Hands signs in Marfan syndrome: thin fingers, long hand shape & hypermobility!
Marfan syndrome is a connective tissue disorder characterised by a tall, slender body featured with long limbs & long thin fingers. The most serious complications are the defects of the heart valves and the aorta, which could lead to an aortic rupture (due to too much stress on the aorta), which is usually fatal. However, many people who have this disorder are not aware of it – partly because Marfan syndrome typically becomes manifest only after the age of 5. But there are hand signs that have a highly reliable diagnostic value!
Marfan syndrome is featured with many typical hand characteristics, however a combination of two specific hands signs related to a long hand shape (hand signs) & hand motorics (joint hypermobility) is often enough to identify the disorder.
THE STEINBERG SIGN (a):
This test is used for the clinical evaluation of Marfan patients.
Procedure:
Instruct the patient to fold his thumb into the closed fist. This test is positive if the thumb tip extends from palm of hand (see figure a).
THE WALKER-MURDOCH SIGN (b):
This test is used for the evaluation of patients with Marfan syndrome.
Procedure:
Instruct the patient to grip his wrist with his opposite hand. If thumb and fifth finger of the hand overlap with each other, this represents a positive Walker-Murdoch sign (see figure b).
JOINT HYPERMOBILITY
How to check if a person has hypermobility? You can check this easily by doing the 5 tests that are included in the so-called ‘Beighton score‘: see figure 1.
A ‘Beighton score’ of 4 or above usually indicates hypermobility.
And if a person has the Sternberg sign + Walker-Murdoch sign + hypermobility, the chances are close to 90% that the person has Marfan syndrome.
The presence of other related hand markers such as: skin quality (hyperextensiblity), a simian crease, extra digital transverse creases, or a high positioned axial triradius provide other hand signs which are indicative for a person to have a medical diagnosis for Marfan syndrome.
Marfan hands.
Palm Reading Perspectives 
[…] The presence of other related hand markers such as: skin quality (hyperextensiblity), a simian crease, extra digital transverse creases, or a high positioned axial triradius provide other significant hand signs which are indicative for a person to have a medical test for Marfan syndrome. […]
The hand in Marfan syndrome: thin fingers, hand signs, hypermobility, skin quality & more! | Hand Facts: News about hands!
May 24, 2011 at 5:00 am
Good web page
Matthew Syracuse
May 29, 2011 at 3:55 pm
Thank you! 🙂
martijnvanmensvoort
May 29, 2011 at 4:13 pm
Nice to see that this post featured at:
http://marfan-syndrome.crossfingers.info/term/marfan+syndrome+pictures+hands
martijnvanmensvoort
June 1, 2011 at 4:34 am
Hello Hein, feel free to share more details about how you made your discovery exactly….
martijnvanmensvoort
August 17, 2011 at 12:36 pm
i just discovered i have it too.
for years i knew i could do ‘abnormal’ things that my friends couldnt.
i score a 9 on the Beighton score. and i can do the hand gestures.
although, i am not tall. i have long limbs and a small torso. i am only 5″2 and 16 years old.
bek
August 17, 2011 at 7:43 pm
Hello Bek, thank you for your report!
martijnvanmensvoort
August 17, 2011 at 7:50 pm
Just read this post. I have the EDTC on my left pinky. I also am short 5′ as are other members of my family with a diagnosis that mostly fits in the category of Ehlers Danlos syndrome hypermobility type. But I am the only one with EDTC. The genetics of hereditary disorders of connective tissue is not a cut and dried issue. Even in immediate family there are some differences in phenotype. Mostly EDSIII is diagnosed on clinical grounds. I also have features of EDSIV – ‘old looking hands’ and short stature. There is overlap between the various types. The EDTC is also found in Marfan’s syndrome. There is also a Marfanoid phenotype in EDSIII. Totally confused yet?
Marilyn Weate
February 29, 2012 at 1:52 am
I think it’s important to note that a diagnosis requires more than just these findings. These are the diagnostic criteria for Marfan syndrome: http://www.ncbi.nlm.nih.gov/books/NBK1335/#marfan.Diagnosis
GCNerd
August 22, 2011 at 12:17 am
Agreed – its really important to look at the actual criteria for diagnosing Marfan Syndrome, because these tests don’t even come close to addressing the most important two (aortic dilation and ectopia lentis). In fact, most of the hypermobility issues aren’t even included in a real Marfan diagnosis.
Martin, could you please inform us where you found this data? What medical studies have shown that having these signs makes the chances of having Marfan Syndrome 90% or more? It’s important to raise awareness, but if this information has backing from real medical data and real studies, it’s also important for your readers to see it. In the meantime, I suggest readers check out this information: http://www.marfan.org/marfan/4265/Diagnostic-Criteria
ConcernedAboutTheValidityOfThisArticle
February 21, 2012 at 1:31 am
Hello … (?),
Thank you for your question!
The 90% percentage concerns only a rough estimate (my estimate), but I can share some evidence that my estimate is actually rather ‘conservative’:
The 1997 study ‘Evolving phenotype of Marfan’s syndrome‘ reports the following for a sample of 40 Marfan children:
“The presence of arachnodactyly was defined clinically by a positive Steinberg sign when the thumb protrudes from the ulnar border of the hand when apposed across the palm20 and a positive Walker-Murdoch sign when a combination of slim wrists and long digits results in noticeable overlap of the thumb and little finger when the wrist is grasped.21 This was found in all except three children and was identifiable from birth in five.
thirty one children had pes planus which in most was associated with joint hypermobility with a Beighton score of greater than 5/9.”
This implicates:
– the combination of the Steinberg sign and the Walker-Murdoch sign was found in 37 out of 40 Marfan children (=92%);
– and additinonally in (almost) 30 of the 40 children (=75%) a Beighton score higher than
five
was found.
Combining these two figures confirms that my 90% estimate mentioned in my article is sensible + realistic. And as a matter of fact, my estimate could even be described as ‘conservative’ – especially since
I have used in my article a Beighton score higher than four
, which indicates that in an arbitrary Marfan population the 90% percentage could very well turn out to be even higher in practice.
How does this sound to you? Have I answered your question?
Greetings from The Netherlands!!
martijnvanmensvoort
February 21, 2012 at 4:03 pm
Hello martijnvanmensvoort,
You are forgetting one crucial thing when estimating your 90%: the fact that 90% of Marfan patients have these symptoms does NOT mean that 90% of those who have these symptoms have Marfan syndrome! To sum up, you are doing logic wrong.
To give an example: 100% of dogs are mammals, but <1% of mammals are dogs.
sdggd
March 9, 2012 at 8:12 pm
Hello sdggd,
Yes, I understand your argument by logic. But I think your example does not adres the fact that I have been talking about a relatively rare combination of 3 features (counting the Steinberg sign and the Walker-Murdoch sign as seperate characteristics, and joint hypermobility as the 3rd feature) – which has a highly specific diagnostic value for Marfan syndrome.
Because even just the combination of the Steinberg sign & the Walker-Murdoch sign have been recognized as a ‘high specific’ combination for Marfan syndrome, which implicates that there is a very small chance that your will find it in other people, see for example:
http://www.jbjs.org/article.aspx?Volume=92&page=1868
“Musculoskeletal clinicians should be aware of the diagnostic features of Marfan syndrome. Patients with three to four physically evident features, or two highly specific features (e.g., thumb and wrist signs, craniofacial features, dural ectasia, or protrusio), should be carefully reexamined and possibly referred for an echocardiogram or a genetics consultation.”
Now, I would like to invite you to find just 1 single person who meets this MULTI-DIMENSION combination of 3 features that I described… and you will find that it is extremely hard to any person who has this combination – except among people who have Marfan syndrome.
Because even just the combination of the Steinberg sign & the Walker-Murdoch sign (= a combination of just 2 of the 3 signs that I mentioned) have been recognized as a ‘high specific’ combination for Marfan syndrome, which implicates that there is a very small chance that your will find it in other people, see for example:
http://www.jbjs.org/article.aspx?Volume=92&page=1868
“Musculoskeletal clinicians should be aware of the diagnostic features of Marfan syndrome. Patients with three to four physically evident features, or two highly specific features (e.g., thumb and wrist signs, craniofacial features, dural ectasia, or protrusio), should be carefully reexamined and possibly referred for an echocardiogram or a genetics consultation.”
So, I think your ‘dogs vs. animals example’ is an oversimplification of the highly specific materials that I have presented.
My article only serves as an introduction to how Marfan syndrome can often be recognized via the hand – but again, I have not suggested nor claimed that the combination of 3 features is a requirement for Marfan syndrome.
I hope this will help you to recognize the fundamental difference between: my article about
how to recognize hand features
that have a highly reliable ‘diagnostic value’ for Marfan syndrome, versus your (implicite) suggestion that I sort of tried to present guidelines for making an exclusive (medical) diagnosis for Marfan syndrome – which is not the focus nor the purpose of my article at all.
Does this make sense for you?
PS. Sometimes it requires specific expertise in order to recognize why your sensible argument may loose it’s value in this context – therefore I raised the issue regarding your experience in this matter (I hope you understand).
martijnvanmensvoort
March 9, 2012 at 10:11 pm
[…] • 5 Hand signs in Marfan syndrome […]
Can hand signs signal disease & psychiatric disorders? | Hand Facts: News about hands!
August 25, 2011 at 12:40 am
I got a seven on the Beighton score :O
kat
October 14, 2011 at 1:52 am
if I only scored 2, and couldn’t do A or B does this mean I do not have marfan sydrome? bare in mind that i have a sunken chest, a moderately arched pallete, close teeth, long slender finger, and 6ft 135 pounds, i also have stretch marks on my back when i had a growth spurt.
Would really appreciate if someone would reply as I’m extremely worried. also I’m 22 years old
Terry
December 16, 2011 at 11:54 pm
Yes Terry, you probably don’t have Marfan syndrome – since you don’t have the Steinberg sign, nor the Walker-Murdoch sign, nor do you qualify for hypermobility.
If you have more worries regarding your body, please consult your general-practitioner in order to ease your mind.
Thank you for your comment.
martijnvanmensvoort
December 31, 2011 at 2:55 am
Wow… I think I have it. I can do both a and b and got a 7 on the 3rd test. But I’m not tall. I just have long thin fingers and arms.
Bori
February 11, 2012 at 5:44 pm
Hello Bori,
While many individuals who have Marfan syndrome grow to above height, many have normal height. So, you do appear to have most of the typical characteristics.
Do you have any problems with your wrist, spine or eyes?
(At wikipedia you can find the most common problems in Marfan syndrome for these body parts: http://en.wikipedia.org/wiki/Marfan_syndrome#Signs_and_symptoms )
Anyway, thank you for your report so far!
Greetings from The Netherlands.
martijnvanmensvoort
February 11, 2012 at 7:28 pm
My son has long slender fingers does that mean he has it he didnt pass the Steinberg sign but passed the walker murdoch sign im so worried he didnt pass some of the other test either cant you just have long slender hands without have Marfan please put my mind at ease shall i do the test of hand span when you measure the distance to your height is thst good indicator as i have read this is another test boy am i worried thanks susan
susan
February 12, 2012 at 11:38 am
Hello Susan,
First of all, ‘long slender fingers’ & the ‘hand span when you measure the distance to your height’ should not be considered as representing reliable indicators for Marfan syndrome.
Second, I think your son doesn’t show (m)any signs of joint hypermobility; and because he basically only passes the Walker-Murdoch sign… therefore I think you son has a pretty high chance that he DOES NOT have Marfan syndrome.
I hope this will easy your mind (permanently).
Thank you for sharing your question!
martijnvanmensvoort
February 12, 2012 at 4:52 pm
There are many differential diagnosis for long fingers/positive wrist or thumb signs. Also people can have Marfan without being tall or have long fingers. I think it is appalling that you are on here diagnosing people? when you are not a doctor but a psychologist (albeit with an interest in hand therapy!).
Do you have any idea of the implications for someone to believe but not KNOW that they may have Marfans? Insurance? Self esteem? body image? no cure? etc etc . I cannot believe the arrogance and naive stupidity/cynical manoeuvring that is on here!
The only way to diagnose/rule out marfan is to see a PROPER doctor – and then only if there is a reason why you feel you need to! if something has no cure perhaps it is better to not know? and take your chances? and live a happy life? rather than one ruled by possibly endless/needless fear and anxiety! (and thereby keep charlatans out of the equation) :s
Mag
February 25, 2012 at 5:22 am
Hello Mag,
I think you are confusing the process of observing/discussing the chances (based on scientific evidence) that an individual has Marfan syndrome with the process of diagnosing Marfan syndrome.
There is no clear cut (blood) test available for diagnosing Marfan syndrome, and even the genetic test are arbitrary due to the fact that the proces of diagnosis Marfan syndrome is still a matter of ‘clinical evaluation’. Which implicates that even when consulting a doctor for diagnosing Marfan syndrome, the result will depend on which criteria he/she decides to use.
Basically, I have only been discussing here the perspective of the hand… but I have reported in the last sentence of my article additional (hand related) criteria – which could also make the difference of whether it would be appropriate for an individual to have a check-out for a Marfan diagnosis.
In the article & my responses to questions I have talked about chances (in the perspective of ‘diagnostic value’) – I never claimed to be able to make a permanent diagnosis.
(By the way, you forget to mention whether you have yourself any experience in the perspective of Marfan syndrome – which could indicate that you are judging my article/responses without knowing much about this topic…?)
You are welcome to respond & share your additional views/experience!
martijnvanmensvoort
February 25, 2012 at 2:29 pm
“you forget to mention whether you have yourself any experience in the perspective of Marfan syndrome – which could indicate that you are judging my article/responses without knowing much about this topic”
This does not disprove the fact that you are not a doctor and yet try to diagnose people, by picking out three criteria from the numerous list of symptoms associated with Marfan. Not to mention that you are trying to diagnose ppl through the internet, without having ever seen them…
sdggd
March 9, 2012 at 8:20 pm
Hi–I have been doing some research and I don’t know if I should be concerned. I have the wrist and thumb sign. I can also easily extend thumb to forearm on both hands and lift finger at 90 degree angle. I am taller than anyone in my family–although not incredibly tall 5 foot 8 inches (I’m a woman). I also have some other signs–my shoulder used to dislocate easily when I was a kid. When I turned 20 I started suffering from joint pain–in many different joints–very severe at times in my knees and also sometimes bone pain (at night). They did x-rays and found nothing. Should I be concerned–how important is the hand sign?
layla
March 25, 2012 at 11:59 pm
Hello Layla,
Regarding the x-ray, you didn’t inform us why the x-ray was made exactly… but in Marfan syndrome an x-ray usually only becomes involved in order to find confirmation for a conditions called ‘dural ectasia’ (which relates to the weakening of a connective tissue outside the spinal cord) or in order to find abnormalities in the aorta of the heart, the lungs or the hips.
So, in Marfan syndrome x-rays are not (typically) being used to assess the joints!
Does this answer your question?
(By the way, yes I think you have described more than enough reasons to request a check up for Marfan syndrome by your general practitioner – because it could explain the connection between the various symptoms that you described; when you decide to follow my advice … you are also very welcome to report the outcome of your check up!)
Anyway, thank you kindly for your sharing so far!
martijnvanmensvoort
March 26, 2012 at 1:47 am
I would like permission to use the striking image of the boy with Marfan Syndrome in a blog about my friend’s discovering he has it. May I have your permission?
Pat Shipman
May 8, 2012 at 5:00 pm
I am worried about possibly having Marfan’s. no one in my family has it, however I can do the second hand sign. just the tip of my nail/finger shows on the first hand sign. I am not flexible at all. i do have long arms and legs. i have huge hands and long fingers and toes. i just had an echo stress test and my heart is normal. i mentioned Marfan’s to my dr and she laughed it off. my dad is 6’5 and wears like a 14 shoe. i am 5’9 and 145 lbs. i dont know if I am just big because of my dad or if it is marfan’s. advice?
nikki
May 10, 2012 at 3:32 am
Hello Nikki,
Your feedback indicates that you have only 1 of the significant hand features listed in my article. For this reason it is quite unlikely that you have Marfan syndrome, and therefore you have not much to worry about – because the missing of the other hand markers should be understood as a contra-indicator.
I hope this eases your mind.
martijnvanmensvoort
May 22, 2012 at 9:53 am
Thank you for this article. I have long wondered if I have Marfan Syndrome; I am 6’2” tall, with very long and slender limbs and fingers, and score a very definite positive on both the Steinberg and Walker-Murdoch signs. I score just over 4 on the Beighton tests; I say ‘just over’ because my thumbs don’t quite touch my forearms but get pretty close (also I can lick my elbow which I’m told is impossible for most people!). I also suffer with shortness of breath, have a very low lung capacity (I have never been a smoker), serious fatigue, have regular heart flutters and palpitations, have cold limbs and poor circulation, aching limbs and muscles as well as ‘electric shock’ type pains in my legs which only go away if I lay flat on my back. I have asked my doctor about the possibility of Marfan Syndrome in the past but he was very dismissive and said I was just tall and I should expect height related aches and pains. So I am unsure. I don’t present with some of the classic signs; I don’t have a deep or narrow pallet, I don’t have narrow shoulders, I don’t have eye problems, nor to I have a concave mid chest – however my sternum is ‘bumby’ and often makes a loud ‘crack’ or clicking sound when I move which other people are able to hear. My sternum can also be painful at times. As I present with a good number of Marfan Syndrome identifiers, but not others, I am quite confused and would welcome any thoughts.
With thanks and kindest regards,
Gemma
Troy Books Publishing
May 22, 2012 at 1:16 pm
Hello Gemma,
Sorry for not responding earlier.
Have you tried the new ‘hand-test’?:
https://palmreadingperspectives.wordpress.com/2012/06/07/a-hand-test-for-recognizing-marfan-syndrome/
Sounds like your score is at least 8 – which puts you in the category ‘likely’ Marfan syndrome. But since your score of 8 relates to only one of the three hand-dimensions (hand shape), I think it is important that in your case your score should become higher than 8 in order to proceed on the possibility that you might have Down syndrome. Do have any of the other 6 hand signs displayed in the hand test?
Regarding your body height, please consider your arm-span as well: does it exceed your body height? (I am asking because in Marfan syndrome the arm-span versus body height ratio is typically > 1.05).
And does your body height exceed the body height of family members?
Only if your answers are 3x a ‘yes’ then your chances for having Marfan syndrome would become very significant.
(Looking forward to see your answers)
Greetings from The Netherlands!
martijnvanmensvoort
June 10, 2012 at 2:19 am
hope you can reply 🙂 I don’t look extremely different with the limbs and the height. I am 5’7 and havve a thin torso. Anyways. I can do all those things with my hands and I have really thin wrists so that I can wrap smallestfinger-thumb around my wrist. I also have the fingers bending extremely far backwards and the thumb being able to bend down to the wrist on my left hand. I can’t touch the floor with my pals though, and I can’t bend my knee backwards. But you think there is still a chance I could have this syndrome? 🙂
Amalie 17 yrs
June 8, 2012 at 5:33 pm
Hello Amalie,
Your body height could indicate that you probably don’t have Marfan syndrome – unless your body height exceeds the body height of your parents. I assume that is not the case?
Also, Marfan syndrome usually featured with an arm span that is at least about 5 cm longer than your body length. Your body length signals that you don’t have this characteristic as well. Correct?
So, in case your answer to both of these questions is a ‘yes’ then your probably do not have Marfan syndrome.
Does this ease your worries so far?
Greetings from The Netherlands, Martijn.
PS Just in case my answer raises new questions, you’re welcome to share these as well.
martijnvanmensvoort
June 9, 2012 at 12:22 am
May I have a reply also please (comment May 22nd)
Thank you,
Gemma
Troy Books Publishing
June 9, 2012 at 9:53 pm
Hi, in about May I learned about this syndrome. I did a bit more research and now I am afraid I may have this. (Btw I am 13) I have both the thumb and wrist signs. Also, I got a 7 on the chart. Though, I’m not that tall. 5 foot 5. I am very skinny though, 88 pounds. I am nearsighted and my shoulders stick out a lot… I don’t think I have scoliosis. I may have a slight chest sticking out. Long face too. Last, my legs ache all the time, but these are probably just growing pains. My arms are 5 or 6 cm longer than my height. I wanna go to the doctors because I am worried, but my parents think I’m crazy and that I’m just skinny. What do you think? Please help.
Jackson
September 6, 2012 at 7:45 am
Hello Jackson,
I would like to invite you to do this ‘hand-test’ for recognizing Marfan syndrome:
https://palmreadingperspectives.wordpress.com/2012/06/07/a-hand-test-for-recognizing-marfan-syndrome
Let me know your score, then I might be able to advice you regarding contacting your general practitioner.
NOTICE: Please read the guidelines for each of the 10 hand signs carefully as presented in the 2nd picture; also regarding your age, many people of your age tend to have a ‘slender’ posture so I would advice you not to link related features (shoulder sticking out & weight) with Marfan.
(Your first response indicates that you probably will probably qualify for the ‘Marfan syndrome hand-test’, but please consider all details in the guidelines)
PS. I also have presented this article, which might be helpful to rule out various related conditions:
http://www.handresearch.com/diagnostics/marfan-syndrome-hand-test.htm
(You are welcome to report if you have some of the other hand features listed in that more detailed article)
martijnvanmensvoort
September 7, 2012 at 3:38 am
Ok, I got a 21 on the new test. No other hand signs
Jackson
September 8, 2012 at 12:48 am
Hi. Im 24 years old male. I have really long and skinny arms and legs and my torso is pretty short compared to my legs (my height is 6’2, while my father is 5’8 and mother 5’4). I am positive for both Steinberg sign & the Walker-Murdoch. My thumb can touch the forearm but not exactly with ease. These are the only symptoms of Marfans which I can relate to myself, because other than these, my chest and spine are normal. So do you believe these symptoms are enough?
hsafdar
August 16, 2014 at 9:17 pm
Hello hsafdar,
The details that you describe suggest that you only score 9 points (basically due to long, slender posture + extraordinary flexible thumb), not enough to pass the hand test for Marfan syndrome… and likely not enough to get associated with Marfan syndrome because that would require the presence of more abnormalities at other dimensions.
Anyway, thanks for asking!
martijnvanmensvoort
September 29, 2014 at 3:27 pm
I have most of the hand signs of marfans. I have a beighton score of 7. I am extremely hypermobilite with mild skin extensibility. I have been to a geneticist. I have Ehlers-Danlos syndrome hypermobility type.
Elhers danlos sufferer
November 21, 2016 at 8:49 pm
Rodricus miller i am 22 years old i have marfan syndromes i have back problems an they put a rod in my back an i have solilses in my back
Rodricus
November 17, 2017 at 3:17 am
This is a very interesting and somehow useful, but there is one problem – the features described (combination of BOTH wrist and thumb signs + hypermobility) points to a marfanoid habitus, which is often present in both Ehlers-Danlos and Marfan’s.
Myself – I have the wrist sign and Beighton score of 4. My fingers are long but not extremely long, my metacarpal index (I calcualted that based on my hand radiograph) is about 8-8.1, i.e. slightly above the norm.
Petr
October 13, 2020 at 9:02 am