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Neonatal Emergencies. Alyssa Brzenski. Overview. Tracheoesphageal Fistulas Congenital Diaphragmatic Hernias Omphaloceles and Gastroschisis Necrotizing Enterocolitis Myelomeningocele. TEF. Background. TEF/EA associated with 1:2,500-4,000 live births 30% of the neonate are premature

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neonatal emergencies

Neonatal Emergencies

Alyssa Brzenski

overview
Overview

Tracheoesphageal Fistulas

Congenital Diaphragmatic Hernias

Omphaloceles and Gastroschisis

Necrotizing Enterocolitis

Myelomeningocele

background
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
the evidence behind the pre repair bronch
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
thorascopic vs open repair
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
anesthesia for thorascopic
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.

anesthetic considerations
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
extubate or not
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

background2
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
co morbidities2
Co-morbidities

Trisomy 13, 18, 21

Goldenhar syndrome

Beckwith-Wiedemann syndrome

Survival in patients with co-morbidities 15%

diagnosis
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
pathophysiology
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
postnatal management
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
postnatal ventilatory strategy
Postnatal Ventilatory Strategy
  • Gentle ventilation- PIP less than 25cm H20
  • pH> 7.25
  • paCO2<65
  • Preductal Sat>90%
  • Rescue Ventilatory Strategies
    • iNO
    • HFOV
    • ECMO
when can we operate
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
anesthesia for cdh repiars
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

intraoperative complications
Intraoperative Complications

Exacerbation of Pulmonary HTN

PTX on contralateral lung

Hemorrhage

Hypothermia

background omphalocele
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%

co morbidities omphalocele
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
background gastroschisis
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

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

surgical closure
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
preoperative considerations
Preoperative Considerations

Optimize the fluid status–

Correct hypoglycemia

Maintain euthermia

Cover mucosal surfaces with plastic wrap

NGT decompression

Labs

Type and Cross

+/- ECHO

anesthetic considerations1
Anesthetic Considerations

Standard ASA monitors

Adequate IV access

Avoid nitrous oxide

Balanced anesthetic technique– most babies will remain intubated

Fluid, fluid, fluid

abdominal compartment syndrome
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!!!!
background3
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%)
term babies
Term babies
  • Unusual in term neonates
  • First 1-3 days of life
  • Occurs before feedings begin
  • Associations
    • Perinatal asphyxia
    • Congenital Heart Disease
    • Respiratory Distress
risk factors
Risk Factors

Prematurity

Enteral Feeds

Hyperosmolar formula

Bacterial infections

Umbilical arterial catheters

what else is affected
What else is affected?
  • Cardiovascular
    • Hypotension
  • Metabolic
    • Hyperglycemia
    • Metabolic Acidosis
  • Hematologic
    • Thrombocytopenia
    • Coagulopathy
    • Anemia
  • Renal
treatment
Treatment
  • Prevention
    • Feed with breast milk
  • Medical management
    • Stop feeds
    • Optimize hemodynamics and treat with antibiotics
  • Peritoneal drain
  • Surgical exploration
intraoperative management
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

basics of mmc
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

co morbidities3
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
latex allergies
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

post natal mmc repair
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