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Cystic duplications of the esophagus and neurenteric cysts
Insights
This review of 19 pediatric cases highlights that intraspinal anomalies often accompany mediastinal masses and can be asymptomatic. Myelography is recommended for esophageal duplications with vertebral anomalies.
Area of Science:
- Pediatric Surgery
- Thoracic Surgery
- Gastrointestinal Surgery
Background:
- Cystic duplications of the esophagus are rare congenital anomalies.
- These lesions can present with thoracic or neurological symptoms, or be incidentally discovered.
Purpose of the Study:
- To review 25 years of experience with pediatric cystic duplications of the esophagus.
- To analyze clinical presentation, diagnostic methods, surgical management, and outcomes.
Main Methods:
- Retrospective review of 19 pediatric patients treated at The Hospital for Sick Children, Toronto.
- Analysis of clinical data, imaging studies (myelography, technetium scans), and surgical findings.
Main Results:
- 19 patients (9 <1 year) treated; 15 had chest masses, 4 neurological symptoms only.
- 14 lesions had associated vertebral anomalies; 8/9 myelograms showed abnormalities.
- 9/15 chest masses contained gastric mucosa; 95% long-term survival. Intraspinal anomalies co-existed in ~25%.
Conclusions:
- Intraspinal anomalies frequently co-exist with mediastinal masses and may be asymptomatic.
- Myelography is recommended for all patients with cystic duplications of the esophagus and vertebral anomalies.
- Technetium scans can aid diagnosis when clinical presentation is unclear.
Abstract:
This paper reviews the past 25 years of experience with these lesions at The Hospital for Sick Children, Toronto, during which 19 infants and children were treated. There were 8 females and 11 males. Nine of these patients were under 1 year of age at presentation, 5 were between the ages of 1 and 10 years, 5 were older than 10 years. Eleven presented with symptoms referrable to the chest, and six with predominantly neurological symptoms. Two asymptomatic children were referred because of chest masses found incidentally on chest x-rays. Fifteen chest masses were noted; the other four had neurological symptoms only. Fourteen of these 19 lesions had associated vertebral anomalies. Nine patients underwent myelograms and 8 abnormalities were demonstrated, 2 of which were neurologically asymptomatic. Four patients had both myelographic abnormalities and mediastinal masses. Two out of three technetium scans were positive. Fourteen thoracotomies and six laminectomies were carried out. Nine of the 15 chest masses contained gastric mucosa including 2 which had perforated. None of the intraspinal masses contained gastric mucosa. The long-term survival was 95%. In this series, intraspinal anomalies co-existed with mediastinal masses in almost 25% of patients and were often initially asymptomatic. It is recommended that myelography be carried out in all patients with cystic duplications of the esophagus who have vertebral anomalies. Technetium scans may be useful if the diagnosis is obscure.