Endoscopic management of a rare case of nasal glioma in Meckel's cave in an adult: case report.

BACKGROUND: Nasal glioma or glial heterotopia is a rare embryologic anomaly that heralds its presence shortly after birth or in childhood. Nasal glioma in an adult is very rare, often asymptomatic and the occurrence of nasal glioma in Meckel's cave in an adult has not been previously reported. CASE REPORT: The authors encountered a case of an incidentally diagnosed Meckel's cave nasal glioma in a 40-year-old male which was successfully excised by an endonasal endoscopic transmaxillary transpterygoid approach. CONCLUSION: The occurrence of a nasal glioma in Meckel's cave an adult is very rare. Considering the deep skull base location, endonasal endoscopic surgery provides a minimal access technique to reach this location with excellent results.

Three-dimensional neurostereoendoscopy: subjective and objective comparison to 2D.

Neuroendoscopic procedures, particularly transnasal skull-base procedures, are currently performed with 2D endoscopes that lack stereoscopic vision and depth of field. In principal, 3D vision should be preferable to the operating surgeon, but the previously existing systems have not been adopted. We evaluated a novel 3D endoscope to compare with 2D endoscopy. 33 neurosurgeons and skull-base otolaryngologists were recruited, and randomized to complete two runs of a task-based simulator paradigm using 2D and/or 3D visualization. After the two trials, each subject completed a questionnaire assessing professional demographics and preferences for visualization. The task paradigm had objective endpoints that measured speed, efficiency, and error rates. 75% of respondents preferred 3D endoscopy, and 87.5% determined that 3D visualization either somewhat or greatly helped with the assigned tasks. In the second run, subjects using 3D demonstrated a significantly higher efficiency than subjects using 2D (p=0.04). Subjects' speed and efficiency improved significantly when moving from 2D to 3D, and speed and efficiency improved significantly from Run 1 to Run 2 for 3D visualization. Subjective and objective outcomes support the utility of 3D visualization for neuroendoscopic techniques. Visualization that provides real-time, high-resolution binocular depth perception has a role in endoscopic skull base surgery and other neuroendoscopic procedures.

Combined simultaneous endoscopic transsphenoidal and endoscopic transventricular resection of a giant pituitary macroadenoma.

Intrasellar and sellar-suprasellar adenomas are generally removed through a transsphenoidal approach. Giant adenomas with significant suprasellar extension often require a craniotomy or combined "above and below" approach. The use of endoscopes has increased the visualization capacity of the transsphenoidal route and made these surgeries less invasive. In this report, we describe a novel combination of the endoscopic transsphenoidal approach with the endoscopic transventricular approach to remove a giant pituitary macroadenoma extending into the third and lateral ventricles. The tumor was initially removed via an endoscopic transnasal transsphenoidal, transtuberculum, transplanum approach. A second endoscope was then advanced into the lateral ventricle through a pre-coronal burr hole to assist in mobilizing the tumor and assure a complete resection. Multilayer closure and a ventriculo-peritoneal shunt were performed to insure a watertight seal of the skull base. Giant pituitary adenomas have traditionally been removed with staged or combined transsphenoidal and transcranial approaches. We describe the successful implementation of a minimal access endoscopic combined extended transsphenoidal and transventricular approach that avoids craniotomy and brain retraction.

Disseminated infection with Cryptococcus neoformans var neoformans in an 8 years immunocompetent girl.

Disseminated cryptococcosis is a rare and often fatal disease in children. The majority of cases usually occur in individuals with defective cell-mediated immunity, most commonly due to HIV infection. The authors here in report an 8-year-old girl from Nepal who presented with fever, cough, headache, lymphadenopathy, hepatosplenomegaly and cutaneous lesions. Lymph node biopsy revealed multiple granulomas composed of histiocytes and epitheliold cells along with numerous yeast forms of cryptococcus. Cultures of CSF, sputum and urine yielded cryptococcus neoformans. Surprisingly,the immune function in terms of T-cell number, CD4 : CD8 ratio, serum immunoglobulins and HIV serology was normal. After the diagnosis of disseminated cryptococcosis was established, the patient was treated with 5-fluorocytosine (100 mg/kg/day) for initial two weeks and amphotericin B (1 mg/kg/day) for 13 weeks. Patient responded well to the treatment with disappearance of presenting symptoms, cutaneous lesions, and lymphadenopathy, though she still had hepatosplenomegaly, which also decreased. Unfortunately, she developed loss of vision in 10th week of therapy. The patient was discharged on oral fluconazole (6 mg/kg/day) and no recurrence was found during the follow-up period of more than 9 months. This is the first case of disseminated cryptococcosis with no detectable immune deficit, from India.

Serum prolactin in celiac disease.

Serum prolactin levels (SPL) were estimated in patients with celiac disease (CD), diagnosed as per ESPGAN criteria, on unrestricted gluten containing diet (group 1), as well as those consuming a gluten-free diet (GFD) (group 2). Forty-one children with CD, with 20 cases in group 1 (mean age 5.67 +/- 2.14, range 2.5-10.5 years) and 21 cases in group 2 (mean duration of follow-up 2 years, range 1-4 years), and 41 age- and sex-matched controls were studied. Hyperprolactinemia was defined as serum prolactin > 18 ng/ml in males and > 24 ng/ml in females. Upper gastrointestinal endoscopic biopsy was performed in both study groups for initial and follow-up evaluation. Hyperprolactinemia was detected in all the patients of group 1 and one patient of group 2 who had severe villous atrophy. The SPL in group 1 (mean 48.3 +/- 17.4; range 20-90 ng/ml) and group 2 (mean 18.3 +/- 6.9, range 10-39 ng/ml) was significantly higher compared with the controls (mean 9.3 +/- 4.5; range 2.4-20 ng/ml; p < 0.001). Among the patients with CD, mean SPL in group 1 was significantly higher than in group 2 (p < 0.001). In group 1, there was a positive correlation between SPL and duration of symptoms (p = 0.006, r = 0.768) and age of diagnosis (p < 0.001, r = 0.842). A positive correlation also existed in group 2 between SPL and degree of villous atrophy (p < 0.001, r = 0.71) and lamina propria infiltrate (p < 0.001, r = 0.568). Our results suggest that SPL has a significant correlation with activity of CD. Therefore serum prolactin estimation may provide an additional marker of disease activity in CD and may be a more viable option economically.

Progressive bulbur paralysis (Fazio-Londe disease).

Progressive bulbar paralysis of childhood is characterised by progressive paralysis of muscles innervated by cranial nerves. The authors report a case of progressive bulbar paralysis of childhood in a 12-year-old child. Child was admitted with the complaints of drooping of eyelids, difficulty in swallowing and hoarse voice. She had involvement of III, VII, IX, X, XI and XII cranial nerves and the corticospinal tracts. Electromyography revealed spontaneous activity in the form of fasciculations, giant motor unit potential and discrete recruitment of motor neurons suggestive of denervation pattern. Hearing assessment was normal. Muscle biopsy was also suggestive of neurogenic atrophy.

Iron supplementation in children with celiac disease.

OBJECTIVE: To evaluate the effect of iron supplementation, in addition to gluten free diet (GFD), on hematological profile of children with Celiac Disease (CD). METHODS: Children diagnosed as CD as per modified ESPGAN criteria were prospectively evaluated for their hematological profile at the time of their enrolment and after consuming GFD for at least one year. The results were compared with age and sex matched controls. Evaluation of hematological profile included hemoglobin estimation, complete blood counts, peripheral blood smear examination, serum iron, total iron binding capacity (TIBC), and serum ferritin estimation. All the enrolled cases were given iron supplementation in addition to exclusion of gluten from their diet. Repeat intestinal biopsy was performed in all the cases after completing 1 year on GFD. RESULTS: Twenty one children (mean age 6.67 years, range 4-11 years) diagnosed as CD who completed at least one year of regular follow up on GFD (mean 1.5 years, range 1-2 years) were analysed for their hematological profile at the time of enrolment and after consuming GFD and iron supplementation. At the time of enrolment all the children had hemoglobin level <11 gm%, 78% had microcytic hypochromic anemia and 22% had dimorphic anemia, with lower mean MCV, MCH and serum ferritin levels, and a significantly higher mean TIBC as compared to controls (p<0.001). In the follow up evaluation of these cases on GFD, mean hemoglobin levels were comparable with controls but the cases continued to have lower mean MCV, MCH serum ferritin levels (p<0.05) and higher mean TIBC (p<0.05). Seven children had mild anemia. Serum ferritin levels showed a negative correlation with the grade of villous atrophy and lamina propria infiltrate. CONCLUSION: Our results suggest that iron deficiency anemia (IDA) is commonly associated with CD and iron deficiency state continues for a longer time even after excluding gluten from the diet and iron supplementation. Apart from offering them GFD rich in iron, early detection and treatment of IDA and prophylactic iron folic acid supplementation will go a long way to optimize their mental and psychomotor functions.

Diagnostic modalities for gastroesophageal reflux.

OBJECTIVE: To evaluate commonly utilized diagnostic modalities to detecting Gastroesophageal Reflux (GER). METHODS: Sixty children aged 1-72 months (mean age 14.7 months) with symptoms suggestive of Gastroesosphageal Reflux (GER) were investigated and subjected to upper gastrointestinal endoscopy and esophageal biopsy (EB), gastroesophageal scintiscanning (GS) and 24 hour ambulatory pH monitoring. RESULT: GER was detected in 28 (46.7%) cases by one or more diagnostic modalities. Ambulatory 24 hour pH monitoring was positive in higher proportion (43.3%) of cases in comparison to other modalities, followed by EB (38.3%) and GS (30%). Considering 24 hour pH monitoring as the gold standard, esophageal biopsy was positive in 22/26 cases (84.6%) detected by 24 hour pH monitoring with a specificity of 97.1% as compared to 17/26 cases (65.4%) by gastroesophageal scintiscanning with a specificity of 97.1%. When compared with EB results, amongst various parameters measured during 24 hour pH monitoring, Reflux index (RI) ranked highest (sensitivity 95.6 % and specificity 89.2 %) followed by duration of longest episode > 20 minutes and Euler Byrne score. Oscillatory index, calculated from tracings of pH monitoring, even though ranked lower because of its low sensitivity helped to pick up 2 cases missed by EB and RI. CONCLUSION: Our results suggest that a combination of diagnostic modalities may be required to diagnose GER in young children. Ambulatory 24 hour pH monitoring appears to be the single best investigation and combining it with EB and/or GS can help to detect maximum number of cases.

Association of gastroesophageal reflux disease in young children with persistent respiratory symptoms.

Forty children aged between 3 months and 3 years (median age 14 months) with persistent respiratory symptoms beyond 4 weeks or recurrence of respiratory symptoms were investigated for gastroesophageal reflux (GER). Diagnostic tests included upper gastrointestinal endoscopy, oesophageal biopsy, gastroesophageal scintiscan and 24 h ambulatory oesophageal pH monitoring. GER was detected in 14 (35 per cent) of these patients; which included 38 per cent of the enrolled cases of recurrent bronchopneumonia, 40 per cent cases of reactive airway disease, and 22 per cent cases of persistent cough. Amongst the cases detected to have GER, the age of onset of respiratory symptoms was less than 1 year in 86 per cent of cases (p < 0.01), nocturnal symptoms of cough and wheeze were reported in 78 per cent (p < 0.05), and 86 per cent cases did not present with typical gastrointestinal symptoms (p < 0.01). Family history of asthma was absent in all cases of GER-related reactive airway disease (p < 0.01). Cases detected to have GER were followed for 3-6 months after starting anti-reflux therapy. A significant (p < 0.01) decrease was noticed in the number of further episodes in children with GER-related recurrent bronchopneumonia and reactive airway disease after starting anti-reflux therapy. Improvement was also noticed in nocturnal symptoms and nutritional status after anti-reflux therapy was started. Our results suggest that GER may be one of the possible contributing factors in any child with recurrent and persistent respiratory complaints. Early diagnosis and anti-reflux therapy in cases with GER-related respiratory complaints can result in significant improvement in symptoms.

Sinusitis complicated by meningitis: current management.

OBJECTIVES: Meningitis is the most common intracranial complication of sinusitis. We review the incidence, current management, outcomes, and complications of this serious infection. Our study also examines the evolving roles of endoscopic sinus surgery and other new therapeutic and diagnostic modalities in our armamentarium. STUDY DESIGN: A retrospective chart review was performed at a tertiary academic medical center of all patients diagnosed with sinusitis with complications between January 1985 and December 1999. METHODS: The patients were divided into two main groups: intracranial versus orbital complications. Meningitis was the most common intracranial complication. Data on patients with sinusitis and meningitis were collected and analyzed. RESULTS: Intracranial complications were present in 39 of 82 patients whereas orbital complications were noted in 43 patients. Twenty-one of the 39 intracranial complications were meningitis. The most common computed tomography finding in adults (54%) was sphenoid sinusitis. All patients with AIDS (6 of 21) had unique cryptococcus meningitis. In patients without AIDS, the most common organism was Streptococcus pneumoniae (10 of 21). The most common sequela was seizure disorder (4 of 21). Endoscopic sinus surgery was performed on 7 of 21 patients. One patient with AIDS who had sinusitis and meningitis died. CONCLUSIONS: Meningitis as a complication of sinusitis may still pose a serious threat. Although outcomes are encouraging, sequelae such as seizure disorders and hearing loss are common complications. The introduction of high-resolution computed tomography scans and magnetic resonance imaging and the availability of wide-spectrum antibiotics have improved our management significantly.

Brush cytology: an adjunct to diagnostic upper GI endoscopy.

Endoscopic brush cytology (EBC) was performed in antral and duodenal brushings of children subjected to upper GI endoscopy for the detection of H. pylori (Hp) and trophozoites of Giardia lamblia (Glt) in addition to routine endoscopic grasp biopsy (EGB). It was hospital based prospective study. EBC was performed in children subjected to upper GI endoscopy with a sheathed cytology brush. Mucosal brushings were collected from antrum, body of the stomach and second or third part of duodenum by gently rubbing the surface of the brush with the mucosal wall in all the directions, brush withdrawn and brushings performed on a glass slide. The smears were placed in 95% ethyl alcohol and later examined for Glt and Hp using Giemsa and Hematoxylin & Eosin stain. EGB was taken from antrum, body of the stomach and duodenum from sites other than those used for brushings. One hundred and seventy children between 1-13 years (median age = 5 years) were subjected to upper GI endoscopy for malabsorption (n = 94), recurrent abdominal pain (n = 49), failure to thrive (n = 16) and recurrent vomiting/regurgitation (n = 11) and EBC was performed in addition to routine EGB. Thirty five children (20.4%) were colonized by Hp, 14 (8.2%) were detected to have Glt and in 6 cases (3.5%) both Hp as well as Glt were detected. Out of 41 cases colonized by Hp, 24 cases (58.5%) were detected by EGB and 27 cases (65.8%) were detected by EBC. Out of 20 children in whom Glt were detected from their duodenum, the detection was by EBG in 12 cases (60%) and by EBC in as many as 19 cases (95%). Comparison of EGB and EBC suggested that detection rates with EBC were higher than EGB. Detection by EBC was significantly higher for Glt than Hp. There were no complications attributed to EBC and procedure time for endoscopy was not significantly prolonged. On the contrary, detection of Hp and particularly Glt in higher proportion of cases with the help of EBC was helpful in their appropriate management. Our results suggest that EBC is a safe and useful tool to enhance the value of diagnostic endoscopic procedure when used in combination with routine EGB.

Evaluation of the utility and cost-effectiveness of obtaining histopathologic diagnosis on all routine tonsillectomy specimens.

OBJECTIVES: To evaluate the use and necessity of obtaining histopathology on patients undergoing tonsillectomy, and to provide indications and guidelines for requesting histopathology on tonsillectomy patients. STUDY DESIGN: At the University of Mississippi Medical Center, we have been routinely obtaining histopathologic diagnoses on every patient undergoing tonsillectomy or tonsillectomy and adenoidectomy. Specimens are separated into left and right tonsils and adenoids. This study was designed at our tertiary care academic center to evaluate the necessity for obtaining histopathologic diagnosis on each of these patients. METHODS: A retrospective review of histopathologic reports on all patients in both pediatric and adult age groups undergoing tonsillectomy or tonsillectomy and adenoidectomy between January 1994 and December 1999 was performed. RESULTS: A total of 2438 reports were reviewed. There were 2099 in the pediatric age group and 339 were adults. None of the children had an unusual histopathology finding other than lymphoid hyperplasia. Of the 339 adults, 34 had squamous cell carcinoma and 6 had lymphoma; however, these findings were suspected preoperatively by history and clinical manifestations. CONCLUSIONS: The general practice guidelines mandate obtaining histopathologic diagnoses on most of the tissues received. Based on our review, histopathology of tonsillectomy and/or adenoidectomy may not be necessary, especially in children. In this era of cutting excess costs of health care dollars, waving histopathology in these cases may have major implications without compromising delivery of quality care.

Tracheobronchial extension of recurrent respiratory papillomatosis.

Endobronchial and pulmonary dissemination reportedly occurs in 5% of patients with laryngeal papillomatosis. It is more frequently observed in the juvenile form, and carries significant implications for treatment. In this study, we review our experiences with pulmonary and endobronchial extension of laryngeal papillomatosis. The records of the 52 patients with laryngeal papillomas treated at our institution since 1980 were reviewed. Twenty-nine percent of the patients (N = 15) developed tracheobronchial extension; 7% (N = 4) demonstrated pulmonary involvement. The birth history, race, sex, age at onset of symptoms, presenting symptoms, site and type of papillomas, surgical procedures, other methods of treatment, complications, and mortality data were analyzed. Eighty percent of the patients with tracheobronchial involvement were born to mothers with a positive history of vaginal condylomas. Pulmonary changes in 4 patients were observed on either chest radiographs or computed tomography and were manifested as multiple parenchymal nodules in 3 of the 4 patients. Pneumatoceles, cavitary empyema, and multiple recurrent pneumonias were the predominant complications of pulmonary involvement. Of the 15 patients with tracheobronchial extension, 80% (N = 12) required tracheotomy before their presentation to us. The number of multiple surgical procedures performed to remove papillomas ranged from 7 procedures in 1 patient with a solitary tracheobronchial lesion to more than 100 procedures in 2 patients with diffuse bronchopulmonary papillomatosis. As our study shows, tracheobronchial involvement continues to complicate surgical treatment in this challenging disease process.

Goldenhar syndrome with rare associations.

Goldenhar syndrome is a malformation complex involving the structures arising from first and second branchial arches, the first pharyngeal pouch, first branchial cleft and primordia of the temporal bone. Though the syndrome itself is not very rare, the presence of polydactyly and hydrocephalus, which are rare associations, prompted us to report this case.

Value of radiologic imaging and computer assisted surgery in surgical decisions of the anterior skull base lesions.

The role of radiologic imaging in surgical decision making of anterior skull base lesions has been found to be critical in the endoscopic surgical management of these lesions. The non-invasive radiologic imaging may include the use of CT scans, MRI scans, MRA scans and their subtraction technique. The imaging offers an understanding of the vascularity of the lesion, the relationship to the nearby neurovascular structures and the type of tissue density of these lesions. The addition of image guided applications offers one a sagittal reconstruction and a 3-D imaging capacity which has immensely improved the accuracy and precision in endoscopic surgical applications in these areas.

Yolk sac tumor of the temporal bone: report of a case.

Yolk sac tumor (endodermal sinus tumor) is rarely encountered in the temporal bone. Facial nerve paralysis can be a primary manifestation of this condition. Histologically, the tumor can be difficult to diagnose, although elevated levels of alpha fetoprotein can facilitate its identification. In this report, we describe the case of an 18-month-old girl who developed peripheral VIIth nerve palsy and a polypoid mass in the left external ear canal 3 months following myringotomy. Computed tomography and magnetic resonance imaging revealed that the tumor involved the left external ear canal, middle ear space, and mastoid air cells. Biopsies were consistent with a yolk sac tumor. Special staining demonstrated that only a very few tumor cells were positive for alpha fetoprotein, despite the markedly elevated level of alpha fetoprotein in her serum. The patient was treated with chemotherapy, which included cisplatin, etoposide, and bleomycin. Within a period of weeks, she experienced a complete reversal of her left VIIth nerve palsy, a marked decrease in her serum alpha fetoprotein levels, and a dramatic resolution of the tumor as demonstrated radiographically. Such a successful chemotherapeutic response in this case argues against surgical intervention in other cases, particularly in view of the risk of serious complications with surgery.

Magnetic resonance navigation for head and neck lesions.

OBJECTIVE: Review applications of interventional magnetic resonance imaging and describe methods, procedures, and additional instrumentation for the magnetic resonance "operating theater." Describe advantages of magnetic resonance navigation for biopsies of head and neck tumors. STUDY DESIGN: Patients with palpable and nonpalpable head and neck and cranial base tumors were recruited into the study. Patients underwent magnetic resonance-guided biopsy. Retrospective analysis of 21 patients was conducted. METHODS: 0.5 Tesla superconducting open magnetic resonance imaging was used for navigation of the biopsy needle. Patient records and magnetic resonance images were reviewed. The type, size, and location of the lesions were tabulated. Type of anesthesia and monitoring method were analyzed. The histopathologic correlation was conducted in patients who required further surgeries or open surgical biopsies. RESULTS: Twenty-two biopsies were carried out in the magnetic resonance suite. One patient required general anesthesia and the other biopsies were conducted under intravenous sedation. There was only one case of nonconcurrence in a patient with Wegener's granulomatosis of the posterior orbit. Overall, a 92% concurrence rate between image-directed fine-needle aspiration, open biopsy, and surgical therapy was encountered. No complications occurred. CONCLUSIONS: The use of interventional magnetic resonance imaging to assist with fine-needle aspiration core biopsy has made the biopsy procedure safer and more accurate. Potentially morbid and disfiguring surgeries have been avoided in some patients. Deeper lesions have been more easily approached, as the needle for biopsy is under constant magnetic resonance guidance. Improved visualization for critical structures allows safer performance of biopsies. The primary difficulties of open magnetic resonance imaging relate to the need for nonferromagnetic instrumentation and equipment and their high costs. An inverse relationship exists between the imaging quality and the "dead time" required to acquire images.