Re: Conjoined twins with different genders
From: Wolfgang Moroder, M.D. (wolfgang.moroder@tin.it)
Tue Oct 21 14:35:12 2003
This discussion is continuing and I'd like to share with the forum the
thoughts and ideas which came up at this point from a discussion between
Viv Souter, Albert Reece, Terry DuBose and me.
Dear Al,
I remember and was impressed to see you doing the embryoscopies at Yale
with the
thought of early fetal therapy. It came immediatly to my mind with this
discussion.
Now is monochorionic pregnancy the clue?. Blood chimerism is induced by
very early
twin to twin transfusion in monochorionic pregnancies. At the embryonic
stage stemm
cells are transfered as an allograft from one embryo to the other via
common placental
blood vessels and cause a blood chimerism while other tissues like skin
are not
affected.
At this point we have only one question to solve: why do they share the
placenta or
why are they monochorionic.
I'm quite exited at these thoughts
Wolfgang
Albert Reece wrote:
Dear Viv:
I agree with you that these are blood chimeras with low levels of
chimerisms.
However, chimerisms are not expressed in all tissues, and so it is not
unreasonable to
see selective tissue types with manifest expression. For whatever
reason, the blood
carries to chimeras and the skin fibroblasts do not. This is very
interesting. Regards.
Sincerely,
E. Albert Reece,
viv souter <vsouter@yahoo.com> writes:
>Hi Albert
>In the twins that we described in the NEJM the twins were identical in
terms of the
cytogenetics of their blood lymphocytes (both 95% 46,XY and 5% 46,XX)
however
the boy was 46,XY and the girl 46,XX in skin fibroblasts ie they are
blood chimeras.
Blood chimerism in dizygotic twins has been recognised for at least 30
years. They
typical presentation was with when difficultly was encountered cross
matching these
individuals for blood transfusions (Tippett et al rported a series of
these cases). A
>more recent study using very sensitive techniques found 8% of non
identical
multiples had blood chimerism, most at low levels.
>I think the DZ MC twins are most likely chimeric only in hemopoietic
cells rather
than total body chimeras (although obviously without testing every
tissue we cannot
be 100% sure).
>I would be interested on your thoughts re this.
>Viv
>Good Samaritan Hospital
>Phoenix
>AZ
>
Dear Wolfgang:
I don't mind. I just heard from Viv Souter and I shared with her that
this is probably a case of chimerism, with chimeras being most
manifest in some tissues (e.g.) blood and not in others (skin
fibroblast). Regards.
Sincerely,
E. Albert Reece,
wolfgang.moroder@tin.it writes:
>Dear Al:
>It is good to hear from you: it is a small world.
>I thought of chimerism when we karyotyped the newborns but their blood
>was mixed by definition since we were sure it was a monochorionic
>pregnancy. The proof are the pictures of the first trimester scan here:
>
>http://www.obgyn.net/us/us.asp?page=/us/present/1003/moroder_monochorionic
>
>I am convinced that the lambda, delta or twin peak sign, you name it, is
>highly sensitive and specific but they are not direct signs of
>monoamnionicity like the single extraembryonic space or the paired yolk
>sacs. The unequivocal demonstration of these signs is possible only in
>the first trimester with high resolution, high frequency transvaginal scanning.
>On the skin fibroblasts of each newborn a distinctive normal
>heterogender karyotype was demonstrated. I don't know if the chimerism
>could be confined to the gonads, I confess my great ignorance in
>genetics. We have not done any DNA analysis yet to demonstrate
>monozygosity but asked the geneticists to work on it. I surely would
>publish the case as a case where there was sonoembryologic proof of
>chorionicity, sonographic proof at 20 weeks of heterogenic genitals
>while everything else has to be proven
>besides genotypic and phenotypic sex. Besides all that I believe that
>nature does not have any strict rules, what is normal in one species is
>very rare in the other. I have written to Prof. Kurt Benirschke about
>the case and he gave various suggestions.
>
>Al, if you don't mind I would put all our private discussion on the
>obgyn.net Ultrasound forum with your permission so we can sahre it with others.
>I'm looking forward to see you soon again at the next SMFM meeting.
>
>Best regards
>Wolfgang
>Albert Reece <reeceealbert@uams.edu> wrote:
>
>Dear Wolfgang:
>I just received this e-mail which was sent to me, and see that you are
indeed
breaking new ground. Your finding violates traditional teaching, but in
my mind is
more consistent with chimeras (XX/XY) with two sets of karyotype and in
one case,
one is phenotypically expressed. This is a very good case and is worth
publishing. I
hope you and Gabriel are doing well. Hope to see you one day soon.
Regards.
>Sincerely,
>E. Albert Reece, M.D., Ph.D., M.B.A.
>Vice Chancellor, UAMS
>Dean, College of Medicine
>University of Arkansas for Medical Sciences
>4301 W. Markham, Slot 550
>Little Rock, AR 72205
>(501) 686-5350
>FAX: (501) 686-8160
>Email: reeceealbert@uams.edu
At Tue, 21 Oct 2003, Wolfgang Moroder, M.D. wrote:
>
>Terry,
>I believe ultrasound plays a key role in these "breaking ground facts",
>to put it as Prof. Alert Reece wrote me. In particular it is
>sonoembryology that allows us to do a routine and anatomic assessment of
>chorionicity in twin pregnancies. Before sonoembryology only accurate
>observation of the placentas and/or targeted pathology allowed us to
>define exactly chorionicity. Maybe Internet (in this case OBGYN.net)
>plays also it's part with sharing qickly, informally and worldwide
>precious information. Ciao Wolfgang
>
>At Mon, 20 Oct 2003, Terry J DuBose wrote:
>>
>>This message is in MIME format. Since your mail reader does not understand
>>this format, some or all of this message may not be legible.
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>>I have probably misunderstood something here. Moroder, Souter, and
>>Jeanty have all seen cases of sex-discordant twins with that appear to be
>>monochorionic twins, correct? And at least one of these are "conjoined",
>>or is more than one set of male/female twins conjoined. With 3 cases, I
>>don't understand why we have not seen this before.
>>
>>Or is it the case that we have not had the genome and sonography together
>>long enough, and no one looked? Has this been going on all along, and we
>>just did not recognize it?
>>
>>Terry
>>
>>On Sat, 18 Oct 2003 07:27:09 -0500 wolfgang.moroder@tin.it (Wolfgang
>>Moroder, M.D.) writes:
>>> Terry, I don't think we can talk about prevalence of "heterogender"
>>> monochorionic twins in literature since the case published by Vav
>>> Souter
>>> on the NEJM is the first to my knowledge. Thank you for publishing
>>> my
>>> images on the Ultrasound section as a Interactive Informal Case
>>> here:
>>>
>>http://www.obgyn.net/us/us.asp?page=/us/present/1003/moroder_monochorioni
>>c
>>>
>>> I'd like to remember the fact that the paired yolk sac sign (8 sign)
>>> is
>>> pathognomonic of a monochorionic pregnancy and quite common. The
>>> yolk
>>> sacs in monochorionic pregnancy are located in one space which is
>>> the
>>> extraembryonic coelom and sometimes, who knows why?, they seem to
>>> be
>>> attracted by each other... even if they are of the same sex!
>>> Ciao Wolfgang
>>>
>>> At Thu, 16 Oct 2003, Terry J. DuBose wrote:
>>> >
>>> >So what is the prevelence of these hetrogender twins?... in the
>>> literature? Terry
>>> >
>>> >ultrasound@obgyn.net writes:
>>> >>In a message dated 10/16/2003 4:53:18 PM Eastern Daylight Time,
>>> vsouter@yahoo.com writes:
>>> >>
>>> >>> We postulated a fusion event as the cause of our unusual twins.
>>> Rowena Spencer has is a proponent of fusion rather than
>>> >>> fission as the cause of amny conjoined twins.
>>> >>
>>> >>Is Rowena still working?
>>> >>
>>> >>Joe P.
>>> >
>>> >--
>>> >Terry J. DuBose, M.S., RDMS, FSDMS, FAIUM
>>> >Assistant Professor & Director, Diagnostic Medical Sonography
>>> Program
>>> >University of Arkansas for Medical Sciences, CHRP
>>> >4301 West Markham St. Mail Slot #563
>>> >Little Rock, Arkansas, 72205 USA
>>> >501-686-6510
>>> >DuBoseTerryJ@UAMS.edu
>>> >http://www.io.com/~dubose/
>>> >http://www.uams.edu/chrp/dms/default.asp
>>> >http://www.obgyn.net/us/panel/panel.htm
>>> >
>>>
>>> --
>>> Wolfgang Moroder, MD
>>> Prenatal Unit
>>> Bolzano General Hospital Italy
>>>
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>>Content-Transfer-Encoding: quoted-printable
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>><!DOCTYPE HTML PUBLIC "-//W3C//DTD HTML 4.0 Transitional//EN">
>>
>>I have probably misunderstood something here. Moroder, Souter, and
>>Jeanty have all seen cases of sex-discordant twins with that appear to
>>be monochorionic twins, correct? And at least one of these are
>>"conjoined", or is more than one set of male/female twins conjoined. With
>>3 cases, I don't understand why we have not seen this before.
>>
>>Or is it the case that we have not had the genome and sonography together
>>long enough, and no one looked? Has this been going on all along, an we
>>just did not recognize it?
>>
>>Terry
>>
>>On Sat, 18 Oct 2003 07:27:09 -0500 <A
>>href="mailto:wolfgang.moroder@tin.it">wolfgang.moroder@tin.it Wolfgang
>>Moroder, M.D.) writes:> Terry, I don't think we can talk about prevalence
>>of "heterogender"> monochorionic twins in literature since the cas
>>published by Vav > Souter> on the NEJM is the first to my
>>knowledge. Thank you for publishing > my> images on th
>>Ultrasound section as a Interactive Informal Case > here:>
>>> > <A
>>href="http://www.obgyn.net/us/us.asp?page=/us/present/1003/moroder monochorionic">http://www.obgyn.net/us/us.asp?page=/us/present/1003 moroder_monochorionic>
>>> I'd like to remember the fact that the paired yolk sac sign (8 sign)
>>> is> pathognomonic of a monochorionic pregnancy and quite
>>common. The > yolk> sacs in monochorionic pregnancy ar
>>located in one space which is > the> extraembryonic coelom and
>>sometimes, who knows why?, they seem to > be> attracted by each
>>other... even if they are of the same sex!> Ciao Wolfgang gt;
>>> At Thu, 16 Oct 2003, Terry J. DuBose wrote:> >> gt;So
>>what is the prevelence of these hetrogender twins?... in the >
>>literature? Terry> >> ><A
>>href="mailto:ultrasound@obgyn.net">ultrasound@obgyn.net writes: gt;
>>>>In a message dated 10/16/2003 4:53:18 PM Eastern Daylight Time, >
>>vsouter@yahoo.com writes:>
>>>>> >>> We postulated a fusion event as the cause of our
>>unusual twins. > Rowena Spencer has is a proponent of fusion rathe
>>than> >>> fission as the cause of amny conjoined twins.<BR >
>>>>> >>Is Rowena still working?> >>>
>>>>Joe P.> >> >--> >Terry J. DuBose, M. .,
>>RDMS, FSDMS, FAIUM> >Assistant Professor & Director, Diagnostic
>>Medical Sonography > Program> >University of Arkansas fo
>>Medical Sciences, CHRP> >4301 West Markham St. Mail Slot #563<BR >
>>>Little Rock, Arkansas, 72205 USA> >501-686-6510> > A
>>href="mailto:DuBoseTerryJ@UAMS.edu">DuBoseTerryJ@UAMS.edu> &g ;<A
>>href="http://www.io.com/~dubose/">http://www.io.com/~dubose/> ><A
>>href="http://www.uams.edu/chrp/dms/default.asp">http://www.uams.edu/chrp dms/default.asp>
>>><A
>>href="http://www.obgyn.net/us/panel/panel.htm">http://www.obgyn.net/us panel/panel.htm>
>>>> > --> Wolfgang Moroder, MD> Prenatal
>>Unit> Bolzano General Hospital Italy> >
>>
>>
>>----__JNP_000_1c7e.32ef.1b2e--
>
>--
>Wolfgang Moroder, MD
>Prenatal Unit
>Bolzano General Hospital Italy
>
--
Wolfgang Moroder, MD
Prenatal Unit
Bolzano General Hospital Italy
