Esophageal Perforation and Rupture

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Background

• Esophageal Rupture - spontaneous perforation, Boerhaave syndrome, effort perforation, or esophageal apoplexy. No instrumentation or intubation of the esophagus. Suggestive of intrinsic anatomical abnormality or dysfunction present within the esophageal wall (i.e. EoE with food impaction can rupture in the setting of forceful vomiting)
  
• Esophageal Perforation - direct result of instrumentation or object in the esophageal lumen (Traumatic) often a result of Dx or Tx manipulation (Iatrogenic) 0.4-1.2% complication rate for esophagoscopy and esophageal dilation procedures.  See causes below

Causes of esophageal perforation and rupture

Trauma	Iatrogenic	Other
MVC GSW Child Abuse	Cardiac Massage Heimlich NG Tube placement Laryngoscopy or ET Tube intubation Excessive vigorous postpartum sxn Difficult Upper Endoscopy Sclerotherapy of esophageal varices Esophageal compression by a cuffed ET tube Dilation for Tx of achalasia or strictures	s/p Forceful Vomiting, coughing, straining s/p Esophageal injury due to caustic ingestion Foreign body ingestion Food impaction Pill esophagitis EoE Drinking Cold, carbonated beverages rapidly

Clinical Manifestations

• Esophageal Rupture in Neonate - Full term, uncomplicated pregnancy (most frequent) Respiratory Distress w/ cyanosis may be present at birth or may develop hrs later.
  
• Respiratory Distress caused by PTX (typically R sided). tension may develop
• Boerhaave will, more frequently in infants, develop as R sided PTX due to close adherence of aorta which provides additional support on L side of esophagus at that age
  
• If respiratory distress not initially present, Sx may manifest with first feeding
• cough
• vomiting
• choking
• mild hematemesis
• Thoracentesis or tube thoracostomy may reveal serosanguineous or grossly bloody pleural fluid or contents of feeding. Breastmilk/formula will appear in thoracostomy tube promptly if feeding attempted
• Esophageal Rupture in Neonate: Premature infant with difficulty passing NG tube (63% of cases) 75% of patients will show:
• difficulty swallowing
• drooling
• increased oral secretions
• feeding problems
• Older children (typically left sided lesion - distal left lateral esophageal wall where smooth muscle layer is the weakest)
  
• severe pleuritic or substernal chest pain is the hallmark of perforation due to esophagoscopy or dilatation procedures
• Fever
• Toxic progression to shock
• Subcutaneous emphysema (rare in infants unless seen with massive pneumomediastinum) crepitus
• may have cold water polydipsia

Dx

• Rapid and accurate diagnosis essential  - requires imaging
• Plain films - PA and Lat (consider CT of neck and chest if plain film indeterminate)
• typical appearance of  rupture and traumatic injury is Right Sided Tension PTX or hydropneumothorax on upright radiograph or pneumomediastinum
• Iatrogenic perforation may reveal malpositioning of NG tube usually into R pleural cavity, may also be in submucosal or mediastinal position causing a "pseudodiverticulum" of the esophagus
• Perforation during esophageal dilation - look for pneumomediastinum or Left chest pneumothorax
• Lateral chest film may show mediastinal air early
• Premature infants with iatogenic perforation may be thought to have esophageal atresia until NG or OG tube returns blood-tinged secretions and tube displaced away from midline on plain film
• Esophagography - required to establish Dx, localize perforation, and direct Tx
• Esophagogram with water-soluble contrast media
• esophagograms will miss >30% of cervical perforations
• negative water soluble study (or if anatomic definition inadequate) follow with a Barium study
• +barium study will identify smaller defects but can lead to higher risk of inflammatory mediastinitus
• note: surgeons do not like barium in the thorax!
• Alternative: CT with oral contrast
• Esophagoscopy: 30% false negative rate, may enlarge perforation and compromise effective repair

Tx

• Decision regarding appropriate therapy depends on:
• Location: site of perforation
• If perforation is free or contained
• Time interval from injury to Dx
• Response to Injury
• Spontaneous rupture not related to instrumentation
• usually results in substantial contamination of mediatinum or pleural space
• resulting in systemic response of fever, sepsis, pleuritic pain, +/- shock
• Preop Prep
• NPO
  
• IV fluids
• Abx
• Thoracentesis
• Tube Thoracostomy
• Operative Procedure
• Esophagography - define location of perforation
• Thoracotomy immediately after esophagography
• Irrigation of pleural space
• Suture or patch of the laceration if found
• Flex esophagoscopy during the exploration may help localize the perforation
• A flap of pleura or pericardium can be used to reinforce the esophageal closure
• Placement of multiple Large chest tubes
• pleural cavity is generously irrigated and drained with chest tubes
• in some cases ancillary procedures are used including (not extensively used in children):
• diverting or tube cervical esophagostomy
• esophageal division without anastomosis
• gastrostomy
• feeding jejunostomy
• stenting techniques
  
• note: free pleural perforation seconday to instrumentation should be managed in the same way
• Perforation caused by placement of ET or NG tube (Iatrogenic instrumentation)
• Can be managed nonsurgically
• May affect mediastinum only or may extend into the pleural space (R sided)
• Remove NG Tube and replace under fluoroscopy with contrast injected to confirm placement in stomach
• Chest tube if PTX
• Abx
• TPN,
  
• advance to NG tube feeding after 2-3 days
• If reflux occurs, advance through pylorus to feed duodenum
  
• NPO
• Monitor for Sepsis which suggests mediastinal abscess, confirm with CT if positive, plan for surgical drainage
• Regardless of treatment type (surgical or nonsurgical)
• Contrast esophagography performed 7-10 days after injury or repair
• if completely healed, chest/mediastinal tubes removed and PO feeding resumes
• if extravasation is observed, continue conservative therapy for another 7-10 days
• persistence of fistula suggests distal esophageal or gastric outlet obstruction

Complications

•  Late stricture occasionally encountered at the site of surgical repair which responds well to dilation
• several case reports emphasize the uniform mortality with nonsurgical therapy although many authors advocate nonsurgical therapy for upper esophageal perforations
• when nonsurgical therapy selectively applied to infants with no systemic symptoms of perforation (submucosal or well contained in mediastinum, so called pseudodiverticulum of esophagus), the survival rate approaches 100%

References:

1. Kliegman, Robert. Nelson Textbook of Pediatrics. Edition 21. Philadelphia, PA: Elsevier, 2020.
2. Tortora, Gerard J. Principles of Anatomy and Physiology. 15th ed. Hoboken, NJ: J. Wiley, 2009. Print.
3. Moore, Keith L.,, Arthur F. Dalley, II, and Keith L Moore. Clinically Oriented Anatomy. Fifth edition. Baltimore: Wolters Kluwer Health, 2009. Print.
4. Kleinman RE, Goulet O, Mieli-Vergani G, et al, eds. Pediatric Gastrointestinal Disease: Pathophysiology, Diagnosis, and Management. 5th ed. Hamilton, Ontario: BC Decker; 2008.
5. The NASPGHAN fellows concise review of pediatric gastroenterology, hepatology, and nutrition. 1st edition (2011)
6. Wyllie, Robert & Hyams, J.S.. (2011). Pediatric Gastrointestinal and Liver Disease. 10.1016/C2009-0-53242-4.  (Accessed online Feb 2020)
7. Coran, Arnold G, and N S. Adzick. Pediatric Surgery. Philadelphia, PA: Elsevier Mosby, 2012. Internet resource.