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  • Title: Successful treatment with the Smith-Hodge pessary of cervical incompetence due to defective connective tissue in Ehlers-Danlos syndrome.
    Author: Leduc L, Wasserstrum N.
    Journal: Am J Perinatol; 1992 Jan; 9(1):25-7. PubMed ID: 1550628.
    Abstract:
    Ehlers-Danlos syndrome (EDS) is a heterogeneous group of inherited connective tissue disorders with at least ten distinct types. We report a case of EDS complicated by cervical incompetence. Treatment was undertaken after confirmation that the patient's EDS was not type IV, hence not associated with potentially serious vascular complications. Because of concern about stretching or tearing of the hyperextensible connective tissue associated with EDS, cerclage was not performed. The cervical incompetence due to defective connective tissue was treated with a Smith-Hodge pessary. 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.
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