Liver Cysts, Myasthenia Gravis and Orf
CLINICAL Q&A
Our team of experts answer queries on orf, sudden ptosis, syncope and poly cystic livers
Q The liver of my 57-year-old patient is covered in cysts. She has had numerous operations to de-roof the cysts, but it is becoming increasingly dangerous each time it is done. Can you suggest the next course of action?
A it is likely that your patient has polycystic liver disease (PLD). Is there a family history and does she have cysts elsewhere, particularly in the kidneys?
PLD is associated with kidney cysts in more than 50 per cent of cases. Cysts can also be detected in some patients at other sites, including the pancreas, thyroid and choroid plexus. Assuming your patient has PLD, treatment will depend on her symptoms.
In our experience, there is little to be gained from de-roofing the cysts, because they just grow back and any relief is only temporary. If the patient develops sudden pain or fever, she may have bled into a cyst or developed an infection in a cyst. In these circumstances, it is worth aspirating the affected cyst under radiological control. Otherwise I would leave the liver alone.
Patients with PLD do not develop liver failure, although there is a small risk of massive bleeding into the cysts.
The main problems patients have are symptoms of discomfort and compression related to the massive enlargement of the liver. If symptoms become intolerable, liver transplantation is a good option.
It is important to be aware of and treat the other manifestations of this syndrome.
More than 50 per cent of patients with polycystic kidney disease (PKD) are hypertensive, regardless of whether they have renal involvement. This should be treated aggressively to prevent accelerating the renal damage.
These patients also have a higher incidence of berry aneurysms than the general population.
The mechanisms of cyst development and growth are beginning to be understood and it is possible in the future that novel drugs aimed at inhibiting growth factor receptors in cancer will also be effective in PKD.
Professor David Adams, consultant hepatologist, Queen Elizabeth Hospital, Birmingham and professor of hepatology, liver research laboratories, Birmingham University
Q Occasionally I see a case of orf among my farming patients. Is there an effective treatment for this condition?
A Orf is usually a disease of sheep and goats, and is caused by parapox virus. Humans acquire the disease through direct contact with infected animals or with contaminated fences or troughs.
The lesions (ecthyma contagiosum) usually appear on hands and arms. They last several weeks and are painful. The virus is identified by electron microscopy or tissue culture.
Orf regresses spontaneously and conservative management is indicated. Surgical intervention delays healing and aggravates the lesion. Bacterial superinfections may need to be treated with an antibiotic, such as flucloxacillin.
The disease may be severe in immunocompromised patients, and no clear treatments have emerged. Only case reports have been published.
The antiviral drug cidofovir has been used topically with success. Topical idoxuridine has been used with varying results. Lesions have also responded to cryotherapy.
Dr Peter Wilson, consultant microbiologist, Middlesex Hospital, London
Q I was recently consulted by a young girl who may have myasthenia gravis. She has a history of sudden closure of both eyelids for a few seconds three to four times a day. She then has to open her eyes manually. She has no visual problems.
A Myasthenia gravis is worth considering, although more commonly it causes asymmetrical ptosis which varies through the day and may be caused by fatigue. Complete ptosis is unusual unless there are other symptoms such as diplopia, though unilateral complete ptosis will abolish diplopia.
This case sounds more like an apraxia of eye opening, which is not common. It is sometimes seen in older people as an isolated idiopathic condition or as neurodegenerative disease. The youngest case I have seen was middle aged.
Blepharospasm is another possibility, although these patients tend to complain of eye twitching, not closure.
It is worth considering whether this is organic. A neurological or neuro-ophthalmic opinion should be sought, but with no great urgency if the patient remains otherwise well. The patient is ineligible to drive and must inform the DVLA.
I would not offer any treatment, pending specialist opinion. I would consider FBC, ESR, B12, U&E, LFTs, calcium, glucose, protein strip, T4, TSH and thyroid and antiacetyl choline receptor antibodies. If there was sufficient concern, then MRI of the brain and orbits and magnetic resonance angiography of the circle of Willis might be considered.
Single-fibre electromyography of the orbicularis oculi is another option. Most results will probably be normal.
If this is blepharospasm, then no investigations may be needed, and there may be a good response to botulinum toxin.
Dr Giles Elrington, consultant neurologist, The City of London Medical Centre
Q I recently came across the diagnosis of ‘reflex anoxic syndrome episode’ on the records of a patient visiting A&E on three occasions. What are the symptoms and signs?
A I think ‘reflex anoxic syndrome episode’ means syncope. Syncope is sometimes accompanied by jerking and other features reminiscent of epilepsy. It is very important not to confuse this with epilepsy.
More commonly, the term ‘reflex anoxic seizure’ is used but this leads the unwary to believe that an epileptic seizure has been diagnosed.
Syncope begins with nausea, sweating, and blackness of vision and may proceed to collapse with pallor, brief unconsciousness, sometimes stiffness, jerking, tongue or lip biting, and incontinence. Recovery is usually brisk without postdrome, although anxiety, panic, and propping the patient upright may cloud the picture.
Episodes of collapse or altered awareness are difficult to diagnose. A witness account is essential. It is important to be open- minded about diagnostic labels of ‘funny turns’. I recommend your patient be referred to a neurologist.
Dr Giles Elrington
Polycystic liver disease is not normally improved by surgical de- roofing
Unilateral ptosis Is more commonly associated with myasthenia gravis
Copyright Haymarket Business Publications Ltd. Sep 2, 2005