Charles A. James

Intracranial aneurysms in infants and children

Background. The diagnosis and imaging of pediatric aneurysms has changed since the advent of MR and MRA. Objective. To update the literature on pediatric aneurysms and better define the appropriate work-up of childhood aneurysms in 1997. Materials and methods. Retrospective review of 21 children (12 boys, 9 girls) with 25 aneurysms from three institutions over a 20-year period was performed. Imaging studies were mixed and included CT (19 patients), MR (11 patients), MRA (6 patients) and angiography (18 patients). Results. Eighteen of 25 aneurysms were congenital saccular, 6 were mycotic, and 1 was post-traumatic. Of these, 44 % were in the posterior circulation. Nine aneurysms arose from distal arterial branches. Forty percent were large (between 1–2.5 cm) and 16 % were giant (> 2.5 cm). CT and MR showed hemorrhage, and frequently revealed the aneurysms as a focal mass with or without enhancement and flow void. Six children had MRA which revealed aneurysms in four patients. All patients with MRA had corresponding conventional angiography. Conclusion. Characteristics of pediatric aneurysms include diversity of type, increased incidence in the posterior fossa, peripheral location, and large size. CT, MR and MRA are useful in the diagnosis with conventional angiography essential for preoperative planning.

Transcranial Doppler, MRA, and MRI as a screening examination for cerebrovascular disease in patients with sickle cell anemia: an 8-year study

Objective. The authors previously reported five transcranial Doppler ultrasonography (TCD) findings as significant in detecting clinical cerebrovascular disease in a 4-year study in patients with sickle cell disease. This is a follow-up to evaluate the validity of the original findings over another 4-year period during which the study population doubled. A clinical follow-up of the original asymptomatic sickle cell patients with positive TCD, MRA, and MRI was also made. Materials and methods. Over an 8-year period TCD, MRI, and MRA were prospectively performed in 90 sickle cell patients who were clinically asymptomatic for stroke and in 27 sickle cell patients with clinical stroke. Results. Of the 4 out of original 46 control patients in 1992 who had positive MRA and TCD, 3 have subsequently had clinical stroke. None of the 9 original patients with positive TCD and positive MRI but negative MRA have developed stroke. All five original TCD indicators of disease were still significant (P < 0.05) for detecting clinical disease: maximum velocity in ophthalmic artery (OA) > 35 cm/s, mean velocity in middle cerebral artery (MCA) > 170 cm/s, resistive index (RI) in OA < 50, velocity in OA greater than in MCA, and velocity in posterior cerebral (PCA), vertebral, or basilar arteries greater than in MCA. Four additional factors were also significant: turbulence, PCA or ACA without MCA, RI < 30, and maximum velocity in MCA > 200 cm/s. Conclusion. Positive MRA with a positive TCD in an asymptomatic patient in long-term follow-up suggests a trend for developing clinical stroke. A 4- to 8-year follow-up of nine patients with positive TCD, positive MRI, but not positive MRA did not show development of clinical stroke. Nine Doppler findings are significant in screening for clinically symptomatic vascular disease in sickle cell patients. It is recommended that children with sickle cell disease be screened for cerebrovascular disease with TCD. If one or two indicators of abnormality are present, MRA is recommended. If the MRA is positive, the patient may be considered for transfusion therapy or other treatment for prevention of stroke.

Microbubble-augmented Ultrasound Declotting of Thrombosed Arteriovenous Dialysis Grafts in Dogs

PURPOSE Transcutaneous low-frequency ultrasound (LFUS) can effectively lyse clots in the presence of microbubbles. This study was designed to test the commercially available human albumin microspheres injectable suspension octafluoropropane formulation, Optison, to establish efficacy and assess US parameters of intensity and wave modes in a canine model of a thrombosed arteriovenous (dialysis) graft. MATERIALS AND METHODS Arteriovenous grafts in five dogs were cannulated, temporarily ligated, and thrombosed. Different declotting techniques were randomized to treat nine groups. Control groups involved direct saline (4.5 mL) clot injection in 0.5-1.0-mL increments. One group underwent peripheral intravenous microbubble injection (13.5 mL). Six groups underwent direct incremental clot injection of 4.5 mL of microspheres with LFUS for 30 minutes in 3-5-minute increments with use of various intensity settings in continuous-wave and pulsed-wave (PW) modes. At each increment, angiography was used to grade flow, declotting, and overall success. RESULTS One hundred four procedures showed success in all 24 high-intensity PW modes (1.2-2.0 W/cm(2)); only one of 20 control experiments was successful (P <.0001). Medium-intensity modes yielded intermediate success rates. Lowest-intensity direct-injection groups and intravenous and control groups ranked lower. Results at 30 minutes were better than at 15 minutes (P <.0001). CONCLUSIONS LFUS with direct injection of microbubbles is effective in lysing moderate-sized clots and recanalizing thrombosed arteriovenous grafts. It best succeeds at the higher range of intensity settings tested in PW mode. Further development is justified.

Arteriovenous Malformations of the Tongue: A Spectrum of Disease†

Objectives: To examine our vascular anomalies teams experience with tongue arteriovenous malformations (AVM) with specific emphasis on the spectrum on disease and surgical management.

Diagnosis and management of pediatric cervicofacial venous malformations: Retrospective review from a vascular anomalies center

Venous malformations are rare congenital aberrations of vein development frequently presenting in the head and neck. Without treatment, venous malformations cause progressive disfigurement, dysfunction, and bleeding. In this study, we analyzed a cohort of pediatric patients with cervicofacial venous malformations (CFVM), with the goal of developing a treatment algorithm for these complex lesions.

Gastric teratoma with intramural extension.

Gastric teratoma is an extremely rare neoplasm which accounts for less than two percent of all teratomas. Unlike other teratomas, gastric teratomas are all benign and predominantly occur in males. As gastric teratomas generally present as a palpable abdominal mass, more aggressive solid masses of childhood must be excluded. In this case, CT imaging delineates both cystic and fatty components characteristic of teratoma and displays the rare gastric origin of the lesion.

Reconversion of bone marrow in Gaucher disease treated with enzyme therapy documented by MR

Background. Skeletal complications are responsible for significant morbidity in Gaucher patients. Plain radiographs have been unreliable in assessing bone marrow infiltration and activity. A way to assess bone marrow improvement is needed during enzyme therapy. Objective. The purpose of this paper is to assess the usefulness of MR in following improvement of abnormal bone marrow in Gaucher patients on enzyme therapy. Materials and methods. Three patients aged 2, 7, and 24 years underwent serial MR scans of the lower extremities before and during treatment with Alglucerase (two patients) and Imiglucerase (one patient). T1-weighted, T2-weighted, STIR and FSE T2-weighted images were utilized. Two patients were imaged after 16 months of therapy, and one patient was imaged after 6 months of therapy. Results. All patients had improvement in marrow signal consistent with partial reconversion to fatty marrow during treatment. The findings were more marked after prolonged therapy. T1-weighted images demonstrated findings most clearly. Conclusion. MR consistently showed improvement in marrow signal in Gaucher patients on enzyme therapy. As smaller doses of enzyme therapy are the trend, MR can be utilized to determine if therapy is effecting a change in the bone marrow.

Preoperative sclerotherapy of facial venous malformations: impact on surgical parameters and long-term follow-up.

PURPOSE To analyze the operative benefit of preoperative sclerotherapy of facial venous malformations and assess long-term patient outcome. MATERIALS AND METHODS Preoperative sclerotherapy was performed in 24 consecutive patients referred before resection of facial venous malformation. Pretreatment imaging was reviewed for malformation dimensions (length, width, and height), and volumes were estimated. Sclerotherapy was performed with 3% sodium tetradecyl in the first 15 patients and 98% dehydrated alcohol in the remaining 9 patients. Operative blood loss, operative time, transfusion requirement, and hospital stay were recorded. Operative time per lesion volume and operative blood loss per lesion volume were calculated. Results were compared with 15 historical control patients who underwent resection of facial venous malformations without preoperative sclerotherapy. Long-term follow-up of study and control patients was performed. RESULTS Compared with controls, patients undergoing preoperative venous sclerotherapy were significantly older (P = .0206) and had larger lesions in all three dimensions (height, P = .0002; length, P = .0010; width, P = .0004). Patients receiving sclerotherapy had shorter operative time per lesion volume (P < .0001) and reduced blood loss per lesion volume (P < .0001). Neither hospital stay nor the need for blood transfusion differed from the control patients (P = .2449 and P = .6857). Mild periprocedural complications were encountered in 12.5% of cases, and nerve paresis occurred in 8.3% of cases. Long-term follow-up revealed retreatment was required in 2 of 24 patients (8.3%). CONCLUSIONS Preoperative sclerotherapy of venous malformations was associated with less operative time per lesion volume and less operative blood loss per lesion volume. Long-term follow-up revealed a low need for retreatment.

Pediatric interventional radiology workforce survey summary

This report summarizes the results of a workforce survey of pediatric interventional radiology (PIR) performed by the 2005 SPR Vascular Interventional Radiology (VIR) Committee. Detailed results, discussion and proposed solutions to the challenges reported will be presented in a subsequent article. A link to an online survey was sent to all known pediatric interventionalists and to the members of the Society of Chairpersons of Radiology in Children’s Hospitals (SCORCH). The survey was announced at the 48th Annual SPR in New Orleans and was open from 18 February 2005 until 18 May 2005. The survey was sent to a maximum of 77 potentially different sites and there were 41 responses. Therefore, the response rate was at least 53%. Due to the anonymity of the survey, the exact response rate cannot be calculated. Out of 41 responses, 38 sites reported having an active PIR service. These 38 responses form the basis of the remainder of this report. The original question is stated or paraphrased and the responses are listed below it. In the instances when not all sites answered a question, the number of responses is noted in parentheses.

MR and CT diagnosis of carotid pseudoaneurysm in children following surgical resection of craniopharyngioma.

We report the cases of two children who underwent CT, MR, MRA and angiography in the diagnosis of postoperative aneurysmal dilatation of the supraclinoid carotid arteries following surgical resection of craniopharyngioma. Craniopharyngiomas are relatively common lesions, accounting for 6–7% of brain tumors in children. They are histologically benign, causing symptoms by their growth within the sella and suprasellar cistern with compression of adjacent structures, especially the pituitary gland, hypothalamus and optic nerves, chiasm, and tracts. Complete surgical resection, particularly of large tumors, is complicated by the fact that the lesions are usually found within the circle of Willis, with displacement and adherence to the adventitia of these vessels [1, 2]. Recent reports in the neurosurgical literature have described aneurysmal dilatation of the supraclinoid internal carotid arteries following aggressive surgical resection of craniopharyngioma [3, 4].