Ieal Atresia and Total Colonic Hirschsprung Disease in a 36-week Neonate : Case Report

doi:10.21203/rs.3.rs-1875233/v1

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Ieal Atresia and Total Colonic Hirschsprung Disease in a 36-week Neonate : Case Report

https://doi.org/10.21203/rs.3.rs-1875233/v1

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Intestinal atresia and Hirschsprung disease are two common causes of bowel obstruction in neonates, and simultaneous occurrence is rare.

This report delineates a 36-week newborn with ileal atresia and total colonic Hirschsprung.

A newborn male (gestational week 36; birth weight,2500 gram) was referred to our unit due to failure of meconium Passage during the first 48 hours after birth, abdominal distension, and bilious vomiting.

Physical examination revealed a prominent abdomen, decreased bowel sounds, and an empty rectum in digital rectal examination.

The abdominopelvic radiograph showed dilated intestinal loops. No gas was observed in the rectum.

Intraoperative findings were: Type three Ileal atresia with classic V shape deformity in the mesentery just 10 mm proximal to the ileocecal valve. All parts of the large bowel were unused (microcolon).

H&E examination of colonic biopsies revealed the absence of Ganglion cells in all parts of the large bowel.

colonic aganglionosis should be in mind in any operated infant with small intestinal atresia repair who continues to exhibit poor bowel function after corrective surgery.

Intestinal atresia is a significant cause of primary bowel obstruction in neonates accounting for 22.4% of all cases [1]. On the other hand, Hirschsprung disease (HD) is the most frequent congenital intestinal motility disorder [2]. HD occurs when bowel segments are not supported by neuronal ganglion formation within the myenteric (Auerbach) and submucosal (Meissner) plexus, which promote gastrointestinal movements.

And in 80% of cases, the absence of ganglion cells is limited to the rectosigmoid area. However, aganglionosis may extend through the entire colon with the distal ileum (10%) or more proximal segments [3]. Also, in these cases, a water-soluble enema shows a "question mark" configuration in the foreshortened colon with no transitional zone in the colon. [4] The rectal biopsy can always confirm the diagnosis.

Concurrent small intestinal tract atresias associated with Hirschsprung are less than 1%.

In this case report, we simultaneously present an infant born with ileal atresia and Hirschsprung disease.

A 36-week male neonate was referred to the pediatric surgery service on the second day of life due to failure to pass meconium, abdominal distension, and bilious emesis.

The patient was born to a 23-year-old healthy primipara Iranian mother without a history of abortion via normal vaginal delivery. Her birth weight was 2500 grams.

On physical examination, The primary vital signs were in the normal range, bowel sounds were decreased, and the rectum was empty.

Initial abdominopelvic radiography revealed Thumb-sized intestinal loops with no gas in the rectum [Figure 1A]. Small-bowel contrast series confirmed dilated proximal blind end [Figure 1B].

Baseline biochemical and blood gases analyses were in the normal range. On the third day, with a diagnosis of intestinal obstruction, the patient underwent exploratory laparotomy after obtaining informed consent from the parent. We identified Ileal atresia with a proximal dilated, blind-ending1 cm distal bud attached to the ileocecal region (Type IIIa). [Figure 2] The colon appeared as an unused microcolon throughout. After resecting the proximal dilated ileal pouch and ileocecal part, surgery was followed by an end-to-end ileo- ascending colonic anastomosis.

Permanent Hematoxylin & Eosin (H&E) pathological examination revealed the absence of ganglion cells in the cecum, the appendix, and rectum and the abundance of ganglia in the ileum—[Figure 3].

Due to the persistence of abdominal distention and no defecation on postoperative days and histopathologic report that was compatible with total segment HD, the patient was a candidate for re-exploration. [Figure 4]

Our findings were an unused colon and a functional intestinal obstruction at the anastomosis site with no anastomotic leak.

After taking multiple levels of frozen section biopsies to reaffirm the total colonic aganglionosis, the patient underwent an ileostomy and colonic mucus fistula bypass

The final permanent pathology studies by calretinin test disclosed total aganglionic colon HD.

Swenson pull-through surgery with removal of all colon and ileoanal anastomosis was performed on three months old. The case tolerated oral feeding after five days and was discharged from the hospital in good condition.

Co-existence of ileal atresia and total colonic HD is a rare event.[5, 6] Conforming to the currently accepted theories, jejunoileal atresia arises due to intrauterine ischemic vascular events in the third trimester such as intussusception, perforation, volvulus, or thromboembolism; maternal smoking as a hypercoagulation state [7, 8] and cocaine use as vasoconstrictive medications [9]

The migration of ganglion cells was completed through the gastrointestinal tract from proximal to distal by 13 weeks postconception. Therefore, it is suggested that early gestational atresia in the 6th to eight weeks of gestation would result from an ischemic insult to interrupt the caudal migration of ganglion cells and lead to total colonic HD [10, 11]. Finding a very small micro colon, no fibrotic of the left colon, and no meconium distal to atretic segments strengthen this theory.

The other hypothesis that can justify this concurrency is an increased colonic intraluminal pressure and subsequent perforation ileocecal portion due to a developed HD and the secondary small bowel atresia. However, in our case, there was no evidence of meconium spillage into the peritoneal space during our laparotomy, which weakens the second assumption.

As the common occurrence of microcolon in the cases of distal small intestinal atresia, it is tough to differentiate this colonic appearance during surgery with concomitant total colonic aganglionosis and small bowel atresia.

Therefore, it appears rationale to do per-operative colonic biopsies looking for ganglion cells on a frozen section to exclude or confirm the underlying HD in suspicious cases.

A definitive reconstructive operation should be planned once we have established the diagnosis and done a proximal ileostomy.

However, there are controversial questions about the correct timing and the most appropriate treatment options [12, 13].

Several approaches have been described to treat this, Such as primary pull-through without ileostomy or total colectomy with standard techniques (Swenson, Duhamel, or Soave), and neither is superior to the others.

Albeit discriminating the best operative approach should be constructed based on the surgeon's level of expertise, [13] in our case, we did total colectomy with an ileoanal Swenson procedure when the patient status was allowed.

In conclusion, this rare concurrency should be considered in cases of small bowel atresia with poor bowel function after the corrective operation.

Acknowledgments

The authors would like to acknowledge all the hospital staff who assisted us in treating and studying the patient

Authors' contributions

MH designed the work and had the role of directing and supervision.

KA supervised the project process and did scientific corrections.

AS and MP prepared the manuscript and interpreted the data.

All authors read and approved the manuscript

Funding

The authors received no funds to conduct the study. It was self-funded.

Competing interests

The authors declare no competing interests relating to this original work.

Ethics approval and consent to participate

The Research Ethics Committees approved this Ardabil University of Medical Sciences study with the code: "IR.ARUMS.REC.1400.246"

Consent to Participate

Informed consent was obtained from the patient to participate.

Written Consent for Publication

The patient obtained written consent to publish this case report and any accompanying images.

Data Availability Data will be made available upon request

Code availability

Not applicable

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Ieal Atresia and Total Colonic Hirschsprung Disease in a 36-week Neonate : Case Report

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