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Table of Contents
Table of Contents.................................................................................................................1 Congenital Diaphramatic Hernia Guidelines.......................................................................1 Delivery Room Management...........................................................................................1 Respiratory Management: Goal Minimi!e "ung #n$ury..............................................1 %edation............................................................................................................................& Hemodynamic Management............................................................................................& General Management.......................................................................................................' (CM)..............................................................................................................................' #ssues in *ostoperative Management...............................................................................' %evere+ Chronic or Rebound pulmonary hypertension................................................' G(R.............................................................................................................................., -eurologic....................................................................................................................,

Congenital Diaphramatic Hernia Guidelines

The guidelines outlined belo. are not to be a substitute for clinical $udgment. /ariation from the guidelines shall depend on the patient0s condition and the clinical $udgment of the health care providers. This guideline is a 1uality assurance document used for the purpose of education and improvement of patient care. This document is not meant to replace an ongoing dialogue .ith the attending physician regarding patient care.

Delivery Room Management

#ntubation 2-o bag mas3 ventilation4 -asogastric tube 2*referrably a Replogle 15 6r4 for gut decompression /entilation ob$ectives: *reductal %a)7 8,9+ *#* : 7, mm Hg o *reductal %a)7 ;,<8,9 acceptable in first 1<7 hrs if there is not significant respiratory distress #f at all possible allo. parents to briefly hold infant as this may be their only chance for an e=tended time after surgery or .ithdra.al.

Respiratory Management: Goal = Minimize Lung Injury

#nitial /ent %ettings o %tart .ith %#M/ *% o 6i)7 1.5+ #M/ '5+ *#* 75<7,+ *((* ,+ #< time 5.,. o #ncrease #M/ up to >5 if severe respiratory distress 2severe retractions+ inade1uate or labile o=ygenation+ tachypnea4. Decrease #<time to 5.&<5.' s4

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7 o Ma= *#* 7, mm Hg o @ean 6i)7 to maintain preductal sats A5<A,9 Targets for %a)7+ pC)7+ p)7 o 6ocus on preductal saturation. o *reductal %a)7 A5<A,9. *reductal saturations of 8,<8A9 are acceptable if there is not significant respiratory distress or increasing acidosis. Bse minimum 6i)7 to achieve goal. o pC)7 : >5. *C)7 >1<>, are acceptable if there is not significant respiratory distress or increasing acidosis. o pH ;.7, 2pH ;.75<;.7' is acceptable if o=ygenation and hemodynamic parameters are ade1uate4 o Regional tissue saturation monitoring Renal C Cerebral saturation >5 o #nade1uate urine output and increasing acidosis are indicators of poor o=ygen delivery and may suggest alteration of target parameters H6) o #ndications for H6) -eed for *#* D 7, mm Hg on %#M/ pC)7 D >, mmg Hg *ersistent preductal %a)7 E 8,<A59 *ersistent respiratory distress -eed for i-) pneumothora= o *arameters Fttempt to maintain MF* 1> 2Most lungs .ith CDH do not represent recruitable tissue4 %tart .ith H! of 15 Goal is for 8<15 rib e=pansion on contralateral side i-) o #ndications *ersistent preductal %a)7 E A59 .ith fi)7 85<1559 )# 7, %ignificant ductal shunting (levated R/ pressures especially if poor R/ function o Dose %tart at 75 ppm @ean .hen fi)7 >59 or improvement in R/ function @eaning guidelines o %ee Targets above o Fttempt to .ean fi)7 first o @ean fi)7 no faster than &<,9 1 &5 min )ther

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& o %urfactant is generally not indicated for treatment of CDH unless there is evidence of RD% on CGR or the infant is premature o Consider milrinone 25., mcg?3g?min if poor response to i-)4 and systemic blood pressure ade1uate Fcute deterioration o Ma3e sure -G tube decompressing the stomach o (nsure (TT patent < suction Fre H%?chest movement?.iggle ade1uate o #f hypotensive+ give normal saline bolus o CGR to rule out airlea3 o (cho .ith .orsening R/ function start i-) if not already started #f already on i-) consider adding milrinone %tart *G( if associated .ith ductal closing Consider #loprost?*G# 2#loprost preferred4 *rostacyclin 2for use in combination .ith i-)4 o #nhaled: *G#: ,5 ng?3g?min & mcg?3g?hr. since neb gives 15cc?hr dilute *G# to 5.& mcg?3g?ml. trial & hrs. o #/: ,<175 ng?3g?min %ide effect: hypotension #loprost inhalation: 5., I 7 mcg?3g?dose every 7<' hours

Sedation
Fvoid routine use of paralytic agents. May use if severe respiratory dysynchrony despite sedation. )piates preferred for sedation. %tart .ith bolus administration. #f needs persistent sedation start continuous infusion Ftivan may be used in con$unction .ith opiates

Hemodynamic Management
Maintain mean H* '5 mm Hg 2term4 #f evidence of **H-+ H* ', < ,5 mm Hg #nitial treatment for hypotension is 15 ml?3g of normal saline. *atients .ith CDH may have significant mediastinal shift inhibiting venous return .hich can be overcome .ith improving intravascular volume status. *ersistent hypotension treated .ith dopamine starting at , mcg?3g?min. Consider adding epinephrine if dose of dopamine D 15 mcg?3g?min Consider milrinone 25., mcg?3g?min4 especially if R/ dysfunction .ith elevated R/ and *F pressures despite i-). Consider *RHC transfusion if hematocrit E '59

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General Management
-*) Replogle 215 6r4 to "#% BFC?B/C Total fluids >5<85 cc?3g?d T*- 2see T*- guidelines4 Maintain Hct '59 preoperatively. Hct &,<'59 is acceptable if lo. vent settings and no hemodynamic support. Regional saturation monitoring *ediatric %urgery consult (chocardiogram Head ultrasound (valuation for dysmorphic features I if present chromosomes and genetics evaluation.

E M!
#ndications I failure of medical management o *reductal %a)7 E 8,9 despite i-)+ inotropic support o %evere lability Contraindications o @eight E 7 3g o GF E &' .ee3s o %evere pulmonary hypoplasia %evere hypercarbia 2see prognostic indicators4 *reductal %a)7 never 8,9 6amilies are provided prenatal consultation regarding these parameters as ones that .e may recommend not going to (CM). They can be told that these parameters lead to either death or significant morbidity+ and .e support them in not providing (CM) o "ac3 of parental consent

Issues in "ostoperative Management

Severe, Chronic or Rebound pulmonary hypertension
o @ho *rolonged mechanical ventilation *ersistent fi)7 need o Monitoring (chocardiogram to evaluate */R monthly Consider initiation of chronic therapy if

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, %uprasystemic *F pressures High *F pressures .ith fi)7 D >59 High *F pressure .ith R/ dysfunction Reecho after initiation of therapy to evaluate effect o *otential therapies Reinitiation or continuation of i-) i-) via nasal cannula if e=tubated %ildenifil 5.& mg?3g?dose 1 8 <17 hrs #loprost inhalation: 5., I 7 mcg?3g?dose every 7<' hours *rostacyclin 2for use in combination .ith i-)4 #nhaled: *G#: ,5 ng?3g?min & mcg?3g?hr. since neb gives 15cc?hr dilute *G# to 5.& mcg?3g?ml. trial & hrs. #/: ,<175 ng?3g?min o %ide effect: hypotension

GER
o "an!opra!ole

Neurologic
o @ithdra.al from sedation? pain management Conversion of fentanyl drip to morphine Convert morphine to methadone iv or )G Methadone 159 .ean a day Ftivan started to help .ith .ean o Fc3no.ledge and socially manage normal baby crying behaviors 2i.e. normal infant .ill cry about & hours a day at ><8 .ee3s. This can be prolonged for sic3 or preterm infants4

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