1/125. Tracheal agenesis revisited: analysis of associated anomalies. We describe five new cases of tracheal agenesis and report on epidemiological and numerical analyses of nearly 100 such cases with multiple congenital anomalies. Malformations seen with tracheal agenesis form patterns which overlap with, but are distinct from, VACTERL association. They have a high frequency of other lower respiratory tract anomalies; e.g., laryngeal atresia and lung lobation defects, and complex heart anomalies, but fewer anal and vertebral malformations. Cluster analysis of the malformations in 86 patients identified four consistent groups. Anomalies in the first group were primarily restricted to the trachea, larynx, and cardiovascular system. In the second group, the patients had more severe cardiac defects, and lung lobation anomalies, while in the third they had a caudal component in addition to thoracic abnormalities, with anal and renal anomalies being common. Each of these groups showed a male excess and may represent increasingly severe perturbations in development fields encompassing the developing respiratory tract. Although the nature of the causative insult is unknown and probably heterogenous, one underlying pathogenetic mechanism may be abnormal epithelial-mesenchymal interactions. patients in the fourth group also had multisystem involvement with a high incidence of aberrant vessels, complex cardiac malformations, lung lobation defects, and anomalies of other foregut derivatives. The sex ratio in this group was normal and such cases could represent a disturbance in the primary development field during blastogenesis with secondary vascular disruptions. Complete tracheal agenesis is a lethal anomaly. However, segmental forms may be correctable and, in this group of infants, the nature of associated anomalies may well determine long-term prognosis. ( info) |
2/125. pregnancy outcome in aboriginal women with NIDDM in the Sioux Lookout Zone. PURPOSE: To review the pregnancy outcomes of Aboriginal women with non-insulin-dependent diabetes mellitus (NIDDM) in the Sioux Lookout Zone of Northwestern ontario, canada. METHOD: Retrospective chart review of deliveries of all women with a confirmed diagnosis of NIDDM was carried out between 1989 and 1992. RESULTS: During this period, 26 infants were born to 19 women with the diagnosis of NIDDM. Mean birth weight was 4,075 grams, with an average gestational age at delivery of 38 weeks. Three newborns required cesarean delivery, one required forceps, and one a vacuum extraction. There were four cases of shoulder dystocia. There were one stillbirth, one maternal death, and two cases of congenital heart disease. Ten newborns had neonatal jaundice and only two had neonatal hypoglycemia. These results suggest there is significant risk associated with NIDDM in pregnancy. ( info) |
| We report a case of previously healthy woman who suffered cerebral embolism after delivery. echocardiography with contrast medium confirmed the patent foramen ovale (PFO). PFO may be a mechanism of paradoxical embolism causing a transient ischemic attack or stroke. ( info) |
4/125. Episodic hyperglycaemia in pregnant women with well-controlled Type 1 diabetes mellitus: a major potential factor underlying macrosomia. AIMS: To test the common assumption that pregnant women who are sufficiently motivated to achieve very good HbA1c levels will record home blood glucose data accurately. methods: A new device was used to download information from electronic blood glucose meters to assess the extent of selectivity in patient glucose diary-keeping. RESULTS: In an index case, a woman with excellent ambient HbA1c (5.9%; upper limit of normal 6.1%) was observed to have 68% of preprandial blood glucose readings above the target range of 3.5-6.5 mmol/l and a mean ( /- SD) level of 8.9 /-3.9 mmol/l in the corresponding period. No such impression was conveyed by the home monitoring diary. Six pregnant women with well controlled Type 1 diabetes (mean HbA1c 6.6 /-0.2%) exhibited between 42 and 68% of preprandial readings above the target range. CONCLUSIONS: The frequency of hyperglycaemia has hitherto been underestimated in well controlled pregnant women whose near-perfect home monitoring record is apparently corroborated by near-normal HbA1c levels. These observations provide a hypothesis for understanding of the disappointing continuance of macrosomia despite excellent HbA1c levels throughout pregnancy. ( info) |
5/125. Abnormal presentation in labor and fetal growth of affected infants with type III ehlers-danlos syndrome. Information about labor presentation and birth weight of infants with connective tissue disorders such as ehlers-danlos syndrome is scarce. We report a case of abnormal fetal presentation and decreased birth weight in 2 infants affected by type III ehlers-danlos syndrome; their siblings were unaffected. ( info) |
6/125. Intracardiac fungal masses in high-risk neonates: clinical observations. Four cases of intracardiac fungal masses occurred over 2 y amongst 7 cases of systemic candidiasis in a neonatal referral unit. The gestations and birthweights were 25, 23, 24 and 30 wk and 805, 605, 640 and 1395 g, respectively. The pedunculated, solitary right atrial masses were detected 2-17 d after diagnosing candidemia in 3 cases, whereas it was the presenting feature in the 4th. All had indwelling right atrial catheters and received multiple courses of broad-spectrum antibiotics. The masses were removed successfully in two cases fit for surgery. None survived despite antifungal therapy, including liposomal amphotericin b at 6 mg/kg/d. Early introduction of enteral feeds, minimization of prolonged exposure to broad-spectrum antibiotics and judicious use of central catheters may reduce the incidence of systemic candidiasis in high-risk neonates. Surveillance echocardiography and timely surgical intervention may reduce the mortality and/or morbidity related to intracardiac fungal masses. ( info) |
7/125. Intra amniotic candidiasis. Case report and meta-analysis of 54 cases. We present a case of mid pregnancy loss with retained intrauterine contraceptive device associated with fetal candida infection. review of English literature identified 53 additional cases of fetal candidal infection, with 17 associated with an IUCD in situ. The presence of an IUCD was associated with delivery at a statistically significant earlier gestational age when compared to cases not associated with an IUCD (23.3 /- 4.9 vs 31.6 /- 7.0, p < 0.001). Seventy-seven percent of fetal candidal infections associated with an IUCD were systemic (heart, brain, liver, gastrointestinal, lung) compared to 33% of cases not associated with an IUCD. In contrast to bacterial intraamniotic infections there was a low incidence of maternal febrile morbidity. An hypothesis as to the pathogenesis of Candidal infections in the presence and absence of an IUCD is offered as well as a paradigm for the management of the gravid patient with an IUCD in situ. ( info) |
8/125. One day old infant with acyanotic congenital heart disease: critical aortic stenosis. Congenital aortic stenosis accounts for about 5% of cardiac malformations recognized in childhood. It belongs to the category of acyanotic congenital heart disease. These lesions produce a load on the heart because of left ventricular outflow tract obstruction. Severe aortic stenosis in the newborn period (critical aortic stenosis) presents with signs of left sided heart failure (pulmonary edema, poor perfusion), right sided heart failure (hepatomegaly, peripheral edema) and may progress rapidly to total circulatory collapse. We present a case of an infant with critical aortic stenosis presenting with cyanosis, who was entirely dependent on ductal patency for systemic output. When oxygen was given, the ductus started to close, with a worsening of the left sided output and subsequent acidosis. With the right to left shunt across the ductus, the baby was cyanotic and dependent on prostaglandin to keep the ductus open. There was minimal flow across the aortic valve because of the stenosis and extremely poor left ventricular function prior to surgery. After relief of the aortic valvular obstruction, there was finally good antegrade flow across the aortic valve, terminating cyanosis. ( info) |
9/125. Emotional factors in juvenile diabetes mellitus: a study of the early life experiences of eight diabetic children. The developmental history of eight diabetic children was compared with that of their nondiabetic siblings. On the basis of data obtained through interviews with the children and their families, as well as a chart review, we advance the hypothesis that an as yet unspecified metabolic "pre-diabetic" pathophysiological condition may be reflected in disturbances of appetite, satiation and weight regulation appearing early in life in those children who may later develop clinical diabetes mellitus. We further hypothesize that these dysfunctions are accompanied by a disturbance in important psychological developmental processes, compounding the liability a physiologically susceptible individual suffers. The results are discussed in terms of previous psychosomatic research. ( info) |
10/125. Obstetric emergencies precipitated by malignant brain tumors. OBJECTIVE: Our goal was to present a case series of pregnancy-associated malignant brain tumors. STUDY DESIGN: A review was conducted from 1978-1998 at 5 hospitals. RESULTS: Ten women were diagnosed with a malignant brain tumor during pregnancy (n = 8) or post partum (n = 2). patients diagnosed antenatally exhibited severe symptoms, manifest between 27 and 32 weeks' gestation. Six were emergently delivered of their infants because of maternal deterioration, and 2 were delivered electively in the early third trimester after documentation of fetal pulmonary maturity. There were 4 maternal deaths and 1 neonatal death; all of the other infants maintained viability. CONCLUSIONS: Malignant brain tumors rarely occur in pregnancy. In contrast to reports that describe an indolent course, each of the 8 antenatal patients experienced a neurologic crisis. If symptoms are amenable to pharmacologic control, we advocate delivery in the early third trimester after documentation of fetal pulmonary maturity. To minimize temporal lobe or cerebellar herniation in neurologically unstable patients, a consideration should be made for cesarean delivery with the patient under general anesthesia, followed by immediate neurosurgical decompression. ( info) |