Senior Case Presentation

Armi Azad, M.D.

Introduction

• S.R. is a 4 y/o HF transferred from MVCH for higher level of care

• Chief Complaint:

Intermittent fever x 1 month (ranging from 100.4 to 103º) and R hip pain for the past couple of weeks; no hx. of trauma

Review of Systems

• On further questioning:

+occasional abdominal pain

generalized weakness and fatigue

?decreased appetite

• ROS otherwise negative

PMHx

Strep Throat x 1 month ago, tx’d w/ PO Abx. x10 dys.

PSHx

No Surgical History

Birth Hx

Full Term

C/S 2/2 failure to progress

Birth Wt= 8 lbs.

Family History

Mother Denies

Social History

Lives with both parents

2 older siblings

No sick contacts

No pets

No tobacco exposure

Pre-school waiting List

History Cont’d.

• Development- Appropriate

• Diet- Regular

• Meds- Motrin

• Allergies- Amoxicillin rash

• Immunizations- Up to date

Physical Exam

Vital Signs:

T=101.5

P=115

R=24

BP=84/59

Wt= 17 kg

Height= 103 cm

Physical Exam

• Gen- pale, scared/anxious

• HEENT- normocephalic, PERRLA

neck supple

no lymphadenopathy

throat clear

TM’s clear B/L

Physical Exam

• CV- RRR, 2/6 systolic murmur

• Resp- CTA B/L

• Abd- Soft, ND, +BS

+tenderness to deep palpation RLQ

no CVA tenderness

Physical Exam

• MS- Decreased abduction R hip 2/2 pain,

+TTP R lower back (no bony TTP),

5/5 motor strength x 4 extremities

+sensation intact B/L

DTR’s intact B/L

• Neuro- AAOx3, CN’s II-XII grossly intact,

gait WNL

What tests do you want to order?

Diagnostic Testing

• CBC w/ peripheral smear

• CMP

• UA, UCx/Blood Cx.

• Pelvis and R hip x-rays

• ESR, CRP, ASO titer

Laboratory Results

7.8

6.8 423

23.1

MCV= 76.3

ESR= >140

CRP= 13.7

ASO= 15 (<150)

• Segs= 19 (37-80%)• Lymphs= 57 (25-35%)• Mono= 5 (0-12%)• Eos= 1 (0-7%)• Bands= 17 (0-6%)

Laboratory Results

137 103 12

86

5.0 28 0.4

Albumin= 3.3 Ca= 9.6

Total Protein= 7.0 BiliT= 0.2

AST= 54 AlkPhos= 150

ALT= 14 Retic= 2.4%

Urine Analysis

• Color yellow• Glucose negative• Bili negative• Blood negative• Nitrite moderate• LE 2+• WBC 5-10• RBC 0-2

Laboratory Results

• Urine Cx- negative

• Blood Cx’s- negative x2

X-ray

• Pelvis and R hip x-rays: unremarkable

Any Thoughts?

Differential Diagnoses of Hip Pain1

Now What?

Radiology

• CT Pelvis:– Large fecal collection in rectum– Thickening of urinary bladder– No evidence of acute osteo or a pelvic abscess

Update

• Continued fevers despite IV Abx• Migratory bone pain (i.e. L shoulder, mid

back)• Intermittent RLQ abdominal tenderness to

deep palpation 6.7

8.0 372 19.9

3- Phase Bone Scan

• Vascular Flow Study-No abn. Increased vascular flow in lower abdomen,

pelvis, and thighs

• Blood Pool Phase-

Slight hyperemia in the right medial ilium

3- Phase Bone Scan

• 3-hr Delayed Static Images-

Abn. accumulation in R mastoid and clivus

Abn. accumulation in body of C2/C3

Mild generalized accumulation in bodies of L3/L4 and multiple levels of the thoracic vertebrae

Non-functional R kidney

Bone Scan Images

Impression

Metastases

vs.

Extensive Diffuse Osteomyelitis

More Radiology

• CT Chest w/ contrast-

Multiple osteolytic and osteoblastic lesions in the thoracic spine

• CT Abd/Pelvis w/ contrast-

3.6 cm L adrenal mass

No ascites or adenopathy

Osteolytic lesion in R posteromedial ilium w/ sclerotic rim

Neuroblastoma

• What is it?– 4th most common malignancy of childhood– Cancerous tumor that begins in nerve tissue of

infants and very young children– Usually begins in tissues of adrenal gland

(sometimes in neck, chest, and/or pelvis)

Neuroblastoma

• Metastasizes quickly (i.e. LN’s, liver, lungs, bones, CNS, bone marrow)

• 70% of all children diagnosed have some metastatic disease

Frequency3

• Approx. 650 new cases dx’d in U.S./year

• More common in whites

• Male-to-Female ratio 1.3:1

• 79% of children diagnosed by age 4

• 97% of cases diagnosed before age 10

Etiology4

• Chromosome #1

• Deletion/rearrangement on short arm of C’some 1

• Amplification of n-myc oncogene

• Inherited mutation + 2nd mutation after birth

vs.

2 acquired mutations after birth

Symptoms

• Abdominal mass/distention• Uncontrolled eye movement if facial

involvement• Changes in urination, diarrhea• Pain, limping, paralysis, weakness if bone

marrow involvement• Fever• High BP and increased HR

Staging5

Treatment

• Surgery

• Chemotherapy

• Radiation

• Bone marrow transplant

• Antibiotics

• Supportive care

• Continuous follow-up

Prognosis6

• Depends on a variety of factors including age of pt. and stage of disease

• Survival significantly better for children less than 1 year of age

• N-myc amplification confers worse prognosis

• Increased ploidy has better prognosis

Survival6

• Patients risk stratified into 3 groups

• 5-year survival by risk group:

1) Low Risk- 95%

2) Intermediate Risk- 85-90%

3) High Risk- 30-50%

Back to S.R.

• Chemotherapy: completing 4 of 6 cycles today

• Surgery to remove tumor

• Bone marrow transplant

References

1. An Evidence-Based Approach to the Evaluation and Management of Hip Pain in Children. Pediatric Case Reviews, Volume 2:1; January 2002

2. http://www.emedicine.com/med/topic2836.htm

3. http://www.okstate.edu/artsci/zoology/ravdb/3024.htm

4. Staging of Neuroblastoma at Imaging. Report of the Radiology Oncology Diagnostic Group 2002; Volume 223:1 (p. 168-175)

5. http://www.cancer.org/docroot/cri/content/cri_2_4_3x_how_is_neuroblastoma_staged_31.asp