Controversy concerning the most appropriate treatment guidelines for intersex children currently exists. This is due to a lack of long-term information regarding medical, surgical, and psychosexual outcome in affected adults. We have assessed by questionnaire and medical examination the physical and psychosexual status of 14 women with documented complete androgen insensitivity syndrome (CAIS). We have also determined participant knowledge of CAIS as well as opinion of medical and surgical treatment. As a whole, secondary sexual development of these women was satisfactory, as judged by both participants and physicians. In general, most women were satisfied with their psychosexual development and sexual function. Factors reported to contribute to dissatisfaction were sexual abuse in one case and marked obesity in another. All of the women who participated were satisfied with having been raised as females, and none desired a gender reassignment. Although not perfect, the medical, surgical, and psychosexual outcomes for women with CAIS were satisfactory; however, specific ways for improving long-term treatment of this population were identified. (J Clin Endocrinol

Routine neonatal assignment of genetic males to female sex because of severe phallic inadequacy can result in unpredictable sexual identification. Clinical interventions in such children should be reexamined in the light of these findings.

OBJECTIVETo identify genetic and nongenetic factors contributing to the risk of bladder exstrophyepispadias complex (BEEC). PATIENTS AND METHODSIn all, 285 families with BEEC were invited to participate in the study, and 232 of them were recruited. Epidemiological information was obtained from 151 of the consenting families, with a detailed clinical genetic examination of 94 probands. In all, 440 DNA samples were collected from 163 families for molecular analysis.

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The authors from Copenhagen write about their 15‐year consistent strategy in the treatment of antenatally suspected PUJ obstruction. The group deals with this controversial subject in some detail, and they outline data which they feel are helpful for urologists giving advice to parents about the advisability of having the condition treated by operative or conservative means. The Cohen technique for the treatment of bilateral VUR, using a common submucosal tunnel, over an 18‐year period is described by authors from Athens; they found that the technique offers excellent long‐term results, and that crossing one ureter upon the other within the tunnel does not predispose to obstruction. OBJECTIVE To review of the sexual and urogynaecological issues faced by a large cohort of women with the exstrophy‐epispadias complex (EEC). PATIENTS AND METHODS The study comprised 83 women and girls with EEC; a confidential survey was mailed to identify their social and sexual concerns. Fifty‐six women had classical bladder exstrophy (CBE), 13 had female epispadias (FE) and 14 had cloacal exstrophy (CE). Data on the initial method of reconstruction and urogynaecological problems were obtained from a review of the hospital records. Information on continence, infection and sexual function was obtained from 34 completed surveys. RESULTS The bladder was closed in 51 patients with CBE and 13 with CE. Urinary calculi developed in 10 patients with CBE, two with FE and three with CE. Vaginal and uterine prolapse occurred an earlier age in patients with EEC. Eight women had 13 pregnancies, eight of which resulted in normal healthy children. Overall continence was achieved in 85% of the women surveyed. Urinary tract infections remained a frequent problem for women with EEC; only 27% of respondents indicated that they were infection‐free. Women aged> 18 years (24) who responded indicated that they had appropriate sexual desire; 16 were sexually active and the mean age for commencing sexual activity was 19.9 years. Six patients had dyspareunia and 10 indicated that they had orgasms. However, five additional patients indicated that they had restricted intercourse, as they were dissatisfied with the cosmesis of their external genitalia. CONCLUSIONS Sexual and gynaecological issues become increasingly important in patients with EEC as they become adults. Understanding these issues faced by patients with EEC as they mature will permit better counselling of future patients.

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Purpose-To identify causative non-genetic and genetic risk factors to the bladder exstrophyepispadias complex (BEEC).Materials and Methods-237 BEEC families were invited to participate and information was obtained from 214 families, mainly comprising European countries.Results-Two families showed familial occurrence. Male predominance (p 0.001) was found among all BEEC subgroups comprising epispadias (E), classical exstrophy of the bladder (CBE) or cloacal exstrophy (CE), with male to female ratios of 1.4, 2.8, and 2.0, respectively. No association with parental age, maternal reproductive history or with periconceptional maternal exposure to alcohol, drugs, chemical noxa, radiation or infections was found. However, periconceptional maternal exposure to smoking (p 0.009) was significantly more common for CE patients than for the combined group of E/CBE patients. Only 16.8% of mothers followed the current recommendations *Correspondence to: Michael Ludwig, Ph.D., Dept. of Clinical Biochemistry and Clinical Pharmacology, University of Bonn, SigmundFreud-Str. 25, D-53105 Bonn, Germany. E-mail: E-mail: mludwig@uni-bonn.de. Publisher's Disclaimer: This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. NIH Public Access NIH-PA Author ManuscriptNIH-PA Author Manuscript NIH-PA Author Manuscript of periconceptional folic acid supplementation. 17.6% had started supplementation before the 10 th week of gestation. Interestingly, in the latter group, mothers of CE patients were more compliant (p 0.037) than mothers of the combined group of E/CBE patients. Furthermore, mothers of CE children knew significantly more often prenatally that their child would have a congenital malformation (p < 0.0001) than mothers of E/CBE children.Conclusions-Our study corroborates the hypothesis that E, CBE and CE are causally related, representing a spectrum of the same developmental defect, with a small risk of recurrence within families. Embryonic exposure to maternal smoking appears to enforce the severity, whereas periconceptional folic acid supplementation does not seem to alleviate it. There is a disproportional prenatal ultrasound detection rate between severe and mild phenotypes, possibly due to the neglect of imaging of full urinary bladders with focus on neural tube defects.