Online Clinical Case Study (November 2017)

Clinical Cardiology Series
臨床心臟科個案研究

The content of the November Cardiology Series is provided by:
Dr. TAN GuangMing
MBChB, MRCP, FHKCP, FHKAM (Med), Specialist in Cardiology
Dr. CHEUNG Shing Him, Gary
MBBS, MRCP, FHKCP, FHKAM (Med), Specialist in Cardiology
十一月臨床心臟科個案研究之內容承蒙譚廣明醫生張誠謙醫生提供。

CASE 1 - A young patient with abdominal pain and weight loss

A 34-year-old gentleman with good past health presented for 1 year onset of progressive abdominal pain. The pain localized at the epigastrium. There was positional change of pain severity especially when he was lying on his left side. Other than that, there was no other relieving or aggravating factors of the pain. He also complained of loss of appetite and progressive weight loss of > 20lb in the past year. He had been a cyclist and regular jogger, but he noted a marked progressive decrease of his exercise tolerance.

Physical examination was essentially unremarkable. Notably there was no abdominal bruit on auscultation when patient is in supine position.

Extensive investigations including upper and lower endoscopy, CT and MRI abdomen with contrast could not identify a cause for his symptoms. A Duplex ultrasound of the mesenteric vessels were performed. Selected images are shown below.


Figure 1. Abdominal Aorta Spectral Doppler


Figure 2. Celia Artery Spectral Doppler during Inspiration and Expiration


Figure 3. SMA and IMA Spectral Doppler

1.

What did the Duplex ultrasound demonstrate?
A. Celiac Artery stenosis
B. Aortic stenosis
C. Superior Mesenteric Artery stenosis
D. Inferior Mesenteric Artery stenosis
E. Dynamic compression of the celiac artery

 
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2.

What is your diagnosis?
A. Chronic mesenteric ischemia
B. Heyde's syndrome
C. Anorexia Nervosa
D. Chronic pancreatitis
E. Median Arcuate Ligament Syndrome (MALS)

 
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3.

What are the treatment options?
A. Medical treatment with antacid
B. percutaneous angioplasty
C. Decompression of the celiac artery
D. Neurolysis of celiac plexus
E. The combination of both C & D

   
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1. E, 2. E, 3. E

Discussion
Median arcuate ligament syndrome (MALS), also known as celiac artery compression syndrome, results from ananatomical compression of the celiac axis and/or celiac ganglion by the median arcuate ligament and diaphragmatic crura. It is characterized by chronic abdominal pain, nausea, vomiting, diarrhea, and unintentional weight loss. In addition, the pain may be positional, mitigated by leaning forward or drawing the knees to the chest. It is a rare syndrome commonly considered a diagnosis of exclusion of other causes of chronic abdominal pain. Duplex abdominal ultrasonography during inspiration and deep expiration may be used as a preliminary anatomic and physiologic assessment of celiac compression. There are several hypotheses concerning the pathogenesis of the abdominal pain in MALS. One proposes that compression of the celiac artery causes ischemia, or decreased blood flow, to abdominal organs, leading to pain. However, given the extensive communication between the 3 mesenteric vessels, intestinal ischemia is unlikely unless 2 out of the 3 vessels are compromised. Another theory hypothesizes that the pain is actually resulted from the compression of the celiac ganglion. Therefore, the combination of surgical decompression (either open or laparoscopic) of celiac artery and neurolysis with wide excision of the involved celiac plexus are recommended to address both the vascular and neuropathic component of compression.

Reference:

  1. Kim EN, Lamb K, Relles D, Moudgill N, DiMuzio PJ, Eisenberg JA. Median Arcuate Ligament Syndrome-Review of This Rare Disease. JAMA Surg. 2016 May 1;151(5):471-7.
  2. Duffy AJ, Panait L, Eisenberg D, Bell RL, Roberts KE, Sumpio B. Management of median arcuate ligament syndrome: a new paradigm. Ann Vasc Surg. 2009;23(6):778-784.
  3. Duncan AA. Median arcuate ligament syndrome. Curr Treat Options Cardiovasc Med. 2008;10(2):112-116.
   
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The content of the November Cardiology Series is provided by:
Dr. WONG Chi Yuen
MBBS (HK), MRCP (UK), FHKCP, FHKAM (Medicine), Specialist in Cardiology
Dr. CHUI Shing Fung
MBChB (CUHK), MRCP (UK), FHKCP, FHKAM (Medicine), Specialist in Cardiology
十一月臨床心臟科個案研究之內容承蒙黃志遠醫生徐城烽醫生提供。

CASE 2 - An 84-year-old gentleman with exertional chest pain and shortness of breath

An 84-year-old gentleman, independent of daily living, with known history of diabetes mellitus, hypertension and peripheral vascular disease, presented with exertional chest pain and shortness of breath in recent few months. Physical examination revealed loud ejection systolic murmur best heard over right upper sternal border of 2nd intercostal space with radiation to the neck.

1.

What investigation(s) would be helpful in this case?

1. ECG
2. Chest X-ray
3. Echocardiogram
4. Treadmill exercise stress test

A. 1 and 4
B. 2 and 4
C. 3 and 4
D. 1, 2 and 4
E. All except 4

 
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2.

ECG was performed and showed sinus rhythm with left ventricular hypertrophy (Figure 1). Echocardiogram revealed satisfactory left ventricular systolic function, the aortic valve was calcified and thickened with severe aortic stenosis, the mean gradient across aortic valve was 46mmHg (Figure 2 and 3).


Figure 1


Figure 2


Figure 3

2. Which of the following medication should be avoided?
A. Aspirin
B. Metoprolol
C. Frusemide
D. TNG
E. Panadol

 
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3.

Which of the following is/are the appropriate DEFINITIVE treatment of this patient?

1. Conservative management
2. Balloon Aortic Valvuloplasty
3. Open surgical aortic valve replacement
4. Transcatheter aortic valve implantation (TAVI)

A. 1
B. 2
C. 1 and 2
D. 3 or 4

 

   
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1. E
This patient had heart murmur of aortic stenosis with exertional chest pain and shortness of breath. For patients with symptomatic severe aortic stenosis, treadmill exercise stress test should be avoided in most circumstances.

2. D
In patients with symptomatic severe aortic stenosis, their blood pressure is heavily dependent on preload. The use of nitrates may cause profound hypotension because of their inability to augment stroke volume in the face of nitrate induced vasodilation and deceased preload due to the presence of aortic outflow obstruction. Although some studies supported the judicious use of nitroglycerin in patients with hard-to-manage acute pulmonary oedema who have known aortic stenosis (a), their use should be of great caution with intensive hemodynamic monitoring. (b)

3. D.
In patient with symptomatic severe aortic stenosis, if left untreated, the mortality can be very high. The gold standard of treatment in these patients is surgical aortic valve replacement. And for those with high operative risks, either surgical aortic valve replacement or transcatheter aortic valve implantation, after consideration by a multidisciplinary Heart Team, is recommended. (b)

References

  1. D Claveau, A Poha-Gossack, et al. Complications Associated with Nitrate Use in Patients Presenting with Acute Pulmonary Edema and Concomitant Moderate or Severe Aortic Stenosis. Ann Emerg Med, 66 (2015), pp 365-362.
  2. Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP 3rd, Fleisher LA, et al. 2017 AHA/ACC focused update of the 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines J Am Coll Cardiol 2017;Mar 15
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Dermatology Series 皮膚科病例研究

Dermatology Series for November 2017 is provided by:
Dr. KWAN Chi Keung, Dr. TANG Yuk Ming, William, Dr. CHAN Hau Ngai, Kingsley, Dr. LEUNG Wai Yiu and Dr. CHANG Mee, Mimi
Specialists in Dermatology & Venereology
十一月皮膚科個案研究之內容承蒙關志強醫生鄧旭明醫生陳厚毅醫生梁偉耀醫生張苗醫生提供。

CASE 1 - Nodules on the lip

A 88 year-old fisherman complained of a few nodules on his lower lip for nearly 4 years. The nodules were asymptomatic but increasing in size very slowly. There was no trauma history and no spontaneous bleeding. The largest one was around 1.0 cm in diameter which was deep blue in colour. It was not tender. The surface was smooth. There was no abnormality detected in the oral cavity.

Answers

1. What is your diagnosis?
a) Basal cell carcinoma
b) Squamous cell carcinoma
c) Benign Melanocytic Naevus
d) Venous Lakes
e) Melanoma
D (Venous Lake)
Venous lakes commonly occur in people over 50 years old. The typical presentation is a slow growing papule which is asymptomatic on lips. Typically, it is a relatively well-demarcated, soft, compressible and dark-blue papules.
 

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2. Helix of ear is also the common area for this lesion. (True or False)
True
Helix of ears and lips are common sites for venous lakes.
 

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3. Long term sun exposure is one of the precipitating factors for this lesion. (True or False)
True
The long term sun exposure may damage the dermal elastic tissue and vascular adventitia that causes superficial venous dilatation.
 

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4. What investigation can help you to make the diagnosis?
a) No investigation is needed for diagnosis
b) Diascopy
c) Dermoscope
d) Skin biopsy
e) All of the above
E (All of the above)
The diagnosis of venous lakes is clinical and no investigation is needed in simple case. Diascopy can compress the lesion causing blanch to identify the lesion is mainly vascular in nature. The dermoscopy helps to differentiate the venous lake from other melanocytic lesions. Skin biopsy may be necessary in difficult cases.
 

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5. Incisional and Drainage (I & D) is the best treatment for this gentleman. (True or False)
False
I and D is not the best treatment for this gentleman. Venous lake is asymptomatic and harmless, conservative approach particular in this 88 year-old gentleman may be a good option of treatment. Cryotherpay, sclerotherapy and laser therapy are other options. Surgical excision is also the choice.
 

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Dermatology Series for November 2017 is provided by:
Dr. CHANG Mee, Mimi, Dr. TANG Yuk Ming, William, Dr. CHAN Hau Ngai, Kingsley, Dr. LEUNG Wai Yiu and Dr. KWAN Chi Keung
Specialists in Dermatology & Venereology
十一月皮膚科個案研究之內容承蒙張苗醫生鄧旭明醫生陳厚毅醫生梁偉耀醫生關志強醫生提供。

CASE 2 - A 50 year-old man with painful skin rash

A 50 year-old man, who newly started allopurinol for 2 weeks for gouty attack, was admitted for generalized painful skin rash (> 50% body surface area) and flaccid blisters involving his face, body and limbs for 3 days. There were also oral and genital erosions. He was febrile, and with a blood pressure of 120/80 and pulse 120. His baseline bloods were normal. He enjoyed good past health.

1. What is the most likely diagnosis?
a. Stevens Johnson syndrome
b. Toxic epidermal necrolysis
c. Acute generalized exanthematous pustulosis
d. Pemphigus vulgaris
e. Infective exanthem
B
Toxic epidermal necrolysis (TEN) is a rare but life-threatening mucocutaneous reaction, characterized by rapid onset, progressive extensive epidermal necrosis. Patients will have skin pain, erythematous to dusky patches which evolve into blisters and erosions quickly, involvement of mucosal surfaces and systemic symptoms. TEN patients have more than 30% epidermal detachment while patients with Stevens Johnson Syndrome (SJS) have less than 10% epidermal detachment.
 

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2. What is the most common cause of the problem?
a. Idiopathic
b. Viral infections
c. Drug
d. HIV seroconversion
e. Autoantibodies targeted at the hemidesmosomes of the epidermal keratinocytes
C
In more than 90% of the cases, TEN is due to drug. In very few cases, infections, autoimmune disease (acute SLE) can trigger TEN and rarely, no cause is found. Allopurinol is considered to be one of the high-risk culprits for TEN.
 

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3. Which type of HLA allele test could be ordered to predict the increased individual risk of Stevens Johnson Syndrome and Toxic Epidermal Necrolysis in allopurinol use?
a.HLA-B*5801
b.HLA-B*5701
c.HLA-A*3101
d.HLA-B*1502
e.HLA-B*1301
A
HLA B*5801 is found to be positive in patients with allopurinol-induced SJS and TEN.
 

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4.

What is the best course of management?

i. The patient should be given best supportive medical treatment
ii. Allopurinol should be stopped immediately
iii. Intravenous immunoglobulin is the first-line treatment with strongest evidence with survival benefit
iv. Ophthalmologist should be consulted to look for eye involvement
v. Active debridement of skin is important to reduce risk of infection

A. i and ii
B. i, ii and iv
C. ii, iii, and v
D. i, ii, iii and iv
E. all of the above

B
Early withdrawal of offending agent and multi-disciplinary best supportive medical therapy are the most important and essential measures for SJS/TEN. As of this moment, there has been no conclusive evidence that favours any active intervention (immunoglobulin, steroid, and cyclosporine) that confers significant survival benefit compared to best supportive medical therapy. Debridement should be avoided. Keeping the roof of the blister (epidermis) intact is important to prevent secondary bacterial infection and hasten recovery in TEN.
 

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