=?us-ascii?Q?RE:_Informal_Case:_Exencephaly_-_Anencephaly_Sequence_and_it? =?us-ascii?Q?s_Sonographic_Features?

From: Philippe Jeanty, MD, PhD (jeanty@TheFetus.net)
Thu Oct 9 12:21:54 2003


I very disagree with the hypothesis that anencephaly is the end result of acrania. Why would acrania be visible in older fetuses then those with anencephaly ? The paper of Dani and Waldo is very interesting and I would agree that it is quite suggestive that in some case this could be the pathway in a specific subset. However the problem is that there are no good definition of acrania and anencephaly (how little brain should you have to be considered anencephaly and how much to be called acrania ?).

The embryological mechanism for the 2 conditions is also different (failure of closure of the anterior neuropore in anencephaly versus lack of migration of the mesoderm over the neural tube in acrania.

Finally the recurrence rate of neural tube defect in anencephaly is well known, while recurrence of acrania is exceedingly rare (notwithstanding the case in their series).

-----Original Message----- From: ultrasound@obgyn.net [mailto:ultrasound@obgyn.net] On Behalf Of Terry J. DuBose Sent: Thursday, October 09, 2003 10:12 AM To: Multiple recipients of list ULTRASOUND Subject: Informal Case: Exencephaly - Anencephaly Sequence and its Sonographic Features

Re: http://www.obgyn.net/us/us.asp?page=/us/cotm/0006/Exencephaly-Anencephaly

There is an excellent article that also documents the change from Exencephaly to Anencephaly with images in the current JUM. See: http://www.jultrasoundmed.org/cgi/content/full/22/10/1075 Unfortunately, this article did not reference our earlier publication of the same type of series with images... I guess electronic peer-review and publication just doesn't count. If it isn't in Medline it doesn't count. :-(

However the article title raises an academic question for me. According to my handy-dandy Stedman's Electronic Medical Dictionary "Acrania" and "Exencephaly" are very similar if not the same... so is there a difference in the two terms and how they should be used? Thanks, Terry

J Ultrasound Med 22:1075-1079 . 0278-4297 http://www.jultrasoundmed.org/cgi/content/full/22/10/1075 Case Series

First-Trimester Echogenic Amniotic Fluid in the Acrania-Anencephaly Sequence

By Daniel Cafici, MD and Waldo Sepulveda, MD Ultrasound and Prenatal Diagnosis Unit, Ecodiagnostico Alem, Buenos Aires, Argentina (D.C.); and Fetal Medicine Center, Department of Obstetrics and Gynecology, Clinica Las Condes, Santiago, Chile (W.S.).

Abstract

Objective. To describe the association between echogenic amniotic fluid and first-trimester fetal acrania. Methods. Nine fetuses with acrania were examined between 11 weeks' and 13 weeks 6 days' menstrual age for the presence of echogenic free-floating particles in the amniotic fluid. Cases were classified into 3 types according to the echogenicity of the amniotic fluid: similar to (type 0), slightly greater than (type 1), and clearly more echogenic than (type 2) that of the extracelomic fluid. Results. In 1 pregnancy, no free-floating particles were identified (type 0). In 6 cases, small free-floating particles scattered within the amniotic cavity were identified, making the amniotic fluid slightly more echogenic than the extracelomic fluid (type 1). In the remaining 2 cases, the amniotic fluid was homogeneously and clearly more echogenic than the extracelomic fluid (type 2). Conclusions. A high percentage (89%) of fetuses with acrania had echogenic amniotic fluid, suggesting that this finding could potentially be used as a marker of fetal acrania in the first trimester. This finding also supports the hypothesis of the transition from acrania to anencephaly, with the unprotected brain undergoing progressive destruction from the first trimester, leading to the classic finding of anencephaly in the second trimester.

Key Words: acrania . anencephaly . fetal sonography . first trimester . prenatal diagnosis

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




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