This book is the product of long years of close collaboration between two physicians, a pediatric endocrinologist and a pediatric surgeon, who are interested in the complex field of human malformations. Their efforts have set a new standard in the treatment of children with intersexual or mal­ formed female genitalia. It is impossible to convey the details of complex surgical procedures without the help of meticulous illustrations. It is fortunate that happened to meet an academically trained artist, Mrs. Siri Mills, M.A., while visiting in the United States. Her excellent knowledge of anatomy, her remarkable skills as an artist and technical illustrator, and her readiness to include all details of interest to the surgeon have found their fruitful expression in the illustrations of this book. We express our thanks to Springer-Verlag and especially to Prof. Diet­ rich Goetze, at whose suggestion this book was written, for their amiable compliance with all our wishes, and particularly for their willingness to retain Mrs. Mills as our illustrator. Munich, May 1985 WALDEMAR HECKER Contents In trod ucti on 1 Part 1 : Endocrinologic Diagnosis in Pediatric Patients with Genital Anomalies General Rules . . . . . . . . . . . 4 Endocrinology of Antenatal Sex Differentiation 6 Male Differentiation 6 Female Differentiation 7 Specific Endocrinologic Diagnosis and Therapy 8 Virilization of the Female External Genitalia 8 Congenital Adrenogenital Syndrome with a Defect of 21-Hydr- ylase . . . . . . . . . . . . . . . . . . . . . . . . . . 8 Intersexual Genitals Due to Deficient Androgenization of the Male 11 Defects of Testosterone Biosynthesis 11 Androgen Receptor Defects . . .

This book is the product of long years of close collaboration between two physicians, a pediatric endocrinologist and a pediatric surgeon, who are interested in the complex field of human malformations. Their efforts have set a new standard in the treatment of children with intersexual or mal­ formed female genitalia. It is impossible to convey the details of complex surgical procedures without the help of meticulous illustrations. It is fortunate that happened to meet an academically trained artist, Mrs. Siri Mills, M.A., while visiting in the United States. Her excellent knowledge of anatomy, her remarkable skills as an artist and technical illustrator, and her readiness to include all details of interest to the surgeon have found their fruitful expression in the illustrations of this book. We express our thanks to Springer-Verlag and especially to Prof. Diet­ rich Goetze, at whose suggestion this book was written, for their amiable compliance with all our wishes, and particularly for their willingness to retain Mrs. Mills as our illustrator. Munich, May 1985 WALDEMAR HECKER Contents In trod ucti on 1 Part 1 : Endocrinologic Diagnosis in Pediatric Patients with Genital Anomalies General Rules . . . . . . . . . . . 4 Endocrinology of Antenatal Sex Differentiation 6 Male Differentiation 6 Female Differentiation 7 Specific Endocrinologic Diagnosis and Therapy 8 Virilization of the Female External Genitalia 8 Congenital Adrenogenital Syndrome with a Defect of 21-Hydr- ylase . . . . . . . . . . . . . . . . . . . . . . . . . . 8 Intersexual Genitals Due to Deficient Androgenization of the Male 11 Defects of Testosterone Biosynthesis 11 Androgen Receptor Defects . . .

Das glückliche Zusammentreffen zweier auf dem komplizierten Gebiet menschlicher Fehlbildungen interessierter Ärzte, eines Kinderendokrinolo­ gen und eines Kinderchirurgen, und eine langjährige engste Zusammenarbeit erlaubten es, einen hohen Standard bei der Behandlung von Kindern mit einem intersexuellen oder fehlgebildeten weiblichen Genitale zu schaffen, was in dem vorliegenden Buch seinen Niederschlag findet. Die Darstellung komplizierter operativer Techniken ist ohne subtile und übersichtliche zeichnerische Darstellung nicht möglich. Die in den Staaten zufällig gemachte Bekanntschaft mit der akademisch ausgebildeten Zeichne­ rin Frau Siri Mills M.A. erwies sich als ein Glücksfall ganz besonderer Art. Das hervorragende anatomische Wissen, die exzellente Auffassungs­ gabe, operative Vorgänge zeichnerisch zu erfassen und künstlerisch auszuar­ beiten und ihre Bereitschaft, jedwede Detailwünsche des Operateurs zu be­ rücksichtigen, fanden ihren fruchtbaren Niederschlag in den Abbildungen dieses Buchs. Dem Springer-Verlag, und hier besonders Herrn Professor Dr. Dietrich Götze, von dem die Anregung zur Publikation dieses Werks ausging, sei gedankt für das bereitwillige Eingehen auf all unsere Wünsche, insbesondere für die Bereitschaft, Frau Mills als Zeichnerin zu gewinnen.

This book is the product of long years of close collaboration between two physicians, a pediatric endocrinologist and a pediatric surgeon, who are interested in the complex field of human malformations. Their efforts have set a new standard in the treatment of children with intersexual or mal­ formed female genitalia. It is impossible to convey the details of complex surgical procedures without the help of meticulous illustrations. It is fortunate that happened to meet an academically trained artist, Mrs. Siri Mills, M.A., while visiting in the United States. Her excellent knowledge of anatomy, her remarkable skills as an artist and technical illustrator, and her readiness to include all details of interest to the surgeon have found their fruitful expression in the illustrations of this book. We express our thanks to Springer-Verlag and especially to Prof. Diet­ rich Goetze, at whose suggestion this book was written, for their amiable compliance with all our wishes, and particularly for their willingness to retain Mrs. Mills as our illustrator. Munich, May 1985 WALDEMAR HECKER Contents In trod ucti on 1 Part 1 : Endocrinologic Diagnosis in Pediatric Patients with Genital Anomalies General Rules . . . . . . . . . . . 4 Endocrinology of Antenatal Sex Differentiation 6 Male Differentiation 6 Female Differentiation 7 Specific Endocrinologic Diagnosis and Therapy 8 Virilization of the Female External Genitalia 8 Congenital Adrenogenital Syndrome with a Defect of 21-Hydr- ylase . . . . . . . . . . . . . . . . . . . . . . . . . . 8 Intersexual Genitals Due to Deficient Androgenization of the Male 11 Defects of Testosterone Biosynthesis 11 Androgen Receptor Defects . . .

This book is the product of long years of close collaboration between two physicians, a pediatric endocrinologist and a pediatric surgeon, who are interested in the complex field of human malformations. Their efforts have set a new standard in the treatment of children with intersexual or mal­ formed female genitalia. It is impossible to convey the details of complex surgical procedures without the help of meticulous illustrations. It is fortunate that happened to meet an academically trained artist, Mrs. Siri Mills, M.A., while visiting in the United States. Her excellent knowledge of anatomy, her remarkable skills as an artist and technical illustrator, and her readiness to include all details of interest to the surgeon have found their fruitful expression in the illustrations of this book. We express our thanks to Springer-Verlag and especially to Prof. Diet­ rich Goetze, at whose suggestion this book was written, for their amiable compliance with all our wishes, and particularly for their willingness to retain Mrs. Mills as our illustrator. Munich, May 1985 WALDEMAR HECKER Contents In trod ucti on 1 Part 1 : Endocrinologic Diagnosis in Pediatric Patients with Genital Anomalies General Rules . . . . . . . . . . . 4 Endocrinology of Antenatal Sex Differentiation 6 Male Differentiation 6 Female Differentiation 7 Specific Endocrinologic Diagnosis and Therapy 8 Virilization of the Female External Genitalia 8 Congenital Adrenogenital Syndrome with a Defect of 21-Hydr- ylase . . . . . . . . . . . . . . . . . . . . . . . . . . 8 Intersexual Genitals Due to Deficient Androgenization of the Male 11 Defects of Testosterone Biosynthesis 11 Androgen Receptor Defects . . .

Das glückliche Zusammentreffen zweier auf dem komplizierten Gebiet menschlicher Fehlbildungen interessierter Ärzte, eines Kinderendokrinolo­ gen und eines Kinderchirurgen, und eine langjährige engste Zusammenarbeit erlaubten es, einen hohen Standard bei der Behandlung von Kindern mit einem intersexuellen oder fehlgebildeten weiblichen Genitale zu schaffen, was in dem vorliegenden Buch seinen Niederschlag findet. Die Darstellung komplizierter operativer Techniken ist ohne subtile und übersichtliche zeichnerische Darstellung nicht möglich. Die in den Staaten zufällig gemachte Bekanntschaft mit der akademisch ausgebildeten Zeichne­ rin Frau Siri Mills M.A. erwies sich als ein Glücksfall ganz besonderer Art. Das hervorragende anatomische Wissen, die exzellente Auffassungs­ gabe, operative Vorgänge zeichnerisch zu erfassen und künstlerisch auszuar­ beiten und ihre Bereitschaft, jedwede Detailwünsche des Operateurs zu be­ rücksichtigen, fanden ihren fruchtbaren Niederschlag in den Abbildungen dieses Buchs. Dem Springer-Verlag, und hier besonders Herrn Professor Dr. Dietrich Götze, von dem die Anregung zur Publikation dieses Werks ausging, sei gedankt für das bereitwillige Eingehen auf all unsere Wünsche, insbesondere für die Bereitschaft, Frau Mills als Zeichnerin zu gewinnen.