Challenging Cases in Pediatric Anesthesia Claude Abdallah, MD, MSc Children’s National Medical Center Washington DC On Call A newborn with a combination of congenital malformations Abdallah, C. et al.: Management of a neonate with a rare combination of unrepaired major malformations. Annual Meeting of the American Society of Anesthesiologists, San Francisco, CA. Case Presentation One day old female transferred from another hospital. Called to schedule ASAP for rigid bronchoscopy, gastrostomy (concern of tracheoesophageal fistula), with possible closure of an omphalocele. Admitted to NICU few hours ago: o Fluid and electrolyte status optimized (as per NICU) o NPO and head elevated o Bradycardia episodes resolving with stimulation Omphalocele: Gastroschesis: Embryologic process: Failure of abdominal wall to develop. Defect: Central defect; Membranous sac covers the gut; however, the sac may rupture. Incidence:1/5000-1/10,000 (more frequent) Incidence of prematurity: 30% Associated congenital anomalies: 80% Embryologic process: Intrauterine occlusion of the omphalo mesenteric artery. Defect: In abdominal wall lateral to umbilicus; No sac covers intestine Incidence: 1/15,000-1/30,000 Incidence of prematurity: 60% Associated congenital anomalies: Rare Anomalies Associated with Omphalocele • Gastrointestinal: Biliary atresia, imperforated anus. • Cardiovascular: Tetralogy of Fallot, Septal defects. • Urinary: Bladder extrophy, vesicointestinal fistula. • Craniofacial: Cleft lip/palate- • Oro/maxillary tumor-- Potential difficult airway? Differential Syndromes Beckwith-Wiedemann syndrome: (infantile gigantism) - 50% born prematurely, - Birth weight>4kg - Macroglossia, exophtalmos - Visceromegaly, omphalocele - Congenital heart disease - Neonatal hypoglycemia Potentially difficult airway ( Pancreatic islet cell hyperplasia) Requires constant glucose infusion, with close monitoring of blood glucose - Medullary Renal dysplasia Impaired renal drug excretion - Polycythemia Differential Syndromes • Vater Syndrome or VACTERL association: combines: - V: Vertebral Anomalies - A: Imperforate Anus/ Anal atresia - C: Congenital heart disease (VSD usually) - TE: Tracheo-esophageal fistula - R: Renal dysfunction - L: Limb defects, Absent radius Differential Syndromes Pentalogy of Cantrell: Association of: Defects in abdominal wall Diaphragmatic abonormalities: Hernia Sternal deformities Cardiac and Pericardial defects Request Cardiac Echocardiography Hypoplastic LV, coarctation of the aorta with hypoplastic transverse arch, hypoplastic anomalous left pulmonary artery branch, hypoplastic mitral valve, large atrial septal defect and patent ductus arteriosus: Hypoplastic Left Heart Syndrome: HLHS Cardiac Anesthesia Considerations The neonate’s survival is dependent upon ductal patency, as mixed blood in the RV is ejected out the single great vessel (PA) to both the lungs and (through the PDA) the systemic circulation. Severe congestive failure may be present. HLHS • The right ventricle functions as the systemic ventricle, • The ratio of pulmonary to systemic blood flow depends on the balance between systemic vascular resistance (SVR) and pulmonary vascular resistance (PVR). PVR is affected by changes in Paco2, Pao2, acid-base status, body temperature, and lung volumes. • Insure patency of the ductus arteriosus Cardiac Anesthesia Considerations Maintenance of cardiac output and oxygen delivery o Inotropic support, if needed o Blood transfusion Meticulous administration of IV fluids Narcotic/air/oxygen technique Adequate muscle relaxation Unusual Presentation: Defects Association with TEF Congenital diaphragmatic Hernia/ lung hypoplasia Linked to genetic background (trisomy 13,18, deletion at chromosome 15q24-26) and environmental factors (insecticides), deficit in Vit A. One Infant with similar three midline defects: Omphalocele, esophageal atresia, tracheoesophageal fistula, tetralogy of fallot. Pediatr. Surg. Int. 7(1992):37-40. Anesthesia Considerations Tracheoesophageal Fistula (TEF): Esophageal atresia with distal TEF (close to the carina) is most common. RISK OF ASPIRATION. RISK OF GASTRIC DISTENSION with mask ventilation. GOAL: Endotracheal tube (ETT) distal to the fistula an above the carina: Place the ETT into the right mainstem bronchus and slowly withdrawing until bilateral breath sounds are confirmed. Aim is to avoid gastric distension, which may decrease ventilation and venous return to the heart. Anesthesia Considerations AVOID Aspiration pneumoniae (Cardiac!) AVOID OG/NG suctioning (TEF!!) Rapid sequence induction? Modified rapid sequence induction ? Awake intubation? (Cardiac!) Rigid BRONCHOSCOPY part with spontaneous ventilation! RIGID BRONCHOSCOPY Induction Technique: - Anesthetic plan to balance multiple organ system function - Spontaneous ventilation - General Anesthesia - Difficulty monitoring level of anesthesia - Titrate to effect, while avoiding coughing /movement and laryngospam major consequences in this patient. - Avoid urgent gastrostomy with present omphalocele - Increased risk of pulmonary complications from aspiration -Anesthetic plan to maintain hemodynamic stability, Ketamine Pro • Antagonizes the NMDA receptor central dissociation of the cortex from the limbic system good sedation and analgesia while preserving upper airway muscular tone and respiratory drive. • Relaxes the smooth musculature of the airway . Con • Increase the amount of oral secretions (laryngospasm). Glycopyrrolate. • Correlation of ketamine with increased neuronal apoptosis during rapid synaptogenesis after birth. PROPOFOL: Bad Media? Parents Sue After Teen Dies During Wisdom Tooth Surgery By KATIE MOISSE | ABC News – Wed, Dec 14, 2011 Wisdom teeth death: Man dies after removal of wisdom teeth Top News April 4, 2013 By: Bruce Baker Propofol Safe hemodynamic Profile Rapid onset and Recovery Versatile: Short or prolonged effect Titration for all levels of sedation as well as for general anesthesia. • No side effects: Nausea/ Vomiting/ Delirium….. • >DRUG OF CHOICE : MRI sedation with spontaneous ventilation. Delivery Method: Manual Titration Versus Infusion pump? • • • • Total Amount of Fluid Infused Total Amount of Fluid Infused (ml/min) 1.6 1.2 0.8 0.4 0 Infusion Pump Metered Burette *p=0.01 *corrected to the time of infusion *including volume of propofol infused • The desired level of sedation was not statistically significant between both groups. • Both infusion techniques, preserved hemodynamic stability . Other anesthesia agents • Etomidate: Good Hemodynamic stability ?SuppressionAdrenal function • Dexmedetomidine: Spontaneous Ventilation ? Hemodynamic stability • Topical local anesthetics to vocal cords during laryngoscopy INTRAOPERATIVE COURSE IV induction with ketamine/propofol titration. Cover omphalocele with warm sterile saline-soaked gauze and plastic wrap Maintain a neutral thermal environment Prevention of infection: High risk of sepsis would delay cardiac repair, and increased mortality-- Aseptic technique + Strict Antibiotic regimen. Rigid bronchoscopy/ Endotracheal Intubation : Muscle relaxation (rocuronium) and opioid based technique (fentanyl). Surgical Intent: Primary Closure of omphalocele NO access to umbilical vessels Arterial line- Baseline and Follow Up: Hematocrit/acid base status/ Electrolytes: Avoid Hemoconcentration and Metabolic Acidosis: Albumin /(crystalloids) Anesthesia Considerations Omphalocele closure (primary closure) effect on: - pulmonary ventilation pressures - venous return, cardiac output and other hemodynamic parameters 1) Bowel ischemia and eventual wound dehiscence 2) Renal compromise: Oliguria, HTN. Post operative mechanical ventilation management: - At least 24-48 hours - Immature lungs - Limitation of PEEP application … Consider TPN ( even with gastrostomy, postoperative ileus) Bronchoscopy revealed complete tracheal rings below the trachoesophgeal fistula (TEF) and tracheal stenosis. Patient underwent a rigid bronchoscopy, gastrostomy and omphalocele closure. Stable to NICU A week later, a TEF, tracheostomy and an open heart surgery repair were done. For long term growth: Gastrojejunostomy tube in preparation for esophageal atresia repair. Post Anesthesia Care Unit WOULD YOU PLEASE ASSESS THIS PATIENT? ? Review of the anesthesia record: 12 yrs. old female patient , 29 kgs, Severe developmental delay, Severe scoliosis ORIF of lower extremity fracture Lennox-Gastaut syndrome (Difficult-to-treat form of childhood- onset epilepsy, characterized by frequent and different types of seizures.) • Previous surgical history: Botox injections, G tube placement. • Medications: Multivitamins, Valproic acid (Depakote) . • • • • Intraoperative Course • Easy mask inhalation, difficult IV insertion , maintenance with sevoflurane , rocuronium , fentanyl 3mcg/kg and morphine 0.2mg/kg. • Estimated blood loss intraoperative: 80 ml of blood loss. • 500 cc of LR given. • To Recovery Room, uneventful stay for >1 hour then patient became more tired with discrepancy in blood pressure cuff measurements (100/5080/40-70/30 changed to LE 80/30…) • On assessment, patient was noticed to be pale with blood saturation of surgical dressing • A bedside hemocue showed a hemoglobin of 4 g/dl. • Rescuscitation with albumin and PRBC transient improvement followed by deterioration in vital signs, treated with vasopressors. Central line placed. • Transferred to PICU. • In PICU, management necessitating supplementary investigative laboratory testing along with intensive management with blood, blood products and coagulation factors. • Patient subsequently recovered after 3 days and was discharged home. Valproic acid • Valproic acid (VPA) is one of the most frequently prescribed antiepileptic drugs. • Effective in the management of Lennox-Gastaut syndrome and infantile spasms. • Inhibition of repetitive firing of neurons by blockade of voltagesensitive sodium channels, increasing membrane potassium conduction • Increase GABA brain concentrations (>synthesis , blocking conversion ) Abdallah C.: Valproic acid and acquired coagulopathy. Ped. Anaesth. In print. Acquired Coagulopthy - Thrombocytopenia, abnormal platelet function. - Hypofibrinogenemia, and decreased concentrations of von Willebrand factor. - Decreased factor VII and VIII, XIII levels, Protein C, and increased lipoprotein (a) levels even during short-term therapy (1). - The incidence of coagulation disorders related to VPA in children: 4% -20.7% (2, 3). References: 1) J Child Neurol. 2009 Dec;24(12):1493-8 2) Epilepsia. 2006 Jul; 47(7):1136-43 3) J Child Neurol. 2002 Jan; 17(1):41-3 4) Epilepsia. 1992 Jan-Feb; 33(1):178-84. FACTS • Valproate may cause a variety of laboratory abnormalities affecting hemostasis • Always associated with thrombocytopenia? NO • The mechanism of VPA- induced coagulopathy is not well identified • Relationship between plasma VPA levels, duration of therapy and incidence of VPA induced coagulopathy in patients receiving VPA not well defined (VPA dose, length of treatment). • Special attention to this side effect in the preoperative assessment would be highly recommended Acute Epiglottitis • Inflammatory edema of the arytenoids, aryepiglottic folds and the epiglottis; therefore, supraglottitis may be used instead or preferred to the term acute epiglottitis. • A late referral to an acute care setting with its serious consequences • Life-threatening disorder: Potential for laryngospasm and irrevocable loss of the airway. • Acute epiglottitis can occur at any age. Incidence and Pathogens • Used to be Hemophilus influenzae type B (Hib) • Infection with group A b-hemolytic Streptococci has become more frequent after the widespread use of Hemophilus influenzae vaccination. • Incidence of acute epiglottitis in adults : 0.97 to 3.1 per 100,000. Mortality of approximately 7.1%. • The mean annual incidence of acute epiglottitis per 100,000 adults significantly increased from 0.88 (from 1986 to 1990) to 2.1 (from 1991 to 1995) and to 3.1 (from 1996 to 2000). , Pathogens • There is more diversity in the cause of epiglottitis in adults. with often negative sputum cultures and negative blood cultures to Hib.  • Some cases of epiglottitis have been attributed to Candida spp. • Noninfectious causes of epiglottitis may include trauma by foreign objects, inhalation and chemical burns, thermal or caustic injury ( mental disorders or communication difficulties), or are associated with systemic disease or reactions to chemotherapy. • In young adults, acute epiglottitis has been described as being caused by inhalation of heated objects when smoking illicit drugs. Pediatric Patient Review of epiglottitis admissions: 8-year retrospective (19982006): - Epiglottitis continues to be a significant entity, - Two uniquely vulnerable populations: Infants (<1 year old) and the elderly (>85 years old). -Examining the pediatric cohort of patients (patients <18 years of age), 34.4% were <1 year of age. - This category of age <1 year seemed to have increased in frequency: 26.8% of pediatric patients in 1998 to 41.1% in 2006.  - A case of epiglottitis with negative cultures has been reported in a neonate within hours of birth.  Differential Diagnosis Croup • • • • • • • Viral laryngotracheobronchitis, swelling of the mucosa in the subglottic area of the larynx, more prevalent during the wintertime. Croup has a more gradual onset than acute epiglottitis, commonly associated with lowgrade fever. Same symptoms of inspiratory stridor, suprasternal, intercostal and substernal retractions and hoarseness, differentiation in early illness is possible by additional observation of barking cough and absence of drooling and dysphagia in croup. Epiiglottitis • no seasonal predilection to • and by the additional observation of drooling and dysphagia with absence of coughing in epiglottitis. • Additional reliable signs of epiglottitis are a preference to sit, dysphagia and refusal to swallow. The radiological "thumb sign" in acute epiglottitis Alphabet P sign" formed by acoustic shadow of hyoid bone " (HY), swollen epiglottis (pointed by white arrows). Hung TY et al. Am J Emerg Med. 2011; 29:359. Inflammatory edema of the arytenoids, aryepiglottic folds and the epiglottis. Tracheal intubation of a patient with epiglottitis must be regarded as a potentially difficult procedure. • Difficulty in breathing and stridor are common signs of epiglottitis in children, but are less frequent in adults. • The most common presenting symptom in adults is odynophagia (100%), followed by dysphagia (85%) and voice change (75%). • In adults, stridor is regarded as a warning sign for occlusion of the upper airway. • Stridor, tachycardia, tachypnea, rapid onset of symptoms and a "thumb-sign" present in 79% of the cases on lateral X-rays of the neck are significant predictors for imminent airway compromise with rapid clinical deterioration.