[Extracranial vascular anomalies (hemangiomas and vascular malformations) in children and adolescents--diagnosis, clinic, and therapy]. / Extrakranielle vaskuläre Fehlbildungen (Hämangiome und vaskuläre Malformationen) im Kindes- und Jugendalter--Diagnostik, Klinik und Therapie.

The field of extracranial vascular anomalies is considered as special focus of pediatric otolaryngology and it has shown a rapid development during the last years. The reason for this interest is finally also due to the global acceptance of the classification introduced by the ISSVA (International Society for the Study of Vascular Anomalies). Hemangiomas are the most frequently observed vascular tumors. Today the systemic propranolol therapy is mostly used for therapy of hemangiomas requiring treatment. Increasingly, the topical application of beta blocker is discussed while the benefit in the head and neck seems to be limited. Vascular malformations are classified according to the morphology of the affected part of the vascular system in arterial, venous, arterio-venous, lymphatic, capillary, and combined vascular malformations. Conventional surgery, sclerosing therapy, and laser treatment are invasive options for the treatment of lymphatic malformations. The options for the treatment of venous malformations could be significantly improved during the last years. In this context, the use of Nd:YAG laser, the conservative treatment of the localized disseminated intravascular coagulation with low-molecular weight heparin, the re-discovery of bleomycin as effective sclerosing agent, and the improvement of alcohol-based embolization agents must be mentioned. Today the treatment with dye laser is the preferred therapy for capillary malformations and it is superior to other therapeutic options as for example photodynamic therapy. Arterio-venous malformations as representatives for high-flow lesions are the high-risk lesions. Frequently they are compared to malignant head and neck tumors, in particular when a curative treatment can no longer be assured because of diffuse or multifocal extent and when the disease shows a progressive course. The combined treatment of embolization and surgical resection and if necessary consecutive defect reconstruction have turned out to be appropriate for arterio-venous malformations. Incurable findings are still a major challenge. Despite the introduction of antiangiogenetic drugs in oncology, the medicamentous therapeutic approach could not be established for arterio-venous malformations up to now.

[Lymphatic malformations in the head and neck region. Clinical aspects and therapeutic options]. / Lymphatische Malformationen im Kopf-Hals-Bereich. Klinik und therapeutische Möglichkeiten.

Lymphatic malformations are congenital abnormalities of the lymphatic system which occur predominantly in the head and neck region. According to their dominant clinical and morphological characteristics, these are classified into micro- and macrocystic forms. Established therapies for lymphatic malformations include conventional surgery, sclerotherapy and laser treatment. Despite the significant improvements in therapeutic options seen in recent years, treatment of extensive lymphatic malformations remains an interdisciplinary challenge. Close-knit interdisciplinary cooperation is necessary to provide optimized care for affected individuals.

[Venous and arteriovenous malformations in the head and neck region. Therapeutic options and challenges]. / Venöse und arteriovenöse Malformationen im Kopf-Hals-Bereich. Therapeutische Möglichkeiten und Herausforderungen.

Venous malformations are the prototype low-flow malformations in the head and neck region. Arteriovenous malformations (AVM) represent the main high-flow malformations. In recent years it has been possible to significantly optimize the therapeutic options for venous malformations. In addition to conventional surgery, laser treatment and sclerotherapy have become established techniques and the importance of embolization with new alcohol-based materials is increasing. AVM are progressive and destructive diseases. Therapy of choice is usually a combined treatment comprising embolization and surgical removal of the arteriovenous nidus. This curative approach is usually possible if diagnosis is made at an early stage. Incomplete embolization or sole ligation of the arterial supply causes progression. There is a clear need for improved therapeutic methods and pharmacotherapeutic approaches.

Prospective evaluation of reliability of cone-beam computed tomography in detecting different position of vibroplasty middle ear implants.

OBJECTIVES: This study had the following objectives: (i) to determine the accuracy of determination of Vibrant Soundbridge position in the spectrum of typically implanted sites in the middle ear, (ii) to assess interobserver agreement between three observers with different levels of radiology experience and (iii) to determine the suitability of cone-beam computed tomography (CT) to be used as the baseline radiological assessment post implantation, confirm ferromagnetic transducer (FMT) position. DESIGN: Prospective research study. Using four fresh human cadaveric heads, different types of vibroplasty were performed. After each step, cone-beam CT was performed for each of the four cadaveric heads. SETTING: University hospital (ENT and Neuroradiology). PARTICIPANTS: Four fresh cadaveric heads of human donors were operated and analysed by radiological imaging. MAIN OUTCOME MEASURES: There are different ways of coupling an ferromagnetic transducer to the anatomical structures of the middle and inner ear. Possibilities of differentiation between these coupling variants should be presented. RESULTS: The individual reconstruction view was significantly different from a standardised view for each observer (observer 1: P = 0.003; observer 2: P = 0.001; observer 3: P = 0.002) for all coupling variants combined as well as for each individual coupling variant (overall correct diagnosis: 100% versus 60%). Regarding the frequency of correct diagnosis, no significant differences were found between the three observers (P > 0.500) for each individual coupling variant as well as for all coupling variants combined. The worst rates of correct diagnosis were found in the standardised view for incus (42%), stapes (0%) and TORP (17%) vibroplasty. CONCLUSION: Cone-beam CT as a radiological control for Vibrant Soundbridge is safe and adequately sensitive and reliable and is therefore suitable for clinical investigation. The position of the ferromagnetic transducer in the middle ear and the presence or absence of an additional coupler could be determined in this study. Therefore, cone-beam-CT is useful for the assessment of device failure when there has been gross displacement of the ferromagnetic transducer (or smaller displacements in case of a baseline postoperative cone-beam CT). Regarding the quality of imaging, cone-beam CT produced accurate results with different observers with widely varying radiological experience.

Phleboliths from venous malformations of the head and neck.

OBJECTIVES/HYPOTHESIS: Phleboliths in venous malformations (VM) of the head and neck are often observed and may cause significant symptoms. Only a few articles refer to the morphology and composition of the phleboliths in VM. The objective of this study was to analyse and to demonstrate their composition and morphology. METHODS: Patients with VM presenting to a vascular anomalies centre during a three-year period were identified. The incidence of phleboliths was analysed followed by morphological and structural analysis with cone beam tomography and X-ray diffraction. RESULTS: Phleboliths were identified in 28/98 patients with VM of the head and neck. Seven patients underwent conventional surgery to reduce the volume of the VM or to remove the phleboliths, which were localized in the cheek (3 cases), submandibular region (2 cases), infrahyoidal neck or upper eyelid (1 case each). The structural analysis showed that more advanced lamination and an increasing radiopacity of the cortex was observed in larger phleboliths. X-ray powder diffraction analysis revealed that the main constituent in the pulverized phleboliths was carbonate-fluorohydroxylapatite. CONCLUSION: This study shows in a vivid way that phleboliths from VM of the head and neck area show a laminar structure and consist of apatite, without any indication of differences in their chemical composition. Treatment of localized intravascular coagulopathy in VM might be able to prevent the formation and the progression of phleboliths. Hypothetically, another option might be lithotripsy.

[Intracochlear electrode position: evaluation after deep insertion using cone beam computed tomography]. / Intracochleäre Elektrodenlage : Beurteilung mittels "cone beam computed tomography" nach tiefer Insertion.

INTRODUCTION: Due to the increasing number of cochlear implantations (CI), postoperative radiological verification of the electrode position, e.g., with respect to quality control, plays a central role. The aim of this study was to evaluate the intracochlear position of deep inserted electrodes by cone beam computed tomography (CBCT). MATERIALS AND METHODS: CBCT data sets (Accu-I-tomo, Morita, Kyoto, Japan) of 22 patients (28 ears operated between 2008 and 2011) were retrospectively analyzed. All patients underwent a CI (round window approach) with deep insertion of the electrode (Flex soft or standard electrode from MedEl©). CBCT data were analyzed for intracochlear position of the electrode (scala vestibuli, scala tympani, malposition between the scalae) and the certainty of this evaluation. RESULTS: All ears could be evaluated with the status certain or relatively certain in the basal turn of the cochlea. Thereby, the electrode array was inserted into the scala tympani in 93% (n = 26). Primary insertion into the scala vestibuli and the scala media was observed in 3.5% of the ears, respectively. In the apical part of the cochlea, only 32% (n = 9 ears) could be evaluated with relative certainty. The remaining 68% of cases could not be evaluated. Of the 32% interpretable cases in the apical part of the cochlea, 25% (n = 7) were inserted into the scala tympani, 3.5% (n = 1) into the scala vestibuli, and 3.5% (n = 1) were malpositioned between the scalae. CONCLUSION: The exact evaluation of the intracochlear position of the electrode by CBCT is only possible in the basal turn of the cochlea. In deep insertion, determination of the position in the medial and apical parts of the cochlea by CBCT is still not possible. Furthermore, the round window approach allows reliable implantation into the scala tympani.

Lymphatic malformations involving the parotid gland.

INTRODUCTION: Cases of salivary gland involvement of lymphatic malformations have been occasionally reported in the literature. Of all the lymphatic malformations in the salivary glands, the parotid is the most common site. The present study aimed to analyze a series of lymphatic malformations of the parotid gland. MATERIALS AND METHODS: A retrospective analysis of the localization, symptoms, management and outcome was performed. RESULTS: Out of a total of 20 patients with lymphatic malformations of the parotid gland, 4 patients suffered from lymphatic malformations limited to the parotid gland (type I) and 16 patients from extensive cervicofacial lymphatic malformations involving the parotid gland (typeII). In 2 cases with type I disease and 4 cases with type II disease the malformations could be completely resected. In 3 patients with type II lymphatic malformations a partial resection was performed. The other patients were closely observed. 8 of them had already been treated elsewhere with surgery, sclerotherapy or laser therapy. One patient suffered from facial paralysis and 1 from transient facial nerve weakness immediately after surgery. In all, 11 patients suffered from persistent lymphatic malformations despite several attempts to reduce or resect the lymphatic malformation. CONCLUSION: The treatment of lymphatic malformations of the parotid gland remains challenging and persistent disease after therapy is common. Care should be taken to excise the entire malformation during initial surgery in order to avoid recurrence.

Hemangiomas of the nasal tip: an approach to a therapeutic challenge.

OBJECTIVE: Hemangiomas of the nasal tip (HNT) are commonly described as "Cyrano" or "Pinocchio nose". They may cause significant aesthetic and functional impairment. It is still controversial how and when HNT should be treated. The risk of severe complications like irreversible contour deformities, growth disturbance, ulcerations, and nasal obstruction may obligate a therapeutic intervention. The aim of this study is to overview and to analyze different therapeutic approaches which were practiced over the last decade in two specialized centers. METHODS: This is a retrospective study which includes the analysis of demographic parameters, extent of the lesion, therapeutic interventions, outcome, and follow-up interventions. The analysis includes a blinded evaluation of photographic series for evaluation of the extension at the time of presentation and of the final outcome. RESULTS: Twenty-three children with HNT were analyzed who presented from 04/01/1998 to 03/31/2009. The age at presentation ranged from 1 to 63 months. On 6 patients (26%) conventional surgery was performed, 6 (26%) were treated with Nd:YAG laser, 6 (26%) were only observed, in 3 cases (13%) cryotherapy and in 2 patients (9%) treatment with Propranolol was performed. The results were evaluated between 9 months and 10.5 years after treatment (mean: 35.5 months). Limited lesions which were only observed showed a good tendency of regression. Significant wound healing disturbance and scar formation was observed after Nd:YAG laser therapy. Secondary rhinoplasty in adulthood was recommended to two patients. CONCLUSION: Limited lesions do not require therapy. The results with Propranolol are encouraging. Laser- and cryo-therapy have to be critically reevaluated. Treatment of choice for lesions that are not suitable for beta blockers and residual disease is conventional surgery.

Wyburn-Mason syndrome: a case report.

OBJECTIVE: Wyburn-Mason syndrome is a rare disease associated with multiple arteriovenous malformations of the brain, orbit, and face resulting from an insult occurring during embryonic development. CASE REPORT: We present the clinical and radiological features of a 47-year-old-man with Wyburn-Mason syndrome who suffered from recurrent bleeding episodes primarily at the nasal corner of the left orbit. After radiotherapy and several angioembolisations, surgical reduction with exenteration of the left orbit was performed and resulted in reduced bleeding. Two years later the patient presented with maxillary sinus empyema. Due to massive endonasal bleeding, endoscopy could not be performed and the maxillary empyema was treated via a transorbital approach. CONCLUSION: Although close observation represents the standard of care in Wyburn-Mason syndrome, patient-specific management decisions are required in the presence of symptoms or complications. In the presented case, surgical intervention proved to be successful.

Analysis of clinically suspected orbital cavernomas.

BACKGROUND: Orbital cavernomas are low-flow vascular malformations that are the most common benign neoplasms of the orbit in adults, typically becoming symptomatic in the middle age. METHODS: The medical records of six patients with clinically suspected orbital cavernomas receiving elective surgical excision were analysed concerning symptoms, physical findings, treatment results and visual outcome. The pathologic slides were evaluated, and additional immunohistochemical stains were done if necessary to obtain diagnosis. RESULTS: Histologic evaluation revealed three of six cases not being cavernomas, although the clinical and macroscopic findings were consistent with orbital cavernomas. Two of them were haemorrhagic lymphangiomas, and one was a solitary fibrous tumour. CONCLUSIONS: Haemorrhagic lymphangiomas and other vascular tumours may mimic orbital cavernomas regarding anamnesis, radiologic and intraoperative findings and gross examination. Therefore, exact histologic evaluation is necessary to get the correct diagnosis.

Propranolol therapy for infantile haemangiomas: review of the literature.

OBJECTIVES: Haemangiomas are the most common tumors of infancy affecting approximately 1 in 10 children. Unlike other tumors, haemangiomas enter an involution phase, during which they usually regress over the next several months to years. Sometimes intervention is required due to proliferative growth which is complicated by ulceration, bleeding, persistent aesthetic deformity or infection. METHODS: Review of the literature. RESULTS: Propranolol, a nonselective beta-blocker, has recently been introduced as a novel modality for the treatment of proliferating haemangiomas. The exact mechanism of action of propranolol in the treatment of haemangiomas remains unclear, but vasoconstriction, down-regulation of angiogenic factors such as VEGF and bFGF and up-regulation of apoptosis of capillary endothelial cells may be responsible for the reduction of haemangiomas. Besides, an inhibition of MMP-9 and HBMEC expression by propanolol is discussed as possible mechanism influencing the growth of haemangiomas. However, there are different case reports of successfully treated infants in the current literature. CONCLUSION: There is the obtain that propranolol will detach steroids in the therapy for infantile haemangiomas.

[Differential diagnosis of vascular malformations of the upper aerodigestive tract]. / Zur Differenzialdiagnostik vaskulärer Malformationen im oberen Aerodigestivtrakt.

The differential diagnosis of vascular malformations of the upper aero-digestive tract may represent a diagnostic challenge and become a hindrance for therapy planning. The terminus "extracranial vascular anomalies" of the Head and Neck integrates hemangiomas and vascular malformations. The differential diagnosis contains benign and malign neoplasms, naevi, pigmentations and purpura.

[Therapy for prenatally diagnosed lymphangioma--multimodal procedure and interdisciplinary challenge]. / Die Therapie von pränatal diagnostizierten Lymphangiomen--Multimodales Vorgehen und eine interdisziplinäre Herausforderung.

INTRODUCTION: Ultrasound diagnostics enable the prenatal diagnosis of extensive lymphatic malformations. The diagnosis is made prenatally or directly after birth in 50-60% of the cases, in about 80% of the afflicted in the first year of life and up to 90% of the cases in the second year of life. PRENATAL DIAGNOSTICS AND POSSIBILITIES OF PRE- AND PERINATAL INTERVENTIONS: The so called hygroma colli is a primary symptom, which is detected during the routine ultrasound examination. Lymphatic malformations, which are diagnosed antenatally are often associated with chromosome abnormalities. The EXIT procedure allows invasive therapeutic or diagnostic interventions. Hereby the airway safety of Newborns with extended cervical or zerviko-facial lymphangiomas can be warranted. The survival chance and the prognosis may be significantly improved. MANIFESTATION AND ENT-CLINICS: 72 patients (mean age 12.3 yrs) with lymphatic malformations of the Head and Neck presented for the first time during a 5-years period until 31.12.2008 at the Department of Otolaryngology of the University of Marburg. Therapeutic interventions consisted in conventional surgery, laser assisted therapy, sclerosing therapy or a combined therapeutic approach. CONCLUSION: Extensive lymphatic malformations of the Head and Neck challenge treating physicians and affected families. Advancements of prenatal diagnostics and opportunities of peri- and postnatal interventions contribute to an improved survival chance and prognosis. Intensive interdisciplinary cooperation in specialised centers is of outstanding importance for an optimal care of these patients.

Orbital lymphatic malformation showing the symptoms of orbital complications of acute rhinosinusitis in children: a report of 2 cases.

Orbital lymphatic malformations are benign cystic malformations of the lymphatic system. The present report shows two cases with symptoms of orbital complications of acute rhinosinusitis with proptosis, compressive optic neuropathy, loss of vision and cellulites in children. Magnetic resonance imaging (MRI) revealed a well-demarcated intraorbital mass with heterogeneous signal conformable with lymphatic malformation in both cases. A tumor extirpation was performed via lateral orbitotomy in both cases. Postoperatively the symptoms and especially the loss of vision improved completely. Histological analysis of the surgical specimens verified lymphatic malformations of the orbit. Orbital lymphatic malformations can mimic the symptoms of orbital complications of acute rhinosinusitis. The existence of lymphatic malformation should be considered in every orbital complication of rhinosinusitis in children.

Surgery in patients with vascular malformations of the head and neck: value of coagulation disorders.

OBJECTIVES: The surgical treatment of congenital vascular malformations always bears the risk of severe bleeding. The aim of this study was to investigate the prevalence of haemostatic abnormalities in patients with congenital vascular malformations of the head and neck which could additionally complicate the complex surgery of vascular malformations. METHODS: Thirteen patients with congenital vascular malformations of the head and neck, who underwent scheduled surgery, were reviewed regarding their haemostatic tests, including prothrombin time, activated partial thromboplastin time, fibrinogen level, platelet count, platelet function analyzer-epinephrine (PFA-Epi) and -adenosine diphosphate (ADP) (PFA-100), von Willebrand factor (vWF) and D-dimers. RESULTS: In six of 13 patients an impaired primary haemostasis was detected by prolonged PFA-Epi and one of these patients also had a slightly prolonged PFA-ADP. Additionally, two of the patients showed mild coagulation factor deficiency. One patient had a mild vWF deficiency that was not detected by PFA-100. CONCLUSION: Prior to surgery of congenital vascular malformations of the head and neck, a detailed coagulation analysis is needed in order to identify those patients at increased risk of bleeding. This proceeding is supported by the fact that a relevant portion of the patients with congenital vascular malformations of the head and neck show an impaired primary haemostasis.

[State of the art of classification, diagnostics and therapy for cervicofacial hemangiomas and vascular malformations]. / "State of the Art" zur Klassifikation, Diagnostik und Therapie von zervikofazialen Hämangiomen und vaskulären Malformationen.

The successful treatment of vascular anomalies depends on profound knowledge of the biologic behavior of vascular lesions and their correct classification. On the base of the clinical course Mulliken and Glowacki developed a biologic classification that was accepted as official classification by the ISSVA (International Society for the Study of Vascular Anomalies). Based on an extended literature research, this manuscript will give an overview of different internationally accepted treatment concepts. Even if a wait-and-see strategy can be recommended in many cases of uneventful hemangiomas in infants the proliferative growth of such lesions requires an adequate treatment indication. Vascular malformations that persist lifelong require treatment in the majority of the cases, especially when clinical symptoms occur. Based on individual parameters such as the diameter, location or growth behavior, different therapeutic options as cryotherapy, corticosteroids, laser therapy, sclerotherapy, surgical intervention and/or embolisation can be performed successfully. None of those treatment concepts, however, represents the only treatment method of choice.

Bipolar radiofrequency induced thermotherapeutic volumetric reduction of VX2 metastases in an animal model.

BACKGROUND: Radiofrequency-induced thermotherapy has shown promising results in the palliative treatment of various tumor entities. The purpose of this study was to investigate the effectiveness of bipolar radiofrequency volumetric tissue reduction (VTR) on lymph node (LN) metastases in the VX2 SCC model. MATERIALS AND METHODS: Six male New Zealand white rabbits, with palpable metastatic disease within the parotid lymph nodes, were treated using the Celon-ProSurge probe, needle length 10 mm, diameter of 2.3 mm. The animals were sacrificed on the 4th, 8th, 11th, 14th, 18th and 22nd postoperative days respectively. RESULTS AND CONCLUSION: Bipolar radiofrequency VTR could prevent progression of local metastatic disease in one-third of the animals compared to the control group of untreated VX2 carcinoma rabbits. These results encourage further studies, directed at whether this treatment modality could play a role in the palliative therapy of metastatic LN. Future studies should concentrate on the refinement of the treatment parameters and optimization of the treatment duration.