Article - What is EDS?

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WHAT IS EHLERS-DANLOS SYNDROME?

Ehlers-Danlos syndrome (EDS) is a group of hereditary connective tissue

disorders characterized by defects of the major structural protein in the

body (collagen). Collagen, a tough, fibrous protein, plays an essential role

in " holding together, " strengthening, and providing elasticity to bodily

cells and tissues. Due to defects of collagen, primary EDS symptoms and

findings include abnormally flexible, loose joints (articular hypermobility)

that may easily become dislocated; unusually loose, thin, " stretchy "

(elastic) skin; and excessive fragility of the skin, blood vessels, and

other bodily tissues and membranes.

The different types of EDS were originally categorized in a classification

system that used Roman numerals (e.g., EDS I to EDS XI), based upon each

form's associated symptoms and findings (clinical evidence) and underlying

cause. A revised, simplified classification system (revised nosology) has

since been described in the medical literature that categorizes EDS into six

major subtypes, based upon clinical evidence, underlying biochemical

defects, and mode of inheritance.

Each subtype of EDS is a distinct hereditary disorder that may affect

individuals within certain families (kindreds). In other words, parents with

one subtype of EDS will not have children with another EDS subtype.

Depending upon the specific subtype present, Ehlers-Danlos syndrome is

usually transmitted as an autosomal dominant or autosomal recessive trait.

Symptoms

The symptoms and findings associated with Ehlers-Danlos syndrome (EDS) may

vary greatly in range and severity from case to case, depending upon the

specific form of the disorder present and other factors. However, the

primary findings associated with EDS typically include abnormal " looseness "

(laxity) and excessive extension (hyperextension) of joints; susceptibility

to partial or complete joint dislocations; chronic joint pain; a tendency to

develop degenerative joint disease (osteoarthritis) at an early age;

unusually loose, thin, elastic skin; and excessive fragility of the skin,

blood vessels, and other bodily tissues and membranes. Due to tissue

fragility, affected individuals may easily bruise; experience prolonged

bleeding (hemorrhaging) after trauma; have poor wound healing; develop

" parchment-like, " thin scarring; and/or have other associated abnormalities.

In many individuals with EDS, associated symptoms and findings may become

apparent during childhood. More rarely, depending upon the specific disorder

subtype present, certain abnormalities may be apparent beginning at birth

(congenital). In addition, in other individuals, such as those with mild

disease manifestations, the disorder may not be recognized until adulthood.

The different forms of EDS were formally classified in the 1980s using a

Roman numeral system. This categorization identified at least 10 major forms

of the disorder based upon genetic and biochemical abnormalities as well as

associated symptoms and findings. However, a simplified, revised, updated

classification system has since been published in the medical literature

that classifies EDS into six primary subtypes as well as some other forms of

EDS, based upon the specific underlying biochemical cause, mode of

inheritance, major and minor symptoms, and physical findings. The revised

classification system serves to further differentiate between the various

forms of the disorder as well as some related disorders.

The original classification system differentiates between severe and mild

forms of classic EDS (EDS I and II). In the revised categorization, EDS I

and II are reclassified as one subtype, known as EDS classical type.

According to reports in the medical literature, in individuals with this

subtype, associated skin abnormalities may vary greatly, ranging from mild,

moderate, to severe in certain affected families (kindreds). EDS classical

type may be characterized by excessive laxity and extension of the joints

(hypermobility); susceptibility to recurrent sprains and dislocations of

certain joints, such as the knees and shoulders; abnormally increased

elasticity and extension (hyperextensibility) of the skin; and tissue

fragility, potentially leading to degeneration or " splitting " of the skin,

abnormal healing of skin wounds, and characteristic, thin, " parchment- " or

" paper-like " (papyraceous) scarring that often becomes discolored and

widened. Such scarring may occur primarily over certain prominent bony areas

(pressure points), such as the shins, knees, elbows, and forehead. In

individuals with EDS classical type, additional findings may include the

formation of relatively small, fleshy, tumor-like skin growths (molluscoid

pseudotumors) and/or hard, round, movable lumps (calcified spheroids) under

the skin; unusually " velvety " skin; diminished muscle tone (hypotonia);

and/or flat feet (pes planus). EDS classical type may also be characterized

by easy bruisability, often occurring in the same areas; abnormal

displacement (prolapse) of certain organs due to tissue fragility, such as

protrusion of part of the stomach upward through an opening in the diaphragm

(hiatal hernia); and/or an increased risk of certain complications after

surgical procedures. For example, postsurgical complications may include

protrusion of certain organs through weak areas in surrounding membranes,

muscles, or other tissues (postsurgical hernias). In addition, some

individuals with this subtype may have a deformity of one of the heart

valves (mitral valve prolapse), allowing blood to leak backwards into the

left upper chamber of the heart (mitral insufficiency), and/or, more rarely,

abnormal widening (dilatation) of a region of the aorta, the major blood

vessel of the body.

EDS hypermobility type was formerly classified as EDS III or benign

hypermobility syndrome. This form of the disorder is primarily characterized

by generalized, excessive extension (hypermobility) of the large and small

joints. Additional findings may include abnormally increased skin

elasticity, an unusually smooth or " velvet-like " consistency of the skin,

and/or easy bruising. Skin abnormalities and bruising susceptibility may be

extremely variable from case to case. Some individuals with EDS

hypermobility type may develop chronic, potentially disabling joint pain and

be prone to recurrent dislocations, particularly of the knee, shoulder, and

jaw (i.e., temporomandibular) joints.

EDS vascular type (formerly EDS IV or EDS arterial-ecchymotic type) is

primarily characterized by unusually thin, transparent skin with prominent

underlying veins, particularly in the chest and abdominal areas; a

susceptibility to severe bruising from minor trauma; and tissue fragility,

potentially resulting in spontaneous rupture of certain membranes and

tissues. For example, affected individuals may be prone to spontaneous

rupture of certain mid-sized or large arteries or the intestine (bowel),

leading to life-threatening complications. Because acute pain in the

abdominal or flank area may indicate possible arterial or intestinal

rupture, such symptoms require immediate, emergency medical attention.

Individuals with EDS vascular type may also be prone to developing abnormal

channels between certain arteries and veins (arteriovenous fistula, e.g.,

carotid-cavernous sinus fistula) and have an increased risk of weakening of

arterial walls and associated bulging of certain arteries (aneurysms), such

as those supplying the head and neck (carotid arteries) and within the skull

(intracranial). Aneurysms may be prone to rupturing, potentially resulting

in life-threatening complications. Females with EDS vascular type may also

be at risk for arterial bleeding and rupture of the uterus during pregnancy

as well as vaginal tearing, uterine rupture, and/or other complications

during delivery. In addition, affected individuals may be prone to

experiencing certain complications during and after surgical procedures,

such as separation of the layers of a surgical wound (dehiscence).

Individuals with EDS vascular type may also have abnormally decreased levels

of fatty tissue under skin layers (subcutaneous adipose tissue) of the

hands, arms, legs, feet, and face. As a result, some affected individuals

may have a characteristic facial appearance, including thin lips; a thin,

pinched nose; relatively large, prominent eyes; hollow cheeks; and tight,

lobeless ears. In addition, skin of the hands and feet may appear

prematurely aged (acrogeria). Additional symptoms and findings associated

with this EDS subtype may include a deformity in which the foot is twisted

out of position at birth (clubfoot); hypermobility that may be limited to

joints of the fingers and toes (digits); the early onset of varicose veins,

which are unusually widened, twisted veins visible under the skin; and

spontaneous rupture of muscles and tendons. In addition, some with this EDS

subtype may be susceptible to abnormal accumulations of air and blood in the

chest cavity (pneumohemothorax) and/or associated collapse of the lungs

(pneumothorax).

In individuals with EDS kyphoscoliosis type (formerly EDS VI), certain

symptoms and findings may be apparent at birth (congenital). These include

abnormal sideways curvature of the spine (congenital scoliosis) that becomes

progressively severe; diminished muscle tone (hypotonia); and generalized,

excessive extension and looseness (laxity) of the joints. In children with

the disorder, severe hypotonia may cause delays in the acquisition of

certain motor skills, and affected adults may lose the ability to walk by

the second or third decade of life. Additional findings associated with EDS

kyphoscoliosis type may include easy bruising, tissue fragility and

associated degenerative (atrophic) scarring of the skin, a risk of

spontaneous arterial rupture, abnormally reduced bone mass (osteopenia), and

unusually small corneas (microcornea). In addition, because the opaque,

inelastic membrane covering the eyeballs (sclera) may be unusually fragile,

minor trauma may result in rupture of the sclera, rupture of the transparent

region in the front of the eyes (cornea), and/or detachment of the

nerve-rich membrane in the back of the eyes (retina).

EDS arthrochalasia type (formerly EDS VII, Autosomal Dominant [EDS VIIA and

VIIB]) is primarily characterized by dislocation of the hips at birth

(congenital hip dislocation); severe, generalized, excessive extension of

the joints (hypermobility); and recurrent partial dislocations of affected

joints (subluxations), such as those of the elbows, knees, hips, and feet.

Affected individuals may also have diminished muscle tone (hypotonia),

abnormal front-to-back and sideways curvature of the spine (kyphoscoliosis),

and mildly reduced bone mass (osteopenia). Additional findings typically

include abnormally increased elasticity and extension of the skin

(hyperextensibility), easy bruising, and tissue fragility, with associated

scarring of the skin.

Primary symptoms and findings associated with EDS dermatosparaxis type

(formerly EDS VII, Autosomal Recessive [EDS VIIC]) include severe skin

fragility; soft, sagging, redundant skin; and extensive bruising. In some

cases, certain tissues or organs may abnormally protrude through a weak area

in a surrounding membrane, muscle, or other tissue (e.g., umbilical hernia,

inguinal hernia).

In addition to the six primary EDS subtypes described above, there are some

additional, rare forms of EDS. For example, X-linked EDS (formerly EDS Type

V) has been described in individuals within at least one family (kindred).

Associated symptoms and findings include easy bruising, hyperextensible

skin, minor skin fragility, and deformity of one of the heart valves (mitral

valve prolapse), allowing blood to leak backwards into the left upper

chamber of the heart (mitral insufficiency). Because this form of EDS is

transmitted as an X-linked recessive trait, it is fully expressed in males

only. (For more information on X-linked inheritance, please see the " Causes "

section of this report below.)

The symptoms and findings associated with EDS periodontosis type (formerly

EDS Type VIII) are considered similar to those seen in EDS classical type.

Additional findings typically include disease of the tissues surrounding and

supporting the teeth (periodontal disease), potentially resulting in

premature tooth loss.

EDS progeroid form, another rare variant of the disorder, is characterized

by loose, elastic skin; hypermobile joints; slow wound healing; degenerative

(atrophic) skin scars; and reduced bone mass (osteopenia). Additional

findings may include delayed mental development, short stature, and a

prematurely aged appearance (progeroid appearance) due to premature

wrinkling of facial skin; scanty scalp hair, eyebrows, and eyelashes; and

other findings.

According to reports in the literature, some individuals may be affected by

additional, rare subtypes of EDS, which are currently referred to as EDS

unspecified forms. Such subtypes are characterized by joint hypermobility,

loose, elastic skin, and other symptoms and findings commonly seen in

individuals with the disorder.

The EDS subtype originally referred to as EDS type X (or EDS

dysfibronectinemic type) is extremely rare, affecting only one reported

family (kindred). This subtype is characterized by abnormally extensible,

loose joints; thin, elastic skin; and abnormalities of the specialized blood

cells that play an essential role in blood clotting (platelets). Associated

findings typically include the appearance of tiny purplish or reddish spots

on the skin due to abnormal bleeding within or under skin layers (petechiae)

and/or pinkish, depressed scar-like skin lesions that may later become white

(striae distensae). These lesions, which may occur on the thighs, abdomen,

buttocks, and breasts, develop due to weakening of elastic tissues.

Some subtypes of EDS included within the original disease classification

have been redefined and are no longer part of the original nor the revised

EDS categorization. For example, what was previously known as EDS type IX

has been redefined and is now termed occipital horn syndrome. In addition,

EDS type XI is currently known as familial hypermobility syndrome. For more

information on these disorders, please see the " Related Disorders " section

of this report below.

Causes

Most forms of Ehlers-Danlos syndrome (EDS) are transmitted as an autosomal

dominant or autosomal recessive trait. Each EDS subtype is a distinct

hereditary disorder that may affect individuals within certain families

(kindreds). In other words, individuals with one subtype of EDS will not

have children with another EDS subtype.

The disease genes that cause some forms of EDS have been mapped to

particular chromosomes. Although the specific underlying cause of EDS is not

known for all EDS subtypes, the disorder is known to result from various

defects of collagen, the major structural protein in the body. Collagen is

the tough, fibrous protein that serves to provide elasticity to and

strengthen bodily cells and tissues.

EDS classical type is inherited as an autosomal dominant trait. Human traits

including the classic genetic diseases, are the product of the interaction

of two genes for that condition, one received from the father and one from

the mother.

In dominant disorders, a single copy of the disease gene (received from

either the mother or father) will be expressed " dominating " the other normal

gene and resulting in the appearance of the disease. The risk of

transmitting the disorder from affected parent to offspring is 50 percent

for each pregnancy regardless of the sex of the resulting child.

According to researchers, in at least some affected individuals, EDS

classical type may result from abnormal changes (mutations) in the gene

known as collagen type V, alpha-1 (COL5A1), which has been mapped to the

long arm (q) of chromosome 9 (9q34.2-q34.3), or the gene collagen type V,

alpha-2 (COL5A2), located on the long arm of chromosome 2 (2q31).

Chromosomes are found in the nucleus of all body cells. They carry the

genetic characteristics of each individual. Pairs of human chromosomes are

numbered from 1 through 22, with an unequal 23rd pair of X and Y chromosomes

for males and two X chromosomes for females. Each chromosome has a short arm

designated as " p " and a long arm identified by the letter " q. " Chromosomes

are further subdivided into bands that are numbered.

EDS hypermobility type is transmitted as an autosomal dominant trait. A

specific underlying collagen defect responsible for this form of the

disorder has not been identified. EDS vascular type is also inherited as an

autosomal dominant trait. This subtype is caused by abnormal changes

(mutations) of the gene known as collagen type III, alpha-1 (COL3A1), which

is located on the long arm of chromosome 2 (2q31).

EDS kyphoscoliosis type is inherited as an autosomal recessive trait. In

recessive disorders, the condition does not appear unless a person inherits

the same defective gene for the same trait from each parent. If an

individual receives one normal gene and one gene for the disease, the person

will be a carrier for the disease, but usually will not show symptoms. The

risk of transmitting the disease to the children of a couple, both of whom

are carriers for a recessive disorder, is 25 percent. Fifty percent of their

children risk being carriers of the disease, but generally will not show

symptoms of the disorder. Twenty-five percent of their children may receive

both normal genes, one from each parent, and will be genetically normal (for

that particular trait). The risk is the same for each pregnancy.

In some affected individuals, the kyphoscoliosis subtype is thought to

result from mutations of a gene (called " procollagen-lysine, 2-oxoglutarate

5-dioxygenase " [PLOD]) that encodes a collagen-modifying enzyme known as

lysyl hydroxylase. Deficiency of this enzyme may result in the symptoms and

findings associated with this form of EDS. The PLOD gene has been mapped to

the short arm of chromosome 1 (1p36.3-p36.2).

EDS arthrochalasia type is transmitted as an autosomal dominant trait. This

subtype may result from mutations of the gene known as collagen type I,

alpha-1 (COL1A1), which has been mapped to the long arm of chromosome 17

(17q21.31-q22.05), or the gene called collagen type I, alpha-2 (COL1A2),

located on the long arm of chromosome 7 (7q22.1).

EDS dermatosparaxis type has autosomal recessive inheritance. This EDS

subtype is thought to be caused by mutations of a gene or genes that encode

a collagen-modifying enzyme known as procollagen I N-terminal peptidase.

As discussed above (see " Symptoms " ), in addition to the six primary EDS

subtypes, there are some other, rare forms of EDS. The rare subtype known as

X-linked EDS is, as its name indicates, transmitted as an X-linked trait.

X-linked recessive disorders are conditions that are coded on the X

chromosome. Females have two X chromosomes, but males have one X chromosome

and one Y chromosome. Therefore, in females, disease traits on the X

chromosome may be masked by the normal gene on the other X chromosome. Since

males only have one X chromosome, if they inherit a gene for a disease

present on the X, it will be expressed. Males with X-linked disorders

transmit the gene to all their daughters, who are carriers, but never to

their sons. Females who are carriers of an X-linked disorder have a 50

percent risk of transmitting the carrier condition to their daughters and a

50 percent risk of transmitting the disease to their sons. In some females

who inherit a single copy of a disease gene for an X-linked recessive trait

(heterozygotes), disease traits on the X chromosome may not always be masked

by the normal gene on the other X chromosome. Therefore, it is possible that

some female carriers of the disease gene may exhibit some of the symptoms

associated with the disorder; however, according to reports in the medical

literature, to date, no female carriers of the disease gene for X-linked EDS

have experienced symptoms (asymptomatic carriers).

EDS periodontosis type, another rare subtype, has autosomal dominant

inheritance. EDS progeroid form, which is thought to be inherited as an

autosomal dominant trait, may be caused by gene mutations that result in

deficiency of a particular enzyme (XGPT deficiency). The subtype known as

EDS type X (or EDS dysfibronectinemic type), which has been described in

several siblings in one affected family (kindred), is thought to have

autosomal recessive inheritance.

According to reports in the medical literature, there appear to be

additional, rare subtypes of EDS that may have autosomal dominant or

autosomal recessive inheritance (e.g., EDS, autosomal dominant, unspecified

type; EDS, autosomal recessive, unspecified type).

Affected Populations

Males and females are equally affected by autosomal dominant and autosomal

recessive forms of Ehlers-Danlos syndrome (EDS). The X-linked subtype of EDS

is fully expressed in males only. It is possible that some females who carry

a single copy of the disease gene (heterozygotes) for X-linked EDS may

develop some symptoms; however, according to the medical literature, reports

indicate that no female carriers have developed associated symptoms

(asymptomatic).

In many individuals with EDS, associated symptoms and findings may become

apparent during childhood. However, depending upon the form of the disorder

present, some abnormalities may be apparent at birth. In other cases, such

as those with relatively mild disease manifestations, EDS may not be

recognized until adulthood.

Reported estimates concerning the disorder's overall frequency have varied,

ranging from one in 5,000 to 10,000 births. However, because those with mild

joint and skin manifestations may not seek medical attention or remain

undiagnosed, it is difficult to determine the true frequency of EDS in the

general population. EDS classical, hypermobility, and vascular types account

for most reported cases of the disorder. EDS kyphoscoliosis, arthrochalasia,

dermatosparaxis, and other subtypes are considered much less common. For

example, some forms of EDS (e.g., EDS type X or EDS dysfibronectinemic type)

may have only been reported in individuals within one affected family

(kindred).

The first published accounts of Ehlers-Danlos syndrome occurred in 1892. The

syndrome was furthered clarified by Ehlers in 1901 and Danlos in 1908.

Related Disorders

Some of the symptoms of the following disorders may be similar to those seen

in Ehlers-Danlos syndrome (EDS). Comparisons may be useful for a

differential diagnosis:

Occipital horn syndrome (OHS), also known as X-linked cutis laxa, is a rare

disorder that was formerly classified as a subtype of EDS (EDS type IX). The

disorder has been recategorized with other connective tissue diseases that

result from defects of copper metabolism. OHS is characterized by abnormally

loose skin that tends to hang in folds (cutis laxa); abnormalities of the

muscular organ that stores urine (bladder); the formation of " horn-like "

bony protuberances on both sides of the back of the skull (occipital horns)

and other skeletal abnormalities; excessive extension (hypermobility) of the

fingers and toes; and limited extension of the elbows and knees. In some

cases, affected individuals may have a prematurely aged facial appearance, a

hooked nose, sagging cheeks, downwardly slanting eyelid folds (palpebral

fissures), and/or other facial abnormalities. The disorder may also be

characterized by mild mental retardation. OHS is transmitted as an X-linked

recessive trait and is caused by deficiency of an enzyme (lysyl oxidase

deficiency) that results in abnormalities of copper metabolism.

Familial hypermobility syndrome was also formerly categorized as a subtype

of EDS (EDS type XI). However, researchers since suggested that the

designation of EDS be reserved for the association of joint hypermobility

with distinctive skin changes, resulting in the disorder's separate

categorization. Familial hypermobility syndrome is characterized by

looseness (laxity) and excessive extension of the joints; recurrent

dislocation of certain joints, such as those of the shoulders and knees;

and, in some cases, dislocation of the hip joints at birth (congenital).

This disorder is transmitted as an autosomal dominant trait.

There are additional disorders that may be characterized by joint

hypermobility, skin changes, and/or other abnormalities similar to those

associated with EDS, such as other forms of cutis laxa or other related

disorders.

Standard Therapies

Diagnosis

Ehlers-Danlos syndrome (EDS) is diagnosed based upon a thorough clinical

evaluation, characteristic physical findings, a careful patient and family

history, and specialized tests.

Specialized diagnostic laboratory tests may be available for certain EDS

subtypes in which the specific underlying biochemical defect has been

identified and characterized. In addition, in some families (kindreds)

affected by a particular EDS subtype who have identified gene mutations,

precise genetic testing may be available that enables diagnosis before or

after birth (prenatal or postnatal diagnosis). However, it is possible that

such testing may only be accessible through research laboratories with a

special interest in EDS.

In addition, in some cases, diagnostic testing includes the removal (biopsy)

and microscopic examination (e.g., electron microscopy) of small samples of

skin tissue. Such examination may reveal characteristic abnormalities in

collagen structure seen in certain EDS subtypes.

The clinical evaluation of individuals with suspected or diagnosed EDS

typically includes assessments to detect and determine the extent of skin

and joint hyperextensibility. For example, physicians may measure skin

hyperextensibility by carefully pulling up skin at a neutral site until the

point of resistance, and joint hyperextensibility may be evaluated using a

clinical rating scale (i.e., Beighton scale). In addition, in some cases,

specialized imaging tests, such as computerized tomography (CT) scanning,

magnetic resonance imaging (MRI), and echocardiography, are used to detect

and characterize mitral valve prolapse and aortic dilatation. During a CT

scan, a computer and x-rays create a film showing cross-sectional images of

certain bodily structures. MRI uses a magnetic field to create

cross-sectional images of particular organs and tissues. During an

echocardiogram, sound waves are directed toward the heart, enabling

physicians to study cardiac function and motion.

In addition, in some individuals with EDS, specialized x-ray studies may be

used to characterize round, movable lumps (calcified spheroids) under the

skin; to detect and determine the extent of abnormal spinal curvature

(scoliosis and/or kyphosis) and/or reduced bone mass (ostepenia) (e.g., in

those with EDS kyphoscoliosis or arthrochalasia types); and/or to confirm

and characterize certain other abnormalities.

In some cases, physicians may recommend that individuals with EDS vascular

type be monitored with appropriate noninvasive imaging techniques (e.g., CT

scanning, MRI, ultrasonography) to ensure early detection of arterial

changes (e.g., aneurysms) that may result in spontaneous arterial rupture

and potentially life-threatening complications. Angiography, a diagnostic

test that is often used to detect aneurysms, must be avoided, since this

technique may be hazardous to individuals with EDS, particularly those with

EDS vascular type. During angiography, a substance that is impenetrable by

x-rays (contrast medium) is injected into an artery via a flexible plastic

tube (catheter) and an x-ray series is taken that visualizes blood flow

through certain blood vessels.

Treatment

The treatment of individuals with EDS is directed toward the specific

symptoms that are apparent in each individual. Treatment may require the

coordinated efforts of a team of specialists who may need to systematically

and comprehensively plan an affected individual's treatment. Such

specialists may include pediatricians or internists; specialists who

diagnose and treat disorders of the skeleton, joints, muscles, and related

tissues (orthopedists); physicians who diagnose and treatment skin disorders

(dermatologists); specialists who diagnose and treat connective tissue

diseases (rheumatologists); surgeons; physical and occupational therapists;

and other health care professionals.

In individuals with EDS, the use of special braces may help to stabilize

affected joints. In addition, specialized physical and occupational therapy

techniques may help to preserve the joints and strengthen muscles. Parents

of young children with the disorder and affected individuals should also

take necessary precautions to prevent injuries and trauma, such as may occur

during contact sports. Wearing protective clothing and special padding over

pressure points (e.g., shins, knees, elbows) may be beneficial.

Females with EDS vascular type should be counseled concerning the increased

risk of certain complications during pregnancy and delivery and the need for

meticulous obstetric care. In addition, appropriate precautions and careful

monitoring are essential before, during, and after dental or surgical

procedures. Because fragile tissues and stitched (i.e., sutured) incisions

or wounds may easily tear during or after surgery, unnecessary surgical

procedures should be avoided. Accordingly, when surgery is necessary in

individuals with EDS, specific surgical approaches require careful

evaluation.

Genetic counseling will be of benefit for affected individuals and family

members. Other treatment for individuals with EDS is symptomatic and

supportive.