Synchronous bilateral ovarian torsion followed by recurrent unilateral torsion in a patient undergoing fertility treatment: A case report and literature review.

BACKGROUND: Ovarian torsion (OT) is a gynaecological emergency and requires prompt recognition and treatment in order to prevent the loss of ovarian function. Patients who are undergoing fertility treatment are at an increased risk of developing OT. OBJECTIVE: The diagnosis of OT in patients undergoing fertility treatment can be challenging as they often present with abdominal pain and other non-specific symptoms. We highlight the importance of early diagnosis of suspected torsion and performed a literature review on cases of bilateral OT to review its presentation, investigation, and subsequent management. CASE REPORT: A 32-year-old nulliparous woman who was undergoing controlled ovarian stimulation presented with lower abdominal pain and was initially managed as ovarian hyperstimulation syndrome (OHSS). Her pain did not subside following conservative management and she proceeded to have a laparoscopy which demonstrated synchronous bilateral ovarian torsion (SBOT), both ovaries were detorted. Eight months later, she was preparing for her frozen embryo transfer (FET) cycle, patient again presented with significant right sided abdominal pain and was found to have a recurrent torsion of the right ovary which was again detorted successfully. CONCLUSIONS: Clinicians should have a low-threshold to investigate and rule out OT in patients who present with lower abdominal pain, especially in those with additional risk factors for torsion. Patients with confirmed torsion can be successfully managed with detorsion of the ovaries. Further research is needed to determine the best management option for patients with recurrent torsion episodes.

Hypermobile Ehlers-Danlos syndrome and pregnancy.

Ehlers-Danlos syndromes are a clinically and genetically heterogeneous group of rare inherited connective tissue disorders. Hypermobile Ehlers-Danlos syndrome is one of the common types and not infrequently encountered in pregnancy. While, in the majority of women with hypermobile Ehlers-Danlos syndrome, the pregnancy is uncomplicated, it is important to be aware of the condition in view of potential complications such as recurrent joint dislocations and history of surgical joint stabilization procedures, secondary autonomic pain and postural orthostatic tachycardia syndrome. Increased awareness of the condition and a multi-disciplinary approach to the management of these women in pregnancy result in good outcome for the mother and the baby. We report the clinical characteristics and outcome of pregnancies in eight women with hypermobile Ehlers-Danlos syndrome and present a review of the literature with particular reference to management in a pregnant woman with joint hypermobility syndrome.

Successful pregnancy outcome following maternal intravenous immunoglobulin treatment in a woman with recurrent perinatal haemochromatosis.

OBJECTIVE: We report a case of successful pregnancy outcome following maternal intravenous immunoglobulin treatment in a woman with previous history of recurrent fetal hydrops secondary to perinatal haemochromatosis. METHODS: A 32-year old woman had two successive pregnancies complicated by fetal hydrops and perinatal deaths. Pathological examination of the fetus showed severe liver destruction with siderosis of hepatocytes at extrahepatic sites, but sparing of the reticulo-endothelial elements, consistent with the diagnosis of perinatal haemochromatosis. In the subsequent pregnancy, maternal intravenous immunoglobulin was administered weekly from the 18th week of gestation until delivery by elective caesarean section at 38 weeks. The infant was treated with desferrioxamine, N-acetylcysteine, vitamins K and E. RESULTS: The infant was born in good health, but had high serum ferritin levels, markedly elevated percent transferrin saturation, and mild transient derangement of liver and coagulation function. The infant made an excellent recovery and the treatment was stopped at 7 weeks of age. The liver and coagulation parameters and the serum ferritin levels returned to normal values. CONCLUSIONS: Haemochromatosis should be considered in the differential diagnosis of hydrops fetalis. The recurrence risk is high, and immunomodulation with intravenous immunoglobulin treatment appears to alter the course of the disease with better infant survival.

Recurrent fetal hydrops due to mucopolysaccharidoses type VII.

Hydrops fetalis is associated with a wide range of abnormalities. In about 20% of cases of non-immune fetal hydrops, no cause is found despite investigations including routine post-mortem examination and enzyme studies may be indicated to detect an underlying metabolic storage disease. Fetal hydrops due to mucopolysaccharidosis type VII is very rare and a prenatal diagnosis is not usually made. We report a case of mucopolysaccharidosis type VII presenting as recurrent fetal hydrops and review the clinico-pathological features of this disorder.