All posts by alp37

VA Morning report 6.19.17: Puffy hands syndrome

Case summary: Ivan de Kouchkovsky presented a case with a unique presenting complaint that generated tons of great learning this morning of a 73M with metastatic, castrate-resistant prostate cancer who presented with bilateral upper extremity edema and contractures.
Top pearls: 
1) Bonus pearl from the daily ditty: wall thickness can help you distinguish bulla (<1mm) versus cyst (1-4mm) versus cavity (>4mm) on chest CT.
2) There is a real diagnosis called “puffy hands syndrome”! This usually refers to IVDU-related bilateral hand swelling, however, a host of rheumatologic and other diseases can present with this finding.
3) The instances in which patients with heart failure can have low-normal BNP (~100) are: obesity, isolated R heart failure, and pericardial effusion.
 

Cystic lung disease:
  • Recall that cystic lung diseases are an important cause of pneumothorax
  • Nifty nomenclature based on wall thickness on CT chest
 
Wall thickness
Nomenclature
<1mm
bulla (bleb if at parenchyma surface)
1-4mm
cyst
>4mm
cavity
 

Differential diagnosis of bilateral swollen digits or “puffy hands syndrome”:
  • chronic IVDU (causes sclerosis leading to lymphedema)
  • CPPD
  • gout
  • infectious tenosynovitis
  • reflex sympathetic dystrophy
  • eosinophilic fasciitis
  • palmar fasciitis-polyarthritis
  • palmar fibromatosis
  • HCV arthropathy
  • amyloidosis
  • cryoglobulimemia
  • diabetic cheiroarthropathy
  • RS3PE syndrome (remitting seronegative symmetrical synovitis with pitting edema)
  • early scleroderma
  • rheumatoid arthritis
  • paraneoplatic inflammatory arthritis
  • medication reaction
Note that Stemmer’s sign is a physical exam finding characterized by an inability to pinch or lift the thickened skin fold at the base of an affected digit and is found in lymphedema.
Del Giudice P, Durant J, Dellamonica P. Hand Edema and Acrocyanosis: “Puffy Hand Syndrome”. Arch Dermatol. 2006;142(8):1065-1086.
Alexandroff, BL, et al. Woody hands. Lancet, 361 (2003), p. 1344.

HF + low-normal BNP (~100):
  • obesity
  • isolated R heart failure
  • pericardial effusion
Note that Entresto (sacubitril/valsartan), in contrast, will artificially elevate the BNP, as it prevents cleavage.

VA Morning Report: Solid liver lesion without cirrhosis

Case summary: Rockstar MS3 Axel Adams did a fantastic job presenting the case of a 70M with PMH schizophrenia and HCV without cirrhosis, who presented with LE edema and was found to have an IVC thrombus and multifocal hepatic mass.

Top pearls:
1. Recall that 80-90% of hepatocellular carcinomas are associated with cirrhosis. HCC can develop without cirrhosis in patients with HBV, NASH, alpha-1-antitrypsin, and transformation from hepatic adenoma.
2. Work-up of a solid liver lesion without HBV, cirrhosis or extrahepatic malignancy should include checking tumor markers, getting CT or MRI, and considering biopsy versus resection if the preceding tests are non-diagnostic.
3. The three cancers that classically can be diagnosed without a biopsy are: hepatocellular carcinoma, renal cell carcinoma, and testicular cancer.

Solid liver lesion WITHOUT HBV, cirrhosis or extrahepatic malignancy
Thanks to Chief of Medicine/Gastroenterologist Ken McQuaid for joining us today and giving us this great framework
 
Conditions leading to HCC without cirrhosis: NASH, alpha 1 antitrypsin, conversion from adenoma
 
Work-up:
– Lesions <1cm: get MRI with contrast, resect if confirmed HCC, or follow serial imaging if not.
– Lesions >1cm:
1. Check AFP and obtain triple-phase CT or MRI with contrast.
2. If imaging non-diagnostic for HCC, check CA 19-9 for cholangiocarcinoma (can be falsely elevated in biliary obstruction).
3. If work-up remains non-diagnostic, consider biopsy versus resection.
 
Differential diagnosis:
Benign
– hepatic hemangioma
– focal nodular hyperplasia
– hepatic adenoma
– idiopathic noncirrhotic portal hypertension
– regenerative nodules
Malignant
– HCC (including fibrolamellar variant)
– cholangiocarcinoma
– non-Hodgkins lymphoma
– mucinous cystic neoplasms
– mesenchymal tumors (rare soft tissue/vascular sarcomas)
– hepatoblastoma
– metastases

Cancers where tissue is not the issue

1. Renal cell carcinoma- tissue diagnosis obtained via nephrectomy or partial nephrectomy
2. Testicular cancer- tissue diagnosis obtained via orchiectomy
3. Hepatocellular carcinoma- diagnosed radiographically on triple-phase CT or MRI with contrast
Mittal S, El-Serag HB. Epidemiology of hepatocellular carcinoma: consider the population. J Clin Gastroenterol. 2013;47(Suppl):S2–S6.
El-Serag HB. Hepatocellular carcinoma. N Engl J Med. 2011;365:1118-1127.

Gupta S, Bent S, Kohlwes J. Test characteristics of alpha-fetoprotein for detecting hepatocellular carcinoma in patients with hepatitis C. A systematic review and critical analysis. Ann Intern Med. 2003 Jul 1; 139(1):46-50.

VA ICU Report: Neuro-ICU pearls

Case summary:
Thanks to Vijay Kotecha for presenting the case of a 61M with PMH pAF, COPD admitted to the ICU for post-operative monitoring after an elective meningioma resection who became acutely unresponsive.

Top pearls:

  • In an unresponsive patient, your neurologic exam should focus on assessing response to painful stimuli, reflexes/tone, and brainstem reflexes (pupils, oculocephalic maneuver, corneal reflex, cough/gag).
  • The differential diagnosis for pin-point pupils is short: opiates, metabolic derangement, or pontine ischemic/hemorrhagic stroke
  • NCHCT is our first-line radiology exam for an unresponsive patient but can miss recent ischemic stroke, cerebral venous thrombosis, and detailed information about the posterior fossa.

Evidence-based coma exam:

  • 2 localizations of coma: 1) brainstem or 2) bilateral  hemispheres
  • Step 1: Assess level of consciousness with response to noise and/or painful stimuli
    • looking for vocalization, eye opening or limb movement
  • Step 2: Assess tone and reflexes:
    • looking for reflex asymmetry, clonus, and myoclonus
  • Step 3: Assess brainstem reflexes:
    • Pupillary light (CN II)
      • Uneven pupils: point to structural cause
      • If fixed: dilated (7-9mm) early in coma; mid-position (3-5mm) late in coma
    • Oculocephalic maneuver/”Dolls eyes”/”vestibuloocular reflex or VOR” (CN III, IV, VI)
      • when the head is abruptly rotated from one side to the other in the horizontal plane, the patient’s eyes should NOT turn with the head
    • Corneal reflex (V, VII)
      • gently touching the cornea with a tissue or swab should produce a responsive blink
    • Cough, gag, spontaneous breathing (IX, X)
      • can be elicited by deep suctioning

Ddx for pin-point pupils:

  • opiates
  • metabolic derangement
  • pontine ischemic or hemorrhagic stroke

What can’t be seen on NCHCT:

  • recent ischemic stroke
    • a recent systematic review showed that the early findings of ischemia on NCHCT (eg hyperattenuation of a large vessel or cortical sulcal effacement) were only present in ~60% of cases of acute ischemic stroke
    • get a CT stroke protocol, which gets you CT perfusion and CT angiogram (which also gives you information on the posterior fossa like basilar thrombosis)
  • cerebral venous thrombosis (get an MR venogram)
  • detail in the posterior fossa (get either CT w/ contrast, CTA or MRI)

VA Intern Report Pearls 6.8.17: Behcet’s and the Silk Road

Case summary

Thanks to Colin Purmal for presenting the case of a 26F presenting with diffuse abdominal pain, bloody diarrhea and oral ulcers, who was diagnosed with Behcet’s disease.


Top pearls

1. Oral ulcers have a fascinating differential, including infection, autoimmune disease, malignancy, medication-induced, and vitamin deficiencies. But the most common cause is the aphthous ulcer!

2. Consider treating severe infectious bloody diarrhea (e.g. systemically ill, immunocompromised or elderly) with ciprofloxacin. The benefits outweigh the risks except in cases with high concern for EHEC or Salmonella typhi.

3. Behcet’s disease is a key “Crohn’s disease mimicker.”


Oral ulcers

  • Pocket reference Ddx:

Infection– primary HSV, HIV,TB, CMV, EBV, coxsackie, GC/CT, syphilis, fungal

Autoimmune– Behcet’s, SLE, Crohn’s, blistering skin disease (Pemphigus)

Malignant– SCC, lymphoma

Aphthous

Meds- DIHS, SJS

Vit Deficiencies


Antibiotics in infectious bloody diarrhea

  • Most acute infectious diarrhea does not require antibiotic therapy.
  • 2001 IDSA guidelines for dysentery (to use the Oregon Trail-approved terminology) recommend obtaining stool cultures and treating empirically for 3 days in 3 cases: 1) toxic-appearing, 2) elderly, 3) immunocompromise.
  • The main benefit of antibiotic therapy is reduced duration of symptoms.
  • Ciprofloxacin is first-line, but Azithromycin can be used in pregnancy or if high suspicion for resistant Campylobacter (e.g. recent SE Asia travel).
  • Contraindications: 1) high concern for enterohemorrhagic E. coli (concern for precipitating HUS), 2) high concern for Salmonella typhi (concern for promoting carrier state and relapse)

IDSA Guidelines: http://www.uphs.upenn.edu/bugdrug/antibiotic_manual/idsadiarrhea.pdf


Crohn’s disease mimickers

  • Thinking about Crohn’s disease?
  • Consider Behcet’s disease in…patients from eastern Asia or the Middle East (the Silk Road!) with oral ulcerations (more frequent and more severe); genital ulcerations (more specific but less sensitive); systemic symptoms, including ocular symptoms, rashes, pan-vasculitis, weird clots (Budd-Chiari or cerebral venous thrombosis), pathergy (a 20G needle prick causes a papule or pustule within 48 hours).
  • International Study Group for Behcet’s Disease Diagnostic Criteria:
Recurrent oral ulceration: Aphthous (idiopathic) ulceration, observed by physician or patient, with at least three episodes in any 12-month period
Plus any 2 of the following:  
Recurrent genital ulceration Aphthous ulceration or scarring, observed by physician or patient
Eye lesions Anterior or posterior uveitis cells in vitreous in slit-lamp examination; or retinal vasculitis documented by ophthalmologist
Skin lesions Erythema nodosum-like lesions observed by physician or patient; papulopustular skin lesions or pseudofolliculitis with characteristic acnelform nodules observed by physician
Pathergy test Interpreted at 24 to 48 hours by physician

Behcet’s review: https://www.nature.com/nrrheum/journal/v9/n2/abs/nrrheum.2012.156.html

VA Morning Report 6.5.17: Large bowel obstruction and C. diff

Case Summary

72F with a PMH of a prolonged hospital course who developed acute-onset abdominal pain, n/v and obstipation and was found to have a large bowel obstruction attributed to C. diff colitis.


Top Pearls
  1. Don’t forget “things next to the abdomen” that can present as abdominal pain- lower lobe pneumonia, inferior MI, superficial causes (eg zoster), ovaries/uterus
  2. Large bowel obstruction is less common and requires surgery more often than small bowel obstruction. Causes include functional (Ogilvie’s, due to impaired autonomic nervous system) and mechanical obstruction.
  3. Complicated C. diff can cause either total or segmental nonobstructive colonic dilatation, and there is some data to support PR vancoymin in this patient population due to impaired colonic transit.

Bowel obstruction
  • Functional versus mechanical obstruction-
    • Functional- impaired colonic transit due to dysfunctional autonomic nervous system
    • Mechanical- extrinsic, intrinsic, or intraluminal blockage
  • Small bowel obstruction– ~75% of obstructions, more often resolve with medical management
    • Causes (in rough order of incidence)- post-operative adhesions, stricture (IBD, ischemia, XRT), hernias, malignancy, intussusception, volvulus
    • Treatment-
      • Many resolve with decompression from above with NG and supportive care
      • Surgery is indicated if the patient has a closed loop or complete obstruction, perforation, signs of intestinal ischemia and/or is toxic-appearing
  • Large bowel obstruction- ~25% of obstructions, more often require surgical management
    • Functional- Ogilvie’s/pseudo-obstruction (most often in critically ill or post-operative patients), C diff (can be pan-colonic or segmental)
    • Mechanical-
      • Extrinsic to bowel- adhesions, hernias, extraintestinal malignant compression
      • Intrinsic to bowel- colonic malignancy, volvulus (typically sigmoid), inflammation leading to stricture (prior diverticulosis, IBD, ischemic colitis, XRT)
      • Intraluminal- bezoars, feces, foreign bodies including inspissated barium, gallstones (entering the lumen via a cholecystoenteric fistula)
    • Treatment-
      • 75% ultimately require surgery
      • NG if nausea/vomiting and IVF
      • Important to rule out malignancy, stricture with endoscopic evaluation
      • Subsequent treatment depends upon the etiology and location (eg. neostigmine for Ogilvie’s, stenting of a malignant LBO, colectomy in one or multiple stages)

Severe, complicated Clostridium difficile infection
  • This patient had a recent flexible sigmoidoscopy that did not show malignancy or stricture and tested positive for C diff, supporting a diagnosis of severe, complicated C. diff
  • American College of Gastroenterology C. diff severity scoring system and summary of recommended treatments (one of many, all with slightly differing criteria and not validated :)):
Severity criteria     Treatment Comment
Mild-to-moderate– diarrhea plus any additional signs or symptoms not meeting severe or complicated criteria Metronidazole 500mg PO TID x 10 days (or vancomycin 125mg QID x 10 days) Many guidelines suggest starting with PO vancomycin
Severe– albumin <3g/dL plus 1 of the following: WBC >15,000, abd tenderness Vancomycin 125 mg orally four times a day for 10 days
Severe, complicated- ICU admission, hypotension, fever >38.5, ileus or abd distension, mental status changes, WBC >35,000 or <2,000, serum lactate >2.2, end organ failure Vancomycin 500 mg orally four times a day and metronidazole 500 mg IV every 8 h, and vancomycin per rectum (vancomycin 500 mg in 500 ml saline as enema) four times a day Surgical consultation