Sunil Kamath MD Post-Doctoral Fellow Childrens Hospital Los Angeles Pediatric Pulmonary Case Conference.

Sunil Kamath MDPost-Doctoral Fellow

Childrens Hospital Los Angeles

Pediatric Pulmonary Case Conference

HPI

6 month old male with no significant PMH3/17

cough, rhinorrhea, nasal congestion, Fever 101cranky and NBNB emesis x 1

3/18 "moaning" while breathingPMD diagnosed a URI and pt. was sent homedeveloped subcostal retractions and taken to outside ED

where he received breathing treatments, improved, and was discharged home

3/19Irritable and had subcostal retractionsReturned to outside ED

ED Course

Persistent retractions and pale

SpO2 71% placed on O2 “pinked up”

Received continuous aerosol treatments

Transferred to outside hospital PICU for further care with

the presumptive diagnosis of bronchiolitis

FT, NSVD, no complications, home on DOL 2Surgical history: noneNKDAImmunizations: has not received 6 month

vaccinationsDiet: Enfamil 6oz TID, baby foodsFamily History: father with bronchitis as a childSocial History: Lives with mother, father and 2

yo sister, no tobacco exposure, no petsAll other ROS negative

Outside Hospital Physical ExamVS:

Temp: 36.7 CHR: 174 bpmRR: 53 breaths per minuteBP: 98/67 mmHgSpO2: 98% on 1.5 LPM via NC

PE:General: Awake in mild/moderate respiratory distress with

subcostal retractionsResp: Coarse breath sounds bilaterally. + Rhonchi. No

Wheezing.Heart: RRR. Normal S1 and S2

Labs

18.3 \ 10.7 / 334

  / 36 \ 149 107 8 149 Ca:9.8

5.1 21 0.4Respiratory culture – Negative for bacteriaRSV DAA – negativeInfluenza DAA – negativeTotal IgG, IgA, IgM, IgE – normalCXR

Outside Hospital Course3/20 Intubated for worsening respiratory distress HFOV x 1

weekStarted on ABX and steroids

3/25 ETT viral culture: Adenovirus (not typed)3/30 DVT of right leg Rx Lovenox4/11Extubated to HFNC and steroids were weanedDeveloped wheezing, prolonged expiratory phase, increasing

distress IV steroids were re-started and patient improved5/4 Changed to Prednisone 5mg BID and transferred to the floor 5/5 MSSA bacteremia Rx oxacillin5/6 Developed increased tachypnea with nasal flaring and

fatigue during feeding5/6 Chest CT

consolidation of RLL and LUL with associated cylindrical bronchiectasis

5/7 Transferred to CHLAVS

Temp: 37.9 deg CHR: 148 bpmRR: 38 Breaths/MinBP: 144/90 mm HgSpO2: 99% on ½ LPM

PEGeneral Appearance: laying in bed, moderate respiratory

distress, becomes fearful with examChest: symmetric chest rise, subcostal retractionsRespiratory: diffuse crackles, wheezing, forceful expiration

with gruntingCardiovascular: RRR, no m/r/g, 2+ pulses

Labs18.72 \ 11.5 / 557

  / 35.9 \ Segs 44, Bands 0, Lymph 42, Mono 13, Baso 0, Eos 1

139 97 11 123 Ca:9.9

5 32 0.2

CBG: 7.46/50//36

“The lungs are hyperinflated. There is streaky perihilar disease with peribronchial thickening bilaterally.”

What is your assessment and plan?

Hospital CoursePlan: chest CT, bronchoscopy, lung biopsy, and iPFT when

stable5/10 SCINTI: normal5/11 ECHO

Small secundum atrial septal defect vs. patent foramen ovale.No evidence of PHTN

5/13 MBSS: normal5/18 Wheezing. Prolonged expiratory phase. Increasing

respiratory distress. Prednisone Solumederol5/21 Admitted to the PICU for stabilization and repeat CT

scan5/24 RV panel: negativeImmunology workup: unremarkable

Template

progression of bronchiectasis and scattered areas of groundglass opacity

What is your management plan?

Management

Bronchiolitis Obliterans:Azithromycin (5mg/kg QMWF)Methotrexate (10-15mg/m2/dose SQ Qwk)Continued IV steroids

5/25 Developed thick secretions and was difficult to ventilateEmpirically started on Vanc and ZosynTrach cult (Many Haemophilus influenzae, Beta lactamase

negative) Ceftriaxone

PICUadmit

Intubated

Azithro

MTX

Extubated

ABX started

IV steroids

Bronchiolitis Obliterans

Rare form of chronic obstructive lung disease that occurs after an insult to the lower respiratory tract

Etiology:

Bronchiolitis Obliterans in Children. Pediatric Pulmonology 39:193-208 (2005)

Pathophsiology:Inflammation and fibrosis of the terminal and

respiratory bronchioles narrowing and/or complete obliteration of the airway lumen

Bronchiolitis Obliterans in Children. Pediatric Pulmonology 39:193-208 (2005)

Kumar: Robbins and Cotran Pathologic Basis of Disease, Professional Edition , 8th ed.

Diagnosis:CXRPFTBronchoscopy - neutrophilia HRCT: mosaic patternOpen lung biopsy:

Sampling error due to patchy airway involvement 2 categories:

proliferative bronchiolitis (intraluminal polyps) constrictive bronchiolitis (peribronchiolar fibrosis)

TreatmentSupportive careSteroidsImmune modulators

Thank You