Introduction: Swyer's syndrome is a distinct type of pure gonadal dysgenesis characterized by a 46 XY karyotype in female phenotypic patients. It shows an abnormality in testicular differentiation. Objective: To present cases of Swyer syndrome.Material and methods: We present the clinical, sonographic, endocrine findings, genetic analyses and treatment in two cases of phenotypic females with XY karyotype and gonadal dysgenesis.Results: All patients presented with primary amenorrhea. All patients had female-type external genitalia. Secondary sexual characters were developed in all cases. FSH levels were high. Chromosome analyses revealed a 46, XY male karyotype with no detectable mosaicism. The surgical findings were steak gonads, one of them with bilateral gonadoblastoma. Intervention(s): Bilateral gonadectomy followed by hormone replacement therapy. Conclusion:We aimed to underline the necessity of considering 46, XY complete pure gonadal dysgenesis in the differential diagnosis in every adolescent female patient with delayed puberty and the importance of early gonadectomy in order to avoid the risk for gonadal tumor development.

A 25-year-old woman developed HELLP syndrome and antepartum eclamptic fit complicated with ruptured subcapsular liver hematoma during the 28th week of pregnancy. Intervention: Midline abdominal exploratory laparotomy, with delivery by caesarean section. Tight abdominal packing for the hematoma and Pringle maneuver were done. Partial couvelaire uterus was managed by prostaglandins and B-Lynch brace sutures to minimize uterine bleeding and atony. The patient developed postoperative hepatic, renal failure, coagulopathy, deterioration and finally death. Conclusion(s):Ruptured subcapsular liver hematoma is a life-threatening condition that should be considered in pregnant women with HELLP syndrome and severe preeclampsia presenting with symptoms and signs of hemorrhagic shock, hemoperitoneum and the liver should be evaluated with ultrasound before delivery. In these patients delivery of the fetus is the first step and the best approach is a midline abdominal incision. Also, regular antenatal care is very important through all trimesters.

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