delivered through the incision. Care must be taken to evaluate the extent of the
intussusceptum before delivering it, because it can extend into the rectosigmoid region in
severe cases. Extension of the incision is often required in such cases.
Once the leading edge of the intussusceptum is identified, it is gently manipulated
back toward its normal position in the terminal ileum (Fig. 39-10). Excessive force or pulling
is avoided to prevent injury or perforation of the bowel and subsequent contamination.
Inability to manually reduce the intussusception, the finding of ischemic bowel, or
identification of a pathologic lesion requires surgical resection and bowel anastomosis or
diversion, depending on the condition of the bowel and child (see Figs. 39-1 and 39-11).
If surgical reduction is possible, the bowel is then evaluated for viability, perforation,
or a pathologic lead point (especially in children older than 2 years of age). Questionable
ischemic bowel can be warmed with saline-soaked laparotomy pads and reevaluated by the
coloration of the bowel, peristalsis, presence of Doppler signals, or Wood’s lamp evaluation
using fluorescein. After complete reduction of the intussusception, an incidental
appendectomy is usually performed because the location of the abdominal scar is similar to
an open appendectomy incision.

Laparoscopic Approach
Initially, the use of laparoscopy in intussusception was strictly diagnostic and used in
cases with equivocal radiographic studies or those with suspected pathologic lesions. Once
the diagnosis was confirmed, the operation was converted to a laparotomy. Recent small
studies have demonstrated laparoscopic reduction of intussusception with variable degrees of
success.41-46
Various techniques have been reported, but a majority of minimally invasive
approaches describe the use of three abdominal ports: one in the infraumbilical region with
two other ports along the left side of the abdomen. Laparoscopic reduction is accomplished
by applying gentle pressure distal to the intussusceptum using atraumatic graspers. Although
counterintuitive to the conventional open method, traction is usually required proximal to the
intussuscipiens to complete the reduction (Figs. 39-12 and 39-13). Careful inspection is then
performed to evaluate for any signs of ischemia, necrosis, or perforation. If a resection is
required, this can sometimes be accomplished by exteriorizing the bowel through the
periumbilical incision. If this cannot be accomplished safely, the operation should be
converted to an open laparotomy.

RECURRENT INTUSSUSCEPTION
Recurrent intussusception has been described in 2% to 20% of cases (average about 5%),
with about one third occurring within 24 hours and the majority within 6 months of the initial
episode.7,47-49 Recurrences usually have no defined lead point, and they are less likely to
occur after surgical reduction or resection. Multiple recurrences can occur in the same
patient. Success rates with enema reduction after one recurrence are comparable to those with
the first episode and are better if the child did not previously require operative reduction.
Patients tend to be seen earlier with recurrent intussusception, and they have fewer
symptoms. Irritability and discomfort may be the only clues during the early stage of a
recurrence.
An overriding concern in recurrent intussusception is occult malignancy, although
multiple recurrences are not a contraindication to attempted radiologic reduction. 48
Unfortunately, the clinical findings or pattern of recurrence do not predict the presence of a
pathologic lead point. A careful imaging search is mandatory, and ultrasonography has been
recommended as the imaging study of choice.50 Indications for operation include (1)
irreducible recurrence, (2) clinical evidence to suggest a pathologic lead point, (3)
documentation of a pathologic lead point by an imaging procedure, or (4) persistence of
clinical symptoms after the completion of the enema.47

POSTOPERATIVE INTUSSUSCEPTION
Intussusception accounts for 3% to 10% of cases of postoperative bowel obstruction
during childhood and may occur after operations performed for a variety of conditions. 51
Thoracic and abdominal operations have been followed by latent intussusception. Because
ileus and adhesive obstruction more frequently come to mind as a cause for postoperative
intestinal obstruction, these intussusceptions may not be diagnosed preoperatively, although
ultrasonography has proved to be a successful diagnostic modality.51 Most postoperative
intussusceptions occur within a month of the initial procedure. An interval of about 10 days
between initial operation and development of symptoms is average.52 Most postoperative
intussusceptions are ileoileal and respond to operative reduction without resection.51,52