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Summary Introduction and aims Sclerotherapy has become first line treatment for most venous malformations and some lymphatic malformations. We aimed to measure our sclerotherapy treatment success using 3% sodium tetradecyl sulphate (STD) and describe our experience. Materials and methods Retrospective five year review (Jan 04–09) of children with vascular malformations treated at our centre with 3% STD. Patients were classified using the Birmingham classification and treatment success was measured through case note review and pre- and post-treatment photographs. Findings and results Forty-three (84.3%) of the 51 patients with vascular malformations (VM) who underwent sclerotherapy derived a benefit. Twelve patients (23.5%) had an excellent result, 31 (60.8%) were improved whilst eight (15.7%) were unchanged. Using Fisher's exact test, there was a statistically significant difference in achieving complete resolution of superficial VMs compared to lesions involving the deeper layers of the head and neck. 17.6% of patients developed a complication with an overall complication rate of 12.2% per injection. There was one major complication with the remainder consisting of superficial skin necrosis that resolved conservatively. Conclusions Treatment with 3% STD sclerotherapy is effective in venous and some lymphatic vascular malformations. It should be considered an important treatment modality within a multi-disciplinary setting in these difficult problems.

The management of vascular anomalies in upper and lower limbs is complex. The current practice at Birmingham Children's Hospital is based on a multidisciplinary approach, involving plastic surgeons, interventional radiologists, vascular surgeons, dermatologists and laser specialists. This study reviews the management strategies for peripheral venous malformations (VMs) and proposes a simple classification system to aid treatment. A retrospective review was undertaken involving all paediatric patients presenting with (VMs) of the upper and lower limbs, managed by the same multidisciplinary team over a period of 3 years. A total of 33 patients were identified, of whom 19 had lesions located in the upper limb. Treatment modalities included surgery, sclerotherapy, a combination of the two and conservative management. The indications for treatment included: (1) worsening pain, (2) increased swelling, (3) reduced function, (4) bleeding or ulceration and finally, (5) cosmetic deformity. Following treatment, outcome measures with regards to the symptoms were graded into (1) improved, (2) worsened and (3) unchanged. Based on magnetic resonance imaging, we were able to apply our classification to separate the lesions into Type 1a (superficial localised): nine, Type 1b (superficial diffused): five, Type 2 (Fascia/muscle infiltration): nine, Type 3 (Bone/joint infiltration): seven and Type 4 (Extensive whole-limb infiltration): three. In patients with upper limb VMs (n=19), eight lesions (42%) were superficial and localised (Type 1a) while the rest were diffused lesions. In contrast, in the lower limb (n=14), only one lesion (7%) was superficial while the rest were diffused lesions. Lower success rate for treatment was noted in lower limb malformations (p0.05). In eight patients with recurrence of symptoms, six had Type 3 (intra-articular) lesions. There was one major and three minor complications following treatment. An outline of the management strategies for VMs in peripheral limbs is discussed in this article. An anatomical classification is described which aids in management and communication.

Bilateral Renal Cell Carcinoma (RCC) is an uncommon clinical entity, affecting 3%-6% of patients with localized RCC. Sunitinib has proven efficacy in the management of metastatic RCC (mRCC), however, there is very limited evidence of primary tumor response. With the changing treatment paradigm, the role of sunitinib should be extended to the neoadjuvant setting, to downstage locally advanced primary renal tumors, to facilitate nephron-sparing surgery (NSS), and to select responding patients with mRCC for continuation of treatment after cytoreductive nephrectomy. The role of sunitinib in downstaging primary renal tumors to facilitate curative NSS has not been previously reported. We report the case of recurrent renal tumors in a solitary kidney, where neoadjuvant sunitinib downstaged the tumors enough to allow NSS.

Clinical Summary A 31-year-old woman was admitted to a peripheral hospital with an episode of massive hematemesis. She had undergone a patchtechnique coarctation repair at age 3 years and required balloon dilatation for recoarctation at age 14 years. Annual cardiologic follow-up detected, at age 26 years, a pseudoaneurysm at the coarctation site with dimensions of 2.4 1.9 cm. The pseudoaneurysm size remained stable, but 8 months before the hematemesis, TEVAR was recommended to avert any risk of rupture and a Jomed 48 20-mm covered stent (Abbott Vascular Ltd, Kent, United Kingdom) was deployed at the coarctation site pseudoaneurysm. Post-TEVAR imaging suggested satisfactory exclusion. After the hematemesis, she underwent esophagogastroduodenoscopy, which showed a 1.5-cm bluish polypoid lesion 26 cm from the incisura. Biopsy revealed blood clot only. She was discharged but readmitted 4 weeks later with a further massive hematemesis. A repeat esophagogastroduodenoscopy revealed that the lesion had enlarged 3-fold. This finding, together with the history of coarctation surgery, prompted a computed tomographic (CT) scan (Figure 1), which demonstrated a perigraft hematoma suggestive of AEF. After transfer to our center, aortography confirmed peri–stent-graft extravasation, further supporting a diagnosis of AEF. Another covered stent (38 12 mm, Jostent; Abbott Vascular Ltd) was deployed and the hematemesis abated. However, a postprocedure angiogram demonstrated persistent filling of the pseudoaneurysm. The incomplete pseudoaneurysm exclusion and the perceived risk of further complications prompted surgical referral. Echocardiography demonstrated moderate bicuspid aortic valve stenosis. Surgery was performed via a transsternal thoracotomy in the left fourth intercostal space and comprised mechanical aortic valve replacement, retrocardiac ascending–descending aortic conduit insertion, and an interposition graft between the conduit and the left subclavian artery. The aortic arch was staple-ligated between the left common carotid artery and the left subclavian artery. The descending aorta was stapled below the stent site. The aortic pseudoaneurysm was opened and the previously placed stents were removed. Inspection revealed a section of necrotic and partially absent aortic wall with erosion of the longitudinal esophageal muscle but no mucosal breach. It was assumed that stent deployment had perforated or disintegrated the aortic wall leading to an AEF. The aortic remnant was left open, a feeding enterostomy and decompression gastrostomy performed, and the chest closed with drainage. Recovery was uneventful, with a contrast swallow examination showing no abnormalities. At 12 months, the patient remains free of symptoms, and CT scan (Figure 2) demonstrates resolution of perigraft hematoma and satisfactory anatomic reconfiguration.

T cell activation is commonly assayed in vitro by measuring the proliferative response of primed cells to an antigenic stimulus. We have modified the conventional form of this assay by dividing up the response into two stages. During the first stage, antigen drives the specific expression of IL-2 receptor expression. This phase is carried out in the presence of homologous mouse serum, in order to reduce non-specific responses to a minimum. During the second phase, proliferation of these activated T cells is driven by the addition of excess exogenous IL-2. This modified form of proliferatiion assay significantly increased the signal to noise ratio which can be attained, and is of particular value when looking at the T cell response to weak (e.g., cross-reactive) antigens, or low concentrations of antigen.