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Morbidity and Mortality. January 23, 2001 Randy Hoover MD. Eponyms: Livedo reticularis associated with stroke-like episodes is known as?. Sly’s Syndrome Sneddon’s Syndrome Riley-Day Syndrome Shwachman’s Syndrome Richter’s Syndrome.
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Morbidity and Mortality January 23, 2001 Randy Hoover MD
Eponyms: Livedo reticularis associated with stroke-like episodes is known as? • Sly’s Syndrome • Sneddon’s Syndrome • Riley-Day Syndrome • Shwachman’s Syndrome • Richter’s Syndrome
73 year old woman presents to an outside acute care clinic with a chief complaint of back pain. • Upper-thoracic region • Described as a “bunch,” mild in severity • Constant, no radiation or change with position, not respirophasic • Similar to recent transient episodes
History of Present Illness • Associated with fatigue and malaise • Night prior to presentation, unable to get comfortable; sweats and nausea • Recent nose bleeds • No fevers or rigors • No chest pain, SOB or abdominal pain • No bowel or bladder symptoms
Chronic A.Fib Anticoagulated on warfarin H/O Atypical Chest Pain Cath 12/00, normal Chronic Low Back Pain HTN CRI Baseline Creatinine 1.5 COPD Chronic Diarrhea Temporal Lobe epilepsy S/P Appendectomy, herniated bowel repair Past Medical History
Medications • Diltiazem CD 360 mg po qd • Losartan 50 mg po qd • Triamterene 50 mg po qd • Warfarin 5 mg po qhs • Metoprolol XL 50 mg po qd • Amlodipine 5 mg po qd ADR’s: Morphine, ACE Inhibitors
Social History • Widowed mother of 2 • Consumes a glass of sherry and of cognac daily • Current 2 ppd smoker • Approx 100 pk year history • Lives alone and functions independently
Physical Exam Gen: 73 yowf, pleasant, NAD, who appeared older than her stated age T=97.9 P=89 R=18 BP=126/90 Heent: EOMI, PERLA, OP pink and moist. Sclera anicteric Neck: Supple, JVP =6 cm Lungs: Poor air movement but otherwise clear CV: Irreg Irreg no MRG and variable S1 AB: + Bs, soft, non-tender, non-distended, no masses, no hepatosplenomegaly Back: Tender in the mid-dorsal region. Pain could be reproduced. No paravertebral or bony tenderness. No muscular spasm Ext: No c/c/e
Labs 141 104 22 4.2 22 1.4 Total Bili = 0.5 Alk Phos= 190 Ast = 23 Alt = 21 Amylase = 219 Lipase = 285 ESR = 67 99 13.3 4.7 234
Initial Radiology • RUQ Ultrasound: Multiple gallstones, no wall thickening, no free fluid or dilated ducts • CT Abdomen: Gallbladder is distended, no gallstones, slightly enlarged common hepatic and common bile ducts
2 weeks later: Seen by general surgery at DHMC for possible symptomatic cholelithiasis Pt extremely reluctant to undergo surgery “ I’ve not been significantly bothered by this” Referred to GI for possible ERCP 1 month later: Seen by GI Persisently elevated alk phos and amylase Thought secondary to etoh vs stone passage Further Evaluation
-Management Options-What would you do next? • Ursodeoxycholic acid • HIDA scan • MRCP • ERCP • Recommend Surgery • Watchful waiting
-Test Characteristics-Magnetic Resonance Cholangiopancreatography (MRCP) SensitivitySpecificity Choledocholithiasis1,2 40%-100% 85% Cystic duct calculi3 100% 93% Pancreatic cancer4 84% 97% Chronic Pancreatitis4 88% 94% • Scheiman JM, et al. Can endoscopic U/S or MRCP Replace ERCP in suspected biliary disease? American Journal of Gastroenterology 2001;96: 2900-2904. • Chan YL, et al. Choledocholithiasis: comparison of MRCP and ERCP. Radiology 1996;200: 85-89. • Park MS, et al. Acute cholecystitis: comparison of MRCP and US. Radiology 1998;209: 781-785. • Adamek HE, et al. Pancraetic cancer detection with MRCP and ERCP; a prospective controlled study. The Lancet 2000;356:190-193.
MRCP (Thin Slab) 4-5 mm slices, 15 slices, 30 sec scan
Choledocholithiasis MRCP 5mm MRCP MIP ERCP
ERCP • Could only cannulate pancreatic duct • Dye injected into pancreatic duct showed local dilatation • Brushings of pancreatic duct • Sent to IR for transhepatic cholangiogram • Percutaneous Transhepatic Cholangiogram • Mildly distended intra/extrahepatic ducts • Narrowing of distal common bile duct • No dye spilling into duodenum, cholecystostomy tube placed Admitted for monitoring
Physical Exam Gen: 73 yowf, lethargic but arousable, anicteric T=37.4 , P=114 irreg, R=18, BP=210/130, 93% on 3 L Heent: Eomi, perrla, op pink and moist, fundi not examined. Neck: Supple, no lad, JVP=7cm CV: Irreg irreg, no m/r/g, variable s1, nl S2 Lungs: Few crackles at bases fair air movement, otherwise clear Ab: + Bs, soft, distended, mildly tender in RUQ, no masses, no rebound, no guarding. Biliary drain without oozing Ext: Moves all 4, no c/c/e Neuro: Sleepy but arousable, ambulates with unsteady gait, grossly non focal
Labs Total Bili = 1.0 Alk Phos= 176 Ast = 43 Alt = 32 INR = 1.1 PTT = 26 140 101 16 3.5 23 0.8 UA: pH 1.032, protein >300, trace ketones, negative urobilinogen, no wbc, no rbc 16.5 10 193
Assessment and Plan • Hypertensive urgency • EKG without signs of ischemia. Pt with lethargy and + proteinuria • IV Labetalol PRN until SBP decreased < 180 • Restart oral antihypertensive agents: diltiazem, losartan, metoprolol, and amlodipine • Ductal dilatation s/p ERCP and PTC • Hydrate • Monitor LFTs and for signs of post-ERCP pancreatitis • Cefotetan for prophylaxis • F/U on Brushings
The incidence of post-ERCP pancreatitis is estimated to be: • 5% • 15% • 30% • 50% • 75%
Post-ERCP Pancreatitis • Serum amylase elevated in 75% of patients • 5% have clinical pancreatitis • MOST mild/moderate, rarely (0.4%) severe • Usually with therapeutic (versus diagnostic) • Prediction rules • Amylase < 276, lipase < 1000 @ 2 hours • Prevention • Technical, stents, pharmacologic • Antibiotics, calcitonin, glucagon, nifedipine, C1-inhibitors, secretin, anticoagulation, corticosteroids, somatostatin, octreotide, gabexate mesilate, IL-10 Freeman ML, et al. Complications of Endoscopic Biliary Sphincterotomy. NEJM 1996;335:909-918. Loperfido S, et al. Major early complications from diagnostic and therapeutic ERCP: a prospective multicenter study. Gastrointestinal Endoscopy 1998; 48: 1-10.
Hospital Days 2-4 • Hypertension/A.fib • Improved with oral agents • Post ERCP pancreatitis • Amylase 600 • Lipase 3780 • NPO, pain control, continue IV Hydration • Cholecystostomy tube falls out • IR contacted: recommend monitoring LFTs • Day 4 • Feeling much better, tolerating clear liquids, LFTs stable at baseline
Hospital Day 5 • C/o Increasing RUQ pain, worsening abdominal distention, and nausea • Labs: • Plan: NPO, adequate pain management, follow LFTs, place PICC line and begin TPN Amylase = 167 Lipase = 324 T.Bili = 1.6 D. Bili = 0.7 Alk Phos = 152 AST = 22 ALT = 17
Hospital Day 6 • Worsened abdominal pain and distention. • New rhonchi bilateral lung bases • Labs: T.Bili = 1.7 D. Bili = 1.2 Alk Phos = 152 AST = 129 ALT = 173 Amylase = 93 Lipase = 121 16.3 9.6 179 • CT Abdomen and Pelvis
Hospital Day 7 • Worsening abdominal pain and distention • Return to IR • Attempted to drain bile pool around liver, but unable to do so • Replace cholecystostomy tube • Somnolent and short of breath • ABG: 7.25/50/77 on 2 L, oxygen increased to 4 liters • CXR: CHF • Lasix 20 mg IV • Appeared to stabilize
Somnolent and unarousable Acute Abdomen Absent bowel sounds, + guarding and rebound Urgent surgical consultation Exploratory Laparatomy Bile Leak from right medial lobe of liver at previous puncture site, cultures sent Cholecystectomy: gallbladder full of stones, signs of chronic cholecystitis T-Tube inserted No masses noted Hospital Day 8 • Transferred to ICU on ventilator
Fever spikes Peritoneal fluid growing Enterococci Hospital acquired pneumonia Hospital Day 9-13 • Brushings Returned: • Bile Duct: negative for malignancy, + inflammation • Pancreatic Duct: ATYPICAL; atypical ductal epithelial cells. Metaplastic and benign mucosal cells present
Hospital Day 14 • Defervesced • Oliguric, rising BUN/CR • Increased ventilatory requirements • Increasing LFTs Total Bili = 4.5 Alk Phos= 123 Ast = 21 Alt = 27 TP = 4.3 Alb = 0.7 133 104 40 4.0 16 1.8 UA: no wbc, rbc, nitrites, many granular casts 99
Family Meeting Daughter indicated that her mother would not want her life prolonged by aggressive measures Family requested to withdraw support Pt made DNR/DNI Support withdrawn Pt died peacefully 3 hours later Family refused autopsy Family Meeting
Haunting Questions At what point did this go wrong? What was her diagnosis?