April 12th 2018

To Cut or Not to Cut?

What is the Role of Surgery in the

management of pediatric Lung Lesions

Canadian Respiratory Conference: A breath of fresh air

Sarah Bouchard,

MDCM, FRCSC, FACS

Associate professor of Surgery

University of Montreal

Financial Interest Disclosure(over the past 24 months)

Dr. Sarah Bouchard

I have no conflict of interest.

Prenatal diagnosis of lung lesions Natural history & exclusion

of other anomalies

Serial ultrasounds

MRI

Karyotype

Pathophysiology survival

Monitoring of size & effect

Treatment plan based on prognosis

Postnatal therapy planned & coordinated

Prenatal treatment

?

Timing of anomalies during lung development

Classification of Congenital Lung Lesions, Langston C, 2003

Bronchopulmonary malformation Bronchogenic cyst

Bronchial atresia

Congenital pulmonary airway malformation (Stocker type 1 and 2) (most common: 0.66 /10 000)

Bronchopulmonary sequestration (10%)

Pulmonary hyperplasia and related lesions Laryngeal atresia

Congenital pulmonary airway malformation (Stocker type 3)

Polyalveolar lobe

Congenital lobar emphysema

Other cystic lesions Lymphatic/lymphangiomatous cysts

Enteric cysts

Mesothelial cysts

Simple parenchymal cysts

Low-grade cystic pleuropulmonary blastoma

Bronchopulmonary sequestration Non-functionning lung mass

Systemic blood supply

Rarely produces symptoms antenatally,

except massive pleural effusion.

Can be associated with congenital

diaphragmatic hernia, or Scimitar

syndrome

Can represent hybrid lesions

Intralobar are more common (75–85%)

and usually present later in life with

recurrent infections

As pediatric surgeons we tend to see

extralobar sequestration more often.

Congenital pulmonary airway malformation (CPAM)

CCAM prevalence 0.66 / 10 000

Associated anomalies in up to 20%

Only 25% symptomatic at birth

CPAM classification Stocker classification

Type 0: Solid appearance

Type 1: large cyst, multiloculated, > 2 cm

Type 2: small cyst < 2 cm

Type 3: solid (microcystic)

Type 4: Very large cyst

Type I and II could be isolated or

hybrid (intralobar sequestration)

Prenatal:1. Macrocystic: cyst 5mm in diam or

larger

2. Microcystic: solid echogenic mass

Overall prognosis depends on

size rather than type of lesion

Congenital lung lesionsRegression: 20% CPAM

> 2/3 Sequestration

Compression:

Esophagus Polyhydramnios

Lung Hypoplasia

Inferior Hydrops Mortality

vena cava or

Hydrothorax

Prenatal surveillance: CPAM VOLUME RATIOCrombelhome T, et al., J Pediatr Surg, March 2002

0.52 x Lenght x Height x Width

Head circumference

< 1.6 (n=42): hydrops in 7 pts (16.7%); 6 with main cyst

> 1.6 (n=16): hydrops in 12 pts (75%)

1.6 : risk of hydrops F/U 2x/week

1.2 – 1.6 2x/week initially, then weekly

1.2 weekly initially, then q 2 weeks

Prenatal natural history of CPAM

• Growth plateau at around 30

weeks

• Rarely regresses, but stabilizes

in size, thus seems smaller given

continued fetal growth

Pathophysiology of large fetal lung lesions

• Esophageal compression

• interference with swallowing of amniotic fluids

• Polyhydramnios

• Cysts hyperinflation from liquid trapping

• Vena caval obstruction + cardiac compression

• extreme mediastinal shift

• Hydrops

• Placentomegaly

• Fetal demise

Adzick NS, et al., Seminars in Pediatric Surgery, February 2003

Prenatal management if hydrops

• Steroids administration

• Pleuroamniotic shunts

• Fetal lobectomy

• If maternal mirror syndrome? Pregnancy termination

< 32 weeks

• Delivery with postnatal management> 32 weeks

A,B: before steroids, C,D: post steroids

Diaphragmatic

eversionMediastinal

shift

Decreased CPAM after steroids administration

Variable response to steroids in high risk lesions (Cincinnati)

15 patients treated

13 hydropic fetuses responded initially

7/15 patients resulted in fetal demise or postnatal death

Overall survival rate 53%

Second course of steroids was not helpful

Consider shunting and/or open fetal surgery in non-responder

Morris et al, JPS 2009; 44:60-65

Best delivery mode for fetuses

with large lung lesions causing

mediastinal shift and

polyhydramnsios?

EXIT

EX utero Intrapartum Treatment

Risks:

Hypoventilation

from lack of space

Hypoxia

Acidosis

EXIT C-section

Minimal anesthesia

time:

Fetal exposure

Newborn respiratory

depression

Low anesthetics MAC

Preserve uterus tone

Prevent maternal

hemorhage

C-section

Fetal anesthesia and analgesia desired

Deep anesthesia

Uterine relaxation

Risk of maternal

hemorrhage

EXIT

Postnatal management

of pediatric pulmonary lesions

Resection of symptomatic CPAM

Acute respiratory distress 1/3 neonates prenatally diagnosed

No arguments! Next slide!

Open approach

If less than 5 kilos

Cannot tolerate one lung ventilation during general anesthesia

Lesion too large to allow access and safe dissection via

thoracoscopy

Otherwiswe thoracoscopy can be attempted

Presentation from childhood to adulthood

60% Symptoms < 1 mo

10% Symptoms 1 to 6 mo

15% Symptoms 6 mo to 14 years ( risk cancer)

97% Dx < 1 an

Symptoms: Recurrent respiratory infections

Dyspnea

Hemoptysis

Hemothorax

Empyema

Pneumothorax

Malignant transformation

Incidental finding

Imaging studies: asymptomatic

patient Usefulness of CXR?

Baseline

Allows for follow-up if lesion is visible

Timing of thoracic CT scan

Wait for clearance of lung fluid

2 to 6 months

Other – MRI – less radiation for follow up

Barium swallow – differentiation from CDH

Extralobar Bronchopulmonary sequestration

18 weeks At birth 5 months

9 month old healthy baby boy, with

extralobar sequestration as seen on

previous slide.

Asymptomatic

Resection or no resection?

Approach?

Vignette

Treatment of extralobar sequestration

sequestration Resection

Wait for sufficient size to allow thoracoscopy if patient

asymtomatic (6 months)

Thoracoscopic approach preferred

Embolisation reported but

Pain

Fever

Tissue never really disappear

Not a definitive treatment

Resection of intralobar sequestration

If no symtpoms it will depends on size

Reluctance to remove a “functioning” lobe

For symptomatic patient a lobectomy will be

performed

Preferably via thoracoscopy

Postnatal surgical management of lung

anomalies in asymptomatic patients

«To doubt everything or to believe everything are

equally easy solutions, for both protect us from

thinking»

(Poincaré)

Asymptomatic 6 month old baby

girl

Vignette

Resection

or no

resection?

Asymptomatic 2 month old

baby boy

Vignette

Resection

or no

resection?

Asymptomatic 3 month old

baby boy

Vignette

Resection

or no

resection?

day 2 day 21 3 months

Why resect?

Arguments for routine resection of asymptomatic CPAM

Long-term risk of infection

Some argue that the surgical risk is higher if the lesion has

already been infected (not all studies have documented this

difference).

Early resection results in better compensatory lung growth

Hypothetical advantage which is not universally supported in long-

term follow-up studies

Possibility that the lesion is not a benign CPAM, but is instead a

Type I pleuropulmonary blastoma (PPB).

Risk of Infection

Risk of infection

10% to 30%of CPAM will present with infection

10% per year, so cumulative incidence

May have higher rate of complication if surgery performed after an

episode of infection

Higher loss of productive time for patient and parent

Risk of malignancy

Really?

Multitude of reported cases and registries

Difficult to differentiate between CPAM and PBS

Broncho alveolar Carcinoma

Rhabdomyosarcoma

Epidermoid carcinoma

Mucinous adenocarcinoma

Mucinous adenocarcinoma in 10 yo boy

Can congenital pulmonary airway malformation be distinguished from Type I

pleuropulmonary blastoma based on clinical and radiological features?Journal of Pediatric Surgery , Volume 51, Issue 1, Pages 33-37 (January 2016)

Decision-Making Criteria for Observational Management of

Congenital Pulmonary Airway Malformations (CPAMs)

Number of lesions,

Presence of a solid component

Size of the cysts.

This said, these criteria are

poorly defined and arbitrary

representing “expert opinion”

only - Grade V level of

evidence.

Survey of CAPS membership on management of CPAM, especially

observation, In press 2018

Size of asymptomatic CPAM lesions at which Canadian pediatric

surgeons considered appropirate to observe

Complications of surgery for patients operated on

when becoming symptomatic

Longer hospital stay

Longer pleural drainage and invasive ventilation

Higher rate of postoperative complications

Fistula

Hemorrhage

Second surgery

No difference in mortality

Elective surgery, eliminating CPAM complications, is thus

considered to provide better outcome than emergency surgery.

Optimal timing for elective resection of asymptomatic

congenital pulmonary airway malformations Eric B. Jelin a,⁎, Elizabeth M. O'Hare a, Tim Jancelewicz b, Isam Nasr a,

Emily Boss c, Daniel S. Rhee a

In press, Journal of Pediatric Surgery 2018

Between 1 months and 2 years old

anesthetic and surgical risks decrease after the first months of life

OR time increases with age

Lung maintains the capacity to expand and develop until 4 years of

age allowing better compensation when early surgery

ASA >2 and prolonged OR time associated with complications

Procedure = Lobectomy

• Well tolerated

• Good long term

pulmonary capacity

• Complete resection

• Segmentectomy can be

performed in special

situation

–Lesion well demarcated and

can be visualied

–15% incomplete resection

Complications of lobectomy

Early

Bleeding

Infection

Injury to other thoracic organs

Air leak / bronchopleural fistula

Pain

Death

Late – if using open approach, without muscle-sparing

Chest wall deformity

Scoliosis

Winged scapula

Thoracoscopy

Slowly replacing thoracotomy

As safe, if not safer

More challenging for surgeon

and anesthetist

Better cosmetic results

Shorter LOS

Less pain and narcotic use

Similar OR time past learning

curve

Pulmonary capacity post lobectomy

All studies comparing pulmonary function test after

lobectomy in early life versus normal controls show no

statistical differences

Congenital lobar

emphysema

Who would observe

an asymptomatic

patient?

Congenital lobar emphysema

Resection for symptomatic patients

Asymptomatic patients can be observed

Easily followed with CXR

In 1976, study compared PFTs in patients who had surgery

(symptomatic) and children observed (asymptomatic or mild

symptoms)

Same results in both: diminished PFTs

Duplication cysts

Lined by enteric type mucosa

No cartilage

May contain ciliated epithelium

May extend in spinal canal

In the esophageal wall

Can cause dysphagia

Can contain gastric mucosa

bleeding

Malignant potential

Must be excised

Bronchogenic cyst

Resect even if

asymptomatic

VATS

Can be located in various

parts in the thorax

Follow up if no surgery

CT in first few months of life

Clinical follow-up after that if no change in

appearance

Surgery for

Symptoms

Parental wishes

Questions