Recommendations Based on Experiences of 23 years of Workgroup Vascular Anomalies Rotterdam (WEVAR)

Odijk, S.E.P. van (Sytze) (2015) Recommendations Based on Experiences of 23 years of Workgroup Vascular Anomalies Rotterdam (WEVAR). thesis, Medicine.

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Abstract

WEVAR) which has been operating continuously over the past 23 years. We evaluated the effect of the ISSVA-classification in the daily practice of the WEVAR with regard to diagnosis and treatment. We evaluated the interplay between the different medical specialties working together with the WEVAR. We compared our findings to similar studies performed by other vascular anomalies centers and other literature. Our findings result in recommendations for future research, recommendations for the functioning of the WEVAR itself, and recommendations for other physicians working in the field of vascular anomalies. Materials and Methods: A literature review was performed describing the latest developments with respect to diagnosis and treatment in the field of vascular anomalies. Consecutive cases of patients with vascular anomalies presenting at the WEVAR were retrospectively analyzed from January 1993 until June 2015. Basic patient characteristics were described for both vascular tumors and vascular malformations, including the diagnosis according to the 2014 classification of the International Society for the Study of Vascular Anomalies (ISSVA), location of the lesion, and status of follow-up. With regard to vascular malformations we also reviewed the age of first presentation, diagnostic tests, treatment, and physical and psychosocial development. Results: We examined 1,078 patient records with 1,090 lesions. We found 655 vascular tumors (60%), 358 vascular malformations (33%) and 2 provisionally unclassified lesions (0,1%). The remaining 75 lesions (6,9%) were classified as either non-vascular lesions (n=65), or lesions that could not be classified due to limited information (n=10). Vascular tumors consisted of 640 benign vascular tumors (mostly infantile hemangiomas), 14 ‘locally aggressive or borderline’ tumors (mostly kaposiform hemangioendotheliomas), and 1 malignant sarcoma. The 358 vascular malformations consisted of 292 simple vascular malformations (27%) with 56 capillary malformations (5,1%), 116 lymphatic malformations (11%), 109 venous malformations (10%), 9 arteriovenous malformations (0,8%), and 2 arteriovenous fistulas (0,1%). We found 34 combined vascular malformations (3,1%) and 32 malformations associated with other anomalies (2,9%). Not all ISSVA-diagnoses are represented in the WEVAR population. With respect to 358 vascular malformations we found a mean follow-up time of 8 years + 2 months, a longer period than described in similar studies by other vascular teams. In total 50% of patients were seen before the age of 3 years and 3 months old, 55 patients had a follow-up time of 10 years or more, and 31% of patients still receive regular follow-up by the WEVAR. A total of 55% percent of the patients were female. Most frequently observed locations were the head and neck region (32%) and lower extremities (28%). The concordance between clinical diagnosis and outcome of diagnostic tests was least high for Pathological- (35% congruence), Ultrasound- (59% ~) and MRI evaluations (65% ~). Despite these incongruences, the WEVAR was able to reach a final clinical diagnosis in the majority of cases. Evaluations on the effects of treatment were retrievable in less than 50% of the cases. Sclerotherapy and excision in simple vascular malformations were better documented (<50% unknown outcomes). Orthopedics, Plastic Surgery, Interventional Radiology, Ophthalmology and Neurology were the most often consulted specialties outside the WEVAR. Steps are taken by the WEVAR to work with a broader range of medical specialties. Coagulopathy symptoms were detected 2 in 42 patients. Analysis of pharmacological treatment in case of coagulopathy showed that 23/42 patients were prescribed acetylsalicylic acid and 5/42 patients were prescribed either dalteparine or acenocoumarol. Acetylsalicylic acid proved beneficial in certain patients. We found 76% of patients being evaluated for physical and psychosocial complaints. Patients with simple vascular malformations reported 10% complaints in both domains. Patients with combined vascular malformations reported 20-25% complaints in both domains. The effectiveness of interventions targeted at improving the psychosocial development has not been documented in the patient records. Conclusion: The WEVAR has treated 1,078 patients with 1,090 lesions covering a large part of the complete spectrum of vascular anomalies. Various types of vascular malformations listed in the ISSVA-classification were not represented in the WEVAR population. These types of malformations were possibly treated outside the WEVAR. We found incongruence between the clinical diagnosis and the diagnosis described by the departments of Pathology and Radiology. In the daily functioning of the WEVAR however, there appears to be little confusion about the final clinical diagnosis. This positive outcome is hypothesized to be the result of 23 years of experience, tackling these incongruences. In hospitals that function without a multidisciplinary vascular team, these incongruences possibly lead to misdiagnosis. Only a small part of the simple vascular malformations is actively treated. In more than 50% of cases the outcome of treatments for the patient remains undocumented for unknown reasons. Post-treatment experiences in the form of patient reported outcome measures (PROMs) should improve this situation. The WEVAR team works together with a diverse group of specialists. Patients with complex vascular diseases benefit from being treated by a larger team rather than different physicians in one hospital. Coagulopathy resulting from vascular malformations might be insufficiently recognized in our patient population. A standardized diagnosis and treatment protocol should include laboratory tests on coagulation. Literature suggests that treatment of first choice in coagulopathy with elevated D-dimers is dalteparine or acenocoumarol. Acetylsalicylic acid as prescribed by the WEVAR also shows improvements in a distinct proportion of patients. This incongruence should be topic of future research. The use of standardized questionnaires can improve the monitoring of the physical and psychosocial development of the child. Overal we recommend implementation of standardized protocols for all patients in WEVAR.

Item Type:	Thesis (Thesis)
Supervisor name:	Supervisor from Rijksuniversiteit Groningen (facultair begel and Houwing, R.H. MD
Supervisor name:	Pasmans, S.G.M.A. MD and Laat, P.C.J. de MD and Department of Pediatric Dermatology | Erasmus MC - Sophia Ch
Faculty:	Medical Sciences
Date Deposited:	25 Jun 2020 10:40
Last Modified:	25 Jun 2020 10:40
URI:	https://umcg.studenttheses.ub.rug.nl/id/eprint/196

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