PEDIATRIC UROLOGY

A preliminary investigation into quality of life, psychological distress and social competence in children with cloacal exstrophy
Towell DMB, Towell AD
From the University of East London, Great Ormond Street Hospital for Children and University of Westminster, London, United Kingdom
J Urol. 2003; 169: 1850-3

• Purpose: Cloacal exstrophy is a complex multisystem anomaly. Due to ambiguous genitalia gender assignment or reassignment is common. The psychological, emotional and behavioral impact of this condition has rarely been investigated.
• Materials and Methods: We recruited 8 children with cloacal exstrophy born with genital ambiguity and a control group of 12 with cloacal anomalies born without genital ambiguity were recruited via urology-endocrine clinics at Great Ormond Street Hospital for Children. Patient age was 5 to 18 years (average 11.3). The child behavior checklist, child health related quality of life and social cognition questionnaire were administered to assess perceived levels of social competence and adjustment, emotional and behavioral distress, and perceived quality of life.
• Results: Social and behavioral competence as well as psychological problems were comparable with normative data for the 2 groups. There were no statistically significant differences in the 2 groups on any competence, problem or social adjustment scale. A quality of life measure again revealed no significant differences in the groups. The scores obtained were comparable with those reported for other chronic illnesses.
• Conclusions: Results suggest that being born with cloacal exstrophy or anomaly and gender assignment or reassignment does not necessarily result in childhood psychological, emotional or behavioral distress and/or problems, lower levels of social competence or subjective reports of poor quality of life. It is suggested that longitudinal and larger studies are required to assess the long-term implications of this condition.
  
• Editorial Comment
  This is an extremely important report of the psychological evaluation of children with cloacal exstrophy, with particular emphasis on 6 patients with 46 XY chromosomes who were raised as females. The researcher was blinded as to the chromosomal diagnosis in the cases and there was a control group of patients with cloacal anomalies, but not cloacal exstrophy or gender conversion. The authors found no statistical differences between the 2 groups, with scores comparable to those reported for other chronic illnesses.
  The gender of rearing is highly controversial in patients with cloacal exstrophy and 46 XY chromosomes. Because the phallic structures are usually too small to be adequately reconstructed as a normal male phallus, most patients have, in the past, been raised as females. On the other hand, they have already had their central nervous system “imprinted” with androgens prenatally. Recently, fears of gender dysphoria and ultimately reassignment (no good published reports in peer-reviewed journals), have led many physicians to recommend raising these children as males, despite the fact that they will have an inadequate phallus. Hence the development of these patients as normal females is of major interest. On the other hand, this study has several cautions. First, follow-up is still too short with a mean age of 11.6 years. Second, 5 of the 16 patients eligible refused to be interviewed and 3 of the others were undergoing psychiatric treatment and were deem ineligible. Data from these patients and follow-up through puberty could ultimately change the results. We anxiously await more information in this critical area.

Dr. Barry A. Kogan
Chief and Professor of Urology and Pediatrics
Albany Medical College
Albany, New York, USA