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Duplications of the alimentary tract: clues to the missing links
1Department of Paediatric Surgery, All India Institute of Medical Sciences, New Delhi.
Insights
Alimentary tract duplications, particularly esophageal, were reviewed in 14 children. Histological and imaging studies suggest embryonic endoderm potential and a link between esophageal duplications and spinal defects.
Area of Science:
- Pediatric Surgery
- Developmental Biology
- Gastroenterology
Background:
- Alimentary tract duplications are rare congenital anomalies.
- Esophageal duplications constitute the majority of these malformations.
- Unexplained associations necessitate further investigation into their etiology.
Purpose of the Study:
- To review a decade of experience with alimentary tract duplications.
- To investigate the high incidence of gastric mucosa in duplication cysts.
- To explore the association between esophageal duplications and spinal pathology.
Main Methods:
- Retrospective review of 15 cases of alimentary tract duplications.
- Histological examination of duplication cyst walls.
- Myelography and computerized axial tomography scans for spinal assessment.
Main Results:
- Esophageal duplications were the most common type (55%).
- Gastric mucosa was the most frequent ectopic tissue found in cyst walls.
- Three out of four myelograms in esophageal duplication cases revealed intraspinal pathology and vertebral defects.
Conclusions:
- Embryonic endoderm's potential to form gastric mucosa explains its presence in duplications.
- The close embryonic relationship between the esophagus and vertebral column underlies associated vertebral defects.
- Findings support the split notochord syndrome theory for these malformations.
Abstract:
This is a review of the past 10 years' experience with duplications of the alimentary tract at the All India Institute of Medical Sciences, New Delhi, during which 14 infants and children with a total of 15 duplications were treated. Esophageal duplications represented the majority of cases (55%). Certain unexplained associations in this malformation led the authors to perform (1) detailed histological studies of the duplication cysts to seek explanation for the high incidence of gastric mucosa in their walls and its presence in duplications even in the distal parts of the gut, and (2) myelography and postmyelography computerized axial tomography scans to substantiate the spinal pathology. Three of four myelograms in patients with esophageal duplications showed an intraspinal pathology, and all three had vertebral defects. Histological evaluation of the cyst wall showed that gastric mucosa was the most common ectopic tissue in the walls of the cysts, and the tissues were in various primitive stages of differentiation. On the basis of these data, the authors hypothesize that (1) the embryonic endoderm of the entire alimentary tract has the potential to develop into gastric mucosa at the time of inception of duplications, which explains the high incidence of gastric mucosa and its presence in duplications even in the distal parts of the gut, (2) persistence of the embryonic relationship of the esophagus with the vertebral column is responsible for the high incidence of vertebral defects in esophageal duplications. The results of myelography in this series also support the theory of split notochord syndrome.