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Oncology Emergencies in PICU. Norah Khathlan MD Pediatric Intensivist Director PICU November 2006. Oncology Emergencies in the PICU. MEDIASTINAL MASSES SVC Syndrome HYPERLEUKOCYTOSIS TUMOR LYSIS SYNDROME SEPTIC SHOCK ARDS SPINAL Cord Compression. CNS Events.
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Oncology Emergenciesin PICU Norah Khathlan MD Pediatric Intensivist Director PICU November 2006
Oncology Emergencies in the PICU • MEDIASTINAL MASSES • SVC Syndrome • HYPERLEUKOCYTOSIS • TUMOR LYSIS SYNDROME • SEPTIC SHOCK • ARDS • SPINAL Cord Compression. • CNS Events
Oncology Emergencies in the PICU 1- TUMOR LYSIS SYNDROME • Metabolic abnormalities occurring as a result of tumor cell death: • Spontaneously • Chemotherapy • Starting chemotherapy on rapidly growing-chemo-sensitive tumors release of intracellular contents into circulation.
TUMOR LYSIS SYNDROME • Hyperkalemia. • Hyperphospatemia. • 2ry Hypocalcemia. • Hyperuricemia. • Uremia. • High creatinine. • Oliguria.
TUMOR LYSIS SYNDROME • Incidence: • 70% of hematological malignancies laboratory criteria of TLS. • 3% with clinical TLS. • Associated with hematological malignancies: • ALL • AML • Lymphomas • Solid tumors
TUMOR LYSIS SYNDROME • Maybe precipitated by : • Chemotherapy • steroids • Radiotherapy. • Hormonal agents. • Risk factors: • Tumor type • Dehydration • Preexisting renal insufficiency • Nephrotoxic medications • High LDH in TLS is indicative of likely progression to ARF
TUMOR LYSIS SYNDROME • MANAGEMENT: • Identify at risk patients. • Admit to PICU. • Consult Nephrology service • Establish good venous access prefer. CVC. • Frequent lab monitoring of: - Na+ - Ca++ - K+ - Uric acid - Cl- - Creatinine - PO4++ - Urea - Bicarbonate - LDH
TUMOR LYSIS SYNDROME • MANAGEMENT: cont. • Urine analysis and pH • HYDRATION THERAPY: • 2-3 L/m2/day OR 1 1/2 to 2 x maintenance • Start 24-48 hrs prior to chemotherapy. • Isotonic NS or Hypotonic saline if Urine Na <150 meq/L • Alkalinization of the urine to pH = 6-7 controversial ! • Diuretics controversial ! • Mannitol if suboptimal diuresis • Avoid P.O. or exogenous K+, potassium sparing diuretics, ACE inhibitors and uric acid tubular re-absorption blockers.
TUMOR LYSIS SYNDROME • Specific management: • Hyperkalemia: • Ca gluoconate • Na Bicarbonate • Insulin & Glucose • Salbutamol • K binding resins • DIALYSIS or CRRT “CVVHD” for K>5
TUMOR LYSIS SYNDROME • Hyperphosphatemia & 2ry Hypocalcemia: • Phosphate binders eg. Aluminum antacids. • Avoid unnecessary Ca supplements. • PO4 > 4 is an indication for dialysis. • Consider CRRT.
TUMOR LYSIS SYNDROME • Hyperuricemia: • Urine Alkalinization maximizes Uric acid solubility • Urine pH > 6 and < 7.5 • Avoid urine pH more than 7.5 “may lead to massive phosphate crystalluria and phosphate precipitates”.
Tumor Lysis Syndrome • Allopurinol: • Xanthine oxidase inhibitor: Xanthine Hypoxanthine Xanthine oxidae - -ve allopurinol Uric Acid • Blocks production of new Uric acid • Increased levels of uric acid precursors; Xanthine nephrotoxic • Impairs chemotherapy metabolism
TUMOR LYSIS SYNDROME • Hyperuricemia: cont. • Non recombinant urate oxidase (Uricozyme) urate oxidase Uric Acid -------------------------- Allantoins “highly soluble in urine” • Recombinant Urate Oxidase (Rasburicase) • Effective: Single dose decreases uric acid from 15 to 0.4 mg/dl in 24 hrs • Costs • C.I. in G6PD deficiency ====================
Oncologic Emergencies in PICU 2- Hyperleukocytosis • WBC counts > 100,000/ul in 5-20% children ALL • Clinically significant if > 300,000 in ALL • Marked elevation of blood viscosity: erythrocyte + Leukocyte volumes and deformability of cells. • Normal = 1.5 relative to water • Clinical manifestation if > 4 • Mainly affects CNS & Lungs • Leukocyte aggregation. • Small vessel obstruction. • Decreased perfusion of microcirculation. • Vascular stasis, Leukostasis. • Risk of Intra Cranial Hmg and /or IVH & SAH • Role of Cytokines !!
Hyperleukocytosis • Management: • Lack of controlled trials. • Avoid Packed RBCs transfusion • Avoid diuretics. • Maintain platelets > 20,000 • Correct coagulopathy • Hydration,? Alkalinization and allopurinol: • Used in ALL & WBCs > 100,000 80% reduction in 36hrs no complications. • Exchange transfusion & Leukapheresis; • Needs anticoagulants and vascular access. • Rebound WBC count. • No effect on pulmonary status, CNS outcome or mortality. NO ROLE FOR STEROIDS NOR emergency CRANIAL RADIATION
Oncology Emergencies in the PICU 3- Anterior Mediastinal Mass Airway & circulatory compromise posed by mediastinal masses provide some of the great challenges in the PICU and in OR This is a genuine emergency!!
Anterior: Lymphomas Teratomas Middle: Lymphoma Posterior: neuroblastoma Anterior Mediastinal Mass
Anterior Mediastinal Mass • Signs & Symptoms: • Respiratory symptoms predominate: > 50% narrowing • Air hunger • Dyspnea • Wheezing • Anxiety • Position of comfort. • SVC obstruction symptoms: • Facial swelling • Periorbital edema • Conjunctival suffusion • Headache & Dizziness
Anterior Mediastinal Mass • Evaluation: • Quick & cautious approach is a must!!! • Inappropriate delay, investigation and /or management may be catastrophic !! • CXR: PA & Lat. wide mediastinum • CT Chest: • No sedation May lead to cardio- respiratory arrest • No supine position
Anterior Mediastinal Mass • CBC & blood film • LDH • Β-HCG & α-fetoprotein • BMA & biopsy • Pleural fluid LOCAL Anesthetic • Pericardial fluid Only • Lymph node biopsy
Anterior Mediastinal Mass If still no diagnosis:nearly 27% of cases Empiric therapy • Steroids • Chemotherapy • Radiation OR More Invasive testing
Anterior Mediastinal Mass Accurate diagnosis is preferable but Significant risk of induction of general anesthesia must be considered Predictors of safe G.A.: • Echo to evaluate cardiac motility & venous return • PFT : PEF rates > 5o% predicted • Tracheal cross-sectional area > 50% • Different Protocols for different PICUs depending on the available support.
Anterior Mediastinal Mass In a Study to assess risk of G.A in patients with SVC syndrome,163 children with anterior mediastinal masses were reviewed: • 44 underwent G.A. prior to therapy: • Seven (16%) developed life-threatening airway compromise. • Three needed chemotherapy or radiation prior to extubation. • However all survived. • Ferrari et al; General Anesthesia prior to treatment of anterior mediastinal masses in Pediatric cancer patients, Anesthesiology 1990
Anterior Mediastinal Mass • Intubation should be performed: • Awake with FOB. • Spontaneously breathing. • Sitting position. • Lower extremities venous access. • Standby ECMO or CPB • If above is not feasible: Seriously consider empiric steroids +/- chemo or radiotherapy
Oncology emergencies in the PICU • Coordination of care is essential for optimal care. • Communication and collaboration among the members of the health care team improves quality and efficiency of patient care. • Everyone has an important role in the team, BUT there must be a “Captain of the Ship”.