Oesophageal pathology
Key points
Congenital oeosphageal stenosis
Mucosal webbing 16% - Upper/Middle 1/3
Fibromuscular hypertrophy 54% - Middle/Lower 1/3
Tracheobronchial rests and remnants 30% - Lower 1/3
Failure of tracheo-oesophageal separation
Possibly on the same spectrum as OA/TOF
Web + iron deficiency - Plummer-Vinson/Paterson-Brown-Kelly
Iron deficiency leads to loss of iron dependent enzymes + mucosal degeneration
Associated with autosomal recessive form of epidermolysis bullosa
Investigations
UGI contrast
Manometry to differentiate from Achalasia
Iron studies for web
Endoscopy - web will be thin eccentric lesion, normal mucosa
Management
Trial of dilatations
95% success rate for web
Unlikely to be successful in TBR
Segmental oesophagectomy if dilatations fail
Thoracoscopy or thoracotomy
Identify level with on table endoscopy
Preserve vagus nerves
TAT tube
Persistent symptoms in 50% after procedure
Oesophageal diverticula
Pharyngoesophageal - Zenker - between cricopharyngeus + inferior constrictor - pulsion - false diverticulum lacking outside muscle
Midoesophageal - complication of histoplasmosis/TB/sarcoid/lymphoma, inflammation causes external traction - true diverticula containing all oesophageal wall layers
Epiphrenic - pulsion - false diverticulum lacking outside muscle - right posterior wall - secondary to functional obstruction e.g., Achalasia, fundoplication
Also can be acquired as a result of OA/TOF
Investigations
Contrast
Endoscopy
Management
Pharyngoesophageal diverticulum - Cricopharyngeal myotomy + resection
Midoesophageal - Right thoracotomy, adhesiolysis, resection + closure in layers + intercostal flap
Epiphrenic - Left thoracotomy + stapled resection + myotomy on opposite side
Oesophageal replacement
2 most common indications:
Long gap OA - should do at 3 months if failed anastomosis (ERNICA)
Corrosive stricture
Ideal replacement:
Allows normal feeding
Permits replacement of the entire oesophagus when indicated
Does not cause respiratory compromise
Does not become tortuous or redundant
Gastro-oesophageal reflux in the conduit should be minimal
Does not have increased malignant risk
Should function normally for the life-time of the individual (i.e. over 70 years)
Gastric - most common
Should bring up through mediastinum - 2 incisions - abdomen and cervical - do not open thorax, do not perform thoracic anastomosis
Thoracic stomach will be dysmotile
Can treat with domperidone
Do pyloroplasty (Recommended by ERNICA)
Do jejunostomy
If dilated - thoracic gastroplasty
Other options:
Gastric tube (use greater curvature and rotate up)
Jejunum - cannot do if has oesophagostomy
Right hemicolon - isoperistaltic (ERNICA) - need to do fundo and pyloroplasty as well
Page edited by Mrs Charnjit Seehra BSc November 2024
References
Jensen, Amanda R, et al. "Congenital Esophageal Stenosis." Pediatric Surgery NaT, American Pediatric Surgical Association, 2020. Pediatric Surgery Library, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829675/all/Congenital_Esophageal_Stenosis.
Trappey, Francois , et al. "Esophageal Diverticula." Pediatric Surgery NaT, American Pediatric Surgical Association, 2020. Pediatric Surgery Library, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829745/all/Esophageal_Diverticula.
Dingemann C, et al. ERNICA Consensus Conference on the Management of Patients with Long-Gap Esophageal Atresia: Perioperative, Surgical, and Long-Term Management. Eur J Pediatr Surg. 2021 Jun;31(3):214-225. doi: 10.1055/s-0040-1713932. Epub 2020 Jul 15. PMID: 32668485.
Arul GS, Parikh D. Oesophageal replacement in children. Ann R Coll Surg Engl. 2008 Jan;90(1):7-12. doi: 10.1308/003588408X242222. PMID: 18201490; PMCID: PMC2216706.