The Coffin-Lowry syndrome: a case report and review of the literature.

BACKGROUND: Prenatal ultrasound has afforded insights into many structural and syndromic fetal disorders. In this report, the ultrasound findings were of assistance when counselling a patient who presented with a history of Coffin-Lowry syndrome (CLS). CASE: A 39-year-old woman presented in the third trimester of pregnancy asking whether CLS could be diagnosed in utero. Three of her male offspring had been found to have this syndrome in childhood. Ultrasound assessment of the fetus was able to confirm female sex, which provided direction for counselling. An additional finding was of short and stubby digits, which have been well described as part of this syndrome. This information provided direction for pediatric management. CONCLUSION: CLS is a rare syndrome that is typically diagnosed in childhood. To our knowledge, this is the first report of a case in which prenatal ultrasound provided assistance for counselling before delivery.

Compliance with a perinatal prophylaxis policy for prevention of venous thromboembolism after caesarean section.

OBJECTIVE: To assess physician compliance, before and after a quality improvement intervention, with a regional policy on postpartum thromboprophylaxis following Caesarean section (CS), and to compare clinical outcomes (reduction of venous thromboembolism or increase in postpartum bleeding) between groups. METHODS: We performed a retrospective chart review of deliveries by CS, 404 prior to and 451 subsequent to a quality improvement intervention. All subjects were classified as being at either moderate or high risk for venous thromboembolism based on a risk-factor assessment, and warranted postpartum thromboprophylaxis according to the regional policy. Data regarding thromboembolism risk factors, postpartum thromboprophylaxis received, and clinical outcomes were recorded. RESULTS: Initial compliance with the regional policy was poor, but improved following the intervention. The use of intermittent pneumatic compression devices increased from 32% to 84% (P < 0.001), use of anticoagulation increased from 6.2% to 46% (P < 0.001), and full compliance with the policy increased from 1.7% to 30% (P < 0.001). Clinical outcomes were not significantly different between the groups. CONCLUSION: A quality improvement intervention markedly increased physician compliance with a regional policy on postpartum thromboprophylaxis among women at moderate to high risk of venous thromboembolism after CS. Adverse clinical outcomes were infrequent in this small study population.

Investigating adverse outcomes in obstetrics.

Investigation of adverse outcomes in health care requires a systematic and systemically oriented approach. This is true in obstetrics where, in Western countries such as Canada, adverse outcomes in the form of maternal mortality are rare. A model for such investigation is presented, based on answers to 3 questions: What? How? and Why? These questions correspond to Donabedian's 3 components of quality assurance: Outcome, Process, and Structure, respectively. Aspects of Reason's model of accidents are also included, in the form of "active failures" and "latent conditions." The latter are divided into 5 basic contributory factors, 4 of which are derived from Helmreich's model of human factors influencing behaviour in flight-crew environments. To illustrate its basic nature, the model is applied to a previously well-investigated air crash at Dryden, Ontario, and 3 obstetric cases of maternal mortality. Application of the model to the reactive investigation of close calls is discussed, as is application to the proactive interrogation of the system for the presence and nature of hazards that pose threats to safety.

A rare case of antepartum spontaneous septostomy in a monochorionic diamniotic twin pregnancy.

Spontaneous septostomy in a monochorionic diamniotic twin pregnancy is a rare phenomenon. We present a case of monochorionic diamniotic twin pregnancy with an intact dividing membrane seen in the 1st half of the pregnancy. At 26 weeks, when she was assessed for preterm contractions, the dividing membrane was not documented, which suggested spontaneous septostomy. There had been no invasive procedures during the pregnancy. She subsequently delivered at 29 weeks, secondary to preterm labor. No dividing membrane was noticed at the time of caesarian section. Spontaneous septostomy can complicate the management of monochorionic diamniotic twins by creating a pseudomonoamniotic environment resulting in cord entanglement, and difficulty in the diagnosis and management of twin-twin transfusion syndrome. We believe that such a case should be managed as monochorionic monoamniotic twin gestation.