Marton König

Treatment of esthesioneuroblastomas.

OBJECTIVE To analyze the clinical features, treatment outcomes, pattern of failures, and course of the disease of a cohort of patients treated for esthesioneuroblastoma (ENB) with craniofacial resection (CFR) at a single institution during a 12-year period. MATERIAL AND METHODS Retrospective analysis of 11 patients with ENB treated with CFR in a tertiary care academic medical center from 1998 to 2009. RESULTS Median age at diagnosis was 51 years (range 41-67 years). The most common presenting symptom was nasal obstruction (91%). Four patients (36%) presented with Kadish stage B, six patients (55%) with Kadish stage C, and one patient (9%) with Kadish stage D. The initial treatment was craniofacial resection (CFR) alone for three patients (23%), CFR followed by postoperative radiation therapy (RT) in seven patients (64%), while one patient (9%) received both neoadjuvant and adjuvant RT in addition to surgery. The mean and median follow-up times were 66 and 58 months, respectively (range 23-158 months). Seven patients are currently alive with no evidence of disease (64%), while two patients are alive with disease (18%). Overall survival was 100% at one year postoperatively and 80% five years after the primary treatment. The progression free survival was calculated to 73% at one year and 64% at five years. CONCLUSIONS ENB is an uncommon diagnosis with an incidence of 0.037/100,000 persons/year in the catchment area of our institution. Treatment can be challenging, especially with advanced disease. CFR with RT offers good oncologic disease control with minimal morbidity.

Reactive Expansive Intracerebral Process as a Complication of Endovascular Coil Treatment of an Unruptured Intracranial Aneurysm: Case Report

BACKGROUND AND IMPORTANCE:As a consequence of the increased use of endovascular coiling of intracranial aneurysms, a growing number of case reports on complications are being reported. This article presents a case with a previously undescribed complication of coil treatment: a reactive, noninfectious process after coiling of an unruptured intracranial aneurysm CLINICAL PRESENTATION:A 60-year-old hypertensive woman with hypoxic encephalopathy after respiratory arrest following a total thyroidectomy had extensive intentional myoclonus and reduced quality of life as sequelae. An asymptomatic 15-mm internal carotid artery bifurcation aneurysm was discovered on magnetic resonance imaging (MRI) 6 months after the thyroidectomy. After documented growth, the aneurysm was treated endovascularly with bare platinum Guglielmi detachable coils. Three months later, an expansion in the right frontal lobe cranially to the coiled aneurysm was observed. The lesion had grown at the 12-month postcoil MRI and, because of its increasing mass effect, was resected through a craniotomy 2 years after the coiling. As a result of lesion regrowth and cyst formation, she underwent a new craniotomy 5 years later with excision of the now 21-mm large coiled aneurysm, internal carotid artery clip reconstruction, and lesionectomy. Five months postoperatively, the process had not recurred. No signs of malignancy or infection were observed during the histological evaluation of the resected tissue. The tissue is described as a reactive, noninfectious process, most likely resulting from the coils acting as a foreign body. CONCLUSION:This article presents a case with a reactive expansive intracerebral process as a complication to endovascular coil treatment of an unruptured intracranial aneurysm.

Multimodal treatment of osteogenic sarcoma of the jaw.

Osteosarcomas (OSs) account for 40 to 60% of primary malignant bone tumors. About 10% occur in the head and neck region, frequently in the mandibula or maxilla. We treated a 30-year-old patient with 26-month history of right-sided facial pain and paresthesia. Investigation showed high-grade OS of the right mandibular coronoid process, affecting the mandibular nerve, middle cranial fossa, internal jugular vein, and internal carotid artery (ICA). True en bloc resection was performed after upfront adjuvant chemotherapy. The ICA was trap-ligated intradurally, whereafter the floor of the middle fossa, including the mandibular nerve and the glenoid fossa, was detached from the skull base in one piece. Subsequently, a hemimandibulectomy, total parotidectomy, ICA sacrifice, and removal of the pterygoid plates and muscles were performed, and the abovementioned structures were removed as a solitary specimen, including the facial nerve branches overlying the tumor. A sural nerve graft was interposed between five major facial nerve branches to reanimate the face. The patient had an uneventful recovery, is able to eat, and has a partial facial nerve palsy. He has no tumor recurrence 26 months after surgery. OS of the jaw should be treated with radical surgery as the primary modality.

Median Facial Cleft with a Frontoethmoidal Encephalocele Treated with Craniofacial Bipartition and Free Radial Forearm Flap: A Case Report

We describe a patient with a median facial cleft with a frontoethmoidal encephalocele, hypertelorism, hydrocephalus, and cerebrospinal fluid (CSF) leakage referred to our department due to numerous complications after previous surgical treatments. An 8-year-old girl, born with median cleft syndrome, underwent neurosurgical repair of the encephalocele at another hospital and cleft lip/palate repair later in the same year. Her hydrocephalus was treated with a ventriculoperitoneal shunt, but she underwent numerous shunt revisions due to recurrent intracerebral infections. In 2008, she was rehospitalized due to a gram-negative meningitis and cerebral abscess. She underwent surgery where part of her frontal bone was removed due to osteomyelitis. She was referred to our department due to persistent CSF leakage, recurrent infections, and significant dura defect. In addition, she had hypertelorism and a strongly reduced vision. We performed a monobloc and facial bipartition osteotomy where 15 mm of her frontal and nasal bone was removed after facial bipartiton. The dura defect was closed using a free fasciocutanous flap. The patient had no CSF leakage or infections postoperatively, and her hypertelorism was reduced. The case represents the first monobloc and facial bipartition osteotomy performed in Norway as a part of the treatment of median cleft syndrome with a nasoethmoidal encephalocele.