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You - an emergency doctor was called to a child of 6 months old after 8 hours from the disease onset.
The mother said that, against the background of full wellbeing, the attacks of agitation, food refusal, vomiting and feet stamping occurred.
On physical examination a palpable abdominal mass in the right upper quadrant was found.
Imaging
Abdominal X-ray. A 5.5 cm soft tissue mass is seen in the right abdomen. There are mildly dilated loops of small bowel seen in the pelvis and right lower quadrant. Little air or stool is seen in the left colon. No free air or free identified. The osseous structures are unremarkable except for mild rightward splinting.
Abdominal ultrasonograhy. This transverse sonographic image shows the alternating rings of low and high echogenicity (called the target sign).
Diagnosis???
Incarcerated hernia, Internal hernia, Bowel intussusception, Volvulus, Meckel diverticulum, Gatroenteritis.
Answer
N. Testemitanu State University of Medicine and Pharmacy Department of Pediatric Surgery, Orthopedics and Anesthesiology
Intussusception
Epidemiology
o Intussusception is one of the most frequent causes of bowel obstruction in infants and toddlers. o Nevertheless, an individual pediatrician may encounter this condition only rarely. o 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 (Fig.1). o John Hutchinson reported the first successful operation for intussusception in 1873. o In 1876, Harold Hirschsprung described hydrostatic reduction, which led to a 23% reduction in mortality. o Ravitch popularized the use of contrast enema reduction for intussusception, which gradually became the accepted initial treatment for pediatric intussusception in stable patients.
Pathophysiology
Intussusception is the acquired invagination of one portion of the intestine into the adjacent bowel (Fig.1). It is described by the proximal, inner segment of intestine (intussusceptum) first (black arrow) and the outer distal, receiving portion of intestine (intussuscipiens) last (white arrow).
Fig. 1.
Pathophysiology
Eighty to 95 percent of pediatric intussusceptions are ileocolic (Fig. 2). The ileoileal, cecocolic, colocolic, and jejunojejunal varieties occur with increasing rarity. Occasionally, an intussusception may have an identifiable lesion that serves as a lead point, drawing the intussusceptum into the distal bowel by peristaltic activity.
Pathophysiology
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 (Fig. 3). If reduction of the intussusception does not occur, arterial insufficiency will ultimately lead to ischemia and bowel wall necrosis.
Pathophysiology
For many reasons, the morbidity and mortality rates have decreased dramatically at childrens hospitals worldwide since the mid of 1940s.
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 Peyers patches within the ileal wall (Fig. 4).
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. 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. The most recent immunization, Rotashield, has not been associated with a similar increase in intussusception.
Secondary intussusception
These anatomic lead points tend to increase in proportion to age, especially after 2 years of age.
The incidence of a definite anatomic lead point ranges from 1.5% to 12%.
Secondary intussusception
The most common pathologic lead point is a Meckels diverticulum (Fig. 5) followed by polyps (Fig. 6) and duplications (Fig. 7).
Secondary intussusception
The most common pathologic lead point is a Meckels diverticulum (Fig. 5) followed by polyps (Fig. 6) and duplications (Fig. 7).
Secondary intussusception
The most common pathologic lead point is a Meckels diverticulum (Fig. 5) followed by polyps (Fig. 6) and duplications (Fig. 7).
Secondary intussusception
Other benign lead points are the appendix, hemangiomas, carcinoid tumors, foreign bodies, ectopic pancreas or gastric mucosa, hamartomas from Peutz-Jeghers syndrome (Fig. 8), and lipomas.
Fig. 8. A, Operative view of the outside of the jejunum shows a palpable mass as the lead point of a reduced intussusception. B, A hamartomatous polyp is characteristic of Peutz-Jeghers syndrome. C, Mucocutaneous macular lesions are seen in this patient with Peutz-Jeghers syndrome. Note extension of the pigmentation beyond the vermilion border.
Secondary intussusception
Malignant causes, which are very rare, include lymphomas, lymphosarcomas, small bowel tumors, and melanomas. 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-Schnlein purpura and cystic fibrosis, may also be complicated by intussusception. The majority of abdominal complaints in Henoch-Schnlein purpura are due to vasculitis in the gastrointestinal tract. However, submucosal hemorrhages within the bowel wall can function as lead points in Henoch-Schnlein 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. Other rare diseases associated with intussusception are celiac disease and Clostridium difficile colitis.
Incidence
Idiopathic intussusception can occur at any age. However, the greatest incidence occurs in infants between ages 5 and 10 months. 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. Most patients are wellnourished, healthy infants. Approximately two thirds are male.
Clinical presentation
The classic presentation of intussusception is a young child with: intermittent, crampy abdominal pain associated with recurrent vomiting, currant jelly stools and a palpable mass on physical examination, although this triad is seen in less than a fourth of children. 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 (Fig. 9).
Clinical presentation
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.
Clinical presentation
Physical examination
The childs 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.
Physical examination
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. 11).
Fig. 11. This 10-year-old boy has a palpable sausageshaped mass (arrows) due to an intussusception.
Physical examination
The right lower abdominal quadrant may appear flat or empty (Dances
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.
Physical examination
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.
Physical examination
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.
Although there are no specific laboratory studies that aid with the specific diagnosis of intussusception, as the process progresses there may be associated:
anemia, and/or
leukocytosis.
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 (Fig. 12).
The meniscus (crescent) sign is a crescent-shaped lucency in the colon outlining the distal end of the intussusception (Fig. 12).
Fig. 12. This is a 3 year old female. There is a prominent crescent sign (arrow) in the left upper quadrant. In this case, the crescent sign truly is crescent shaped. Note that it again points in the direction of normal colon transit. If the shape of the crescent is pointing the wrong way, consider the possibility of situs inversus or that this sign is somehow not due to intussusception. There is a target sign in the right upper quadrant. The target is smaller in this case and not as easy to identify. Once it is pointed out, you should be able to appreciate the target sign, which is subtle.
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.
Fig. 12. This is a 3 year old female. There is a prominent crescent sign (arrow) in the left upper quadrant. In this case, the crescent sign truly is crescent shaped. Note that it again points in the direction of normal colon transit. If the shape of the crescent is pointing the wrong way, consider the possibility of situs inversus or that this sign is somehow not due to intussusception. There is a target sign in the right upper quadrant. The target is smaller in this case and not as easy to identify.
However, plain films have limited value in confirming the diagnosis and cannot be used as the sole diagnostic test.
DIAGNOSIS Ultrasonography
Since then, many institutions have adopted its use as a screening tool because of the lack of radiation exposure and decreased cost.
DIAGNOSIS Ultrasonography
Fig. 13. Intussusception: sonographic images through the mid abdomen showing the concentric appearance (target or doughnut sign) on a transverse section through an intussusception (A, arrows) and the hay fork (sandwich, pseudokidney) appearance on a longitudinal section (B). Black arrows indicate intussuscipiens; and white arrows, intussusceptum.
DIAGNOSIS Ultrasonography
This pattern is similar to a sandwich and represents the edematous walls of the intussusceptum within the intussuscipiens (Fig. 13B).
Fig. 13. Intussusception: sonographic images through the mid abdomen showing the concentric appearance (target or doughnut sign) on a transverse section through an intussusception (A, arrows) and the hay fork (sandwich, pseudokidney) appearance on a longitudinal section (B). Black arrows indicate intussuscipiens; and white arrows, intussusceptum.
DIAGNOSIS Ultrasonography
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. 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.
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. 14C).
Fig. 14. Concurrent contrast enema and pelvic CT image of an intussusception. A, Contrast study showing the intussusception low in pelvis. B, CT image of the intussusception. C, CT image of the layered intussuscepted mass. This is the target sign on CT.
Transient small bowel intussusceptions that are discovered on CT or MRI are usually not clinically significant. 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. Laparoscopy is an excellent means to evaluate these patients if surgical intervention is needed.
The methodology for hydrostatic reduction has not changed significantly since its first description in 1876. Although hydrostatic reduction with barium under fluoroscopic guidance has been the historic method since the mid 1980s, 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.
Fig. 15. Fluoroscopic examination using isotonic contrast for hydrostatic reduction of intussusception. A, Intussusception (arrow) seen in midtransverse colon. B, Reduction has occurred to the hepatic flexure. C, Complete reduction with reflux of contrast medium into the terminal ileum. Note the edematous ileocecal valve (arrow).
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%. There is less success with long-segment intussusceptions, 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.
Advocates of the air enema believe that the method is quicker and safer, is
less messy, and decreases the exposure time to radiation.
The procedure is fluoroscopically monitored as air is insufflated into the rectum (Fig. 16). The maximum safe air pressure is 80 mm Hg for younger infants and 110 to 120 mm Hg for older infants.
Fig. 16. Plain radiography and fluoroscopic examination using air for pneumatic reduction of an intussusception. A, Plain radiograph showing a mass effect in the right upper quadrant. B, Pneumatic reduction to the vicinity of the cecum with the intussusception still present (arrow). C, Complete reduction with reflux of air into multiple loops of small intestine.
Accurate pressure measurements are possible, and reduction rates are higher than with hydrostatic techniques.
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.
However, the risks of the increasing radiation burden must be weighed against the risks of emergency surgery and anesthesia.
If nonoperative reduction is successful either by hydrostatic or pneumatic technique, the patient should be admitted for observation and should
Any clinical signs of abdominal pain after reduction could be a sign of ischemic bowel or recurrent intussusception (see later).
OPERATIVE TREATMENT
Surgery is indicated when nonoperative reduction is unsuccessful or incomplete (Fig. 17), for signs of peritonitis, for the presence of a pathologic lead point, or for radiographic evidence of pneumoperitoneum.
Fig. 17. Contrast enema view after hydrostatic reduction of an intussusception to the ileocecal junction. A persistent filling defect (arrow) is present without free reflux into the terminal ileum.
OPERATIVE TREATMENT
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 (Fig. 18).
OPERATIVE TREATMENT
OPERATIVE TREATMENT
Once the leading edge of the intussusceptum is identified, it is gently manipulated back toward its normal position in the terminal ileum (Fig. 19).
Excessive force or pulling is avoided to prevent injury or perforation of the bowel and subsequent contamination.
Fig. 19. Gentle and continuous massage from distal to proximal usually results in reduction of the intussusception.
OPERATIVE TREATMENT
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. 20, 21 and 22).
Fig. 20. A, Operative view of the outside of the jejunum shows a palpable mass as the lead point of a reduced intussusception. B, A hamartomatous polyp is characteristic of Peutz-Jeghers syndrome. C, Mucocutaneous macular lesions are seen in this patient with Peutz-Jeghers syndrome. Note extension of the pigmentation beyond the vermilion border.
OPERATIVE TREATMENT
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. 20, 21 and 22).
Fig. 21. This operative view shows an incompletely reduced intussusception with the intussusceptum (white arrow) telescoping into the intussuscipiens (black arrow). A pathologic lead point due to Hodgkins lymphoma was found.
OPERATIVE TREATMENT
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. 20, 21 and 22).
Fig. 22. A, Ileoileal intussusception remaining after reduction of the ileum from the colon. B, The ileoileal intussusception has been completely reduced. Note the edema and induration in the wall of the small bowel in both photographs.
OPERATIVE TREATMENT
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 Woods 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.
In a suspected jejunal or ileal intussusception or in the case of postoperative or chronic intussusception, laparoscopy might be a diagnostic tool or the treatment might even be laparoscopically.
A routine diagnosis of a primary intussusception by laparoscopy should be exceptional since ultrasound is perfect.
A primary attempt to reduce an ileo-colic intussusception by laparoscopic instruments does not correspond to the treatment of gentle squeezing the
In the literature a warning is given because laparoscopical instruments may easily damage the vulnerable bowel.
Fig. 23. For laparoscopic reduction of an ileocolic intussusception, the small bowel (intussusceptum) is grasped with an atraumatic bowel clamp. It is best to completely grasp across the entire intussusceptum so that the bowel is not torn when attempting to distract it from the colon. A larger clamp (5 vs. 3 mm) is therefore often helpful. The cecum is then pushed away from the small bowel with an intestinal grasping forceps.
In a suspected jejunal or ileal intussusception or in the case of postoperative or chronic intussusception, laparoscopy might be a diagnostic tool or the treatment might even be laparoscopically.
A routine diagnosis of a primary intussusception by laparoscopy should be exceptional since ultrasound is perfect.
A primary attempt to reduce an ileo-colic intussusception by laparoscopic instruments does not correspond to the treatment of gentle squeezing the
In the literature a warning is given because laparoscopical instruments may easily damage the vulnerable bowel.
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.
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.
RECURRENT INTUSSUSCEPTION
An overriding concern in recurrent intussusception is occult malignancy, although multiple recurrences are not a contraindication to attempted radiologic reduction.
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. 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.
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. 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. 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. Most postoperative intussusceptions are ileoileal and respond to operative reduction without resection.