Acute colonic pseudo-obstruction (ACPO), or Ogilvie syndrome, is a gastrointestinal motility disorder characterized by marked colonic dilation in the absence of mechanical obstruction. It is an uncommon but potentially fatal condition with significant morbidity and mortality that can be prevented by early diagnosis and prompt management (1,2).
A 5-year-old child with an unremarkable past history presented to the pediatric intensive care unit with persistent fever, pneumonia, pleuro-pericardial effusion, and respiratory failure. There was no history of constipation, trauma, toxic ingestion, or diarrhea. Family history was negative for abnormal gastrointestinal motility, connective tissue disorder, autonomic dysfunction, and mitochondrial defects. His electrolyte levels were normal: sodium, 140 mEq/L; potassium, 4.3 mEq/L; calcium, 8.7 mg/dL; phosphorus, 4.3 mg/dL; and magnesium, 2.4 mg/dL. His white cell count was 33,860/mm3, hemoglobin was 9.6 g/dL, platelet count was 1,120,000/mm3, C-reactive protein was 28.7 mg/dL, erythrocyte sedimentation rate was 122, alanine aminotransferase was 107 U/L, γ-glutamyl transpeptidase was 93 U/L, lactate dehydrogenase was 1188 IU/L, and stool guaiac test results were negative. His blood, urine, pleural fluid, fungal, and bacterial cultures were negative. His echocardiogram was normal with no coronary artery involvement. He tested negative for cytomegalovirus, respiratory syncytial virus, Coxsackie virus, HIV, hepatitis, and Epstein-Barr virus. He screened negative for rheumatoid factor, ANA, and anti-DNA. He was treated with antibiotics, diuretics, midazolam, fentanyl, and intravenous immunoglobulin for suspected atypical Kawasaki disease (KD). KD was suspected because of his prolonged fever, mucous membrane changes, increasing markers of inflammation with pneumonia, and pleuro-pericardial involvement without an apparent diagnosis.
He developed abdominal pain and distension on day 9 of admission. On day 10 of hospitalization he developed rapid progression of abdominal distension and increasing volume of bilious nasogastric aspirate. On examination the abdomen was tense and tender with hypoactive bowel sounds. He developed hypokalemia (3.2 mg/dL) and hypophosphatemia (2.9 mg/dL). Abdominal girth increased from 49 to 54.9 cm. Rectal examination showed ballooning of the rectum without fecal impaction. An abdominal radiograph and computed tomography scan revealed massive dilation of the large bowel and cecum with minimal dilation of the small bowel. There were neither air/fluid levels nor free air. The cecal diameter was 6 cm. The diagnosis of ACPO was entertained because of massive abdominal distension with preferential colonic dilation without signs of mechanical obstruction or perforation (Fig. 1).
The child was managed conservatively with bowel rest, nasogastric tube drainage, electrolyte correction, and weaning of sedative-hypnotic medications. (His condition deteriorated with increasing abdominal distension.) His colon was decompressed with a rectal tube with prompt evacuation of flatus and stool with reduction in abdominal circumference to 50.5 cm and cecal diameter to 5 cm on subsequent radiography. There was complete clinical and radiological resolution gradually over the next 3 days with conservative management and rectal tube decompression. He did not require surgical intervention. Subsequently, he had no further gastrointestinal complications during the hospitalization and 3 years of follow-up.
Ogilvie syndrome was named after Sir Heneage Ogilvie, who in 1948 described 2 patients with colonic obstruction without evidence of organic obstruction to intestinal flow (3). Hence, ACPO is also known as Ogilvie syndrome. It is seen in the adult population, but rarely in children (4–6). (Table 1). The pathophysiology is not completely understood. It is believed to result from either a suppression of sacral parasympathetic nerves or an increase in sympathetic tone leading to inhibition of colonic motility. It is accompanied by dilation of the proximal colon resulting in retention of large quantities of gas and fluid (7–10). This leads to distension and increased intraluminal pressure in the proximal colon and cecum. This intraluminal pressure impedes the cecal capillary circulation leading to ischemia, gangrene, and perforation. This syndrome occurs in patients with serious medical and surgical illnesses including stroke, myocardial infarction, neoplasia, metabolic disturbances, spinal injury, peritonitis, sepsis, and shock, and with various medications such as narcotics, tricyclic antidepressants, and sedatives or hypnotics (11). Mechanical obstruction, perforation, and ischemia are to be excluded before the diagnosis is made. The clinical features are nausea, vomiting, abdominal pain, obstipation, and fever. Abdominal distension can develop in 24 hours to approximately 7 days (10). Bowel sounds may be hyperactive, hypoactive, or absent.
The diagnosis of Ogilvie syndrome is based on history, physical and radiological examination, and exclusion of other diagnoses. There are no specific laboratory tests but the plain abdominal radiographs are the most useful diagnostic tool for this disorder. In ACPO, there is no mechanical obstruction, preferential colonic and cecal dilation with normal haustral markings, and thin colonic wall (Fig. 1). The radiograph of ACPO shows gaseous distention with little fluid in the bowel lumen, in contrast to mechanical obstruction, which contains multiple air/fluid levels. Specific attention to the diameter of the cecum and colon is important (2). A threshold cecal diameter of 9 to 12 cm is commonly used in adult patients for decompression to avoid the risk of spontaneous perforation (10,12). There are no established criteria for the pediatric age group.
The primary aim of the treatment is to halt the evolution to ischemia or perforation. The initial step is to ascertain that there is no mechanical obstruction that requires an operative intervention. Conservative management is the initial mode of therapy because spontaneous resolution is seen in 85% of cases (13). The management consists of bowel rest, nasogastric decompression, fluid and electrolyte correction, and aggressive treatment of any underlying reversible medical conditions. Discontinuation of any causative medications is crucial. Rectal tube placement is helpful if the colonic distention extends to the rectosigmoid region. An abdominal radiograph should be obtained daily and abdominal circumference measurements taken multiple times per day. Studies have shown the use of pharmacological agents like erythromycin and neostigmine in the management of ACPO (6,13–16). Several controlled and noncontrolled open-label case series supported the use of neostigmine in this condition, with a success rate of as high as 91% (8). The experience of neostigmine in the pediatric age group is limited (6). The failure of pharmacological and conservative management requires colonoscopic decompression. Success rate are 60% to 80% and immediate recurrence rates are 18% to 40% (10,17). Cecostomy is reserved for impending ischemia, perforation, peritonitis, or failure of all other measures. The subsequent perforation may require subtotal colectomy. The mortality rate in the pediatric age is unknown, but is approximately 15% in the adult population. The risk increases to 36% in the presence of ischemia or perforation (10,13).
Intestinal pseudoobstruction is an infrequent but important manifestation of KD (18). ACPO has never shown to be associated with KD. Intestinal pseudoobstruction can occur at the onset or during the course of the disease. The pathogenesis of pseudoobstruction in KD may relate to mesenteric artery vasculitis with bowel ischemia and associated dysfunction of the myenteric plexus (19,20). The reported treatment includes bowel rest, intravenous immunoglobulin, and intravenous steroids (18–20).
We believe the etiology of ACPO in our case could be multifactorial. Atypical KD, severe systemic disease, use of narcotic/sedative medications, and electrolyte imbalance could be contributing factors in the pathogenesis of ACPO.
There are only 2 more reported cases of ACPO in the pediatric literature as far as we are aware (Table 1). A 4-year-old child with electrolyte imbalance responded to neostigmine therapy and an 18-year-old patient with sepsis and surgery responded to conservative management.
Sepsis, hypokalemia, atypical KD, and the administration of narcotics may have contributed to ACPO in our patient. Although it is uncommon in children, it should be considered in all patients with significant abdominal distention. It is of paramount importance that physicians recognize this clinical entity, as early diagnosis and prompt management can reverse the outcome of this potentially fatal condition.
1. Di Lorenzo C. Pseudo-obstruction: current approaches. Gastroenterology 1999; 116:980–987.
2. Laine L. Management of acute colonic pseudo-obstruction. N Engl J Med 1999; 341:192–193.
3. Ogilvie WH. Large intestine colic due to sympathetic deprivation: a new clinical syndrome. Br Med J 1948; 2:671–673.
4. Vadala G, Santonocito G, Mangiameli A, et al
. Ogilvie's syndrome. Minerva Med 1998; 89:185–188.
5. Irabor DO, Ladipo JK, Ogunsanya WF, et al
. Ogilvie's syndrome (colonic pseudo-obstruction) occurring in an 18-year-old Nigerian female. West Afr J Med 2001; 20:75–77.
6. Gmora S, Poenaru D, Tsai E. Neostigmine for the treatment of pediatric acute colonic pseudo-obstruction. J Pediatr Surg 2002; 37:E28.
7. Baker DA, Morin ME, Tan A, et al
. Colonic ileus: indication for prompt decompression. JAMA 1979; 241:2633–2634.
8. Saunders AD. Acute colonic pseudoobstruction. Curr Gastroenterol Rep 2004; 6:410–416.
9. Ponec RJ, Saunders MD, Kimmey MB. Neostigmine for the treatment of acute colonic pseudo-obstruction. N Engl J Med 1999; 341:137–141.
10. Vanek VW, Al-Salti M. Acute pseudo-obstruction of the colon (Ogilvie's syndrome): an analysis of 400 cases. Dis Colon Rectum 1986; 29:203–210.
11. Peck SN, Altschuler SM. Pseudo-obstruction in children. Gastroenterol Nurs 1992; 14:184–188.
12. Gierson ED, Storm FK, Shaw W, et al
. Caecal rupture due to colonic ileus. Br J Surg 1975; 62:383–386.
13. Fazel A, Verne GN. New solutions to an old problem: acute colonic pseudo-obstruction. J Clin Gastroenterol 2005; 39:17–120.
14. Stephenson BM, Morgan AR, Salaman JR, et al
. Ogilvie's syndrome a new approach to an old problem. Dis Colon Rectum 1995; 38:424–427.
15. Turegano-Fuentes F, Munoz-Jimenez F, Del Valle-Hernandez E, et al
. Early resolution of Ogilvie's syndrome with intravenous neostigmine: a simple effective treatment. Dis Colon Rectum 1997; 40:1353–1357.
16. Rovira A, Lopez A, Cambray C, et al
. Acute colonic pseudo-obstruction (Ogilvie's syndrome) treated with erythromycin. Intens Care Med 1997; 23:798.
17. Geller A, Patersen BT, Gostout CJ. Endoscopic decompression for acute colonic pseudoobstruction. Gastrointest Endosc 1996; 44:144–150.
18. Akikusa JD, Laxer RM, Friedman JN. Intestinal pseudoobstruction in Kawasaki disease. Pediatrics 2004; 113:e504–e506.
19. Miyake T, Kawamori J, Yoshida T, et al
. Small bowel pseudo-obstruction in Kawasaki disease. Pediatr Radiol 1987; 17:383–386.
20. Franken EA Jr, Kleiman MB, Norins AL, et al
. Intestinal pseudo-obstruction in mucocutaneous lymph-node syndrome. Radiology 1979; 130:649–651.