Imaging of pediatric congenital heart disease.

The evaluation of a patient with a heart murmur and congenital heart disease is diagnostically very challenging. Multiple advanced techniques aid in the diagnosis of simple and complex malformations. Interventional procedures and surgical corrections have allowed most patients to enjoy a good and productive lifestyle.

The use of inferior vena cava filters in pediatric patients for pulmonary embolus prophylaxis.

PURPOSE: To report our experience with inferior vena cava (IVC) filters in pediatric patients. METHODS: Over a 19-month period, eight low-profile percutaneously introducible IVC filters were placed in four male and four female patients aged 6-16 years (mean 11 years). Indications were contraindication to heparin in six patients, anticoagulation failure in one, and idiopathic infrarenal IVC thrombosis in one. Six of the eight devices placed were titanium Greenfield filters. One LGM and one Bird's Nest filter were also placed. Two of the filters were introduced via the right internal jugular vein by cutdown, and the remainder were placed percutaneously via the right internal jugular vein or the right common femoral vein. Patients received follow-up abdominal radiographs from 2 to 13 months after IVC filter placement. RESULTS: All filters were inserted successfully without complication. Three of the patients died during the follow-up period: two due to underlying brain tumors at 2 and 12 months and a third at 6 weeks due to progressive idiopathic renal vein and IVC thrombosis. The remaining five patients were all alive and well at follow-up without evidence of IVC thrombosis, pulmonary emboli, or filter migration. CONCLUSION: IVC filter placement using available devices for percutaneous delivery is technically feasible, safe, and effective in children.

Preoperative embolization of the spleen in children with hypersplenism.

Splenomegaly associated with myelodysplastic disorders in children may be massive and can result in pancytopenia, abdominal discomfort, and respiratory distress. When these symptoms cannot be relieved by nonsurgical means, splenectomy may be indicated. Under such conditions, surgical splenectomy carries increased risks, as the thrombocytopenia is difficult to correct secondary to splenic sequestration. Additionally, the surgical anatomy is often distorted secondary to the massive spleen and dissection can be difficult. These factors can lead to uncontrollable hemorrhage. In an attempt to decrease intraoperative blood loss, the authors successfully performed preoperative splenic artery embolization in 11 of 12 children (age range, 1-11 years) with pancytopenia due to hypersplenism. Hypersplenism requiring surgical splenectomy was due to leukemia (n = 9), myelodysplastic syndrome (n = 1), immune thrombocytopenia (n = 1), and osteopetrosis (n = 1). Embolization was performed under general anesthesia, prior to surgery, with gelatin sponge particles alone, Gianturco coils alone, or a combination of polyvinyl alcohol sponge particles and Gianturco coils. Embolization allowed for safe surgical splenectomy.

Arteriovenous fistula after biopsy of renal transplant kidney: diagnosis and treatment.

An 11-year-old renal transplant recipient was noted to have a bruit over her transplant graft 26 months post transplant and 17 months following percutaneous renal biopsy during an episode of rejection. Diagnosis of an arteriovenous (AV) fistula was made by ultrasound examination with Doppler flow and was confirmed with arteriography. The AV fistula was occluded by transcatheter embolotherapy with placement of a steel coil into the fistula from the renal vein approach. This procedure allowed nonsurgical closure of the AV shunt without significant change in renal function.

Bronchial arteriography and embolotherapy for hemoptysis in patients with cystic fibrosis.

Bronchial arteriography and embolotherapy were performed to control hemoptysis in 11 patients with advanced stages of cystic fibrosis. Two patients suffered massive, 1 moderate, and 8 mild but recurrent hemoptysis. The embolization procedures were performed with Gelfoam, Ivalon, and coils in one to four separate procedures. Altogether, 19 of 20 procedures were successful, with follow-up periods ranging from 9 months to 8 years. No serious complications were encountered except for one femoral artery pseudoaneurysm which required surgical repair. Rapid digital subtraction angiography and "roadmapping" were considered helpful in avoiding the complication of reflux embolization and expediting the procedures. Bronchial embolization is a life-saving procedure for severe hemoptysis in patients with cystic fibrosis and is considered safe enough to include the indications of moderate and mild/recurrent hemoptysis to improve the quality of life in these patients.

Polysplenia syndrome in the asymptomatic adult: computed tomography evaluation.

Although the majority of patients with polysplenia syndrome will present during infancy or childhood with congenital heart disease, 5% to 10% will have no cardiac disease; and the associated abnormalities may not be discovered until adulthood. Physicians should be familiar with the chest and abdominal radiographic findings so as not to confuse this syndrome with other, more common pathologic conditions in asymptomatic adult patients.

High risk of recurrent stroke after discontinuance of five to twelve years of transfusion therapy in patients with sickle cell disease.

Although long-term transfusion therapy is at least 90% effective in preventing recurrent strokes after an initial cerebrovascular accident in patients with sickle cell disease, it is unknown how long transfusion therapy should be continued. To address this question, we prospectively discontinued transfusions in 10 patients with sickle cell disease whose median duration of transfusion therapy after an initial stroke was 9 1/2 years (range 5 to 12 years). Before the transfusions were discontinued, patients were examined by cerebral angiography, magnetic resonance imaging of the head, neuropsychologic testing, electroencephalography, and a complete neurologic examination. Within 12 months after transfusion therapy was stopped, 5 of 10 patients had had an ischemic event. Three events caused relatively mild deficits in the same areas as those originally affected. Two were associated with massive intracranial hemorrhage, including one on the contralateral side of original involvement. An additional patient died suddenly of unknown causes. Of the four remaining patients, three declined to resume transfusion and are relatively well at greater than or equal to 18 months after therapy was stopped. The studies performed before transfusions were stopped were not predictive of recurrent stroke. The risk of recurrent cerebrovascular accident in this group was significantly greater than the estimated risk of 10% in patients who are receiving long-term transfusion therapy (p = 0.002). This adverse outcome suggests that patients with sickle cell disease who have had a stroke must receive long-term transfusion indefinitely or a suitable therapeutic alternative must be devised.

Initial experience with a universal-length copolymer ureteral stent.

A universal-length ureteral stent made of a biocompatible copolymer was designed specifically for easy antegrade internal placement. The double-looped distal end of the stent eliminates the need to measure the distance from the ureteropelvic to ureterovesical junction. If additional length is required, a portion of the distal loop is incorporated into the stent shaft. Interventional radiology practices benefit economically, as the universal-length stent is the only size required for the treatment of most ureteral obstructions.

The hyperdense choroid plexus: a CT finding associated with aortic arch obstruction in the newborn.

We report the first observation of choroid plexus hyperdensity depicted by CT in two newborns with aortic arch obstruction. Neither infant had intracranial hemorrhage or infarction demonstrated by autopsy or cranial ultrasonography. Although not proven, we believe that such hyperdensity in these two cases represents abnormal vascularity within the choroid plexus related to upper extremity hypertension.

Interventional catheterization in congenital heart disease.

The field of interventional catheterization in congenital heart disease has seen an explosion in application and scope over the past 10 years. In some lesions, such as valvar pulmonic stenosis, the transcatheter approach has replaced surgery as the treatment of choice. Applications currently considered experimental will no doubt become standard therapy as experience is gained with their use. Being able to avoid or postpone surgery has emotional, aesthetic, and practical advantages to patients and their families. The role of the catheter interventionist will continue to grow as new technologies such as fiberoptics, lasers, and miniaturized electromechanical devices are incorporated.

The spectrum of heterotaxic syndromes.

The heterotaxic syndromes may manifest as complex disorders with multiple anomalies, which have been described in the polysplenia/asplenia syndromes, or as a single isolated anomaly. Clinically insignificant anomalies may be incidentally discovered and should be recognized. Early diagnosis in the prenatal and neonatal periods allows for early intervention and correction, particularly of cardiac anomalies. Chest radiography, sonography, CT, radionuclide imaging, and MR imaging are useful diagnostic tools in evaluating these patients.

Digital subtraction angiography in the evaluation of renal vascular hypertension in children.

Twenty children were evaluated with digital subtraction angiography (DSA) for renal vascular hypertension. IV DSA from a basilic vein approach was performed in four patients and femoral vein approach in two patients. Intraarterial DSA was performed in 14 patients with suspected renal vascular hypertension. Renal vein renin samples were obtained from 17 patients. Two renal artery angioplasties were performed in association with intraarterial DSA. IV DSA was diagnostic in five of six patients with one false-negative result for fibromuscular dysplasia of the tertiary vessels. Intraarterial DSA images were diagnostic in 14 of 14 patients, and renal vein renin results correlated well with DSA results. In 11 patients, DSA was performed as an outpatient (hospital day-care unit) procedure. Of the 20 patients evaluated, 13 patients had abnormal DSA findings which included a hypoplastic but functioning kidney in three patients, distal small vessel abnormalities in five patients, unilateral or peripheral renal artery stenosis in four patients, and middle aortic syndrome in one patient. DSA with renal vein renin samples can be used as a diagnostic procedure for the evaluation of renal vascular hypertension in children as well as adult patients. In addition, it is a useful tool for therapeutic vascular procedures in children.

The continued value of angiography in planning surgical resection of benign and malignant hepatic tumors in children.

We assessed the accuracy of angiography or digital subtraction angiography (DSA) in diagnosing malignancy in hepatic tumors in children. In addition, these results were correlated with sonographic and computed tomographic findings of the liver in selected patients. Twenty-seven patients with primary liver tumors were examined with celiac or selective hepatic arteriography. Sonography was performed in 15 and computed tomography in 15 of the 27 patients. Angiographic criteria for malignancy or benignancy were established. These findings were correlated with computed tomographic and sonographic findings of the liver vascularity in selected patients. The final pathologic diagnosis was established surgically or by percutaneous biopsy. Sonography and computed tomography can be used as the initial procedure for evaluating tumor size, location and hepatic vascularity. However, the exact vascular anatomy demonstrated by angiography in children is more accurate and is often needed prior to surgical resection of primary liver tumors.

Imaging of foregut duplication cysts.

There are many types of diagnostic imaging studies available for the evaluation of foregut duplication cysts. Esophagography may be helpful in confirming the presence of a mediastinal mass, explaining esophageal symptoms, or defining the location of a lesion, but it is rarely diagnostic. Digital subtraction angiography can successfully be used in place of pulmonary arteriography to define vascular anatomy. Computed tomography is often diagnostic, although the attenuation number may be slightly elevated and not that of water. Computed tomography will clarify the relationship of the mass to adjacent mediastinal structures, and may also assist in percutaneous guided aspiration. Surgical resection is often necessary in an infant because of respiratory distress with airway compromise. In asymptomatic patients with a round to oval, nonenhancing, thin walled, cystic mass demonstrated on CT, a thoracotomy may be avoided and the patients may be followed by chest radiography.