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 Etiology unknown
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 Postnatal 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 hypoplastic ipsilateral 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 Management Balloon 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%)- cloacal extrophy
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 g 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. Reduced Mesenteric Blood Flow
Pathophysiology
Reduced Mesenteric Blood Flow
Mucosal Ischemia
Intestinal Mucosal Injury
Reduced blood flow is 2/2 hypotension, PDA

46. Pathophysiology

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

49. Treatment Prevention Medical management Peritoneal drain
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

51. Myelomeningocele

52. What is Spina Bifida?
Back to the basics of embryology… In utero the neural tube is created when the notochord causes its overlying ectoderm to differentiate into the neural plate. This plate then folds along its axis with the two neural crests ultimately fusing to create the neural tube. Failure of this fusion results in a neural tube defect depending on the location of the opening.

53. Varying Neural Tube Defects
Iniencephaly- defect of the cervical vertebra with cervical spina bifida and back bending of the cervical spine

54. Spina Bifida
Spinal Bifida is a neural tube defect with varied neural tissue involvement. SB Occulta has an isolated deficit of the vertebrae. This defect can be ducted by ultrasound. Clinically, a tuff of hair may overlie the defect. Spina bifida with meningocele had vertebral defect with a cystic thecal sac protruding out of the skin. SB with myelomeningocele is a vertebral defect with protrusion of the thecal sac with the spinal cord. Finally SB with myeloschisis is a vertebral defect with exposed neural tissue.

55. Basics of MMC
3.4:10,000 births
Related to low folate levels, anticonvulsants (carbamazepine, valproic acid)
Previous child with same partner is a risk factor
NTD are the second most common congenital disorder behind congenital heart disease. Myelomeningocele is the most common neural tube defect making up 85% of all NTD. This defect has been associated with low folate levels. As such the current recommendation is that any female of childbearing age who may become pregnant should take Folic Acid supplement(0.4mg). This single intervention has decreased the rate of occurrence to 3.4:10000 live births, down from 2:1,000 live births. Women taking anticonvulsants such as carbamazepine and valproic acid face higher rates, as high as 1-2% of these women will deliver a baby with a NTD.

56. Co-morbidities Sensory motor deficits Bowel and Bladder Incontinence
Arnold Chiari Type II
Caudal displacement of cerebellar vermis, fourth ventricle, and lower brainstem
Hydrocephalus
Cognitive delay
Lower risk if no VP Shunt needed
Neurologic deficits reflect the location of the defect. Sensory motor deficit is related to the level of the lesion. This can lead to drastic differences in the ability of a child to walk vs remain wheelchair bound(50% of patients). Bowel and bladder incontinence can be a problem as well, 40-85% have bladder control while 50-85% have bowel control. It is thought that these defects are a result of a 2 hit hypothesis, an open neurotube (hit #1) is then exposed to the amniotic fluid (hit#2) which can worsen prognosis as motor function can be lost throughout the prenatal period.
*Note- Arnold Chiari I—caudal displacement of the cerebellar tonsils
Arnold Chiari II- caudal displacement of the cerebellar vermis, fourth ventricle, lower brainstem

57. Co-morbidities
MMC is associated with Arnold-Chiari Type II lesions(caudal displacement of the cerebellar vermis, fourth ventricle, and lower brainstem below the foramen magnum). This is thought to develop as CSF leaves the thecal sac there is downward displacement of the hindbrain. Hydrocephalus can then develop which may require VP Shunt placement. In addition cranial nerves can be affects, thus vocal cord paralysis with stridor and respiratory distress, apnea, abnormal swallowing and pulmonary aspiration. This may require a trach and g-tube. Also, CO2 and O2 responsiveness may be decreased.

58. Latex Allergies All patients with MMC are labeled as latex allergic
High rates due to recurrent procedures including urinary catheterization
Cross reaction to avocados, banana, passion fruit, kiwi, tomato
All Children with MMC are labeled as Latex allergic. Now that we label these patients the rates of true latex allergy am much lower. It is thought that the rates of latex patients with MMC is due to their high rates of recurrent procedures such as urinary catheterization. FYI… If someone has a latex allergy the protein in some of the great fruits may be similar enough to cause anaphylaxis. Thus, latex individuals should also avoid avocados, banana, passion fruit, kiwi and banana.

59. Management of Myelomeningocele Study
Very few of the fetal interventions that are currently undertaken have prospective studies documenting their superiority. However, with MMC we do have a prospective study that suggests it may improve outcomes, notably that there is a decreased rate of death or VP Shunt within the first year. In addition, the motor function in the children treated with prenatal repairs is significantly better than those treated postnatally. Finally, there was a significant regression in the amount of hindbrain herniation.

62. Post-natal MMC Repair Infants repaired early after birth
Must be cautious to not injury the neural tissue during moving or intubation
Routine ASA monitors
Prone position for repair
May or may not receive VP Shunt at the same time
Typically remain intubated as infant should not lie supine for the first day

63. VP Shunts have Complications
Ventricular Shunts in the Neonatal Period are not a benign procedure. Depending on the size of the neonate the surgeon may not be able to place the shunt in the peritoneal cavity and may instead place in the subgael region only to revise at a later time. In addition, shunt revisions are more common in kids and even more common in neonates as they grow and are more likely to develop infections.