Juwarat Ains Kadiri, Harvard Medical School Year III...

32
Juwarat Ains Kadiri, Harvard Medical School Year III Gillian Lieberman, MD August 2011 Juwarat Ains Kadiri, HMS III Gillian Lieberman, MD

Transcript of Juwarat Ains Kadiri, Harvard Medical School Year III...

Page 1: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Juwarat

Ains

Kadiri, Harvard Medical School Year IIIGillian Lieberman, MD

August 2011Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

Page 2: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Agenda

Anatomy and embryology of CDH

Prenatal diagnosis of CDH

Using imaging to determine prognosis

Postnatal diagnosis of CDH

Post treatment imaging

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

2

Page 3: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Meet Our Patient

A 29y G5P2022 presented at routine second trimester full fetal survey ultrasound, this was seen…

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

3

Page 4: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Our Patient Fetal US at 19w6dJuwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

From the US we can see-R sided heart -Anechoic foci in thorax (fluid filled stomach)-Liver in thorax-These findings are consistent with CDH

Children’s Hospital BostonAxial view through fetal thorax

4

Page 5: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Ddx

for Thoracic Anechoic Foci on Fetal US

CDHPulmonary sequestration Congenital Pulmonary Adenomatoid

Malformation (CPAM)Bronchogenic

cystsBronchial atresiaEnteric cystsTeratomas

The presence of liver in our patient’s thorax however clinches the diagnosis of CDH

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

5

Page 6: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

CDH Overview

CDH is a communication between the thoracic and abdominal cavity due to an embryonic defect in the diaphragm.

Incidence of 1/2,000-1/5,000 live births, with females affected 2X as often as males

2 major types exist:

Bochdalek

(posterolateral

hernia) seen in 95% of cases

Morgagni

(retrosternal

hernia)

Left CDH are more commonly symptomatic

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

Nelson Textbook of Pediatrics, 19th ed

6

Page 7: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Embryology of the Diaphragm

Chavhan G B et al. Radiographics 2010;30:1797-1817

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

Slide courtesy of Behroze Vachha MD 7

Page 8: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Embryonic Development of CDH

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

Chavhan G B et al. Radiographics 2010;30:1797-1817

8

Page 9: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Prenatal Diagnosis of CDH The Role of US

US detection rate ranges from 59%- 93% at gestational age19-24Wks the

wide range is attributed to the operator dependence of US

There are multiple features on US that clue in the diagnosis of CDH…

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

9

Page 10: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Companion Pt 1 Fetal US showing bowel in thorax at different gestational ages

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

•Both axial views show heart, stomach and bowel at 21w(left) and 28w (right). •As the fetus gets older, inhomogeneous collapsed echogenic bowel becomes more dilated and fluid filled making it easier to differentiate from normal lung tissue.

Kline-Fath, B.M. Congenital diaphragmatic hernia Pediatr RadiolAxial views through fetal thorax

10

Page 11: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Companion Pt 2 and 3 Fetal US comparing CDH with and without liver herniation

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

•Pt 3 (left) has a left CDH with stomach herniating into the thoracic cavity. •Contrast with pt 4 (right) , much like our patient, with left CDH with liver herniation. The stomach is displaced from the anterior thoracic wall because liver is herniated anteriorly.•The heart is seen in both images

Kline-Fath, B.M. Congenital diaphragmatic hernia Pediatr Radiol 2011 Jul 8Axial views through fetal thorax

11

Page 12: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Our Pt Fetal Doppler US showing abnormal increased vascularity

in the thorax

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

12

Doppler shows increased blood flow in the thorax due to the blood flowing to the bowel in its abnormal location. Recall that blood flow is diverted from the fetal lung while in utero

Children’s Hospital BostonCoronal view through fetal thorax at 26w5d

Page 13: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Our Pt Fetal US showing absent left hemidiapragm

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

Children’s Hospital BostonCoronal image through fetal thorax at 26w5d

The findings on this view include:Intact right hemidiaphragmAbsent left hemidiaphragm

13

Page 14: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

We have viewed the findings on fetal ultrasound that are diagnostic of CDH

Lets move on to Fetal MRI…

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

14

Page 15: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Prenatal Diagnosis of CDH The role of MRI

MRI is highly sensitive and specific for CDH, however it is not the routine test of choice. It is reserved for patients with suspicious findings on US.

MRI serves to:

Confirm US findings

Detail the anatomy

Look for associated anomalies

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

15

Page 16: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Our Pt Fetal MRI at 19w6d showing fluid filled bowel in thorax

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

Enclosed in the box are fluid filled bowel loops in thorax that are high signal (bright) on T2

Children’s Hospital BostonSagittal T2 through fetal midline

16

Page 17: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Our Pt Fetal MR at 36w5dJuwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

Findings include: Right sided esophagusleading into the stomach in its abnormal location in the thorax and fluid filled bowel loops in thorax. These fluid filled structures appear high signal (bright) on T2

Children’s Hospital BostonCoronal T2 Fetal MR 17

Page 18: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

We just looked at early and late gestation MRI findings in CDH

Lets move on to the use of imaging modalities to determine fetal prognosis…

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

18

Page 19: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Prognostic Predictors on Fetal US

The degree of development of fetal lungs is the most important determinant of fetal prognosis in patients with CDH.

Therefore, the ability to estimate the degree of pulmonary hypoplasia

and pulmonary HTN are important in

determining prognosis

US measurement of lung-to-head ratio(LHR) provides estimate of the degree of pulmonary hypoplasia.

LHR>0.85 predicts survival in isolated left sided CDH (Sens: 95% Spec: 64%).

LHR ≥

1 signifies better prognosis

Pulmonary HTN can be estimated using US Doppler

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

19

Aspelund G, et al. Prenatal Lung-Head Ratio: Threshold to Predict Outcome for Congenital Diaphragmatic Hernia.

Page 20: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Prognostic Predictors on Fetal MRI

MRI estimated total lung volume (TLV) predicts degree of pulmonary hypoplasia.

A TLV >40 mL

had a 90% survival vs. 35% survival for a TLV <20 mL

MRI can also be used to measure the Percent Predicted Lung Volume (PPLV)

A PPLV < 15% required prolonged ECMO support and had a 40% survival rate.

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

20

Barnewolt CE, Kunisaki SM, Fauza DO, et al. Percent predicted lung volumes as measured on fetal magnetic resonance imaging: a useful biometric parameter for risk stratification in CDHChavhan G B et al. Multimodality imaging of the pediatric diaphragm: anatomy and pathologic conditions.

Page 21: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Other Prognostic Predictors

Presence of associated congenital anomalies worsens prognosis

Echocardiogram assess cardiac anomalies

US guided amniocentesis for karyotype

Presence of liver in the thorax

associated with worse prognosis (sensitivity= 73%, specificity= 54%)

Bilateral herniation

associated with worse prognosis

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

21

Page 22: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Our patient’s prognostic indicators

LHR (recall >0.85 predicts survival (Sens: 95% Spec: 64%))

at 19w6d = 0.8

at 22w =0.7 (measured at a different institution)

Percent predicted lung volume (PPLV) (recall <15% associated with need for ECMO)

At 19w6d = 24%

At 22w = 18%

At 36w = 14%

Besides right sided heart, normal echocardiogram

Normal karyotype

(46XX)

Overall, our patient is estimated to have severe pulmonary hypoplasia

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

22

Page 23: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

We have looked at the role of US and MRI in prenatal diagnoses of CDH

We have reviewed the role of both modalities in determining prognosis in patients with CDH

Lets move on to the postnatal diagnosis and treatment of CDH and the postnatal outcome of our patient

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

23

Page 24: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Postnatal DiagnosisPostnatal diagnoses is made on the bases of:

Clinical presentation. The patient presents with:

Respiratory distress

PMI displaced to the right

Bowel sounds in chest

Increased chest diameter

Scaphoid

abdomen

Plain film

Demonstration of abdominal contents in thorax

In prenatally diagnosed patients, postnatal plain film is used to confirm diagnosis

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

24

Page 25: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Our Pt 1 day old Postnatal plain film showing CDH

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

Children’s Hospital Boston plain film of chest and abdomen

•Air filled bowel loops in chest•Absence of bowel loops in abdomen•Right sided cardiac silhouette•Carina shifted right with endotracheal tube in place•Esophageal tube in place terminating in stomach in its abnormal position in the thorax

25

Despite prenatal prediction of severe pulmonary hypoplasia, our patient never required ECMO, she did however need to be intubated.

Page 26: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Treatment of CDH

Fetal Intervention:

fetal tracheal obstruction (experimental).○

Amniotic fluid produced in the lungs stay within the lungs and provide an expansive force to promote lung development.

Postnatal Intervention:

Physiologic stability of the newborn

Followed by surgical repair○

Reduction of abdominal viscera○

Primary closure of defect or○

Closure of defect with patch

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

26

Page 27: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Our Pt Surgical FindingsComplete agenesis of left diaphragmSpleen, colon, stomach, small bowel

and liver in herniaSurgically repaired with 6 ×

7cm

porous polytetrafluoroethylene (Gortex) patch

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

27

Page 28: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Our Pt Age:4 days 1 day Post surgical repair

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

Children’s Hospital Boston; plain film of chest and abdomen

The plain film findings •Endotracheal tube in place•Esophageal tube terminates in stomach below diaphragm•Air filled bowel loops in abdominal cavity•Right sided cardiac silhouette •Small left lung•Flattened left hemidiaphragm

28

Page 29: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

Pt 1 Age: 34days 30 days post surgical repair

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

Children’s Hospital BostonPlain film of chest

From the film, we can see that our patient is.•Extubated•Esophageal tube in place•Improvement in left lung size•Left sided cardiac silhouette

29

Our patient was discharged home for the first time at 4months of age. She is currently being treated for pulmonary HTN

Page 30: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

In Summary

Imaging (US, MRI and Plain film) in CDH serves to:

Establish the diagnosis ○

prenatally (US and MRI) and postnatally

(plain film)

Determine prognosis

Detail associated anomalies

Inform postnatal treatment plan

Inform maternal decision making

Evaluate response to treatment

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

30

Page 31: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

AcknowledgementsElizabeth Asch MD, BIDMCAmmar

Sarwar

MD, BIDMC

Emily Hanson, BIDMCTejas

Mehta MD, BIDMC

Behroze

Vachha

MD, BIDMCAmee

Patel MD, BIDMC

Gillian Lieberman MD, BIDMCAugust 2011 core students

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

31

Page 32: Juwarat Ains Kadiri, Harvard Medical School Year III ...eradiology.bidmc.harvard.edu/LearningLab/genito/kadiri.pdf · Juwarat Ains Kadiri, Harvard Medical School Year III. ... Juwarat

References

Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD

•Aspelund G, et al. Prenatal Lung-Head Ratio: Threshold to Predict Outcome for Congenital Diaphragmatic Hernia. J Matern Fetal Neonatal Med. 2011 Aug 4. [Epub ahead of print]•Barnewolt CE, Kunisaki SM, Fauza DO, et al. Percent predicted lung volumes as measured on fetal magnetic resonance imaging: a useful biometric parameter for risk stratification in congenital diaphragmatic hernia. J Pediatr Surg 2007;42:193-7.•Carlo, W.A., Diaphragmatic Hernia, Nelson Textbook of Pediatrics 19th ed. Philadelphia, PA W.B Saunders, 2011 http://www.mdconsult.com.ezp- prod1.hul.harvard.edu/books/page.do?eid=4-u1.0-B978-1-4377-0755-7..00095-6--sc0035&isbn=978-1-4377-0755-7&sid=1191646665&uniqId=273299958- 3#4-u1.0-B978-1-4377-0755-7..00095-6--sc0035 08/2011•Chavhan G B et al. Multimodality imaging of the pediatric diaphragm: anatomy and pathologic conditions. Radiographics 2010;30:1797-1817•Clugston,R.D., John J. Greer, J.J. PhD. Diaphragm development and congenital diaphragmatic hernia. Seminars in Pediatric Surgery (2007) 16, 94-100•Garne E, Haeusler M, Barisic I et al (2002) Congenital diaphragmatic hernia: evaluation of prenatal diagnosis in 20 European regions. Ultrasound Obstet Gynecol 19:329–333•Guibaud L, Filiatrault D, Garel L et al (1996) Fetal congenital diaphragmatic hernia: accuracy of sonography in the diagnosis and prediction of the outcome after birth. AJR 166:1195–1202•Hedrick , H.L. Management of prenatally diagnosed congenital diaphragmatic hernia. Seminars in Fetal & Neonatal Medicine 15 (2010) 21–27•Kline-Fath, B.M. Congenital diaphragmatic hernia Pediatr Radiol 2011 Jul 8 [Epub ahead of print]•Lee, T.C. et al. Late gestation fetal magnetic resonance imaging–derived total lung volume predicts postnatal survival and need for extracorporeal membrane oxygenation support in isolated congenital diaphragmatic hernia. Journal of Pediatric Surgery (2011) 46, 1165–1171•Maheshwari, A.,, Mullassery D, Ba'ath ME, Jesudason EC, et al. Value of liver herniation in prediction of outcome in fetal congenital diaphragmatic hernia: a systematic review and meta-analysis. Ultrasound Obstet Gynecol 2010;35:609-14.

32