Melanoma of sphenoid sinus. Case presentation and review of literature

The primary malignant melanomas of sphenoidal sinus are very uncommon tumours. We are reporting a clinical case of a 56 years-old male who was sent from the Neurology Department with ocular pain and headache of several months evolution and without personal history. Exploration revealed a VIth palsy, diplopia and ptosis. The complementary imaging tests (CT and MR) showed and expansive tumour of the sphenoidal sinus involving the right cavenous sinus. A F.E.S.S. with biopsy was performed to confirm the diagnosis. Our patient was treated by Radiotherapy and after a 4 year follow-up period he is still alive. We have carried to a bibliographic review of this pathology whose differential diagnosis must be done with epidermoid carcinomas, adenocarcinomas and lymphomas on that location.     

Superior orbital fissure syndrome in trigemino-facial zoster

A case of trigemino-facial zoster presenting as Superior Orbital Fissure Syndrome is reported. Geniculate ganglion involvement was limited to the vestibular branch of the cochleo-vestibular nerve, without any hearing impairment or facial palsy. This case clearly illustrates that herpes zoster cranialis is a polyneuropathy of multifocal asynchronous viral activity and can present in numerous forms.     

Intra-thyroidal hemangioma—A rare congenital anomaly: Case presentation and literature review

Infantile hemangiomas (IH) present and evolve in a predictable way. In certain cases, however, they can be of clinical significance. IHs in the airway can cause significant respiratory distress. We present the first reported case of an intrathyroidal hemangioma to cause significant respiratory distress, which was successfully treated with propranolol and dexamethasone. A review of the literature was also performed.     

Cogan syndrome. Case report, review of the literature, therapy

We present a case report of Cogan's syndrome and a review of the literature. Cogan's syndrome is most likely due to autoimmune disease primarily affecting the vestibulocochlear organ and cornea and secondarily resulting in systemic disease. 40% of patients develop bilateral complete deafness and 70% show signs of systemic disease mostly in the form of cardiovascular symptoms. The mortality rate of 10% is mainly due to vasculitis. It is important to consider Cogan's syndrome in the differential diagnosis of sudden hearing loss accompanied by ocular signs in young patients, as high morbidity and mortality rates are effectively lowered by early immunosuppressive treatment.     

Sphenoid wing meningioma occurring as a lateral orbital mass.

The differential diagnosis of a mass presenting in the lateral aspect of the orbit with bony erosion and involving the skin includes many malignancies, predominantly sarcomas. A total evaluation of the mass to include histopathologic characteristics and the true extent of the disease is critical to proper management of the patient. A 68-year-old man was referred for therapy with a diagnosis of fibrosarcoma. Evaluation of the disease revealed contiguous tumor from the right temporal region into the posterior orbit, with displacement of the globe and extension intracranially. During operation a frozen section analysis of the tumor was also interpreted as fibrosarcoma. A craniofacial resection was performed, including orbital exenteration and resection of a large 10 × 12 cm segment of attached dura. There was no evidence of extension into the brain. Final pathologic evaluation of the tumor was meningioma rather than fibrosarcoma. This unusual presentation of a meningioma has never been reported in the medical literature. The difficulty with interpretation of the original biopies will be discussed, as well as the management of this case.     

Retropharyngeal abscess: An unusual presentation of Kawasaki disease. Case report and review of the literature

We report the case of a patient with Kawasaki disease whose initial presentation mimicked a retropharyngeal abscess and review the literature of this topic (16 cases reported). Fever and deep neck infection like symptoms were the only clinical findings at admission in 87.5% children. All children had a neck CT scan performed showing findings suggestive of retropharyngeal abscess. All children were started antibiotic therapy without clinical improvement and 31% of patients underwent unproductive surgical drainage of the retropharyngeal space. Otolaryngologist should be aware of atypical presentation of Kawasaki disease presentation mimicking retropharyngeal abscess. Early diagnosis is pivotal for preventing cardiac complications and avoiding the risk associated to unnecessary surgical intervention.     

Sphenoid sinus involvement in neuroblastoma: a case report and review of the literature

Neuroblastoma is the most common extracranial solid tumors of childhood. The most frequent anatomic site of the primary tumor is abdomen. Metastatic spread of neuroblastoma to lymph node, bone, bone marrow and liver are common at presentation. However, primary or metastatic involvement of sphenoid sinus is extremely rare. Here, we report a case with stage IV neuroblastoma presents with the involvement of the sphenoid sinus. To our knowledge this is the fourth case of neuroblastoma involved sphenoid sinus in English literature.     

Nasopharyngeal craniopharyngioma. Case report and literature review

Craniopharyngioma is an intracranial tumor that occurs rarely in the infrasellar region. Eight patients with craniopharyngioma located within the nasopharynx have been reported previously. These cases originated in the nasopharynx and involved the sella turcica (6), the sphenoid sinus (1), and the vomer (1). Craniopharyngioma usually originates intracranially. When there is no evidence of sellar involvement, the tumor most likely arises along the path of the craniopharyngeal duct. According to the neurosurgical literature, the optimal treatment consists of total surgical excision. Incomplete tumor removal is supplemented by adjunctive radiotherapy, which has been shown to significantly increase the survival rate. A patient with craniopharyngioma of the nasopharynx and paranasal sinuses who presented with nasal obstruction is reported. Radiographic studies were employed for tumor evaluation, and biopsy was done to establish the histopathologic diagnosis. Treatment included a combination of surgical excision and irradiation.     

Bilateral facial paralysis: a case presentation and literature review.

Bilateral facial paralysis is a diagnostic challenge, which may manifest itself as either a simultaneous or alternating form, occurring in 0.3-2.0% of patients that present with facial paralysis. The differential diagnosis of facial paralysis includes congenital, traumatic, neurologic, infectious, metabolic, neoplastic, toxic, iatrogenic and idiopathic etiologies. While idiopathic facial paralysis is the most common diagnosis, a comprehensive evaluation must be completed prior to this diagnosis in patients with bilateral facial paralysis. A representative case of simultaneous bilateral facial paralysis is presented. The literature is reviewed and discussed. A diagnostic protocol for the evaluation of bilateral facial paralysis is proposed. Bilateral facial paralysis requires a thorough evaluation and may prove to be a diagnostic dilemma.     

Case of Waardenburg Shah syndrome in a family with review of literature

Waardenburg syndrome is a rare disease characterized by sensorineural deafness in association with pigmentary defects. Depending on additional symptoms, WS have been classified into four types. Waardenburg syndrome type 4, also called as Waardenburg Shah Syndrome is a very rare congenital disorder with astounding variable clinical expression, characterized by pigmentary abnormalities of the hair (A white forelock of hair, premature graying) and pigmentary changes of the iris such as heterochromia or homochromia irides, sensorineural deafness and Hirschsprung disease. Three genes have been bestowed so far in consociation with EDNRB, EDN3, and SOX10 genes. The pattern of inheritance is multifarious with the SOX10 mutation affiliation with autosomal dominant inheritance whereas the EDNRB and EDN3 genes are passed down in an autosomally recessive pattern.     

Superior orbital fissure syndrome due to sinusitis: report of two cases

BACKGROUND: The potential morbidity and mortality of sphenoid sinus infectious processes are related to their proximity to vital structures within the orbit, such as the cavernous sinus and the brain. Involvement of the posterior orbit can result in superior orbital fissure syndrome, a rare entity affecting structures that cross this anatomic region. Early recognition of this syndrome is mandatory. Delays in adequate treatment may compromise the patient's prognosis. METHODS: We present two cases of incomplete superior orbital fissure syndrome due to infectious processes of the posterior ethmoid and sphenoid sinuses. CONCLUSIONS: In our experience, endoscopic decompression of the lamina papyracea shows complete recovery of extrinsic ocular motility in these patients and should be combined with intravenous antibiotic therapy.     

Conservative treatment of bilateral condylar fractures in children: Case report and review of the literature

Two children (11 year old) with bilateral condylar fractures associated with symphysis fracture were conservatively treated. Both of them were followed up for about 1 year. A review of 21 cases of bilateral condylar fracture available in the literature revealed the younger the patient, the better the outcome of TMJ function or in radiographic remodeling. However, the longer the time elapsed, the higher the incidence of remodeling deformity and dysfunction. Thus, it must be better that a close follow-up of bilateral condylar fracture in children should be continued until the end of growth period.     

Silent sinus syndrome: lack of orbital findings in early presentation

BACKGROUND: Silent sinus syndrome (SSS) is an uncommon disease process, classically described as unilateral maxillary sinus opacification that presents with enophthalmos and atelectasis of bony sinus walls from chronic negative pressure. Patients are largely free of characteristic rhinosinusitis symptoms. METHODS: Operative reports, clinic notes, and radiological studies were reviewed for cases of SSS treated in a tertiary care institution over a 7-year period. Presenting symptoms, radiological features, and surgical findings were evaluated. RESULTS: Seventeen cases of SSS were identified. Presentation ranged from incidental computed tomography (CT) scan findings to unilateral enophthalmos with altered midface anatomy. Facial pain ipsilateral to the disease process occurred in 30% of cases. Vision changes were uncommon. Radiological studies universally revealed an opacified maxillary sinus on the affected side. Maxillary sinus walls were contracted and demineralized with significant orbital floor depression in 11 advanced cases. Four early cases revealed only lateralized uncinate process on CT scan without increased orbital volume, and two moderate cases showed increased orbital volumes on CT scan without clinical enophthalmos. All patients had a significantly lateralized uncinate process at surgery, often closely apposed to demineralized orbital walls. CONCLUSION: Some authors maintain that SSS presentation must include enophthalmos. However, our series shows cases of lateralized uncinate processes and increased orbital volumes on CT scan, as would be seen in classic SSS, but lacking clinical enophthalmos. Such cases should be considered as potentially representing early SSS, before the development of clinical orbital findings.     

Lingual thyroid. Case report and review of the literature.

We review the world literature on lingual thyroid. One of our cases of lingual thyroid is added to this body of information specifically because of the patient's unusual lack of any response to hormonal suppression treatment. This behavior was suggestive of a malignant neoplasm and was, in our opinion, indication for excisional biopsy, even without any of the usual symptoms associated with lingual thyroid. To our knowledge, this behavior has not been previously reported.     

Postseptal orbital cellulitis and sinusitis. Descriptive study of 8 cases and literature review

This is a retrospective study of 8 patients with postseptal orbital cellulitis (stages II-V of Chandler) caused by sinusitis during 1999-2003. The variables analyzed in this study included age, sex, delay in diagnosis, aetiology, imaging studies, microbiology specimens collected and treatment. Delays in diagnosis were frecuent. CT was the best radiologic study. Polymicrobial infections were the rule, including anaerobes. 4 patients of 8 needed surgical drainage. A general guideline for the management of periorbital and orbital cellulitis according to the clinical staging of the process is presented.     

鳃裂癌一例报告并文献复习

正鳃裂癌是原发于鳃裂残迹的恶性肿瘤,以鳞状上皮癌为主要病理类型。发病率很低,目前文献报道多为个案。现结合广东省中医院大学城医院耳鼻喉科收治1例鳃裂癌患者的病历资料,并复习相关文献,总结鳃裂癌的诊断要点,重点讨论鳃裂癌的治疗方法,报道如下。1资料与方法.1一般资料患者男,61岁,2014年10月发现