Congenital diaphragmatic hernia2

CONGENITAL CONGENITAL DIAPHRAGMATIC HERNIADIAPHRAGMATIC HERNIA

HOW I DO IT?HOW I DO IT?

Dr.P.NARASIMHA REDDY M.D,DADr.P.NARASIMHA REDDY M.D,DA PROFESSOR AND HOD,PROFESSOR AND HOD, DEPT OF ANAESTHESIOLOGY,DEPT OF ANAESTHESIOLOGY, KURNOOL MEDICAL COLLEGE,KURNOOL MEDICAL COLLEGE,

KURNOOLKURNOOL..

CONGENITAL DIAPHRAGMATIC CONGENITAL DIAPHRAGMATIC HERNIAHERNIA

A 4 hours male infant weighing 3.5kg was A 4 hours male infant weighing 3.5kg was referred from a taluk hospitalreferred from a taluk hospital

Patient cyanosed and in respiratory distressPatient cyanosed and in respiratory distress

How I should proceed? How I should proceed?

PreopPreop

PeropPerop

PostopPostop

CONGENITAL DIAPHRAGMATIC HERNIACONGENITAL DIAPHRAGMATIC HERNIA

INITIAL WORKUPINITIAL WORKUP::

IV line, ?intra arterial line.IV line, ?intra arterial line.Oxygen by mask.Oxygen by mask.Nasogastric tube- decompress the stomach.Nasogastric tube- decompress the stomach.Blood for INV. Blood for INV.

O/EO/E: : Infant in respiratory distress.Infant in respiratory distress.

CyanosedCyanosed

PR:160/minPR:160/min

RR:60/minRR:60/min

BP:50/30mmHgBP:50/30mmHg

Scaphoid abdomenScaphoid abdomen

Barrel chestBarrel chest

Breath sounds decreased on left sideBreath sounds decreased on left side

Apparent DextrocardiaApparent Dextrocardia

Bowel sounds on the left side of chest.Bowel sounds on the left side of chest.


SCAPHIOD ABDOMEN

INV:INV:

X ray chest: bowel loops in the chestX ray chest: bowel loops in the chest Mediastinal shiftMediastinal shift with Ryles tube in situ showing the tip.with Ryles tube in situ showing the tip. X ray abdomenX ray abdomen: Abdomen-relatively devoid of gas.: Abdomen-relatively devoid of gas. Prenatal sonographyPrenatal sonography: Bowel loops in the chest.: Bowel loops in the chest. CT& MRICT& MRI: Defect in the diaphragm: Defect in the diaphragm compressed fetal lungcompressed fetal lung intestinal loops in the thorax.intestinal loops in the thorax. ABGABG: pH:7.2: pH:7.2 paCOpaCO22>40mmHg>40mmHg paOpaO22>60mmHg>60mmHg


COILED FEEDING TUBE

DIFFERENTIAL DIAGNOSISDIFFERENTIAL DIAGNOSIS

Congenital cystic adenomatoid MalformationCongenital cystic adenomatoid MalformationPneumatocoelePneumatocoeleCongenital lobar emphysemaCongenital lobar emphysemaMediastinal cystMediastinal cystEventration of diaphragmEventration of diaphragmAspiration syndromesAspiration syndromesPleural effusionPleural effusion

PREDICTION OF OUTCOMEPREDICTION OF OUTCOME

1)1) SymptomsSymptoms::

<<1hr – grave1hr – grave

<6hrs-moderate(68%)<6hrs-moderate(68%)

>6hrs up to 24hrs-good(59%)>6hrs up to 24hrs-good(59%)

>24hrs-very good(22%)>24hrs-very good(22%)

2) PCO2) PCO22 of 40mmHg, critical ventilation index of 1000, PaO of 40mmHg, critical ventilation index of 1000, PaO22

of 60-100mmHg, Post ductal PaOof 60-100mmHg, Post ductal PaO22>100 at least once in >100 at least once in

24hrs.24hrs.

3) 5 minute APGAR, birth wt, ventilation index, PaCO3) 5 minute APGAR, birth wt, ventilation index, PaCO2 2 & &

PaOPaO22- Keshen et al, a complex equation.- Keshen et al, a complex equation.

4) Echo- left ventricular hypoplasia, prognosis-very bad.4) Echo- left ventricular hypoplasia, prognosis-very bad.

5) Other congenital associated problems- poor prognosis.5) Other congenital associated problems- poor prognosis.

INITIAL TREATMENTINITIAL TREATMENT

1)1) Naso gastric tube- Naso gastric tube- for continuous aspirationfor continuous aspiration

2)2) Patient in propped up Patient in propped up positionposition

3)3) Mask Mask ventilationventilation should not be done should not be done

4)4) IntubatedIntubated, if not done already, if not done already

5)5) VentilatedVentilated with low pressures<30cms of H with low pressures<30cms of H22OO

6)6) Respiratory rateRespiratory rate 30-80/minute 30-80/minute

7)7) ParalysedParalysed

Beware of pneumothorax - ipsi and Beware of pneumothorax - ipsi and contralateral side.contralateral side.


8) 8) SedationSedation- must be haemodynamically - must be haemodynamically stable.stable.

9) 9) Surfactant therapySurfactant therapy as early as possible to as early as possible to avoid barotrauma and valotraumaavoid barotrauma and valotrauma

10) Umbilical artery and venous 10) Umbilical artery and venous catheterscatheters

11) 11) Pulse oxymetryPulse oxymetry to know the shunting of to know the shunting of bloodblood

12) 12) FluidsFluids: optimum doses of crystalloids or : optimum doses of crystalloids or colloids (be ware of pulmonary oedema) colloids (be ware of pulmonary oedema)


13) 13) IonotropesIonotropes: Dopamine/Dobutamine : Dopamine/Dobutamine to maintain BP at around 50mmHg.to maintain BP at around 50mmHg.

14) 14) Metabolic acidosisMetabolic acidosis: Soda bicarb or : Soda bicarb or THAM to maintain pH at 7.25 THAM to maintain pH at 7.25 PCOPCO22<60mmHg, SpO<60mmHg, SpO22 at 75-85%. at 75-85%.

15) 15) Temperature:Temperature: maintained at optimal maintained at optimal levels.levels.

OTHER MODALITIES OF TREATMENTOTHER MODALITIES OF TREATMENT

AIMSAIMS:: To reduce PVR To reduce PVR To prevent RT to lt shuntTo prevent RT to lt shunt This can be accomplished byThis can be accomplished by 1) Hyperventilation1) Hyperventilation 2) Pharmacological therapy2) Pharmacological therapy1)Hyperventiation1)Hyperventiation: HFO reduces PA pressures and : HFO reduces PA pressures and

resistance resulting in better oxygenationresistance resulting in better oxygenation2)Pharmacological:2)Pharmacological: Tolazoline,PGE Tolazoline,PGE1 1 and other drugs and other drugs

and ECMO.and ECMO.TolazolineTolazoline: : αα-adrenergic blocking agent, not specific -adrenergic blocking agent, not specific

to lungs. Complications like hypotension, to lungs. Complications like hypotension, tachycardia, thrombocytopenia seizures and tachycardia, thrombocytopenia seizures and arrhythmias.arrhythmias.

PGEPGE11:: it improves oxygenation by reducing PVR. it improves oxygenation by reducing PVR.

Specialized treatmentSpecialized treatmentHyperventilation and alkalinizationHyperventilation and alkalinization:: GOAL: pH>7.5GOAL: pH>7.5 Complications: CNS and Ear problems and ODC shifted to left.Complications: CNS and Ear problems and ODC shifted to left.Surfactant therapySurfactant therapy:: It improves oxygenation and reduces pulmonary vascular It improves oxygenation and reduces pulmonary vascular

resistance. It is very effective if given prophylactically. resistance. It is very effective if given prophylactically. High frequency jet ventilationHigh frequency jet ventilation: : Very good control over PCOVery good control over PCO22 patient improves within 1hour. If patient improves within 1hour. If

there is no improvement ECMO. Very good for patients who there is no improvement ECMO. Very good for patients who develop pneumothorax.develop pneumothorax.

Inhaled Nitric oxideInhaled Nitric oxide:: It is very good in PP HTN of new born Karama noukian- NO before It is very good in PP HTN of new born Karama noukian- NO before

ECMO- little responseECMO- little response 1week after ECMO- sustained improvement1week after ECMO- sustained improvement Pts treated with surfactant, very good results.Pts treated with surfactant, very good results.Liquid ventilation:Liquid ventilation: Perflurocarbons are used to distend the lung Perflurocarbons are used to distend the lung

provide gas exchange. Animal experiments show good results.provide gas exchange. Animal experiments show good results.

ECMOECMO

ECMOECMO: : VV/VA.VV/VA. Indications: 1) Failure of medical treatment to reverse hypoxia.Indications: 1) Failure of medical treatment to reverse hypoxia. 2) Acute clinical deterioration2) Acute clinical deterioration 3) P[(A-a)O3) P[(A-a)O22]>600mmHg for 8hrs]>600mmHg for 8hrs

4) O4) O22 Index Index

[(FIO[(FIO22X MAP)/PaO] of 51 for 5hrs.X MAP)/PaO] of 51 for 5hrs. 5) Failure to respond to maximal therapy.5) Failure to respond to maximal therapy. 6) Cardiac arrest.6) Cardiac arrest.

VA ECMO-VA ECMO- Flow 100ML/KG/MIN Flow 100ML/KG/MIN Goals: PaO2:60-100mmHgGoals: PaO2:60-100mmHg PaCOPaCO22;30-45mmHg.;30-45mmHg.

VV ECMO-VV ECMO- Does not support cardiovascular function and PaO Does not support cardiovascular function and PaO2 2 less. less.

ECMOECMO Inclusion criteriaInclusion criteria:: 1)80% predicted mortality with conventional therapy1)80% predicted mortality with conventional therapy 2)34 week completed gestation.2)34 week completed gestation. 3)Body wt 2kg.3)Body wt 2kg.

Exclusion criteriaExclusion criteria:: 1) Evidence of IVH 1) Evidence of IVH 2) Patient on mechanical ventilation for >10days.2) Patient on mechanical ventilation for >10days. 3) hypoxia secondary to CHD.3) hypoxia secondary to CHD. 4)Other anomalies influencing survival.4)Other anomalies influencing survival.

ECMOECMO

Complications:Complications:

1) Canulation, ligation of right common 1) Canulation, ligation of right common carotid, right Internal Jugular veincarotid, right Internal Jugular vein

2) Heparinization 2) Heparinization

3) Blood products exposure3) Blood products exposure

4) Sickness to face ECMO4) Sickness to face ECMO

5) ECMO circuit malfunction5) ECMO circuit malfunction

6) Bleeding (CNS)6) Bleeding (CNS)

7) Hypertension7) Hypertension

IDEAL TIME FOR SURGERYIDEAL TIME FOR SURGERY

Early intervention is not necessary as Early intervention is not necessary as previously thought.previously thought.

Trouble is with hypoplasia, low surfactant Trouble is with hypoplasia, low surfactant and Pul. Vas. Resistance but not due to and Pul. Vas. Resistance but not due to herniation of viscera.herniation of viscera.

Ideal time – controversial, some say Ideal time – controversial, some say 24hrs,but operated 7-10days after good 24hrs,but operated 7-10days after good stabilization and Echo findings showing stabilization and Echo findings showing decreased PVR is Idealdecreased PVR is Ideal..

Types of surgeriesTypes of surgeries

Repair of CDHRepair of CDHLung transplantLung transplantLobe transplantLobe transplant

PREMEDICATIONPREMEDICATION No premedication required generally.No premedication required generally.

MONITORSMONITORSRESPRESP: Precordial and esophageal steth.: Precordial and esophageal steth. CapnometerCapnometer Inspiratory pressure gaugeInspiratory pressure gauge ABG.ABG.CVSCVS: ECG: ECG NIBPNIBP CVPCVP Arterial lineArterial lineTEMPTEMP: Oesophageal and rectal temperature.: Oesophageal and rectal temperature.

INDUCTION & MAINTENANCEINDUCTION & MAINTENANCE

Awake intubation after preoxygenation.Awake intubation after preoxygenation.Patient is vigorous muscle relaxant is usedPatient is vigorous muscle relaxant is usedNo IPPV before intubation. No IPPV before intubation. Inhalational anaesthetics depending on the Inhalational anaesthetics depending on the

patient.patient.Muscle relaxant and IPPV.Muscle relaxant and IPPV.Nitrous oxide should not be used.Nitrous oxide should not be used.Ventilation with low pressures, high Ventilation with low pressures, high

respiratory rate. respiratory rate.

TEMPERATURE MAINTENANCETEMPERATURE MAINTENANCE

Switch off the Air conditioners. Switch off the Air conditioners. Maintain OT temp at 27Maintain OT temp at 27ooC.C.Radiant warmers and heating blankets. Radiant warmers and heating blankets. Warm and humidified inspired gases.Warm and humidified inspired gases.Warm IV, irrigating fluids and blood.Warm IV, irrigating fluids and blood.

PEROPERATIVE PROBLEMSPEROPERATIVE PROBLEMS

Inability to close the abdomen - Silastic Inability to close the abdomen - Silastic poutch (be ware of increased intra-poutch (be ware of increased intra-abdominal pressure).abdominal pressure).

Sudden deterioration at the end of surgery Sudden deterioration at the end of surgery (pneumothorax).(pneumothorax).

HypothermiaHypothermiaBlood lossBlood lossSometimes thoracic incisions may be Sometimes thoracic incisions may be

necessarynecessary

FLUID MAINTAINANCEFLUID MAINTAINANCE

Aims: to correct preop deficits, provide Aims: to correct preop deficits, provide maintenance fluids, intra operative evaporative, maintenance fluids, intra operative evaporative, third space and blood losses.third space and blood losses.

Kidneys are immature and neonates are obligate Kidneys are immature and neonates are obligate sodium loosers, decreased glycogen storesodium loosers, decreased glycogen store

Maintainence fluids: 5% dextrose with1/2NS Maintainence fluids: 5% dextrose with1/2NS 4ML/KG/HR for preoperative looses, 4ML/KG/HR for preoperative looses, 8-10ml/kg/hr RL for peroperative looses. Blood 8-10ml/kg/hr RL for peroperative looses. Blood loss if >10% must be replaced with blood.loss if >10% must be replaced with blood.

EXTUBATE OR CONTINUE VENTILATIONEXTUBATE OR CONTINUE VENTILATION

If patient is vigorous and hernia is small patient, If patient is vigorous and hernia is small patient, can be extubated after good assessment.can be extubated after good assessment.

If not elective ventilation is continued If not elective ventilation is continued postoperatively.postoperatively.

Hypoxemia may be improved by use of PEEP or Hypoxemia may be improved by use of PEEP or CPAP.CPAP.

ICD must be placed on the diseased side and if ICD must be placed on the diseased side and if necessary on the opposite side. No suction should necessary on the opposite side. No suction should be attached to this.be attached to this.

POST-OP MANAGEMENTPOST-OP MANAGEMENT

““Honeymoon periodHoneymoon period” – Rapid recovery ” – Rapid recovery followed by sudden deterioration. followed by sudden deterioration.

Mortality depends on: Mortality depends on: 1) Pulmonary hypoplasia1) Pulmonary hypoplasia 2) Associated congenital defects 2) Associated congenital defects 3) Inadequate preop preparation3) Inadequate preop preparation 4) Ineffective postop management4) Ineffective postop management

Long term follow upLong term follow up

Severely affected neonates will have chronic Severely affected neonates will have chronic lung problems treated with supplemental lung problems treated with supplemental oxygen, diuretics and steroids.oxygen, diuretics and steroids.

Long term steroid controversial- evaluate Long term steroid controversial- evaluate CNS and ear function.CNS and ear function.

Incidence of GERD medical and surgical Incidence of GERD medical and surgical (Nissen or Thal) treatment(Nissen or Thal) treatment..

REFERENCESREFERENCES Areechon W, Reid L: Hypoplasia of the lung associated Areechon W, Reid L: Hypoplasia of the lung associated

with congenital diaphragmatic hernia. Br Med J 1963; i: with congenital diaphragmatic hernia. Br Med J 1963; i: 230-233 230-233

Clark RH, Hardin WD Jr, Hirschl RB: Current surgical Clark RH, Hardin WD Jr, Hirschl RB: Current surgical management of congenital diaphragmatic hernia: a report management of congenital diaphragmatic hernia: a report from the Congenital Diaphragmatic Hernia Study Group. from the Congenital Diaphragmatic Hernia Study Group. J Pediatr Surg 1998 Jul; 33(7): 1004-9 J Pediatr Surg 1998 Jul; 33(7): 1004-9

Lally KP: Extracorporeal membrane oxygenation in Lally KP: Extracorporeal membrane oxygenation in patients with congenital diaphragmatic hernia. Semin patients with congenital diaphragmatic hernia. Semin Pediatr Surg 1996 Nov; 5(4): 249-55 Pediatr Surg 1996 Nov; 5(4): 249-55

NINOS: Inhaled nitric oxide and hypoxic respiratory NINOS: Inhaled nitric oxide and hypoxic respiratory failure in infants with congenital diaphragmatic hernia. failure in infants with congenital diaphragmatic hernia. The Neonatal Inhaled Nitric Oxide Study Group (NINOS). The Neonatal Inhaled Nitric Oxide Study Group (NINOS). Pediatrics 1997 Jun; 99(6): 838-45 Pediatrics 1997 Jun; 99(6): 838-45

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Congenital diaphragmatic hernia2

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