foregut duplication cysts - suny downstate medical...

Foregut Duplication Cysts

Maria Georgiades April 25, 2013 Grand Rounds

www.downstatesurgery.org

Case Presentation

25 yo female presents with 3 year history of progressive dysphasia and weight loss

PMH/PSH: rheumatoid arthritis NKDA Medications: methotrexate


Case Presentation

4.65 11

33 295

138

4.4

13

8

8

0.6 82

9.9 2.0

3.6

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


CXR

CXR


Operative Summary

Bronchoscopy, esophagoscopy, Right thoracotomy, resection of posterior mediastinal mass


Pathology

Esophageal Duplication Cyst


Postoperative Course

Patient tolerated clear liquids on POD#1.

Chest tubes were removed on POD#2 and 3.

Discharged on POD #5


Discussion

History of foregut cysts Different types of foregut cysts Surgical Technique Questions


History of foregut cysts

1674- Charles James Blasius Esophageal duplication cyst

1881- Roth 1884- Fitz - (“duplication”)

Omphalomesenteric duct remnants within abdomen


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%)


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


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


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


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


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


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


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.


Treatment

If untreated obstruction, infection, rupture

Aspiration inadequate

Surgical Resection Extramucosal resection or enucleation


Surgical Managment

Indications: Symptomatic Malignancy cannot be ruled out


Surgical Approach

Thoracotomy Thoracoscopic resection

Large cysts initially drained Trocar sites directed toward post mediastinum 3 ports- 1- 5 mm camera port , 2- 5mm


Thoracoscopic Resection of Esophageal Duplication Cyst


Bronchogenic cysts

Most are primary cysts of mediastinum

Common in pediatric

population Men> females


Histology


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


Clinical Presentation

Not associated with other congenital anomalies

Symptoms: Obstructive- neonate Infectious/inflammatory- older child Dysphagia

Congenital lobar emphysema


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


Bronchogenic cysts


Bronchiogenic cyst in middle mediastinum


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


Operative Approach


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


GASTRIC DUPLICATION CYST www.downstatesurgery.org

Diagnosis and Treatment of gastric duplication cysts

Diagnosis CT scan, MRI

Treatment Surgical cystectomy or partial gastrectomy


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.


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.


DaVinci resection of esophageal cyst

JSLS. 2011 Apr-Jun; 15(2): 244–247.


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


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


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


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.


http://www.mdconsult.com.newproxy.downstate.edu/books/linkTo?type=bookHome&isbn=978-1-4377-1560-6&eid=4-u1.0-B978-1-4377-1560-6..C2009-0-60514-6--TOP&uniq=409356851-3



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