Eagle's syndrome - A report of two cases.

INTRODUCTION: Eagle's syndrome is defined as elongation of the styloid process or the stylohyoid ligament mineralization complex which consist of styloid process, stylohyoid ligament and lesser horn of hyoid bone. It is a rare entity, is not commonly suspected in clinical practice. It is characterized by recurrent facial and throat pain, dysphagia, odynophagia, parapharingeal foreign body sensation, otalgia and neck pain. Eagle's syndrome can be treated conservatively (lacing local anesthetic into the styloid process and stylomandibular ligament attachment) or surgically. Its pathogenesis and threatment modalities are still being debated while different theories have been presented. CASE REPORT: The two traditional surgical approaches to styloidectomy (removal of the elongated portion of the styloid process) were presented the intraoral approach and the extraoral approach. We presented two cases (49 years and 34 years old males), with bilateral and unilateral elongated styloid process. The surgical treatment included unilateral right side stiloidectomy by intraoral approach in the first case and right styloidectomy by extraoral approach in the second case. In both eases post-operative course passed regularly with no complaints at regular postoperative control. CONCLUSION: Surgical techniques for treatment of Eagle's syndrome have many advantages and disadvantages. We believe that the length of the styloid process or the calcified ligament is a decisive parameter for the selection of techniques and approach.

A 5-year retrospective analysis of necrotizing fasciitis--a single center experiences.

BACKGROUND/AIM: Necrotizing fasciitis (NF) is usually an acute infection of superficial fascia with rapid progression in around soft tissue. If not promptly recognized and aggressively treated NF usualy leads to sepsis and multiorgan failure with fatal outcome, thus early diagnosis and prompt surgical treatment are crucial for healing of these patients. The aim of this article was to evaluate the clinical presentation of all patients with acute NF diagnosed and treated in surgical clinics of Clinical Center of Vojvodina, Novi Sad, Serbia. METHODS: The medical records of patients treated for acute NF localized on a different parts of the body in Clinical Center of Vojvodina, Novi Sad, Serbia, during a 5-year period (from January 2008 to December 2012) were retrospectively evaluated. This study enrolled patients admitted via Emergency Center of Vojvodina with the diagnosis of acute NF either as the primary diagnosis or with the diagnosis at discharge after surgical treatment. RESULTS: During a 5-year period there were 216 patients with final diagnosis of acute NF. Most of our patients (140-64.81%) were admitted with the initial diagnosis of cellulitis, abscesses, phlegmons or sepsis. Unfortunately, the clinical symptoms of acute NF were atypical at time of initial examination. Pain and swelling of the affected localization were the most presented bias of symptoms (183-84.72%). The majority of our patients were male (164-75.92%). Among the 216 patients, the most common pre-existing single factor was drug abuse (39-18.05%), followed by obesity (38-17.59%) and diabetes mellitus (31-14.35%). Trauma was most common etiological factor (22-10.8%) in infected wounds, followed by abdominal (15-6.94%) and orthopedic (11-5.09%) surgical intervention. In the present study idiopathic acute NF was diagnosed in 22 (10.18%) patients and more than one etiological factor were diagnosed in 20 (9.25%) patients. The majority of our pa- tients had type I acute NF (172-79.62%) with Streptococcal species as the most common microorganism (125-71.02%). The most common localization was an extremity (151-69.90%). The minority of our patients had head and neck lo- calization of infection (7-3.24%). Surgical treatment was performed in all the patients and most of them (183-84.72%) received the first surgery within 24 h. Other patients (23-10.64%) received operation after stabilization of general status or after getting the diagnosis of acute NF (unclear diagnosis on admission). During hospitalization, the most common complication among our patients was sepsis (156-72.22%). The mortality rate was 14.35%. CONCLUSION: Acute NF is a rare but very difficult and sometimes life-threatening disease of superficial fascia and around soft tissue. If acute NF is suspected, early radical excision of all the affected tissue with exploration and excision of superficial fascia with pathological and microbiological assessment are most significant for treatment. Appropriate antibiotics and intensive care set ting to manage other organ failure of NF are recommended at the same time with surgery.

Li-Fraumeni syndrome: a case report.

INTRODUCTION: Li-Fraumeni syndrome (LFS) is a very rare familial disease with the predisposition to the development of malignant tumors, such as osteosarcoma, breast cancer, brain neoplasm, leukemia, and adrenal tumors. Inheritance is autosomal dominant and is caused by heterozygous mutations in the p53 gene. The diagnosis is based on clinical criteria: a person under the age of 45 years suffering from sarcoma, the closest relative younger than 45 years diagnosed with cancer and a relative of the first or second degree, which is up to 45 years, was diagnosed with cancer and was diagnosed with sarcoma at any age. CASE REPORT: The presented family with three members diagnosed with malignant disease typical for LFS suggests the need to carefully follow those diagnosed with LFS related tumor. A 24-year-old man diagnosed and treated for osteosarcoma of the maxilla died in the first year. His younger brother was submitted to surgery due to osteosarcoma of the mandible three years later, and a year later in his 24 year he had no signs of locoregional recurrence. Their mother was operated in 1996 for glioblastoma multiform brain cancer and ductal carcinoma, and died two years later at the age of 33. CONCLUSION: The presented family highlights the need for careful examination, inspection and notification of the risks of family members diagnosed with LFS related tumors.

[Optimal postoperative radiotherapy treatment of orbital rhabdomyosarcoma].

INTRODUCTION: Rhabdomyosarcoma (RMS) is the most common sarcoma of the soft tissue, mostly affecting the region of head and neck (orbit, paranasal sinus). Histological types include embryonal (66-70%) with better prognosis, and alveolar type (20%) with poorer prognosis. There are also diffuse anaplastic and undifferentiated sarcomas (10%). Due to multimodal therapy approach (surgery, chemotherapy and radiotherapy), RMS survival rate is considerably improving. Nevertheless, early diagnosis of RMS is of crucial importance for the outcome of treatment. Standard conformal radiation therapy is very complex due to closeness of many critical structures of head and neck, thus limiting optimal tumor dose coverage. CASE OUTLINE: Patient aged 59 years, surgically treated for RMS of nasal cavity several times before radiation therapy treatment. Due to relapse, patient was re-operated, when subtotal re-resection of the maxilla with exenteration of the right orbit was done. The patient received IV cycles of polychemotherapy postoperatively. Standard procedure and planning for conformal radiation therapy did not lead to acceptable irradiation plan, and hence modification in the patient's preparation was done, resulting in optimal therapeutic plan according to internationally recognized recommendations. CONCLUSION: The reported case shows a rare pediatric tumor, which often occurs in children but extremely rare in adults. The problem of inadequate isodose distribution obtained by standard conformal plan was solved by placing bolus material into the orbital cavity.The outcome of the treatment plan showed much better isodose distribution and tumor bed coverage.

[Chondroma of the skull base and maxilla].

INTRODUCTION: Chondromas are uncommon benign tumors of cartilaginous origin. Although chonodroma of the jaw is extremely rare, the commonly involved are the anterior portion of the maxilla, condyle and coronoid process. A chondroma is a painless, slow growing tumor causing destruction and exfoliation of teeth. On a radiograph, the tumor appears as a cyst-like radiolucent lesion, while some are sclerotic. The borders are usually ill-defined. Irregular calcifications may be seen with radiolucencies and then it is an osteochondroma. MATERIAL AND METHODS: We present a patient with a resected and histologically proven chondroma of the skull base and maxilla. A 65-year-old female was admitted to our clinic with swelling and breathing difficulties. MRI showed a large soft tissue mass of the skull base and maxilla. Clinicopathological and radiological features were examined by computed tomography (CT) and magnetic resonance imaging (MRI). DISCUSSION AND CONCLUSION: Chondromyxoid fibroma (CMF) is a rare, benign cartilaginous tumor that often occurs in the metaphyses of proximal tibia, proximal and distal femur and small bones of the foot. The differential diagnosis is wide and includes simple or aneyrismal bone cyst, giant cell tumor, nonossifying fibroma, fibrous dysplasia, enchondroma, chondroblastoma, eosinophilic granuloma and fibrous cortical defect. Our case demonstrates an uncommon occurrence in the maxillary sinus: CMF with nasal, pterygoid and orbital infiltration. In the diagnosis of an intracranial chondrocytic tumor, it is important to distinguish it from enchondroma and chondrosarcoma. MR provides a detailed assessment of soft tissue masses of the craniofacial region, while CT offers superior analysis of bone structure involvement. The present case underlines the importance of MR examination in the diagnosis of soft tissue masses in the craniofacial region.