Presentazione standard di PowerPoint...Different professional background (physical therapist vs...

Francesco MoriniDepartment of Medical and Surgical NeonatologyOspedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy

francesco.morini@opbg.net

Prenatal diagnosis & treatment

Mechanical ventilation

Surgery

+/- ECMO

DISCHARGE

3 francesco.morini@opbg.net

CDH: long term sequelae

“Now this is not the end. It is not even the

beginning of the end.

But it is, perhaps, the end of the beginning.”

Sir Winston Churchill, El Alamein,November 1942

Auxological Up to 70%Cardio-pulmonary Up to 60%Gastrointestinal Up to 70%NDO Up to 40%Orthopedic Up to 30%Surgical Up to 50%

Diaphragmatic defect

Pulmonary hypoplasia

Pulmonary hypertension

Stoll C et al., Genet Couns, 2015

Prenatal treatment Mechanical ventilationFiO2Pressure

Drugs ECMO Minimal access surgery Patch repair

CDH sequelae

Putnam LR et al., Pediatrics, 2016

Hollinger LE & Buchmiller TL, Semin Perinatol, 2019

Giorgia

• GA 33 wks; BW 1.6 kg

• STABILZATION: 72 h

• LEFT CDH (Type D defect, Liver up, Stomach up)

• REPAIR: Patch

• PO COURSE: Uneventful (O2 dependent at 30 days)

• DISCHARGE: 60 days after birth

• No O2-support

Iacusso C et al., Eur J Pediatr Surg Rep, 2018

Giorgia

• Regular follow up

check-visits

• Unremarkable first 9

years of life…

4 years-old…climbing!JANUARY 21st, 2007

Abdominal pain, vomiting… Emergency admission…Iacusso C et al., Eur J Pediatr Surg Rep, 2018

Giorgia

2007 2008 2009 2010 2011 2012 2014

JanuaryPHTnGERD

June, 16thH/L Tx

March,23rdListed for H/L

July, 28thIntestinal obstruction

CDH recurrence(bowel resection, ileostomy)

July, 1stPleural effusion (thoracic drainage)

Ascites (peritoneal drainage)

March, 28thNissen + ileostomy

closurePost-op Lung Abscess

Dec,27thRe-Listed for

Dec,28thLTx

March, 14thObliteration of left main

bronchus

May, 15thPost vertebral

Arthrodesis

Tracheostomy

July, 27thExitus

SildenafilBosentan

Furosemide

PEJNIV

CDH & Persistent/recurrent PHTn

Mesas Burgos C et al., J Pediatr Surg, 2017

CDH & lung transplantation

CDH & PHTn

Bagolan P & Morini F, Semin Pediatr Surg, 2007

CDH & gastroesophageal reflux

Gastroesophageal reflux (%)

6 months 12 months 24 monthsValfrè L et al., J Pediatr Surg, 2011

61 CDH survivors

61 @ 6 mos

49 @ 12 mos

43 @ 24 mos

Morando A et al., Eur J Pediatr Surg, 2016

12 CDH survivors

Mean age 14.5 yrs

75% asymptomatic

Gastroesophageal reflux

Recurrent RTI

Abnormal PFT

Oral aversion

Failure to thrive

Feeding tube

Maier S et al., J Pediatr Surg, 2011

79 L-CDH survivors

36 wARS

43 controls

CDH & obstructive manifestations

1 year 2 years 8 years 12 years

Spoel et al2012

Valfrè et al2011

Basek et al2008

Ijsselstijn et al1997

Spoel et al2013

27 years Follow-up

Peetsold MG et al., Eur Respir J, 2009

53 CDH survivors

Mean age 12 yrs

Asthma 28%

Toussaint-Duyster LCC et al., Pediatr Pulmonol, 2019

Obstructive manifestations vs PFT abnormalities (%)

Basek et al2008

Peetsold et al2009

Valfrè et al2011

Clinical

CDH & Fitness

Trachsel D et al., Pediatr Pulmonol, 2006

CDH & Fitness

Turchetta A et al., Pediatr Pulmonol, 2011

CDH & Neurodevelopmental outcome

Different diagnostic tool(Bayley II vs III)Different professional background(physical therapist vs developmental psychologist)Different patients’ population

Snoek KG et al., Neonatology, 2016

Bevilacqua F et al., Am J Perinatol, 2017

22% delay

Danzer E et al., J Pediatr Surg, 2017

CDH & Psychiatric problems

Danzer E et al., J Pediatr Surg, 2017

May be severe

May be smoldering

Tend to worsen over time

Hollinger LE & Buchmiller TL, Semin Perinatol, 2019

CDH: long term follow-up

Morini F et al., Semin Pediatr Surg, 2017

CDH follow-up

AAP Section on Surgery and Committee on Fetus and Newborn, Pediatrics, 2007

CDH sequelae: our long term follow-up

Multidisciplinary follow-up clinic:

& physiotherapist, neurologist, radiologist, ENT, …

Pediatrician Pediatric surgeon Nurse

Psychologist

6 mos 12 mos 18 mos 24 mos 3 yrs 5 yrs 10 yrs

Multidisciplinary follow-up clinic:

CDH sequelae: our long term follow-up

CDH follow-up: the tale of three cities

Morini F et al., Semin Pediatr Surg, 2017

CDH follow-up

Safavi A et al., J Pediatr Surg, 2012

CDH follow-up

Ijsselstijn A et al., Pediatr Res, 2018

CDH follow-up

Ijsselstijn A et al., Pediatr Res, 2018

CDH parents: who cares?

Aite L et al., Am J Perinatol, 2016

Failed projectUncertainty for the futureFear for lossChange of familial routine

No symptoms

1 symptom

2 symptoms

3 symptoms

CDH parents: who cares?

Bevilacqua F et al., Am J Perinatol, 2018

6 months 12 months

Parents with PTSD (%)

Mothers Fathers

Failed projectUncertainty for the futureFear for lossChange of familial routine

Take home messages CDH patients may have serious long

term sequelae

In CDH patients long term sequelae

may be smoldering

Long term, family-centered follow-up programs are needed

Standardization of follow-up programs is desirable

Future: TRANSITION

Thank you

Presentazione standard di PowerPoint...Different professional background (physical therapist vs...

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