1/619. E.N.T. manifestations of Von Recklinghausen's disease. Von Recklinghausen's disease (VRD) is a neurocutaneous, systemic disease characterized by CNS tumors and disorders, cafe-au-lait spots, generalized cutaneous neurofibromata, skeletal deformities, and somatic and endocrine abnormalities. It is an autosomal dominant, hereditary disorder found in approximately 1:2500 to 3300 births. There are many manifestations of this disease in the head and neck region of interest to the otolaryngologist. case reports of three patients with multiple ENT involvements are detailed. A review of the literature is presented with a brief discussion of diagnosis and treatment. The most common intracranial tumor in the adult is the acoustic neuroma, usually bilateral, while in the child it is the astrocytoma. A defect in the sphenoid bone is common and may produce temporal lobe herniation into the orbit causing pulsatile exophthalmos. Involvement of the facial bones usually causes radiolucent defects secondary to neurofibromata within nerve pathways, and a variety of asymmetrical changes, especially within the mandible. "elephantiasis" of the face is a hypertrophy of the soft tissues overlying a neurofibroma, often quite extensive and disfiguring. Laryngeal and neck involvement may compromise the airway and early and repeated surgical intervention is required. The over-all malignancy rate approaches 30%, indicating that the patient with VRD may be predisposed to developing a malignancy. There appears to be an increased surgical risk in these patients, with some demonstrating abnormal responses to neuromuscular blockade. ( info) |
| The aetiological spectrum of sphenoid sinus mucocele includes congenital anomaly, trauma, infection, allergy and surgery of the sphenoid sinus. Enlargement of the mucocele, even with a short history, can result in progressive expansion of the sinus and extension of the lesion into the pituitary fossa, the suprasellar region, nasopharynx, orbits, clivus or ethmoid air cells. It is a benign cystic lesion with an excellent prognosis when treated appropriately. Generally, these lesions are managed by an ear, nose and throat surgeon, but when there is extension into the sellar and parasellar (especially suprasellar) regions they are managed by the neurological surgeon. sphenoid sinus mucocele should be considered in the differential diagnosis when there is suspicion of a cystic lesion in these regions. Three cases of large sphenoid sinus mucocele are presented, with discussion on their neurosurgical management and a review of the literature. ( info) |
3/619. mucocele of the maxillary antrum in an eight-year-old boy. mucocele is a rare clinical entity in children. It is considered to be associated with fibrocystic disease. The capacity of mucocele to erode through the bone walls along with the increased density of its content, make the diagnosis difficult when mucocele has to be differentiated from benign or malignant tumors. In this paper, we present the case of an 8-year-old boy with mucocele of the left maxillary antrum. Both the differential diagnosis and the surgical treatment are described. ( info) |
| A 23-year-old woman was admitted with headache, nausea, vomiting and blurred vision on the left side. Neurological examination showed ptosis with a complete internal and external ophthalmoplegia and a red fullness around the left orbita. Computed tomographic scanning of the brain revealed no abnormalities. As she improved on high doses of steroids a diagnosis of tolosa-hunt syndrome (THS) seemed to be indicated. However, magnetic resonance imaging (MRI) showed a lesion with intermediate signal intensity in the left cavernous sinus. craniotomy was performed when symptoms of THS recurred. Histopathological examination revealed a meningioma with a papillary aspect and some mitoses. This case illustrates that: (1) THS is still a diagnosis by exclusion; (2) MRI and histopathological examination are important if there is any doubt about the diagnosis; and (3) also when there is no doubt, improvement after steroid therapy may be a diagnostic pitfall. Therefore, not only MRI but also orbital phlebography and angiography should seriously be considered. ( info) |
5/619. frontal sinus pneumocele. A case report. A pneumocele refers to an aerated sinus with either focal or generalized thinning of the bony sinus wall. Although the pathogenesis of a pneumocele is not yet known, it is presumed that increased intrasinusal pressure, due to a one-way valve between the nasal cavity and the affected sinus, is responsible for this condition. A 37-year-old man with frontal bossing, who underwent surgery for cosmetic reasons, is presented. ( info) |
6/619. Concha bullosa pyocele--undiagnosed for 3 years. We report a rare case of post-traumatic concha bullosa pyocele in a diabetic teenager that has gone undiagnosed for 3 years. The clinical findings, radiological features and management are discussed. The literature is reviewed. ( info) |
7/619. Non-randomized comparison of surgical modalities for paranasal sinus mycoses with intracranial extension. Mycotic infections of paranasal sinuses are frequently reported in southern asia. aspergillus and mucor species are the predominant ones. Intracranial extension of paranasal sinus mycoses is a difficult problem to manage. We report 18 cases of paranasal sinus mycoses with intracranial extensions. The commonest manifestations were nasal discharge (67%), nasal obstruction (50%), ocular symptoms such as proptosis (44%), telecanthus (39%) and ophthalmoplegia. Computerized tomography scans were found to be quite informative regarding the nature and extent of the disease (100% sensitivity and 78% specificity). A combined intracranial-extracranial approach (six cases) gave a distinct advantage over only adopting an extracranial approach (12 cases). A 17% incidence of CSF leak was noted by adopting only an extracranial approach as well as a recurrence in four cases out of the 12 that were treated using this method (P < 0.05). A slight increase in morbidity was associated with the combined intracranial-extracranial treatment, but no recurrence or significant complications were noted in this approach. ( info) |
8/619. Acute angle closure glaucoma precipitated by intranasal application of cocaine. We describe a patient who developed acute angle-closure glaucoma following the application of topical intranasal cocaine. A 46-year-old woman underwent an elective antral washout under general anaesthesia and with local application of 25 per cent cocaine paste to the nasal mucosa. Twenty-four hours post-operatively the patient developed sudden painful blindness which was found to be due to acute glaucoma. cocaine with its indirect sympathomimetic activity causes mydriasis, that can precipitate acute angle-closure glaucoma in predisposed individuals with a shallow anterior chamber. Although the incidence is rare, otolaryngologists need to be aware of this potential complication. ( info) |
9/619. dentigerous cyst: report of an unusual case. A case of dentigerous cyst in the maxillary sinus of a 16-year-old boy is reported due to its unusual presentation and extensive nature. ( info) |
10/619. Hyperaeration of the sphenoid sinus: cause for concern? A case of exuberant pneumatization of the left sphenoid sinus into the pterygoid process and floor of the middle cranial fossa is presented. The fact that pneumatization of the sphenoid sinus is frequently atypical is of clinical import because there is an intimate relationship between the contents of a hyperaerated sinus and adjacent vital facial and cranial structures. It is imperative that clinicians determine the location and extent of the walls of the sphenoid sinus and its relationship to adjacent vital structures whenever endoscopic sinus surgery is contemplated to avoid morbid consequences during surgery. ( info) |