Prenatal treatment of fetal hydrops associated with the hypertelorism-dysphagia syndrome (Opitz-G syndrome).
Non-immunological fetal hydrops diagnosed prenatally presents a difficult diagnostic and therapeutic problem. In the case presented, fetal hydrops was recognized at 19 weeks gestation and no specific cause was found prenatally in spite of extensive investigations. The fetal hydrops was treated in utero by thoracocentesis and an intravenous infusion of albumin carried out at fetoscopy. After birth the infant was recognized to have the hypertelorism-dysphagia syndrome (or Opitz-G syndrome, McK no. 30710). This autosomal dominant syndrome consists of hypertelorism, laryngeal abnormalities, swallowing difficulties, hyprospadias and an imperforate anus. Fetal hydrops has been reported on one previous occasion in this syndrome. The intrauterine treatment given in this case may have been successful in reducing the neonatal complications of the Opitz-G syndrome.
D000328:Adult; D000418:Albumins / Q000008:administration & dosage / Q000627:therapeutic use; D001848:Bone Diseases, Developmental / Q000150:complications*; D003680:Deglutition Disorders / Q000150:complications*; D004487:Edema / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*; D005260:Female; D005332:Fetoscopy; D006801:Humans; D006972:Hypertelorism / Q000150:complications*; D007223:Infant; D007231:Infant, Newborn; D007263:Infusions, Parenteral; D008297:Male; D011247:Pregnancy; D011677:Punctures; D014463:Ultrasonography
D000418:Albumins; C028426:hyperoncotic albumin