Transient neonatal arthrogryposis: Another case

✍ Scribed by Robinow, Meinhard; Miller, Marvin

• Publisher: John Wiley and Sons
• Year: 1996
• Tongue: English
• Weight: 84 KB
• Volume: 66
• Category: Article
• ISSN: 0148-7299
• DOI: 10.1002/(sici)1096-8628(19961230)66:4<475::aid-ajmg19>3.0.co;2-r

✦ Synopsis

In 1986, one of us [Robinow, 19861 described monozygotic diamniotic twin girls with the twin-twin transfusion syndrome. At birth, twin A, the transfusion recipient, had polyhydramnios of 2,000-3,000 ml. Her weight was 2.58 kg. She was in cardiac failure (tachypnea, hepatomegaly, gallop rhythm and generalized edema). An echocardiogram showed increased thickness of the septum and the left ventricular wall. Her muscles were unduly firm and rigid. Knees, hips and elbows could not be fully extended. WBC were 55,000, SGOT was 355 units, SGPT 1,005 units, CPK 2,200 units. Within 3 hours after birth, she voided more than 180 ml and after a few more hours all signs of cardiac failure had resolved. A week later the muscles had become softer and the flexion contractures had almost completely disappeared. SGOT had decreased to 36, SGPT to 71, and CPK to 21 units. She seemed to have fully recovered, but a few weeks later she was found to have mild spastic diplegia. Twin B, the transfusion donor, weighed 1.93 kg at birth and was asymptomatic.