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.

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.

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.