SHAR-PEI DOGS & CONGENITAL ENTROPION

I consider it critical to share my extensive personal experiences and findings on this subject with the public, in particular Shar-Pei lovers.

The big question is – is congenital entropion hereditary? To set the record straight:

CONGENITAL ENTROPION IS A PEDIGREE-RELATED HEREDITARY DISEASE, A GENETIC DISORDER TRANSMITTED THROUGH BREEDING!

The Shar-Pei breed is predisposed to congenital entropion. Scientists believe that the transmission is polygenic. Hereditary diseases are present at birth or manifest themselves soon thereafter, but some breeders continue to downplay the consequences of this problem. They classify the illness simply as some sort of an acquired spastic entropion, insisting that it is often caused by the paws of siblings inadvertently scratching the eyes of others while suckling, or some such lame excuse.

It is easy for a vet to distinguish the two forms of entropion: “If the eyelids remain closed or continue to squint after administering local anesthetic, it is clearly not spastic entropion but rather congenital entropion.”
A puppy opens its eyes in a week or two after birth, marking the first instance when it is actually able to see something. Quickly thereafter follows the second instance when a puppy with this disease sheds tears. It immediately closes either one eye or at times both – to avoid severe pain! This is the unfortunate outcome of congenital entropion, a consequence of selected mating of its parents! It would have been possible to avoid transmission of congenital entropion, if the breeder had acted responsibly in selecting the sire and dam. Dogs with pedigree-related diseases, even entropion, should never be bred. 
Entropion & its causes
Entropion is an eyelid disorder in which the eyelid’s border (usually the lower one) inverts along a part or all of its length. The word entropion refers to this pathological inversion, commonly called rolling in of the eyelids. This prevents the eyelids from functioning properly to protect the eye.
Eyelids are made of two movable skin folds, an upper and a lower one. They have an important job – to protect the eyeball against foreign objects and strong light. On sensing danger, they blink and close by reflex in a flash. The eyelid borders have several glands, with openings along the edge that look like tiny yellow points with fine oily secretions. Normally, eyes blink involuntarily six to twelve times a minute and spread a stable film of tears from the glands evenly over the corneas to keep them moist and protected. 
The eyelid borders themselves are hairless (no eyelashes), but as the eyelids invert the coat hair tend to rub the eyeball. Entropion is always accompanied by runny eyes and pain, squinting, conjunctivitis, and inflammation of the cornea. Constant rubbing of the cornea by the hair can lead to tiny defects, such as a chronic state of irritation of the conjunctiva (red eye) and flowing tears.
Affected animals react by squinting, referred to as blepharospasm. Mechanical rubbing by the hair can also develop into an inflammation of the eye, followed by corneal ulceration and scarring – which in turn reduce visual acuity of the affected dog. In severe cases, keratohelcosis or corneal ulceration that develops can burst, punching a hole in the cornea. This is a very serious condition that can lead to loss of the eye. In the face of such serious risks, vets view entropion as an ophthalmological emergency. 

Symptoms

•    Inverting of the eyelids, mostly the lower ones
•    Foreign body sensation in the eye, causing the dog to rub it with its paws
•    Excessive blinking, flowing tears
•    Sensitivity to light
•    Squinting of affected eye, which is held tightly closed
•    Chronic irritation of the conjunctiva
•    Conjunctivitis, eye is red and runny
•    Corneal erosion/wear
•    Corneal susceptibility to infections 
•    Formation of corneal ulcers (keratohelcosis)
•    Pathologic vasculogenesis  
•    Suppuration (heavy mucoid or pus eye)
•    Sticky eyes 
•    Loss of appetite
•    Cessation of weight gain by affected puppies

Forms of entropion

There are several forms of eyelid abnormalities that can occur:

A.    Congenital entropion
Congenital entropion is gene-mediated and it develops over the first few weeks or months of life, but sometimes also later in life. This hereditary disease is like torture for the growing puppy.

It is easy to diagnose this eyelid disorder. The vet uses a slit lamp to identify how far the cornea has been rubbed by hair and the extent of the damage caused. Certain breeds are predisposed to congenital entropion that frequently affects the entire family. It is believed that the root cause is polygenic, involving simple transmission of dominant or recessive genes. Even though scientists are unable to identify the specific gene, responsible breeders should consider this suspicion as adequate justification for excluding breeding stock with congenital entropion!

To maintain silence as a breeder and to continue using affected dogs for breeding – for reasons of profit – not only doesn’t help our dogs, it clearly undermines the practice of purebred breeding. Personally, I consider such behavior tantamount to criminal acts of a malicious nature, aimed at torturing the offspring of the affected parents!

Although I have said it often enough, I will repeat my stance once more: dogs with entropion should not be bred, even if their eyelids return to normality after an operation. Not using affected dogs for breeding would at least halt further transmission of the genetic disorder. Many scientists and experts worldwide have been demanding strict compliance with this practice. However, for serious breeders interested in healthy Shar-Pei pedigrees, the only solution is to:
Ban the breeding of animals with congenital entropion!

B.    Acquired entropion
Acquired entropion develops as a sequela of an injury of the eyes that causes scarring, or some other painful ailment of the eyes that develops into spastic entropion.

C.    Spastic entropion
As mentioned above, this form of painful spastic entropion is easy to diagnose. A vet simply applies a local anesthetic to suppress the pain, upon which the dog opens its eye. A checkup is critical, since it allows the vet to narrow down and identify the form of entropion, and appropriately treat the source of pain and inflammation.

D.    Senile entropion
This form of age-related entropion occurs in adult dogs following laxity of muscular tonus, which results in loss of horizontal tension in eyelids and their ability to protect the eye. Thereafter, the respective eyelid tends to invert.

Treatments
There is no prophylactic treatment. Only an operation can correct such an eyelid abnormality. Ocular ailments should never be treated with chamomile, since that dries out the eyes and promotes irritation.

A.    Tacking – a temporary solution
The main purpose of eye tacking is to hinder serious damage and/or prevent chronic inflammation of the eye and possible corneal opacity. This is not a surgical procedure. In almost 90% of the cases, tacking (or gathering) is a temporary solution that may need to be repeated. The eyelids of affected Shar-Pei puppies are often tacked four times in the first year of their life.

Congenital entropion surfaces in the early weeks of life, shortly after the pups open their eyes for the first time. Experienced breeders tend to tack or gather these eyelids right then, typically without any local anesthetic. Since this is a recurring condition, these pups may be tacked again before being sold.

In my opinion, any breeders who tack eyes of pups without anesthesia are practicing cruelty to animals, and those who just deaden the local area are drug abusers. Only trained vets should perform any tacking (or gathering) of the eyelids.

B.    Surgical correction
As stated earlier, there are a multitude of eyelid syndromes that lead to inversion or entropion. No topical ointments or drops effectively heal the resulting inflammation. Only specific surgical procedures offer a long-term solution. 

An entropion operation takes time, and it is done under local anesthesia for cases with a good prognosis. Excessive eyelid tissue is removed along with muscle under the eye to shorten the eyelid laterally. This weakens the eye’s annular sphincter muscle. Suturing the resulting wounds helps strengthen the canthus (corner of the eye). A special three suture procedure creates outward tension in such a way that the lids evert or roll out away from the eyeball. An antibiotic is injected into the wound to preclude any infection.

The symptoms disappear quickly after an operation, since the cornea is no longer irritated. It is important for the animal to wear a lampshade collar for typically two weeks after the operation. The most common postoperative problem is that the eyelids do not function perfectly, especially in dogs suffering from entropion for a long time.

C.    Recommendations
It is quite evident that entropion is very common among Shar-Pei dogs. Such a high frequency of occurrence alone makes it imperative to combat this situation by banning the breeding of all dogs that test positive. One should also ensure that the sire and dam are not of the same ancestry. In other words, pay due attention to the coefficient of inbreeding of the selected pair, especially if the two show signs of specific characteristics.

I propose that responsible breeders exercising such self-control should be willing to accept a decline in the breeding pool. This will significantly curtail spreading of the hereditary diseases. This effort should, however, not lead to increased inbreeding – a proliferation technique that only irresponsible breeders would use to offset any loss in revenues.

Buyers beware! Check for this hereditary disease when buying a puppy! Make sure its eyelids are open and the eyeball is a shiny brown, without any signs of opacity/cloudiness. Insist on a written guaranty from the breeder that the ancestry of the parents was and is free of eye diseases, especially entropion!

I strongly urge the VDH and pedigree clubs like the 1st DSPC 85 e.V. and the “Club for Exotic Purebreds” (CER e.V.) to set up a register and record all necessary details of dogs with congenital entropion, such as the ancestry, breeding stock, and breeders. This register should naturally be accessible to all potential buyers.

I urge all three of these associations to compile this register jointly with the DOK, the Dortmund Regional Society for Congenital Ocular Diseases. DOK regularly examines many pedigree dogs for hereditary eye diseases. Hence, it should be possible to organize and run a program aimed at benefiting the Shar-Pei breed.

I implore all special judges and breed judges to pay particular attention in coming years to Shar-Pei dogs that have entropion and/or have undergone corrective surgery. Such animals should be banned from participating in shows or competitions, as stated clearly in the notes to the FCI Breed Standard No. 309D:

“Any Shar-Pei with physical repair of especially the flews and eyelids shall be banned from competitions.”

THE TOPIC OF THE HOUR IS OPENNESS! LET’S ALL TALK TURKEY AND STOP BEATING AROUND THE BUSH!

This is an open call to all owners with Shar-Pei dogs suffering from entropion to help advance the state of awareness & knowledge on this hereditary disease!

DO NOT MAINTAIN SILENCE!

Please contact me about personal experiences with your Shar-Pei and inform us of your dog’s ancestry and the name of its breeder.

Hanspeter Kobold
Bremen, December 2007

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