Is Barium Enema Reliable for the Diagnosis of Total Colonic Aganglionosis?

Abstract Background Preoperative diagnosis of total colonic aganglionosis is important for the rational choice of treatment. The present study aimed to evaluate the diagnostic performance of radiographic signs on preoperative barium enema in patients with total colonic aganglionosis. Methods Forty-four patients [41 (3-659) days] with total colonic aganglionosis, including 17 neonatal patients, who received preoperative barium enema at our center, from January 2007 to December 2019 were included. All radiographs were retrospectively restudied by 2 pediatric radiologists to ascertain radiographic signs including rectosigmoid index, transition zone, irregular contraction, gas-filled small bowel, microcolon, question-mark-shaped colon and ileocecal valve reflux. Kappa test was performed to assess the accuracy and consistency of the radiographic signs. Results The 2 radiologists showed slight agreement for gas-filled small bowel, microcolon and rectosigmoid index, fair agreement for transition zone and irregular contraction, and moderate agreement for question-mark-shaped colon and ileocecal valve reflux (Kappa values, 0.043, 0.075, 0.103, 0.244, 0.397, 0.458 and 0.545, respectively). In neonatal patients, the 2 radiologists showed moderate agreement for ileocecal valve reflux and substantial agreement for question-mark-shaped colon (Kappa values, 0.469 and 0.667, respectively). In non-neonatal patients, the 2 radiologists showed substantial agreement for ileocecal valve reflux (Kappa value, 0.628). In 36 patients with total colonic aganglionosis extending to the ileum, the accuracies of question-mark-shaped colon, ileocecal valve reflux and the combination of both were 47%, 53%, and 75%, respectively, in one radiologist and 53%, 50% and 72%, respectively, in the other. Conclusion Ileocecal valve reflux was a reliable radiographic sign for diagnosing total colonic aganglionosis and could improve the accuracy by combination with question-mark-shaped colon.

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Barium enema findings in total colonic aganglionosis: a single-center, retrospective study

BMC Pediatrics ◽

10.1186/s12887-020-02403-3 ◽

2020 ◽

Vol 20 (1) ◽

Author(s):

Jiayu Yan ◽

Jihang Sun ◽

Rongchang Wu ◽

Sarah Siyin Tan ◽

Yongwei Chen ◽

...

Keyword(s):

Small Bowel ◽

Transition Zone ◽

Barium Enema ◽

Ileocecal Valve ◽

Substantial Agreement ◽

Total Colonic Aganglionosis ◽

Question Mark ◽

Kappa Test ◽

Moderate Agreement ◽

Choice Of Treatment

Abstract Background Preoperative diagnosis of total colonic aganglionosis is important for the rational choice of treatment. The present study aimed to evaluate the diagnostic performance of radiographic signs on preoperative barium enema in patients with total colonic aganglionosis. Methods Forty-four patients [41 (3-659) days] with total colonic aganglionosis, including 17 neonatal patients, who received preoperative barium enema at Beijing Children’s Hospital, from January 2007 to December 2019 were included. All radiographs were retrospectively restudied by 2 pediatric radiologists to ascertain radiographic signs including rectosigmoid index, transition zone, irregular contraction, gas-filled small bowel, microcolon, question-mark-shaped colon and ileocecal valve reflux. Kappa test was performed to assess the accuracy and consistency of the radiographic signs. Results The 2 radiologists showed slight agreement for gas-filled small bowel, microcolon and rectosigmoid index, fair agreement for transition zone and irregular contraction, and moderate agreement for question-mark-shaped colon and ileocecal valve reflux (Kappa values, 0.043, 0.075, 0.103, 0.244, 0.397, 0.458 and 0.545, respectively). In neonatal patients, the 2 radiologists showed moderate agreement for ileocecal valve reflux and substantial agreement for question-mark-shaped colon (Kappa values, 0.469 and 0.667, respectively). In non-neonatal patients, the 2 radiologists showed substantial agreement for ileocecal valve reflux (Kappa value, 0.628). In 36 patients with total colonic aganglionosis extending to the ileum, the accuracies of question-mark-shaped colon, ileocecal valve reflux and the combination of both were 47%, 53%, and 75%, respectively, in one radiologist and 53%, 50% and 72%, respectively, in the other radiologist. Conclusions Ileocecal valve reflux is a relatively reliable radiographic sign for diagnosing total colonic aganglionosis and could improve the diagnostic accuracy upon combination with question-mark-shaped colon.

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Two Siblings with Total Colonic Aganglionosis Extended to the Ileum

Swiss Surgery ◽

10.1024/1023-9332.9.4.187 ◽

2003 ◽

Vol 9 (4) ◽

pp. 187-189

Author(s):

Aslan ◽

Caglar ◽

Karagüzel ◽

Melikoglu

Keyword(s):

Intestinal Obstruction ◽

Loop Ileostomy ◽

Good Condition ◽

Disease Type ◽

Definitive Treatment ◽

Entire Colon ◽

Total Colonic Aganglionosis ◽

Protective Ileostomy ◽

Definitive Surgery ◽

Partial Intestinal Obstruction

Total colonic aganglionosis (TCA) extended to the ileum is seen quite rare among infants with Hirschsprung's disease. Type and timing of definitive surgery in these patients are controversial. This report was presented to discuss the management of two siblings with TCA. Case 1: A two-day-old girl was operated for partial intestinal obstruction. During laparotomy, serial frozen biopsies proved TCA extended to the terminal ileum and a loop ileostomy was performed. At five months of age, a modified Duhamel-Martin procedure without protective ileostomy was performed. An endo-GIA stapler was transanally used for colo-ileal anastomosis. She is doing well for the last five years. Case 2: A one-day-old boy admitted to the hospital with similar findings to his sister. Frozen biopsies during first laparotomy proved that majority of ileum and entire colon was aganglionic and a proximal ileostomy was performed. At 10 months of age, he underwent a similar Duhamel-Martin operation. He is in a good condition for the last four years. Conclusion: In infants, our modification on Duhamel-Martin procedure, which is based on the use of an endo-GIA stapler transanally for colo-ileal anastomosis without protective ileostomy, may be utilized as an alternative method in the definitive treatment of patients with TCA.

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Analysis of air contrast barium enema, computed tomographic colonography, and colonoscopy: prospective comparison

The Lancet ◽

10.1016/s0140-6736(05)70194-x ◽

2005 ◽

Vol 365 (9456) ◽

pp. 305-311 ◽

Cited By ~ 284

Author(s):

D ROCKEY ◽

E PAULSON ◽

D NIEDZWIECKI ◽

W DAVIS ◽

H BOSWORTH ◽

...

Keyword(s):

Barium Enema ◽

Computed Tomographic Colonography ◽

Computed Tomographic ◽

Prospective Comparison

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Enterogastroesophageal reflux during barium enema : report of a case

Journal of the Korean Radiological Society ◽

10.3348/jkrs.1972.8.3.191 ◽

1972 ◽

Vol 8 (3) ◽

pp. 191

Author(s):

HJ Shin ◽

HS Rhee

Keyword(s):

Barium Enema

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Diagnosis of Hirschsprung's Disease: Accuracy of Barium Enema Findings

Journal of the Korean Radiological Society ◽

10.3348/jkrs.1997.36.4.631 ◽

1997 ◽

Vol 36 (4) ◽

pp. 631

Author(s):

Sue Yun Yu ◽

Gye Yeon Lim ◽

Ji Yeong Yun ◽

Seong Tae Hahn ◽

Hak Hee Kim ◽

...

Keyword(s):

Barium Enema ◽

Hirschsprung's Disease ◽

Hirschsprung’S Disease

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CASE REPORT: RARE FORM OF HIRSCHPRUNG’S DISEASE

10.35988/sm-hs.2020.118 ◽

2020 ◽

Vol 30 (5) ◽

pp. 82-84

Author(s):

Ilja Skalskis

Keyword(s):

Down Syndrome ◽

Case Report ◽

Clinical Symptoms ◽

Hirschsprung Disease ◽

Distal Colon ◽

Total Colonic Aganglionosis ◽

Sphincter Function ◽

Anal Sphincter Function ◽

History Of ◽

High Index Of Suspicion

Hirschsprung disease (HD) is a developmental disorder characterized by the absence of ganglia in the distal colon, resulting in a functional obstruction. Incidence of total colonic aganglionosis (TCA) is 1 in 500 000 and it accounts for 5-10% of all cases of HD. HD should be suspected in patients with typical clinical symptoms and a high index of suspicion is appropriate for infants with a predisposing condition such as Down Syndrome (DS), or for those with a family history of HD. The treatment of choice for HD is surgical, such as Swenson, Soave, and Duhamel procedures. The goals are to resect the affected segment of the colon, bring the normal ganglionic bowel down close to the anus, and preserve internal anal sphincter function. We present a clinical case report of TCA in a child with Down syndrome (DS) and review of literature.

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