Congenital diaphragmatic hernia2

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Congenital diaphragmatic hernia2

  1. 3. CONGENITAL DIAPHRAGMATIC HERNIA HOW I DO IT? Dr.P.NARASIMHA REDDY M.D,DA PROFESSOR AND HOD, DEPT OF ANAESTHESIOLOGY, KURNOOL MEDICAL COLLEGE, KURNOOL .
  2. 5. CONGENITAL DIAPHRAGMATIC HERNIA <ul><li>A 4 hours male infant weighing 3.5kg was referred from a taluk hospital </li></ul><ul><li>Patient cyanosed and in respiratory distress </li></ul><ul><li>How I should proceed? </li></ul><ul><li>Preop </li></ul><ul><li>Perop </li></ul><ul><li>Postop </li></ul>
  3. 6. CONGENITAL DIAPHRAGMATIC HERNIA <ul><li>INITIAL WORKUP : </li></ul><ul><li>IV line, ?intra arterial line. </li></ul><ul><li>Oxygen by mask. </li></ul><ul><li>Nasogastric tube- decompress the stomach. </li></ul><ul><li>Blood for INV. </li></ul>
  4. 7. <ul><li>O/E : Infant in respiratory distress. </li></ul><ul><li>Cyanosed </li></ul><ul><li>PR:160/min </li></ul><ul><li>RR:60/min </li></ul><ul><li>BP:50/30mmHg </li></ul><ul><li>Scaphoid abdomen </li></ul><ul><li>Barrel chest </li></ul><ul><li>Breath sounds decreased on left side </li></ul><ul><li>Apparent Dextrocardia </li></ul><ul><li>Bowel sounds on the left side of chest. </li></ul>CONGENITAL DIAPHRAGMATIC HERNIA
  5. 8. SCAPHIOD ABDOMEN
  6. 9. <ul><li>INV: </li></ul><ul><li>X ray chest: bowel loops in the chest </li></ul><ul><li>Mediastinal shift </li></ul><ul><li>with Ryles tube in situ showing the tip. </li></ul><ul><li>X ray abdomen : Abdomen-relatively devoid of gas. </li></ul><ul><li>Prenatal sonography : Bowel loops in the chest. </li></ul><ul><li>CT& MRI : Defect in the diaphragm </li></ul><ul><li>compressed fetal lung </li></ul><ul><li>intestinal loops in the thorax. </li></ul><ul><li>ABG : pH:7.2 </li></ul><ul><li>paCO 2 >40mmHg </li></ul><ul><li>paO 2 >60mmHg </li></ul>CONGENITAL DIAPHRAGMATIC HERNIA
  7. 10. COILED FEEDING TUBE
  8. 14. DIFFERENTIAL DIAGNOSIS <ul><li>Congenital cystic adenomatoid Malformation </li></ul><ul><li>Pneumatocoele </li></ul><ul><li>Congenital lobar emphysema </li></ul><ul><li>Mediastinal cyst </li></ul><ul><li>Eventration of diaphragm </li></ul><ul><li>Aspiration syndromes </li></ul><ul><li>Pleural effusion </li></ul>
  9. 15. PREDICTION OF OUTCOME <ul><li>Symptoms : </li></ul><ul><li>< 1hr – grave </li></ul><ul><li><6hrs-moderate(68%) </li></ul><ul><li>>6hrs up to 24hrs-good(59%) </li></ul><ul><li>>24hrs-very good(22%) </li></ul><ul><li>2) PCO 2 of 40mmHg, critical ventilation index of 1000, PaO 2 of 60-100mmHg, Post ductal PaO 2 >100 at least once in 24hrs. </li></ul><ul><li>3) 5 minute APGAR, birth wt, ventilation index, PaCO 2 & PaO 2 - Keshen et al, a complex equation. </li></ul><ul><li>4) Echo- left ventricular hypoplasia, prognosis-very bad. </li></ul><ul><li>5) Other congenital associated problems- poor prognosis. </li></ul>
  10. 16. INITIAL TREATMENT <ul><li>Naso gastric tube- for continuous aspiration </li></ul><ul><li>Patient in propped up position </li></ul><ul><li>Mask ventilation should not be done </li></ul><ul><li>Intubated , if not done already </li></ul><ul><li>Ventilated with low pressures<30cms of H 2 O </li></ul><ul><li>Respiratory rate 30-80/minute </li></ul><ul><li>Paralysed </li></ul><ul><li>Beware of pneumothorax - ipsi and </li></ul><ul><li>contralateral side. </li></ul>
  11. 17. INITIAL TREATMENT <ul><li>8) Sedation - must be haemodynamically stable. </li></ul><ul><li>9) Surfactant therapy as early as possible to avoid barotrauma and valotrauma </li></ul><ul><li>10) Umbilical artery and venous catheters </li></ul><ul><li>11) Pulse oxymetry to know the shunting of blood </li></ul><ul><li>12) Fluids : optimum doses of crystalloids or colloids (be ware of pulmonary oedema) </li></ul>
  12. 18. INITIAL TREATMENT <ul><li>13) Ionotropes : Dopamine/Dobutamine to maintain BP at around 50mmHg. </li></ul><ul><li>14) Metabolic acidosis : Soda bicarb or THAM to maintain pH at 7.25 PCO 2 <60mmHg, SpO 2 at 75-85%. </li></ul><ul><li>15) Temperature: maintained at optimal levels. </li></ul>
  13. 19. OTHER MODALITIES OF TREATMENT <ul><li>AIMS : To reduce PVR </li></ul><ul><li>To prevent RT to lt shunt </li></ul><ul><li>This can be accomplished by </li></ul><ul><li>1) Hyperventilation </li></ul><ul><li>2) Pharmacological therapy </li></ul><ul><li>1)Hyperventiation : HFO reduces PA pressures and resistance resulting in better oxygenation </li></ul><ul><li>2)Pharmacological: Tolazoline,PGE 1 and other drugs and ECMO. </li></ul><ul><li>Tolazoline : α -adrenergic blocking agent, not specific to lungs. Complications like hypotension, tachycardia, thrombocytopenia seizures and arrhythmias. </li></ul><ul><li>PGE 1 : it improves oxygenation by reducing PVR. </li></ul>
  14. 20. Specialized treatment <ul><li>Hyperventilation and alkalinization : </li></ul><ul><li>GOAL: pH>7.5 </li></ul><ul><li>Complications: CNS and Ear problems and ODC shifted to left. </li></ul><ul><li>Surfactant therapy : </li></ul><ul><li>It improves oxygenation and reduces pulmonary vascular resistance. It is very effective if given prophylactically. </li></ul><ul><li>High frequency jet ventilation : </li></ul><ul><li>Very good control over PCO 2 patient improves within 1hour. If there is no improvement ECMO. Very good for patients who develop pneumothorax. </li></ul><ul><li>Inhaled Nitric oxide : </li></ul><ul><li>It is very good in PP HTN of new born Karama noukian- NO before ECMO- little response </li></ul><ul><li>1week after ECMO- sustained improvement </li></ul><ul><li>Pts treated with surfactant, very good results. </li></ul><ul><li>Liquid ventilation: Perflurocarbons are used to distend the lung provide gas exchange. Animal experiments show good results. </li></ul>
  15. 21. ECMO <ul><li>ECMO : VV/VA. </li></ul><ul><li>Indications: 1) Failure of medical treatment to reverse hypoxia. </li></ul><ul><li>2) Acute clinical deterioration </li></ul><ul><li>3) P[(A-a)O 2 ]>600mmHg for 8hrs </li></ul><ul><li>4) O 2 Index </li></ul><ul><li>[(FIO 2 X MAP)/PaO] of 51 for 5hrs. </li></ul><ul><li>5) Failure to respond to maximal therapy. </li></ul><ul><li>6) Cardiac arrest. </li></ul><ul><li>VA ECMO- Flow 100ML/KG/MIN </li></ul><ul><li>Goals: PaO2:60-100mmHg </li></ul><ul><li>PaCO 2 ;30-45mmHg. </li></ul><ul><li>VV ECMO- Does not support cardiovascular function and PaO 2 less. </li></ul>
  16. 22. ECMO <ul><li>Inclusion criteria : </li></ul><ul><li>1)80% predicted mortality with conventional therapy </li></ul><ul><li>2)34 week completed gestation. </li></ul><ul><li>3)Body wt 2kg. </li></ul><ul><li>Exclusion criteria : </li></ul><ul><li>1) Evidence of IVH </li></ul><ul><li>2) Patient on mechanical ventilation for >10days. </li></ul><ul><li>3) hypoxia secondary to CHD. </li></ul><ul><li>4)Other anomalies influencing survival. </li></ul>
  17. 23. ECMO <ul><li>Complications: </li></ul><ul><li>1) Canulation, ligation of right common carotid, right Internal Jugular vein </li></ul><ul><li>2) Heparinization </li></ul><ul><li>3) Blood products exposure </li></ul><ul><li>4) Sickness to face ECMO </li></ul><ul><li>5) ECMO circuit malfunction </li></ul><ul><li>6) Bleeding (CNS) </li></ul><ul><li>7) Hypertension </li></ul>
  18. 24. IDEAL TIME FOR SURGERY <ul><li>Early intervention is not necessary as previously thought. </li></ul><ul><li>Trouble is with hypoplasia, low surfactant and Pul. Vas. Resistance but not due to herniation of viscera. </li></ul><ul><li>Ideal time – controversial, some say 24hrs,but operated 7-10days after good stabilization and Echo findings showing decreased PVR is Ideal . </li></ul>
  19. 25. Types of surgeries <ul><li>Repair of CDH </li></ul><ul><li>Lung transplant </li></ul><ul><li>Lobe transplant </li></ul>
  20. 26. PREMEDICATION <ul><li>No premedication required generally. </li></ul><ul><li>MONITORS </li></ul><ul><li>RESP : Precordial and esophageal steth. </li></ul><ul><li>Capnometer </li></ul><ul><li>Inspiratory pressure gauge </li></ul><ul><li>ABG. </li></ul><ul><li>CVS : ECG </li></ul><ul><li>NIBP </li></ul><ul><li>CVP </li></ul><ul><li>Arterial line </li></ul><ul><li>TEMP : Oesophageal and rectal temperature. </li></ul>
  21. 27. INDUCTION & MAINTENANCE <ul><li>Awake intubation after preoxygenation. </li></ul><ul><li>Patient is vigorous muscle relaxant is used </li></ul><ul><li>No IPPV before intubation. </li></ul><ul><li>Inhalational anaesthetics depending on the patient. </li></ul><ul><li>Muscle relaxant and IPPV. </li></ul><ul><li>Nitrous oxide should not be used. </li></ul><ul><li>Ventilation with low pressures, high respiratory rate. </li></ul>
  22. 28. TEMPERATURE MAINTENANCE <ul><li>Switch off the Air conditioners. </li></ul><ul><li>Maintain OT temp at 27 o C. </li></ul><ul><li>Radiant warmers and heating blankets. </li></ul><ul><li>Warm and humidified inspired gases. </li></ul><ul><li>Warm IV, irrigating fluids and blood. </li></ul>
  23. 29. PEROPERATIVE PROBLEMS <ul><li>Inability to close the abdomen - Silastic poutch (be ware of increased intra-abdominal pressure). </li></ul><ul><li>Sudden deterioration at the end of surgery (pneumothorax). </li></ul><ul><li>Hypothermia </li></ul><ul><li>Blood loss </li></ul><ul><li>Sometimes thoracic incisions may be necessary </li></ul>
  24. 30. FLUID MAINTAINANCE <ul><li>Aims: to correct preop deficits, provide maintenance fluids, intra operative evaporative, third space and blood losses. </li></ul><ul><li>Kidneys are immature and neonates are obligate sodium loosers, decreased glycogen store </li></ul><ul><li>Maintainence fluids: 5% dextrose with1/2NS 4ML/KG/HR for preoperative looses, 8-10ml/kg/hr RL for peroperative looses. Blood loss if >10% must be replaced with blood. </li></ul>
  25. 31. EXTUBATE OR CONTINUE VENTILATION <ul><li>If patient is vigorous and hernia is small patient, can be extubated after good assessment. </li></ul><ul><li>If not elective ventilation is continued postoperatively. </li></ul><ul><li>Hypoxemia may be improved by use of PEEP or CPAP. </li></ul><ul><li>ICD must be placed on the diseased side and if necessary on the opposite side. No suction should be attached to this. </li></ul>
  26. 33. POST-OP MANAGEMENT <ul><li>“ Honeymoon period ” – Rapid recovery followed by sudden deterioration. </li></ul><ul><li>Mortality depends on: </li></ul><ul><li>1) Pulmonary hypoplasia </li></ul><ul><li>2) Associated congenital defects </li></ul><ul><li>3) Inadequate preop preparation </li></ul><ul><li>4) Ineffective postop management </li></ul>
  27. 34. Long term follow up <ul><li>Severely affected neonates will have chronic lung problems treated with supplemental oxygen, diuretics and steroids. </li></ul><ul><li>Long term steroid controversial- evaluate CNS and ear function. </li></ul><ul><li>Incidence of GERD medical and surgical (Nissen or Thal) treatment . </li></ul>
  28. 35. REFERENCES <ul><li>Areechon W, Reid L: Hypoplasia of the lung associated with congenital diaphragmatic hernia. Br Med J 1963; i: 230-233 </li></ul><ul><li>Clark RH, Hardin WD Jr, Hirschl RB: Current surgical management of congenital diaphragmatic hernia: a report from the Congenital Diaphragmatic Hernia Study Group. J Pediatr Surg 1998 Jul; 33(7): 1004-9 </li></ul><ul><li>Lally KP: Extracorporeal membrane oxygenation in patients with congenital diaphragmatic hernia. Semin Pediatr Surg 1996 Nov; 5(4): 249-55 </li></ul><ul><li>NINOS: Inhaled nitric oxide and hypoxic respiratory failure in infants with congenital diaphragmatic hernia. The Neonatal Inhaled Nitric Oxide Study Group (NINOS). Pediatrics 1997 Jun; 99(6): 838-45 </li></ul>
  29. 36. HAPPY MOTHER AND CHILD
  30. 37. Hey Guys Thanks for listening

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