Nabeela Ata, Samee Akhtar.
Ehler Danlos syndrome with pregnancy
Professional Med J Jul - Sep 2006;13(3):476-7.
Nishtar Hospital, Multan

A 32 years old lage, G5 POA4 was admitted with gestational amenorrhea of 20 weeks with the history of fall on the same day, as an emergency case on 13-5-2004. On examination, abdomen was soft, consistent with period of gestation, no marks of external injury was noted all over the body, except 3 bruises over her arm and forearms. On investigation, her blood group was found to he A +ve, Hb 10.5% and platelets count 18200 lakhs. Rest of biochemical investigations were normal except her prolonged APTT and PT. Sonographic finding showed single active fetus of 20 weeks of fetal biometery. There is an episode of bleeding vaginally, which was mild. Her echocardiography was normal, but she was given Tab. Lasoride and digoxin but had only symptomatic relief. She was diagnosed as a case of Ehler Dalson syndrome, on the basis of hypermobile joints, high arched palate, bruises, due to fragile blood vessels, proximal muscle weaken. The final management planned was to built up her Hb%, omit digoxin and lasoride, having one pint of blood in hand, termination is planned after counseling the patient. As the blood arranged, at 26 week of gestation repairing initiated with prostaglandins. She did not start any uterine contraction, it was repeated again and after the failure of change in chemical status establishment of uterine contraction, her caesarean section was planned. It was done on list, in the presence of senior anaesthetist and obstetrician. She delivered a baby girl of 0/10 A/S weighing 1.5 kg. Bilateral tubal ligation was done. She did well in her postoperative period except dyspnoea, which resolved spontaneously.

Category: Case Reports
Keywords: Ehler Danlos Syndrome. Pregnancy.

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