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(BQ) Part 2 book has contents: Gastrointestinal diseases, metabolic diseases, musculoskeletal, syndromes, off-site anesthesia, adults with congenital diseases, pain, fontan physiology, eisenmenger syndrome, juvenile idiopathic arthritis, pain management after scoliosis repair,… and other contents.
PART7 GASTROINTESTINAL DISEASES This page intentionally left blank 53 ESOPHAGOGASTRODUODENOSCOPY PhilippJ Houck, MD YOUR PATIENT An 8-year-old presents for esophagogastroduodenoscopy (EGD) for gastroesophageal reflux disease (GERD) PREOPERATIVE CONSIDERATIONS Gastroesophageal reflux disease is one of the most common comorbidities in our field It is more prevalent in patients with neurologic impairment, obesity, repaired esophageal atresia or other congenital esophageal diseases, and cystic fibrosis GER is a normal physiologic process that occurs several times per day in healthy infants, children, and adults GERD is present when the reflux of gastric contents causes troublesome symptoms and/or complications Patients may present with asthma bronchopulmonary dysplasia, or apparent life-threatening events that may be related to pulmonary aspiration ANESTHETIC MANAGEMENT • Rapid-sequence induction, which has its own risks in pediatric anesthesia, may be necessary • If pulmonary aspiration is not a leading concern (ie, symptoms are not severe and are mostly postprandial) and the patient is schoolaged, tracheal intubation can be avoided and a total intravenous anesthesia technique can be used, with supplemental oxygen being supplied through a nasal cannula • In smaller children, a mask induction and endotracheal intubation is the most practical choice This allows insertion and manipulation of the endoscope without impeding the patency of the airway • The most stimulating part of this procedure is the insertion of the endoscope To facilitate the insertion of the endoscope in the esophagus, the head can be flexed while the patient is in left lateral position 183 184 PART7 Gastrointestinal Diseases POSTOPERATIVE CONSIDERATIONS In infants, prolonged insufflation and the use of high insufflation pressures can lead to a distended abdomen that may impede ventilation Intestinal perforation can present with a similar picture DOs and DON'Ts / / ® / Do flex the head during the insertion ofthe scope Do choose a rapid-sequence induction in patients with severe GERD, particularly when the patient regurgitates food even after adequate fasting Do not use high insufflation pressures in infants Do intubate young children CONTROVERSIES Topicalization ofthe larynx may decrease the incidence oflaryngospasm during insertion ofthe endoscope, but it results in an unprotected airway during and after emergence from anesthesia SURGICAL CONCERNS The incidence of intestinal perforation is low {0.1 %) Signs of a perforation are an unusually distended abdomen, abdominal pain, fever, and subcutaneous or mediastinal air Injuries during an EGD in a stable patient without peritonitis can be managed conservatively; colonic perforation during a sigmoidoscopy requires immediate surgical repair FACTOID More than 25% of adults in the United States use antisecretory medications at least times per month 54 CONTROL OF UPPER GASTROINTESTINAL BLEEDING Manon Hache, MD YOUR PATIENT A 10-year-old presented to the emergency room after having emesis of bright red blood times Hematocrit on admission was 15 The patient received 20 mL/kg of packed red blood cells and was booked for an emergency diagnostic esophagogastroduodenoscopy with banding ofvarices Pertinent past medical history reveals that he was diagnosed at age months with cystic fibrosis (CF) He has had banding ofesophageal varices times in the past He also has chronic lung disease requiring chest physical therapy (PT), a cough assist vest inhaled tobramycin, and DNase, fluticasone, and albuterol inhalers He just finished a course of IV antibiotics last week for increased pulmonary secretions and presumed pneumonia PREOPERATIVE CONS I DERATION$ Upper gastrointestinal bleeding can originate from any area proximal to the ligament ofTreitz Most commonly, any bleeding that occurs distally will present as melena The most common differential diagnosis ofupper gastrointestinal bleeding in children includes gastritis, peptic ulcer disease, Mallory-Weiss syndrome, and esophageal varices Given this patient's history, the most likely cause is variceal bleeding Cystic fibrosis is a genetic disorder associated with chronic lung disease and pancreatic insufficiency Pulmonary disease is responsible for more than 90% of the morbidity in these patients Classically, patients are diagnosed by performing a sweat test Chloride in sweat will be markedly increased These patients can present at birth with meconium ileus with rare perforation They may also present with severe pulmonary manifestations; thick secretions or mucus plugs, leading to multiple infections; or severe persistent episodes of obstructive airway disease This may eventually lead to pulmonaryhypertension and cor pulmonale They may also present with malnutrition or diabetes resulting from exocrine or endocrine pancreatic dysfunction They may also present with liver cirrhosis They can develop upper gastrointestinal bleeding from esophageal varices secondary to portal hypertension and also have coagulopathy from liver failure or cirrhosis and malnutrition 185 186 PART7 Gastrointestinal Diseases ANESTHETIC MANAGEMENT • Thoroughly assess the severity ofpulmonary disease in order to better plan intraoperative and postoperative management; oxygen should be available as well as biphasic positive airway pressure if it is used preoperatively • Humidify gases Frequent suctioning or lavage of secretions may be necessary • Assess the degree of pancreatic involvement and manage diabetes if present; frequent blood glucose monitoring is indicated • Sinus involvement may act as a bacterial reservoir and also trigger bronchospasm • Upper gastrointestinal bleeding may be severe, and adequate volume resuscitation as well as preparation fur possible blood transfusion should be made • Manage coagulopathy, if present • Have blood products available POSTOPERATIVE CONSIDERATIONS Early extubation should be the goal with all CF patients, as prolonged intubation may increase morbidity and mortality Early chest PT and mobilization should be encouraged DOs and DON'Ts / / ® / / Do get a good sense ofbaseline pulmonary function in CF patients Do make sure that the patient has been adequately volume resuscitated Do not forget about pancreatic involvement Do a rapid-sequence induction and have two suctions available in case one clots Do plan for early extubation and chest PT ifpossible CONTROVERSIES The risk ofbleeding when banding esophageal varices electively (without recent bleeding) is very low These patients may be managed without endotracheal intubation Control of Upper Gastrointestinal Bleeding CHAPTER 54 187 SURGICAL CONCERNS In CF patients, it may be hard to distinguish massive upper gastrointestinal bleeding from hemoptysis These patients may have a bronchiectasis that causes massive hemoptysis and requires bronchial artery embolization FACTOID The gene causing CF was identified in 1989, but so far gene therapy has not proven successful There are more than 1000 genetic mutations that have been identified 55 LIVER BIOPSY Manon Hache, MD YOUR PATIENT A 2-month-old female infant with persistent jaundice is scheduled for a liver biopsy She was born at 35 weeks' gestation LMoratoryfindings: Total bilirubin 10.3; conjugated 6.0; international normalized ratio 1.25; partial thromboplastin time 32; albumin 20 PREOPERATIVE CONSIDERATIONS Physiologic unconjugated hyperbilirubinemia is very common and occurs in about two-thirds of newborns It usually becomes apparent on day or of life and lasts around 10 days Persistent or severe hyperbilirubinemia warrants further evaluation It can result from increased bilirubin production, deficiency of hepatic uptake, defects of conjugation, and increased enterohepatic circulation Increased values of conjugated bilirubin warrant a cholestasis workup to evaluate for biliary atresia Time is of the essence because the success ofthe Kasai procedure is thought to be better when it is performed early, preferably before months ofage ANESTHETIC MANAGEMENT • Use general anesthesia without an endotracheal tube • Sevoflurane mask induction and maintenance or propofol can be used • Use opioids judiciously; the surgery is not very painful, but patients are expected to remain flat for a prolonged period postoperatively Fentanyl!-2 jlg/kg is a good choice POSTOPERATIVE CONSIDERATIONS Patients must remain in the postanesthesia care unit for hours for monitoring of possible hemodynamic consequences of bleeding at the site of the liver biopsy; they must take nothing by mouth for the first hours 188 Liver Biopsy CHAPTER 55 189 DOs and DON'Ts / / ® / Do perform brief general anesthesia Patients must remain absolutely still Do be prepared for significant bleeding Have an active type and cross Ifpossible, not delay surgery Do keep an IV in place in case bleeding develops later CONTROVERSIES Tylenol administration {10 mg!kg) is most likely fine for patients with hyperbilirubinemia without liver failure SURGICAL CONCERNS Guidance ofthe needle biopsy is by percussion or ultrasound, especially for patients with transplanted livers There is a small risk ofsignificant bleeding, less so with transplanted livers, because of adhesions to surrounding structures FACTOID The implementation of stool color cards in Taiwan in 2004 helped increase the number of patients diagnosed and treated with the Kasai operation before age months 56 LIVER TRANSPLANTATION Philipp J Houck, MD YOUR PATIENT A 2-year-old patient with biliary atresia presents with portal hypertension, esophageal varices, and massive ascites for liver transplantation after a failed Kasai procedure Laboratory findings: International normalized ratio 4.2; total bilirubin 16 PREOPERATIVE CONSIDERATIONS Biliary atresia is the most common indication for pediatric liver transplantation Up to 20% of patients have other congenital malformations, including splenic malformations, situs inversus, or absence of the inferior vena cava After diagnosis in infancy, a Kasai portoenterostomy (a Roux-en-Y loop is anastomosed to exposed ductules at the surface of the portahepatis), which corrects hyperbilirubinemia within months if successful, is the standard of care This has to be performed befure months of age, and the failure rate is high Those patients develop portal fibrosis, cirrhosis, and portal hypertension Patients may have the usual stigmata of liver failure and can be hypervolemic, hyperdynamic, and coagulopathic ANESTHETIC MANAGEMENT • Use rapid-sequence induction for patients with a full stomach or massive ascites; use a cuffed endotracheal tube • Arterial line and central venous access must be obtained above the diaphragm because of interruption of blood flow from the aorta and the inferior vena cava during cross-clamping • Assess arterial blood gases, hemoglobin, and urine output hourly Every hours determine complete blood cell count, coagulation factors, and fibrinogen • Avoid hypothermia • Preanhepatic phase: Prepare patient for caval cross-clamp, including optimizing hemodynamics with volume (central venous pressure 190 334 APPENDIX3 VT, Wide-Compln:Irreplar Rhythm SVT, Tachyarrbythmiaa with Pulae Biphasic J!kg, then Jlkg for additional shocks Synchronized cardioversion 0.5-1 Jlkg then J/kg for additional shocks Read Out Hlk'l's Hypovolemia Hypoxemia Hydrogen ion Thrombosis (acidosis) Hyperkalemia Trauma Hypoglycemia Hypothermia Tension pneumothorax Tamponade Toxin APPENDIX3 335 TRANSFUSION: MASSIVE HEMORRHAGE (REPLACEMENT OF> HALF OF TOTAL BLOOD VOLUME [TBV] PER HOUR OR TBV < 24 H) / / / / Call for help Notify blood bank immediately of massive transfusion need • RBC: FFP: Platelets= 1:1:1 • Use uncross-matched negative blood until cross-matched blood available • Give cryoprecipitate to maintain fibrinogen > 100 Obtain additional vascular access ifneeded Send labs q30min • Type and cross • CBC, platelets, PT/PTT/INR, fibrinogen • ABG, Na, K, Ca, lactate / Warm the room ./ Blood product administration • Use 140-micron filter for all products • Use a blood warmer for RBC and FFP transfusion (not for platelets) • Rapid transfuser pumps may be used when increased flow is needed Monitoring for hypothermia, hypocalcemia, electrolyte, blood gas, and add-base disturbances Consider rFactor VIla for refractory hemorrhage if above measures are corrected Terminate the massive transfusion protocol once bleeding is under control o/ / / MAINTAIN o/ / / o/ HCT>21%orHB>7 Platelet count> 50,000 {> 1OOk brain injury) INR< 1.5 (< 1.3 brain injury) Fibrinogen> 100 336 APPENDIX3 TRANSFUSION REACTIONS (REACTIONS MAY OCCUR WITH ANY TYPE OF PRODUCT IMPORTANT TO DETERMINE TYPE OF REACTION.) FOR ALL REACTIONS Call for help• / / / / / / / Stop transfusion Disconnect donor product and IV tubing Infuse normal saline through clean tubing Examine blood product ID; determine correct pt Send product to blood bank Document per Institutional Policy HEMOLYTIC-HGBEMIA, HGBURIA, DIC,J BP, i HR, BRONCHOSPASM / / / / / / Furosemide 0.1 mglkg Mannitol 0.5 glkg (2 rnLJkg of25% mannitol) Dopamine (2-4 }lglkg/min) Maintain urine output at least 1-2 mL/kglh Prepare for cardiovascular instability Send blood and urine sample to laboratory NONHEMOLYTIC-J BP, BRONCHOSPASM, PULMONARY EDEMA, FEVER, RASH / / / / Stop transfusion Send blood to blood bank Treat fever Observe for signs of hemolysis ANAPHYLACTIC-ERYTHEMA, URTICARIA, ANGIOEDEMA, BRONCHOSPASM, TACHYCARDIA, SHOCK / Stop transfusion ./ / / / / Support airway and circulation as necessary Epinephrine 10 }lg/kgiV Diphenhydramine mglkg IV Hydrocortisone 2-5 mglkg Maintain intravascular volume APPENDIX3 337 TRAUMA (INITIAL MANAGEMENT OF TRAUMA) PRIOR TO PT ARRIVAL TO OR / / / / Assemble team and assign roles Estimate weight and prepare emergency drugs Gather equipment: • Airway supplies • Invasive monitors • Fluid warmer • Rapid infusion device • Code cart with programmed defibrillator Type and cross blood products ON PT ARRIVAL TO OR / / / / / / / / / / Maintain c-spine precautions for transport Secure/confinn airway (aspiration risk, unstable c-spine) Ensure adequate ventilation (maintain PIP 60 mm Hg Maintain cerebral perfusion pressure • (MAP - ICP) > 40 mm Hg and systolic BP > 5th percentile for age (see "Hypotension" card) • Use CVP in place of ICP ifno ICP monitor available Treat elevated ICP with: • Hyperventilation • Propofol or etomidate • Mannitol ( glkg) • Hypertonic saline (3% via central venous catheter; mUkg) Maintain nonnoglycemia • Avoid glucose-containing solutions ifhyperglycemic INDEX Note: Page numbers in italics refer to figures; page numbers followed by t indicate tables A Achilles tendon release and tendon transfer, 224 Achondroplasia, 21 Acidosis, lactic, 204 Acquired methemoglobinemia, 176 Acrocephalosyndactyly syndrome, 260 Acute arterial stroke, 251 Acute chest syndrome, 171, 295 Acute hypertension, pediatric critical ~nts checldist for, 324 Acute lymphoblasticleukemia, 160 Adenoidectomy, 24 concurrent 26 in patient with obstructive sleep apnea, 17-22, 17t Adenoids, Adenotonsillar hypertrophy, 17 Adenotonsillectomy, 19,23 posttonsillectomy bleeding following, 23-24 Adults with congenital diseases cystic fibrosis, 277-280 Down syndrome, 273-276 Eisenmenger syndrome, 284-286 Fontan physiology, 281-283 juvenile idiopathic arthritis, 287-289 Air embolism, pediatric critical events checldist for, 314 Airway aspirated foreign body; 44-45 bilateral myringtotomy and tubes in a patient with an upper respiratory tract infection, 25-26 cleft lip and palate repair, 27-30 cystic hygroma, 41-43 difficult airway management, 48-51,50 epiglottis, 31-33 infant, 1-2,2 laryngeal papillomatosis, 46-47 lower, 2-3, 2t postoperative stridor, 34-36 posttonsillectomy bleeding, 23-24 subglottic stenosis, 37-40, 40t tonsillectomy and adenoidectomy in a patient with obstructive sleep apnea, 17-22, 17t Airway fire, pediatric critical ~nts checklist for, 321 Allergic rhinitis, 17 Allergies, egg and soy,197-198 Alzheimer's disease, 274 Analgesia, patient-controlled, 203, 295 intravenous,298-301,299t Analgesics, 305 Anaphylaxis, pediatric critical events checklist for, 315 Anemia, 158, 247 sickle cell, 295 Anesthesia off-site (See Off-site anesthesia) Anesthesia-induced rhabdomyolysis, 204 Anesthesia induction with intracardiac shunt, Ankylosing spondylitis, 193 Anterior mediastinal mass, 160-162 Antibiotics, 306-307 Antihypertensives, 307 Antiemetics, 307 Antipruritics, 308 Anus, imperforate, 111-113 Aortic coarctation, 251 Apert syndrome, 18 Apnea,4 neonatal, Apnea-Hypopnea Index 201 Amold-Chiari malformations, 128, 131 Arthritis, juvenile idiopathic, 287-289 Arthrogryposis, 224-225 Arthroscopy, shoulder, 215-217,216, 216t, 217 Asphyxia, 339 340 INDEX Aspirated foreign body, 44-45 Aspiration, chronic or recurrent, 213 Aspiration pneumonia, 87-89 Asthma, 77-79, 201 Atelectasis, postoperative, 274 Atlantoaxial instability, 274 Atraumatic subluxation, 215 Atresia biliary, 190 duodenal, 105-106 esophageal, 101, 105 Atrial septal defect (ASD), 55, 251,273 Avascular necrosis, 214 Becker's muscular dystrophy, 204 Beckwith-Wiedemann syndrome, 103,157 Benzodiazepine, 10 Bilateral hemifacial microsomia, 234 Bilateral myringtotomy and tubes in a patient with an upper respiratory tract infection, 25-26 Biliary atresia, 190 Biopsies endomyocardial, 74 liver, 188-189 Blalock-Taussig shunt, 65 Bleeding control of upper gastrointestinal, 185-187 posttonsillectomy, 23-24 Bloodletting, 173 Bluetet, 61 Body composition, 4-5, 5t Brachmann-de Lange syndrome, 245 Bradycardia, pediatric critical events checklist for unstable, 316 Brainstem auditory evoked responses (BAERs), 129, 131 Brain tumor, MRI for, 257-259 Broncho~ors,308 Bronchopulmonary dysplasia, 80-83 Brown fat, 6-7 Bucking, 51,85 Butterfly children, 248 c Cardiac abnormalities, 201 Cardiac arrest, 133, 135 pediatric critical events checklist for asystole, PEA, 317 prone CPR, 319 VFNT,318 Cardiac catheterization after heart transplantation, 73-74 Cardiac defects, 251 isolated conotruncal, 233 Cardiacntedications,303-304 Cardiovascular system cardiac catheterization after heart transplantation, 73-74 cardiopulntonary bypass, 55-58 pulntonary hypertension, 70-72 single ventricle physiology, 65-69 tetralogy of Fallot, 61-64 ventricular septum defect repair, 59-60 Cataracts, 251 CATCH-22, 232 Caudal block, 12, 12-13, 13 Central core disease, 204 Cerebellar hypoplasia, 251 Cerebral palsy; 143 Cerebral sinus malformation, 251 Cerebrospinal fluid leaks, 131 CHARGE syndrome, 243-244 CHD7 ntutation, 244 Chiarimalformation, 114,128-131 Choanal atresia, 243, 244 Cholecystectomy, postoperative pain management in sickle cell disease for laparoscopic, 295-297 Cholelithiasis, 295 Cholesteatonta, 26 Chrontosonte 22q11 deletion, 61, 62 Chronic lung disease, 213 Chronic obstructive sleep apnea, 229 Chronic progressive external ophthalmoplegia, 249 Cleft lip, repair of, 27-30 Cleft palate, 25 repair of, 27-30 INDEX Clubbing, 72 Clubfoot, 218-221, 219,220 Coloboma, 251 Congenital cystic adenomatoid malformation, 89 Congenital diaphragmatic hernia, 89, 99-100 Congenital diseases, adults with cystic fibrosis, 277-280 Down syndrome, 273-276 Eisenmenger syndrome, 284-286 Fontan physiology, 281-283 juvenile idiopathic arthritis, 287-289 Congenital heart disease, 89 Congenital hypothyroidism, 251 Congenital lobar emphysema, 89 Congenital myasthenia gravis, 146 Congenital myotonic dystrophy, 136 Conotruncal anomaly face syndrome, 61 Contraction alkalosis, 97 Cornelia de Lange syndrome, 245-246 Cor pulmonale, 21, 81, 185, 234 Cortriatriatum, 251 Cranial vault remodeling 260 Craniofacial syndromes, 21 Craniosynostosis, scan for, 260-262 Cricoid cartilage, 1, Crohn's disease, 193-194 Croup 84-86 86t membranous, 85 postintubation, 1, 85 subglottic, 84 Crouzon syndrome, 18 Cysticfibrosis, 110, 185,277-280 Cystic fibrosis transmembrane regulator (CFTR), 277 Cystic hygroma, 41-43 Cystic lymphangioma See Cystic hygroma D Dandy-Walker syndrome, 251 Defibrillation/cardioversion, 303 Dehydration, assessment of; St Dementia, 274 Denys-Drash syndrome, 157 341 Depression, 201 Developmental delay, 213 Deviated septum, 17 Diabetes mellitus, 207-209 Diabetic ketoacidosis, 207 Diaphragm, Diaphragmatic hernia, congenital, 99-100 Diastrophic dwarfism, 218 Difficult airway: after induction, pediatric critical events checklist for, 320 Difficult airway management, 48-51,50 DiGeorge syndrome, 61, 232-233 Disseminated intravascular coagulation, 95 Down syndrome, 18, 21 25, 41 105, 229-231, 233 adult with, 273-276 Drug dosages, 303-309 Duchenne muscular dystrophy, 132, 134,204 Duodenal atresia, 105-106 Duodenoduodenostomy, 106 Duodenojejunostomy, 106 Dural arteriovenous malformation, 251 D~.diastrophic,218 Dysplasia, bronchopulmonary, 80-83 E Egg allergy, 197-198 Ehlers-Danlos syndrome, 215 Eisenmenger syndrome, 59, 284-286 Endocardial cushion defects, 273 Endomyocardial biopsies, 74 Endoscopic craniectomy; 260 End-stage renal disease (ESRD), 199-200 Enterocolitis, necrotizing, 95-96 Epidermolysis bullosa, 247-248 Epiglottis, 1, 31-33 Esophageal atresia, 101,105 Esophageal diverticulum, 251 Esophageal varices, 185 Esophagogastroduodenoscopy, 183-184 Extrapulmonary sequestration, 89, 92 INDEX 342 F Fatty liver, 20 Feeding problems, 213 Fentanyl Oralet, 10 Fetal alcohol syndrome, 234 Fistula, tracheoesophageal, 101-102 Fluids/electrolytes/diuretics, 306 Fluoro-2-D-glucooe (FDG) scanning, 264 Fontan physiology, 281-283 Fontan procedure, 65-66, 68, 69, 281 Foreign body aspirated, 44-45 wheezing and, 79 Frontotemporal lesions, 253 Functional residual capacity (FRC), 3,3t G Gamma Knife radioourgery; 267-270 Gastritis, 185 Gastroesophageal reflux disease (GERD), 183, 203, 213,279 Gastrointestinal diseases Crohn's disease, 193-194 esophagogastroduodenoscopy, 183-184 liver biopsy, 188-189 liver transplantation, 190-192 upper gastrointestinal bleeding in, 185-187 Gastroschisis, 103-104 Gene expression profiling of peripheral blood samples, 74 Glomerulonephritis, 199 H H2 blockers, 307 Head trauma, pediatric critical events checklist for, 338 Heart transplantation, cardiac catheterization after, 73-74 Hemangioma, 251,252 subglottic, 252, 253 Hematology/oncology anterior mediastinal mass, 160-162 heparin-induced thrombocytopenia, 177-179 massive transfusion, 172-173 methemoglobinemia, 174-176 ooteosarcoma, 163-165 posttransplant lymphoproliferative disorder, 166-168 sickle cell disease, 169-171 W!lm's tumor, 157-159 Hemoglobin SS (HbSS), 169 Hemorrhage, pediatric critical events checklist for massive, 335 Heparin-associated thrombocytopenia and thrombosis (HITT), 177 Heparin-induced thrombocytopenia, 177-179 Hepatic artery thrombosis, 192 Hernia, congenital diaphragmatic, 99-100 Hib vaccine, 33 Hip osteotomy, 213-214 Hippocrates,60,262 Hirschsprung's disease, 229 Hodgkin's lymphoma, 160 Horner's syndrome, 251 Human papillomavirus (HPV), 46,47 Hunter syndrome, 21 Hurler syndrome, 21 Hydration, assessment of, St Hydrocephalus, 114, 121-123, 131,213 Hygroma, cystic, 41-43 Hyperaldo~,157,158 Hypercarbia, 203 Hyperglycemia, 207,208 Hyperkalemia, 132, 199-200 pediatric critical events checklist for, 323 Hypertension, 201 pediatric critical events checklist for acute, 324 pulmonary,21, 60,70-72, 185,229 Hyperthermia, 132 malignant, 204, 223, 225 pediatric critical events checklist for malignant, 330 postoperative, 224 Hyperventilation, 126, 127 Hypoalbuminemia, 193 INDEX Hypocalcemia, 126 Hypoglossal paresis, 131 Hypoglycemia, 5-6, 126 Hypokalemia, 158 Hypomagnesemia, 126 Hyponatremia, 126 Hypoplasia mandibular, 18 maxillary, 18 Hypopnea, 17 Hyposplasia of the corpus callosum, 251 Hypotension, 200 pediatric critical events checklist for, 325 Hypotonia, 273 Hypoxemia, neonatal, 41 Hypoxia, pediatric critical events checklist for, 326 loss ofETCO,, 327 Infant fluid replacement, Infant temperature regulation, 6-7, 7t lnhalational anesthesia, Insulin resistance, 20 lnterscalene block, 215-217,216 Intracardiac shunts, Intracranial hemorrhage, 213 Intrapulmonary sequestration, 89,92 Intravenous patient-controlled analgesia, 298-301, 299t Ivan the Boneless, 223 Juvenile idiopathic arthritis, 287-289 Juvenile myasthenia gravis, 146 Juvenile myotonic dystrophy, 136 Juvenile rheumatoid arthritis, 287 K Kasai procedure, 188 Kearns-Sayre syndrome, 249-250 King-Denborough syndrome, 204 Klinefelter syndrome, 41 343 Klippel-Feil syndrome, 240-242 Kyphoscoliosis, 134 L Lactic acidosis, 204 Ladd'sprocedure,107,108 Laryngeal atresia, 37 Laryngeal papillomas, 46 Laryngeal papillomatosis, 46-47 Laryngomalacia, 243, 273 Laryngoscopy, 1, 28 Laryngotracheal reconstruction complications of, 39 single-stage, 38 stages o:t; 37 Laryngotracheobronchitis,84 viral, 85 Larynx.1 Leukemia, 274 acute lymphoblastic, 160 Liver biopsy o:t; 188-189 fatty,201 transplantation of, 190-192 Local anesthetics, 11, 11 t Local anesthetic toxicity, pediatric critical events checklist for,328 Loss of evoked potentials, pediatric critical events checklist for,329 M Macroglossia, 18, 229, 273 Malignant hyperthermia, 204 223,225 pediatric critical events checklist for,330 Mallory-Weiss syndrome, 185 Malnutrition, 193,213 ~on,107-108 Mandibular distraction, 235, 237 Mandibular hypoplasia, 18 Mandibularlesions,253 Marfan syndrome, 215 Massive transfusion, 172-173 ~constriction,17 ~hypoplasia, 18 Meconium ileus, 109-110 344 INDEX Meconium peritonitis, 109 Membranous croup, 85 Metabolic diseases diabetes mellitus, 207-209 egg and soy allergy, 197-198 hyperkalemia, 199-200 mitochondrial diseases, 204-206 morbid obesity, 201-203 Metabolic syndrome, 201 Methemoglobinemia, 174-176 acquired, 176 rebound, 175 Microcephaly, 251 Micrognathia, 232, 243, 251 Microphthalmos, 251 Midazolam, 10 Mini-multicore disease with RYRI,204 Minimum alveolar concentration (MAC), changes with age, 8,8 Mitochondrial diseases, 204-206 Mitochondrial myopathy, 204, 206 Mitral valve insufficiency, 274 Mitten deformity, 247 Model II heart-lung device, 58 Morbid obesity, 201-203 Morning glory retinal deformity, 251 Moyamoya disease, 150-152,251 Muc:opolysatduuidoses, 21 Muscle relaxants, 304 Muscular dystrophy, 132-135 Becker's, 204 Duchenne, 132, 134, 204 pseudohypertrophic, 132 Musculoskeletal system arthrogryposis, 224-225 clubfoot, 218-221,219,220 hip osteotomy, 213-214 osteogenesis imperfecta, 222-223 shoulder arthroscopy; 215-217,216, 216t,217 Myasthenia gravis, 146-149 congenital 146 juvenile, 146 neonatal, 146 Myelodysplasia, 115, 121 Myelomeningocele, 114-115 Myer-Cotton staging system, 39-40, 40t Myocardial ischemia, pediatric critical events checklist for, 331-332 Myoglobinuria, 133 Myopathy congenital, 206 mitochondrial, 204, 206 Myotonic dystrophy, 136-139,206 congenital, 136 juvenile, 136 Myringtotomy; bilateral, and tubes in a patient with an upper respiratory tract infection, 25-26 N Nasopharyngeal hypoplasia, 261 Nasopharynx, 273 Necrosis, avascular, 214 Necrotizing enterocolitis, 95-96, 213 Neonatal hypoglycemia, 5-6 Neonatal myasthenia gravis, 146 Neonatal renal function, 4-7 Neonates congenital diaphragmatic hernia, 99-100 duodenal atresia, 105-106 gastroschisis and omphalocele, 103-104 imperforate anus, 111-113 malrotation, 107-108 meconium ileus, 109-110 myelomeningocele, 114-115 necrotizing enterocolitis, 95-96 pyloric stenosis, 97-98 sacrococcygealtumor,116-117 tracheoesophageal fistula, 101-102 Nephropathy, 207 Neuro Chiari malformation, 128-131 hydrocephalus, 121-123 moyamoya disease, 150-152 muscular dystrophy, 132-135 myasthenia gravis, 146-149 myotonic dystrophy, 136-139 selective dorsal rhizotomy, 143-145 spinal muscular atrophy, 140-142 INDEX status epilepticus, 124-127 tethered spinal cord, 153-154 Nisaen fundoplkation, 89, 243 Non-Hodgkin's lymphoma, 160 Pancreatic insufficiency, 277, 280 Papillomatosis, laryngeal, 46-47 Parasinusitis, 278 Norepinephrine,? Norwood procedure, 65 Patient-controlled analgesia, 203, Obesity, 201 morbid, 201-203 Obesity hypoventilation syndrome, 201 Obstructive sleep apnea, 17-22, 17t, 24,201,202,274 Oa:iput, Oculocraniosomatic neuromuscular disease, 249 Off-site anesthesia Gama Knife radiosurgery, 267-270 MRI for brain tumor, 257-259 st:an for craniosynostosis, 260-262 SPECT scan, 263-266 Omphalocele, 103-104 Oncology See Hematology/ oncology Open strip craniectomy, 260 Optic nerve atrophy, 251 Oral premedications, 10 OR fire: nonairway Pediatric critical events checklist for, 322 Ortner's syndrome, 72 Osteogenesis imperfecta, 222-223 Osteosarcoma, 163-165 Osteotomy; hip, 213-214 Otitis media with effusion, 25 Overnight polysomnography, 18 p Pain intravenous patient-controlled analgesia, 298-301, 299t management of, after scoliosis repair, 293-294 postoperative, in sickle cell disease for laparoscopic cholecystectomy, 295-297 Palatoplasties, 27, 29 345 Patent ductus arteriosus, 65, 251 295,308 intravenous, 298-301, 299t Pediatric critical events checklist acute hypertension, 324 air embolism, 314 airway fire, 321 anaphylaxis, 315 bradycardia: unstable, 316 cardiac arrest asystole, pea, 317 prone CPR, 319 vf/vt, 318 difficult airway: after induction, 320 head trauma, 338 hyperkalemia, 323 hypotension, 325 hypoxia, 326 loss ofETC02, 327 local anesthetic toxicity, 328 loss of evoked potentials, 329 malignant hyperthennia, 330 myocardial ischemia, 331-332 OR fire: nonairway, 322 tachycardia with pulses, associated with hypotension, 333-334 transfusion: massive hemorrhage, 335 transfusion reactions, 336 trauma, 337 Pediatric respiratory physiology lower airway, 2-3, 2t lung volumes, 3, 3t neonatal apnea, neonatal hypoxemia, Peptic ulcer disease, 185 Peritonitis, meconium, 109 Pfeiffer syndrome, 18 PHACE syndrome, 251-253 Pharyngeal hypoplasia, 237 Pharyngeal muscle hypotonia, 229 Pharyngoplasties, 27 Pierre Robin syndrome, 18, 21, 27, 48, 234-236 Pinktet,61 346 INDEX Pneumonia, 274 aspiration, 87-89 Pneumoperitoneum, 203 Polycythemia, 159 Polydipsia, 158 Polysomnography components of, 22 overnight, 18 Popliteal block, 218-219, 219, 220 Positive end-expiratory pressure, 225 Positron emission tomography (PET) s'anning,264-265 Posterior fossa malformation, 251 Postintubation croup, 1, 85 Postoperative hyperthermia, 224 Postoperative pain management in siclde 'ell disease for laparos~k cllolecystectomy, 295-297 Postoperative stridor, 34-36 Posttonsille,tomy bleeding, 23-24 Posttransplant coronary vas'ulopathies, 74 Posttransplant lymphoproliferative disorder,166-168 Premature birth, as cause of developmental delay, 213 Pseudohypertrophic muscular dystrophy, 132 Pseudomeningocele, 131 Pseudosyndactyly, 247 Pulmonary hypertension, 21, 60, 70-72, 185,229 Pulmonary sequestration, 90-92, 92t Pulmonary stenosis, 251 Pulmonaryvascularresunance,59,61 Pyloric stenosis, 97-98 R REAR (renal, ear, anal, and radial) syndrome, 111 Rebound methemoglobinemia, 175 Re,urrent tonsillitis, 24 Refractory seizures, 263 Regional anesthesia, 10-13, 11 t Renal dysfunruon, 158 Respiratory system aspiration pneumonia, 87-89 asthma, 77-79 broncllopulmonary dysplasia, 80-83 croup, 84-86, 86t development of, 2t pulmonary sequestration, 90-92, 92t Restenosis, 244 Resuscitation medications, 303 Retinopathy, 207 Retinopathy of prematurity, 213 Retrognathia, 232 Reversals, 306 Rhabdomyolysis, 132, 133 anesthesia-induced, 204 Ribcage, Right-to-left shunting, Right ventrkular failure, 73 Right ventricular hypertrophy, 21 Rocuronium, 9-10 s sa,cular aneurysm, 251 Sacrococcygeal tumor, 116-117 Sano modification, 65 Scoliosis idiopathic, 293 pain management after repair, 293-294 Sedati~/iv anesthetics, 304-305 Seizures, 127,213 ~tory,263 temporal I~ 263 Seleruve dorsal rhizotomy, 143-145 Sensorineural hearing loss, 251 Septic meningitis, 131 Sequestration extrapulmonary, 92 intrapulmonary 89, 92 Shoulder arthroscopy, 215-217,216, 216t,217 Shprintzen syndrome, 233 Siclde B thalassemia, 295 Siclde cell anemia, 295 Siclde cell disease, 21, 169-171 postoperati~ pain management in, for laparoscopic cholecystectomy, 295-297 Simian crease, 231 Sizing cllart, 311-312t INDEX Klippel-F~ 240-242 Marfan,215 PHACE, 251-253 Pierre Robin, 234-236 Shprintzen, 233 Stickler, 234 Takao,233 Treacher Collins, 237-239 22q 11.2 deletion, 232, 233 Sleep apnea, 17 obsbnlctive,17-22,17~201,274 Somatosensory evoked potential (SSEP) monitoring 129,131 Soto's syndrome, 157 Soy allergy, 197-198 Spasticity, 143 Spina bifida, 218,251 Spinal cord injury to, 143 tethered, 153-154 Spinal muscular atrophy, 140-142 Spring-assisted cranioplasty, 260 Status epilepticus, 124-127 Steinert's disease, 136 Stenosis pyloric, 97-98 subglottic, 35, 37-40, 40~ 229 Steroids, 307 Stickler syndrome, 234 Stop-bang-tool, 22 Stridor, 34 acquired, 36 congenital, 36 postextubation, 34-35 postoperative, 34-36 Subglottic croup 84 Subglottic hemangioma, 252, 253 Subglottic stenosis, 35, 37-40, 40t,229 congenital, 37 Succinylcholine, Superposition,266 Swaddling, 214 Sweat test, 278 Syncope, 173 Syndromes acrocephalosyndactyly, 260 Brachmann-de lange, 245 CHARGE, 243-244 Cornelia de Lange, 245-246 Dandy-Walker, 251 DiGeorge, 232-233 Down 229-231, 233 Ehlers-Danlos, 215 epidermolysis bullosa, 247-248 fetal alcoho~ 234 Homer's, 251 Kearns-Sayre, 249-250 347 velocardio~.232,233,234 Systemic vascular resistance, 61 T Tachycardia, 132, 133 Tachycardia with pulses, associated with hypotension, pediatric critical events checklist for, 333-334 Takao syndrome, 233 Temporal lobe seizures, 263 Tethered spinal cord 153-154 Tetralogy ofFallot, 232 Tet spells, therapeutic maneuvers for, 62 Thermal conductance, Thermogenesis, 6-7 Third nerve palsy, 251 Thrombocytopenia, 158 heparin-induced.177-179 Thromboelastography, 191 Thymectomy,146,149 Tongue, Tongue-lip adhesion, 234 Tonsillectomy, 24 in patient with obstructive sleep apnea, 17-22, 17t Tonsillitis, recurrent, 24 Tonsils, Tracheoesophageal fistula, 101-102 Transfusion pediatric critical evt:nts checklist for, massive hemorrhage, 335 pediatric critical evt:nts checklist for reactions, 336 Transplantation cardiac catheterization after heart, 73-74 liver, 190-192 348 INDEX Trauma, 172 v pediatric critical events checklist for, 337 pediatric critical events checklist for head, 338 Traumatic brain injury, 143 Treacher Collins syndrome, 18, 21, 48, 51,237-239 'fricuspid atresia, 69 'frisomy 13, 41, 229 Trisomy 18, 41, 157 Trisomy21, 273 Truncus arteriosus, 232 Tumors MRI for brain, 257-259 sacrococcygeal, 116-117 Wilm's, 157-159 Thrbinate hypertrophy, 17 1\uner syndrome, 41 22q 11.2 deletion syndrome, 232, 233 Type diabetes mellitus, 201 Vaccines, Hib, 33 Vallecula sign, 33 Vascular diseasse, 207 Vaso-occlusive crisis, 295 Velocardiofacial syndrome, 61, 232, 233,234 Velopharyngeal insufficiency, 27,232 Ventral defects, 251 Ventricular septal defects, 8, 251,273 Ventriculoperitoneal shunt, 115, 121, 213 Vrrallaryngotracheobronchitis, 85 Vital signs, 5, 5t Vocal cords, von Willebrand's disease, 157 u Upper gastrointestinal bleeding, control of, 185-187 Upper respiratory tract infection, bilateral myringtotomy and tubes in a patient with an, 25-26 w WAGR {Wilms' tumor, aniridia, genitourinary anomalies, and mental retardation), 157 Wheezing, 79 Wilm'stumor, 157-159 X X-linked recessive disease, 132 ... manipulation of the endoscope without impeding the patency of the airway • The most stimulating part of this procedure is the insertion of the endoscope To facilitate the insertion of the endoscope... glucose is >20 0 mgldL 20 7 20 8 PARTS Metabolic Diseases • If the patient does not have a pump, an infusion of regular insulin can be started at 0.05 U /kglh with a maintenance solution of D5% dextrose... the use of propofol (egg/soy in Australia, soy/peanut in the United Kingdom) ANESTHETIC MANAGEMENT • Propofol may be used • Ifyou choose to avoid propofol, you have multiple options: Sevoflurane