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Morbidity and Mortality Conference. January 9, 2002 A# 50239521-3 (K1304) Carole Bibeau, MD. Initial Presentation. 69 yo WM establishing care at the WRJ VA Overall felt well ROS: DOE x 6 months, “dizziness,” easy bruising. Past Medical History Diabetes insipidus HTN CVA 1997

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morbidity and mortality conference

Morbidity and Mortality Conference

January 9, 2002

A# 50239521-3 (K1304)

Carole Bibeau, MD

initial presentation
Initial Presentation
  • 69 yo WM establishing care at the WRJ VA
  • Overall felt well
  • ROS: DOE x 6 months, “dizziness,” easy bruising
history
Past Medical History

Diabetes insipidus

HTN

CVA 1997

PVOD with LE claudication

Medications

Desmopressin nasal spray qd

Amlodipine 10 mg PO qd

Benazepril 20 mg PO qd

ASA 325 mg PO qd

ALLS: NKDA

History
social and family history
Social History

Married, lived in New London, NH

Tobacco: none

EtOH: 1 drink/day

Retired salesperson for surgical cleaning supply company

Family History

Mother alive age 94, lived alone, healthy

Father deceased age 74, unknown cause

Sister alive, well

No FHx CAD, DM, cancer

Social and Family History
physical exam
Physical Exam

Gen: Looks slightly older than stated age

VS: BP 110/60 HR 72 wt 160#

HEENT: PERRL

Card: RRR with occasional early beat; extra sound mid-systole; no S3 or S4; possible diastolic murmur

Resp: Clear

Abd: Soft, NT, ND, +BS

Extr: No edema

Neuro: No nystagmus; gait stable

laboratory data
Laboratory Data

Fe 22 (50-180)

TIBC 300 (205-395)

Fe saturation 7.3%

ESR 33

B12, Folate, Ferritin WNL

11.3 MCV 71

7.8 329 RDW 19.4

N70 L15 M12 E3

2+ anisocytosis, microcytosis, hypochromia

134 102 21

65

4.2 23 1.1

Ca, albumin, LFT’s, TSH, lipids, PSA WNL

two months later
Two months later...
  • Follow up visit
    • Interval history: new ecchymoses on forearms
    • VS: BP 96/70 HR 56 wt up 3#
    • Exam:
      • HEENT: no mucosal bleeding
      • Card: RRR, systolic murmur
      • Resp: decreased BS at bases bilaterally
      • Extr: ecchymoses on forearms
  • CXR
  • Echocardiogram
admission to wrj vah
Admission to WRJ VAH
  • Patient admitted for further evaluation
  • ROS: denied chest pain, tearing sensation, back pain, abdominal pain, SOB at rest, HA, vision change, F/C/S, BRBPR, N/V
  • Positive for decreased appetite and intermittent constipation x 4 months, estimated 8 pound weight loss
slide9
Exam

Gen: Very pleasant 69 yo WM, A+Ox3, NAD

VS: T 96.3 HR 87 BP 108/78 RR 20 sat 98% RA no pulsus

HEENT: PERRL, EOMI, op moist, no petechiae

Neck: Supple, no LAD, JVP 7 cm, carotids 2+ without bruits

Card: RRR, muffled S1; S2 physiologically split, II/VI SEM LLSB

Resp: Diminished BS left base

Abd: NABS, soft, NT, ND, no bruits

Rectal: Nl tone, prostate somewhat enlarged, no masses, soft brown stool, heme positive

Extr: Toes cool, femoral pulse 1+ L, 2+ R, no femoral bruits, pedal pulses obtainable via Doppler (monophasic), 1+ pitting ankle edema, multiple small ecchymoses on UE’s

Neuro: Grossly non-focal

assessment and plan
Pericardial effusion

Aortic dissection

Malignancy

Inflammatory process

Infectious process

Coagulopathy

Aortic dilatation

Aortic dissection

Collagen vascular disease

Syphilis

Atrial abnormality

Thrombus

Tumor

Anemia, heme positive stools

Malignancy

Aorto-enteric fistula

Assessment and Plan
additional data
Additional Data

Reticulocyte 1.1%

ANA POSITIVE

1:320 speckled

1:160 homogeneous

RF <20

ANCA (c and p) negative

Anti-DS DNA negative

RPR negative

Blood cx x 1 negative

PTT 53.4

PT WNL

THROMBOSIS SCREEN

Lupus anticoagulant positive

Negative: Anticardiolipin Ab, Factor V Leiden

ECG:

—NSR 64, nl axis, RBBB (new)

—low voltage, no pathologic q’s

—ST depressions V2-V6

—non-specific T changes

U/A: pH 6.5, s.g 1.015, WNL

consultations findings
Consultations/Findings
  • Colonoscopy
    • Mass at splenic flexure suspicious for malignancy, near complete obstruction; cannot pass scope beyond it
    • Path: Adenocarcinoma, moderately differentiated
  • Rheumatology
    • +ANA non-specific, likely due to malignancy
    • Aneurysm not due to aortitis; ?paraneoplastic syndrome
  • Vascular + CT Surgery
    • Not feasible to operate
  • Cardiology
    • Lipomatous hypertrophy; no further evaluation needed
left hemicolectomy hd 10
Left Hemicolectomy, HD #10
  • Findings
    • Moderately to poorly differentiated adenocarcinoma, extension to pericolic fat
    • 11/26 nodes positive, no obvious liver involvement
    • Tumor-associated thrombosis; start LMWH
    • Tumor board: offer adjuvant chemo when more stable
  • Complications (HD #10-23)
    • Post-op atrial fibrillation with chest pain, hypotension
    • Delirium
    • Prolonged ileus
    • Fluid and electrolyte abnormalities
hospital day 24 pod 14
Hypotensive sitting up in chair

Telemetry alarm: junctional rhythm with PAC’s, rate 60

Medicine consult requested

Medications

Metoprolol, desmopressin, heparin SC

VS

Afebrile

BP 92/54, HR 60 irr, RR 24

I>>O, est 8 liters positive

Exam

Chronically ill appearing

JVP 7

Upper airway ronchi, copious frothy sputum

Can’t auscultate heart sounds

Abd without BS, mildly tender

ECG

Afib vs junctional rhythm

RBBB

CXR

Early CHF

Hospital Day 24 (POD #14)
hospital days 28 36
Cardiac

HD #28: Afib with RVRIV diltiazem bradycardic with 3-6 second pauses

Temporary pacer placed; amiodarone initiated

PA catheter placed

RV 41/13

PA 41/17

PCWP 30

CI 2.4, SVR 1249

ID

HD#24:

MRSA in sputum

E. faecalis 2/4 bottles in blood; start ampicillin

HD#27:

Line tip (+) E. faecalis

HD#36:

Blood cultures neg x 5 days

Dispo

Transfer back to medicine

Hospital Days #28-36
nursing notes
Nursing Notes
  • Hospital Day #35
    • “I really felt good before I came in. I thought that I had some minor problem and it would be fixed. I never thought I had all of this going on.”
    • “I would like to die peacefully and with dignity. Could you see to it that happens?”
transfer to dhmc hd 41
Transfer to DHMC (HD #41)
  • Tachy-brady syndrome, symptomatic
  • Thoracic aortic aneurysm
  • Pericardial effusion
  • Pleural effusions
  • Stage III colon cancer, s/p resection
  • Lupus anticoagulant, tumor embolus, on anticoagulation
  • Enterococcal bacteremia, MRSA + sputum
  • Diabetes insipidus
  • Deconditioning, prolonged hospitalization (41 days)
medications on transfer
Medications on Transfer

Amiodarone 400 mg PO qd

ASA 81 mg PO qd

Furosemide 80 mg IV bid

Metoprolol 2 mg IV q4hr

Spironolactone 25 mg PO qd

Ampicillin 2 gm IV q4hr

Desmopressin nasal spray bid

Ferrous sulfate 325 mg PO tid

Magnesium oxide 1 tab PO qd

Heparin 5000 u SC bid

Rabeprazole 20 mg PO bid

PRN:

Acetaminophen, albuterol nebs, AlOH/MgOH, docusate, ipratropium nebs, lubriderm, miconazole powder, NTG SL, prochlorperazine

hospital course hd 41 43
Hospital Course (#HD 41-43)
  • HD #43: DDDR pacer placed
  • Patient wishes to transfer back to VA
hospital day 44
Hospital Day #44
  • Am VS check: 112 afib, 107/70, RR 30, 80-83% 3 liters, “very wet sounding cough” per nursing. Patient denied SOB.
  • Team recommended thoracentesis for symptoms
  • Patient refused care; did not want wife to know
  • Team discussion with patientCMO instituted
  • Wife notified of grave situation
  • Patient died peacefully with wife at bedside
  • Permission for autopsy granted
slide22

Aorta:

Dilated from arch to iliac bifurcation

Severe atherosclerosis

Aneurysm with mural thrombus

slide24

Left kidney

Right kidney and adrenal

slide25

Left kidney

Right kidney

slide26

Periadrenal fat

Peripancreatic fat

slide27

Right lung, septum

Right lung, pleura

slide30

Right atrium and ventricle, schematic

Right atrium and ventricle, A-01-153

autopsy finding disseminated xanthogranulomatous process
Cardiovascular

Periaortic

Atrial myocardial

Pulmonary

Pleural

Septal

Bronchovascular bundles

CNS

Dural nodules

Hypophyseal stalk

?pituitary

Retroperitoneal

Perirenal

Periadrenal

peripancreatic

Autopsy Finding:Disseminated Xanthogranulomatous process
disseminated xanthogranulomatous inflammation differential diagnosis
Disseminated Xanthogranulomatous Inflammation:Differential Diagnosis
  • Infection: Syphilis, atypical mycobacteria, fungi
  • Malignancy: inflammatory fibrosarcoma, lymphoproliferative disorders
  • Histiocytoses
  • Other: inflammatory pseudotumor, lipid storage disorders
histiocytoses
Histiocytoses
  • Class I: Langerhans cell histiocytosis
    • Eosinophilic granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease
  • Class II: non-Langerhans cell histiocytosis
    • Hemophagocytic lymphohistiocytosis (familial erythrophagocytic lymphohistiocytosis), virus-associated hemophagocytic syndrome, sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease), Erdheim-Chester disease, xanthogranuloma, xanthoma disseminatium, reticulohistiocytoma, juvenile xanthogranulomatosis
  • Class III: malignant histiocytic disorders
    • Acute monocytic leukemia, malignant histiocytosis, histiocytic lymphoma
langerhans vs non langerhans cell histiocytoses
Langerhans cell

Children and juveniles

Bone-marrow derived antigen presenting cells not of monocyte/macrophage lineage

Immunohistochemical staining: CD1a +, S-100+, CD68 -

Non-Langerhans cell

Adults: mean age 53

Monocyte-macrophage lineage

Immunohistochemical staining: CD1a -, S-100 essentially negative, CD68 +

Langerhans vs. Non-Langerhans cell histiocytoses
non langerhans cell histiocytoses
Hemophagocytic lymphohistiocytosis

virus-associated hemophagocytic syndrome

Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy

Erdheim-Chester disease (Lipid granulomatosis)

xanthoma disseminatium

Reticulohistiocytoma

juvenile xanthogranulomatosis

xanthogranuloma

Non-Langerhans Cell Histiocytoses
erdheim chester disease
Histopathology: Infiltration of multiple organ sites by foamy histiocytes, lymphocytesand scattered Touton giantcells with extensive fibrosis

Clinical manifestations:

Symmetric, sclerotic lesions of long bones

dyspnea and respiratory failure due to interstitial lung disease

Hydronephrosis and renal failure secondary to retroperitoneal xanthogranulomatosis

Diabetes insipidus

Extra-axial masses(dural nodules)

Retroorbital masses with visual disturbances

Ataxia secondary to cerebellar involvement

Pericardial infiltration --> pericardial effusion

Erdheim-Chester Disease