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 Table of Contents  
Year : 2017  |  Volume : 3  |  Issue : 1  |  Page : 194-196

Giant megacolon: An unusual surgical emergency

1 Department of Surgery, St. Luke's University Health Network, Bethlehem, PA, USA
2 Temple University School of Medicine, St. Luke's University Hospital Campus, Bethlehem, PA, USA
3 Department of Surgery, Division of Acute and Adult Trauma Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA

Date of Web Publication7-Jul-2017

Correspondence Address:
Stanislaw P Stawicki
Department of Surgery, St. Luke's University Health Network, 801 Ostrum Street, Bethlehem, PA 18015
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2455-5568.209861

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Giant megacolon (GMC), an advanced form of chronic megacolon, is an uncommon diagnosis that may evolve into a surgical emergency. Here, we demonstrate a case of an elderly male who presented to our hospital with complaints of acute abdominal distention and diffuse abdominal pain. He was emergently taken to the operating room where necrotic cecum was found. The patient underwent total abdominal colectomy with end ileostomy. Radiographic and operative images of GMC are presented. The authors also discuss the pathophysiology and clinical management of this surgical condition.
The following core competencies are addressed in this article: Medical knowledge, Patient care.

Keywords: Colonic dilatation, colonic motility disorder, giant megacolon, megacolon, surgical emergency

How to cite this article:
Wojda TR, Yanagawa F, Wallner A, Hillman Terzian W T, Smith EA, Stawicki SP, Cipolla J. Giant megacolon: An unusual surgical emergency. Int J Acad Med 2017;3:194-6

How to cite this URL:
Wojda TR, Yanagawa F, Wallner A, Hillman Terzian W T, Smith EA, Stawicki SP, Cipolla J. Giant megacolon: An unusual surgical emergency. Int J Acad Med [serial online] 2017 [cited 2022 Dec 8];3:194-6. Available from: https://www.ijam-web.org/text.asp?2017/3/1/194/209861

  Introduction Top

Megacolon can be defined as colonic dilatation >6.0 cm in one or more colonic segments.[1],[2] Some authors have suggested that measured diameter of >6.5 cm at the transverse and rectosigmoid locations,[3] >8 cm for the ascending colon,[4],[5] and >12 for the cecum [4],[5] may provide a more precise categorization.[3],[4],[5],[6] Megacolon can present either acutely or as a chronic condition.[7] This heterogeneous entity can be broadly grouped into Hirschsprung's (e.g., congenital)[8],[9] or non-Hirschsprung's (e.g., acquired)[3],[10],[11] megacolon types. The latter form is the focus of the current report.

The genesis of chronic megacolon is poorly understood and involves pathologic changes in the intestinal connective tissue, smooth muscle cells, and the enteric nervous system.[10],[12],[13] Clinical hallmarks of chronic megacolon include abdominal pain, bloating, and constipation.[10] Among chronically institutionalized patients, megacolon can progress to “giant megacolon” (GMC), often accompanied by the clinical picture of “large-bowel obstruction with obstipation.”[14] Herein, we describe a case of GMC with acute presentation requiring the performance of emergency total abdominal colectomy with end ileostomy.

  Clinical Vignette Top

A 76-year-old male presented from a nursing home with progressive abdominal distention. Due to cognitive decline, the patient was only able to provide a limited history of “abdominal pain.” His medical record indicated that he suffered from progressive dementia, hypertensive, and an insulin-dependent diabetic. On physical examination, he was tachycardic at 114 beats/min, tachypneic at 24 breaths/min, and hypotensive with blood pressure of 78/53 mmHg. His abdomen was severely distended, with diffuse tenderness to palpation. The patient's laboratory results were significant for leukocytosis of 14,500 cells/μL, hypokalemia at 3.3 mmol/dL, and hypomagnesemia of 1.2 mmol/dL. Abdominal radiograph demonstrated massively dilated large bowel [Figure 1].
Figure 1: Abdominal film showing massive and diffuse colonic dilation, with greatest colonic diameter measured at 15 cm

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Given the patient's acute clinical presentation, signs of systemic inflammatory response, and the presence of peritonitis on examination, he was taken urgently to the operating room. Upon entering the peritoneum, the patient was found to have a necrotic and friable cecum. A subtotal colectomy was performed [Figure 2] for photograph of surgical specimen, and end ileostomy was created. The patient subsequently spent 3 days in the intensive care, followed by an additional week in the hospital. He eventually recovered sufficiently to allow discharge back to the nursing home.
Figure 2: Total colectomy specimen removed at the time of laparotomy

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  Discussion Top

Chronic megacolon is a progressive condition that can present acutely in its terminal stages.[7] This heterogeneous entity can be broadly categorized either as Hirschsprung's disease that primarily affects pediatric patients [8],[9] or adult-onset, non-Hirschsprung's megacolon.[3],[10] Of note, the latter idiopathic entity is distinct from Ogilvie's syndrome or acute colonic pseudo-obstruction.[15] Among institutionalized patients, chronic megacolon can progress to so-called GMC, characterized by the presence of obstipation and large-bowel obstruction.[14]

The pathophysiology of chronic megacolon is poorly understood and involves progressive changes within the intestinal connective tissue, smooth muscle cells, and the associated enteric/extrinsic nerves.[10],[12] Various neurological, metabolic, endocrine, connective tissue, and muscle disorders have been associated with the appearance of megacolon.[16],[17] Certain pharmacologic therapies (e.g., tricyclic antidepressants, phenothiazines, and clonidine) may also be contributory.[16] Idiopathic slow-transit constipation, likely a result of several of the above factors presents simultaneously, is hypothesized to lead to the development of chronic megacolon.[18],[19] Mechanistically, chronic megacolon is associated with the loss of enteric neuronal function with submucosal connective tissue and smooth muscle destruction. In a histopathologic study of patients with idiopathic megacolon, it was found that approximately 36% had myopathy, 61% had neuropathy, and 32% had mesenchymyopathy.[20] GMC is thought to represent the end-stage manifestation of chronic megacolon.[21]

Clinical hallmarks of chronic megacolon include abdominal pain (84% patients), bloating, and abdominal distention (95% cases).[10] Chronic constipation combined with the evidence of decreased gastrointestinal motility is important components of associated medical history, with an average of 1.5 bowel movements per week in affected patients.[10] When evaluating chronically institutionalized patients presenting with abdominal distention, it is important to consider other serious colonic disorders such as toxic megacolon, Ogilvie's syndrome, and various forms of mechanical obstruction.[22],[23] As exemplified by the current case scenario, chronic megacolon can evolve into a surgical emergency, with associated life-threatening tissue ischemia and/or colonic perforation.[24] Obtaining detailed history of the disease is important, including past treatments and a complete list of medications.

Cases of chronic megacolon are initially treated conservatively, with a combination of laxatives, enemas, and avoidance of constipating medications. Optimized management of any underlying medical condition is critical. For refractory nonemergent cases, surgery is generally well tolerated, with the most commonly performed operation being total abdominal colectomy with ileorectal anastomosis. Success rates after subtotal colectomy, defined as improvement in symptoms and at least 1–2 bowel movements per day, are in excess of 80%.[25]

  Conclusions Top

The current case demonstrates the end-stage presentation of chronic megacolon – a serious, progressive condition that continues to be poorly understood. The patient described herein presented acutely with massively and diffusely dilated colon, also known as “GMC.” Due to hemodynamic instability and cecal necrosis, the patient underwent emergent total abdominal colectomy with end ileostomy. An overview of pathophysiologic and clinical management considerations pertinent to GMC is also provided.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Harari D, Minaker KL. Megacolon in patients with chronic spinal cord injury. Spinal Cord 2000;38:331-9.  Back to cited text no. 1
Gan SI, Beck PL. A new look at toxic megacolon: An update and review of incidence, etiology, pathogenesis, and management. Am J Gastroenterol 2003;98:2363-71.  Back to cited text no. 2
Barnes PR, Lennard-Jones JE, Hawley PR, Todd IP. Hirschsprung's disease and idiopathic megacolon in adults and adolescents. Gut 1986;27:534-41.  Back to cited text no. 3
Min BH, Son HJ, Kim JJ, Rhee JC, Lee SJ, Rhee PL. Idiopathic proximal hemimegacolon: Radiologic findings and analyses of clinical and physiological characteristics. Abdom Imaging 2010;35:291-5.  Back to cited text no. 4
Preston DM, Lennard-Jones JE, Thomas BM. Towards a radiologic definition of idiopathic megacolon. Gastrointest Radiol 1985;10:167-9.  Back to cited text no. 5
Jones JH, Chapman M. Definition of megacolon in colitis. Gut 1969;10:562-4.  Back to cited text no. 6
Hanauer SB, Wald A. Acute and chronic megacolon. Curr Treat Options Gastroenterol 2007;10:237-47.  Back to cited text no. 7
Sullivan PB. Hirschprung's disease. Arch Dis Child 1996;74:5-7.  Back to cited text no. 8
Tam PK. Hirschsprung's disease: A bridge for science and surgery. J Pediatr Surg 2016;51:18-22.  Back to cited text no. 9
O'Dwyer RH, Acosta A, Camilleri M, Burton D, Busciglio I, Bharucha AE. Clinical features and colonic motor disturbances in chronic megacolon in adults. Dig Dis Sci 2015;60:2398-407.  Back to cited text no. 10
Coremans GE. Surgical aspects of severe chronic non-Hirschsprung constipation. Hepatogastroenterology 1990;37:588-95.  Back to cited text no. 11
Gattuso JM, Kamm MA. Clinical features of idiopathic megarectum and idiopathic megacolon. Gut 1997;41:93-9.  Back to cited text no. 12
Iantorno G, Bassotti G, Kogan Z, Lumi CM, Cabanne AM, Fisogni S, et al. The enteric nervous system in chagasic and idiopathic megacolon. Am J Surg Pathol 2007;31:460-8.  Back to cited text no. 13
Watkins GL, Oliver GA, Rosenberg BF. Giant megacolon in the insane. Subtotal colectomy as a method of management. Ann Surg 1961;153:409-17.  Back to cited text no. 14
Nanni C, Garbini A, Luchetti P, Nanni G, Ronconi P, Castagneto M . Ogilvie's syndrome (acute colonic pseudo-obstruction). Dis Colon Rectum 1982;25:157-66.  Back to cited text no. 15
Faulk DL, Anuras S, Christensen J. Chronic intestinal pseudoobstruction. Gastroenterology 1978;74(5 Pt 1):922-31.  Back to cited text no. 16
Bharucha AE, Phillips SF. Megacolon: Acute, toxic, and chronic. Curr Treat Options Gastroenterol 1999;2:517-23.  Back to cited text no. 17
Tack J, Müller-Lissner S, Stanghellini V, Boeckxstaens G, Kamm MA, Simren M, et al. Diagnosis and treatment of chronic constipation – A European perspective. Neurogastroenterol Motil 2011;23:697-710.  Back to cited text no. 18
Knowles CH, Scott SM, Wellmer A, Misra VP, Pilot MA, Williams NS, et al. Sensory and autonomic neuropathy in patients with idiopathic slow-transit constipation. Br J Surg 1999;86:54-60.  Back to cited text no. 19
Ohkubo H, Masaki T, Matsuhashi N, Kawahara H, Yokoyama T, Nakajima A, et al. Histopathologic findings in patients with idiopathic megacolon: A comparison between dilated and non-dilated loops. Neurogastroenterol Motil 2014;26:571-80.  Back to cited text no. 20
Prokić B, Todorović V, Mitrović O, Vignjević S, Savić-Stevanović V . Ethiopathogenesis, diagnosis and therapy of acquired megacolon in dogs. Acta Vet 2010;60:273-84.  Back to cited text no. 21
Sheikh RA, Yasmeen S, Pauly MP, Trudeau WL. Pseudomembranous colitis without diarrhea presenting clinically as acute intestinal pseudo-obstruction. J Gastroenterol 2001;36:629-32.  Back to cited text no. 22
Vanek VW, Al-Salti M. Acute pseudo-obstruction of the colon (Ogilvie's syndrome). An analysis of 400 cases. Dis Colon Rectum 1986;29:203-10.  Back to cited text no. 23
Stephenson BM, Morgan AR, Salaman JR, Wheeler MH. Ogilvie's syndrome: A new approach to an old problem. Dis Colon Rectum 1995;38:424-7.  Back to cited text no. 24
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  [Figure 1], [Figure 2]


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