Tuesday, October 8, 2013

Spina Bifida: It Doesn't Just Affect Humans

Spina bifida is a birth defect involving abnormal prenatal development of one or more of a dog’s vertebrae, which are the bones of the spine. What causes spina bifida isn't well-understood, although there probably is a strong genetic component. Malnutrition and ingestion of toxins during pregnancy may play a role. The vertebrae of dogs with this condition aren't properly fused, which interferes with the function of the spine and the shock-absorbing intervertebral disks. This defect allows the spinal cord to squeeze out through gaps in the spine, causing a variety of symptoms. Owners usually notice signs when affected puppies try to walk, between one and two weeks of age. They show hind-end weakness, poor muscle tone, incontinence, incoordination and abnormal use of their tail. There may be a draining mass or skin dimpling on the dog’s back, over the site of the spinal defect. Fortunately, spina bifida doesn't worsen with time. 
By definition, spina bifida is a congenital disorder, meaning that it is present at birth. Spina bifida is not progressive and does not worsen with time. Signs of this condition usually are first noticed when affected puppies try to walk, between one and two weeks of age. Sometimes, dogs with spina bifida never develop observable symptoms.
As mentioned above, some dogs born with spina bifida never show signs of their condition. Owners of dogs that do develop clinical signs may notice one or more of the following signs:
  • Rear limb paresis (weakness)
  • Slight to moderate incomplete paralysis of one or both hind legs
  • Decreased muscle tone (hind end)
  • Muscle atrophy (hind end)
  • Ataxia (lack of coordination)
  • Fecal incontinence
  • Urinary incontinence
  • Loss of sensation in the perineal area (the region between the tail and the genitalia)
  • Decreased anal sphincter tone
  • Decreased ability to use the tail
In rare cases, there may be a draining mass on the dog’s back, towards its tail. Other possible signs are dimpling of the skin and/or abnormal direction of hair growth over the affected area of the animal’s spine.

Dogs at Increased Risk

Spina bifida can occur in any dog of any breed or mixed breed and of either sex. However, it is most commonly seen in English Bulldogs (and in Manx cats), which is why a hereditary component to this condition is suspected.

Diagnostic Procedures

If the spinal cord is unaffected by the defective bone fusion that is called spina bifida, the dog probably will never have symptoms of neurological abnormalities, and the condition actually may never be diagnosed. However, when the spinal cord pushes out through the gap in the abnormal vertebrae, the dog’s rear-end ambulation and control of its urinary and rectal function typically are adversely affected.
A veterinarian should be consulted when a young puppy is not able to walk normally as compared with the abilities of its littermates. The veterinarian will take a history of the puppy’s environment and development and will perform thorough physical and neurological examinations. She may or may not be able to localize which areas of the spine are affected based upon the results of those examinations. Additionally, a miminum data base of blood work (a complete blood count and serum biochemistry profile) and a urinalysis are almost always advised to assess the puppy’s overall health. This is especially important before heavy sedation or general anesthesia is administered to facilitate a more in-depth neurological examination. An electrocardiogram (ECG) to evaluate heart health, and a thyroid panel to evaluate thyroid gland function, may also be recommended.
Spina bifida normally can be definitively diagnosed with simple radiographs (X-rays). Often, it is discovered incidentally when thoracic (chest), abdominal or hip X-rays are taken for some unrelated reason. Radiographs usually will reveal the anatomical defect in the upper parts of affected vertebrae. Computed tomography (CT scan) and magnetic resonance imaging (MRI) are available in some specialty clinics and at veterinary teaching hospitals; these can be helpful to pinpoint the precise location and the extent of bony and soft tissue abnormalities in dogs with spina bifida. A number of other advanced laboratory and electrodiagnostic tests, such as myelography and cerebrospinal fluid analysis, are available if the veterinarian thinks that they may be helpful in a given case.

Special Notes

It is important for owners to remember that spina bifida may or may not cause any discomfort or noticeable signs in affected animals. If the spinal cord does not protrude from the opening in the unfused vertebrae, the disorder may never be diagnosed and may never need any sort of treatment or medical management.

Treatment Options

The management of spine and spinal cord disorders depends upon the nature and severity of the particular abnormality. Treatment is rarely attempted for dogs with spina bifida, mainly because there is no reliable way to “treat” - or fuse - the defect in the abnormal vertebral bones. If a dog has clinical symptoms as a result of a spinal malformation, it means that the spinal cord has already been damaged. At that point, treatment is almost never available or effective.
There is no current treatment for spina bifida in domestic dogs. Attempts to correct the vertebral defects surgically have not been successful, and management with drugs, supplements or dietary modification have no meaningful impact on the condition. Puppies with spina bifida are born with one or more vertebrae that did not develop normally, leaving a gap at the uppermost side of the bone that exposes the spinal cord. The abnormal opening makes the spinal cord vulnerable to trauma and provides a pathway for it to “pop” or squeeze out of its normally protected canal. When this happens, it usually leads to significant neurological damage, because the edges of the gaps in the vertebral bones are rarely smooth and because the spinal cord is extremely fragile. Once the spinal cord is damaged, the adverse consequences are almost always irreversible. Euthanasia is often the most humane alternative for puppies that are severely affected by spina bifida.
However, if the spinal cord of an affected dog does not bulge out through the gap in the upper side of the vertebrae, and if the spinal cord and associated nerves otherwise remain undamaged, the dog may never have noticeable symptoms associated with its condition. In those cases, the animal probably will live a full, happy and pain-free life – at least, without complications of spina bifida - even without any medical treatment for the disorder.

Prognosis

The prognosis and life-expectancy for dogs born with this anatomical spine defect is quite variable. If a dog has significant spinal cord damage caused by the congenital failure of one or more of its vertebrae to fuse, and if it shows severe signs of neurological deficits, its outlook is guarded to grim. These dogs – usually young puppies at the time of diagnosis – often are in a great deal of pain and discomfort. Their inability to walk normally, combined with both fecal and urinary incontinence, probably will prevent them from living an acceptably long or good quality of life. Many caring owners opt for euthanasia when their puppies are so disabled from the disorder. On the other hand, the prognosis for dogs that have no observable symptoms of spina bifida is quite good. They probably should be restricted from engaging in vigorous athletic activities, be well-trained and be encouraged to be calm, in order to enhance their chances of avoiding future spinal cord damage if at all possible.
While spina bifida has been reported in a wide variety of dogs and, there is a high incidence of this condition in young English Bulldogs and Manx Cats. Spina Bifida has also been reported in the German Shepherd, Weimeraner, Pekingese, Dalmatian, Chinese Crested and Swedish Vallhund.

The following links are of two of my favorite animals with Spina Bifida. 

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