Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer,...

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Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September 22, 2010

Transcript of Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer,...

Page 1: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Western States Pediatric Pulmonary Case Conference

Cough, Hypoxia, and Down Syndrome

Emily DeBoer, MD

The Children’s Hospital

University of Colorado

September 22, 2010

Page 2: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Outline

Case presentation: Cough, Hypoxia, and Down Syndrome

Differential Diagnosis Multi-system complications of patients with

Trisomy 21 Treatment and Monitoring

Page 3: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Chief Complaint

9 month old female with Down Syndrome referred to pulmonary clinic for initial evaluation because of cough and persistent oxygen requirement

Page 4: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

History of Present Illness

Daily cough for months - day and night No increased work of breathing Snoring Gags with jejunal feeds

Treated with RanitidineSevere oral aversion, tastes by mouth

Page 5: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Past medical/surgical history Trisomy 21 Born at 36 weeks in Colorado Esophageal atresia without TEF

GT placment DOL 1Gastric pull-through at 4 months

On and off oxygen Ventilated x 1 week after surgery Discharged at 5 months on ¼ lpm O2 via nasal

cannula

Page 6: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

PMH/PSH

PDA ligation at 5 months of age Recent echo revealed – “normal function,

small left to right ASD, mild TR” Monthly esophageal dilations – tolerated

well

Page 7: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Meds at visit

Ranitidine Spironolactone/hydrochlorothiazide ¼ lpm oxygen

No inhaled medicines No steroids

Page 8: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Review of Systems

No fevers Adequate growth No hemoptysis No vomiting No steatorrhea Normal thyroid No hematuria Sitting with support

Page 9: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Family and Social History

No asthma, allergies, or lung disease in the family

Lives with mom, “adopted grandparents” in Denver Parents are from Senegal No known TB exposures No pets No smokers

Page 10: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Physical Exam Vitals HR 136 | RR 28 | Ht 65 cm (43%) | Wt 7.5 kg

(39%) SaO2 88% RA | SaO2 95% ¼ lpm

General: happy baby, + drooling HEENT: Down’s facies, small nares, +rhinorrhea Chest: Easy work of breathing, clear to auscultation,

prolonged expiratory phase CVS: RRR, normal S1 and S2, no murmur Abd: Soft, non-tender, no hepatosplenomegaly Ext: No clubbing Neuro: Decreased truncal tone

Page 11: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Chest Xray at 8 months of age

Page 12: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

What should we do?

Page 13: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

What should we do?

Differential Diagnosis – cough, hypoxia Reflux / aspiration Pulmonary edema Airway anomaly / poor airway clearance Asthma Interstitial lung disease Pneumonia/infection

Page 14: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

What should we do?

Differential Diagnosis – cough, hypoxia Reflux / aspiration Pulmonary edema Airway anomaly / poor airway clearance Asthma Interstitial lung disease Pneumonia/infection

Because of her Down Syndrome, she is at risk for aspiration, heart disease, tracheal anomalies, obstructive sleep apnea, pulmonary hypertension

Page 15: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.
Page 16: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Bronchoscopy Erythematous mucosa Copious clear secretions Mild-mod malacia of trachea and both mainstem bronchi

LIPID INDEX = 0. IRON INDEX = 270.

Bacterial and viral cultures: negativeCOLOR COLORLESSCHARACTER HAZYNUCLEATED CELLS 910RBC 1705RBC MORPH NORMALSEGS 55LYMPHS 18MONOCYTES 7MACROPHAGES 17

Page 17: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.
Page 18: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

What should we do now?

Cause of increased iron index?

Treatment?

Page 19: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.
Page 20: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Disorders with pulmonary capillaritis Disorders without pulmonary capillaritis

NoncardiovascularCardiovascular

Chronic heart failure Pulmary Hypertension Pulmonary veno-occlusive disease

Page 21: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Follow-up via phone

New symptomsRhinorrhea Increased cough, respiratory rateSleeping moreRequiring ½ - 1 lpm O2

Intervention5 days of oral steroids

Page 22: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Follow-up

Outpatient echocardiogram scheduled Improved for 2 weeks, then symptoms

returned Treated with 5 more days oral steroids by her

PCP Symptoms do not resolve – present to ED Further history – taking liquids by mouth for 6

weeks as instructed by therapy

Page 23: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Physical Exam in ED

Vitals HR 180 | RR 62 | SaO2 94% 3 lpm General: Infant in moderate respiratory distress HEENT: Down’s facies, +rhinorrhea Chest: Subcostal retractions, tachypneic,

coarse symmetric breath sounds CVS: Tachycardic, prominent S2, 2/6 systolic

flow murmur at LLSB Abd: Soft, non-tender, Liver down 3 cm

Page 24: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

8 months of age 12 months of age in ED

Page 25: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

What should we do?

Page 26: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

What should we do?

Differential Diagnosis – cough, hypoxemia, prominent S2, hepatomegaly

Pulmonary hypertension Aspiration Heart failure Infection

Page 27: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Laboratory

CBC 11.6 WBC 81% Segs, 8% Lymphs, 10% Monos Hb 16.3 g/dL / Hct 51.4 % Platelets 221

CBG pH 7.43CO2 34 mm Hg

Page 28: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Echocardiogram Small secundum ASD with bidirectional flow. Moderate right heart enlargement and moderate

septal flattening. Normal left ventricular size and systolic function. Low

normal RV systolic function. Systemic pulmonary hypertension (on 3 lpm NC O2).

TV jet 4.48 m/secondRV-RA grad 80 mm Hg (SBP 90/69)

Page 29: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Pulmonary Hypertension Causes?

Page 30: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Pulmonary Hypertension Causes of secondary PAH

Cardiac/Vascular anomaly Chronic lung disease

Trisomy 21 Aspiration / reflux Overcirculation from PDA (repaired at 5 months)

Obstructive sleep apnea Thromboembolic disease Collagen vascular disease Thyroid disease HIV

Page 31: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Pulmonary Hypertension Causes of secondary PAH

Cardiac/Vascular anomaly Chronic lung disease

Trisomy 21 Aspiration / reflux Overcirculation from PDA (repaired at 5 months)

Obstructive sleep apnea Thromboembolic disease Collagen vascular disease Thyroid disease HIV

Page 32: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Cardiac catheterization30% FiO2Qp/Qs = 1.25:1; Rp/Rs = 0.46

21% FiO2Qp/Qs = 1.25:1; Rp/Rs = 0.64

100% FiO2 with 40 ppm iNOQp/Qs = 1.14:1; Rp/Rs = 0.47

Page 33: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Pulmonary Hypertension Causes of secondary PAH

Cardiac/Vascular anomaly Chronic lung disease

Trisomy 21 Aspiration / reflux Overcirculation from PDA (repaired at 5 months)

Obstructive sleep apnea Thromboembolic disease Collagen vascular disease Thyroid disease HIV

Page 34: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Chest CT

Page 35: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Chest CT - prone

Page 36: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Esophagram

•Thread-like appearance of distal esophagus•Fundus of stomach superior to the diaphragm•No normal peristalsis – movement of feeds only with gravity

Swallow study•Deep laryngeal penetration with thin liquids. •No aspiration with pureeds.

Page 37: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Pulmonary Hypertension Causes of secondary PAH

Cardiac/Vascular anomaly Chronic lung disease

Trisomy 21 Aspiration / reflux Overcirculation from PDA (repaired at 5 months)

Obstructive sleep apnea Thromboembolic disease Collagen vascular disease Thyroid disease HIV

Page 38: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Polysomnogram

Mild sleep-disordered

breathing Apnea-hypopnea

index of 3 events/hour SpO2 in low 80s in RA SpO2 in mid 90s on

¼ lpm NC (≥92% for 99% of TST)

Obstructive Sleep Apnea does not explain PAH

Page 39: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Pulmonary Hypertension Causes of secondary PAH

Cardiac/Vascular anomaly Chronic lung disease

Trisomy 21 Aspiration / reflux Overcirculation from PDA (repaired at 5 months)

Obstructive sleep apnea Thromboembolic disease Collagen vascular disease Thyroid disease HIV

Page 40: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Further laboratories Protein C and S Prothrombin Antithrombin III Lupus Anticoagulant Factor V Leiden Homocysteine Beta 2 GP1 (antiphospholipid) antibodies Cardiolipin IgG and IgM

Page 41: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Pulmonary Hypertension Causes of secondary PAH

Cardiac/Vascular anomaly Chronic lung disease

Trisomy 21 Aspiration / reflux Overcirculation from PDA (repaired at 5 months)

Obstructive sleep apnea Thromboembolic disease Collagen vascular disease Thyroid disease HIV

Page 42: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Further laboratories ANA, ESR, CRP TSH and free T4 HIV

Page 43: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Treatment of PAH – Vasodilation and Diuretics Continuous oxygen Oral Sildenafil – started at 0.5 mg/kg/dose

and titrated to 2 mg/kg/dose q6h Furosemide 1 mg/kg/dose TID

Page 44: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Thromboembolic disease can contribute to PAH Elevated Beta 2 GP1 antibodies and low

antithrombin III Discussed aspirin or coumadin therapy

Page 45: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Patient’s Echo Changes with Therapy

Date of Echo

Oxygen use via nasal cannula

RV-RA gradient (calculated from TR jet)

Degree of septal flattening

Right heart enlargement

Week 0 3 lpm 80 mm Hg Moderate Moderate

Week 1 1 lpm 60 mm Hg Moderate Moderate

Week 2 ½ lpm / off 50 mm Hg / 70 mm Hg

Mild / Moderate

Mild

Week 6 ½ lpm Unable to estimate; no TR jet

Normal geometry

None

Page 46: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.
Page 47: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Conclusions

Cause of Pulmonary HypertensionChronic lung disease

Primary Aspiration Trisomy 21 Overcirculation prior to PDA closure

?Thromboembolic disease Cause of increased iron index

Pulmonary Hypertension

Page 48: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Conclusions

Cause of her hypoxiaPulmonary hypertensionChronic lung disease

Cause of her coughChronic lung diseaseAirway protection (aspiration/reflux)Airway Malacia

Page 49: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Discussion

Open lung biopsy?

Repeat bronchoscopy and BAL?

Page 50: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Outline

Case presentation: Cough, Hypoxia, and Down Syndrome

Differential Diagnosis of Cough and Hypoxia Multi-system complications of patients with

Trisomy 21 Evaluation for Elevated Iron Index and

Pulmonary Hypertension Treatment and Monitoring of PAH

Page 51: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

Thank you!

Page 52: Western States Pediatric Pulmonary Case Conference Cough, Hypoxia, and Down Syndrome Emily DeBoer, MD The Children’s Hospital University of Colorado September.

4 infants with acute pulmonnary hemorrhageHemosiderin stain first seen 50 hours –

5 days from eventClearance in 1-2 weeks


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