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Foregut Duplication Cysts
Maria Georgiades
April 25, 2013
Grand Rounds
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Case Presentation
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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
11
4.65
33
295
9.9
2.0
3.6
12
138
13 8
4.4 8 0.6
82
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
30
1.1
Upreg: negative
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CXR
CXR
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Operative Summary
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Bronchoscopy,
esophagoscopy, Right
thoracotomy, resection
of posterior mediastinal
mass
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Pathology
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Esophageal Duplication Cyst
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Postoperative Course
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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
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History of foregut cysts
Different types of foregut cysts
Surgical Technique
Questions
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History of foregut cysts
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1674- Charles James Blasius
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Esophageal duplication cyst
1881- Roth
1884- Fitz - (“duplication”)
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Omphalomesenteric duct remnants within
abdomen
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Foregut cysts histology
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Remnants from primitive foregut
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May contain organized histologic architecture
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respiratory tract or alimentary tract compoments
Heterotropic lung tissue, ganglia, gastric
Ileum (50%), esophagus (23%), colon
(15%), stomach (5%), duodenum (4%),
pancreas (1%)
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Esophageal Duplication Cysts
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“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
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Palmer criteria:
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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
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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…
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Contiguous or distal alimentary tract 2nd
cyst
Associated with congenital anomalies
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VACTERL
Skeletal anomalies
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Spina bifida, hemivertebrae, vertebral fusion
Genitourinary duplications, intestinal
malrotation, hindgut anomalies, atresia
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How they come about
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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
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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
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CXR
Barium esophagram
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CT scan
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Smooth oval mass
obstructing lumen
Smooth, well defined
cystic lesion devoid
of calcifications
EUS
**Townsend: Sabiston Textbook of Surgery, 19th ed.
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Treatment
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If untreated  obstruction, infection, rupture
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Aspiration inadequate
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Surgical Resection
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Extramucosal resection or enucleation
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Surgical Managment
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Indications:
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Symptomatic
Malignancy cannot be ruled out
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Surgical Approach
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Thoracotomy
Thoracoscopic resection
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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
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Most are primary
cysts of
mediastinum
Common in pediatric
population
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Men> females
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Histology
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How do they develop?
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Abnormal budding or branching of
tracheobranchial tree
Location depends on developmental
stage
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Paratracheal-usually to the right
Adhered to esophageal wall
Most common- right hilar and subcarinal
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Clinical Presentation
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Not associated with other congenital
anomalies
Symptoms:
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Obstructive- neonate
Infectious/inflammatory- older child
Dysphagia
Congenital lobar emphysema
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Diagnosis
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CXR
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CT scan
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Mediastinal opacification if cyst autonomous
Radiolucent if communicates with airway
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
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Assessment of airway
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Need a secure airway if in respiratory
distress
Clear infection prior to surgical
resection
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Minimize adhesions and postoperative
infectious complications
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Operative Approach
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Gastric Duplication Cysts
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2 male: 1 female
3 morphologic criteria:
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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
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Diagnosis and Treatment of
gastric duplication cysts
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Diagnosis
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CT scan, MRI
Treatment
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Surgical cystectomy or partial gastrectomy
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Excision of Esophageal Duplication Cysts
with Robotic-Assisted thoracoscopic
Surgery
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Case 1:
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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
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Technique:
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Left lateral decubitus
Double lumen tube
8mm port at 6th intercostal space-midaxilla
3- 8 mm ports inserted
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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
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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
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The criteria for esophageal duplication
cyst include all of the following except:
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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
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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
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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.