Utility of cardiac imaging in diagnosis of atypical presentation of cardiac fibroma.

Primary cardiac tumours are relatively rare in the paediatric population, with benign tumours accounting for >90% of cases. Cardiac fibromas are rare primary tumours that typically reside in the ventricles. Symptoms are usually the result of blood outflow obstruction or disruption of the cardiac conduction system. They do not typically regress and usually require surgical intervention. In this case, we report a rare finding of a right atrial fibroma in an 18-month-old female who presented with lethargy and vomiting. Chest X-ray revealed an enlarged cardiac silhouette, and follow-up CT showed a 3.7×3.2×3.7 cm hypodense lesion in the right atrium. Cardiac MRI revealed the diagnosis, which was confirmed on pathology.

Fetal heterotaxy with tricuspid atresia, pulmonary atresia, and isomerism of the right atrial appendages at 22 weeks.

We report the accurate prenatal diagnosis at 22 weeks gestation of right atrial isomerism in association with tricuspid atresia. Several distinctive sonographic features of isomerism of the right atrial appendages were present in this fetus: complex cardiac abnormality, ventriculoarterial discordance, juxtaposition of the aorta and the inferior vena cava to the right side, pulmonary atresia, and anomalous pulmonary venous return to the morphological right atrium. Tricuspid atresia, which is an extremely rare lesion within heterotaxy spectrum disorders, was present. Postnatal investigations confirmed all prenatally diagnosed abnormalities, with additional findings of pulmonary atresia with discontinuous pulmonary arteries and bilateral arterial ducts, asplenia, and bilateral eparterial bronchi. To our knowledge, tricuspid atresia in the setting of isomerism of the right atrial appendages has not previously been diagnosed or reported prenatally. Because of the complexity of cardiac lesions that may be present in cases of atrial isomerism, these disorders should be considered even if sonographic findings are uncommon or atypical.

A rare opportunity for conservative treatment in a case of blunt trauma to the supradiaphragmatic inferior vena cava.

Injuries to the inferior vena cava (IVC) secondary to blunt trauma are rare and occur in only 1-10% of all blunt trauma patients. Management of these injuries has not been subjected to major studies, but several case reports and small retrospective studies have demonstrated that management can be tailored to the hemodynamic status of the patient; this is similar to the management of blunt liver injuries. Stable patients whose injuries have achieved local venous tamponade have been successfully treated without surgical intervention, while unstable patients require operative management. Regardless of patient status, however, IVC injuries are highly fatal with mortality rates between 70 and 90%. This report describes the case of a patient with a blunt traumatic injury to the supradiaphragmatic IVC with development of a pseudoaneurysm who was successfully managed conservatively.

3-Dimensional magnetic resonance genitography: a different look at cloacal malformations.

PURPOSE: Standard contrast genitography and endoscopy have been the cornerstone of preoperative investigation of anatomy in complex cloacal malformations. In many cases standard genitography is ambiguous and lengthy endoscopy may be required to define the anatomy. Nonenhanced magnetic resonance imaging lacks definition of the nondistended common channel and confluence. To our knowledge we introduce 3-dimensional magnetic resonance genitography for delineating the unique anatomical features of these complex anomalies. MATERIALS AND METHODS: Contrast genitography, endoscopy and 3-dimensional magnetic resonance genitography were performed preoperatively in 4 female infants with cloacal malformations. Three-dimensional magnetic resonance genitography was performed using a 3-dimensional spoiled gradient technique after the instillation of gadolinium-diethylenetetramine pentaacetic acid solution via the common channel and cutaneous vesicostomy, when present. Augmented pressure distal colostogram with gadolinium via the mucous fistula was included. Three-dimensional images were constructed using a commercially available workstation. RESULTS: Three-dimensional magnetic resonance genitography yielded anatomical information far superior to that of standard genitography in all cases and it provided information on female genital tract anomalies and hindgut confluence that were not fully appreciated at initial endoscopy. The confluence was most accurately represented by augmented pressure colostogram. The length of the common channel was consistent with that on endoscopy. Rotating 3-dimensional images illustrated the unique anatomical relationships. No magnetic resonance imaging specific complications were observed. CONCLUSIONS: Three-dimensional magnetic resonance genitography provides superior anatomical detail for the preoperative assessment of cloacal malformations. It augments the information obtained by standard magnetic resonance imaging of the pelvic organs, levators and lumbosacral spine. Three-dimensional magnetic resonance genitography has great potential as a standard for preoperative anatomical evaluation and it will likely simplify preoperative endoscopy rather than supplant it.

Children with suspected craniosynostosis: a cost-effectiveness analysis of diagnostic strategies.

OBJECTIVE: Our purpose was to evaluate the clinical and economic impact of three evaluation strategies in children at different risks of craniosynostosis. MATERIALS AND METHODS: A decision-analytic and cost-effectiveness model was constructed to compare three evaluation in strategies in children with suspected synostosis: no imaging, radiography (if abnormal, followed by three-dimensional CT [3D CT]), and 3D CT. Three risk groups were analyzed on the basis of the prevalence (pretest probability) of disease: low (completly healthy children; prevalence, 34/100,000), intermediate (healthy children with head deformity; prevalence, 1/115), and high risk (children with syndromic craniofacial disorders [i.e., Crouzon's syndrome or Apert's syndrome]; prevalence, 9-10/10). Test performance (sensitivity and specificity) of the evaluation strategies was obtained from the literature. Costs (not charge) estimates were obtained from the hospital cost-accounting database and from the Medicaid fee schedule. RESULTS: In the low-risk group, the radiographic and 3D CT strategies resulted in a cost per quality-adjusted life year (QALY) gained of more than $560,000. In the intermediate-risk group, the radiographic strategy resulted in a cost per QALY gained of $54,600. Three-dimensional CT was more effective than the two other strategies but at a higher cost-hence, with a cost per QALY gained of $374,200. In the high-risk group, 3D CT was the most effective strategy with a cost per QALY gained of $33,800. Less experienced radiologists and poor-quality studies increased the evaluation cost per QALY gained for all of the risk groups because of decreased effectiveness. CONCLUSION: Radiologic screening of completely healthy children (low risk) for synostosis is not warranted because of the high cost per QALY gained of the radiographic and 3D CT strategies. In healthy children with head deformity (intermediate risk), the radiographic strategy had a reasonable cost per QALY gained. Three-dimensional CT was more effective but had a high cost per QALY gained. In children with syndromic craniofacial disorders (high risk), 3D CT was the most effective strategy and had a reasonable cost per QALY gained. Selection of children with suspected craniosynostosis based on their risk group and use of the most appropriate evaluation strategy could maximize clinical and economic outcomes for these patients.