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Foregut Duplication Cysts
Maria Georgiades April 25, 2013 Grand Rounds
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Case Presentation
25 yo female presents with 3 year history of progressive dysphasia and weight loss
PMH/PSH: rheumatoid arthritis NKDA Medications: methotrexate
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Case Presentation
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Vital Signs: T 97.5 BP 128/67 HR 76 RR 16 O2 sat 98% General: thin, appearing female CV: RRR, S1S2 normal Pulm: CTA bilaterally Abdom: soft nontender, nondistended Extr: no edema or tenderness
12 30
1.1 Upreg: negative
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CXR
CXR
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Operative Summary
Bronchoscopy, esophagoscopy, Right thoracotomy, resection of posterior mediastinal mass
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Pathology
Esophageal Duplication Cyst
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Postoperative Course
Patient tolerated clear liquids on POD#1.
Chest tubes were removed on POD#2 and 3.
Discharged on POD #5
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Discussion
History of foregut cysts Different types of foregut cysts Surgical Technique Questions
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History of foregut cysts
1674- Charles James Blasius Esophageal duplication cyst
1881- Roth 1884- Fitz - (“duplication”)
Omphalomesenteric duct remnants within abdomen
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Foregut cysts histology
Remnants from primitive foregut respiratory tract or alimentary tract compoments
May contain organized histologic architecture Heterotropic lung tissue, ganglia, gastric
Ileum (50%), esophagus (23%), colon (15%), stomach (5%), duodenum (4%), pancreas (1%)
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Esophageal Duplication Cysts
“Dorsal enteric cysts”, “gastroenteric cysts”, “gastrocytomas”, “enterogenous cysts”
Middle and lower 1/3 of esophagus Congenital 2Men : 1Females ( 75% < 16 yo) Blood supply derived from esophagus
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Criteria to characterize esophageal cyst
Palmer criteria: 1-Attachment to the esophagus 2-Epithelium characteristic of some level of
gastrointestinal tract 3-Presence of 2 layers of muscularis
propria
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Presentation Identified on routine antenatal ultrasound Asymptomatic throughout childhood
Airway compression Perihilar region and younger
Dysphagia, substernal pain Ectopic gastric mucosa in cyst
Pain, bleeding and perforation Fistula formation
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Associations…
Contiguous or distal alimentary tract 2nd cyst
Associated with congenital anomalies VACTERL Skeletal anomalies
Spina bifida, hemivertebrae, vertebral fusion
Genitourinary duplications, intestinal malrotation, hindgut anomalies, atresia
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How they come about
Persistent vacuoles in the wall of the foregut Simple columnar, pseudostratified ciliated
columnar or stratified squamous epithelium Within or in close proximity to esophageal wall Overtime- fill with mucus and size increases
Obstruction
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Presentation Identified on routine antenatal ultrasound Asymptomatic throughout childhood
Airway compression Perihilar region and younger
Dysphagia, substernal pain Ectopic gastric mucosa in cyst
Pain, bleeding and perforation Fistula formation
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Diagnosis
CXR Barium esophagram
Smooth oval mass obstructing lumen
CT scan Smooth, well defined
cystic lesion devoid of calcifications
EUS **Townsend: Sabiston Textbook of Surgery, 19th ed.
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Treatment
If untreated obstruction, infection, rupture
Aspiration inadequate
Surgical Resection Extramucosal resection or enucleation
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Surgical Managment
Indications: Symptomatic Malignancy cannot be ruled out
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Surgical Approach
Thoracotomy Thoracoscopic resection
Large cysts initially drained Trocar sites directed toward post mediastinum 3 ports- 1- 5 mm camera port , 2- 5mm
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Thoracoscopic Resection of Esophageal Duplication Cyst
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Bronchogenic cysts
Most are primary cysts of mediastinum
Common in pediatric
population Men> females
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Histology
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How do they develop?
Abnormal budding or branching of tracheobranchial tree
Location depends on developmental stage Paratracheal-usually to the right Adhered to esophageal wall Most common- right hilar and subcarinal
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Clinical Presentation
Not associated with other congenital anomalies
Symptoms: Obstructive- neonate Infectious/inflammatory- older child Dysphagia
Congenital lobar emphysema
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Diagnosis
CXR Mediastinal opacification if cyst autonomous Radiolucent if communicates with airway
CT scan Air fluid levels in mediastinum Size, location and anatomic relationship
Bronchoscopy
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Bronchogenic cysts
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Bronchiogenic cyst in middle mediastinum
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Management and Surgical Considerations
Assessment of airway Need a secure airway if in respiratory
distress
Clear infection prior to surgical resection Minimize adhesions and postoperative
infectious complications
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Operative Approach
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Gastric Duplication Cysts
2 male: 1 female 3 morphologic criteria:
1-attached to stomach, contiguous with wall 2-at least one layer muscle 3-normal gastric mucosa
2-7% of all GI duplications
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GASTRIC DUPLICATION CYST www.downstatesurgery.org
Diagnosis and Treatment of gastric duplication cysts
Diagnosis CT scan, MRI
Treatment Surgical cystectomy or partial gastrectomy
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Excision of Esophageal Duplication Cysts with Robotic-Assisted thoracoscopic Surgery
Case 1: 12 yo female 2 cm x 1.5cm mass in posterolat mid thoraci
esophagus on right
JSLS. 2011 Apr-Jun; 15(2): 244–247.
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Excision of Esophageal Duplication Cysts with Robotic-Assisted thoracoscopic Surgery
Technique: Left lateral decubitus Double lumen tube 8mm port at 6th intercostal space-midaxilla 3- 8 mm ports inserted
Upper chest behind scapula 2 x right lower chest
JSLS. 2011 Apr-Jun; 15(2): 244–247.
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DaVinci resection of esophageal cyst
JSLS. 2011 Apr-Jun; 15(2): 244–247.
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In Summary
Esophageal duplication cysts can present with dysphagia and respiratory symptoms
Differentiated by histology Operative intervention if patient is
symptomatic or malignancy cannot be ruled out
VATS and robotics
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Question 1
The criteria for esophageal duplication cyst include all of the following except: A- attached to the esophageal wall B- has 2 muscularis layers C- includes cartilage in the wall D-has squamous epithelium
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Question 2 33 year old female presents with 2 month history of
dysphagia and a 2 week history of substernal chest pain. She is hemodynamically normal. She undergoes a barium swallow which is shown below. The next step in management would be: A- CT scan of the chest B- Take to operating room immediately C- Manometry D- Observation and repeat barium study in 6months
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References Townsend: Sabiston Textbook of Surgery, 19th . - 2012 - Saunders, An Imprint of Elsevier Holcomb & Murphy: Ashcraft's Pediatric Surgery, 5th ed.- 2010 - Saunders, An Imprint of
Elsevier Patterson: Pearson's Thoracic and Esophageal Surgery, 3rd ed.- 2008 - Churchill
Livingstone, An Imprint of Elsevier Shew SB, Holcomb GW., III . Alimentary tract duplications. In: Ashcraft KW, Murphy JP,
Holcomb GW III, editors. Pediatric Surgery. 4th ed. Amsterdam: Elsevier; 2005. pp. 543–52.
Lund DP. Alimentary tract duplication. In: Grosfeld J, O’neill J, Fonkalsrud E, Coran A, editors. Pediatric surgery. Toronto: Judith Fletcher; 2006. pp. 1389–98.
Nazem M, Amouee AB, Eidy M, Khan IA, Javed HA. Duplication of cervical oesophagus: A case report and review of literatures. Afr J Paediatr Surg. 2010;7:203–5.
Carachi R, Azmy A. Foregut duplications. Pediatr Surg Int. 2002;18:371–4. Takeda SI, Miyoshi S, Minami M, Ohta M, Masaoka A, Matsuda H. Clinical spectrum of mediastinal cysts. Chest. 2003;124:125–32.
Nakao A, Urushihara N, Yagi T, Choda Y, Hamada M, Kataoka K, et al. Case report: Rapidly enlarging esophageal duplication cyst. J Gastroenterol. 1999;34:246–9. Bravo LO, Walls JG, Ly JQ, Lisanti CJ, Roberts SP. Esophageal duplication cyst presenting as chronic cough. Chest. 2003;124:263–4.
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