Neonatal Emergencies

1. Neonatal Emergencies Alyssa Brzenski

2. Overview Tracheoesphageal Fistulas Congenital Diaphragmatic Hernias Omphaloceles and Gastroschisis Necrotizing Enterocolitis Myelomeningocele

3. TEF

4. Background • TEF/EA associated with • 1:2,500-4,000 live births • 30% of the neonate are premature • Few cases diagnosed prenatally • May present after birth with inability to pass an OGT

5. Background

6. Co-morbidities

8. Waterson Classification

9. Spitz Classification

10. Pre-repair Bronchoscopy

11. The Evidence behind the pre-repair Bronch • May change the operative management (changed operative approach in 57% with 31% being crucial changes) • Bronchoscopy can • Define the fistula location • Determine unusual characteristics of the fistula(double fistula or trifurcation) • Determine presence of tracheobronchitis (surgery contraindicated) • Locate the aortic arch • Influence anesthetic management

12. Thorascopic vs. Open Repair • Reduces Musculocutaneous sequelae • 32% of patients have significant musculocutaeous sequelae • 24% with winged scapula • 20% asymmetry of chest wall 2/2 atrophic serratus anterior • 18% developed thoracic scoliosis • Better visualization • Reduced Pain Post-operatively

13. Anesthesia for Thorascopic Rarely need lung isolation as operative lung compressed by CO2 insufflation (5mmHg) Can be associated with mild desaturation requiring 100% O2 or mild hand ventilation. Some centers using HFOV for these repairs to minimize the movement of the operative side (MAP 14-24, Hz=10-14, delta P=20-27, FiO2 adjusted to Sat of 92%) EtCO2 will be falsely low due to compression of the lung and CO2 insufflation.

14. Patient Position

15. Anesthetic Considerations • Routine ASA monitors +/- A-line • Maintence of spontaneous ventilation during induction • Classic teaching that paralysis can be given after fistula ligated • Balanced anesthetic +/- epidural for post-op pain management • May have difficulty with hypercapnia or difficulty ventilating

16. Fistula Management

17. Extubate or Not? Must consider pre-op lung disease and other comorbidities Spontaneous ventilation decreases the stress placed on the suture line Risk of injury to the repaired fistula with re-intubation

18. Congenital Diaphragmatic Hernia

19. Background • 1 in 2,500 births • Location of the defect • 80% left sided • 20% right sided • 1-2% bilateral • Etiology unknown • 50-70% post-natal survival

20. Co-morbidities

21. Co-morbidities Trisomy 13, 18, 21 Goldenhar syndrome Beckwith-Wiedemann syndrome Survival in patients with co-morbidities 15%

22. Diagnosis • Prenatal diagnosis • Ultrasound can detect 50-60% • Fetal MRI can further delineate • Postnatal diagnosis • Respiratory distress • Scaphoid abdomen • Distended Chest • NGT coiled in the chest

23. Pathophysiology • Impaired lung development bilaterally with hypoplasticipsilateral lung • Decreased bronchial branches and alveoli • Increased muscularization into the intraacinar alveoli • Decreased type II pneumocytes • Pulmonary Hypertension and persistent fetal circulation • Hypoxemia, Hypercapnea, and Acidosis

24. Prenatal ManagementBalloon Tracheal Occlusion

25. Postnatal Management • Not a surgical emergency!!!! • Definitive airway control • Minimize airway pressures to avoid pneumothorax • NGT to decompress the stomach • Cardiac Echocardiogram to assess pulmonary HTN

26. Postnatal Ventilatory Strategy • Gentle ventilation- PIP less than 25cm H20 • pH> 7.25 • paCO2<65 • Preductal Sat>90% • Rescue Ventilatory Strategies • iNO • HFOV • ECMO

27. When can we operate? • Delay surgery for • Physiologic stabilization • Improvement in pHTN • Hemodynamically stable • Minimal vent support • Exact criteria is insitution-dependent • Surgery can occur on the HFOV or on ECMO

28. Anesthesia for CDH Repiars Standard ASA monitors and A-line Have adequate access, blood, iNO and inotropes available Minimize peak inspiratory pressures Avoid nitrous oxide Peak airway pressures may increase from increased abdominal pressure following repair DO NOT try to expand the contralateral lung after the repair

29. Intraoperative Complications Exacerbation of Pulmonary HTN PTX on contralateral lung Hemorrhage Hypothermia

30. Abdominal Wall Defects-Omphalocele and Gastroschisis

31. Background Omphalocele 1 in 4000 live births Gender: Males > females Location: Umbilical Membranous Sac: Present Size of defect: > 4 cm (Giant > 5 cm) Liver involvement: 30-50%

32. Co-morbidities- Omphalocele • 50-75% of patients will have other anomalies • Cardiovascular (30-50%)- tetralogy of fallot • Gastrointestinal(25%)- • Genitourinary (25%)- cloacalextrophy • Beckwith-Wiedemann syndrome (10%) • Chromosomal abnormalities- Trisomy 13, 18, 21 • Multiple anomalies more common in minor omphaloceles

33. Background- Gastroschisis 1 in 4000 births Genders: Male = Female Location: Right of the umbilicus Membranous Sac: Absent Size of defect: 2-5 cm Liver involvement: Rare

34. Co-morbidities-- Gastroschisis • Low association with other anomalies (10-20%) • Gastrointestinal– bowel atresia • Genitourinary– cyrptorchidism • Chromosomal anomalies: Rare • Prematurity common

35. Prenatal Care All children with omphalocele or gastroschisis should be born at a hospital with a NICU Vaginal or C-Section are both acceptable birth plans

36. Surgical Closure • Omphalocele has a membranous covering– emergent surgery not necessary • Unless the membranous covering is ruptured • Gastroschisis does not have a membranous covering- • Primary Closure vs • Staged Closure

37. Staged Closure– Spring Loaded Silo

38. Preoperative Considerations Optimize the fluid status– Correct hypoglycemia Maintain euthermia Cover mucosal surfaces with plastic wrap NGT decompression Labs Type and Cross +/- ECHO

39. Anesthetic Considerations Standard ASA monitors Adequate IV access Avoid nitrous oxide Balanced anesthetic technique– most babies will remain intubated Fluid, fluid, fluid

40. Abdominal Compartment Syndrome • Impaired ventilation • Decreased preload and hypotension • Lower limb venous congestion • Arterial compression • Decreased renal perfusion and oliguria • Decreased perfusion to the lower extremities and bowels • Monitor the peak airway pressures during closure of the fascia!!!!

41. Necrotizing Entercolitis

42. Background • Occurs in 1-5 of every 1000 live births • Most common in premature and ELBW neonates • 11.5% of neonates weighing 401-750g will develop • High mortality (15-30%)

43. Term babies • Unusual in term neonates • First 1-3 days of life • Occurs before feedings begin • Associations • Perinatal asphyxia • Congenital Heart Disease • Respiratory Distress

44. Risk Factors Prematurity Enteral Feeds Hyperosmolar formula Bacterial infections Umbilical arterial catheters

45. Pathophysiology

46. Pathophysiology

47. What else is affected? • Cardiovascular • Hypotension • Metabolic • Hyperglycemia • Metabolic Acidosis • Hematologic • Thrombocytopenia • Coagulopathy • Anemia • Renal

49. Treatment • Prevention • Feed with breast milk • Medical management • Stop feeds • Optimize hemodynamics and treat with antibiotics • Peritoneal drain • Surgical exploration

50. Intraoperative Management Standard ASA monitors plus A-line Adequate IV access Narcotic based anesthetic Large volume fluid resuscitation Have pRBC, FFP and Platelets available Glucose source Keep the baby warm