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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 9  |  Issue : 4  |  Page : 70-72

Perforated Toxic Megacolon: The Dreaded Complication in IBD


Department of General Surgery, Hospital Teluk Intan, Perak, Malaysia

Date of Submission03-Dec-2020
Date of Decision16-Dec-2020
Date of Acceptance24-Dec-2020
Date of Web Publication31-Dec-2020

Correspondence Address:
Dr. Kanmani Murugesu
No 27 Jalan Hang Lekiu, Padang Golf Kuala Kubu Bharu, 44000 Selangor
Malaysia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1941-8213.305993

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  Abstract 

Toxic megacolon is a dreaded complication of inflammatory bowel disease. Unfortunately, it is usually diagnosed late and, in cases of perforation, has a high mortality rate and is associated with a poor prognosis. We present a case of perforated toxic megacolon in a young woman with undiagnosed ulcerative colitis, highlighting the clinical course and outcome of this condition along with the need for prompt detection and intervention. It was difficult to manage this case in a district hospital because of the limited subspecialty support and resources.

Keywords: Keywords: Inflammatory bowel disease, perforation, toxic megacolon


How to cite this article:
Murugesu K, Sivadasan PN, Arvind M, Wei Xin WL. Perforated Toxic Megacolon: The Dreaded Complication in IBD. World J Colorectal Surg 2020;9:70-2

How to cite this URL:
Murugesu K, Sivadasan PN, Arvind M, Wei Xin WL. Perforated Toxic Megacolon: The Dreaded Complication in IBD. World J Colorectal Surg [serial online] 2020 [cited 2021 Aug 2];9:70-2. Available from: https://www.wjcs.us.com/text.asp?2020/9/4/70/305993


  Introduction Top


Toxic megacolon is a potentially fatal complication of any form of colitis, which includes inflammatory, ischemic, infectious, radiation, and pseudomembranous colitis.[1] The incidence of toxic megacolon is approximately 5% in inflammatory bowel disease (IBD). It is more common in ulcerative colitis than in Crohn's Disease.[1] Patients with ulcerative colitis are at a risk of developing this complication, although it rarely is an early form of presentation.[1] The mortality rate varies from 38% to 80% and even higher with perforation, sepsis, and multiorgan failure.[2] Therefore, prompt diagnosis and a multidisciplinary approach are crucial.


  Case Report Top


A 22-year-old woman with no comorbidity presented to the Emergency Department at Hospital Teluk Intan, Malaysia, with a 5-month history of loose stool, worsening lethargy, and abdominal pain for 2 weeks. She had multiple visits to the hospital in the past for chronic diarrhea and abdominal pain, and was managed symptomatically.

Clinically, she was septic and febrile at 38°C with a pulse rate of 120 beats per minute. Her abdomen was distended but not tender. Blood investigations showed white cell count of 33 Χ 109/L, hemoglobin level of 6.4 g/dL, sodium at 122 mmol/L, potassium at 5.5 mmol/L, creatinine at 50 μmol/L, albumin at 15 g/L, and C-reactive protein concentration of 150 mg/L. The x-ray was normal, while the computed tomography (CT) of her abdomen on admission showed marked dilatation of the ascending and transverse colon (7.3 cm) with an irregular mucosal surface, suggestive of ulcerative colitis with no pneumoperitoneum, as seen in [Figure 1].
Figure 1: Initial computed tomography scan showing dilated transverse colon 7.3 cm

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Figure 2: Second computed tomography scan showing gross pneumoperitoneum

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Figure 3: Intra-operative specimen and subtotal colectomy

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She was admitted to the High-Dependency Unit of the hospital and managed conservatively with intravenous (IV) ceftriaxone and corticosteroids. However, 5 days later, she developed peritonitis with septic shock. A repeat CT abdomen [Figure 2] showed gross pneumoperitoneum. She underwent emergency laparotomy and was intraoperatively noted to have a grossly dilated large bowel from the cecum to the descending colon with perforations over both the caecum and transverse colon as shown in [Figure 3]. We performed a subtotal colectomy with a double barrel stoma. Histopathological examination showed fulminant ulcerative colitis with toxic megacolon and perforations characterized by microscopic features of superficial to deep ulcerations and thinned out muscularis propria replaced by the inflamed granulation tissue.

Her postoperative period was stormy and complicated with multiorgan failure, requiring dialysis and tracheostomy for prolonged ventilation. She had multiple bouts of hospital-acquired infections with multiresistant organisms. In addition, she developed abdominal wound dehiscence day 6 postoperatively and required a relaparotomy with wound debridement, abdominal lavage, and fascial closure with vacuum dressing. She required total parenteral nutritional followed by the introduction of enteral feeding. She was started on mesalazine enema for the treatment of ulcerative colitis. Despite severe nosocomial infections and critical illness neuropathy, she recovered and was discharged home ambulating with a wheelchair 3 months after the surgery.


  Discussion Top


Toxic megacolon is an unusual but recognized and highly morbid complication caused by a number of conditions such as IBD, ischemic, infectious, or pseudomembranous colitis. It is more common in the pancolitis form of colitis compared to segmental colitis.[1] Patients with ulcerative colitis are at risk of this complication at the early phase of the disease and it rarely is an initial presentation with a lifetime incidence of 1–2.5%.[1] The microscopic hallmark of toxic megacolon with a background of ulcerative colitis is inflammation extending beyond the mucosa into the colonic smooth muscle and serosa, thus paralyzing the colon and leading to its dilatation.[1] In ulcerative colitis, the predominant inflammatory mediator is nitrous oxide, which further relaxes the smooth muscle and, with repeated inflammation, causes thinning of the muscular layer and its eventual perforation.[1],[3]

The initial diagnosis is based on the clinical signs of toxicity and imaging, which shows colonic dilatation. The systemic signs of toxicity such as altered sensorium, hypotension, fever, and tachycardia predominate. Laboratory findings include leukocytosis, anemia, electrolyte imbalance, and elevated C-reactive protein.[1] The imaging criteria of toxic megacolon is nonobstructive dilatation of the colon (>5.5 cm).[1] The plain abdominal radiograph is an established form of imaging; however, CT provides additional information and leads to early detection of local complications.[1]

Toxic megacolon requires early recognition and prompt involvement of the gastroenterologist, surgeon, and intensivist from the time of diagnosis. The aim of treatment is to reduce inflammation, improve bowel motility, and prevent perforation.[1] Jiten et al. (2020) described medical management of toxic megacolon in the absence of complications such as perforation, massive bleeding, abdominal compartment syndrome, or ischemia. Medical therapy includes close monitoring, fluid resuscitation, intravenous corticosteroids, or second-line agents such as infliximab and cyclosporin.[1] Upon clinical deterioration, failure of response to second-line agents or evidence of complications such as perforation, which was seen in our patient, surgical intervention is life-saving.[1]

In an emergency setting, the surgical intervention of choice is subtotal colectomy with end ileostomy or a sigmoid mucous fistula;[4] the latter was performed in our patient. This is a safe and relatively quick procedure in patients with toxic megacolon as they are usually critically ill. It has a lower mortality rate than single-stage proctocolectomy.[4] Diversion alone with decompression is not recommended as it carries a high risk of postoperative bleeding while retaining the primary bulk of the diseased bowel.[3] Postoperative restorative procedures such as an ileal pouch anal anastomosis can be performed later once patients have recovered from the initial phase.[5]

Following the rectal conserving surgery, medical treatment can be initiated after the acute flare.[6] Mesalazine is effective in maintaining the remission. Topical mesalazine including enema or suppository, which was used in our patient, is more effective in maintaining the remission than oral mesalazine, particularly in ulcerative colitis.[6],[7] In addition to the surgical stress, patients with IBD are usually malnourished.[8] In emergency surgery for IBD patients, medical nutrition (enteral or parenteral) should be started if the oral diet cannot be commenced within 7 days of surgery.[8] Parenteral nutrition was started in our patient as she had prolonged ileus and was subsequently returned to enteral feeding.[8] The benefits and importance of adequate nutrition for recovery in postoperative patients is beyond the scope of this article.

Unfortunately, due to the extent of our patient's condition requiring steroids and neuromuscular blocking agents during her postoperative period, she also developed another debilitating issue. Critical illness neuropathy (CIN), also known as ICU neuropathy, is a frequent complication of chronic illness. It is characterized by limb and respiratory muscle weakness.[9] CIN was seen in our patient who required prolonged ventilation with a tracheostomy and developed lower limb weakness, which persisted upon discharge. Nerve conduction study (NCS), electromyography, and muscle biopsy can be used to diagnose and rule out other associated diseases such as the Guillain-Barrι syndrome.[9] Our patient underwent NCS, which was negative for the demyelinating disease. Upon discharge, she was given frequent follow-up with the rehabilitation team in a nearby healthcare facility. Early rehabilitation involving mobilization and physiotherapy is the mainstay of the treatment. Avoiding neuromuscular blocking agents and corticosteroids may reduce the risk of developing CIN.[10] Management in a district hospital may have contributed to her poor functional recovery at the end due to a lack of goal-orientated programs and intervention for rehabilitation.


  Conclusion Top


Perforated toxic megacolon is a life-threatening condition. Failure to recognize this disorder and late intervention are associated with poor prognosis and high mortality. Its management is multidisciplinary and involvement of the surgeon, gastroenterologist, and intensivist is vital for a favorable outcome. Close monitoring and supportive management is the initial route of management; however, in the event of perforation, bleeding, or clinical deterioration, surgical intervention is life-saving. Long-term management with a physiotherapist and a dietician will also ensure good functional status for patients after surgery.

Acknowledgement

We acknowledge the support of Dr. Kadir (Pathologist Hospital Teluk Intan) in the conductance of this work.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Desai J, Elnaggar M, Hanfy AA, Doshi R. Toxic megacolon: Background, pathophysiology, management challenges and solutions. Clin Exp Gastroenterol 2020;13:203-10.   Back to cited text no. 1
    
2.
Strong SA. Management of acute colitis and toxic megacolon. Clin Colon Rectal Surg 2010;23:274-84.   Back to cited text no. 2
    
3.
Autenrieth DM, Baumgart DC. Toxic megacolon. Inflamm Bowel Dis 2012;18:584-91.   Back to cited text no. 3
    
4.
Ross H, Steele SR, Varma M, Dykes S, Cima R, Buie WD, et al. Practice parameters for the surgical treatment of ulcerative colitis. Dis Colon Rectum 2014;57:5-22.   Back to cited text no. 4
    
5.
Harbord M, Eliakim R, Bettenworth D, Karmiris K, Katsanos K, Kopylov U, et al. Third European evidence-based consensus on diagnosis and management of ulcerative colitis. Part 2: Current management. J Crohns Colitis 2017;11:769-84.   Back to cited text no. 5
    
6.
Matsuoka K, Kobayashi T, Ueno F, Matsui T, Hirai F, Inoue N, et al. Evidence-based clinical practice guidelines for inflammatory bowel disease. J Gastroenterol 2018;53:305-53.   Back to cited text no. 6
    
7.
Kornbluth A, Sachar DB. Ulcerative colitis practice guidelines in adults: American college of gastroenterology, practice parameters committee. Am J Gastroenterol 2010;105:501-23.   Back to cited text no. 7
    
8.
Bischoff SC, Escher J, Hébuterne X, Kłęk S, Krznaric Z, Schneider S, et al. ESPEN practical guideline: Clinical Nutrition in inflammatory bowel disease. Clin Nutr 2020;39:632-53.   Back to cited text no. 8
    
9.
Chawla J, Gruener G. Management of critical illness polyneuropathy and myopathy. Neurol Clin 2010 28:961-77.   Back to cited text no. 9
    
10.
Baek WK, Kim YS, Kim JT, Yoon BN. Critical illness neuromyopathy complicating cardiac surgery. Acute Crit Care 2018;33:51-6.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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