PubMed · 10857109
The source did not provide an abstract. Follow the original record for more information.
Explore connections, maps & timelines
A Orren. 2000. Screening for complement deficiency.. https://doi.org/10.1385/1-59259-056-x%3A139
Cite the original work for its findings. Save a collection to share your selection of sources.
KEEP EXPLORING
Abnormal surgical bleeding may be caused by congenital or acquired plasmatic and/or thrombocytic coagulation disorders. They may be known at the time of operation, or they may occur during the perioperative course for the first time. A detailed identification of haemostatic defects can be performed by a diagnostic spectrum of multiple tests. However, in clinical practice global screening tests are used first. In diagnostic routine three groups of patients have to be considered: (1) patients with no personal or family history of bleeding and no operative bleeding risk; (2) patients with no history, but increased bleeding risk by the planned procedure; (3) patients with a known haemorrhagic diathesis in their own history. In all difficult situations a specialist in haemostasiology should be consulted, because only rational diagnosis and therapeutic monitoring achieve an optimal and cost-effective operative result. The surgeon should always be aware that surgical bleeding from an operative cause must be considered in the differential diagnosis.
We present a neonate with a cervicofacial haemangioma complicated by the Kasabach-Merritt syndrome, respiratory distress due to airway compression and high-output heart failure. This haemangioma and intravascular disseminated coagulation, treated initially by aspirin, ticlopidine and corticosteroids, required more invasive treatment with superselective embolisation and interferon alpha-2a. The clinical outcome was good.
BACKGROUND: Ebstein's anomaly in the severely symptomatic neonate is usually fatal. Because the mortality for various surgical interventions has been prohibitively high, the indications for operation in these critically ill neonates are unclear. METHODS: We reviewed our results with biventricular repair of three consecutive severely symptomatic neonates (2.8 to 3.2 kg) at our institution since 1994. Each had associated complex cardiac pathology, including multiple muscular ventricular septal defects (n = 1), pulmonary stenosis with functional pulmonary atresia (n = 1), and anatomic pulmonary atresia (n = 1). Preoperatively, all infants had severe tricuspid regurgitation, Great Ormond Street Ebstein echocardiogram scores greater than 1.3:1 (grade 3 or 4) and cardiothoracic ratio greater than 0.85. Two patients were severely cyanotic. Hepatic and renal insufficiency with diffuse coagulopathy was present preoperatively in two patients. Surgical repair consisted of (1) reconstruction of a competent monocuspid tricuspid valve, (2) right ventriculorrhaphy, (3) subtotal closure of atrial septal defect (ASD), (4) aggressive reduction atrioplasty, and (5) repair of all associated cardiac defects. RESULTS: There were no early or late deaths. All patients are currently asymptomatic, without medications, and in sinus rhythm. At 5-year follow-up, trivial tricuspid regurgitation is present in 1 and mild regurgitation in 2 patients. On the basis of these results and review of the current literature, we propose new indications for surgical repair in the neonate with Ebstein's anomaly. CONCLUSIONS: Biventricular repair of Ebstein's anomaly in the critically ill neonate is feasible and medium-term durability of the repair is excellent. Therefore, conventional management of these patients should be revised and early surgical repair encouraged.