Pregnancy in a woman with congenital adrenal hyperplasia with 11-beta-hydroxylase deficiency: A case report.

Background: Successful pregnancy with congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency is an extremely rare condition. Only two cases have been reported in the literature. Methods and results: Described here is a 30-year-old woman diagnosed as a neonate with congenital adrenal hyperplasia related to 11-beta-hydroxylase deficiency classic type, who subsequently underwent clitoral resection and vaginoplasty. She was started on lifelong steroid therapy after surgery. She developed hypertension at 11 years of age and was on antihypertensive therapy from then on. In later life, she underwent division of vaginal scar tissue and perineal refashioning. She spontaneously conceived but her pregnancy was complicated by severe pre-eclampsia and delivery was required at 33 weeks of gestation by cesarean section. A healthy male infant was delivered. Conclusion: Management of these women is similar to those with more common causes of congenital adrenal hyperplasia, with careful monitoring throughout pregnancy for complications such as gestational diabetes, gestational hypertension, and intrauterine growth restriction.

Decisions to Perform Emergency Caesarean Sections at a University Hospital: Do obstetricians agree?

OBJECTIVES: This study was undertaken to assess the degree of agreement amongst obstetricians regarding decisions to perform emergency Caesarean section (CS) procedures at a university hospital. METHODS: This retrospective clinical audit was carried out on 50 consecutive emergency CS procedures performed between November 2012 and March 2013 on women with singleton pregnancies at the Sultan Qaboos University Hospital in Muscat, Oman. Data on each procedure were collected from electronic patient records and independently reviewed by six senior obstetricians to determine agreement with the decision. RESULTS: Of the 50 women who underwent CS procedures, the mean age was 28.9 ± 5.1 years and 48% were primigravidae. A total of 65% of the CS procedures were category I. The most common indications for a CS was a non-reassuring fetal heart trace (40%) and dystocia (32%). There was complete agreement on the decision to perform 62% of the CS procedures. Five and four obstetricians agreed on 80% and 95% of the procedures, respectively. The range of disagreement was 4-20%. Disagreement occurred primarily with category II and III procedures compared to category I. Additionally, disagreement occurred in cases where the fetal heart trace pattern was interpreted as an indication for a category II CS. CONCLUSION: The majority of obstetricians agreed on the decisions to perform 94% of the emergency CS procedures. Obstetric decision-making could be improved with the implementation of fetal scalp pH testing facilities, fetal heart trace interpretation training and cardiotocography review meetings.

Ruptured Rudimentary Horn Pregnancy Diagnosed by Preoperative Magnetic Resonance Imaging Resulting in Fetal Salvage.

Pregnancy in a rudimentary horn is very rare. The rupture of the horn during pregnancy is an obstetric emergency which can be life-threatening for both the mother and fetus. Preoperative diagnosis of such pregnancies can be challenging and they are usually diagnosed intraoperatively. We report a unique case of a 31-year-old multiparous woman who presented to the Sultan Qaboos University Hospital in Muscat, Oman, in January 2013 at 32 gestational weeks with abdominal pain. Ultrasonography was inconclusive. A rudimentary horn pregnancy was subsequently diagnosed via magnetic resonance imaging (MRI). An emergency laparotomy revealed haemoperitoneum and a ruptured rudimentary horn pregnancy. A live baby with an Apgar score of 2 at one minute and 7 at five minutes was delivered. The rudimentary horn with the placenta in situ was excised and a left salpingo-oophorectomy was performed. The postoperative period was uneventful. The authors recommend MRI as an excellent diagnostic modality to confirm rudimentary horn pregnancies and to expedite appropriate management.

Successful term pregnancies after laparoscopic excision of poorly differentiated Sertoli-Leydig cell tumor of the ovary / 부인종양

Ovarian Sertoli-Leydig cell tumors are rare sex cord-stromal tumors, accounting for less than 1% of ovarian tumors. Majority of these tumors are benign and unilateral, only 3-5% are bilateral. These patients present with clinical features of virilization due to excessive secretion of testosterone from the tumor, however 50% may have no endocrine symptoms. We report a case of poorly differentiated Sertoli-Leydig cell tumour in a woman diagnosed during routine investigation of infertility. She had two spontaneous successful pregnancies after tumor excision laparoscopically.