Intussusception pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sargun Singh Walia M.B.B.S.[2]
Overview
The exact pathogenesis of intussusception is not fully understood. Intussusception occurs if there is an imbalance between the longitudinal and radial smooth muscle forces of intestine that maintain its normal structure. This imbalance leads to a segment of intestine to invaginate into another segment and cause entero-enteral intussusception. Etiology of intussusception is either idiopathic or pathologic (lead point). In children intussusception occurs mostly due to idiopathic causes. Idiopathic causes include seasonal viral gastroenteritis, rotavirus vaccine, adenovirus infection, hypertrophy of intestinal peyers patches, and bacterial enteritis. In adults intussusception occurs mostly due a lead point. Lead point occurs due to Henoch-Schönlein purpura, cystic fibrosis, Celiac disease, Crohn's disease, Meckel's diverticulum etc.
Pathophysiology
- The exact pathogenesis of intussusception is not fully understood.
- Under normal conditions, a balance between the longitudinal and radial smooth muscle forces maintains the normal structure of intestine.
- Intussusception occurs if there is an imbalance between these forces.
- This imbalance leads to a segment of intestine to invaginate into another segment and cause entero-enteral intussusception.
- The proximal portion is called "intussusceptum" and the distal portion is called "intussuscipien".
- If this telescoping of the intestine continues it can extend till the distal colon or sigmoid colon or even through the anus.
- Nitric Oxide Hypothesis[1]
- There is nitrergic innervation in the myentric plexus.
- These receptors are in greater density at Ileocecal valve(ICV) than on terminal ileum and proximal large ileum. in younger age.
- Nitric oxide(NO) acts on these receptors.
- NO is a inhibitory neurotransmitter of the enteric nervous system. It acts by causing relaxation of smooth muscles.
- Overproduction of nitric oxide (NO) occurs during inflammation.
- This leads to relaxation of the ICV and causes altered intestinal motility.
- This can lead to ileocecal intussusception.
- Intussusception is the most common abdominal emergency in children < 2 years of age.
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| Idiopathic- no lead point | Pathologic- Lead point | ||||||||||||||||||||||||||||||||||||||||||
- Uncommon in adults but when it occurs the most common cause is pathological due to lead point.
If the mesentery invaginates along with the intestine it may lead to lymphatic and venous congestion and cause intestinal edema. If not treated, it eventually leads to ischemia which further progress to peritonitis or even perforation.
Etiology
- Idiopathic:- It is the most common cause of intussusception in children and accounts for about 75% of all cases. Any specific disease trigger point or lead point cannot be recognized. It can be further divided among various causes:
- Viral causes
- Seasonal viral gastro-enteritis.[2]
- Rotavirus Vaccine:- Intussusception is known to be caused by certain types of Rotavirus vaccines. An earlier iteration of the vaccine known as the Rotashield was taken out of the market because it was associated with an increased number of cases of intussusception. If there occurs any case of intussusception after vaccination with Rotavirus vaccine, then it should be reported to the Vaccine Adverse Event Reporting System (VAERS) by the providers.[3]
- Adenovirus:- There is a strong association with adenovirus infection. A case control study conducted in Vietnam and Australia shows that Specie C of adenovirus is a strong predictor of intussusception in children.[4]
- Viral infections stimulate the lymphatic tissue in the intestine and lead to the hypertrophy of the Peyers patches in the terminal ileum and cause the formation of a lead point. This may act as an obstruction and lead to intussusception. Due to this association with hyperplasia, use of glucocorticoids may be used to prevent recurrence but it is not encouraged.
- Bacterial enteritis:- A retrospective cohort study done in children aged 0-5 years shows a strong association between bacterial enteritis and intussusception.[5] There is an increased risk of development of intussusception with Shigella, Salmonella, E. coli and Campylobacter infections.
- Viral causes
- Lead Point:- Lead point can be defined as any lesion that gets trapped in the intestine by peristaltic forces, which then gets dragged into the distal segment of the intestine leading to the formation of intussusception. Lead point accounts for 25% cases of iintussusception in childhood and almost 95% of cases of intussusception seen in adults. Lead point can be caused due to various pathological reasons which are as follows:
- Henoch-Schönlein purpura (HSP):- It is is an IgA mediated inflammatory disorder which causes inflammation and bleeding of the small blood vessels in skin, intestine, joints, and kidneys. HSP is most commonly seen in children less than 7 years of age. In HSP, hematoma formation in the small intestine may act as a lead point. Intussusception mostly occur once the abdominal pain subsides.[6][7]. Intussusception in HSP mostly originates in the ileum or jejunum , and more than one-half of cases are confined to the small bowel. In contrast to idiopathic intussusception, where the majority (80%-90%) are ileo-colic.
- Cystic Fibrosis:- Intussusception is one of the complication of cystic fibrosis. In this thick inspissated/impacted stool acts as a lead point.[8][9][10]
- Celiac disease:- Recent studies show that celiac disease is associated with increased risk of intussusception. Celiac disease may lead to small bowel intussusception by causing dysmotility and excessive secretions in bowel wall or by causing small bowel weakness.[11][12][13]
- Crohns disease:- Crohns disease is a chronic granulomatous inflammatory disease which may lead to intussusception due to inflammation and stricture formation in the intestine. [14][15]
- Meckel's diverticulum
- Polyp
- Duplication Cyst
- Lymphomas
- Areas of reactive lymphoid hyperplasia
- Post-operative :- Small bowel intussusception can occur in postoperative cases. Most commonly seen are jejuno- jejunal or ileo-ileal cases. Most cases are seen after open procedures but can also be seen after non abdominal procedures. It can occur due to uncoordinated peristaltic activity and/or traction from sutures or devices such as a gastrojejunal feeding tube.[16][17][18][19]
References
- ↑ Cserni T, Paran S, Puri P (2007). "New hypothesis on the pathogenesis of ileocecal intussusception". J. Pediatr. Surg. 42 (9): 1515–9. doi:10.1016/j.jpedsurg.2007.04.025. PMID 17848241.
- ↑ Buettcher M, Baer G, Bonhoeffer J, Schaad UB, Heininger U (2007). "Three-year surveillance of intussusception in children in Switzerland". Pediatrics. 120 (3): 473–80. doi:10.1542/peds.2007-0035. PMID 17766518.
- ↑ Shimabukuro TT, Nguyen M, Martin D, DeStefano F (2015). "Safety monitoring in the Vaccine Adverse Event Reporting System (VAERS)". Vaccine. 33 (36): 4398–405. doi:10.1016/j.vaccine.2015.07.035. PMC 4632204. PMID 26209838.
- ↑ Bines JE, Liem NT, Justice FA, Son TN, Kirkwood CD, de Campo M, Barnett P, Bishop RF, Robins-Browne R, Carlin JB (2006). "Risk factors for intussusception in infants in Vietnam and Australia: adenovirus implicated, but not rotavirus". J. Pediatr. 149 (4): 452–60. doi:10.1016/j.jpeds.2006.04.010. PMID 17011313.
- ↑ Nylund CM, Denson LA, Noel JM (2010). "Bacterial enteritis as a risk factor for childhood intussusception: a retrospective cohort study". J. Pediatr. 156 (5): 761–5. doi:10.1016/j.jpeds.2009.11.026. PMID 20138300.
- ↑ Ebert EC (2008). "Gastrointestinal manifestations of Henoch-Schonlein Purpura". Dig. Dis. Sci. 53 (8): 2011–9. doi:10.1007/s10620-007-0147-0. PMID 18351468.
- ↑ Little KJ, Danzl DF (1991). "Intussusception associated with Henoch-Schonlein purpura". J Emerg Med. 9 Suppl 1: 29–32. PMID 1955678.
- ↑ Holmes M, Murphy V, Taylor M, Denham B (1991). "Intussusception in cystic fibrosis". Arch. Dis. Child. 66 (6): 726–7. PMC 1793149. PMID 2053797.
- ↑ Webb AK, Khan A (1989). "Chronic intussusception in a young adult with cystic fibrosis". J R Soc Med. 82 Suppl 16: 47–8. PMC 1291920. PMID 2657054.
- ↑ Gross K, Desanto A, Grosfeld JL, West KW, Eigen H (1985). "Intra-abdominal complications of cystic fibrosis". J. Pediatr. Surg. 20 (4): 431–5. PMID 4045671.
- ↑ Ludvigsson JF, Nordenskjöld A, Murray JA, Olén O (2013). "A large nationwide population-based case-control study of the association between intussusception and later celiac disease". BMC Gastroenterol. 13: 89. doi:10.1186/1471-230X-13-89. PMC 3661363. PMID 23679928.
- ↑ Martinez G, Israel NR, White JJ (2001). "Celiac disease presenting as entero-enteral intussusception". Pediatr. Surg. Int. 17 (1): 68–70. doi:10.1007/s003830000395. PMID 11294274.
- ↑ Mushtaq N, Marven S, Walker J, Puntis JW, Rudolf M, Stringer MD (1999). "Small bowel intussusception in celiac disease". J. Pediatr. Surg. 34 (12): 1833–5. PMID 10626866.
- ↑ López-Tomassetti Fernández EM, Lorenzo Rocha N, Arteaga González I, Carrillo Pallarés A (2006). "Ileoileal intussusception as initial manifestation of Crohn's disease". Mcgill J Med. 9 (1): 34–7. PMC 2687895. PMID 19529808.
- ↑ Cohen DM, Conard FU, Treem WR, Hyams JS (1992). "Jejunojejunal intussusception in Crohn's disease". J. Pediatr. Gastroenterol. Nutr. 14 (1): 101–3. PMID 1573498.
- ↑ Ein SH, Ferguson JM (1982). "Intussusception--the forgotten postoperative obstruction". Arch. Dis. Child. 57 (10): 788–90. PMC 1627910. PMID 7138069.
- ↑ Linke F, Eble F, Berger S (1998). "Postoperative intussusception in childhood". Pediatr. Surg. Int. 14 (3): 175–7. doi:10.1007/s003830050479. PMID 9880741.
- ↑ Kidd J, Jackson R, Wagner CW, Smith SD (2000). "Intussusception following the Ladd procedure". Arch Surg. 135 (6): 713–5. PMID 10843370.
- ↑ Klein JD, Turner CG, Kamran SC, Yu AY, Ferrari L, Zurakowski D, Fauza DO (2013). "Pediatric postoperative intussusception in the minimally invasive surgery era: a 13-year, single center experience". J. Am. Coll. Surg. 216 (6): 1089–93. doi:10.1016/j.jamcollsurg.2013.01.059. PMID 23571141.