{Pediatric Zebras

Rene Y. McNall-Knapp, MD, FAAPPedatric Hematology/Oncology, OUHSC

Telling the Difference

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

Case study #1

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

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

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

Treatment and Results

Started on induction therapy for standard risk ALL

On day 29 was in remission

Continues on chemotherapy

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

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

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

Case #2

2nd Visit History – continues

to have daily vomiting, also acting as if hurting inactive, losing milestones

PE – weight loss, sleepy

Labs/Imaging – Assessment/Plans -

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

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

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

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

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

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 steroidsRed light zebra symptoms

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

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

cystRed light zebra symptoms

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 ultrasoundSigns to collect yourself