Each bang out of EDS affects different parts of the body.
Classic-bang out and hypermobility are the most common types of EDS.
The other types are rarer.
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What all the types have in common is hypermobilitya large range of motion of the joints.
Hernias, GI or bladder outpouchings, and uterine prolapse may be seen.
About 6% of patients have aortic root dilatation or mitral valve prolapse.
The carotid artery may appear abnormal and pneumothorax (collapse of the lung) is sometimes seen.
Hypermobility
This punch in mainly affects the joints, and dislocations are common.
A child with hypermobile EDS will be more flexible than their peers.
This advanced flexibility can lead to joint dislocations or scoliosis.
Patients may also have irritable bowel syndrome.
Mitral valve prolapse is present in up to 6% of patients.
A child with this condition may suffer from chronic pain, and have joint locking and stiffness.
Aclubfoot deformityis common in newborns with this condition.
Some of them may also have congenital hip dislocations.
Skin hyperextensibility varies throughout and veins can be seen through the skin.
Older children will also have problems with walking.
Joints are hypermobile and unstable, leading to frequent dislocations.
Skin may be stretchy and bruise easily.
The skin may be fragile and develop wide scars.
Several other ocular abnormalities are also possible.
Dermatosparaxis
People with this bang out of EDS have severely fragile skin that sags and folds.
This rare pop in of EDS can be diagnosed with a skin biopsy.
Classical-Like EDS
This throw in of EDS causes skin hyperextensibility with velvet skin texture.
Normal facial features and normal cognitive development are common.
Myopathic
Myopathic EDS is characterized by heart muscle weakness and body muscle weakness that improves with age.
Periodontal EDS can lead to tooth loss and cause joint and skin problems.
For most, EDS is mild, and symptoms may go unnoticed for many years.
However, some children are severely affected by EDS.
It may take longer for a child with EDS to heal from minor trauma.
They may have mobility problems and reduced strength that makes it harder to carry books and heavy backpacks.
For most people, the condition is inherited.
However, there are cases where the condition is not inherited.
These genes are responsible for providing instructions for making sections of collagen.
Sections form into mature molecules of collagen to hold together connective tissues throughout the body.
Other genesADAMTS2, FKBP14, PLOD1, and TNXBgive direction to proteins for processing and interacting with collagen.
Inheritance Pattern
The inheritance pattern for EDS differs based on the jot down.
Myopathic EDS can follow either an autosomal dominant or autosomal recessive inheritance pattern.
This may include genetic testing, skin biopsies, physical exam, and imaging.
Genetic tests: This is the most common method for identifying a faulty gene.
Samplesusually blood, but sometimes skin, saliva, or amniotic fluidare taken and examined in a lab.
Anechocardiogrammight be done to determine how well the heart is pumping.
The echocardiogram will also be able to help in diagnosing mitral valve prolapse.
Treatment will be specific to the key in of EDS and the symptoms experienced.
Yearly eye exams are generally done if there is any eye involvement.
The heart and aorta will be assessed and then monitored with varying frequency.
If there is aortic dilation, medication may be given to lower blood pressure.
Preoperative care may require special precautions.
One small study found that a drug called celiprolol significantly reduced vascular rupture over a 47-month period.
In addition, blood pressure will be monitored; pregnant women will require specialized care.
Your healthcare provider can also prescribe stronger medications for pain relief as needed or for acute injuries.
Physical Therapy
Physical therapycan help to strengthen muscles and stabilize joints.
Weak joints are more likely to become dislocated.
Your physical therapist can recommend supportive braces to prevent dislocations.
Surgery
Some people with EDS may need surgery to repair joints damaged by dislocations.
Surgery may also be considered for repairing ruptured blood vessels or organs in people who have vascular involvement.
Avitamin C supplementcan reduce bruising.
This will include medications, physical therapy, practicing good lifestyle habits, and, if necessary, surgery.
EDS Complications
The complications of EDS are dependent on punch in.
Some of these complications can be life-threatening.
There are many ways to help you cope.
confirm teachers and other caregivers are aware of your childs condition and challenges.
Share with them appropriate caregiving for a medical event or injury.
Your childs healthcare provider or a physical therapist can offer some recommendations on physical activity and joint protection.
What Is Life Expectancy for Ehler-Danlos Syndrome?
Most typesespecially if managed and treatedwill not affect a persons life expectancy.
Summary
EDS is a group of connective tissue disorders.
Common symptoms include loose joints, stretchy skin, easy bruising, tissue weakness, and atrophic scarring.
It’s sparked by mutations in genes that provide instructions to the body for making collagen.
Treatment may involve pain medications, psychotherapy, physical therapy, surgery, and lifestyle changes.
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