Pediatric Zebras

1. Pediatric Zebras Rene Y. McNall-Knapp, MD, FAAP Pedatric Hematology/Oncology, OUHSC

5. Keep worst possible diagnosis (UGLY ZEBRA) in mind • Family, patient, caregivers partner in care • Expected course of disease • May take more than one visit • Bad things don’t get better on their own • Stay humble Telling the Difference

6. Day 1 • 4 y.o. boy presents with back and leg pain for 2-3 days • History – no fever, hurting mostly at night, usually very active • Physical – unremarkable, scattered bruises of various ages • Labs/Imaging - ? Case study #1

7. Visit 2 • History – pain worsening, not walking, lethargy, bruising, fever • PE – bruising, pallor, lymphadenopathy • Labs/Imaging • WBC 4.4K (80% lymphs) • Hemoglobin 5.2 g/dL • Platelets 10K Case Study #1

8. Leukemia – in this case ALL • ALL/AML – most common cancer in childhood • Presenting symptoms, findings • Anemia – pallor, fatigue • Thrombocytopenia – bruising, bleeding • Leukocytopenia – infections, thrush, fever • Adenopathy – mediastinal, peripheral • Splenomegaly • Bone pain • Leukemia cutis Diagnosis

9. Started on induction therapy for standard risk ALL • On day 29 was in remission • Continues on chemotherapy Treatment and Results

10. Cancer is #1 disease killer in children • Cancer is diagnosed in 1:300 boys and 1:330 girls before 21 • Stage at diagnosis has dramatic effect on prognosis (morbidity and mortality) • 75% cure for all children walking in door Importance of identifying zebras

11. Imagine if you didn’t catch it on visit 2 • Patient worsened and worsened over next 2 weeks • Presents to local ER • Codes due to severe anemia and infection • Revived but multisystem organ dysfunction and disseminated Aspergillus • Must treat leukemia in face of all of this Challenges of delay in diagnosis

12. 1st visit • Almost 2 y.o. girl with complaint of vomiting for last month • No rhyme or reason to the vomiting • PE – small, fluid behind TM, otherwise unremarkable • Labs/imaging • Assessment and plan – otitis, amoxicillin, RTC 2 weeks for ear recheck Case #2

13. 2nd Visit • History – continues to have daily vomiting, also acting as if hurting inactive, losing milestones • PE – weight loss, sleepy • Labs/Imaging – • Assessment/Plans - Case #2

14. Presents to ER 2 weeks later because parents worried she might have a parasite • History – vomiting everything, lost 1/3 of her weight • Physical – emaciated, lethargic • Labs/Imaging – normal labs, US abdomen and Xray normal • Admitted to hospital for observation • That night stopped breathing – Code Blue • Revived and imaging done Case #2 – Imagine If

15. Brain Tumor (Specifically Medulloblastoma) • 2nd most common cancer in childhood • Prognosis depends on type and stage of diagnosis, surgical resection • Presenting symptoms – common for posterior fossa • Nausea/vomiting • Headache • Head tilt • Ataxia • Lethargy • Double vision Diagnosis

16. Infants – macrocephaly, sunset eyes, loss of milestones, wasting • Seizures – not febrile seizures • Abnormal eye movements or looking through peripheral vision • Delayed or precocious puberty • Abnormal growth • Other cranial nerve palsies – drooling, aspirating, facial droop Other Symptoms of Brain Tumors

17. First – do no harm • Least invasive/radioactive test you can do to reassure yourself • Let the kid keep some of his own blood • Second – build rapport with family for close follow-up • Third – act deliberately using history and physical as your guide • Hint – there are algorithms out there to help! Approaches to zebra symptoms

18. Hypertension – should test at least annually and at every sick visit • Varies by age and height • Renal tumors disease, adrenal tumors and disease, coarctation of the aorta (arm > leg), others • W/U – H&P, UA, CMP, renal US • Horses – essential hypertension • Fever > 5 days • 101 F • Leukemia, Kawasaki, unusual infections • W/U – H&P, CBC, appropriate cultures • Horses – I don’t know what but Zithromax will make it better Red light zebra symptoms

19. Eye – abnormal eye movements, proptosis, white reflex • Brain tumor, retinoblastoma, metastatic disease, rhabdomyosarcoma, orbital cellulitis • W/U – urgent ophthalmology referral, MRI brain/orbit • Horses – normal for baby, conjunctivitis • Adenopathy • >1 cm all except inguinal (>1.5 cm) and supraclavicular (any) • Leukemia, lymphoma, EBV, other infections, abdominal malignancy (Virchow’s node) • W/U – CBC, CMP, uric acid, LDH, CXR, response to antibiotics (if appropriate case), biopsy • Horses – common infections • Warning – do not give steroids Red light zebra symptoms

20. Wheezing, shortness of breath • Foreign body, mediastinal mass, vascular ring • W/U – H&P, CXR • Warning – again steroids! • Abdominal mass • Renal disease, tumors, benign lesions, severe constipation • W/U – H&P, Xray or abdominal US Red light zebra symptoms

21. Bone or joint pain • Infection, leukemia, sarcomas, metastatic lesions • W/U – H&P, CBC, Xray entire bone, consider bone scan • Horses – growing pains, injury • Soft tissue mass • Sarcoma • W/U – image (MRI) then excisional biopsy • Horses – lipoma, ganglion cyst Red light zebra symptoms

22. Poor growth – growth charts essential • Brain tumor, Turner’s syndrome, other syndromes • W/U – H&P, ?endocrinology referral, ?imaging • Horses – normal for patient • Delayed or precocious puberty • Brain tumor, Turner’s syndrome, other syndromes • W/U – H&P, ?endocrinology referral, ?imaging • Horses – normal for patient • Testicular mass • Testicular cancer • W/U – H&P, testicular ultrasound Signs to collect yourself