True Hermaphroditism and Cystic Teratoma in an Adult

The authors present the case of a patient with a 46, XX ovotesticular disorder of sex development diagnosed only in adulthood.[1] Cases like this one always result in a challenge for the treating team, as all treatment needs to be individualized. Since the 2005 Chicago consensus for disorders of sex development (DSD), the importance of a standardized terminology has become of significant relevance.[2] From an academic point of view, using the same technical terminology reduces confusion and helps the compilation of data for future studies. Authors fail to use standard terminology despite having the consensus as a reference. The frequency of these conditions makes management very challenging, and it is important to have specialized transdisciplinary teams, as this may improve the quality of care for these patients.[3] [4] [5] The authors present the chronological management for this patient, and it would be interesting to know the evolution and support given to the patient, from a psychosocial perspective, after surgery.[6] It would also be interesting to include in the discussion the rationale for the second laparotomy and how this was discussed with the patient.

I found it confusing that the authors support the possibility of 46, XX ovotesticular diagnosis concomitant with 46, XX congenital adrenal hyperplasia. Pathophysiologically, these two conditions cannot be linked together, and it is difficult to believe that they can present simultaneously. It would also be interesting to know the background of the patient with bilateral non-palpable gonads because currently, in Colombia, access to healthcare is a worrisome component for patients and families with complex conditions at birth.[7] About 50% of the patients with congenital urological anomalies are not being seen by any specialists after birth, and from of the other 50% that have an appointment assigned, 90% are still waiting to be seen for the first time by a specialist by the age of 3 (personal data, not yet published). Our urological community needs to become more active and protect our patients from late diagnosis, which could lead to serious medical and psychological implications, such as in the case presented by the authors.

Lastly, I would emphasize the importance of stating on the manuscript that the patient consented with the publication of his case and photographies, and I would also suggest submitting the consent as a supplementary document to be used in future publications.

• References

• 1 Ballestas C, Torres P, Ballesteros S, Alemán K. Hermafroditismo verdadero y teratoma quístico en adulto: reporte de caso. 2017; 26 (02) 135-139 . DOI: 10.1016/j.uroco.2017.04.001
• 2 Hughes IA, Houk C, Ahmed SF, Lee PA. ; Lawson Wilkins Pediatric Endocrine Society/European Society for Paediatric Endocrinology Consensus Group. Consensus statement on management of intersex disorders. J Pediatr Urol 2006; 2 (03) 148-162
• 3 Cools M, Looijenga LHJ, Wolffenbuttel KP, Drop SLS. Disorders of sex development: update on the genetic background, terminology and risk for the development of germ cell tumors. World J Pediatr 2009; 5 (02) 93-102
• 4 Fernandez N, Moreno O, Rojas A. , et al. Manejo transdisciplinario de pacientes con desórdenes del desarrollo sexual en Colombia: Limitantes para un manejo oportuno e integral. Urol Colomb. 2016; DOI http://dx.doi.org/10.1016/j.uroco.2016.06.004
• 5 De Paula GB, Barros BA, Carpini S. , et al. 408 Cases of Genital Ambiguity Followed by Single Multidisciplinary Team during 23 Years: Etiologic Diagnosis and Sex of Rearing. Int J Endocrinol 2016; 2016: 4963574
• 6 Lenkiewicz NE, Francisco J, Niño P, Ivett N, Mariño A. De genitales a géneros e intersexualidad: trastornos de la diferenciación sexual, un ámbito sociomédico. Urol Colomb. 2017; 26 (01) 1-7
• 7 Fernández N, Lopez C, Monsalve AM, Zarante I. Seguimiento telefónico de pacientes con malformaciones del tracto genitourinario en sus primeros dos años de vida. Urol Colomb. 2008; 17 (02) 9-14