cdh medical & surgical management
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MEDICAL & SURGICAL
MANAGEMENT
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MEDICAL MANAGEMENT
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GOAL OF TREATMENT
Because of associated persistent pulmonary
hypertension of the newborn (PPHN) and
pulmonary hypoplasia, medical therapy in
patients with congenital diaphragmatic hernia
(CDH) is directed toward optimizing
oxygenation while avoiding barotrauma.
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Delivery Room Management
If known or suspected to have congenital
diaphragmatic hernia:
place a vented orogastric tube
connect it to continuous suction to prevent bowel
distension and further lung compression
For the same reason, avoid mask ventilation
and immediately intubate the trachea.
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Mechanical ventilation strategies
targeted at avoiding high peak inspiratory
pressures and synchronizing ventilation with
the infant's respiratory effort. (Deprest et al. ClinPerinatol. Jun 2009)
In some instances, high-frequency ventilation
(HFV) may be helpful in avoiding the use of
high peak inspiratory pressures. Early use ofHFV showed 90% survival rate. (Bohn Am J RespCrit Care Med, 2002)
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Mechanical ventilation strategies
PaO2concentrations greater than 50 mm Hg
typically provide for adequate oxygen delivery
at the tissue level.
Aiming for higher PaO2 concentrations may
lead to increased ventilator support and
barotrauma.
Barotrauma contributes to up to 25% of CDH
deaths.
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Mechanical ventilation strategies
Similarly, infants with congenital diaphragmatichernia often have hypercarbia because ofpulmonary hypoplasia.
Whether to maintain a low PaCO2
for pulmonaryvasodilation, to allow permissive hypercapnia, orto maintain normocarbia remains controversial.
Retrospective studies suggest that gentleventilation and permissive hypercarbia with
stable hypoxemia (>80%), may be associatedwith improved survival (76%). (Boloker et al. J Ped Surg,2002)
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Alkalinization
Alkalinization was frequently used in the past
because of its ability to produce a rapid
pulmonary vasodilation.
Forced alkalosis can be accomplished either by
using hyperventilation to induce hypocarbia
or by alkali infusions.
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Alkalinization
However, benefits of alkalosis have never
been demonstrated in any prospective clinical
trial, and these therapies are considered
controversial. In addition, alkalosis may resultin undesirable side effects.
For instance, hypocarbia constricts the
cerebral vasculature and reduces cerebralblood flow.
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Alkalinization
Extreme alkalosis and hypocarbia are strongly
associated with later neurodevelopmental
deficits, including a high rate of sensorineural
hearing loss.
Previous studies indicate that the use of alkali
infusions may be associated with increased
use of ECMO and an increased use of oxygenat age 28 days (Walsh-Sukys et al. Pediatrics. Jan 2000).
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Cardiac Support
Inotropic support with dopamine, dobutamine,
or milrinone may be helpful in maintaining
adequate systemic blood pressure; dobutamine
and milrinone may be particularly helpful ifmyocardial dysfunction is present.
Epinephrine infusions may be necessary in
severe cases; low-dose epinephrine (< 0.2mcg/kg/min) may help to promote pulmonary
blood flow and improve cardiac output.
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Nitric Oxide
Nitric oxide does not reduce mortality or the
need for ECMO in infants with congenital
diaphragmatic hernia, although it may
immediately stabilize infants with criticalhypoxemia and reduce the chances of
cardiopulmonary arrest.(Fliman et al. J Pediatr. May 2006)
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Nitric Oxide
Inhaled nitric oxide should be used with
caution if ECMO is not immediately available.
New studies indicate a potential role for long-
term low-dose inhaled nitric oxide therapy inthe treatment of late or recurrent pulmonary
hypertension.
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Sedation
Sedation is an important adjunctive therapy,but the use of paralytic agents remains highlycontroversial.
Although diminished swallowing may bebeneficial, paralysis may promote:
both atelectasis of dependent lung regions andventilation-perfusion mismatch
generalized edema
decreased chest wall compliance
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Surfactant administration
Surfactant administration has also been
shown to produce a transient improvement in
oxygenation in some infants with CDH.
Preliminary reports from the CDH Study Group
showed surfactant administration may worsen
outcome. (Lally et al. J Pediatr Surg 2004)
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SURGICAL MANAGEMENT
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Theoretically, fetal surgery for congenital
diaphragmatic hernia provides an elegant
solution to the difficult problem of congenital
diaphragmatic hernia.
However, a randomized trial showed that in
utero repair did not improve survival
compared with standard therapy.(Harrison et al. NEngl J Med. Nov 2003)
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Subsequent trials of fetal intervention focused
on occluding the fetal trachea.
The fetal lung secretes fluid by active ion
transport through gestation, and this lung
fluid provides a template for lung growth.
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Occlusion of the fetal trachea traps this fluid
and stimulates lung growth, either byretention of growth factors within the lung or
stimulation of local growth factors by the
gentle distension provided by the fluid.
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Unfortunately, a randomized trial in humans
found that fetal tracheal occlusion did not
improve outcome compared with standard
treatment. (Jelin Clin Perinatol. Jun 2009)
Currently, fetal intervention is not indicated in
congenital diaphragmatic hernia, although
some groups continue to offer it on anexperimental basis.
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Until recently, specialists believed that
reduction of the herniated viscera and closure
of the diaphragmatic defect should be
emergently performed following birth.
However, a delayed surgical approach that
enables preoperative stabilization decreases
morbidity and mortality.
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This change in protocol is due to the recentunderstanding that the medical problems ofpulmonary hypoplasia and PPHN are largelyresponsible for the outcome of congenital
diaphragmatic hernia and that the severity ofthese pathophysiologies is largely predeterminedin utero.
Herniated viscera in the chest does not appear to
exacerbate the pathophysiology as long as boweldecompression with a nasogastric tube isadequate.
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Several reports indicate that circulatory stability,respiratory mechanics, and gas exchange deteriorateafter surgical repair. The ideal time to repair acongenital diaphragmatic hernia is unknown.
Some suggest that repair 24 hours after stabilization isideal, but delays of up to 7-10 days are typically welltolerated, and many surgeons now adopt thisapproach. Some surgeons prefer to operate on these
neonates when normal pulmonary artery pressure ismaintained for at least 24-48 hours based onechocardiography.
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Chest tube drainage is necessary when a tensionpneumothorax is present; however, whether routinechest drainage following surgical repair has a role iscontroversial. Some clinicians report improved survival
when chest drainage is not used. Others think that balanced intrathoracic drainage, in
which a closed gated pressure system is used tomaintain intrathoracic pressure within the normal
physiologic range, may minimize risk of pulmonaryinjury and improve respiratory mechanics.
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Approaches for surgical repair
Abdominal subcostal
Preferred because the accompanying malrotation
may be addressed if necessary, and the abdominal
wall may be left open with skin only closed or aSilastic pouch applied if abdominal pressure is
considered excessive
Thoracotomy
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Approaches for surgical repair
Laparoscopic vs Thoracoscopic
MIS ideal for Morgagni hernias but can be
challenging because the peumoperitoneum
widens the defect. Laparoscopy for Bochdaleks has a high failure rate
and is associated with pCO2 and acidemia.
Thoracoscopy is better approach for Bochdalekhernias with recurrence of 14%. Open approach 3-
22%.(Marjorie et al, J Ped Surg, Nov 2003.)
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Approaches for surgical repair
Small defect can be repaired primarily. Large
defect will require abdominal or thoracic
muscle flaps, or prosthetic patch (tension
free).
Synthetic patch (polytetrafluoroethylene) is
now preferred over autologous muscle
transfer or tight primary closure for largedefects.
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PROBLEM LIST
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Problem # 1
RESPIRATORY
S>
O>
A> PPHN sec to Bochdaleks Hernia P>
Dx:
Tx:
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Problem # 2
INFECTIOUS
S>
O>
A> R/O Sepsis
P>
Dx:
Tx:
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Problem # 3
FLUIDS & ELECTROLYTES
S>
O>
A> R/O Sepsis
P>
Dx:
Tx: