Case Management

1. Case Management Richard Lirio, M.D. PGY-3 & Rachel Gast, M.D PGY-3 26th January 2010

2. November Cases • HV 15 yo with R distal femoral metastatic, progressive, recurrent Osteosarcoma with cranial mets causing uncal herniation with RF died upon extubation after a DNR was initiated • GS 17 yo with cranipharyngioma with distal R index finger amputation transferred to Union Memorial Hand Trauma Center • SD 5 mo ex-27 week infant with RF ,vent dependant after viral illness with severe subglotttic stenosis, transferred to UM for tracheostomy after failure of steroids

3. History • P.S. 16 y/o healthy Hispanic male • Sustained head trauma from fall from the back of a car while riding/jumping a skateboard w/o a helmet • Report of + LOC and seizure like activity • Emesis x 2 • Arrived at outside hospital as trauma • AF, HR 84, RR 24, BP 157/85 • GCS 15 on arrival – C/O HA; noted to have amnesia to event

4. Physical Examination • Neuro: GCS 15; Awake & oriented to person and time, but not to place. Normal sensation; Motor strength 5/5 throughout; CN intact • HEENT: Left TM perforated with blood; Oropharynx clear; EOMI; PERRLA; Trachea midline; C-collar in place • Chest: CTAB; good Air Entry throughout; • CV: S1S2, RRR, no murmur • Abdo: Normal • MS: No deformities or swelling of extremities • Skin: No apparent abrasions or lacerations

5. Imaging • Head CT: • Diffuse cerebral edema • Multiple hemorrhagic contusions of frontal lobes • Small subarachnoid hemorrhage in b/l cerebral hemispheres • Small epidural hematoma over L occipital bone • Pneumocephalus in occipital area • Fracture L petrous temporal bone • C-spine CT: No fracture or dislocation or vessel injury • CXR : No pneumothorax or parenchymal injury • Pelvic XR : No fracture • Chest, Abdo, Pelvic CT – No evidence of trauma

6. Outside Hospital Course

7. Outside Hospital Course • Throughout stay • Afebrile • Vital signs stable, on RA • HD #2 • Noted to act more agitated – thought to be appropriate for his injuries • Tolerating mechanical soft diet • Cleared to go to rehabilitation facility

8. On Transfer BMP CBC 14.1 138 103 7 152 13.6 3.8 25 .76 9.6 41.8 PE: Obese adolescent, sleeping, difficult to arouse. Neuro: Withdraws from pain; does not follow commands HEENT: Head normocephalic, atraumatic,PERRLA, Oropharynx clear, C-collar Chest: CTAB. RRR. Abdo: Soft, obese, +BS Ext: Full ROM. 3-4/5 strength throughout Skin: Minor L shoulder abrasions noted – otherwise normal ~4h s/p arrival – increasing irritability noted; Oxycodone given <12 hours s/p transfer  Cardiac Arrest  Sinai ED Died

9. Objectives • To discuss Traumatic Brain Injury • To discuss Trauma Scores • To discuss when to image in TBI • To discuss CT surveillance in TBI

10. Traumatic Brain Injury • Head injury is common in children • TBI = Most Common Cause of death & disability in childhood (Krug et al & Luerrson et al) • CDC estimates ~475,000 ER visits for TBI’s in 0-14 y/o (2006) • Schneier et al (2006) noted in 2000, ~50,000 children </= 17y/o hospitalized for TBI • Dunning et al (2004) noted 98% of children presenting to the ED with head injuries had a GCS of 15 • However, 2 studies in the 1980s by Mayer et al note that ~75% of children with multiple trauma have TBI & almost 80% of all trauma deaths are associated with TBI • Langlois et al estimates overall mortality among children with TBI is ~4.5% (vs. 10.4% among adults)

11. Glasgow Coma Scale Mild (GCS 13-15) Moderate (GCS 9-12) Severe (GCS <9) Peds Trauma Score Combines parameters of: Weight Airway SBP CNS Skin Skeletal system Revised Trauma Score RR SBP GCS Definitions

13. Types of Brain Injury • Diffuse brain injury • MC type of severe brain injury in children • Usually produced by accel/decel forces • Concussions – mildest form of DBI • Diffuse axonal injury – more severe form • Result of tissue shearing of grey & white matter

14. Types of Brain Injury • Focal injuries • Brain contusions (accel/decel; coup/contrecoup) • Intracranial haemorrhage (from either blunt or penetrating trauma) • Epidural, subdural, or subarachnoid haemorrhages usually occur from blunt trauma • Subdural & subarachnoid haemorrhages usually occur secondary to severe trauma;associated with other intracranial injuries • Chung et al noted CT findings of swelling/edema, subdural, & intracerebral haemorrhage worse outcomes; while subarachnoid & epidural haemorrhages better outcomes

15. Pathophysiology of TBI • 2 phases • Initial – direct injury to brain parenchyma • Secondary – resulting from biochemical, cellular, & metabolic responses  hypoxia, hypotension • Cerebral swelling peaks 24-72 hours after initial injury • Resulting in decreased cerebral perfusion more ischemia, swelling, herniation, death

16. Cyt Timing in days of cytokine production, cerebral edema, scar formation, and delayed cell death after TBI. Walker et al Walker et al.. Journal of Trauma, Injury, Infection, & Crit Care. 67,2:S120-127

17. Evaluation • Hx: prolonged LOC, persistent vomiting, severe HA • PE: VS (hypoxic? hypotensive? abnormal breathing?), C-spine; open wounds; Neurological status • Labs: Hct, Type & screen, Lytes, US • Imaging: CT-head (moderate to severe TBI)

18. Imaging • National Institute of Health & Clinical Excellence (NICE): • GCS <13 at any point since injury • GCS 13 or 14 at 2h s/p injury • >1 vomiting episode Dunning et al. The implications of NICE guidelines on the management of children presenting with head injury. Arch Dis Child 2004; 89:763

19. Issues • No widely recognized protocol currently exists to address the recommended interval or duration of CT surveillance • Increasing public concern about radiation exposure in pediatric patients during CT imaging • In numerous studies, a common conclusion noted that despite CT-documented progression of a traumatic intracranial lesion, the decision to undertake delayed neurosurgical intervention is typically based on changes in the patient’s clinical status rather than neuroimaging findings

20. Durham et al (2006) • Retrospective cohort study • 268 patients at Level 1 Trauma Center <18 y/o who underwent repeated Head CT scanning within 24h of their initial Head CT • In 61 of the 214 pts with abnormal findings on initial CT  progression was noted • Pts with epidural hematoma, subdural hematoma, cerebral edema & intraparenchymal hemorrhage found to be at a significantly increased risk for progression & to require delayed neurosurgical intervention • No significantly increased risk for pts w/ subarachnoid hemorrhage, intraventricular hemorrhage, diffuse axonal injury, or skull fracture (if no clinical deterioration) Durham et al. Utility of serial computed tomography imaging in pediatric patients with head trauma. J Neurosurg (5 Suppl Pediatrics) 105:365-369. 2006

21. Recommendations • In light of pt’s hx • LOC • Emesis x2 • Diffuse cerebral edema • Epidural hematoma • Hemorrhagic contusions • Slight mass effect • ? irritability (pain??) • Longer observation at trauma center probably would have been beneficial to the patient – to at least encompass the 72 hour period of maximal cerebral edema

22. Bibliography Krug et al. The global burden of injuries. Am J Public Health 2000;90:523 Langlois et al. Traumatic brain injury in the US: ED visits, hospitalizations, & deaths. Atlanta (GA): CDC&P, Nat’l Center for Prevention & Control;2006 Schneier et al. Incidence of pediatric traumatic brain injury & associated hospital resource utilization in the US. Pediatrics 2006;118:483 Dunning et al. The implications of NICE guidelines on the management of children presenting with head injury. Arch Dis Child 2004; 89:763 Mayer et al. Causes of morbidity & mortality in severe pediatric trauma. JAMA 1981; 245:719 Mayer et al. The modified injury severity scale in pediatric multiple trauma patients. J Pediatr Surg 1980; 15:719 Langlois et al. The incidence of traumatic brain injury among children in the US:differences by race. J Head Trauma Rehabil 2005;20:229 Walker et al. Modern approaches to Pediatric Brain Injury Therapy. Journal of Trauma, Injury, Infection, & Crit Care. 67,2:S120-127 Martin et al. Pediatric traumatic brain injury: an update of research to understand and improve outcomes. Curr Opin Pediatr. 2008. 20:294-299 Chung et al. Critical score of GCS for pediatric traumatic brain injury. Ped Neurol 2006. 34;379-387 Durham et al. Utility of serial computed tomography imaging in pediatric patients with head trauma. J Neurosurg (5 Suppl Pediatrics) 105:365-369. 2006

23. Chief Complaint • D.V. 8 month old female • Transferred from G.B.M.C. to Sinai Pediatric Ward on 12/17/09 • Bacteremia • Fever • Refusing to bear weight on right leg

24. H.P.I. l • 12/14 • Fever for 4 days • Seen by PMD and placed on Amoxicillin for O.M. • 12/15 • Increasingly febrile and irritable • Emesis • Taken to G.B.M.C.; partial septic work-up • WBC = 22 • CXR = normal • Blood culture done, Ceftriaxone • 12/16 • Continued fever • Blood culture grew out gram negative coccobacillus; mom called and child admitted to G.B.M.C. • Ceftriaxone, Vancomycin x 3 • Repeated blood culture • Spinal tap with 1 WBC, latex antigen negative for H. influenza

25. Past History Birth hx FT, SVD, Breastfed Meds No medications NKDA Imm Noimmunizations G & D Appropriate Family hx Older sister (3 y.o.) with seizure activity after vaccination (fully Hib immunized); older brother (2 y.o.) cried for 6 hours after vaccination Soc hx Lives with parents and siblings, no smokers, no daycare

26. Physical Exam – G.B.M.C. • VS: T 38.5 (R), HR 159, RR 44, O2 Sat 100% on RA • H : 73 cm (95th%) • Wt: 8.9 Kg (75-90th%) • HC: 45 cm (75-90th%) • General: Well-hydrated, irritable but consolable • Normal PE except… • Held right knee flexed; refused to bear weight; no erythema or swelling; “When completely distracted, allowed passive movement of leg.” • Transferred to Sinai for Orthopedic consult

27. Labs – G.B.M.C. 12/16 113 8.8 139 105 5 19.5 490 3.8 21 0.2 26.8 9.6 N = 55; Bands = 12; L = 27; M = 3 MCV = 80 Albumin = 3.5 Protein = 6.3 Alk Phos = 114 AST = 32 ALT = 17 Total Bilirubin = 0.4 ICa = 5.01 CRP = 13.8 CSF = Negative Gram Stain, Glu = 72; Pro = 12; WBC = 1, RBC = 2 Bacterial Antigen test negative Blood Culture (12/15) = H. Influenzae Blood culture (12/16) = No growth

28. Consults • Orthopedics • Possible transient synovitis • Imaging – normal • Motrin/Toradol • I.D. • Leg – muscular soreness 2/2 fighting LP • 72 hours meningitic dose of Ceftriaxone • 7-10 days parenteral antibiotics • Prophylactic Rifampin for all household members • Hib vaccine 1 month post discharge and 2nd dose after 1 year of age • Neurology • No evidence of radiculopathy related to spinal tap

29. Objectives To discuss the Epidemiology of H. influenzae To discuss the different vaccine types To discuss the efficacy of vaccines To discuss Herd immunity To discuss AAP guidelines for vaccine refusal

30. Gram negative coccobacilli Non-motile Facultative anaerobe Requires 2 erythrocyte factors for growth that are released following RBC lysis: Hemin NAD Carried in nasopharynx of humans (only natural host) Colonization occurs byage 5

31. Encapsulated Strains • 6 serotypes a – f based on polysaccharide capsule • Responsible for invasive disease • Bacteremia • Meningitis • Pneumonia • Epiglottitis • Septic arthritis • Cellulitis • Pericarditis • Endocarditis

32. Non-encapsulated or non-typeable • Mucosal disease • Sinusitis • Otitis media • Bronchitis • Pneumonia • Conjunctivitis

33. Hib - Prior to Vaccine • Responsible for 95% of invasive disease – 3 million cases annually worldwide • 400,000 deaths from pneumonia or meningitis • Leading cause of meningitis in US and worldwide • 1 in 200 children developed invasive disease prior to age 5 • 60% had meningitis • 5% mortality rate • Permanent sequelae in 20-30%, ranging from mild hearing loss to mental retardation

34. Vaccine • 1985 • Hib capsular polysaccharide – polyribosyl-ribitol phosphate (PRP) • Licensed for children 18-59 months • Efficacy 41-88% • Ineffective in infants 3-17 months • Did not activate T-cell response • Limited, short antibody response

35. 1987-1989 PRP – protein conjugate PRP – T : Hib and tetanus conjugate HbOC : Hib and diphtheria CRM197 conjugate PRP – OMP : Hib and meningococcal conjugate Licensed for infants as young as 2 months Schedule = 2, 4, 6 and 12-15 months Carrier protein processed internally by Β cells; peptides presented to T cells

37. 1993 • Incidence of Hib invasive disease declined > 95% • 1995 • >90% of infants in US were covered by vaccine

38. Herd Immunity Occurs if transmitters – individuals or cohorts who have high rate of colonization and transmit the organism to susceptible individuals – are immunized so that they no longer acquire the organism themselves and cannot drive transmission in the population Vaccines serve to reduce oropharyngeal carriage in immunized infants and young children as well as their unimmunized siblings Moulton, Lawrence H., et al. Estimation of the indirect effect of haemophilus influenzae type b conjugate vaccine in an american indian population. International Journal of Epidemiology, 2000; 29: 753-756.

39. Prior to vaccine, carriage at 2-5% of healthy pre-school and school aged children • Lower rates among infants and adults • Non-typeable H. influenzae considered part of normal respiratory flora in 60-90% of healthy children

40. 30% Navajo children <2 years received one or more doses of Hib-OMPC → 50% reduction in Hib invasive disease 50% immunized → reduction > 70% General US population – Hib disease declined in infants <12 months prior to conjugate vaccines; presuming immunization at 15-18 months resulted in herd immunity

41. Prior to vaccine: • Alaskan natives with highest annual incidence of invasive Hib; > 400/100,000 • Hib carriers had higher anti-PRP IgG and IgM concentrations than noncarrier controls • Cases continue to occur in children < 5 at 5.6/100,000 exceeding 2003 US rate of 0.2/100,000

42. PediatricsVol. 108 No. 4 October 2001 • Prevalance of carriage in the Amish communities was similar to pre-vaccination carriage surveys in the US • Incidence of Hib also similar to that of pre-vaccine era • Resistance to vaccine • Lack of knowledge • Low priority • Religious/philosophical objections

43. American Journal of Public Health, March 1999, Vol. 89, No. 3 “Despite striking decline in Hib disease incidence in the United States, the disease persists at low levels several years after the initial decline.”

45. AAFP, 9/09 Coverage with the Hib vaccine decreased nearly 2 percent from the 2007 level but, at 90.9 percent, was still above the Healthy People 2010 goal The CDC attributed the decrease to a shortage of the vaccine that began in December 2007 and that led to a temporary recommendation to defer the booster dose

46. Vaccines 1905 – U.S. Supreme Court – Jacobson v. Massachusetts • Endorsed the rights of states to pass and enforce compulsory vaccination laws • The Court decided that the freedom of the individual must sometimes be subordinated to the common welfare

47. When Parents Refuse Vaccines: AAP Guidelines • Parents are free to make choices regarding medical care unless those choices place their child at substantial risk of serious harm • Restrictions may be placed upon individual choices when there is a potential threat to the community as a whole • Continued refusal after adequate discussion should be respected unless the child is put at significant risk of serious harm

49. WBC – 19 → 16.8 → 12.2 Platelets – 484 → 1,012 → 903 • CRP - >100 → 86 → 4.15 • SED - 80 → 55 • All cultures performed at Sinai = negative • Ceftriaxone x 10 days; 3 days at meningitic dose, 7 days at 75 mg/kg • Parents and 2 older siblings received prophylactic Rifampin, 20 mg/kg, x 4 days