3 Discussion
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 eighth 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 from concomitant total colonic aganglionosis and small
bowel atresia.
Therefore, it appears rational 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). 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.