Juwarat Ains Kadiri, Harvard Medical School Year III...
Transcript of Juwarat Ains Kadiri, Harvard Medical School Year III...
Juwarat
Ains
Kadiri, Harvard Medical School Year IIIGillian Lieberman, MD
August 2011Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD
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
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
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
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
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
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
Embryonic Development of CDH
Juwarat Ains Kadiri, HMS IIIGillian Lieberman, MD
Chavhan G B et al. Radiographics 2010;30:1797-1817
8
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
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
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
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
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
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
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
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
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
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
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.
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.
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
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
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
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
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.
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
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
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
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
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
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
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