Special Issue Article
Pages: 1 - 1Raghad Aljishi
Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly characterized by triad of uterus didelphys, obstructed hemi vagina, and ipsilateral renal agenesis. The most common presentation is abdominal pain, dysmenorrhea, and abdominal mass secondary to hemi-hematometrocolpos. We report an emergency presentation of this syndrome during the pandemic of COVID-19 in Qatif Central Hospital, Saudi Arabia, April 2020. An 11-year-old Saudi girl presented to the emergency room with three months history of cyclical lower abdominal pain. The pain was progressive, continuous and not relieved by analgesics. Abdominal examination revealed a tender abdominal mass mainly in the left iliac fossa, Ultrasound evaluation showed two uterine bodies. The left uterus was distended with complex fluid. Pelvic MRI findings consistent with Herlyn-Werner-Wunderlich (HWW) syndrome were found with uterine didelphys and left sidedhematometra resulting from obstructed Hemi-vagina and ipsilateral agenesis of the left kidney. Resection of the vaginal septum and drainage of hematometra was done. The patient recovered with normal cyclical menstruation. Cyclical or continuous lower abdominal pain with or without amenorrhea is the usual presentation of HWW syndrome during adolescence. Diagnosis is made by ultrasonography and MRI. Early diagnosis and accurate management can provide pain relief, prevent future complications, and preserve fertility.
Special Issue Article
Pages: 2 - 2Armand Kamga Talom*, Florent Fouelifack Ymele, Edmond Mesumbe Nzene, Jeanne Fouedjio, Pascal Foumane
Objectives: The objective of this work was to determine the epidemiological, clinical and prognostic aspects of maternal mortality (MM) in the city of Yaounde. Methods: We conducted a descriptive cross-sectional study with retrospective data collection of maternal deaths according to the World Health Organization (WHO) definition, from January 1, 2017 to December 31, 2019, in two tertiary centers of Yaounde: The Yaounde Gyneco-Obstetric and Pediatric Hospital (YGOPH) and the Yaounde Central Hospital (YCH). Results: During the study period, 208 maternal deaths were identified, with 4/5 (78.4%) from referred cases. The MM ratio was 1532.8 per 100,000 live births (LB) vs. 609.5 per 100,000 live births at YCH and YGOPH respectively. The Main causes where hemorrhage (49%) followed by hypertensive diseases and their complications (21.2%), maternal mortality was associated with 50% of cases of foetal or neonatal deaths (57.4% in YCH vs 28.3% in YGOPH, P < 0.001). Health service systems were related to MM. Conclusion: Our study shows a large increase in maternal mortality ratios in YCH and YGOPH maternities since 2017. The main causes of MM found were hemorrhage followed by hypertensive diseases. These causes can be prevented. Our health systems should be improved if we want to significantly reduce the maternal mortality ratio
Special Issue Article
Pages: 3 - 3Kavya
Gonadal dysgenesis refers to various clinical conditions in which abnormal development of the fetal gonad is present. It consists of 46 XY gonadal dysgenesis, mixed gonadal dysgenesis, and 45 X turner syndrome. Mixed gonadal dysgenesis is a condition of unusual and asymmetrical gonadal development leading to unassigned sex differentiation. A number of differences have been reported in the karyotype, most commonly a mosaicism 45 X / 46 XY.1 We herein report an interesting and rare case of mixed gonadal dysgenesis in a 23-year-old nulliparous unmarried woman. Case: A 23-year-old nulliparous unmarried woman with primary amenorrhea with short stature, webbed neck, breast developed according to Tanner’s stage was presented to our clinic. She had normal external genitalia with axillary and absence of pubic hair. The evaluation of her uterus on ultrasound revealed a hypoplastic uterus measuring 2.6 x 1.4 x 0.9 cm with poorly formed endometrium and myometrium with bilateral ovaries being small and hypoplastic. Her follicle-stimulating hormone (FSH), testosterone, luteinizing hormone, and anti-mulleins hormone levels was 120 IU/L, 15 ng/dL, 24.5 IU/L and 0.02 ng/Ml respectively. Karyotype test showed mosaicism mixed gonadal dysgenesis, i.e., 45 X0 and 46 XY. She underwent laparoscopic bilateral salpingectomy (gonadectomy and salpingectomy).The postoperative period was uneventful. Intraoperatively streak gonads with elongated tubes were visualized. An infantile uterus with no pelvic abnormality was also observed intraoperatively. The histopathology report showed a cut dissection of ovaries. Discussion: Mixed gonadal dysgenesis (MCG) is a rare intersexual disorder, characterized by the presence of a testis and a contralateral streak gonad; in some cases the contralateral gonad may be rudimentary not having differentiated into an ovary or into a testis and in other cases it may be absent. On evaluation of 23-year-old nulliparous unmarried women’s uterus on ultrasound revealed a hypoplastic uterus measuring 2.6 x 1.4 x 0.9 cm with poorly formed endometrium and myometrium with bilateral ovaries being small and hypoplastic. Karyotype investigation revealed mosaicism mixed gonadal dysgenesis, i.e., 45 X0 and 46 XY. Johansen and coworkers reported structural rearrangement of the Y chromosome in 63% of mixed gonadal dysgenesis patients.2,3 Arora R et al reported that in 16-year-old person, karyotype analysis revealed 46XY karyotype and diagnosed with 46XY mixed gonadal dysgenesis.4 With this study, treatment recommended was laparoscopic bilateral salpingectomy (gonadectomy and salpingectomy). Sheela S et al also found similar kind of presentation where right sided gonads and adjacent tubal structures were visualized laparoscopically and performed gonadectomy.5 Similarly, Yadav P et al reported right streak ovary, left sided fallopian tube and streak ovary were noted in exploratory laparoscopy and later it was excised by doing gonadectomy.6. Conclusion: Gonadectomy was done as a prophylaxis measure in a 23-year-old nulliparous unmarried woman presented with gonadoblastoma or dysgerminoma. It was learned from the experience of the present study that patients with mixed gonadal dysgenesis have a different presentation and variant of chromosomal abnormalities. A multidisciplinary approach is essential for early diagnosis and proper management of patients diagnosed with rare mixed gonadal dysgenesis in order to prevent mental and social sequels
Special Issue Article
Pages: 4 - 4Hawa Obeid
Background: Diagnosis of ectopic pregnancy prior to rupture is an arduous task even with the availability of many new investigative methods and imaging modalities. Above all, a high index of suspicion is necessary when dealing with woman who present in early pregnancy with abdominal pain and vaginal bleeding. The use of transvaginal ultrasonography (TVS) will help in earlier diagnosis because of it is advantages over transabdominal ultrasosnography (TAS). Case presentation: A 36 years old woman, G2P0+1 presented at POA of 5 weeks and 5 days with history of an acute abdominal pain for 3 days’ duration and PV bleeding for 1 day. She has a history of primary infertility due to female factors and a laparoscopic ovarian drilling was performed 5 years back. A 2cm uterine fibroid was also noted during that laparoscopy. Subsequently, 3 IUI were performed the following two years but all were failed. She was also diagnosed with Diabetes Mellites one month ago and it is currently well controlled with diet, Metformin and Glibencarmide. Upon examination, she had tenderness of left iliac fossa. There was no palpable abdominal mass. A transvaginal scan done and revealed an empty uterus, no free fluid, no adnexal mass, complex mass at Pouch of Douglas size of 3.3?3.6cm. On review the next day, transvaginal scan repeated with finding of right ovarian cyst with solid cystic component and left adnexal mass size of 3.5?3.5cm. An laparpscopic was done, with operative finding of left tubal pregnancy over the lenght of fallopian tube (4?2cm), left fimbriae cyst of 1?1cm, subserosal fibroid at fundus (8?5cm) and intramural fibroid (3?3cm), right paratubal cyst (3?1cm) containing strawed coloured fluid, minimal adhesion seen over right pelvic wall with bowel, both ovaries were normal, bowel and liver were normal. A left sulphingectomy was carried out. Postoperative period was uneventful. Conclusion: Ectopic pregnancy carries a high morbidity and mortality partly due to the difficulty in establishing and early diagnosis prior to the rupture of the ectopic gestation. A high index of suspicion is necessary. With the recent advances in ultrasonography particularly transvaginal sonogram, diagnosis is made easier and more accurate.
Special Issue Article
Pages: 5 - 5Kristyna Helenicka
It is well known that the incidence of Adenomyosis and uterine fibroids increases significantly with age. In times when the first pregnancy is postponed into the fourth or even fifth decade, there is a growing demand for fertility saving procedures for these uterine diseases. To date, the literature contains a limited number of reports describing the reproductive outcomes of patients after adenomyomectomy for severe diffuse adenomyosis, and no report has compared the reproductive, obstetrical, and surgical outcomesbetween the two different uterus-sparing surgical procedures, namely, adenomyomectomy and intramural myomectomy. Therefore,we have tried to investigate it. Two groups of women who underwent different fertility-saving procedures were compared. Thepregnancy and delivery rates were 52.0% and 43.5%, respectively, in adenomyosis group versus 96.0% and 70.8%, respectively, infibroid group, with no significant differences between the two groups, except for pregnancy rate. The perinatal outcomes of the groupswere also comparable, including the low frequency of severe peripartum complications, such as the abruption or abnormal invasion ofthe placenta. The pregnancy rate was significantly lower in the group with severe form of adenomyosis. It appears, that the severity ofthe disease and the extent of the surgical resection of the uterus may influence the likelihood of a successful gestation. The clinicaloutcomes of women after cytoreductive resection of adenomyosis seems to be comparable with the different types of fertility sparingsurgery on uterine muscularity, namely myomectomy. Despite the technical challenges resulting in longer operation times and a higheconversion rate in comparison witmyomectomy, cytoreductive resection of adenomyosis seems to be feasible option for women withsevere adenomyosis and reproductive plans.
Special Issue Article
Pages: 6 - 6Evgeniya V Kirakosyan*, Tatyana A. Nazarenko, Stanislav V. Pavlovich
Introduction: The continuous development of assisted reproductive technologies (ART) has led to the fact that now all forms of male and female infertility have been overcome, however, the effectiveness of using ART methods remains limited and is considered to be approximately 30%. Why is this so? Purpose: Determine the possible role of genetic, immunological causes, disorders of intra-ovarian steroid?- and folliculogenesis, leading to the formation of an oocyte defect, mitochondrial dysfunction, which, in turn, can contribute to the disruption of early embryogenesis. Materials and methods: The review of world literature was conducted in the databases Scopus, Web of Science, MedLine, Cochrane CENTRAL, Cochrane Database of Systematic Reviews (CDSR), Database of Abstracts of Reviews of Effectiveness (DARE), EMBASE, Global Health, CyberLeninka, RSCI for a comprehensive study of the contribution of various factors to the development of reproductive system disorders. Results: The findings are promising, but further research is needed to study the etiopathogenesis of reproductive system disorders in order to optimize the algorithm of diagnosis and treatment. Particular attention is paid to the most demonstrative clinical model of reproductive system disorders – infertility of unknown origin when the reproductive system appears anatomically and functionally normal, but conception does not occur. Conclusion: We present the algorithm that is currently relevant from a clinical point of view for the diagnosis and treatment of patients with infertility of unknown origin.
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