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 Few cases diagnosed prenatally May present after birth with inability to pass an OGT Background Co-morbidities Waterson Classification Spitz Classification Pre-repair Bronchoscopy 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 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 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. Patient Position 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 Fistula Management 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 reintubation Congenital Diaphragmatic Hernia 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-morbidities Co-morbidities Trisomy 13, 18, 21 Goldenhar syndrome Beckwith-Wiedemann syndrome Survival in patients with co-morbidities 15% 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 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 Prenatal Management Balloon Tracheal Occlusion 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 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? 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 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 Exacerbation of Pulmonary HTN PTX on contralateral lung Hemorrhage Hypothermia Abdominal Wall DefectsOmphalocele and Gastroschisis 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 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 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 Low association with other anomalies (10-20%) Gastrointestinal– bowel atresia Genitourinary– cyrptorchidism Chromosomal anomalies: Rare Prematurity common 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 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 Staged Closure– Spring Loaded Silo Preoperative Considerations Optimize the fluid status– Correct hypoglycemia Maintain euthermia Cover mucosal surfaces with plastic wrap NGT decompression Labs Type and Cross +/- ECHO 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 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!!!! Necrotizing Entercolitis 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 Unusual in term neonates First 1-3 days of life Occurs before feedings begin Associations Perinatal asphyxia Congenital Heart Disease Respiratory Distress Risk Factors Prematurity Enteral Feeds Hyperosmolar formula Bacterial infections Umbilical arterial catheters Pathophysiology Reduced Mesenteric Blood Flow Mucosal Ischemia Intestinal Mucosal Injury Pathophysiology What else is affected? Cardiovascular Hypotension Metabolic Hyperglycemia Metabolic Acidosis Hematologic Thrombocytopenia Coagulopathy Anemia Renal Treatment Prevention Feed with breast milk Medical management Stop feeds Optimize hemodynamics and treat with antibiotics Peritoneal drain Surgical exploration 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 Myelomeningocele What is Spina Bifida? Varying Neural Tube Defects Spina Bifida 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-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 Co-morbidities 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 Management of Myelomeningocele Study 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 VP Shunts have Complications