[Ehlers-Danlos syndrome and pregnancy. Apropos of a case].
Ehlers-Danlos Syndrome is a hereditary dysplasia of connective tissue with an abnormality in collagen synthesis. The syndrome consists of increased elasticity and fragility of the skin, increased laxity of the ligaments of the joints and fragility of the blood vessels. It is rarely associated with pregnancy but when it is several problems arise which are demonstrated in our case. Our patient had a type III syndrome (hypermobility of the joints) or a type IV syndrome (echymoses, Sack-Barabas Syndrome). In the literature the following are reported: premature rupture of the membranes with premature delivery, tears, perineal haematomas and type IV ruptures of the great vessels of the uterus. The best way of delivering and anaesthetising the patient are discussed. The prognosis depends above all on the type of the disease (25% of mothers die in type IV disease). Strict rest and Pfannestiel lower segment Caesarean section operation under general anaesthetic allowed us to achieve a favorable outcome for the mother and for her child.
Charvet
P Y
PY
Service de Gynécologie-Obstétrique, Faculté de Médecine, Lyon Nord, Hôpital Claude-Bernard, Oullins.
Salle
B
B
Rebaud
P
P
Dutruge
J
J
Cordier
M P
MP
Pic
J C
JC
Touraine
J
J
Berland
M
M
Levrat
R
R
Tourniaire
J
J
D000328:Adult; D002585:Cesarean Section; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000503:physiopathology; D005260:Female; D006801:Humans; D011247:Pregnancy; D011248:Pregnancy Complications