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Megacolon, hypoganglionosis, and cerebrovascular disease.

INTRODUCTION

Gastrointestinal neuromuscular disease (GINMD) represents an evolving spectrum of gastrointestinal disorders associated with symptoms of chronic gastrointestinal dysmotility in both pediatric and adult populations. (1) While diseases causing such symptoms have been well recognized and previously described among newborn and pediatric populations, the problem is being recognized with increasing frequency in older populations. The pathogenesis of such symptoms in adults remains incompletely characterized, and represents a rapidly growing field of study.

The possible etiologic association between cerebrovascular accident and gastrointestinal dysmotility has recently been described in the Japanese literature. (2-4) This paper reports a patient with a history of cerebrovascular accident presenting with sigmoid volvulus secondary to megacolon with hypoganglionosis. The clinical data lend further credence to the possible etiologic association between the adult dysmotility diseases and cerebrovascular injury.

CASE REPORT

A 58-year-old man presented with a four-day history of increasing abdominal pain and 24 hours of obstipation. The patient had no prior history of gastrointestinal difficulty, but did have a history of hypertension, type II diabetes mellitus, and hyperlipidemia. He suffered a cerebrovascular accident 10 years previously with a residual 4/5 right-sided weakness. Vital signs on presentation were temperature 96.6[degrees] Fahrenheit, heart rate 67 beats per minute, blood pressure 164/98, respiratory rate 20 breaths per minute, and oxygen saturation 95% on room air. On physical examination, the patient's abdomen was rigid, distended and diffusely tender without rebound or guarding. Bowel sounds were hypoactive and a digital rectal exam revealed normal tone with heme-positive stool. Plain films of the abdomen displayed a severely distended sigmoid colon with wall thickening (Figure 1). With the presumed diagnosis of volvulus, it was decided to attempt decompression by placement of a rectal tube, and follow the patient clinically either for resolution of the patient's complaints or worsening signs of obstruction and bowel ischemia. The patient's pain subsequently subsided, but his abdomen remained distended and grossly obstructed. He underwent exploratory laparotomy on inpatient day six, which revealed a massively dilated sigmoid colon with few adhesions and no collections of pus or fluid. A left hemicolectomy was performed and an end-colostomy and Hartmann's pouch created. The resected segment of sigmoid colon measured 37 cm in length and 31 cm in maximum circumference. On gross examination, the colon wall was markedly thickened, rubbery and edematous with no gross masses (Figure 2). Histology revealed severe regional hypogangliosis and crypt architecture distortion (Figure 3). The postoperative recovery was uneventful, and the patient was discharged on post-operative day four with plans to follow-up at the clinic for rectal biopsy.

[FIGURE 1 OMITTED]

Although the overwhelming likely hood is that the marked hypoganglionosis was a consequence of the central nervous system disease, other possible etiologies needed to be considered. The patient was a diabetic, and this metabolic disease is known to cause generalized neuropathy. However, this histologic abnormality has never been described in colectomy specimens from diabetic patients, even those with neuropathy. Colonic dilation and hypoganglionosis has never been observed as a consequence of inactivity, nor has colonic dilation from a myriad of causes been shown to cause hypoganglionosis. The virtual absence of ganglion cells ruled out the possibility that finding represented a dilution within the increased surface area of the dilated colon. Thus, there is no realistic alternative etiology for the hypoganglionosis other than the patient's neurologic disease.

DISCUSSION

Many forms of central nervous system injury are associated with the development of gastrointestinal dysmotility. A recent review reported complaints of chronic constipation in 80% of patients with spinal cord injury, 70% with multiple sclerosis and 10% with Parkinson's disease. (5) The prevalence of gastrointestinal dysmotility among a population with cerebrovascular disease however, has yet to be reported.

The current understanding of adult GINMD stems from histologic and biochemical investigation of diseased tissue, and involves defects of either structural composition or neuronal function. Idiopathic megacolon has been associated with defects in Collagen III and enteric smooth muscle hypertrophy, (6, 7) as well as phenotypic alteration in the regulatory intestinal cells of Cajal (ICC). (8) Decreases in ICC density have also been implicated in other forms of adult GINMD, notably hypogangliosis and slow transit constipation, (9-12) while adult Hirsphrung's is associated with a lack of function or absence of ganglionic cells in colonic myenteric plexus. (13)

[FIGURE 2 OMITTED]

[FIGURE 3 OMITTED]

While the etiology of all adult gastrointestinal neuromuscular diseases remains incompletely described, damage to the intestinal cells of Cajal represents a likely common cause. The intestinal cells of ICC function as enteric pacemakers, constantly active in the promotion of digestive function by activation of gastric and intestinal musculature. Much like the sinoatrial node of the heart, functional regulation of ICCs is achieved by autonomic innervations from the central nervous system. This system of enteric pacing with central autonomic regulation must be fully functional to achieve physiologic gastrointestinal motility, and advances are being made in understanding the connection between disruptions of this complex brain-gut-axis and the resulting gastrointestinal neuromuscular disease. (14) There is a strong correlation between ICC disruption and congenital gastrointestinal neuromuscular disease, (15) and their absence is associated with adult dysmotility in the forms of diabetic gastroenteropathy (16) and enteric neurodegeneration of the ageing. (17)

Further understanding of the brain-gut axis and the functional connection between central autonomic neurons and enteric intestinal cells of Cajal provides a likely explanation for the enteric dysfunction seen with central neurologic disease. The damage suffered in cerebrovascular accidents and other forms of central nervous system injury may disrupt the brain-gut axis, thus disrupting the regulatory control of gastrointestinal pacing with resultant alteration of ICC phenotype and density now associated with many forms of gastrointestinal dysmotility.

Current recommendations for the treatment of persons suffering cerebrovascular accidents focus largely on acute treatment, and fail to recommend measures to promote colonic health and gastrointestinal motility. While there is a clear association between injury to the central nervous system and subsequent gastrointestinal dysmotility, there remains a lack of research in the preventative treatment of this significant condition. Thus, there have been no evidence-based recommendations. However, empiric treatment, including establishment of a gastrointestinal routine, using stool softeners, prokinetic agents, and increasing intake of bulking agents must be encouraged. Current recommendations should be updated to include gastrointestinal prophylaxis as a standard of treatment for patients with cerebrovascular disease, as untreated dysmotility may progress to the type of emergent condition described above.

CONCLUSION

Since cerebrovascular disease is so common, it is mandatory to consider the long-term gastrointestinal implications of the disease. Central nervous system injury-induced dysmotility can be disabling and warrants routine prophylactic use of such agents as stool softeners, bulking agents, and prokinetic medications to prevent dysmotility.

REFERENCES

(1.) Knowles CH. New horizons in the pathogenesis of gastrointestinal neuromuscular disease. J Pediatr Gastroenterol Nutr 2007;45 Suppl 2:S97-102.

(2.) Nishida K, Kawazoye S, Higashijima M, et al. Surgical treatment of secondary chronic colonic pseudo-obstruction. Nippon Ronen Igakkai Zasshi 1996;33:862-866.

(3.) Nishida K, Nojiri I, Higashijima M, et al. Three cases of acquired megacolon in the elderly. Nippon Ronen Igakkai Zasshi 1993;30:497-502.

(4.) Hamano N, Hashizume K, Saeki S, et al. Two elderly cases of megacolon associated with cerebral infarction and diabetes mellitus. Nippon Ronen Igakkai Zasshi 1993;30:626-631.

(5.) Coggrave M, Wiesel PH, Norton C. Management of faecal incontinence and constipation in adults with central neurological diseases. Cochrane Database Syst Rev 2006:CD002115.

(6.) Meier-Ruge WA, Muller-Lobeck H, Stoss F, et al. The pathogenesis of idiopathic megacolon. Eur J Gastroenterol Hepatol 2006;18:1209-1215.

(7.) Gattuso JM, Kamm MA, Talbot JC. Pathology of idiopathic megarectum and megacolon. Gut 1997;41:252-257.

(8.) Adachi Y, Ishii Y, Yoshimoto M, et al. Phenotypic alteration of interstitial cells of Cajal in idiopathic sigmoid megacolon. J Gastroenterol 2008;43:626-631.

(9.) Lee JI, Park H, Kamm MA, et al. Decreased density of interstitial cells of Cajal and neuronal cells in patients with slow-transit constipation and acquired megacolon. J Gastroenterol Hepatol 2005;20:1292-1298.

(10.) Lyford GL, He CL, Soffer E, et al. Pan-colonic decrease in interstitial cells of Cajal in patients with slow transit constipation. Gut 2002;51:496-501.

(11.) Hasler WL. Is constipation caused by a loss of colonic interstitial cells of Cajal? Gastroenterology 2003;125:264-265.

(12.) Ito T, Kimura T, Yagami T, et al. Megacolon in an adult case of hypoganglionosis, a pseudo-Hirschsprung's disease: an autopsy study. Intern Med 2008;47:421-425.

(13.) Miyamoto M, Egami K, Maeda S, et al. Hirschsprung's disease in adults: report of a case and review of the literature. J Nippon Med Sch 2005;72:113-120.

(14.) Wood JD. Neuropathophysiology of functional gastrointestinal disorders. World J Gastroenterol 2007;13:1313-1332.

(15.) Rolle U, Piaseczna-Piotrowska A, Puri P. Interstitial cells of Cajal in the normal gut and in intestinal motility disorders of childhood. Pediatr Surg Int 2007;23:1139-1152.

(16.) Ordog T. Interstitial cells of Cajal in diabetic gastroenteropathy. Neurogastroenterol Motil 2008;20:8-18.

(17.) Camilleri M, Cowen T, Koch TR. Enteric neurodegeneration in ageing. Neurogastroenterol Motil 2008;20:418-429.

Joshua M. Levy, BS; Clifford McGinness, MD; and Bernard M. Jaffe, MD Drs. Jaffe, McGinness, and Mr. Levy are with the Department of Surgery, Tulane University School of Medicine in New Orleans, Louisiana.
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Author:Levy, Joshua M.; McGinness, Clifford; Jaffe, Bernard M.
Publication:The Journal of the Louisiana State Medical Society
Article Type:Case study
Date:Mar 1, 2010
Words:1528
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