Pregnant women with significant medical conditions: anaesthetic implications

Anaesthetic assessment plays a vital role in the successfulmanagement of the pregnant patient with coexisting medical diseases.This has been highlighted by successive reports of the ConfidentialEnquiries into Maternal Deaths (CEMD). Advances in medical carehave resulted in an increasing number of women with concomitantdiseases becoming pregnant. The effects of the physiologicalchanges of pregnancy on the disease must be given due considerationin patient evaluation and management. In this review, some ofthe common problems will be discussed.     

Spontaneous gastroduodenal disruption in neonate

A 2-day-old female presented with massive abdominal distention and respiratory distress. An erect abdominal X-ray film revealed a pneumoperitoneum of saddlebag configuration. The patient was resuscitated and a peritoneal drain was put through the right flank. Air and bile drained, and the respiratory distress was relieved. After the patient had stabilised haemodynamically, an exploratory laparotomy revealed a total gastroduodenal disruption. A gastrojejunostomy was done; the pyloric and duodenal ends were closed in two layers. The postoperative course was uneventful. We report this case for its unusual and rare presentation. Accepted: 1 March 2001     

Lower cancer incidence in Amsterdam-I criteria families without mismatch repair deficiency: familial colorectal cancer type X

Context  Approximately 60% of families that meet the Amsterdam-I criteria (AC-I) for hereditary nonpolyposis colorectal cancer (HNPCC) have a hereditary abnormality in a DNA mismatch repair (MMR) gene. Cancer incidence in AC-I families with MMR gene mutations is reported to be very high, but cancer incidence for individuals in AC-I families with no evidence of an MMR defect is unknown. Objective  To determine if cancer risks in AC-I families with no apparent deficiency in DNA MMR are different from cancer risks in AC-I families with DNA MMR abnormalities. Design, Setting, and Participants  Identification (1997-2001) of 161 AC-I pedigrees from multiple population- and clinic-based sources in North America and Germany, with families grouped into those with (group A) or without (group B) MMR deficiency by tumor testing. A total of 3422 relatives were included in the analyses. Main Outcome Measures  Cancer incidence in groups A and B (excluding the 3 affected members used to define each pedigree as AC-I) and computed age- and sex-adjusted standardized incidence ratios (SIRs) using Surveillance, Epidemiology, and End Results data. Results  Group A families from both population- and clinic-based series showed increased incidence of the HNPCC-related cancers. Group B families showed increased incidence only for colorectal cancer (SIR, 2.3; 95% confidence interval, 1.7-3.0) and to a lesser extent than group A (SIR, 6.1; 95% confidence interval, 5.2-7.2) (P<.001). Conclusions  Families who fulfill AC-I criteria but who have no evidence of a DNA MMR defect do not share the same cancer incidence as families with HNPCC-Lynch syndrome (ie, hereditary MMR deficiency). Relatives in such families have a lower incidence of colorectal cancer than those in families with HNPCC-Lynch syndrome, and incidence may not be increased for other cancers. These families should not be described or counseled as having HNPCC-Lynch syndrome. To facilitate distinguishing these entities, the designation of "familial colorectal cancer type X" is suggested to describe this type of familial aggregation of colorectal cancer.      

Cardiac MRI in Congenital Heart Disease – Our Experience

A significant recent advance that has occurred world over in the continuously evolving field of Magnetic Resonance Imaging (MRI) practice is the introduction of Cardiac applications. Cardiac MRI has moved to the centre stage of clinical management strategy by non-invasively imaging the structure as well as function of the heart. It has a wide range of specific applications such as delineation of morphological anatomy, quantification of flow and pressure across cardiac valve dysfunction, evaluation of myocardial function, assessment of infarcts, mapping coronary arteries and so on. Evaluation of congenital heart disease (CHD) is an important application of Cardiac MRI since the morphological details of chambers, septum, defects and anomalous connections are depicted accurately. Besides, flow information across valves, chambers, outflow tracts and shunts are also provided. This article describes our experience in the use of cardiac MRI in congenital heart disease.Key Words: Cardiac MRI, Congenital heart disease, Cyanotic and Acyanotic heart disease     

Changing causes of mortality in patients with familial adenomatous polyposis

Widespread use of prophylactic colectomy has resulted in a reduction in the incidence of colorectal cancer in familial adenomatous polyposis (FAP) patients. A retrospective chart review of families registered at the Steve Atanas Stavro Familial Gastrointestinal Cancer Registry in Toronto was performed to determine whether the decrease in the number of patients developing colorectal cancer implies that causes of mortality in FAP patients are shifting to that of extracolonic manifestations of FAP. Information was available on 140 deaths within 158 families and among 461 individuals with FAP. When stratified by decade, from the 1930s to the 1990s, the ratio of deaths caused by extracolonic manifestations of FAP compared with deaths caused by colorectal cancer was noted to have risen. Even though most deaths in FAP patients are still from colorectal cancer, it appears that screening policies and prophylactic colectomy have resulted in a reduction in the number of FAP patients who develop colorectal cancer. Thus, in recent decades, a greater percentage of deaths in FAP patients appears to be attributable to extracolonic manifestations of the disease.     

A Cross Between Truncus Arteriosus Communis and Aortopulmonary Septal Defect: A Hitherto Undescribed Entity

Case history and necropsy findings of a 5-month-old infant with a unique heart defect with features of truncus arteriosus communis and aortopulmonary defect in combination with severe tricuspid stenosis are presented. There is a wide spectrum of remarkable heart defects between truncus arteriosus communis and aortopulmonary septal defect.     

Mycoplasma pneumoniae infection presenting as bullous papular purpuric gloves and socks syndrome: novel association and review of the literature

Papular purpuric gloves and socks syndrome (PPGSS) is a self-limited, often febrile illness with symmetric edema and erythema of the hands and feet; papular, petechial, and purpuric acral dermatosis; and mucosal lesions in children and young adults. Most of the cases of PPGSS have been reported to be caused by parvovirus B19 and other viruses. This study describes a case resulting from Mycoplasma pneumoniae infection in an adolescent male and reviews the literature.