Pregnancy and Delivery in Ehlers-Danlos Syndrome (Hypermobility Type): Review of the Literature

Ehlers-Danlos syndrome (EDS) is a group of connective tissue disorders which are divided into various distinguishable phenotypes. The type of EDS determines the potential obstetric complications. Due to the spectrum of clinical manifestation and overlap between phenotypes, there are no standardised obstetric management guidelines. Existing literature illustrates different obstetric management in hypermobility type of EDS, including uneventful term vaginal deliveries as well as preterm cesarean section deliveries. This paper discusses obstetric management of a woman with EDS hypermobility type. Cesarean section was deemed the most appropriate delivery method in this patient due to the possible complications including risk of joint dislocation and pain morbidity. No obstetric complications were experienced, and good maternal and neonatal outcomes were achieved. 1. Introduction The first comprehensive clinical description of Ehlers-Danlos Syndrome (EDS) was completed by Dr. Tschernogobow in 1892. The syndrome derives its name from a Danish dermatologist, Edward Ehlers in 1901, and a French physician and dermatologist, Henri-Alexandre Danlos in 1908. Later, clinical diagnostic criteria for the types of EDS were proposed by Beighton in 1998. EDS is a rare inherited disorder of connective tissue, characterised by a collagen synthesis defect. The group of related conditions share a common decrease in the tensile strength and integrity of the skin, joints, and other connective tissues. All forms of EDS share common features to varying degrees including joint hypermobility, skin hyper extensibility, tissue fragility, poor wound healing, and easy bruising [1]. Complications relating to EDS are infrequently seen in obstetric practice [2]. It presents with a range of considerations, which are specific to the classification of type. Some types are associated with severe maternal complications, whereas others are associated with more favourable outcomes [1]. There are at least six distinguishable phenotypes; however, overlap between these types is significant, making clinical diagnosis complex. The types include classic, hypermobility, vascular, kyphoscoliosis, arthrochalasia, and dermatosparaxis. Hypermobility type (formerly type III) is considered the least severe type of the syndrome, although important musculoskeletal complications can occur. It is inherited in an autosomal dominant pattern although the causative gene remains unidentified. Diagnosis of this type is based on clinical assessment alone. Major diagnostic criteria include joint hypermobility, skin