1/54. Acquired and isolated asymmetrical palatal palsy. Benign acquired and isolated asymmetrical palatal palsy is a rare condition in childhood. We report on three cases. Typical features include: sudden onset, abnormality of the palatal components of speech (rhinolalia), nasal escape of fluids from the ipsilateral nostril. It is supposed to be caused by viral infection, but attempts at viral isolation were unsuccessful. Complete spontaneous recovery is usual, taking a few weeks. Our paper seems to be the first report of magnetic resonance imaging of the brain in this condition. It did not disclose any abnormalities in the 2 cases in which it was performed. ( info) |
2/54. 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) |
| OBJECTIVES: patients with facial purpura can have acute and dramatic illnesses. For this reason, the clinician must be aware of certain diagnoses that can present as facial purpura and know how to initiate treatment efficiently. According to our review of the literature, no paradigm currently exists for the diagnosis and management of facial purpura Our goal is to develop a schema for the identification and management of facial purpura. STUDY DESIGN: Case series. methods: A case series of five cases is presented followed by a systematic development of a differential diagnosis based on etiology of the lesion. RESULTS: Facial purpura can be classified into conditions that are primarily or secondarily vasculitic, thrombocytopenic, neoplastic, infectious, toxic, and miscellaneous, which encompasses episodic reports that do not fit into the other categories. A paradigm to diagnose patients with facial purpura is developed based on clinical presentation and etiology. To demonstrate the utility of our algorithm, the five cases presented are re-examined using the algorithm. To our knowledge, this represents the first reported series of facial purpura in the modern English literature. A flow chart based on our diagnostic paradigm is presented. CONCLUSION: Clinicians caring for patients with facial purpura must recognize the acute conditions that require emergent medical intervention. Once these diagnoses have been considered, the clinician can employ a systematic approach to the diagnosis based on etiology. This study provides a useful reference for the otolaryngologist when encountering a patient with facial purpura. ( info) |
4/54. Otolaryngological aspects of relapsing polychondritis: course and outcome. Relapsing Polychondritis (RP) is a rare disorder characterised by episodic inflammation of the cartilaginous structures. Differential diagnosis of this pathology is a challenging enigma since it appears only intermittently and none of the clinical features are pathognomonic, although most of the patients are usually referred to an ENT physician initially. The natural history of the disease is unpredictable. Initiating effective treatment sooner considerably reduces the rate of mortality. patients with RP should be seen on a regular basis even if the patient is non-symptomatic. This study reports a retrospective analysis of the otolaryngological manifestations of 7 patients with RP as well as their long-term progress and reviews the symptomatology, histopathology, immunology and management. ( info) |
| mucormycosis often develops in immunocompromised patients, particularly in patients with diabetic ketoacidosis. Unless early diagnosis and treatment is established mucormycosis leads rapidly to death. A 38-year-old woman was admitted to the hospital with a severe diabetic ketoacidosis. Her clinical status improved in 4 days as a result of aggressive medical treatment. She has complained left cheek pain on the 10th day and had a swelling of her left cheek, facial edema, a black eschar on the palate and nasal cavity in association with visual disturbance and total ophthalmology in a short time. CT scan revealed left orbital cellulitis and pansinusitis. Excessive surgical treatment was performed and liposomal amphotericin-B, 4 mg/(kg day) was applied. Extensive fungal invasion of the orbit and the sinuses was demonstrated in the pathological species and rhizomucor species were yielded with culture. Repeated superficial debridement was also performed. After 10 weeks, she was discharged with suggestion of insulin treatment and liposomal amphotericin-B with progressively decreasing doses. At the 13th month following the presentation, the patient was free of disease as confirmed by serial imaging and under good glycaemic control with insulin treatment. Although mucormycosis is a fatal infection, early diagnosis and aggressive treatment may decrease mortality. ( info) |
6/54. Otolaryngological complications of osteopetrosis. osteopetrosis is a rare inherited bone disease that affects both humans and various mammals. The authors report on two cases of osteopetrosis with otolaryngological complications. One patient had the childhood form and presented with chronic otitis media and brain abscess. The second patient had the adult form and presented with sinusitis from tooth extraction which developed into chronic osteomyelitis of the maxillary bone. ( info) |
7/54. sarcoidosis of the upper respiratory tract. Selected cases emphasizing diagnostic and therapeutic difficulties. BACKGROUND: sarcoidosis of the upper respiratory tract (SURT) is a frequently overlooked and inadequately treated complication of sarcoidosis. AIM: The aim of this descriptive study is to present selected cases of sarcoidosis of the upper respiratory tract depicting diagnostic and therapeutic challenges. methods AND RESULTS: Five selected patients who either had an unusual initial presentation or an unusual clinical course were included if they also had long follow up. The diagnosis of SURT was based on the conventional criteria including histological evidence and appropriate microbiological studies. CONCLUSION: These case studies underline problems that may arise when SURT is not recognized early and appropriate and adequate treatment is delayed. ( info) |
| cysticercosis is not likely to be the first diagnosis the otologist has in mind when regarding tumors in the head and neck area. The fact that this disease may present a diagnostic and therapeutic problem is illustrated by three cases, appearing in the tongue, buccal mucosa, and midline of the neck, respectively. The local finding of a soft nontender mass, the typical calcifications in the soft tissues visible on roentgenograms, and the histopathological findings are described and illustrated. attention is drawn to the fact that cerebral cysticercosis, which is a very grave disease, may cause tinnitus, nystagmus, and hearing disturbances. ( info) |
9/54. Bronchogenic cysts of the neck in adults. Bronchogenic cysts are congenital sacs that result from maldevelopment of the primitive foregut. Although they occur predominantly in the chest, there are reports of lesions in extrathoracic locations. The majority of reported bronchogenic cysts located in the neck are found in the pediatric population; a review of the literature reveals few reports of bronchogenic cysts of the neck among adults. The diagnosis of a bronchogenic cyst relies on the histology and location of the lesion. Here, we review our experience in the diagnosis and management of 2 adult patients with pathologically proven bronchogenic cysts. Both patients presented with solitary neck masses that proved to be bronchogenic cysts on histologic examination. Our purpose is to define the histopathologic and clinical characteristics of bronchogenic cysts and discuss the features that distinguish them from other cervical cysts. In conclusion, congenital bronchogenic cysts can occur in the neck of adults and should be considered in the differential diagnosis of cystic cervical masses in adults, as well as children. ( info) |
10/54. Otolaryngologic presentations of amyotrophic lateralsclerosis. OBJECTIVES/HYPOTHESIS: To determine the incidence of voice disturbance as a presenting symptom of amyotrophic lateral sclerosis (ALS) and describe laryngologic features of ALS. STUDY DESIGN: Retrospective review. methods: Records of patients with voice disturbance at a voice center and ALS patients at a neurology clinic were reviewed from January 1998 to March 2003. RESULTS: 15 of 1759 patients with voice disturbance were later diagnosed with ALS. Of 220 ALS patients presenting to neurology clinic, 44 had bulbar symptoms and 19 had initially presented to an otolaryngologist. dysarthria, dysphagia, tongue fasciculation, and incomplete vocal fold closure were common findings. Neuromuscular disease was missed in 8 of 19 ALS patients seen by an otolaryngologist. CONCLUSIONS: Although otolaryngologists rarely encounter undiagnosed ALS patients, a significant portion of bulbar ALS patients are initially evaluated by otolaryngologists. SIGNIFICANCE: Vigilance for neuromuscular abnormalities on otolaryngologic exam is important in patients who present with dysarthria, dysphonia, or dysphagia. EBM rating: C. ( info) |