Deciphering normal and anomalous viscero-atrial situs on multidetector CT angiography.

Viscero-atrial situs encompasses the laterality, relative position and configuration of the abdominal viscera, the atria of the heart and the tracheobronchial tree. Determining the situs and cardiac position is the first step in the commonly used sequential, segmental approach to the imaging evaluation of congenital heart defects (CHD). Abnormalities of visceroatrial situs and cardiac position are frequently associated with the presence of complex CHDs and accurate assessment of situs abnormalities can help predict the probability and type of the defect. Multidetector CT (MDCT) angiography, with its multiplanar reformatting and volume rendering techniques, offers accurate information about the morphology and three-dimensional relationships of the various cardiac and extra cardiac structures. In this pictorial essay, we present the MDCT imaging findings of the spectrum of abnormalities of visceroatrial situs and cardiac position, using a third generation dual source CT scanner.

Histopathological Findings in the Viscera of Malformed Neotropical Viperids.

Congenital malformations (CMs) or developmental abnormalities are pathological processes defined as morphological defects that originate during embryonic stages. A wide variety of CMs is observed in reptiles, most of which are incompatible with normal life. Characterization of CMs and their pathogenesis are of great importance for the conservation of reptiles, including neotropical viperids (NVs). External CMs are often reported in these species, but little is known about concomitant microscopical findings in the viscera of malformed individuals. In order to partially fill in this gap in knowledge, we aimed to investigate and characterize histopathological findings in the viscera of malformed NVs, jararacas (Bothrops jararaca) and South American rattlesnakes (Crotalus durissus), correlating these findings with features of external CMs. From 78 malformed snakes analysed, 100% (19/19) of rattlesnakes and 98.3% (58/59) of jararacas had histopathological changes in the viscera. Liver and/or kidney fatty degeneration was the most common finding in malformed and control animals. Renal glomerulocystic and tubular disease was significantly correlated with brachygnathia (P < 0.05) in jararacas. These results add to the body of knowledge of snake pathology and demonstrate some associations between external CMs and visceral histopathological findings, some of which could have implications for individual survival.

Lipoma pulmonar intraparenquimatoso con comportamiento clínico de neoplasia maligna / Intraparenchymal pulmonary lipoma clinically mimicking malignant neoplasm

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[A neonate with aprosopia, monomelia and celosomy]. / Un cas de nouveau-né affecté d'une aprosopie, d'une monomélie et d'une célosomie.

The specimen of which it is a matter here takes part of the heritage of the Museum of Anatomy and Embryology of the Faculty of Medicine of the Université Libre de Bruxelles. Its medical history is completely unknown, because it takes part of an ancient collection of pathological anatomy saved from destruction and recovered by the laboratory of Anatomy and Embryology. The specimen is strongly folded up on itself ("in extension"), its length so arranged is about 25 cm; unfolded, it develops to 45 cm. Cranial perimeter is of 31 cm. Unfortunately, the brain was removed during the initial analysis, and only the examination of the braincase allows to draw some conclusions on the probable state of the encephalon. The face is rudimentary, without any orbit, and the oral cavity is limited to a vertical slit, revealing small strongly tipped up maxillary bones. A double proboscis is present. The trunk is characterised by a rather broad celosomy, with exhibition of the intestines, the liver and the pancreas. Anal atresia is observed, and the external sexual organs are hypoplastic and ambiguous. The whole left lower limb is absent, including the left half of the pelvis, corresponding to a left unilateral complete ectromelia. The neck is in hyperextension, so that the occipital region seems extremely welded in the cervical spine. However, CT examination does not confirm the presence of such fusion, but on the other hand, reveals a severe axial diversion of the spine with hyperextension. Extremely rare in humans, the aprosopia is more readily present in some animals (sheep). Its association with a monomelia and a celosomy seems not yet described.

Management of visceral vascular anomalies.

Vascular malformations affect the viscera less commonly than the head and neck, extremities, and extra-cavitary soft tissues. They present with a wide spectrum of symptoms and findings including pain, respiratory compromise, hemoptysis, chylothorax, ascites, gastrointestinal bleeding, and obstruction. Management options depend upon the subtype of malformation and anatomic extent and may include sclerotherapy, embolization, surgical extirpation, coloanal pull-through, and occasionally more innovative individualized surgical approaches.

Left-sided liver and right sided Polysplenia: a 77-year-old patient with hetrotaxy syndrome.

Hetrotaxy syndrome is defined as an abnormality where the internal thoraco-abdominal organs demonstrate abnormal arrangement across the left-right axis of the body. Although it is a known condition among physicians taking care of pediatric patients, it is rarely seen in adult day-to-day medicine and most physicians involved in care of adult patients are unaware of it. It is important to recognize this anomaly based on clinical findings,due to its reported association with various medical conditions. We report a case of a 77-year-old patient diagnosed with Hetrotaxy syndrome.

[Hereditary hemorrhagic telangiectasia]. / Maladie de Rendu-Osler.

Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) is a development disorder of the vasculature characterized by telangiectases and arteriovenous malformations in specific locations. Among monogenic disorders, it is one of the most common, though affected individuals are widely underdiagnosed. The most common features of this disorder, nosebleeds, and telangiectases on the lips, hands, and oral mucosa are often quite subtle. Mutations in at least five genes may result in hereditary hemorrhagic telangiectasia, but mutations in two genes (ENG and ACVRL1/ALK1) account for approximately 85% of cases. Optimal management requires understanding the specific clinical patterns of these vascular malformations, especially their locations and timing during life. Therapeutic modulation of angiogenesis may be an effective therapy.

Study for collecting background data on Wistar Hannover [Crl:WI(Han)] rats in embryo-fetal development studies--comparative data to Sprague Dawley rats.

The purpose of the present study was to collect the background data on Wistar Hannover [Crl:WI(Han)] (hereafter Wistar Han) rats in embryo-fetal development studies from the 6 safety research facilities of pharmaceutical companies and contract research organizations. In each facility, 20 or 22 female rats were dosed with vehicle solution during the organogenesis period. As a result, no abnormalities in clinical signs and necropsy findings in dams were found. Body weights and food consumption in dams were lower than those in Sprague Dawley (SD) rats. The number of corpora lutea (13.3 vs. 16.0 in SD) and implantations (11.8 vs. 14.7) were fewer, and fetal body weights (3.66 vs. 3.70) and placental weights (0.42 vs. 0.45) tended to be lower than those in SD rats. Regarding the fetal abnormalities, the incidence of several findings such as the persistent left umbilical artery (10.4% vs. 1.1%) and cervical (5.2% vs. 0.4%), full (7.4% vs. 0.9%) or short supernumerary (64.5% vs. 9.9%) and wavy ribs (6.6% vs. 0.3%) was higher than that in SD rats. Our present study showed that they maintained a sufficient number of live fetuses and the difference in the fetal sex ratio was not observed. In conclusion, Wistar Han rats were considered to be a suitable strain for embryo-fetal development toxicity study. Since the incidence of several abnormalities was higher than that in SD rats, it may be said that to accumulate background control data is important to evaluate the embryo-fetal development toxicity study using Wistar Han rats.

Sensitive windows of skeletal development in rabbits determined by hydroxyurea exposure at different times throughout gestation.

The critical periods of axial skeletal development in rats and mice have been well characterized, however the timing of skeletal development in rabbits is not as well known. It is important to have a more precise understanding of this timing of axial skeletal development in rabbits due to the common use of this species in standard nonclinical studies to assess embryo-fetal developmental toxicity. Hydroxyurea, a teratogen known to induce a variety of fetal skeletal malformations, was administered to New Zealand White rabbits as a single dose (500 mg/kg) on individual days during gestation (gestation day, GD 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, or 19) and fetal external, visceral, and skeletal morphology was examined following cesarean sections on GD 29. A wide range of fetal skeletal effects was observed following hydroxyurea treatment, with a progression of malformations from anterior to posterior structures over time, as well as from proximal to distal structures over time. The sensitive window of axial skeletal development was determined to be GD 8 to 13, while disruption of appendicular and cranio-facial skeletal development occurred primarily from GD 11 to 16 and GD 11 to 12, respectively. The results of this study provide a better understanding of the critical developmental window for different segments of the rabbit skeleton, which will aid in the design of window studies to investigate teratogenicity in rabbits.

Developmental toxicity of lersivirine in rabbits when administered throughout organogenesis and when limited to sensitive windows of axial skeletal development.

BACKGROUND: Lersivirine is a second-generation nonnucleoside reverse transcriptase inhibitor undergoing clinical development for the treatment of human immunodeficiency virus-1. An embryo-fetal development study was performed to evaluate the potential for maternal and developmental toxicity of lersivirine. METHODS: Pregnant New Zealand White rabbits were administered 0, 100, 250, and 500 mg/kg lersivirine by oral gavage once daily on gestation days (GDs) 7 to 19, followed by cesarean section on GD 29 and fetal evaluation. RESULTS: Maternal toxicity was noted at all dose levels (decreased food consumption and body weight gain), with fetal toxicity at 500 mg/kg (decreased fetal weights, increased postimplantation loss). Equivocal findings for axial skeletal malformations were observed in three fetuses at 500 mg/kg. To better understand if these malformations were related to treatment with lersivirine, a follow-up rabbit embryo-fetal development study was performed with 1000 mg/kg/day lersivirine (500 mg/kg BID, 12-hr interdose interval) for two different 3-day windows, GDs 8 to 10 or GDs 11 to 13, which represent the sensitive windows of axial skeletal development in rabbits. Control rabbits were administered vehicle following the same dosing regimen from GDs 8 to 13. Cesarean sections were performed on GD 29, and fetal skeletons were examined for the potential of lersivirine to cause skeletal malformations in rabbits. At maternal exposure levels higher than the initial study, lersivirine did not induce fetal skeletal malformations when administered in the sensitive windows of axial skeletal development. CONCLUSION: The results of these studies indicate that lersivirine did not exhibit any evidence of teratogenicity in rabbits.

Rare case of isolated levocardia with polysplenia including normally structured lung without cardiac anomaly.

Isolated levocardia (IL) is a rare type of situs inversus in which the heart is in the normal left-side position, but the abdominal viscera are in the dextroposition. Polysplenia is a congenital disorder affecting the asymmetric organs, including the heart, lungs, bronchi, liver, stomach, intestines, and spleen. In this report, we present a rare type satisfying the characteristics of both IL and polysplenia, confirmed by several imaging investigations, which revealed normally structured lungs and heart and the existence of the inferior vena cava (IVC), which runs on the left side, returns to the anterior-right side at the liver, and returns to the right atrium directly. Anatomical investigation and careful observation will make it possible to improve the prognosis of IL.

Normal and variant pelvic anatomy on MRI.

The superior tissue contrast and flexible imaging planes afforded by magnetic resonance imaging (MRI) versus competing technologies permit optimal depiction of the pelvic viscera. Targeted protocols developed for specific pelvic visceral organs highlight important anatomic features that may not be imaged by other modalities. Therefore, a solid understanding of normal and variant pelvic anatomy is crucial for appropriate interpretation of pelvic MRI studies. This article discusses the protocol strategies and relevant anatomy with commonly encountered anatomical variants in a segmented/organ-specific manner, using gender as a broad split given the substantial variance in relevant organs.

Maternal and developmental toxicity of ayahuasca in Wistar rats.

INTRODUCTION: Ayahuasca is a psychotropic plant beverage initially used by shamans throughout the Amazon region during traditional religious cult. In recent years, ayahuasca has also been used in ceremonies of a number of modern syncretic religious groups, including pregnant women. However, no documented study has been performed to evaluate the risk of developmental toxicity of ayahuasca. METHODS: In the present work, maternal and developmental toxicity was evaluated in Wistar rats. Ayahuasca was administered to pregnant rats in three different doses [the equivalent typical dose (TD) administered to humans, five-fold TD and 10-fold TD] during the gestational period (6-20 days). RESULTS: Dams treated with the highest ayahuasca dose showed maternal toxicity with decrease of weight gain and food intake. Visceral fetal findings were observed in all treatment groups. Skeletal findings were observed in the intermediate- and high-dose groups. The fetuses deriving from the highest dose group also presented a decrease in body weight. CONCLUSIONS: From these results, it is possible to conclude that there is a risk of maternal and developmental toxicity following ayahuasca exposure and that the level of toxicity appears to be dose-dependent.

Pyridoxine might not have a preventive effect on the retinyl palmitate-induced viscerocranial anomalies.

Viscerocranial anomalies are induced in the presence of various teratogens. Vitamin A-induced cleft palate formation is one of the most frequently used experimental models in these studies. However, the underlying mechanisms are not yet fully understood. Several studies have shown that exogenous vitamin A disrupts the fusion of the palatal shelves by increasing the expression of epidermal growth factor receptor (EGFR). More recently, pyridoxine (vitamin B6) has been reported to have a potentially protective effect in regard to viscerocranial malformations. Therefore, in this study, we aimed to investigate whether pyridoxine has a preventive effect on retinyl palmitate-induced viscerocranial anomalies. The frequency of gross malformations induced by retinyl palmitate, the natural form of vitamin A, has been studied in a dose dependent manner. Low doses of retinyl palmitate (100 mg/kg) exposure on embryonic day (ED) 10 caused no gross anomalies in the rat fetuses. Teratogenic effects were observed only after exposure to higher dosages (1000 mg/kg) and primarily targeted the developing eyes and palates. On the other hand, co-administration of 10mg/kg pyridoxine, at ED 9 and 10, significantly increased the frequencies of anomalies, even in the moderate dosage (500 mg/kg) group. In all cleft palates, sustained expression of EGFR in the medial edge epithelium was detected by immunohistochemistry. These results show that co-administration of pyridoxine has an inductive rather than protective effect on the formation of viscerocranial malformations after exposure to hypervitaminosis-A.

Raoultella ornithinolytica bacteremia in an infant with visceral heterotaxy.

A case of Raoultella ornithinolytica bacteremia in an infant with visceral heterotaxy is reported. Physical examination was remarkable for markedly red skin flushing, not unlike that seen during histamine fish poisoning. R. ornithinolytica is a histamine-producing bacterium recently elucidated as a major cause of histamine fish poisoning. Only 2 other cases of human infection by R. ornithinolytica have been reported.

Anomalies of visceroatrial situs.

OBJECTIVE: Visceroatrial situs refers to the position and configuration of the cardiac atria, the tracheobronchial tree, and the thoracoabdominal viscera. Accurate determination of situs is essential because anomalies of situs are associated with an increased incidence of complex congenital heart disease. CONCLUSION: We propose a methodical diagnostic approach to determining the visceroatrial situs and cardiac configuration that predicts the probability and types of associated congenital heart disease.

Modified primary sutureless repair of total anomalous pulmonary venous connection in heterotaxy.

Early and late mortality remain high after palliative open heart surgery in low-weight neonates. In addition, the need for neonatal repair and the presence of pulmonary venous obstruction are risk factors for mortality in right isomerism. We describe a modified primary sutureless repair of infracardiac total anomalous pulmonary venous connection in a 1,600 g neonate with heterotaxy. Endarterectomy of intimal hyperplasia localized to the anastomotic site was required at bilateral bidirectional cavopulmonary connection 6 months after the initial repair. The patient underwent a successful fenestrated Fontan procedure using an extracardiac conduit at the age of 2 years.

Update on congenital spinal deformities: preoperative evaluation.

STUDY DESIGN: Review. OBJECTIVE: To review and outline the preoperative evaluation and approach in assessing children with congenital vertebral malformation. SUMMARY OF BACKGROUND DATA: Congenital vertebral malformations encompass a broad spectrum of conditions. A high association of renal, cardiac, and intraspinal anomalies with congenital vertebral malformation has been well documented in the literature. Vertebral malformation with involvement of the thoracic cage may lead to the development of thoracic insufficiency. The natural history, the character, and location of the deformity ultimately influence the propensity for progression and the necessity for treatment. Multiple factors should be considered before treatment with the goal of treatment aimed at providing the best possible care to be able to optimize the child's overall function and potential for growth. METHODS: Narrative and review of literature. CONCLUSION: Congenital scoliosis is a multifaceted condition. The presentation of the condition can be quite varied from those presenting with an isolated hemivertebrae to those with severe malformations, complicated by multiple medical conditions. A thorough preoperative evaluation is necessary before the institution of any treatment protocol. The presence of any medical condition must be addressed; the treatment should be tailor-made for each patient putting into consideration the patients' age and the effects of treatment on pulmonary function at maturity.

[Multiple anatomical malformations in 25-year old patient with heterotaxy--case report].

INTRODUCTION: Heterotaxy or situs ambiguus is a rare congenital disorder, characterized by abnormal situs with either left or right isomerism that usually coincides with A complex malformation. The classic left isomerism or bilateral left-sidedness or heterotaxy syndrome with polysplenia implies that patients have bilateral bilobed lungs, bilateral pulmonary atria, a centrally located liver, a stomach in indeterminate position, and multiple spleens, occasionally associated with interruption of the inferior cava with azygos or hemiazygos continuation. CASE REPORT: We present a case of situs ambiguus associated with severe heart and vascular abnormalities in a 25-year-old male patient. On the admittance to our clinic the patient had history of biliary colic, moderate jaundice of the conjunctive and sclerae, and severe central cyanosis. The echocardiography revealed a large atrial septal defect, complete absence of the interventricular septum resulting in the single ventricle and the inversion of atria. The angiography obtained the hypoplastic inferior vena cava continuing with azygos vein that flowed into the left superior vena cava. The computerized tomography scans showed three splenuli in the right upper quadrant of the abdomen, and a single gallstone appeared in the left-positioned gallbladder. The contrast radiography showed complete transposition of the guts and stomach. DISCUSSION AND CONCLUSION: To our knowledge, our patient with described severity of the anomalies had the longest life span in this part of Europe, although the longer survivors have been described in the other parts of the world. Furthermore, clinical characteristics of both left and right isomerism have been obtained in the patients with anatomically pronounced left isomerism.