Quality of life in patients with vascular malformations outside the central nervous system: Comparison with the general Norwegian population.

PURPOSE: The aim of this study was to assess Health-Related Quality of Life (HRQoL) in a population of patients with vascular malformations outside the central nervous system (CNS) and to compare the results with data from a national reference population. METHODS: In total, 111 consecutive patients above 14 years of age and referred for the first time to the national vascular malformation center from September 2011 to December 2012 were included. HRQoL was assessed using the Short-Form 36-item questionnaire (SF-36), which is a validated questionnaire with eight domains, covering both physical and mental aspects of HRQoL. The results were compared with national reference values. Possible association between HRQoL and selected demographic and clinical variables was analyzed using linear regression analysis. RESULTS: The sample consisted of 47 men (42.3%) and 64 women (57.7%). The median age of patients was 27 years (range 14-63 years). Ninety-six patients (86.5%) were diagnosed with venous malformations and nine patients (8.1%) with arteriovenous malformations. Six patients had other types of malformations (9%). The patients had significantly lower SF-36 scores in all domains, except for General health, than the general population. There was a significant association between muscular involvement and lower SF-36 scores in the physical domains Bodily pain and Role limitation due to physical problems. CONCLUSIONS: Our data suggest that patients with vascular malformations outside the CNS have impaired quality of life when compared with the general population. Muscular involvement seems to be associated with worse HRQoL in the physical aspects.

An 8-year population description from a national treatment centre on lymphatic malformations.

INTRODUCTION: Lymphatic malformations are rare developmental non-malignant abnormalities that present as masses consisting of abnormal growth of fluid filled channels or spaces in the skin or deep tissue that result in abnormalities of lymphatic flow. They may cause symptoms like pain, recurrent lymphorrea, abnormal bone growth, and disturbance of bodily functions. They may also be a cosmetic problem. The study aims to describe the population with lymphatic malformations (LM) treated at a national referral centre. MATERIALS AND METHODS: This is a retrospective review of patients with the diagnosis lymphatic malformation seen at the centre between 2006-2013. The authors performed a systematic review of the hospital records and a reevaluation of the radiological findings. Eighty-five patients with 102 lymphatic malformations were included. RESULTS: The majority of the patients (67%) were diagnosed before the age of 2 years. Lesions were located mainly to the head and neck (64%) and half (52%) were macrocystic. Treatment was: sclerotherapy (69%), surgery (49%), laser therapy (19%), or a combination of these treatment modalities (33%). The best outcome was found in the patients with macrocystic lesions (p = .029), and 76% of the patients reported a good or very good outcome. CONCLUSION: Most patients presented before the age of 2 years with complex lesions which required unique, often multimodal treatment plans and in general they experienced good outcome.

New methodology for facial nerve monitoring in extracranial surgeries of vascular malformations.

OBJECTIVE: To develop a more reliable methodology for monitoring the facial nerve in surgeries of vascular malformations where the extracranial segment of the nerve is at risk. METHODS: Our methodology comprises: (1) preoperative mapping to identify the anatomical location of the nerve branches, (2) continuous intraoperative monitoring of the compound muscle action potential (CMAP) by stimulating the facial nerve extracranially, in close proximity to where the trunk of the facial nerve exits the skull at the stylomastoid foramen, (3) intraoperative mapping to identify the nerve branches during surgical dissection and quantify the innervating contribution of each branch to the target muscle. RESULTS: Only three out of 201 surgeries (1.5%) had complete facial nerve trunk injury as a consequence of facial vascular malformation surgery. CONCLUSIONS: We developed a new method to continuously stimulate the facial nerve extracranially eliciting an objective parameter--the CMAP amplitude--to constantly measure changes in the muscle responses throughout surgery, alerting the surgeon before the facial nerve is severely injured. Our methodology notably reduces the complete facial nerve injury during extracranial surgery of facial vascular malformations. SIGNIFICANCE: This comprehensive methodology may also be a valuable tool to prevent facial nerve injury during other types of extracranial surgeries where radical excisions are required.

Current treatment of parotid hemangiomas.

OBJECTIVES/HYPOTHESIS: Parotid hemangiomas are the most common salivary gland tumors in children. Their treatment has posed a challenge because of the lesions' expansive growth, resistance to treatment, and relationship with the facial nerve. Various treatment modalities have been attempted, and promising results have been achieved with surgical resection alone or in conjunction with endovascular sclerotherapy. Recently, bleomycin and oral propranolol have been introduced, and the results thus far are promising. Here we elucidate the treatment options and propose a treatment algorithm for parotid hemangiomas. STUDY DESIGN: Retrospective chart review. METHODS: We conducted a retrospective chart review of patients from 2004 to 2009 with hemangiomas involving the parotid gland. We included 56 patients and relevant parameters. RESULTS: Seventy percent of patients were female. The female-to-male ratio was 2.3 to 1. Thirty-nine percent had unilateral parotid hemangiomas, 12.5% had cutaneous segmental hemangiomas. All 22 patients who underwent systemic steroid therapy responded initially, but 68% of these rebounded after cessation of therapy. Sixteen patients (29%) underwent surgery with excellent results (facial symmetry, restoration of contour, preserved facial nerve function). Seven (44%) patients received sclerotherapy 24 to 48 hours before surgery, and five (8%) received endovascular sclerotherapy alone. Ten patients were treated medically with oral propranolol. Eight of 10 had significant shrinkage of the lesion within the first month of treatment. There were no reported side effects. CONCLUSIONS: Multiple treatment regimens have been used to successfully treat parotid hemangiomas. Although propranolol is a recent addition, it seems most promising. Further evaluation is warranted.