Case reportTransumbilical laparoendoscopic single-site gonadectomy for Turner's syndrome with Y-chromosome mosaicism
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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Cited by (8)
Multi-institutional experience in laparoendoscopic single-site surgery (LESS): For major extirpative and reconstructive procedures in pediatric urology
2016, UrologyCitation Excerpt :Mean OR time was 145 minutes, with two patients having a postoperative leak that spontaneously resolved. Recent studies expanded use of LESS to include gonadectomy for mosaic Turner syndrome,20 orchidopexy,21 and heminephrectomy.22 Our initial experience has been with GelPoint (Applied Medical) standard size, but since then GelPoint Mini has become available.
Laparoendoscopic single-site surgery
2020, Minimally Invasive and Robotic-Assisted Surgery in Pediatric UrologySingle-Site Laparoscopy and Robotic Surgery in Pediatric Urology
2018, Current Urology ReportsProphylactic gonadectomy in children with mixed gonadal dysgenesis - A nine-year-old girl
2018, Paediatria CroaticaDiagnostic procedures for patients with non-palpable testis: Physical examination and diagnostic imagings
2018, Japanese Journal of Clinical Urology