Intussusception in young children

The description of the intussusception in young children covers all aspects of the surgical procedure used for the management of intussusception. Operating room set up, position of patient and equipment, instruments used are thoroughly described. The technical key steps of the surgical procedure are presented in a step by step way: radiographic guidance, reduction, laparotomic approaches. Consequently, this operating technique is well standardized for the management of this condition.

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Intussusception   in   young   children

Authors
F Becmeur, D Christmann, I Kauffmann
Abstract
The description of the intussusception in young children covers all aspects of the surgical procedure used for the management of intussusception.
Operating room set up, position of patient and equipment, instruments used are thoroughly described. The technical key steps of the surgical procedure are presented in a step by step way: radiographic guidance, reduction, laparotomic approaches.
Consequently, this operating technique is well standardized for the management of this condition.
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2001-10
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WeBSurg.com, Oct 2001;1(10).
URL: http://www.websurg.com/doi-ot02en153.htm

Intussusception   in   young   children

1. Introduction
Intussusception in young children is a medical emergency. It should be suspected if a patient presents with a painful abdomen.
Interventional radiology proves to be an effective treatment in more than 90% of patients: surgery is usually performed in cases of contraindication or failure only. Postoperative hospital stay is usually short because recurrences occur early.
The first sonographic descriptions of intussusception were reported by Stanley et al. (1997).
2. Pathology
• Intussusception
Intussusception is the telescoping of one segment of bowel (called the intussusceptum) into a more distal segment (or the intussuscipiens), following the direction of peristalsis.
• Anatomical characteristics
The anatomical characteristics of intussusception are the following:
- during interventional radiology, reduction of an intussusception is performed by forcing back the head of the intussusceptum (1) with the instillation of gas or fluid. Surgical reduction is performed either with a grasper or with the hand.
- a sausage-shaped intraluminal mass (2) may be felt in the abdomen during physical examination. It is composed of an internal cylinder (intussusceptum) passing into the lumen of an external cylinder (intussuscipiens). The distal part of the internal cylinder is the head of the intussusceptum (3).
- the area of constriction where the intussusceptum and its mesentery penetrate the intussuscipiens is called the neck of the intussusception (4), which results in venous and lymphatic stasis, and possibly arterial stasis.
• Classification
Four types of intussusception have been identified. The first part of the name refers to the internal cylinder, the second part refers to an intermediate segment that may be involved, and the third part refers to the external cylinder.

Ileo-colic (transvalvular) (1)
The ileum is invaginated in the colon through the ileocecal valve (Bauhin’s valve). The rigidity of the neck of the intussusception induces early ischemia.

Ileo-cecal-colic (2)
The ileum, cecum and appendix are invaginated into the ascending colon. Intussusception of the cecum occurs in cases of failure of fixation.

Ileo-ileal (3)
The invagination of the ileum within itself is typical of a postoperative intussusception.

Colo-colic (4)
Colo-colic intussusception is most frequently due to a tumor. This anatomical form is relatively well tolerated due to the low degree of rigidity of the intussusception neck.
• Pathophysiology
The intussusceptum is propelled distally through peristalsis. The leading point of the intussusceptum (generally the ileum or cecum in infants) can progress to the sigmoid colon or even to the anus in cases of anal prolapse.
The following elements of the mesentery are constricted:
- the autonomic nerves, hence the pallor and the first fits of vomiting and nausea;
- the veins and lymph nodes, which worsen the edema (often pre-existing in idiopathic forms of intussusception);
- the arteries, which may lead to necrosis and perforation of the intestine.
3. General principles
Reduction of intussusception is most often performed under radiographic guidance. Surgery is mandatory in cases of:
- suspicious/disturbing clinical signs;
- signs of peritoneal irritation;
- frank intestinal occlusion;
- failure of reduction by interventional radiology;
- postoperative intussusception (Pierro et al., 1993; Chung et al., 1994).
Surgery can be performed either via laparotomy or laparoscopy (Schier, 1997; Poddoubnyi et al., 1998; Galatioto, 1999; Cuckow et al., 1996; Lai et al., 2000): we will concentrate on the laparoscopic approach.
4. Etiology
Intussusception in infants is usually idiopathic, ie, its cause is unknown. Predisposing factors may include ENT infections, gastroenteritis and other causes of mesenteric adenitis.
Intussusception may be secondary to a malformation, a tumor or any other organic lesion due to peristalsis. In such cases, the affected intestine is invaginated into the immediately distal segment of bowel (Ong and Beasley, 1990). Meckel\'s diverticulum is involved in half of cases; intestinal duplication cysts, tumors (polyps, Burkitt\'s lymphomas and others) and a hyperplasia of Peyer\'s patches due to anaphylactoid purpura (Henoch-Schoenlein purpura) are less frequently involved.
In the case of cystic fibrosis, the abnormal thickness and viscosity of intestinal contents can lead to intussusception if incorrectly treated.
Postoperative forms of intussusception have become rare following major abdominal surgery (generally performed via a retroperitoneal approach) or chemotherapy (methotrexate).
5. Clinical signs
• Clinical study
Stringer et al. (1992) analyzed 33 deaths due to intussusception that occurred in England and Scotland between 1984 and 1989; the symptoms reported are listed in the table on the left. The seriousness of the condition was directly due to a delay in diagnosis or therapeutic management. Abdominal pain and vomiting in infants should always be considered possible signs of intussusception.
• Symptomatology
Symptoms associated with intussusception constitute a classic triad: paroxysmal and intermittent abdominal pain, vomiting, and rectal bleeding. However, symptoms are not always obvious:
- symptoms of abdominal pain are difficult to interpret at such a young age. Screams with restlessness and flexion of the inferior limbs are common. Episodic pallor that interrupts the child’s play occurs in 45% of cases (Becmeur et al., 1994);
- refusal to eat or nausea often occurs, rather than vomiting or bilious vomiting (rare);
- rectal bleeding (traces of red blood found in the stools or during non-traumatic rectal examination) is rare. Brownish-colored and foul-smelling rectal bleeding are signs of pre-existing ischemia of the digestive tract.
• Physical examination
It is often difficult to examine a newborn who is both restless and experiencing pain. The abdominal mass can be palpated in 25% to 33% of emergency cases. Between 10% and 30% of intestinal intussusception cases show no clear abdominal symptoms. The clinical picture is often misleading (Stringer et al., 1992).
• Clinical presentations
Diarrheal presentations are frequent. Gastroenteritis in infants begins with vomiting and fever, followed by diarrhea. Intussusception should be suspected immediately if the child continues to experience vomiting during the diarrheal phase. Gastroenteritis may indeed be a predisposing factor to intussusception.
Neurological presentations have been reported, including possible episodes of convulsions or lethargy that requires electroencephalography or even a brain scan (Goetting et al., 1990).
Tumoral presentations are characterized by an abdominal mass that can be perceived during abdominal examination and palpated in children who do not seem to be experiencing pain.
Obstructive presentations trigger an emergency assessment and are therefore easily diagnosed.
Postoperative presentations following major abdominal surgery are rare but difficult to diagnose. They should be systematically suspected in infants who experience secondary mechanical obstruction 3 to 5 days after surgery.
• Clinical distinctions by age
Some rare cases of antenatal intussusception have been reported leading to neonatal small bowel atresia and obstruction (Adejuyigbe and Odesanmi, 1990).
Intussusception is exceptionally rare in newborns (up to the 29th day included) and always takes the form of acute intestinal obstruction. It may be confused with necrotizing enterocolitis, but radiology and ultrasonography allow a precise diagnosis. Intussusception requires emergency surgery, while necrotizing enterocolitis requires surgery in only 50% of cases (Gorgen-Pauly et al., 1999).
Children over 36 months of age have a more insidious clinical picture, sometimes showing signs of chronic infection. An organic cause is often suspected, although an intussusception that is reduced by hydrostatic enema seldom has an organic cause (Ein and Ferguson, 1982). Hydrostatic reduction is not possible in patients where organic lesions are identified as the cause of intussusception: surgery becomes necessary in order to reduce the intussusception and to find and treat the causal lesion.
6. Radiological diagnosis
• Radiological diagnosis
Radiological diagnosis is based on a plain film of the abdomen, followed by an abdominal ultrasound. If the ultrasound examination is negative, radiography can be performed again to search for other causes (eg, gastroenteritis).
• Abdominal radiography
• Ratcliffe study
Plain film radiography (PFR) is not sufficient to clearly diagnose or rule out intestinal intussusception because only 78% of abdominal images (141 of 180 patients) enable a diagnosis (Ratcliffe et al., 1992). Ten percent of patients show no sign of intussusception (Lee et al., 1994). However, the absence of specific signs does not rule out the diagnosis of intussusception.

Diagnostic value of air contrast radiography

Conclusion: While air contrast enema may successfully reduce intussusception, it is not 100% accurate as a diagnostic tool (Ratcliffe et al., 1992).
• Suggestive signs
Suggestive radiological signs of intussusception (Eklof and Hartelius, 1980) are observed in 1out of 3 cases. They include:
1. Insufficient amount of feces and disappearance of the ground-glass-like aspect of the cecum;
2. Air-fluid levels suggestive of intestinal obstruction;
3. Presence of a mass-like opacity corresponding to the intraluminal mass;
4. Absence of bowel gas proximal to the intussusception with a crescent sign;
5. Displacement of small bowel loops into the right upper quadrant;
6. Presence of intraperitoneal fluid that separates the small bowel loops; presence of a crescent-shaped pocket of air under the diaphragm in cases of digestive tract perforation.
• Abdominal sonography
Sonography enables the positive diagnosis of intussusception (Shanbhogue et al., 1994; Pracros et al. 1987). In cases of false-negative results, clinical signs and a history suggestive of intestinal intussusception should lead to enema examination to establish a clear diagnosis.
Sonography results show an intussusception mass resembling:
(1) A target or donut measuring 4 cm in transversal section;
(2) A sandwich with adenopathies in longitudinal section.
Sonography can also assess the seriousness of the intussusception by looking for intraperitoneal fluid (3) and checking the vascularity of the intraluminal mass (Doppler ultrasound) (4). Furthermore, sonography is used to check for tumors or a malformation that may have caused the intussusception: irregular and thickened walls in cases of Burkitt’s lymphoma (5), and a double target aspect in cases of Meckel’s diverticulum (6).
It is also useful to assess the possibilities of reducing the intussusception with interventional radiology.
• Contraindications
Abundant intraperitoneal fluid (1) and decreased Doppler flow (2) in the intussusceptum are signs of serious disease that contraindicate hydrostatic reduction. In such cases, surgical reduction should be immediately performed.
The following are relative contraindications for interventional radiology:
- delay in diagnosis;
- severe dehydration;
- clinical and radiological signs of frank small bowel obstruction or perforation.
A combination of several of these elements increases the gravity of the clinical picture and leads to a contraindication for non-surgical management.
Age is not a contraindication.
7. Radiographic guidance
• Interventional radiology
Interventional radiology is used to treat intussusception without resorting to surgery in the majority of cases (Bramson and Blickman, 1992). Non-surgical treatments include barium enema, air enema and saline enema (Hadidi and El Shal, 1999).
Depending on the type of enema used, either sonography or radiography is performed to assess the outcome of the treatment.
Children requiring emergency treatment must be rehydrated, kept warm and calm (parental presence and analgesics) prior to any reduction attempt. The surgeon must be present in the radiology room. Water pressure should never exceed 120 cm, whatever the product used (Bramson and Blickman, 1992; Schmit et al., 1999).
The antiperistaltic force of an injection of air or liquid (barium or saline) reduces the intraluminal mass. This treatment is performed either under radiographic guidance for barium or air injection, or under sonographic guidance for saline, water-soluble or air injection (Shehata et al., 2000).
• Barium enema
The lead point of the intussusceptum is forced back under radiographic guidance with the flow of a barium enema. Barium enema reduction follows well-established rules:
- the enema must fill between 20 and 30 cm of the small bowel, which should no longer show any radiographic sign of ileo-ileal intussusception;
- the cecum must be correctly positioned when the barium enema is over;
- cecal integrity must be preserved;
- the radiographic image obtained after evacuation of the barium must not show signs of intussusceptum.

Advantages
The procedure is well known by most pediatric radiology teams and can be performed anywhere without specific instrumentation.

Disadvantages
The materials used for barium enema are uncomfortable. The procedure is irradiating and messy. The problem of differential diagnosis with fecal analyses has not yet been resolved. The ileocecal valve is not always permeable in a retrograde fashion, even in healthy children.
Gastrointestinal perforations occur on the site of intestinal necrosis and not in proximal areas of dilatation.
The success rate of barium enema is not very high (Chan et al., 1997: 55% of 23 children).
• Air enema
The lead point of the intussusception is visualized by radiography and forced back with a stream of pressured air (Palder et al., 1991).

Advantages
The procedure is rapid, effective (Gorenstein et al., 1998: 91% success rate; Gu et al., 2000: 95% success rate) and is not messy (may be performed as often as necessary with no specific installation requirements).

Disadvantages
Air enema is nevertheless irradiating and uncomfortable for the child. The procedure also requires air insufflation devices, including a feedback system of the pressure exerted in the digestive tract lumen, making it possible to set the insufflator to maximal pressure. The risks of air enema include perforation (more than with barium enema) and a difficult subsequent ultrasound surveillance (Shiels et al., 1993; Daneman et al., 1995).
• Saline enema
The lead point of the intussusceptum is forced back under sonographic guidance with saline (Wood et al., 1992; Riebel et al., 1993; Choi et al., 1994; Rohrschneider and Troger, 1995; Chan et al., 1997).

Advantages
This procedure is non-irradiating, rapid, safe, comfortable and effective (95% success rate in our series; Wang and Liu, 1988: 95%; Wood et al., 1992: 85%; Rohrschneider and Troger, 1995: 71%; Chan et al., 1997: 91%).
Intussusception reduction can be performed under direct sonographic guidance (Rohrschneider and Troger, 1995).
Short-term follow-up using ultrasound can be repeated as often as necessary.

Disadvantages
Interventional sonography requires a qualified and experienced radiologist.
• Reduction failure
The predictive factors for failure of hydrostatic reduction of intussusception include:
- abundant intraperitoneal fluid,
- reduced flow on Doppler imaging in the intussusceptum (true for all procedures concerned).
Surgery may be needed if interventional radiology is not successful.

The diagnostic criteria for gastrointestinal perforation are:
- clinical and radiological examination dominated by a pneumoperitoneum revealing perforation,
- a severe state of shock or frank intestinal obstruction requiring resuscitation: caution is necessary and in some cases immediate surgery is indicated.
Children presenting these signs should be immediately referred to surgery.
8. Operating room set-up
• Patient
The operation is performed under general anesthesia. The surgeon, the laparoscopic unit and the lead point of the intussusceptum should be on the same axis.
The child is placed in a supine position with the arms alongside the body, on the left edge of the operating table.
The child has an IV in place and is intubated and ventilated. A nasogastric aspiration tube is used during general anesthesia. It is mandatory to place an esophageal thermal catheter, a percutaneous measure of oxygen saturation and 3 electrodes for cardiac monitoring. A urinary catheter is not mandatory, although it is often useful for widening the peritoneal space.
• Team
1. The surgeon stands on the patient’s left side at the level of the abdomen.
2. The assistant stands on the surgeon’s right, close to the patient’s head.
3. The scrub nurse stands on the surgeon’s left, close to the patient’s feet.
• Equipment
The laparoscopic unit is placed on the patient’s right side, opposite the surgeon. Insufflation pressure should not exceed 8 mm Hg.
1. Laparoscopic unit
2. Anesthetic unit
3. Instrument table
9. Trocars/instruments
• Principles
Three trocars are required for this procedure. They must be placed according to the triangulation technique in relation to the optical trocar.
• Optical trocar
A: The 5 mm optical trocar is inserted in the umbilicus. The umbilical region is suspended to the trocar with a string.
• Operating trocars
• Trocars B and C
B: The first 2-3 mm operating trocar is inserted in the left iliac fossa.
C: The second 2/3 mm operating trocar is inserted in the left upper quadrant.
The 2 operating trocars B and C are introduced through the internal tip of the optical trocar.
• Variation
Alternately, the operative trocars can be inserted in the right and left iliac fossae. However, this variation is rather uncomfortable for the surgeon since the most difficult and essential operative time is the reduction of the residual mass in the right iliac fossa.
• Instruments
3 mm instruments are used:
1. Optical device
2. Atraumatic graspers (2)
3. Intestinal clamp or long atraumatic grasper
4. Suction-irrigation device
10. Reduction
• Reduction
• Principles
Reduction is performed in 2 operative steps:
- freeing of the 2 cylinders using pneumodissection,
- reduction of the lead point of the intussusceptum.
• Pneumodissection
Freeing of the intussusceptum from the intussuscipiens is performed at the level of the neck of the intussusception. The spontaneous infiltration of CO2 into the neck of the intussusception suppresses the cupping effect that could interfere with the reduction.
• Reduction of lead point
The lead point of the intussusceptum is reduced either with an intestinal clamp or with an atraumatic grasper. Traction is maintained on the bowel distal to the intussusception.
Gentle traction on the intussusceptum can help finalize the reduction.
• Intestinal tear
Strong resistance on the distal bowel while exerting a gentle traction on the proximal bowel may cause an intestinal tear.
• Difficult cases
Laparoscopic manoeuvres may be extremely difficult in certain occlusive forms of intussusception when major distension of the small bowel leads to reduction of the operative space.
In cases of Meckel's diverticulum, tumor or a long-standing, neglected intussusception, reduction may be almost impossible, therefore requiring conversion to laparotomy.
• Intestinal resection
The following elements are difficult to assess:
- viability of the reduced bowel;
- short-term changes in the condition of the bowel;
- risks of necrosis and perforation secondary to bowel separation.
It is often difficult to differentiate a zone of necrosis from a parietal hematoma. There is major difficulty in cases of purpura for which conversion must be performed through a low horizontal small incision in the lower abdomen, guided by laparoscopy.
11. Laparotomy/approaches
A horizontal skin incision is made at the umbilicus or slightly below. The best approach is then to perform a Jalaguier-Buck’s incision (as illustrated) since a McBurney’s incision may be insufficient (in cases of distal progression of the lead point of the intussusceptum) or too low (in cases of ascension of the cecum).
The Jalaguier-Buck approach allows for intussusception reduction and intestinal resection if necessary. Systematic appendectomy is almost routinely performed. The recurrence rate is very low (2%). There is a risk of obstruction caused by a band: 5% of cases of obstruction due to a band have been treated in our department since 1972 (Becmeur et al., 1997).
12. Postoperative management
After interventional radiology
The child can resume food intake as soon as hydrostatic reduction is achieved. If the child is particularly dehydrated and tired, intravenous fluids are maintained for a few hours following the procedure.
If clinical exams and sonographic findings are satisfactory, the child can be discharged within 24 to 48 hours after the procedure.
Immediate recurrences occur in about 5% of cases.
In order to better understand the etiology of intussusception, clinical evaluation and history are assessed during the child’s hospitalization.

After laparoscopic surgery
If no intestinal resection has been performed, the child can progressively resume food intake the day following the operation and is maintained on intravenous fluids to achieve hydro-electric rebalancing. The child may be discharged within 48 hours of admission.
If intestinal resection was performed, the child can resume food intake as soon as intestinal transit is restored. Analgesics must be prescribed for the first postoperative days.
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