The Long and Short of Esophageal Atresiaeradiology.bidmc.harvard.edu/LearningLab/genito/Olszewski.pdf · The long and short of esophageal atresia ... jejunum colon OPTIONS Compiled

The long and short of esophageal atresia

Aleksandra Olszewski,

Harvard Medical School Year III

Gillian Lieberman, MD

BIDMC Radiology Core Clerkship

August 2013

Aleksandra Olszewski, MSIII


Outline

• What is esophageal atresia (EA)?

• Diagnosing EA: the role for imaging

• Management of short-gap vs long-gap EA

• The Foker Method

• One patient’s story through her diagnosis, therapy, complications, and cure

2



EA: Classification

3



1. Pinheiro et al. World Journal of Gastroenterology 2012

July 28; 18(28): 3662-3672.

+TEF -TEF (8%)

Proximal (1%)

Distal (84%)

Both (3%)

*TEF without EA (H type, 4%)

EA: the basics

• Most common esophageal abnormality (1/2500-1/3500)

• Cause: unclear

• Associated with anomalies >50% of the time

– Highest risk of VACTERL with isolated EA (no TEF)

• Presentation: at birth with excessive salivation, suffocation, cyanosis, feeding intolerance, pneumonia

• Treatment: repaired surgically

4



2. Spitz L. Orphanet J Rare Dis 2007; 2: 24

3. Kovesi T, Rubin S. Chest 2004; 126: 915-925

4. Holland AJ, Fitzgerald DA. Paediatr Respir Rev 2010; 11: 100-106; quiz 106-107

EA: Prenatal diagnosis

• Prenatal: difficult to diagnose

– US: polyhydramnios + dilated proximal pouch + absence of gastric bubble

– not specific or sensitive findings, not always present with TEF

– MR: nonvisualization of intra-thoracic esophagus fundus

5



4. Holland AJ, Fitzgerald DA. Paediatr Respir Rev 2010; 11: 100-106; quiz 106-107

5. Houben CH, Curry JI. Prenat Diagn 2008; 28: 667-675

Companion Patient 1: AIUM (ACOG+ACR) Guidelines for Obstetric Ultrasound Exams

6

Second/third trimester screen: evaluation of fetal presentation, amniotic fluid volume, cardiac activity, placental position, fetal biometry, and fetal number, plus an anatomic survey (gastric bubble)

7. AIUM 2012 Practice Guidelines for the Performance of Obstetric Ultrasound Exams

6. Mourali et al. La tunisie Medicale 2011 89(2):213-214.



EA: Post-natal diagnosis

Delivery room:

– Symptoms, impassibility of orogastric catheter past 11-12cm

– AP CXR with air as contrast (avoid aspiration of contrast fluid!): catheter coiled in esophagus

• Air in stomach, indicates TEF increased urgency for surgery, high risk of aspiration

• Absence of air in stomach indicates no TEF

7



Companion Patients 2,3: EA with and without TEF on AP Chest X-Ray

8



8. LearningRadiology.com

9. radiopaedia.org

Left: note nonprogression of orogastric catheter, lack of gastric air bubble

Right: note nonprogression of catheter, presence of gastric air bubble indicating TEF

*

*

*

*

9

EA: Treatment

Pre-op: full aspiration precautions, VACTERL assessment -orogastric tube (suction), head of bed 45°, acid suppression, avoid PPV

-renal US, ECHO, etc.

Primary repair: thoracoscopic or thoracotic ligation -Contraindications: Low birth weight, congenital heart disease, long gap length, compromised physiologic status



1. Pinheiro et al. World Journal of Gastroenterology 2012 July 28; 18(28): 3662-3672.

2. Spitz L. Orphanet Journal of Rare Diseases. 2, 24. 2007.

EA: Recognizing post-operative complications through imaging

• Seven days post-op, esophogram is performed

• Leak 15% of cases

• Strictures common, recurrent, tx: serial endoscopic dilation

• GERD common

• Esophageal dysmotility 75%-100% of cases

• Thoracic wall deformities 24% winged scapula, 21% scoliosis

10




2. Spitz L. Orphanet J Rare Dis 2007; 2: 24

10. Mortell AE, Azizkhan RG. Semin Pediatr Surg 2009; 18:12-19.

Long-Gap EA: Management

• Primary surgery not possible

• Gapogram to assess gap size

– Treatment is controversial

• Esophagus grows from swallowing (proximal) and reflux (distal) repair delay until 1-3 months

• Other options: replacement vs circular myotomy vs surgery under tension vs Foker method

11

Definition: gap too big to repair

(>3cm or 2 vertebral bodies)




11. Bagolan et al. Diseases of the Esophagus 2013; 26: 372-379.

Companion patient 4: Gapogram to assess gap size

• Contrast through enteric tube to assess proximal stump (then removed by enteric tube)

• Contrast (or probe) through gastrostomy to assess distal stump

• Measure gap by vertebral bodies or cm

12



12. Greenfield's Surgery: Scientific Principles and Practice.

Long-gap EA: Controversy surrounding repair

13



Replacement Growth induction

Stomach (gastric pull up or gastric

tube)

colon jejunum

OPTIONS

Compiled information from:

13. Kunisaki SM, Foker JE. Clin Perinatol

39 (2012) 349–361

14. Spitz L. J Pediatr Surg

2006;41(10):1635–40.

15. Tsai JY et al. Ann Thorac Surg

1997;64(3):778–83.

Long-gap EA: Management by Growth Induction (the Foker process)

14



• Sutures applied through external proximal and distal stumps, with tag on end

• Sutures are pulled out of patient’s body attached to external traction devices

• With the patient paralyzed, increasing traction is applied over time

16. Foker et al. Seminars in Pediatric Surgery 2005; 14:8-15.

Companion patient 5: Serial gapograms show decreasing gap size over time in a patient undergoing the Foker process

15



• Over time, traction causes stress leading to natural tissue growth (rather than stretch, which may compromise tissue integrity) • Serial gapograms are a critical part of the procedure, assessing gap size in preparation for repair

16. Foker et al. Seminars in Pediatric Surgery 2005; 14:8-15.

Now that we know about different types of EA, how to

diagnose each by imaging, and how imaging is used with some

of the management options, let’s meet our patient!

16



First, let’s go through our patient’s diagnostic studies

17



Our Patient: AP Chest X-Ray at delivery



Pause to evaluate and continue for labeled findings.

CHB PACS 18

Did you remember to assess for EA AND for TEF? This is a key distinction that changes the management of patients with EA.

19



Our Patient: Impassibility of NG tube on AP Chest X-Ray at delivery

*

NG tube impassible proximally

Paucity of gastric gas, indicating isolated EA (without TEF)

CHB PACS

Our patient: Presentation and Diagnosis

• GT is a 50-day-old ex-32-weeker who was diagnosed prenatally with isolated EA (no TEF).

• Postnatally, a diagnosis of EA without TEF was confirmed, as you saw on chest X-Ray done at delivery.

• She lives in Tennessee, and was transferred to Boston Children’s for definitive care

20



Our Patient: Pre-operative evaluation

Two major consideration:

• Aspiration precautions

• VACTERL assessment

– She showed no signs of any other anomalies (normal ECHO, no other GI/anal anomaly, no limb anomalies, no vertebral anomalies, normal kidneys)



21

Water-soluble barium gapogram with contrast injected into the enteric tube proximally and contrast injected into a catheter passed through the gastric tube distally. A 34 mm gap between distal and proximal esophageal stumps was visualized. Note the ruler behind the patient, to calibrate findings.

22



CHB PACS

Our Patient: Gapogram showing 34 mm gap size

*

*

Remember: -Use water-soluble contrast in case of leak. -Remove contrast from upper esophageal pouch immediately after! These patients have a blind-ending esophagus and it is important to be prepared with suction after imaging is performed to avoid aspiration.



Our Patient: Imaging safety considerations

23

Our Patient: Management

• As seen on gapogram, her gap size is 34 mm (dx: long-gap EA)

• She came to Children’s for the Foker process

• Her Foker 1 process (thoracotomy with placement of sutures and traction devices) was uncomplicated, and the ensuing slides follow the serial gapograms that assessed her stump approximation for esophageal anastomosis



24

25



ET tube

enteral tube

PICC

POD1 after Foker 1 process. Note the external traction devices attached to sutures at each esophageal stump. The proximal and distal stumps are tagged with markers. Barium gapogram is needed for definitive gap size assessment.

Our patient, day 1 status post Foker 1 process: External traction devices and esophageal stump tags on portable

supine chest x-ray

CHB PACS

26



Note the external traction devices, now with spacers to increase the tension on the stumps. The proximal and distal stumps are tagged with markers, and the distance between is measured at 11.63mm (need gapogram for accurate assessment).

Our patient day 5: External traction devices with spacers and esophageal stump tags on portable

supine chest x-ray

CHB PACS

Water soluble barium injected through enteric tube proximally and through catheter in the gastric tube distally. At this point in time, the proximal esophagus and distally the stomach are visualized.

27



Our patient day 5: Injection of contrast proximally and distally on barium gapogram

*

*

In this next image from the same study, the upper esophageal pouch appears intact, but it is evident that high-attenuation contrast has extravasated from the distal gastric catheter into the pleural space, indicating leak.

28



Our patient day 5: High attenuation contrast extravasation on barium gapogram

*

*

CHB PACS

After this, a repeat thoracotomy was performed

and the ruptured distal esophageal stump was

repaired.

29



Water soluble barium study high-lighting approximation of proximal and distal stumps, no extravasation of contrast.

With the stumps well-approximated, thoracotomy with esophageal anastomosis was next performed.

30



Our patient day 30: Close approximation of proximal and distal esophageal stumps on barium gapogram

*

*

CHB PACS

31



Our patient post-operative day 1: Passage of NG tube to stomach on supine chest x-ray

CHB PACS

32



Our patient post-operative day 1: Evidence of stricture

formation on barium esophophagram

CHB PACS

*

33



10-year-old female with chicken stuck in esophagus due to stricture.

Companion patients 6, 7: Strictures on barium esophagrams

30-day-old male with evidence of stricture.

CHB PACS

Summary

We have seen:

• Key imaging signs to diagnose EA +/- TEF, prenatally and postnatally

• Imaging as it is used to evaluate gap size in EA (gapograms)

• How the Foker process uses serial gapograms to evaluate progress

• Imaging of complications in EA, including leak and strictures

We have discussed:

• Key considerations in diagnosis and management of long and short gap EA, including safety precautions and pre-operative planning

• Different treatment options exist for long-gap EA

• Complications are common, but rarely severe

34



Thank you!

Yuri Shif, MD for his generous offering of time and his assistance with image acquisition

Gillian Lieberman, MD, for her excellent teaching and for directing our core radiology clerkship

Claire Odom for her organization and support throughout the core radiology clerkship

35



36

References 1. Fernando P, Pinheiro M, Simoes e Silva AC, Pereira RM . Current knowledge on esophageal atresia.

World Journal of Gastroenterology 2012 July 28; 18(28): 3662-3672.

2. Spitz L. Oesophageal atresia. Orphanet J Rare Dis 2007; 2: 24.

3. Kovesi T, Rubin S. Long-term complications of congenital esophageal atresia and/or tracheoesophageal

fistula. Chest 2004; 126: 915-925.

4. Holland AJ, Fitzgerald DA. Oesophageal atresia and tracheo-oesophageal fistula: current management

strategiesand complications. Paediatr Respir Rev 2010; 11: 100-106; quiz 106-107.

5. Houben CH, Curry JI. Current status of prenatal diagnosis, operative management and outcome of

esophageal atresia/tracheo-esophageal fistula. Prenat Diagn 2008; 28: 667-675.

6. Mourali M, Essoussi-Chikhaoui J, Fatnassi A, El Fekih C, Ghorbel S, Ben Zineb N, Oueslati B. Prenatal

diagnosis of esophageal atresia. La tunisie Medicale 2011; 89(2):213-214.

7. American Institute of Ultrasound in Medicine. AIUM practice guideline for the performance of obstetric

ultrasound examinations. J Ultrasound Med 2013; 32: 1083–1101. doi:10.7863/ultra.32.6.1083LR

8. Learning Radiology case of the week. Esophageal atresia and tracheoesophageal atresia.

LearningRadiology.com 2005.

9. Alsalam H. Oesophageal atresia with trache-oesophageal fistula. Radiopaedia Cases. Radiopaedia.org.

10. Mortell AE, Azizkhan RG. Esophageal atresia repair with thoracotomy: the Cincinnati contemporary

experience. Semin Pediatr Surg 2009; 18:12-19.

11. Bagolan P., Valfre L, Morini F, Conforti A. Long-gap esophageal atresia: traction-growth and

anastomosis-before and beyond. Diseases of the Esophagus 2013; 26:372-379.



37

12. Mulholloand MW, Lillemoe KD, Doherty GM, Maier RV, Simeone DM, Upchurch GR. Greenfield's Surgery:

Scientific Principles and Practice, 5e > Chapter 106. The Pediatric Chest. Lippincott Williams & Wilkins, Sept 11 2012.

13. Kunisaki SM, Foker JE. Surgical advances in the fetus and neonate: esophageal atresia. Clin Perinatol 2012; 39:

349–361.

14. Spitz L. Esophageal atresia. Lessons I have learned in a 40-year experience. J Pediatr Surg 2006; 41(10):1635–40.

15.Tsai JY, Berkery L, Wesson DE, Redo SF, Spigland NA. Esophageal atresia and tracheoesophageal fistula: surgical

experience over two decades. Ann Thorac Surg 1997; 64(3): 778–83.

16.Foker JE, Kendall TC, Catton K, Khan KM. A flexible approach to achieve a true primary repair for all infants with

esophageal atresia. Seminars in Pediatric Surgery 2005; 14: 8-15.

References



Documents

The Long and Short of Esophageal Atresiaeradiology.bidmc.harvard.edu/LearningLab/genito/Olszewski.pdf · The long and short of esophageal atresia ... jejunum colon OPTIONS Compiled