ISG Zoom Meeting with registered ISG members in the company of Mr Raman Malhotra (Consultant ophthalmic and oculoplastic surgeon of Queen Victoria Hospital, West Sussex) on Saturday 17 April 2021 

The call was opened at 10.00am by Mandy Aldwin-Easton and Sarah Griffiths-Little of the ISG.   ISG members from across the globe who had registered to join the call were introduced to Mr Malhotra who had kindly offered his time to share his expertise with us and help to answer questions concerning problems with the management of eye care in those affected by ichthyosis.  Professor Jemima Mellerio (Consultant Dermatologist) also joined the call to share expertise and gain information from Mr Malhotra and our members.  Sue Corbett (ISG secretary) sat muted and taking notes to share with all our members. 

Mr Malhotra showed a detailed diagram of the eye, complete with both upper and lower eyelids.  There are approx. 100 meibomian glands (oil secreting glands) in the upper eyelid and 50 in the lower eyelid whose job it is to produce oil to keep the surface of the eye (cornea) lubricated.  This oily layer is called the lipid layer.  These were shown in the diagram. 

Early years

Monitoring visual development is very important in the first 9 years of a child’s life as, following that period, the child’s vision will not improve.  Clear ocular (eye) surface must be maintained and corrective equipment (spectacles) should be used as and when necessary.  With those people affected by ichthyosis the development of the eyelids is important as the lipid layer is missing, so tears evaporate quickly thereby leaving the ocular surface dry.  The lipid layer is also important to remove waste products and help prevent infection.  Blinking is, therefore, especially important in sufferers of ichthyosis.   

Blinking

In people with normal skin blinking generally occurs every 5 seconds.  However, ichthyosis sufferers lose the drivers to blink so often and need to make a conscious effort to blink – and need to blink more.  When ‘dry eye’ occurs it is usually because of lack of blinking.  With some forms of ichthyosis complete closure of the eye isn’t possible and so ‘dry eye’ will occur.  

Tips to try for ‘dry eye’ 

Eye lubricants – there are many available on the market, and new ones are being developed all the time.  Ensure these do not contain any preservative – and ask your doctor check for new ones (sometimes a GP may prescribe an eye lubricant that he has known to work well for other patients – this, however, may not suit an ichthyosis sufferer and GPs cannot specialize in all areas).  For over-the-counter eye lubricants your pharmacist will help – make sure you ask for preservative–free products. 

Avoid air conditioning.  Instead try humidifiers to increase the moisture content in the air around you. 

When wearing spectacles (even sunglasses) try wraparound frames.  These offer more protection to the eye and helps to lessen tear evaporation. 

Try warm compresses on the eyes.  One sufferer reported using a clean face cloth dipped in warm water, some use ‘eye bags’.  Many are available online (The Eyebag Company is one such company).  Just 2-3 minutes on each eye will work wonders – and also helps to soften the glands along the eyelid edges (remember all the many glands on the edges of the upper and lower eyelids).   

Eyelid stretching – following creaming the eyelids and surrounding areas of skin (Vaseline is fine for this) you can try gently stretching the eyelid daily to encourage better eye closure.  Also, try gently squeezing the edge of the eyelids to encourage the production and secretion of oil to lubricate the ocular surface.  You should use eyelid wipes to gently clean away any excess cream. 

Diet

Consider diet: 

increase intake of clear water 

Reduce caffeine intake to just 2 cups per day 

Reduce alcohol intake 

Use eye drops 4 times daily 

Blink more – especially important when using computer screens as we all tend to blink less in this situation 

Warm eye compresses 

Reduce red meat intake 

Include more oily fish in your diet (or Omega 3 or flaxseed oil supplements) 

Watery Eyes

Watery eyes?  This often happens when there is a change in room temperatures.  This could be dry eye syndrome, so check out the above.  Could also be an eye infection, allergic reaction, foreign body in the eye...

With some ichthyosis sufferers who do not have complete eye closure while sleeping, eye masks can work well.  Cushioned eye masks could help to maintain eye closure.  Good news here!  Currently in development is 3D printer produced eye masks – to exactly fit the contours of individual faces and help to close the eye while sleeping. 

Some of our members reported using HYLO Night eye ointment (previously known as Vita-POS) with good results.  HYLO Forte is also available – this is a stronger cream.  HYLO-TEAR eyedrops forms part of the HYLO range – all these products are preservative free. 

Eyelid grafting

Eyelid skin grafting was discussed. This has been the traditional approach to correcting either eyelid ectropion, or retraction. However, repeat surgery due to skin graft contracture is often necessary and therefore is not always considered the preferred first-line approach where autologous skin may not available and given the other options that are now available, ie hyaluronic filler and oral buccal mucosal grafts. In 2001, Soparkar and Patrinely (Soparkar DN and Patrinely JR 2001) wrote a letter describing and recommending the use of oral buccal mucous membrane (the moist inner lining of the cheek) in preference to skin for grafts of 1.5 cm or less of vertical height. This avoids delayed-healing donor-sites as oral mucosa is unaffected by ichthyosis. The authors had used this approach for many years and showed photographs of an 8 year-old boy, 2 months and 2 years post upper and lower grafting, respectively. Once implanted into the eyelid, mucous membrane grafts rapidly keratinize (become like skin) with little desquamation or significant contraction. Nayak et al (Nayak S et al. 2011) have also reported the use of oral mucosal grafts as a skin substitute in a 26-year old female with LI and ectropion of all 4 eyelids. Left upper and lower eyelid defects were grafted from the left cheek and 4-months later, right eyelids from the right cheek.  

Fillers

Hyaluronic gel filler helps improve eyelid position by tissue-expansion and reinforcement to improve eyelid closure, with faster recovery and fewer complications in comparison to traditional surgical procedures. Mr Malhotra has been treating patients with ichthyosis using this approach for approximately 7 years now. The injections are usually performed under general anaesthesia for children at the same time as an examination.  It can be an effective, repeatable method of eyelid expansion in ichthyosis that can act as a good temporising method, often delaying the need for more invasive surgical procedures. It can give rise to the appearance of fullness of the eyelid. It should however, only be undertaken by those experienced with fillers in the eyelid region. 

This Zoom meeting ended around 11.20am, but not before all questions had been put to Mr Malhotra and answered with good advice added. 

We give our thanks to Mr Malhotra for his time and also to our participating members for their input and engagement which was greatly appreciated.