Case report
Transumbilical laparoendoscopic single-site gonadectomy for Turner's syndrome with Y-chromosome mosaicism

https://doi.org/10.1016/j.jpurol.2012.02.010Get rights and content

Abstract

Laparoendoscopic single-site surgery (LESS), a minimally invasive procedure, is gaining widespread acknowledgment in pediatric urology. We report the case of a 7-year-old girl with Turner's syndrome with 45,XO/46,XY mosaicism, for whom bilateral prophylactic gonadectomy using LESS was performed. Histopathological findings revealed bilateral streak gonads. The surgical and cosmetic outcome was excellent. Diagnostic and therapeutic laparoscopy is essential for accurate clinical management of patients with disorders of sex development. Although this is only a single case report, it supports the theory that LESS is an exceedingly practical and superior technique for such children, since it provides excellent magnification, as well as allowing normal psychological development owing to the concealed scar. Further studies and long-term follow-up are required to evaluate the benefits and limitations of applying LESS in pediatrics.

Introduction

Turner's syndrome (TS) is a relatively common chromosomal disorder, affecting 1 in 2000 live female births [1]. Because more than 30% of patients with a Y-chromosome risk developing gonadoblastoma, the prophylactic gonadectomy of streak gonads is recommended [2].

The minimal invasive nature, absence of scars, efficacy and low morbidity make laparoscopy an optimal modality for the evaluation and treatment of patients with disorders of sex development (DSD). Recently, laparoendoscopic single-site surgery (LESS) has been performed in pediatric urologic surgery, accompanied by modifications to laparoscopic devices [3], [4]. In the present case, we performed single-site transumbilical laparoendoscopic gonadectomy for a girl with TS with Y-chromosome mosaicism.

Section snippets

Case report

A 7-year-old girl of short stature was presented to the pediatric outpatient department of our hospital. She was 103.6 cm (−2.5 SD) tall and weighed 16.0 kg (−1.5 SD). The external genitalia and psychological examination did not reveal any abnormality. Endocrine evaluation showed a high follicle-stimulating hormone level (70.5 mIU/mL; normal female 2.0–8.3 mIU/mL). G-banding showed a 45,XO/46,XY karyotype, and abdominal ultrasonography and MRI revealed a rudimentary uterus but no gonads. There

Discussion

We performed a bilateral gonadectomy with LESS in a case of TS with Y-chromosome mosaicism and obtained a clinically excellent outcome. LESS offers the advantage of reducing the number of abdominal incisions to one. While caring for patients with DSD, special attention should be given to their psychological development. Patients with DSD often have psychiatric vulnerabilities, such as anxiety or mood disorders, and suffer from gender identity disorder or gender dissatisfaction [5]. Because

Conclusion

We report a case of TS with Y-chromosome mosaicism where bilateral gonadectomy using LESS was performed. Diagnostic and therapeutic laparoscopy is essential for accurate clinical management of patients with DSD. Excellent magnification, as well as subsequent normal psychological development due to the concealed scar, renders LESS an exceedingly useful and superior technique in pediatric urology, especially for children with DSD.

Funding

None.

Conflict of interest

None.

Disclosure information

The authors have no conflicts of interest to declare.

References (5)

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