Radiology Case Conference

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Radiology Case Conference

• May 27, 2003
• Paresh Timbadia, MD

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History

• 73 yr old WF admitted with not feeling well,
  increased fatigue and weakness on 9/18/02 at WWH.
  
• Pulmonary consult for SOB and abnormal CXR.
• C/C Increased fatigue

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HPI

• Recently admitted to EUH 9/6/02 to 9/11/02 for
  increased somnolence and decreased appetite.
• Treated with lactulose for encephalopathy
  elevated ammonia levels. D/c on home O2
  2 L/min
• H/o ETOH use 2 glasses of wine/day for 20 yrs
• H/o bronchiectasis for 20 yrs Rx by private MD
• No fever, cough or sputum production
• No hemoptysis

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Past Medical History

• h/o bronchiectasis
• Chronic Obstructive Pulmonary Disease
• Mild dementia NOS
• ETOH use with recent Rx for encephalopathy

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• PSHx (1) Rt Foot Fracture gt 5yrs ago
  
• Allergy None
• Medications
• Thiamine 100 mg p.o. q.d.
• Folate 1 mg p.o. q.d.
• Theo-Dur 300 mg p.o. q.12h.
• Albuterol MDI 2 puffs p.o. q.6h. p.r.n.
• Humibid L.A. 600 mg p.o. b.i.d.

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• Personal/Social Hx
• Lives with her husband
• Ex smoker, 10 Pack yr quit gt20 yrs
• h/o active ETOH use 2 glasses of wine/day gt20 yrs
• PPD status Unknown
• Family Hx No f/h/o epistaxis or hemoptysis
• ROS No epistaxis
• Not contributory

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Examination

• Alert, not in any distress
• Vitals Supine Sitting
• Temperature oF 98
• Blood pressure mm Hg 180/80 150/98
• Heart rate /min 112
  107
• Respiratory rate /min 20
• RA SpO2 89
  86

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Examination contd.

• HEENT normal, No jaundice Neck no LNE
• Chest Normal BS. no wheezing/ronchi/rub.
• CVS Normal S1 S2. No murmurs
• Abdomen no hepatosplenomegaly, no masses, no
  ascites
• CNS No acute focal deficits.
• Skin No Jaundice, cyanosis or telengectasia
• Ext No clubbing

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Laboratory (9/18/02)

• CBC
• WBC16.6 Hb15.4 Hct45.1 Plt566
• G87 L4 E9 M0 B0
• Chemistry
• Na133, K3.7, Cl80, CO232, BUN8, Cr0.6,
  Glucose140, Ca11 Alb3.5, Phos3.4
• Liver Function Tests
• AST/ALT/Alk PO418/13/59 T Bili 1.2
• Ammonia 32
• Hepatitis Serology(A,B,C) negative
• Urine
• U/A negative
• Urine Lytes Na 12, K28.2, Cr109 Cllt15

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• ABG
• O2 2 L/min pH7.38, pCO2 64, pO2 83,
• Room air pH 7.44 pCO2 57, pO2 57
• PFT
• FEV1 0.4 L, FVC of 0.69L
• FEV1/FVC58

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Chest X ray
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Differential Diagnosis

• Pulmonary AVM (Macroscopic or microscopic)
• congenital
• Acquired-
• post op e.g Glenn shunt (SVC to Rt PA)
• Trauma
• Neoplastic infiltration
• Schistosomiasis
• Hereditary Hemorrhagic Telengiectasia or
  Osler-Weber-Rendu syndrome (80 of macro)
• Hepatopulmonary syndrome
• Portal pulmonary HTN
• Bronchopulmonary sequestration
• Pulmonary Artery Aneurysm

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CT Scan
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Final diagnosis

• Idiopathic Intrapulmonary AV-Malformation

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Pulmonary AVM

• HHT prevalence 2.5 to 43/100,000
• Average age43 yrs (9-78 yrs)
• MF for HHT for PAVM F/M 1.61
• 80 of macroscopic PAVM are associated with HHT
• 20 idiopathic

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Clinical Features

• HHT Epistaxis (90-95), Hypoxemia/paradoxical
  embolism (15-30), Oral/Mucocutaneus
  Telengectasia (50-80), anemia due to GI
  telengectasia (20-40), CNS AVM (20-30), Hepatic
  AVM
• Idiopathic No f/h, no epistaxis and no
  telengectasia. More likely polycythemia

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Clinical Features

• Right to left shunt
• Pulmonary Hemorrhage
• Paradoxical Embolism- TIA, Stroke and Cerebral
  Abscess
• Pregnancy risk of enlargement leading to more
  hemorrhage and stroke risk with extensive PAVM

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Diagnosis

• CXR
• CT Angiogram
• DSA
• MRA
• Rt-to left Shunt
• 99mTc-MAA perfusion scan
• TTE with bubble study

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• Three-dimensional SSD of a right lower lobe
  pulmonary AVM evaluated with nonenhanced spiral
  CT angiography (3-mm collimation, pitch of 1.3,
  threshold value of -520 HU). Demonstrated is the
  simple angioarchitecture of the AVM consisting of
  a single feeding artery (arrow) and a single
  draining vein (arrowhead), both connected to the
  aneurysmal sac (open circles).

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Treatment

• Percutaneus balloon or Coil Embolization
• Surgical treatment
• Liver transplantation (Microscopic PAVMs)
• Lung transplantation
• Antibiotic Prophylaxis indefinitely

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Follow Up
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