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Title: A Pediatric Contrast Enema in management of Hirschsprung Disease.



Wellington Bwanali


October 2014

1. Introduction
Hirschsprung disease is the congenital absence of ganglion cells in the rectum, and
sometimes part or all of the large intestine and the lack of these cells causes an obstruction of
the large bowel. It is known as aganglionosis because one of the main characteristics in all
the patients is that they do not have ganglion cells in the rectum. Wagner and Katz (2014)
wrote that both mesenteric and submucosal plexuses are absent in patients with Hirschsprung
According to Haller, Slovis and Josh (2005), infants affected with Hirschsprung disease may
present with abdominal distension, repeated vomiting and failure of passage of meconium
within the first 48 hours of life.

Older infants and children typically present with chronic

constipation that is often refractory to usual treatment protocols and may require daily
enema therapy. Hirschsprung enterocolitis can be a fatal complication of Hirschsprung
disease. It typically presents with abdominal pain, fever, foul-smelling and/or bloody
diarrhoea, as well as vomiting. Enterocolitis may progress to sepsis, transmural intestinal
necrosis, and bowel perforation.

The Patients Clinical History

Patient ABC is an 8 year old male who was referred from Mpilo Hospital with a provisional
diagnosis of Hirschsprung disease. The patient was initially admitted at Mpilo hospital 3 months
ago after presenting with gross abdominal distension. The history of neonatal meconium plug
was not clear. The patient had a 8-year history of abdominal distension that is associated with
periods of constipation. The patient was having bouts of foul-smelling and yellowish diarrhoea
(about 10 bouts daily), with occasional vomiting. He was being managed with oral
dehydration, Zinc Sulphate medication and a number of bowel washout procedures.
The patient is HIV positive and is on anti-retroviral therapy. A chest radiograph that was taken
was normal, and there was no evidence of abdominal ascites. A rectal biopsy was taken to
confirm Hirschsprung disease, and the tests were positive. Hirschsprung disease and
Hirschsprung enterocolitis was diagnosed. A pre-operative contast study was scheduled in
order to look for a transition zone, so that the region of bowel affected may be determined.



The Examination

A Gastrografin contrast enema was done.




An IAE Direct Digital Radiography system was used.

Agfa Computed Radiography Cassettes and Digital Imager

Catheterising equipment; Foleys Catheter

30ml syringes and needles

Gloves, tape,

According to Chapman and Nakielny (2014), dilute contrast medium ionic contrast medium is
ideal, because it has the advantage of not provoking large fluid shifts and is dense enough to
provide satisfactory images.
Therefore, 30ml of Gastrografin diluted with normal saline was used.

Patient Preparation

The child was well hydrated prior to the procedure. A large bowel washout was done 6 hours
before the examination.


A preliminary film was taken to check positioning, exposure factors and patient bowel
preparation. Plain abdominal radiographs were taken to show possible distended bowel
loops and the expected transmission zones. With the patient lying on the left side, the
catheter was placed just inside the anus without inflation of the balloon to avoid distortion of
a low transition zone and perforation. Radiographs are taken immediately after hand
injection of contrast by the surgeon. Supine and lateral radiographs after administration of
contrast were taken.




The arrows show the transition zone in the proximal rectum and sigmoid colon. The Left lateral
image shows a distended ascending colon and splenic flexure.


According to the findings of the consultant surgeon, a narrowed transition zone was
apparent in the proximal rectum and sigmoid colon regions. Retention of rectal contrast 24
hours after the contrast enema was done also confirmed the diagnosis of Hirschsprung


The classic finding of Hirschsprung disease is a narrowed distal colon with proximal dilation, as
was the case in patient ABC. Therefore the disease was confirmed and the narrow transition
zone was determined.
6.0 Discussion
Hirschsprung disease is normally diagnosed in neonates and infants below 3 years. In patients
with Hirschsprung disease, both mesenteric and submucosal plexuses are absent. The anus is
invariably affected, and aganglionosis continues proximally for a variable distance (Wagner
and Katz, 2014). Less commonly, like in the case of patient ABC, older children may be

diagnosed with Hirschsprung disease. They may present chronically malnourished, usually
accompanied by diagnosis of Hirschsprung enterocolitis. Hirschsprung disease is confined to
the rectosigmoid region in about 75% of cases. This was the case with patient ABC. According
to Haller et al (2005), approximately 60% of infants with Hirschsprung disease have an
associated condition, ranging from subtle to severe. In this case the patient had associated
Hirschsprung enterocolitis diagnosed with biopsy.
Wagner and Katz (2014) highlighted the following conditions in the differential diagnosis of
Hirschsprung disease:

Low intestinal obstruction, Intestinal Pseudo-Obstruction and Intestinal Motility Disorders

Ileus and Colonic atresia

Constipation and Irritable Bowel Syndrome

Acute Megacolon, Chronic Megacolon and Toxic Megacolon

However, in the case of patient ABC, the biopsy ruled out other cases of differential diagnosis
and confirmed Hirschsprung disease.


Tests: An anorectal manometry is sometimes done to detect the relaxation reflex of the
internal sphincter after distension of the rectal lumen. This normal inhibitory reflex is presumed
absent in patients with Hirschsprung disease. However, a full-thickness rectal biopsy is a
definitive diagnosis of Hirschsprung disease, as it demonstrates the absence of ganglion cells.
The disadvantages of full-thickness rectal biopsy include the frequent necessity of general
anaesthesia and risks of bleeding and scarring.
In the case of patient ABC, a simple suction rectal biopsy was used to obtain tissue for
histologic examination. Rectal mucosa and submucosa were sucked into the suction device,
and a self-contained cylindrical blade excised the tissue. The distinct advantage of the
suction biopsy is that it can be easily performed at the bedside. However, the diagnostic yield
of the full-thickness rectal biopsy is significantly better than that of the suction biopsy.
Therefore, the diagnosis of a simple suction biopsy can be aided by the contrast enema to
obtain further confirmation. The contrast enema will then demonstrate the affected region of
the colon.

Given that patient ABC was diagnosed with Hirschsprung disease at the age of 8, the
surgeons determined that it was most likely to be limited to the distal sigmoid and proximal
rectum; because, had it been affecting a larger part of the large intestine, the condition
would not have continued for 8 years without causing complications that could have
necessitated immediate intervention earlier in the life of the patient.
Surgical Operation: A transanal endorectal full thickness dissection and pull-through surgical
procedure was done on patient ABC. The procedure was successful. The patient was
monitored 6 days post-operation and was discharged and referred back to Kwekwe hospital
for monitoring and check-ups.


The radiological examination was limited due to lack of radiologist expertise. The surgeon
insisted in limiting the view to an AP and Lateral views, and a 24 hour delayed AP view. The
procedure was done using spot films. Intermittent screening using fluoroscopy would have
helped to check the progress of the contrast, however it was not available. Nevertheless, the
surgeon did not intend to get the contrast all the way to the descending colon, and further
images were deemed unnecessary for radiation protection.
According to Baert, Knauth and Sartor K (2008), findings are difficult to interpret because 25%
cases do not show the transition zone clearly. Therefore, expert interpretation of images was
essential. Barium sulphate would have optimally demonstrated the transition zone, however,
we were limited to the use of water soluble contrast because the patients condition risked
bowel obstruction if Barium was used; needless to highlight that it was a paediatric


Despite limited resources, the procedure was helpful in determining the transition zone due to
the disease. There were potential complications due to the use of iodinated contrast media.
However, there were no problems encountered.




Wagner J P, Katz J, 2014. Medscape: Hirschsprung Disease. WebMD LLC. Reuters Health
Information. California

Chapman S, Nakielny R, 2014. Chapman and Nakielnys Guide To Radiological Procedures.

6th Edition. Watson N (Ed.) Elsevier-Saunders. London

Betts J B, Grossman H, 1998. Duke Radiology Case Review : Imaging, Differential diagnosis,
and Discussion. Provenzale J M, Rendon C N (Eds.) Lippincott Raven Publishers.

Haller J, Slovis T, Josh A, 2005. Pediatric Radiology. 3rd Edition, Springer. Berlin.

Baert A L, Knauth M, Sartor K, 2008. Radiological Imaging of the Digestive Tract in Infants and
Children. In: MEDICAL RADIOLOGY: Diagnostic Imaging. Devos A S, Blickman J G, (Eds.)
Springer. New York.