1/7. Prenatal and postnatal imaging of thoracopagus conjoined twins with a shared six-chamber heart.Management of thoracopagus conjoined twins is predominantly determined by the associated complex cardiovascular abnormalities seen in the majority of these cases. magnetic resonance imaging (MRI), including three-dimensional contrast-enhanced magnetic resonance angiography (MRA) provides a useful complement to conventional cardiac echocardiography or catheterization for detailed evaluation of the extra-cardiac anatomy. We report a pair of female thoracopagus conjoined twins with a shared six-chambered heart. The MRA findings are highlighted.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
2/7. Promogran and complex surgical lesions: a case report.Surgical excision of a venous malformation on a young girl's thigh left a large and deep ulcerative wound. Treatment of the lesion with Promogran dressings suited this patient's specific needs and produced substantial healing within five weeks.- - - - - - - - - - ranking = 4keywords = complex (Clic here for more details about this article) |
3/7. Repair of a thoracoabdominal aortic aneurysm in the presence of a left-sided inferior vena cava.Venous anomalies are not infrequently encountered during aortoiliac reconstruction, because of the complexity of development of the venous system. Retroaortic left renal veins, duplicate inferior vena cava (IVC), and left-sided IVC are occasionally found. Left-sided IVC has been reported with infrarenal aortic aneurysms. We report successful repair of a thoracoabdominal aneurysm in a patient with a left-sided IVC. The embryology and intraoperative management are discussed.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
4/7. Three-dimensional computed tomographic imaging of complex congenital cardiovascular abnormalities.Primary evaluation of congenital cardiac abnormalities traditionally relies upon echocardiography and conventional angiography, both of which have potential limitations. echocardiography is an operator dependant study, limited by a small window and patient movement. Conventional angiography is an invasive procedure with an inherent risk of catheter complication such as vessel damage, bleeding, stroke and infection. During angiography, overlapping of the pulmonary and systemic circulation often provides a confusing picture given complex anatomy. Another limiting factor of particular significance in young children is radiation dose and contrast administration during catherization procedures. Three-dimensional MDCT provides an alternative to alleviate these pitfalls of traditional cardiac diagnostic studies. Development of multi-detector computed tomography (MDCT) and 3D software provides new methods for non-invasive visualization and evaluation of congenital cardiac abnormalities. The multiplanar, volumetric functions allow faster and more complete computed tomography diagnosis and better understanding of clinical relevance of complex cardiac anatomy. In addition, 3D imaging is particularly useful for preoperative planning and postoperative outcomes. This essay provides case studies to illustrate the usefulness of MDCT/3D CT for evaluation of complex congenital heart disease.- - - - - - - - - - ranking = 7keywords = complex (Clic here for more details about this article) |
5/7. Two babies, one heart, and no airway.The medical literature on conjoined twins is replete with descriptions of the complexity of surgical separations. However, airway management, although quite challenging, is neglected in literature. We present experience with conjoined twins united anteriorly at the thorax and abdomen sharing one six-chambered heart. laryngoscopy and intubation recommendations, bronchoscopy techniques, and tracheotomy strategies are outlined for the face-to-face twins. Difficult, unique case report at tertiary care medical center. Conjoined thoraco-omphalopagus twins were successfully intubated at birth using the rigid Seldinger-assisted videotelescopic intubation (SAVI) technique. Flexible airway endoscopy allows for timely management of ventilator-dependent conjoined twins. tracheotomy was later performed with the twins in the upright position with heads slightly rotated. Meticulous attention to detail, monitoring and vigilance are mandatory. Successful management of conjoined twins relies on close communication and cooperation of all members of the multidisciplinary team. Conjoined thoraco-omphalopagus twins united anteriorly require a challenging airway management strategy. A three-dimensional perspective is utilized for intubation and tracheotomy. The SAVI technique is helpful for difficult intubations in the emergent twin airways.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
6/7. A common SCN5A polymorphism attenuates a severe cardiac phenotype caused by a nonsense SCN5A mutation in a Chinese family with an inherited cardiac conduction defect.The SCN5A mutations have been associated with a variety of arrhythmic disorders, including type 3 long qt syndrome (LQT3), brugada syndrome and inherited cardiac conduction defects. The relationship between genotype and phenotype in SCN5A mutations is complex. Some SCN5A mutations may cause death or severe manifestations in some people and may not cause any symptoms or arrhythmias in others. The causes of these unpredictable clinical manifestations remain incompletely understood. The molecular basis of a four-generation family with cardiac conduction abnormalities was studied and whether variants in the SCN5A gene could account for the cardiac phenotypic variability observed in this family was determined. A novel mutation (W1421X) of SCN5A was identified in a four-generation family with cardiac conduction abnormalities and several cases of sudden death. Most family members who carry this W1421X mutation have developed major clinical manifestations or electrocardiographic abnormalities, both of which became more prominent as the patients grew older. However, the 73-year-old grandfather, who carried both the W1421X and R1193Q mutations, had thus far remained healthy and presented with only subtle electrocardiographic abnormalities, whereas most of his offspring, who carried a single mutation (W1421X), had died early or had major disease manifestations. This observation suggests that the R1193Q mutation has a complementary role in alleviating the deleterious effects conferred by W1421X in the function of the SCN5A gene. This report provides a good model to explain the mechanism of penetrance of genetic disorders.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
7/7. prenatal diagnosis of de novo distal 5q duplication associated with hygroma colli, fetal oedema and complex cardiopathy.We report the first prenatal diagnosis of de novo distal 5q duplication after the echographic findings of hygroma colli and complex cardiopathy in a female fetus of 16 weeks' gestation. Cytogenetic studies on amniocytes showed a de novo inverted distal 5q duplication: karyotype: 46,XX, inv dup(5) (pter-->q3.53::q3.53-q3.33::q3.53-->qter). Based on the findings in the literature, a review of the malformative syndrome associated with partial distal 5q trisomy is given.- - - - - - - - - - ranking = 5keywords = complex (Clic here for more details about this article) |