Management of Fibro-adipose Vascular Anomalies (FAVA) in Paediatric Practice.

Fibro-adipose vascular anomaly (FAVA) is a discrete type of vascular anomaly. We describe our experience managing FAVA at a tertiary level paediatric hospital and offer a treatment algorithm. METHODS: A retrospective review of 27 patients with proven FAVA was undertaken. All patients had undergone MRI and USS evaluation. Patient demographics, presenting concerns, treatment methods, and outcomes were recorded and evaluation with the paediatric outcomes data collection instrument (PODCI) completed a minimum of 12 months after definitive treatment. RESULTS: Mean age at presentation was 8.9 years (range: 9 m-17.4 y) and mean post-treatment follow-up was 7.4 y (range: 2 y-11.6 y). Twenty of 27 lesions affected the lower limb. Severe neurogenic-type pain was present in 23 cases and contractures across joints in 11 cases. Sclerotherapy with sodium tetradecyl sulphate was used in 11 cases, with no improvement in symptoms. Cryoablation provided pain relief in 3/4 cases, but contracture subsequently increased in one patient and pain recurred in another.Fourteen cases underwent surgery (four surgical excisions alone, 10 in combination with other procedures). Three patients required four further surgical procedures that include one amputation for intractable pain and poor function.PODCI evaluations suggest overall good function, with surgical management and interventional radiology that provide comparable results. Surgery did correct deformity. CONCLUSION: If conservative measures or cryoablation fail to achieve symptomatic control, surgical excision should be considered, combined with adjunctive procedures, to correct contractures and balance muscle forces.Relief of pain may compensate for the loss of muscle mass and overall improves function. Multidisciplinary team working is essential.

Combined laparoscopic and cystoscopic injection sclerotherapy for bladder venous malformation: a novel technique.

Treatment of vascular malformations of the urinary bladder can be challenging. We report a case of bladder venous malformation treated with sodium tetradecyl sulphate (STS 3%) sclerotherapy, using a combined cystoscopic and percutaneous transperitoneal laparoscopy guided approach. When cystoscopic views are poor, the laparoscopic approach is a useful adjunct to aid sclerotherapy of bladder venous malformation. This technique has not been previously described.

'Mini' free groin flap for treatment of a tufted angioma of the finger.

Tumours in children can be alarming and diagnosis difficult when the pathology is rare. Furthermore tumours on the finger and subsequent treatment may lead to loss of function. The often-difficult decisions regarding treatment of vascular anomalies are often best managed in the multidisciplinary team meeting. Kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) are rare forms of vascular tumours. There is evidence that they may represent variants of the same tumour group. We present a unique case of TA/KHE involving the finger in a three-year-old child.

Limitations with the response evaluation criteria in solid tumors (RECIST) guidance in disseminated pediatric malignancy.

BACKGROUND: Response evaluation criteria in solid tumors (RECIST) guidelines were published in 2000 to evaluate response to treatment in solid tumors. These have sought to unify response assessment, and the new guidelines extend beyond lesion assessment to address modern imaging strategies. The RECIST guidelines, however, become complex and problematic when used to evaluate metastatic disease. METHODS: Ten consecutive oncology cases representative of tumors common to our pediatric practice were selected. All cases were evaluated at initial presentation and follow-up. The RECIST criteria were retrospectively applied in each case. A standardized evaluation form was used. RESULTS: The age range of the patients included in the study was 1 month to 16 years, with a mean age of 3.9 years. A range of tumor responses was identified: partial response (n = 6), stable disease (n = 2), and progressive disease (n = 2). Two of these responses were likely incorrect. Tumor bulk was underestimated in the axial plane, many lesions were either calcified and unmeasurable, or despite being well-defined were too small to be measurable under the strict RECIST guidance. CONCLUSION: In highlighting specific problems with disseminated pediatric tumors, we emphasize the pressing need for debate regarding the application of RECIST in pediatric oncology and encourage the development of a pediatric radiology oncology group to collaborate in future modifications of the RECIST guidance.

Technical report: use of ultrasound guidance in the removal of tunnelled venous access catheter cuffs.

Venous access catheters are employed for a wide variety of reasons. On removal of the catheter, the subcutaneous catheter cuff can be difficult to extract; retention of the cuff has recognized complications. We describe a simple ultrasound technique that assists in the identification of the cuff within the subcutaneous tissues, simplifying subsequent cuff removal. This may lead to a reduction in associated complications.