Posted by Mandy on September 07, 1998 at 13:33:15:
In Reply to: This is the Hypermobility Syndrome posted by Stefan Back on March 17, 1998 at 13:26:40:
I would like to know more about how Hypermobility and/or Ehlers Danlos affect a woman's body from a gynecological point of view i.e. pelvic floor problems and uterine prolapses. Are there any gynecologists in the USA (or elsewhere) who specialize in correcting prolapses in women whose tissues are fragile and where stitches are prone to tearing out? : HYPERMOBILITY SYNDROME
: Thank You in advance
Mandy
: by Rodney Grahame • Taken from Rheumatology by Klippel and Dieppe
:
: CLINICAL FEATURES
:
: • Range of traumatic and overuse lesions (traction injuries, synovitis,
: rotator cuff lesions, etc.).
:
: • Hyperextensible, thin, soft skin.
:
: • Joint instability effects.
:
: • Stress fractures.
:
: • Some patients develop a chronic arthritis, either low-gradeinflammatory
: synovitis or osteoarthritis.
:
:
: Generalized ligamentous laxity, the prerequisite of joint hypermobility, is
: seen in a substantial proportion (perhaps 10%) of healthy individuals (varying
: according to methodology and tothe age, sex and ethnic origin of the
: population studied), the overwhelming majority of whom probably suffer no ill
: effects.
:
: Hypermobility syndrome (HMS), as seen in clinical practice, is a common
: finding. A diagnostic survey of 9275 new referrals to one large general
: rheumatology clinic revealed that HMS was more frequently diagnosed than
: ankylosing spondylitis, crystal synovitis or psoriatic arthritis, comprising
: 3.25% of all female and 0.63% of all male referrals.
:
: These patients present with a wide variety of readily identifiable traumatic
: and overuse lesions, such as traction injuries at tendon or ligament
: insertions, joint or tendon sheath synovitis, chondromalacia
: patellae, rotator cuff lesions, or back pain due to soft tissue injury or disc
: prolapse.
:
: Others suffer the effects of joint instability, such as flat feet, recurrent
: dislocation or subluxation notably of the shoulder, patella,
: metacarpophalangeal joints (MCP’s) or temporomandibular joints. Others develop
: a chronic arthritis either a low-grade inflammatory synovitis of traumatic
: origin or osteoarthritis, which is held by many authorities to be a direct
: complication of HMS.
:
: What sets the HMS patient apart from others at the rheumatology clinic is the
: profusion and spectrum of common lesions occuring in the same individual and
: often spanning his (or more commonly her) whole lifetime.
:
: More difficult to explain in such patients is the frequently encountered
: arthralgia or myalgia in the absence of any demonstrable clinical abnormality.
: Clinicians unfamiliar with the syndrome, or those who fail to observe the
: hypermobility, are tempted to discount the symptoms or to ascribe them to
: neurosis.
:
: The hapless patient, frustrated by this lack of understanding and help from
: doctors, and often unable to lead a normal life, resorts to the dubious
: benefits of alternative medicine. Not surprisingly, features of depression are
: commonplace.
:
: Organs and tissues, which rely for their structural integrity on the tensile
: strength of normal collagen may also become disordered in hypermobile
: subjects. The skin may be thin, soft, hyperextensible and develop striae. But
: this is also typical for Ehlers-Danlos (EDS) and Marfan syndromes and
: osteogenesis imperfecta (OI), with which HMS shares a number of
: common features.
:
: An association between mitral valve prolapse (MVP) and the HMS has been
: reported in controlled studies. Weakness of the musculotendinous supporting
: structures of the anterior abdominal wall and pelvic floor no
: doubt explains the reported increased findings of abdominalhernia and of both
: rectal and uterine prolapse in HMS subjects. Bone fragility may also be
: present, resulting in an increased liability to fracture. Partes
: interarticulares of the lumbar spine are particularly common, the latter
: constituting additional potential sources of low back pain in HMS.
:
: Differential diagnosis
:
: The recognition of joint hypermobility rests on the ability of the person to
: perform a series of passive joint maneuvers. The system that has stood the
: test of time and that has gained virtual universal acceptance is the 9-point
: scale of Beighton 10. The diagnosis is commonly missed for the simple reason
: that clinicians, trained to look for loss of range of joint and spinal motion,
: fail to recognize an increase in range.
:
: Hypermobility syndrome has to be distinguished from the less benign heritable
: connective tissue disorders such as Marfan and Ehlers-Danlos syndromes (EDS)
: and Osteogenesis Imperfecta (OI), with which it shares a number of common
: features. This is not always easy. However, echocardiography, ophthalmic
: examination and a detailed genetic work-up are invaluable in helping to define
: the phenotype in individual families. A panel of 21 expert geneticists from 10
: countries has recently drawn up an agreed comprehensive classification of the
: many syndromes, and variants of syndromes that have been described.
: Unfortunately, the HMS patients described above as so frequently displaying
: overlap features (marfanoid habitus, MVP, skin hyperextensibility and joint
: laxity) do not fit comfortably into the Berlin nosology.
:
: Neither of the two nearest designations, EDS III (hypermobile type) nor
: familial articular hypermobility syndrome (FAHS) are appropriate for this
: group. The skin and joint changes seen in EDS III are more florid; whilst, in
: FAHS, skin involvement and MVP are reportedly absent.