*Department of Pediatrics, Division of Pediatric Gastroenterology & Nutrition, USA
†Department of Pediatrics, Division of Pediatric Surgery, Medical College of Wisconsin, and the Children's Hospital of Wisconsin, Milwaukee, WI, USA
Received 6 June, 2008
Accepted 19 December, 2008
Address correspondence and reprint requests to Manu R. Sood, MD, Department of Pediatrics, Medical College of Wisconsin, 8701 Watertown Plank Rd, Milwaukee, WI 53226 (e-mail: email@example.com).
The authors report no conflicts of interest.
Background: Chronic intestinal pseudo-obstruction (CIP) is a condition characterized by symptoms of bowel obstruction in the absence of an anatomical cause. Patients with CIP and chronic intractable constipation (CIC) can also develop anatomical obstruction, and the presenting symptoms mimic those of underlying pseudo-obstruction.
Objectives: Our objectives were to evaluate the incidence, clinical presentation, and diagnostic investigations of colonic volvulus in children with intestinal motility disorders and to differentiate these episodes of colonic volvulus from the underlying motility disorder based on clinical presentation and imaging techniques.
Materials and Methods: Patients records of children with colonic volvulus cared for at our institution over the previous 20 years were retrospectively reviewed. We identified 8 patients who were between 2 and 22 years of age at the time of diagnosis with colonic volvulus who also had CIP and CIC.
Results: The mean age ± SD at presentation with colonic volvulus was 13.2 ± 5.05 years. All patients presented with worsening of abdominal distension and pain. The mean duration of symptoms of colonic volvulus before seeking medical help was 4.2 days (range 1–7 days). Water-soluble contrast enema was the single most useful investigation for confirming the diagnosis. All patients required surgery. There was no mortality associated with colonic volvulus.
Conclusions: Clinicians should be vigilant and include volvulus in the differential diagnosis of the acute onset of abdominal distension and pain in patients with CIP and CIC. Delay in diagnosis can result in bowel ischemia and perforation.
Chronic intestinal pseudo-obstruction (CIP) is a rare, severe, disabling disorder characterized by recurrent symptoms of bowel obstruction in the absence of an identifiable anatomical cause. Patients with CIP have radiographic evidence of dilated bowel with air and fluid levels but no fixed lumen-occluding lesion. The common symptoms of CIP include vomiting, abdominal distension, pain, diarrhea, constipation, or urinary bladder dilation and retention (1). Patients with predominant colonic involvement can present with chronic intractable constipation (CIC) (2,3). The clinical course of CIP is characterized by periods of acute exacerbation of symptoms that can be precipitated by viral infections, central line infections, electrolyte imbalance, or bacterial overgrowth. During these episodes, the patients may have worsening of abdominal distension, pain, nausea, and vomiting.
Colonic volvulus is a well-recognized complication of chronic constipation in adults but is thought to be rare in children. There are only 7 case reports of colonic volvulus in children with CIP (4–8). The volvulus involved the splenic flexure in 3, ascending colon in 2, and transverse colon in 2 affected children. In 4 of these 7 children, the diagnosis of CIP was made after the episode of volvulus.
The aim of this study was to determine the clinical characteristics of colonic volvulus in children with CIP/CIC at Children's Hospital of Wisconsin, Milwaukee.
METHODS AND METHODS
We performed a retrospective chart review of all patients who were diagnosed with colonic volvulus at Children's Hospital of Wisconsin between July 1987 and June 2007. Children younger than 2 years were excluded from the study because a majority had volvulus secondary to malrotation or a complication secondary to necrotizing enterocolitis. Charts were reviewed for information regarding age, sex, associated motility disorders, presenting signs and symptoms at the time of diagnosis with volvulus, radiographic investigations, operative findings, surgical procedures, morbidity, mortality, and outcome. This study was approved by the institutional review board of Children's Hospital of Wisconsin.
We identified 200 patients with small and/or large bowel volvulus diagnosed at Children's Hospital of Wisconsin in the past 20 years. One hundred thirty-two were younger than 2 years of age and were, therefore, excluded. Of the remaining 68 patients, 18 had colonic volvulus. Eight of the 18 had an associated motility disorder. Four of these cases had small and large bowel dysmotility and 4 had colonic dysmotility presenting with CIC. The mean duration of symptoms of the underlying motility disorders was 7.7 years (range 3–16 years) before the volvulus occurred. The diagnosis of gastrointestinal motility disorders was established following a manometric evaluation in 4 patients, and by a combination of chronic symptoms and radiological studies in the other 4. The antroduodenal manometric study showed low amplitude and poorly organized phase 3 migrating motor complexes. The colorectal manometric studies showed pancolonic myopathic-like CIP patterns. Due to the substantial dilatation of the colon and absence of contractions even after bisacodyl administration, the delineation of myopathic versus neuropathic patterns could not be definitively determined. One of the studies showed increased internal anal sphincter pressure (IASP), but relaxation was normal following balloon distension. The mean age at presentation of colonic volvulus was 13.2 ± 5.05 years (range 3–22 years). Five were males and 3 were females. Five patients previously had gastrostomies placement, 2 had Nissen fundoplications, 1 had Roux-en-Y jejunostomy tube placement, and 1 each had small bowel intussusception reduction and small bowel resection for volvulus. Two patients did not have any previous abdominal surgeries.
There were 9 episodes of colonic volvulus in 8 of these patients. One patient had 2 episodes of volvulus, one involving the transverse colon and the other the sigmoid colon. Of the remaining 7 patients, 5 had volvulus of the sigmoid colon and 2 had cecal volvulus. Small bowel malrotation was present in 2 patients, one of which was diagnosed at the time of surgery for the volvulus. Neurological disorders including developmental delay, cerebral palsy, and seizure disorders were present in 4 patients. One patient had the diagnosis of MELAS syndrome (mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes).
All patients presented with worsening of their baseline abdominal distension, 8 had increasing abdominal pain, and 5 had emesis of which 2 were experiencing bilious emesis. Five patients had not passed a bowel movement for at least 3 days despite rectal suppositories or rectal irrigation at home. These treatments had been used previously in these patients with successful results. All patients had intermittent abdominal pain for several years. The abdominal pain associated with the colonic volvulus was much worse than the pain previously experienced with episodes of acute exacerbation of pseudo-obstruction and chronic constipation. The pain was unremitting and 4 patients required intravenous opioids for pain relief. The mean duration of symptoms of colonic volvulus before seeking medical help was 4.2 days (range 1–7 days). All patients were diagnosed with volvulus in the emergency department on the day of presentation. One of the 2 patients who presented with fever had gangrenous bowel at laparotomy.
As part of their initial evaluation, all patients had an abdominal x-ray and a contrast enema. All had dilated loops of small or large bowel. Rectal gas was absent in 2 patients. Contrast enema findings included a cutoff sign in 3 patients (sigmoid, transverse, and cecum), a beaked appearance of the sigmoid colon in 4 (Fig. 1), and narrowing of the colonic lumen in 3 patients. Colonoscopy, performed in 3 patients, showed narrowing in 1 and complete obstruction in the other 2. One of these patients had ischemic bowel and the other had a transition point showing mucosal congestion and hemorrhage secondary to the volvulized bowel (Fig. 2). Reduction of the volvulus by colonoscopy was unsuccessful in all of the 3 attempted cases.
Treatment of colonic volvulus was surgical in all patients. Of the 6 patients with sigmoid volvulus, sigmoid resection and end-to-end anastomosis was performed in 3, subtotal colectomy with end-to-end anastomosis in 2, and end-ileostomy in 1 patient. The patient who required an end-ileostomy previously had a subtotal colectomy for a previous colonic volvulus. The child with transverse colonic volvulus had subtotal colectomy with end-to-end anastomosis. In the patients with cecal volvulus, a localized cecal resection was performed in one and right hemicolectomy with end-to-end anastomosis in the other. During laparotomy, perforation of the sigmoid colon was identified in 1 patient with sigmoid volvulus. There was no mortality. All patients continued to have their usual symptoms of CIP after their surgical treatment for mechanical volvulus except 1 patient who was lost to follow-up.
In adults, colonic volvulus accounts for only 3% to 5% of episodes of bowel obstruction (9), but no such data are available for children. The most common site of colonic volvulus in children is the sigmoid colon (10,11) followed by the cecum (11–13) and transverse colon (14,15).
The predisposing factors for the development of colonic volvulus in children include elongated or redundant colon, congenital absence of ligamental attachments at the splenic or hepatic flexures, absence or malfixation of the mesentery, high-fiber diet, and prior abdominal surgery. Other conditions associated with the increased risk of developing colon volvulus include Hirschsprung disease, poor intestinal muscle tone (prune belly syndrome), pathological aerophagia, chronic constipation, and chronic recumbencey in neurologically impaired children (12,14–16).
Almost all children in our study presented with worsening of abdominal pain and distension. Half of the patients had experienced no bowel movement for at least 3 days despite rectal suppository and rectal irrigation at home. Bilious emesis was uncommon. The common symptoms reported in children with sigmoid volvulus include abdominal pain, vomiting, constipation, diarrhea, and abdominal distension (10,11,17). Previous pediatric studies suggest that 56% to 79% of the children with colonic volvulus who do not have a bowel motility disorder present with abdominal distension (10,17). All 8 patients in our study had gross abdominal distension, which is likely to be due to a combination of CIP and colonic volvulus. In our patients the symptoms of colonic volvulus were similar to those associated with previous acute exacerbation of CIP and CIC. This may have contributed to the delay in seeking medical help. Worsening of abdominal distension, unremitting abdominal pain, and failure of rectal suppository or rectal irrigation to induce a bowel movement were some of the features that suggested an acute anatomical obstruction in our patients.
The plain radiographic abnormalities reported in children with colon volvulus include a malpositioned and dilated cecum, small bowel dilation and air fluid levels, and absence of gas in the distal colon (9,18,19). Dilated loops of small bowel with air fluid levels are commonly seen in children with CIP and may not be helpful in differentiating underlying CIP from an associated volvulus. Water-soluble contrast enema was the single most informative investigation and confirmed the diagnosis in all of our patients. This is consistent with previous studies in children with colonic volvulus in whom contrast enema was used to confirm the diagnosis (9,10). When a volvulus is suspected, water-soluble contrast medium is preferred to barium because of the risk of bowel perforation and leakage of barium into the peritoneal cavity. Children with CIP who present with acute obstructive symptoms are often dehydrated and fluid resuscitation before contrast enema study should be considered.
One study used color Doppler ultrasonography to detect twisted mesenteric vessels in patients with colon volvulus (13); we did not perform Doppler ultrasound in any of our patients. The characteristic computed tomography findings include a “coffee bean,” “bird beak,” and “whirl” signs. The “whirl pattern” is composed of a spiraled loop of collapsed cecum, with low-attenuating fatty mesentery and engorged mesenteric vessels. The “bird-beak” correlates with the progressively tapering efferent and afferent bowel loops terminating at the site of torsion, and the “coffee bean” sign refers to an axial view of a dilated cecum filled with air and fluid (20).
In adults, endoscopic reduction is successful in almost half of the patients with sigmoid volvulus. We unsuccessfully attempted endoscopic reduction in 3 patients. Endoscopy may be used in an acute setting for the purpose of diagnosis, but water-soluble contrast enema is the preferred method. Fluoroscopic or endoscopic catheter drainage of the proximal dilated colon can help resolve a sigmoid volvulus, but the patient remains at high risk for recurrence. An elective or semielective sigmoidectomy with end-to-end anastomosis and/or end-ostomy should then be considered.
Clinicians treating children with CIP and CIC that can result in dilated and redundant colon should consider colonic volvulus in the differential diagnosis of acute exacerbations of the underlying disorder. Worsening of abdominal pain and distension with failure to pass a bowel movement despite rectal treatment are some of the clinical features that should raise the suspicion of an underlying volvulus. Water-soluble enema helps to confirm the diagnosis.
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