Retrosepktive Analyse von orbitalen und periorbitalen Hämangiomen und vaskulären Malformationen

This retrospective study has analysed 64 patients. They have introduced themselves between 1998 and 2009 in the ENT clinic at the University of Marburg with intra- and/or periorbital hemangiomas or vascular malformations. While 22 of the patients had hemangiomas, 44 patients presented vascular malformations. Out of the patients with vascular malformations, 14 venous- (VM) or lymphatic- (LM) and seven arterio-venous malformations (AVM) occurred as well as three arterio-venous fistulas (AVF). One patient had a venous-lymphatic malformation. Regarding complex vascular malformations like Sturge-Weber syndrome or Wyburn-Mason syndrome two patients were found for each syndrome. In addition, three patients with a segmental spread of their hemangiomas were suspected of having a Sturge-Weber syndrome. This analysis of patients was performed with respect to age, sex, location, symptoms and ophthalmological findings, diagnostics, treatment procedures and outcome. The scope of analysis contained 44 female patients and 20 male patients. The age distribution was very inhomogeneous from infant up to a 79-year old patient. The most common location was in 30 times a purely periorbital manifestation. The second most frequent manifestation with 14 times was a combination of intra- and periorbital spread. In eleven cases the diagnosis was a purely intraorbital expression. In nine cases an intracranial involvement was observed. In two cases an intraorbital and intracranial manifestation were recorded and in seven cases both intra-, periorbital and intracranial manifestation. Overall, 44 out of 64 patients had symptoms or pathological ophthalmological findings. These occurred in 85.7 % of LM-cases, in 80 % of the AVM/AVF-cases, in 75 % of the cases with complex malformations as well as in 71.2 % of VM-cases and in 50 % of hemangioma patients. Out of these 44 cases with symptoms or ophthalmological findings 31 different visual disorders have been expressed. Patients with LM complained about 21 different ophthalmological disturbances. While the VM and the AVM/AVF patients reported 14 various symptoms or ophthalmological findings, the hemangioma patients had only nine. The overall outcome showed a large number of ophthalmological disturbances due to the fact, that these symptoms occurred mostly combined. If the lesion was complex, there was a higher correlation that it spread and that the symptoms were more serious and more frequent. The analyzed cases with hemangiomas never showed a visual reduction whereas VM caused this symptom in two, LM in eight, AVM/AVF in seven, Sturge-Weber syndrome and Wyburn-Mason syndrome in three cases. Based on a specific location an exact forecast concerning the expected symptoms was not possible. The lesions were often too much complex and could only be divided in intra- and/or periorbital. In over half of the cases (51.6 %) an imaging diagnostic has been initiated, most common for patients with complex malformations (75 %). In ten out of 14 cases imaging diagnostics was initiated for patients with a VM or LM (71.4 %). 70 % of the AVM/AVF-, and 13.6 % of the hemangioma patients have undergone imaging diagnostics. The most common application was the MRI (81.8 %) followed by CT (including angiography) (36.4 %) and ultrasonography (30.3 %). This frequency distribution is associated with the potential spread behavior of hemangiomas (self-induced involution) and vascular malformations. For 73.4 % of the patients a therapy was initiated at Marburg ENT clinic as well as alio loco. In 72.3 % it was a combination of therapies and in 27.7 % a monotherapy was followed. In 42.3 % of the cases a conventional surgical method was chosen as therapy method, as in 21.2 % it was a laser therapy, in 18.3 % it was a corticosteroid therapy, in 12.5 % it was an embolization and in 5.8 % it was a sclerotherapy. A monotherapy was selected in eight cases for the treatment of hemangiomas, in three cases as therapeutic trial in VM and in two cases with AVM/AVF. Especially in more complex and larger vascular malformations several therapeutic trials had to be executed. For example for 14 patients with LM had twelve therapy combinations and for all patients with Sturge-Weber syndrome and Wyburn-Mason syndrome. Even hemangiomas and VM had to be treated in six cases several times. Depending on the type of the lesion different therapies were combined like surgery, laser therapy, cortisone, sclerotherapy, embolization, radiation, Interferon, Thalodomid, antiestrogen and cryotherapy. Propranolol as a therapy for hemangiomas could not be considered because this analysis was performed before the determination of effectiveness of this medication. The "wait and see"-strategy was selected most frequently for hemangiomas (47.1 %). The complexity and difficulty of the treatment of intra- and periorbital hemangiomas and vascular malformation is reflected by the numerous different treatment methods, in some cases used on one patient. As the therapy depends on age, symptoms and exact localization, there is no form of therapy which can be advised for all patients. The outcome is related to the respective specific type of malformation and their spread. An interdisciplinary treatment is indispensable.