Congenital Diaphragmatic Hernia
Grand Rounds
5/20/11
Christopher Broussard, MD
Lecture Outline
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Case Report
Pathophysiology
Management
Perioperative Planning
Board Review
Example Case
• Should we fix it immediately?
• Not so fast
Case Presentation
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Pt born via C/S at 39 3/7 weeks
Apgars 5&7
Pregnancy complicated by placenta previa
Third trimester ultrasound demonstrated
polyhydramnios - not CDH
• Pt born in outlying community hospital, stabilized
and transferred to Tulane.
• Large diaphragmatic hernia with R Lung collapse
on CXR.
Case Presentation
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DOL #1
PSV
ABG 7.18/62/78.6/24/-6.5 on 90% O2
A-a gradient 500mmHg
Echo: PDA with R
L shunt, PFO, PASP
68mmHg (suprasystemic), enlarged RA and
RV.
Case Presentation
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DOL # 2
ECMO started in the NICU
Treatment with NO
Pulmonary vasodilators
– Sildenafil
– Bosentan
• Dobutamine
Overview of CDH
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Incidence - 1/2500 – 1/5000
Male/Female ratio – 2:1
Left/Right – 5:1
Types
– Posterolateral (Bochdalek): 80%
– Anterior (Morgagni): 2%
– Paraesophageal: 15-20%
Embryologic Development
• Diaphragm, lungs and GI tract begin
development at 4 weeks.
• Pleuroperitoneal canal separate by 9th week
• Rotation and settling of midgut before
fusion of diaphragm
• Limited thoracic space for lung growth
• Genetic vs. environmental
Review of Fetal Circulation
• High resistance
pulmonary circuit
• Low resistance
systemic circuit
• R > L shunt via
foramen ovale and
ductus arteriosus
Pathophysiology
Pathophysiology
• Pulmonary Hypertension
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Pulmonary hypoplasia
Thickened tunica media and adventita
Hypoxemia, hypercarbia and acidosis
Inflammatory mediators (TxA2, LT C4 & D4)
• Suprasystemic pulmonary pressures
to L shunt via PDA and PFO
• Poor cardiac output due to small LV
R
CHD
SIDS
Diagnosis
• Typically prenatal (40%-60% by
Ultrasound)
• Classic Triad – cyanosis, dyspnea, apparent
dextrocardia.
• Scaphoid abdomen
• Bulging chest
• Variable Presentation
Immediate Management
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Determine adequacy of ventilation
Gastric decompression
Limit mask ventilation
Intubation
Gentle ventilation
Associated Abnormalities
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10-40% overall, often coexist
Cardiovascular 13-23%
CNS 28%
GI 20%
GU 15%
Migliazza L, et al. J Pediatr Surg
2007
Prognosis
• Mortality 20-60%
• Stege G, et al. Pediatrics 2003
– Case selection bias
– Additional defects – 79% v 30%
• Prenatal findings
– LHR, PA diameter, thoracic liver
Timing of Surgery
• Why would repair worsen pulmonary
compliance?
– Increased intra abdominal pressure
– Decreased chest wall compliance
Timing of Surgery
• Historical Management
– Hyperventilate with 100% O2
– Immediate surgical repair
– Use of ECMO post operatively
Timing of Surgery
• Nakayama DK, et al. J Pediatr 1991
– Medical stabilization (2-11 days) vs.
emergency surgery
– Decreased mortality
– Improvement noted in pulmonary mechanics
Protective Ventilation
• Wung JT, et al. J Pediatr Surg 1995
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Spontaneous, pressure support ventilation
Permissive Hypercapnia
Preductal saturation >90%
Improved survival and decreased need for
ECMO with delayed surgery
HFOV
• Typically used as rescue
therapy in CDH
• 3.5-15Hz
HFOV
• No prospective trials of HFOV vs. traditional
ventilation in CDH
• Migliazza L, et al. J Pediatr Surg 2007
– Retrospective review of 111 cases intended to
look at prognostic factors.
– HFOV used as primary mode of ventilation
• Bhuta T, et al. Cochrane Database Syst Rev 2001
– Infants with respiratory failure, all types
– No survival improvement
Nitric Oxide
• Crosses cell membrane, increases cGMP
concentration
• Selective pulmonary vasodilator
• Free radical with short half life
• Potential for methemoglobinemia and
accumulation of NO2 with prolonged use.
Nitric Oxide
• Controversial in CDH
• Finer NN, Barrington KJ. Cochrane Database Syst
Rev 2006
– Supports use of Nitric Oxide in PPH hypoxic
respiratory failure
• NINOS. Pediatrics 1997
– No improvement in mortality
– Increased risk of ECMO
ECMO
• Historically central in
treatment of CDH
• Utilization has fallen
in current practice
• Is ECMO a good idea
for this patient?
ECMO
• CDH Study Group. J Pediatr Surg 1999
– mortality data for ECMO vs. no ECMO based upon
predicted mortality.
– Birth weight and Apgar score
– ECMO improved survival in infants with >80%
predicted mortality only.
– Worse mortality in the remainder of the patients.
ECMO
• Wilson JM, et al. J Pediatr Surg 1997
– Retrospective review of cases at two tertiary
centers.
– Toronto – HFOV as rescue
– Boston – ECMO as rescue
– Similar mortality data between the two centers.
ECMO
• A-a gradient 600mgHg
• PIP > 25cmH2O
• Oxygenation Index
– MAP×FiO2/PaO2
– >40
Case Presentation
• Serial echocardiograms
decreased PAP,
reversal of PFO and PAD shunts L
R
• ECMO weaned on DOL#12
• Sepsis/DIC – delay
• Closure on DOL#16
Preoperative Evaluation
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4kg
NO(40ppm), Sildenafil, Bosentan
Dopamine 12 mcg/Kg/min
Fentanyl/versed
TPN
IJ central line, UAC
PRVC RR 40, PEEP 5, TV 17
Preoperative Preparation
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Warm room, warm fluids
CVP, Art line
Maintain ventilator settings/NO
Paralysis
Maintain ICU drips (including TPN)
Adequate volume access
Two pulse oximeters
Operative Details
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Subcostal incision
Hepatic Pulmonary fusion
Thoracotomy
Liver resection
Gore-Tex patch
80% defect in R diaphragm
Anesthetic Management
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EBL 400 mL
PRBC 200 mL
FFP 160 mL
Plts 40 mL
UOP 20 mL
Postoperative Course
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Pneumothorax
BPD
Tracheostomy
Ventilator dependence
Nissen Fundoplication
G-Tube
Discharged at 3 months
Hepatic Pulmonary Fusion
• Schmuel K, et al. J Pediatr Surg 1998
• Jeffrey W, et al. J Pediatr Surg 2010
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Case presentation plus literature review
Majority of cases identified intraoperatively
50% success rate in separation
MRI identified as modality most likely to
Complications in CDH survivors
• Respiratory – obstructive disease, poor exercise
tolerance
• GI – oral aversion, GERD, failure to thrive
• Neuro – developmental delay, sensorineural
hearing loss
• Musculoskeletal – pectus excavatum, scoliosis
• Reherniation (10%)
Review
• US is only 50% sensitive for prenatal
Diagnosis
• Early recognition and prompt treatment is
essential
• The use of lung protective ventilation is the
most effective therapy.
Appropriate management of a neonate born with congenital
diaphragmatic hernia (CDH) should include
A. Ventilation of the lungs with a bag and mask to keep
saturation greater than 95%
B. Insertion of an orogastric tube
C. Expansion of the hypoplastic lung with positivepressure ventilation
D. Hyperventilation to keep the PaCO2 below 40 and pH
greater than 7.40
E. Rapid transport to the operating room for surgical
correction
Appropriate management of a neonate born with congenital
diaphragmatic hernia (CDH) should include
B. Insertion of an orogastric tube
Congenital Diaphragmatic Hernia may likely
be associated with all of the following
EXCEPT
A. VSD
B. Meningomyelocele
C. Tetralogy of Fallot
D. Gastroschisis
E. Duodenal Atresia
Congenital Diaphragmatic Hernia may likely
be associated with all of the following
EXCEPT
D. Gastroschisis
References
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Yao SF, Stein D, Savarese J: Congenital Diaphragmatic Hernia. In: Yao and Artusio's Problem
Oriented Anesthesia. Yao, F, editor. 6th ed.; 2008. p. 115-129
Motoyama & Davis: Smith's Anesthesia for Infants and Children, 8th ed.
Yamataka P, Puri P. Pulmonary Artery Structural Changes in Pulmonary Hypertension Complicating
Congenital Diaphragmatic Hernia. Journal of Pediatric Surgery, Vol32, No 3 (March), 1997: pp 387390
Migliazza L, et al. Retrospective study of 111 cases of congenital diaphragmatic hernia treated with
early high-frequency oscillatory ventilation and presurgical stabilization, Journal of Pediatric
Surgery, Volume 42, Issue 9, September 2007
Nakayama D.K., Motoyama E.K., Tagge E.M.: Effect of preoperative stabilization on respiratory
system compliance and outcome in newborn infants with congenital diaphragmatic hernia. J
Pediatr 1991; 118:793-799.
Wung JT, Sahni R, Moffitt ST et al. Congenital diaphragmatic hernia: survival treated with very
delayed surgery, spontaneous respiration and no chest tube. J Pediatr Surg 1995; 30: 406–409.
Bhuta T, Clark RH, Henderson- Smart DJ. Rescue high frequency oscillatory ventilation vs
conventional ventilation for infants with severe pulmonary dysfunction born at or near term.
Cochrane Database Syst Rev 2001; 1: CD0002974
References
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Finer NN, Barrington KJ. Nitric oxide for respiratory failure in infants born at or near term. Cochrane
Database Syst Rev 2006; 4: CD000399
de Buys Roessingh AS, Dinh-Xuan AT (2009) Congenital diaphragmatic hernia: current status and
review of the literature. Eur J Pediatr 168(4):393–406
Neonatal Inhaled Nitric Oxide Study Group, Inhaled nitric oxide and hypoxic respiratory failure in
infants with congenital diaphragmatic hernia, Pediatrics 99 (1997), pp. 838–845.
The Congenital Diaphragmatic Hernia Study Group. Does extracorporeal membrane oxygenation
improve survival in neonates with congenital diaphragmatic hernia? J Pediatr Surg 1999; 34: 720–
725
Azarow K., Messineo A., Pearl R., et al: Congenital diaphragmatic hernia—a tale of two cities: the
Toronto experience. J Pediatr Surg 1997; 32:395-400.
Wilson J.M., Lund D.P., Lillehei C.W., et al: Congenital diaphragmatic hernia—a tale of two cities:
the Boston experience. J Pediatr Surg 1997; 32:401-405.
Katz S, Kidron D, Litmanovitz I, et al. Fibrous fusion between the liver and the lung: an unusual
complication of right congenital diaphragmatic hernia. J Pediatr Surg 1998;33:766-7
References
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Keller RL, Aaroz PA, Hawgood S, et al. MR imaging of hepatic pulmonary fusion in neonates. AJR
Am J Roentgenol 2003;180: 438-40.
Gander JW, Kadenhe-Chiweshe A, Fisher C, et al. Hepatic pulmonary fusion in an infant with a
right-sided congenital diaphragmatic hernia and contralateral mediastinal shift . Journal of Pediatric
Surgery (2010) 45, 265–268