Polydactylism & associated conditions

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Hi all,

I realize this may be a bit vague as I have not seen this little person but the daughter of one of my patients has had a baby born with 7 digits on one foot. The foot is also enlarged compared to the other. There does not seem to be any other external abnormalities as yet. I suppose my question is "Is any one aware of specific medical conditions with polydactylism associated?"
The patient is concerned of possible internal problems that may be present with this baby. Baby has eaten & had bowel movement but not urinated as yet. Sorry, post partum approximately 12hrs.
Sorry I cant be anymore detailed but can only relay information I have received but I have googled a bit but nothing specific yet.
Any feedback would be much appreciated so I can inform my patient.

Cheers
Wayne:bang:

 

Wayne

If you want to scare yourself or your patient have a look at this site http://pediatrics.aappublications.org. Search polydactylism and you will find many syndromes and diseases that polydactylism is possibly associated with or a sign of. These links however are tenuous at best it appears and may not be very useful.

I.E.
ULIUS D. METRAKOS PH.D. and F. CLARKE FRASER PH.D., M.D.
We read with great interest the two excellent case histories of chondroectodermal dysplasia (Ellis-van Creveld syndrome) that were reported recently by Smith and Hand (Pediatrics, 21:298, 1958). We must take issue, however, with the conclusion that "The statistical probability of siblings having the complete tetrad of defects is rather remote...." This conclusion appears to be based on the assumptions that the four members of the tetrad (chondrodysplasia, ectodermal dysplasia, polydactylism and congenital heart disease) are caused by four different genes, and that the characteristic association of abnormalities to form a syndrome is due to linkage.


Cheers Dave

 

Goodaye to you Wayne, I had to put my bit in here. I myself am a pre-axial "polydactyl", with both phalanges of both halluces duplicated but fused. With me it's an autosomal dominant trait with incomplete penetrance. Both offspring have 'lucked-out'. The girl has bilateral syndactyly of 1st, 2nd & 3rd digits, whilst the boy had complete duplication of both phalanges of his r/hallux (with syndactyly) and duplication of distal phalanx only on his left foot.
As you are aware they can be part of some pretty unfortunate syndromes. Luckily the only associated trait with me would be my extraordinary good looks. Hoping all goes well, mark

 

Polydactylism can be an isolated entity or can be associated with a whole range of genetic probelms.

Apart from Mark's good looks, we can speculate what other genetic disorders he mght have :empathy:

 

Contact the wife, she'd be more than willing to offer suggestions.
Craig, you'd be watching the results from other games seriously in the last two rounds. Who are you barracking for in the sharks-waratahs match? Good luck!

 

Hi All,

Has been long time since have followed this up but I am looking for further assistance with the polydactyly case. I have further information but dont have any scans available. They parent has informed me the daughter has 7 digits to the right foot with 8 metatarsals & 4 cuneiforms present. They are currently going thru the public system in Brisbane & have been mucked aroound a little as I dont think they know what to do with this case. Thay have stated they have request feedback from a Surgeon in the states who may have experience with dealing with something like this. Initially they were told they could remove the 2 extra digits between D1/2 & extra MTs but my concern is what should happen with the cuneiforms. There would likely be some effect on the keystone mechanism if they remove a cuneiform. This pt is fully active & functional & is now 2yo. They have been instructed from USA to only remove 2MTs & 1 extra phalange, not touching the cuneiforms. This all sounds good in maintaining proper function of foot but she will still be left with very broad forefoot & 6 digits. Footwear will be an ongoing issue for her whole life.

Again I know this is vague without having better information but I am trying to get some scans to post, hopefully they can get them out of the hospital files.

Any feedback or assistance would be helpful. have any of our Surgical colleagues had any previous experience with something like this.

If I get more information I will post it as soon as possible.

Thanks again.