Mixed Gonadal Dysgenesis (45 X0/46 XV Mosaicism): A case report

@#Disorders of sexual development (DSD) defined as congenital conditions associated with atypical development of anatomical, gonadal or chromosomal sex, is a rare condition that may present with ambiguous genitalia. Included in the varied classes of DSD is mixed gonadal dysgenesis which is known to be due to mosaicism, a chromosomal aberration. Mosaic individuals may have concerns on growth, hormone balance, gonadal development, sex of rearing and fertility. This case report presents an 18-year old student who presented with primary amenorrhea, delayed secondary sexual characteristics and phenotypic features of Turner syndrome who, on chromosomal analysis revealed 45X0/46XY mosaicism. The patient underwent operative laparoscopy with bilateral gonadectomy on the basis of the increased risk of development of gonadal malignancy in phenotypic females with Y-chromosome material. Histopathological analysis revealed bilateral streak gonads. Hormone replacement therapy was then initiated for the induction of secondary female sex characteristics, as treatment for estrogen deficiency, for the induction of pubertal growth spurt and for optimization of bone mineral accumulation. Management of disorders of sexual development is challenging, thus the need for a multidisciplinary approach involving experts in endocrinology, gynecology, psychology and genetics.

Practice of minimally invasive gynecologic surgery in the Philippines during the COVID-19 Pandemic / Philippine Journal of Obstetrics and Gynecology

Objective@#This study aims to establish baseline information on the practice of minimally invasive gynecologic surgery (MIGS) among Filipino gynecologic endoscopists amid the COVID-19 pandemic. @*Materials and Methods@#MATERIALS AND METHODS: An online survey was conducted among Fellows of the Philippine Society for Gynecologic Endoscopy (PSGE) practicing in private and government hospitals in the Philippines after informed consent. The survey had five subsections: (1) demographic data, (2) impact of COVID-19 pandemic on MIGS practice, (3) changes of practice during the COVID-19 pandemic, and (4) changes in the conduct of surgery and postoperative care. @*Results@#A total of 119 out of 144 PSGE Fellows based in the Philippines participated in the survey, 83% were Fellows in both laparoscopy and hysteroscopy. The majority had more than 15 years of practice and were practicing in the National Capital Region. Surgeries were canceled initially but have since resumed. The majority were hysteroscopy cases, the most common being polypectomy. Majority of the respondents reduced their clinic hours and appointments. Most have used telemedicine for consultations. Use of face masks, face shields, and personal protective equipment (PPE) were the top precautions taken in the clinics. Screening and precautions per guidelines inside the operating room setting were observed. Modifications during surgery include the use of smoke evacuators, minimizing energy device use, and wearing enhanced PPE.@*Conclusion@#The volume of laparoscopy and hysteroscopy cases was greatly reduced during the pandemic. The pandemic has disrupted the practice of MIGS both in the outpatient clinics and the operating rooms. Most of the changes made are congruent to local and international automotive task force guidelines. Precautionary measures and screening procedures must remain in place to reduce the risk of severe acute respiratory syndrome coronavirus 2 transmission to patients and health-care workers.

A variant of Herlyn Werner Wunderlich Syndrome

@#Herlyn Werner Wunderlich Syndrome is a rare congenital Mullerian anomaly that commonly presents with uterine didelphys, obstructed hemivagina and ipsilateral renal agenesis. Presented here is a rare variant with contralateral dysplastic kidney with ectopic ureteral insertion instead of renal agenesis. The patient initially presented as a case of recurrent pelvic inflammatory disease who eventually underwent excision of vaginal septum with drainage of pyocolpos. Postoperatively, the patient had urinary incontinence and was eventually referred to Urology for further surgical intervention. The wide range of symptomatology and the uncommon anatomic presentation of this case led to the delay in the diagnosis and the consequent gynecologic complications.