Clinical diagnosis
Case 75
【Progress】
She was diagnosed bacteremia associated with chronic intestinal pseudo-obstruction and then, she got venous drip infusion of antibiotics, albumin and diuretics. Pseudo-occlusion was considered to being progressive to cover the entire bowel. She was scheduled to place a central venous port because of the difficult ingestion of the regular meal.
【Discussion】
Intestinal pseudo-obstruction is a disorder of food-content transportation in digestive organs. Intestinal pseudo-obstruction is categorized in acute and chronic types (1-4). Acute intestinal pseudo-obstruction (AIPO) called Ogilvie syndrome occurs mostly after surgical operation probably due to auto-nerve disorder (2, 3). Meanwhile, CIPO is used for movement disorder without mechanical obstruction and nerve plexus abnormality. CIPO occurs at any age (1, 4). CIPO gradually induces symptoms of gastroparesis, megacolon and paralytic ileus. As time pass, the disorder lesion sprawls and seriously worsens (Fig. 5).
CIPO is caused by myogenic, neurogenic, or mesenchymal origin. Bowel movement is controlled with smooth muscle layers composed of circular muscle and longitudinal muscle and entero-nervous system composed of Auerbach (muscular) plexus and Meissner (submucosal) plexus. Recent studies show Cajal cells which are mainly present between circular muscle cell and longitudinal muscle cell produce pace maker activity such as heart (Fig. 6, 7) (5, 6). Electric activity produced by a single Cajal cell spreads to a single muscle layer cell. Cajal cells drive spontaneous rhythmic motility. Intracellular Ca2+ handling has a key role in the generation of intrinsic electrical rhythmicity in the gut pacemaker as well as in the cardiac pacemaker for intrinsic beating (Fig. 8, 9) (5, 6). Cajal cells play a leading role of enteric nervous system.
Congenital and acquired abnormality of digestive organ movement is considered to result from disorders of Cajal cells. For examples, Cajal cells were found to be absent in the hypertrophic circular muscle in patients with gastric pylorus hypertrophy, and damages to Cajal cells in diabetic mellitus causes dysmotility of digestive organ (1,4,7). AIPO and CIPO are considered to be caused by dysfunction of Cajal cells. Therefore, therapeutic targeting of intracellular Ca2+ handling might be a promising approach in order to improve AIPO and CIPO (5).
It is now believed; CIPO is chronically and gradually progressive based on genetic backgrounds of bowel movement disorder and CIPO symptoms pass long asymptomatic (honeymoon) period that daily life is possible by compensatory mechanism, although chronic constipation is present: however, when stress builds up to bowel like surgical resection, compensatory mechanism breaks down, inducing manifestation of CIPO symptoms.It enters vice cycle of transport disorder of food content, megacolon, absorption disorder, infectious enteritis/ colitis and sepsis (8-10).
Nakajima J, et al accumulated ninety two patients with CIPO in Japan during 2001 to 2010 (9). Patients symptoms were least improving and average duration suffering symptoms were 14.6 years (9). Approximately a half of the cases was required by depression treatment of gastrostomy or bowelostomy and thirty percent of the cases received intravenous or intrabowel feeding (8-10).
In our case, she underwent surgical appendectomy in the past followed by partial resection of bowel because of repeated ileus, inducing bowel paresis and megacolon. She suffers from persistent constipation and repeated vomiting. She has received central venous port placement for taking enough calories with less ingestion of food.
In addition, recent studies reveal that gastrointestinal stromal tumor (GIST) is considered to come from interstitial cells of Cajal (11).
【Summary】
We present an eighty two-year-old female suffering from persistent vomiting and constipation. Abdomen radiograph and CT showed megacolon and dilated small bowel with no mechanical obstruction, that was diagnosed by chronic intestinal pseudo-obstruction (CIPO). Her transportation disorder of food content initiated from surgical resection and persistent for more than seven years and gradually worsened, needing a central venous port. We should keep in mind that interstitial cells of Cajal drive spontaneous rhythmic motility with intracellular Ca2+ handling and play a role of gut pacemaker like heart pace maker. Although treatment for CIPO is yet to be found, more clarification of transportation mechanism of digestive organ might lead to overcome this difficult disease.
【References】
1.Coulie B, Camilleri M. Intestinal pseudo-obstruction. Annu Rev Med. 1999. 50:37-55. [Medline].
2.Alwan MH, van Rij AM. Acute colonic pseudo-obstruction. Aust N Z J Surg. 1998 Feb. 68(2):129-32. [Medline].
3.Ogilvie H. Large-intestine colic due to sympathetic deprivation; a new clinical syndrome. Br Med J. 1948 Oct 9. 2(4579):671-3. [Medline].
4.Lacy BE, et al. Diagnosis, treatment and nutritional management of chronic intestinal pseudo-obstruction. Practical Gastroenterlogy. 2009;9-24.
5.Takaki M. Gut pacemaker cells: the interstitial cells of Cajal (ICC). J Smooth Muscle Res. 2003 Oct;39(5):137-61.
6.Al-Shboul OA. The Importance of Interstitial Cells of Cajal in the Gastrointestinal Tract. Saudi J Gastroenterol. 2013 Jan-Feb; 19(1): 3–15. doi: 10.4103/1319-3767.105909 PMCID: PMC3603487
7.Struijs MC, et al. Absence of the interstitial cells of Cajal in a child with chronic pseudoobstruction. J Pediatr Surg. 2008 Dec;43(12):e25-9. doi: 10.1016/j.jpedsurg.2008.09.017.
8.Ohkubo H, et al. An epidemiologic survey of chronic intestinal pseudo-obstruction and evaluation of the newly proposed diagnostic criteria. Digestion. 2012. 86(1):12-9.
9.Nakajima J. http://www. nanbyou.or. jp/entry/2084
10.Lindberg G, Iwarzon M, Tornblom H. Clinical features and long-term survival in chronic intestinal pseudo-obstruction and enteric dysmotility. Scand J Gastroenterol. 2009;44:692-699. http://www.ncbi.nlm.nih.gov/pubmed/19308797
11.Min KW, et al. Interstitial cells of Cajal (ICC) and gastrointestinal stromal tumor (GIST): facts, speculations, and myths. J Cell Mol Med. 2006 Oct-Dec;10(4):995-1013.
2017.10.11
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