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Journal of Pediatric Surgery (2012) 47, 15721575

www.elsevier.com/locate/jpedsurg

Proximal large bowel volvulus in children: 6 new cases and


review of the literature
Semiu Eniola Folaranmi a , Alex Cho a , Farhan Tareen b , Antonino Morabito a ,
George Rakoczy a , Tamas Cserni a,b,c,
a

Royal Manchester Children's University Hospital, Manchester, United Kingdom


Temple Street University Children's Hospital, Dublin, Ireland
c
Medical Health Science Center, University of Debrecen, Hungary
b

Received 26 August 2011; revised 23 October 2011; accepted 23 October 2011

Key words:
Large bowel volvulus;
Cecal volvulus;
Neurodevelopmental delay;
Chronic constipation

Abstract
Background: Proximal large bowel volvulus is considered as an extremely rare surgical emergency in
children. Approximately 40 cases have been reported, and because of its rarity, the diagnosis is often
missed or delayed. The purpose of this study was to review the presentation, treatment, and clinical
outcome of proximal large bowel volvulus.
Methods: A systematic review and analysis of the data relating to 6 patients from the author's practice
and cases published in the English literature from 1965 to 2010 was performed. Detailed information
regarding demographics, clinical presentation and methods of diagnosis, surgical procedure,
complications, and outcome were recorded.
Results: Thirty-six cases of proximal large bowel volvulus were retrieved from the English literature,
and 6 cases, from the author's practice. The male-female ratio was 1:1, with a median age of 10 years.
There were 29 (69%) cases with neurodevelopmental delay. Clinical presentation included 29 (69%)
cases with constipation, 41 (98%) with colicky abdominal pain, 42 (100%) with abdominal distension,
and 35 (83%) with vomiting. Plain radiography was specific in 64% (27/42) of cases, barium enema in
100% (15/15), and computed tomography in 100% (2/2). All patients underwent surgery, with resection
and primary anastomosis in 24 (57%) cases, stoma formation in 11 (26%), and detorsion of volvulus
without resection in 7 (17%) cases. Six patients (14%) died postoperatively.
Conclusion: A child with neurodevelopmental delay and a history of constipation presenting with an
acute onset of colicky abdominal pain and progressive abdominal distension with vomiting should be
suspected of having a cecal and proximal large bowel volvulus.
2012 Elsevier Inc. All rights reserved.

Large bowel volvulus occurs when the bowel undergoes an


axial twist on its mesentery, compromising its blood supply
[1]. The site of torsion may occur anywhere from the cecum to
Corresponding author. Medical Health Science Center, University of
Debrecen, Hungary.
E-mail address: tcserni@yahoo.com (T. Cserni).
0022-3468/$ see front matter 2012 Elsevier Inc. All rights reserved.
doi:10.1016/j.jpedsurg.2011.10.064

the rectum [2,3]. Volvulus of the large bowel as the cause of


intestinal obstruction is extremely uncommon and more rarely
affect the cecum. Most cases of cecal volvulus involve a
variable amount of terminal ileum and ascending colon [4].
According to Vo et al [5], cecal volvulus occurs so rarely in
children that its true incidence is unknown. Only approximately 40 children with cecal volvulus have been reported in

Proximal large bowel volvulus in children


the English literature [6]. The rarity of this condition may lead
to a delay in its diagnosis and treatment. Untreated, the
volvulus of the cecum becomes gangrenous, necrotic, and
eventually perforates resulting in life-threatening sepsis. If the
diagnosis is made early, the condition may be amenable to
nonoperative barium enema reduction [7] or colonoscopic
decompression [8,9]. Most patients will require surgical
intervention involving simple detorsion of the volvulus with
or without fixation to the retroperitoneum or resection of
nonviable bowel followed by temporary stoma formation or
primary anastomosis and restoration of intestinal continuity.
The aim of this study was to review the presentation,
treatment, and clinical outcome of proximal large bowel
volvulus (PLBV) in childhood to aid early diagnosis.

1. Methods
We retrospectively analyzed the case notes of patients
who had a diagnosis of PLBV in 3 institutions: Royal
Manchester Children's Hospital, United Kingdom; Temple
Street University Children's Hospital Dublin, Ireland; and
Medical Health Science Centre University of Debrecen,
Hungary. We looked at patient demographics, presenting
features, investigations, definitive management, and mortality.
A PubMed search was performed for all case reports and
reviews of large bowel volvulus in children and cecal
volvulus in children. We analyzed the literature and included
cases of cecal and PLBV only. We gathered information under
the same headings of demographics, presenting features, investigations, definitive management, and mortality.

2. Results
There were 6 cases from our institutions and 36 cases
identified in the English literature of cecal and PLBV.
The median age was 10 years with a range of 0 to 18
years. There was no sex predominance with a male-female
ratio of 1:1. Twenty-nine (69%) patients had neurodevelopmental delay (ND), 29 (69%) presented with constipation, 41
(98%) with colicky abdominal pain, 42 (100%) with
abdominal distension, and 35 (83%) with vomiting (see
Table 1). All of the patients with constipation with the
exception of 1 had ND. All patients were investigated with a
plain abdominal radiograph, but this was only diagnostic in
27 (64%) cases. The main finding on plain radiography was
grossly distended bowel (Fig. 1). Fifteen patients underwent
a barium enema, and 2 patients had an abdominal/pelvis
computed tomographic (CT) scan, with both investigation
modalities having 100% specificity. The barium enema
findings were typically that of a bird's beak deformity
representing the cutoff point in the large bowel (Fig. 2).
Computed tomography was able to demonstrate the dilated
segment of large bowel with an air-fluid level (Fig. 3). All

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Table 1 Summary of demographics, clinical features,
investigations, management, and mortality
Clinical features

Our
series

Published Summary
data

No. of patients
Age (y), median (range)
Male-female
Neurodevelopmental delay
History of constipation
Colicky abdominal pain
Abdominal distension
Vomiting
Plain abdominal radiograph
Barium enema
Surgery
Mortality
Cecum involved
Terminal ileum involved
Ascending colon involved
Transverse colon involved

6
12 (3-14)
2:4
5/6
6/6
6/6
6/6
5/6
6/6
1/6
6/6
1/6
6/6
1/6
4/6
3/6

36
9.5 (0-18)
19:17
24/36
23/36
35/36
36/36
30/36
36/36
14/36
36/36
5/36
26/36
4/36
12/36
14/36

42
10 (0-18)
21:21
29/42
29/42
41/42
42/42
35/42
42/42
15/42
42/42
6/42
32/42
5/42
16/42
17/42

patients underwent surgery, with resection and primary


anastomosis in 24 (57%) cases, stoma formation in 11 (26%)
cases, and detorsion of volvulus without resection in 7 (17%)
cases. In our series, intraoperatively, we found that all of the
patients were extremely dilated and heavily loaded with
feces. In addition, all of the patients had a volvulus of the
cecum with a variable amount of ascending and transverse
colon involvement; however, only 26 of 36 patients had
cecal involvement among the published cases. One patient
had involvement of the terminal ileum in addition to the cecal
volvulus. Six patients (14%) in total died postoperatively.
The time delay from presentation to operative intervention
was not made clear in all cases in the published literature.
There was 1 death in our series as a result of overwhelming
sepsis because of a chest infection and an abdominal collection, 2 weeks postoperatively. There were 5 deaths among the
previously published cases. One child aspirated and died on
the fourth day postoperatively. The second mortality was in a
10-year-old girl who had extensive bowel gangrene. The third
case was a 2-year-old boy who developed a fecal fistula on
day 16 postoperatively and died because of electrolyte
imbalance and cachexia on day 25 postoperatively. The
fourth case was caused by a serious lower respiratory tract
infection, and the child died 28 days postoperatively. The fifth
patient was a patient with trisomy 18 who died 2 years
postoperatively from influenza encephalopathy.
The breakdown of the anatomical segment involved in the
volvulus is as follows: cecum 32, terminal ileum 5, ascending colon 16, and transverse colon 17 (Table 1).

3. Discussion
Colonic volvulus was first described by Rokitansky in
1836. The sigmoid, cecal, and transverse colon volvulus are

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S.E. Folaranmi et al.

Fig. 2 Barium enema showing the classic bird's-beak deformity


in another child with cecal volvulus including transverse colon. The
arrow points to the bird's-beak deformity.

Fig. 1 Figs. 1 and 2 are findings in a patient with neurodevelomental delay and chronic constipation. The extremely dilated large
bowel (not only cecum but ascending, transverse, and descending
colon as well) was loaded with feces. The volvulus resulted in an
extremely dilated and necrotic cecum. The ascending colon was
involved half way up to the hepatic flexure. The rest of the colon was
dilated as well, but fully viable. Because of the chronic constipation
and fecal loading, the twisted colon was more dilated than the
intestine proximal of the obstruction. Fig. 1. is a plain abdominal
radiograph showing large bowel distension. One arrow points to the
distended bowel loop, whereas the other points to the fecal impaction.
Fine gas pattern can be seen in the transverse colon and rectum. This
is consistent with massive fecal impaction and dilatation.

relatively rare and are responsible for approximately 3% to


5% of all large bowel obstructions in adults and an even
lower percentage in children [10].
Sigmoid colon volvulus is the commonest form of colonic
volvulus, followed in frequency by cecal volvulus and then
volvulus of the transverse colon. Transverse colon volvulus
is much less frequent, accounting for less than 4% of all
reported cases of colonic volvulus [11].
Interestingly, the incidence of intestinal malrotation is
1:500 live births, a mobile cecum and loose mesocolon have
been described even more commonly (15%-26%) in otherwise healthy children [12,13]. Small bowel volvulus is much
more frequent than large bowel volvulus, and cecal volvulus
is quite rare. In our study, the median age of patients with
cecal and PLBV was 10 years. Fifty-five percent of patients
with intestinal malrotation present within the first week of
life with life-threatening complications of volvulus and 80%

manifest symptoms during the first month [14]. These data


suggest that intestinal malrotation is not the major cause of
large bowel volvulus and the predisposing factor leading to
large bowel volvulus is an acquired anomaly rather than a
congenital one.
Neurodevelopmental delay and severe chronic constipation
were found in our study to be the most commonly associated
disorders. This was also noted in a smaller series [12].
It is highly likely that severe stool accumulation in the
large bowel of mentally impaired children because of insufficiently treated chronic constipation results in a heavy and
extremely dilated bowel.
We hypothesize that this may stretch the ligaments
responsible for fixation. By the time the mesentery of the
colon becomes longer, a volvulus can occur.

Fig. 3 Computed tomographic scan demonstrating large bowel


distension with an arrow pointing to the air-fluid level. The septum
seen between the dilated air-fluidfilled loops suggests a volvulus.

Proximal large bowel volvulus in children


Children with colonic volvulus typically present with an
acute onset of abdominal pain and obstruction or chronic
intermittent symptoms such as cramps, bloating, nausea, and
vomiting that increase in severity [13]. Most, if not all, of the
children in our series and children in the published literature
had colicky abdominal pain, abdominal distension, and
vomiting. Unfortunately, these symptoms occur quite
frequently in children with ND and chronic constipation
without volvulus and may respond to enema and laxatives.
The rarity of the volvulus and the lack of adequate
communication in a child with ND is the main reason why
the diagnosis of volvulus may be delayed. Plain abdominal
radiography may be helpful if it demonstrates extreme
colonic dilatation and a relative absence of gas in the colon
distal to the obstruction. The typical finding on contrast
enema is a narrowed, twisted colon with a bird's-beak
deformity. The bird's-beak appearance comes from the
specific shape of the twisted gut. The lumen gradually
narrows at the site of torsion and comes to a sharp end where
the lumen is completely occluded. The grossly dilated bowel
segment with air-fluid level separated with septa has been
considered as specific features of volvulus on CT scan.
Contrast enema in severe chronic constipation is not only
diagnostic but also therapeutic because gastrografin is
known to aid fecal dilution [13]. Contrast enema is not
appropriate in children with evidence of peritonitis or septic
shock. Computed tomographic scan became more available
in the last decade, but many surgeons would prefer to
proceed with laparotomy when peritonitis is noted. This and
the additional anesthesia required for the scan may explain
why CT has not been offered more frequently before surgery.
All of the patients had a plain abdominal radiograph
performed as an initial investigation. Only 38% of patients,
however, had a barium enema as a follow-up investigation.
Early diagnosis significantly influences the outcome in
patients with PLBV. Conservative management of large
bowel volvulus by contrast enema may be successful only
at an early stage. Operative detorsion, decompression of
the involved bowel, resection of the compromised bowel,
primary anastomosis, or temporary fecal diversion in the
form of a colostomy was performed in most of the cases.
This may be because (as discussed above) early diagnosis
is difficult.

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The overall mortality rate of 14% in this study is relatively
high compared with the mortality rate of 9% in patients with
intestinal malrotation [15]. This may be caused by the fact
that patients who develop PLBV tend to have comorbid
conditions such as neurodevelopmental impairment that predisposes them to a more poor postoperative recovery.
Despite PLBV being more common in neurologically
impaired children, it should be considered in any chronically
constipated child with fecal loading, who presents with acute
onset of colicky abdominal pain and vomiting.

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