Table Of ContentPEDIATRIC GENDER
ASSIGNMENT
A Critical Reappraisal
ADVANCES IN EXPERIMENTAL MEDICINE AND BIOLOGY
Editorial Board:
NATHAN BACK, State University of New York at Buffalo
IRUN R. COHEN. The Weizmann Institute of Science
DAVID KRITCHEVSKY, Wistar Institute
ABEL LAJTHA, N. S. Kline Institute for Psychiatric Research
RODOLFO PAOLETTI, University of Milan
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PEDIATRIC GENDER
ASSIGNMENT
A Critical Reappraisal
Edited by
Stephen A. Zderic
Douglas A. Canning
Michael C. Carr and
Howard McC. Snyder, III.
Children s Hospital of Philadelphia
Philadelphia, Pennsylvania
Springer-Science+Business Media, LLC
Library of Congress Cataloging-in-Publication Data
Pediatric gender assignment: a critical reappraisal/edited by Stephen A. Zderic ... [et al.].
p. ; cm.
Includes bibliographical references and index.
ISBN 978-1-4613-5162-7 ISBN 978-1-4615-0621-8 (eBook)
DOI 10.1007/978-1-4615-0621-8
I. Gender identity disorders in children-Congresses. 2. Hermaphroditism-Congresses.
3. Generative organs-Abnormalities-Congresses. 4. Sex orientation-Congresses. I.
Zderic, Stephen A.
[DNLM: l. Sex Differentiation Disorders-therapy-Child-Congresses. 2. Sex
Differentiation Disorders-therapy-Infant-Congresses. 3. Genitalia,
Female-abnormalities-Child-Congresses. 4. Genitalia,
Female-abnormalities-Infant-Congresses. 5. Genitalia,
Male-abnormalities-Child-Congresses. 6. Genitalia,
Male-abnormalities-Infant-Congresses. 7. Hermaphroditism-Congresses. 8. Sex
Determination (Analysis)-Congresses. WS 320 P3687 2002]
RJ506.G35 P436 2002
618.92'65-dc21
2002032120
ISBN 978-1-4613-5162-7
©2002 Springer Science+Business Media New York
Originally published by Kluwer Academic I Plenum Publishers, New York in 2002
Softcover reprint ofthe hardcover 1s t edition 2002
All rights reserved
No part of this book may be reproduced, stored in a retrieval system, or transmitted in any form or by any
means, electronic, mechanical, photocopying, microfilming, recording, or otherwise, without written
permission from the Publisher, with the exception of any material supplied specifically for the purpose of being
entered and executed on a computer system, for exclusive use by the purchaser of the work
CONTRIBUTORS
Anthony Atala
Children's Hospital and Harvard Medical School
Boston, Massachusetts
Laurence S. Baskin
Univeristy of California San Francisco
San Francisco, California
Jonathon Bingham
Columbia University
College of Physicians
Babies and Children's Hospital of New York
New York, New York
Douglas A. Canning
University of Pennsylvania School of Medicine
The Children's Hospital of Philadelphia
Philadelphia, Pennsylvania
Michael C. Carr
University of Pennsylvania School of Medicine
The Children's Hospital of Philadelphia
Philadelphia, Pennsylvania
Wilson C.l. Chung
Netherlands Institute for Brain Research
Amsterdam, The Netherlands and
University of Massachusetts
Amherst, Massachusetts
Timothy M. Crombleholme
University of Pennsylvania School of Medicine
The Children's Hospital of Philadelphia
Philadelphia, Pennsylvania
Patricia K. Donahoe
Pediatric Surgical Research Laboratories
Massachusetts General Hospital
Harvard Medical School
Boston, Massachusetts
v
vi CONTRIBUTORS
Roger A. Gorski
University of California Los Angeles
School of Medicine
Los Angeles, California
Faruk Badziselimovic
The Basel Children's Hospital
Basel, Switzerland
Terry W. Hensle
Columbia University
College of Physicians
Babies and Children's Hospital of New York
New York, New York
Michael A. Hofman
Graduate School Neurosciences Amsterdam
Netherlands Institute for Brain Research
Amsterdam, The Netherlands
Dale Huff
University of Pennsylvania School of Medicine
The Children's Hospital of Philadelphia
Philadelphia, Pennsylvania
Douglas A. Husmann
Mayo Clinic
Rochester, Minnesota
J ulianna Imperato-McGinley
Weill Medical College of Cornell University
New York, New York
Tatjana A. Ishunina
Netherlands Institute for Brain Research
Amsterdam, The Netherlands and
Kursk State Medical University
Kursk, Russia
Brian K. Jordan
University of California Los Angeles
Los Angeles, California
CONTRIBUTORS vii
Gerald H. Jordan
Eastern Virginia School of Medicine
Norfolk, Virginia
Christine Kodman-Jones
The Children's Hospital of Philadelphia
Philadelphia, Pennsylvania
Frank P.M. Kruijver
Graduate School Neurosciences Amsterdam
Netherlands Institute for Brain Research
Amsterdam, The Netherlands
David T. MacLaughlin
Pediatric Surgical Research Laboratories
Massachusetts General Hospital
Harvard Medical School
Boston, Massachusetts
Laurence B. McCullough
Baylor College of Medicine
Houston, Texas
J. William McRoberts
U ni versity of Kentucky School of Medicine
Lexington, Kentucky
Heino F.L. Meyer-Bahlburg
Columbia University
New York, New York
Michael E. Mitchell
University of Washington School of Medicine
Children's Hospital and Regional Medical Center
Seattle, Washington
J. Chadwick Plaire
Children's Hospital and Regional Medical Center
Seattle, W ashi ngton
William G. Reiner
Johns Hopkins Medical Institutions
Baltimore, Maryland
viii CONTRIBUTORS
Natalie E. Rintoul
The Children's Hospital of Philadelphia
Philadelphia, Pennsylvania
Richard C. Sadove
University of Kentucky School of Medicine
Lexington. Kentucky
Howard McC. Snyder, III
University of Pennsylvania School of Medicine
The Children's Hospital of Philadelphia
Philadelphia, Pennsylvania
Dick F. Swaab
Graduate School Neurosciences Amsterdam
Netherlands Institute for Brain Research
Amsterdam, The Netherlands
Eric Vilain
University of California Los Angeles
Los Angeles, California
Perrin C. White
University of Texas Southwestern Medical Center
Dallas. Texas
Stephen A. Zderic
University of Pennsylvania School of Medicine
The Children's Hospital of Philadelphia
Philadelphia, Pennsylvania
PREFACE
This book represents the proceedings from a conference that took place in Dallas
in the spring of 1999 which was entitled "Pediatric Gender Assignment - A Critical
Reappraisal". Some participants rightfully argued that the conference really focused
on the issue of pediatric gender assignment, and that reassignment was not applied in
most cases. Their comments were reflected in the title of this monograph. This
multidisciplinary meeting was sponsored by a conference grant from the National
Institutes of Health, and a broad inquiry into this complex topic took place from
many points of view. Basic scientists offered insight into mechanisms of sexual
differentiation of the gonads, physical phenotype and imprinting of the central
nervous system. Endocrinologists reviewed their experience in diagnosis and
management, surgeons described traditional as well as innovative approaches, and
there was strong representation from the ethical and behavioral sciences. In putting
together such a panel, it was essential that we identify a cast of speakers who could
address their viewpoints with strong convictions, and yet not let their passions render
the meeting counter productive. We were not disappointed. While many differing
points of view were firmly expressed by the panelists and audience, all viewpoints
were accorded the respect they deserved.
The concept behind the meeting and this book really originated in 1997 shortly
after Diamond and Sigmundson published their long term follow up study of the
John/Joan case. Our first thoughts centered around the concept of the numerator
versus the denominator. This high publicized case report represented a disturbing
example of gender reassignment for a high unusual indication, but it represents a
single case report. Even more disturbing was John Colapinto's personal account of
his experience which appeared that spring in Rolling Stone magazine. We began to
ask ourselves if there were any successful cases of neonatal gender reassignment.
We do not know about the denominator. Are there patients out there who are doing
well or are they all doing poorly? What becomes clear within this monograph, is that
more long term follow up information about these patients is needed.
Several themes emerged from this conference. First and foremost was the need
that all these patients and families have for long term counseling. No matter which
direction is taken with these incredibly difficult issues, counseling must be made a
cornerstone of their management. Psychiatrists, psychologists and social workers are
critical to a multidisciplinary team in order to produce the best possible long term
outcomes for the patient and the family. Logical as this might seem, individuals with
these skills and interests are hard to find. A second theme that emerged was that
patients have more medical knowledge than ever before. We must become
comfortable with life in the internet age where much more information is readily
available, some of which may not always be accurate. Patients will always
appreciate getting the information from their physicians, as opposed to finding it on a
web page. Recently after a long initial discussion with two very computer literate
ix
x PREFACE
parents, I referred them to the Intersex Society of North America (lSNAorg) web
site, and asked them to look through it. At the subsequent multidisciplinary team
meeting, they had additional information at their disposal. By empowering parents
and patients, it is possible to take what is initially threatening in the traditional
medical model, and have it benefit everyone. A third major theme that emerged was
that the human brain is a sexual organ. Since the human brain is sexually dimorphic,
it is not always possible to predict whether the adult will be happy with their gender
20 and 30 years after such a critical decision has been made in the first days of life.
This is underscored by the divergence of opinions expressed at the conference and
within this monograph about the issue of early neonatal corrective surgery versus
delaying any corrective surgery until the child can voice their gender identity.
As organizers, we never expected that one conference could resolve so complex
an issue and arrive at some simple management algorithm. Rather, our goals were
simply to initiate a dialogue amongst individuals with varying points of view. In
fact, the exchange of ideas after the presentations were so informative, that we have
included in this monograph the transcripts (with minimal editing) of the discussions
following each major presentation. We are especially hopeful that major centers will
set up a central registry so that long term outcomes can be obtained. The American
Academy of Pediatrics Section on Urology, and the Society for Fetal Urology have
formed committees that are working together to develop such a registry. Many of the
answers to the questions posed at this meeting already exist, but can be obtained only
if we can get as many patients as possible to return for long term evaluation. It is our
hope that at a future meeting, the answers to some of these questions will have
become apparent.
Stephen A Zderic, M.D.
For the Editors
