Successful treatment with the Smith-Hodge pessary of cervical incompetence due to defective connective tissue in Ehlers-Danlos syndrome.

A case of a woman with Ehlers-Danlos syndrome who was able to carry pregnancy to term with bed rest and a Smith-Hodge pessary is described. The 22-year old black woman who had been diagnosed with Ehlers-Danlos syndrome at age 10 was referred at 14 weeks gestation. Her diagnosis was based on history of hypotonia in infancy easy bruising hypermobile joints kyphoscoliosis hyperelastic skin and microcorneas. She had marked kyphoscoliosis severe varicose veins a long closed cervix and severe restrictive lung disease. She was diagnosed with cervical incompetence based on a previous miscarriage. A skin biopsy was performed and severe type IV Ehlers-Danlos disease was ruled out. It was decided not to do cervical cerclage because of the risk of tears. Instead the patient was treated with bed rest at home and a Smith-Hodge pessary which she removed and washed twice daily. At 29 weeks gestation the cervix was dilated 1 cm the fetus was ballottable in vertex and there were no contractions. The woman was hospitalized for bed rest given 12 mg betamethasone im every 24 hours for 2 days then 12 mg weekly. At 33 weeks gestation the cervix had dilated to 5 cm the membranes had ruptured and contractions began. She was delivered of a 1470 gm male over a small midline episiotomy. The total time in labor was 4 hours. Blood loss was 250 cc. The episiotomy scar healed well. Both epidural and general anesthesia would have been contraindicated in this patient because of her vertebral deformities and her lung disease. The infant had Apgar scores of 7 and 9 and 1 and 5 minutes and was normal.