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Morning Report

MARCH 4, 2012 ELIZABETH PARSONS, MD

Case Presentation
 9 year old boy presents to the emergency room with

a 2 day history of fever, cough, congestion, and difficulty breathing. He has had mild abdominal pain and one episode of vomiting. He also complains of a mild headache. On further questioning, his mother states to you that he has a chronic cough that “won’t go away” and that “he always gets pneumonia” She feels that he is never well.

Past Medical History
 Born at 37 weeks gestation without complication.

 He has been treated for pneumonia 2-3 times per

year by his primary care physician and has had 7 admission in the past for respiratory infections.  History of asthma

History Continued
 FH: Notable for a brother with Trisomy 21. His

paternal grandfather has hypertension. Otherwise, no chronic illnesses  SH: Lives with his parents and 2 siblings No smoke exposure. No sick contacts.  Immunizations: Up to date. He has not received a flu vaccine  Medications: Albuterol as needed, Advair, Singulair

Physical Exam
 WT: 25 kg (25%) HT: 134 cm ( 44%)  T: 39.5 HR 141 BP 116/78 R 37 sat 98% on 2 lpm NC

GEN: WDWN young boy in mild respiratory distress. HEENT: NC/AT. TM clear. OP mildly erythematous. No LAD CV: Tachycardic. Normal S1, S2. No murmur. CRT < 3 secs PULM: Tachypneic. Mild suprasternal retractions. Markedly diminished BS on left compared to right with crackles at bases. No wheezes. ABD: soft, non-distended and non tender. + BS. No HSM EXT: Warm and well perfused. No rash. No clubbing, cyanosis or edema.

Diagnostic Studies
 CBC W: 26 (56% bands, 30% poly, 7% Lymps)

 CMP: normal
 CRP: 12  ESR: 28

 Blood culture pending
 VRP negative

Differential Diagnosis?
ID/Immunology Pneumonia (viral vs bacterial) Tuberculosis Immunodeficiency Fungal infection (cocci, histo, etc) HIV/ immunocompromise Pulmonology CCAM Pulmonary sequestration Broncogenic cyst Congenital lobar emphysema Bronchiectasis Cystic Fibrosis Ciliary dyskinesia Asthma Intersitial lung disease CV Heart failure Undiagnosed congenital heart disease Acquired heart disease Pulmonary hypertension Pulmonary Embolism Rheumatology Sarcoidosis SLE Wegener’s granulomatosis Heme/Onc Lymphoma Pulmonary metastases Airway Abnormalities Chronic aspiration Laryngo/tracheomalacia Vascular ring GI - GERD - Chronic aspiration

Pulmonary Sequestration

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Cystic/Solid mass composed of embryonic tissue that DOES NOT communicate with the tracheobronchial tree AND has anomalous SYSTEMIC blood supply. 15-25% have multiple systemic feeding vessels Thought to be due to an accessory lung bud that develops from the ventral primitive foregut. Does NOT participate in gas exchange or result in a L→ R shunt.

Pulmonary Sequestration
INTRAPULMONARY

EXTRAPULMONARY

75% of cases
Males = Females Does not have its OWN pleura (lies within pleura of lung lobe) Systemic blood supply, but PULM VENOUS DRAINAGE!!! Posterior basal segment of lung (L>R) Often delayed diagnosis with presentation of recurrent infections or hemoptysis.

25% of cases
Males: Females = 4:1 Has OWN visceral pleura

Thoracic/Subdiaphragmatic All left sided.
Systemic blood supply AND SYSTEMIC venous drainage Presents earlier as abdominal mass, heart failure, respiratory distress. Associated with CDH, colonic duplication, vertebral anomalies, etc

Pulmonary Sequestration

Berrocal, Teresa et al. Congenital Anomalies of the Tracheobroncial Tree, Lung, and Mediastinum; Embryology, Radiology, and Pathology. Jan 2004. Radiographics. 24. e17.

Pulmonary Sequestration Diagnosis

CXR with recurrent pneumonia that has slow or incomplete resolution. Prental doppler US showing systemic arterial supply to fetal lung lesion

CT/ MR angiography

Intrapulmonary Sequestration

Extrapulmonary Sequestration

Extrapulmonary Sequestration

Pulmonary Sequestration Treatment

Balloon occlusion of systemic arterial supply

Surgical resection is curative
Intrapulmonary- often poorly defined margins so often needs lobectomy Prognosis is good.