Managing disorder of sexual development surgically: A single center experience

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From: Indian Journal of Urology(Vol. 28, Issue 3)
Publisher: Medknow Publications and Media Pvt. Ltd.
Document Type: Report
Length: 4,358 words
Lexile Measure: 1490L

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Byline: Jatinder. Kumar, Vikas. Kumar, Vijaylakshmi. Bhatia, Preeti. Dabadghao, Samit. Chaturvedi, Rakesh. Kapoor, M. Ansari

Introduction: Ambiguous genitalia are a major cause of parental anxiety and create psychological and social problems to patient, if not managed properly. Here we present our experience in managing patients with ambiguous genitalia. Material and Methods: We retrospectively reviewed clinical records of all patients with ambiguous genitalia managed surgically at out institute between December 1989 and January 2011. Relevant history, clinical examination , investigations and surgical procedures performed were analyzed and results were evaluated in terms of anatomical, functional and psychosexual outcomes. Results: Female pseudohermaphroditism was the most common cause of genital ambiguity in our patients. Male and female genitoplasty was done according to gender of rearing, genital anatomy and parental choice. Twenty six patients (86.6%) reported satisfactory cosmetic outcome and 22 (73.3%) satisfactory functional outcome on long term follow-up. Among the 24 patients diagnosed as male pseudohermaphroditism 14 (82.3%) patient have reported satisfactory cosmetic outcome and 13 (76.4%) have reported satisfactory functional outcome. In patients with mixed gonadal dysgenesis and true hermaphroditism satisfactory cosmetic and functional outcome was seen in 70% patient. Conclusion: Managing patients of genital ambiguity according to gender of rearing, genital anatomy and parental choice carries good prognosis in terms of anatomical, functional and psychosexual outcome.

Introduction

Individuals with a congenital discrepancy between external genitalia, gonadal, and chromosomal sex are classified as having a disorder of sexual development (DSD). [sup][1] Whenever there is difficulty in attributing gender to child based on appearance of external genitalia, they are termed "ambiguous genitalia." The appearance of external genitalia is a result of complex interaction between genetic and endocrine processes during fetal development. [sup][1],[2] Abnormalities of external genitalia sufficient to warrant genetic and endocrine studies are said to occur in 1 in 4,500-10,000 births. [sup][3],[4] Diagnosis and management of this condition can be challenging requiring a multidisciplinary approach. [sup][5] Ambiguous genitalia are a major cause of parental anxiety and can create psychological and social problems to the patient, if not managed properly.

Data on disorder of sex development from Indian subcontinent are limited. The purpose of this article is to highlight the various surgical options available and surgical outcome on long-term basis. Herein, we present our experience in surgical management of patients with DSD.

Materials and Methods

The clinical records of all patients with ambiguous genitalia managed surgically at out institute between December 1989 and January 2011 (N=88) were retrospectively reviewed. After thorough history and clinical examination, patient had undergone chromosomal analysis, relevant blood and urinary steroid estimation, abdominal and pelvic ultrasound, as well as retrograde genitogram as indicated. The genitogram was performed by low pressure injection of a water-soluble contrast agent through Foley catheter, inserted into the very distal part of the common perineal opening with care and repeated fluoroscopy in order to identify the perineal internal anatomy. Cystoscopy was done just before the surgery to confirm the findings. The frequency of various disorders according to the classification system utilized by Grumbach and Conte...

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Gale Document Number: GALE|A309358355