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BMJ Case Rep. 2009; 2009: bcr03.2009.1681.
Published online Dec 30, 2009. doi:  10.1136/bcr.03.2009.1681
PMCID: PMC3029588
Unusual presentation of more common disease/injury
A case of fulminant diversion pan-colitis presenting 19 years after colonic diversion for neuronal intestinal dysplasia
Deya Nawrani,1 Philip Turton,2 and Dermot Burke3
1Leeds Teaching Hospitals, Breast and General Surgery, 68 Wensley Road, Leeds LS7 2LS, UK
2Leeds Teaching Hospitals NHS Trust, Breast and General Surgery, Leeds General Infirmary, Great George Street, Leeds LS1 3EX, UK
3Leeds Teaching Hospitals NHS Trust, Colorectal and General Surgery, Leeds General Infirmary, Great George Street, Leeds LS1 3EX, UK
Deya Nawrani, deyanawrani/at/doctors.org.uk
A 20-year old woman with a long history of abdominal symptoms presented with acute abdominal pain associated with nausea and vomiting. She had had a right iliac fossa end-ileostomy previously fashioned at the age of 11 months for neuronal intestinal dysplasia. A few days after her admission she suffered a hypokalaemic cardiac arrest from which she was resuscitated. Her clinical condition deteriorated as she became increasingly septic. She was diagnosed with a fulminant colitis based on the clinical picture and the finding of fluid filled, thick walled rectum suggestive of proctocolitis on a pelvic magnetic resonance imaging scan. She was taken to the operating theatre where she had a subtotal colectomy. The histopathological diagnosis was that of fulminant diversion colitis. The patient recovered from surgery and was discharged home 5 weeks later.
Background
A patient with an abdominal stoma may develop complications relating to a diverted segment of distal bowel. Diversion colitis is an under-reported cause of abdominal symptoms which may manifest insidiously or acutely. An awareness of the condition by treating clinicians is a prerequisite to early diagnosis and appropriate investigation and treatment. Our report is the first in the English literature of a case of fulminant diversion colitis.
A 20-year old woman was admitted acutely under the care of the gastroenterologists with a 4 day history of central abdominal pain of moderate severity associated with nausea and vomiting. For the previous 6 months she had experienced mild non-specific chronic lower abdominal pain, which had been labelled as non-organic pain.
Her past medical history was complex. She had neuronal intestinal dysplasia as a baby which manifested as a pseudo-obstruction. Consequently she required a right iliac fossa end-ileostomy which was fashioned at the age of 11 months. Her colon had been left in situ. There was a family history of neuronal intestinal dysplasia. Her twin sister and brother had also had an end-ileostomy fashioned at a similar age.
She had longstanding psychological problems including fluctuating depression, obsessive compulsive disorder, and apparent psychosomatic disorder. In addition, she was wheelchair bound secondary to congenital spastic paraparesis. She suffered from recurrent urinary tract infections and had ongoing episodes of high stoma output.
For many years she had direct access to the medical gastroenterology ward for parenteral rehydration in case of high stoma output causing dehydration. On this occasion she was admitted to the gastroenterology ward for further management in view of what was thought to be recurrent non-organic symptoms.
On admission, she was apyrexial and haemodynamically normal. There were no signs of peritonitis, and a digital rectal examination was normal. Blood investigations and plain abdominal imaging on admission were unremarkable. A provisional diagnosis of a psychosomatic abdominal pain was made.
After 4 days the patient was transferred to the psychiatric ward as she was refusing to eat or allow the phlebotomists to take blood for routine investigations. Within 24 h of transfer she suffered a cardiac arrest. Attempts at cardiopulmonary resuscitation were eventually successful in restoring a cardiac output. She required intubation and ventilation and was transferred to the intensive care unit (ICU).
She was noted to be profoundly hypokalaemic with a K+ of 1.8 mmol/l. It was presumed that self induced vomiting had caused her hypokalaemia and this in turn had triggered ventricular fibrillation. After 24 h she was noted to have an increasing serum lactate that reached 17 mmol/l. She required increasing inotropic support and so a surgical opinion was sought.
On admission:
  • Haemoglobin 12.2 g/dl, white blood cell count 16.7×109/l
  • C reactive protein <5.0 mg/l, K 3.3, albumin 25 g/l
  • Abdominal x-ray, chest x-ray : normal
Prior to admission:
  • Flexible cystoscopy: normal
  • Rigid sigmoidoscopy: erythematous rectum
In the ICU:
  • K+ 1.8 mmol/l
  • Serum lactate 17 mmol/l
  • Pelvic magnetic resonance imaging (MRI) (fig 1): this showed a fluid filled, thick walled rectum suggestive of proctocolitis.
    Figure 1
    Figure 1
    Fast spin echo T2 weighted magnetic resonance imaging of the pelvis performed to look for a rectovesical fistula showing: (A) a midline sagittal section with a fluid filled dilated rectum with no connection of proximity to the bladder or vagina; (B) a (more ...)
  • Clostridium difficile toxin was not detected in rectal discharge
Postoperative:
  • Histopathology: sections from the colectomy showed features typical of fulminant diversion colitis
Differential diagnosis
  • Fulminant colitis
  • Ischaemic bowel
The surgical review of the patient on the ICU gave rise to a suspicion of the presence of a fulminant colitis. Although it was apparent that the patient had an end-ileostomy there was no initial indication that the patient still had an intact but diverted full length colon. After review of her medical history and clinical deterioration it was noted that a pelvic MRI (fig 1) that had been done just 2 days earlier (previously requested by the urologists) had shown a fluid filled, thick walled rectum suggestive of proctocolitis. C difficile infection was excluded. The clinical picture fitted with possible fulminant colitis and the patient was transferred to the operating theatre for a laparotomy. She was found to have low volume ascites, and a hyperaemic appearance of the serosa of the entire colon. It was noted that she had an end-ileostomy and a previously diverted ileocaecal junction. She underwent a sub-total colectomy and was transferred back to ICU.
Outcome and follow-up
There was a dramatic improvement in the patient’s condition, both clinically and biochemically, post-colectomy. Her lactate returned swiftly to a normal value. She continued to recover slowly and was discharged home 5 weeks later.
The histological features that are described in a diverted bowel segment are universal regardless of the original pathology. They occur to some degree in the majority of defunctioning bowels. An inflammatory picture with lymphoid follicular hyperplasia is the hallmark of diversion colitis. Cryptitis, crypt abscesses, mixed acute and chronic inflammation, as well as aphthous ulceration have also been described.1
In our patient the original colonic pathology was neuronal intestinal dysplasia (NID). This is a rare pathological entity characterised by aganglionosis of the bowel. It may be localised or diffuse. Clinically it presents a similar picture to Hirschsprung’s disease, often with pseudo-obstruction.
Distinguishing diversion colitis clinically poses a challenge, especially so in the absence of an underlying inflammatory bowel disease or an infective source. The diagnosis in this case had been particularly difficult because of a history of apparent psychosomatic disorder. The high stoma output may well have contributed to such a profound hypokalaemia. The cardiac arrest and need for inotropic support may also have precipitated the fulminant picture of her diversion colitis.
The exact pathogenesis of diversion colitis is not fully understood. In all cases affecting the large bowel, proximal diversion of the colonic stream is the common denominator. Several hypotheses have been postulated such as ischaemia,2 and the role of short chain fatty acid deficiency.3 However, the proportion of patients who develop clinical symptoms or signs is unclear. Diversion colitis is usually diagnosed retrospectively on the basis of histopathological findings.
In a small paediatric study of 14 children who underwent colonic diversion for severe gastrointestinal motility disorders, it was found that all patients developed a diffuse, poorly localised abdominal pain, and 11 of them had diversion colitis.4 Diversion colitis therefore needs to be distinguished from other disorders.57 A case of diversion colitis presenting with massive rectal distension and bilateral ureteric obstruction has been previously reported,8 but our report is the first in the English literature of a life threatening fulminant diversion colitis.
There is a need to describe diversion colitis as a clinical syndrome and an entity that is distinct from other colonic pathologies. This is a prerequisite to delineate a practical approach to the diagnosis and treatment of what is essentially an iatrogenic condition. An early full endoscopy of the defunctioned segment is indicated in the pursuit of this diagnosis where there are unresolved symptoms or diagnostic doubt.
Learning points
  • Diversion colitis is a common sequelae to proximal intestinal diversion where the colon has been left in situ. It may develop at any time regardless of the length of the time interval between the original diversion and development of abdominal symptoms.
  • It may manifest insidiously with non-specific abdominal pain, but its diagnosis is often delayed.
  • An understanding of the anatomy and extent of any defunctioned segment is also required.
  • Although this was a rare case with a fulminant presentation, in all cases of diagnostic uncertainty an early full endoscopy of the defunctioned segment in the pursuit of this diagnosis is indicated.
Acknowledgments
The figures were kindly submitted by Dr John A Spencer, consultant radiologist at St James’ University Hospital, Leeds, along with the radiological diagnosis.
Footnotes
Competing interests: none.
Patient consent: Patient/guardian consent was obtained for publication
1. Geraghty JM, Talbot IC. Diversion colitis: histological features in the colon and rectum after defunctioning colostomy. Gut 1991; 32: 1020–3. [PMC free article] [PubMed]
2. Villanacci V, Talbot IC, Rossi E, et al. Ischaemia: a pathogenetic clue in diversion colitis. Colorectal Dis 2007; 9: 601–5. [PubMed]
3. Mortensen PB, Clausen MR. Short-chain fatty acids in the human colon: relation to gastrointestinal health and disease. Scand J Gastroenterol 1996; 216(Suppl): 132–48. [PubMed]
4. Ordein JJ, Di Lorenzo C, Flores A, et al. Diversion colitis in children with severe gastrointestinal motility disorders. Am J Gastroenterol 1992; 87: 88–90. [PubMed]
5. Nielsen OH, Vainer B, Rask-Madsen J. Non-IBD and non-infectious colitis. Research Support, Non-U.S. Gov’t. Nature Clinical Practice Gastroenterology & Hepatology 2008; 5: 28–39. [PubMed]
6. Szczepkowski M, Kobus A, Borycka K. How to treat diversion colitis? Current state of medical knowledge, own research and experience. Acta Chir Iugosl 2008; 55: 77–81. [PubMed]
7. Tsironi E, Irving PM, Feakins RM, et al. “Diversion” colitis caused by Clostridium difficile infection: report of a case. Dis Colon Rectum 2006; 49: 1074–7. [PubMed]
8. Boyce SA, Hendry WS. Diversion colitis presenting with massive rectal distension and bilateral ureteric obstruction. Int J Colorectal Dis 2008; 23: 1143–4. [PubMed]
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