Tangier disease in a Turkish family.

In this report we describe the upper gastrointestinal tractus involvement in a rare genetic disease of lipid metabolism. A 12-year-old boy presented with sore throat and fever. On physical examination, orange-yellow tonsils and adenoid tissue were noted. Mild hepatosplenomegaly was present. Lipid profile was compatible with Tangier disease (TD). Endoscopy of the upper gastrointestinal tract showed white-yellowish fatty deposits on the gastric mucosa. Microscopically, biopsy specimens contained numerous histiocytes with a foamy cytoplasm packed in the lamina propria of the gastric mucosa and at the crypt basement of the duodenum. His sister, 8 years old, was also diagnosed with TD based on abnormal lipid profile and orange-yellow tonsils. TD is a rare familial disorder of lipid metabolism, characterized by deposition of cholesteryl esters, probably involving the entirety of the gastrointestinal tract from the mouth to the anus.

A rapidly growing neck mass in childhood: Castleman's disease concomitant with cavernous lymphangioma in the posterior cervical region.

In this article, we present a 12-year-old girl case with a painless mass in the left posterior region of the neck for two weeks. Two masses were detected during surgery. Histopathologic examination revealed the hyaline vascular type of Castleman's disease for the bigger mass and cavernous lymphangioma for the smaller mass. In the light of literature data, our case was the third childhood cervical posterior triangle Castleman's disease and was the first case reported due to its concomitance with cavernous lymphangioma.

Uncinate process in the repair of nasoseptal perforation.

Septal perforation is an anatomical defect of the nasal septum. Prior septal surgery is the most common reason of this defect. Because repair of a nasoseptal perforation is a challenging surgical procedure, many techniques have been discussed for decades. This article considers the first case of nasoseptal perforation repair using the overmedialized uncinate process. The main aim of this study is to report on the usability and efficiency of regional autogenous grafts in the repair of nasoseptal perforations.

[Sphingomonas paucimobilis infection with underlying submandibular sialolithiasis]. / Submandibüler sialolitiazis zemininde Sphingomonas paucimobilis enfeksiyonu.

Sphingomonas paucimobilis (S. paucimobilis), is a gram-negative, aerobic, non-fermentative, oxidase (+) and catalase (+) bacterium. Although S. paucimobilis is isolated very rarely, it can cause both nosocomial and community-acquired infections. A patient admitted to our clinic had a complaint of swelling in the right mandibular region and pain increasing while eating for the previous week. Bimanual palpation revealed a painful swelling of 1x1x1 cm in size inside the right Wharton's duct. Via massage over the right submandibular gland, a purulent drainage came up from the opening of the Wharton's duct and it was cultivated and S. paucimobilis was isolated. There was no ultrasonographic evidence of calculi in the duct or in the gland, but during the right submandibular gland massage, two stones came out through the Wharton's duct. The infection was observed to be eradicated with a 14-day ampicillin-sulbactam treatment. In the control examination after six months, there were no pathological signs or symptoms and the ultrasonogram was normal. As far as we know, this is the first sialadenitis case with underlying sialolithiasis where S. paucimobilis was isolated.

Magnesium sulphate infusion is not effective on discomfort or emergence phenomenon in paediatric adenoidectomy/tonsillectomy.

OBJECTIVE: This study was performed to evaluate the possible influence of magnesium sulphate which has sedative and analgesic properties on sevoflurane-induced emergence agitation in paediatric patients undergoing adenoidectomy with or without tonsillectomy. METHODS: One hundred and ten paediatric patients aged between 3 and 16 years were randomly allocated to the study. Propofol 2-2.5 mg kg(-1), vecuronium 0.1 mg kg(-1) and fentanyl 1 µg kg(-1) were used for induction of anesthesia and sevoflurane at 1 MAC with nitrous oxide in oxygen (35%) mixture was administered as maintenance. Magnesium sulfate 30 mg kg(-1) in saline (20 mL) in the Group M, or equal volume of saline for controls (Group C) was started 10 min before and infused until the end of the operation. Recovery characteristics included time to extubation, eyes open, emergence and interaction. Patients were evaluated using Modified Aldrete Score (MAS), Pain/Discomfort Scale and Agitation Score. Side effects were determined during emergence and in the recovery. RESULTS: Time to open eyes was significantly higher in the magnesium treatment group (GroupC: 7.7±3.5, Group M: 12.7±17.5 min, p=0.001). Agitation score was significantly lower in Group M at the 60th min (Group C: 1.3±0.7, Group M: 1.0±0.3, p=0.005). Agitation or pain/discomfort scores in the resting observation periods were the same. The initial MAS value was lower in Group M (Group C: 5.0±1.9, Group M: 4.0±1.7, p=0.003). There was no significant difference between groups regarding side effects. CONCLUSION: Magnesium sulphate infusion has no influence on sevoflurane-induced discomfort or emergence agitation.

Effects of septoplasty on pulmonary function tests in patients with nasal septal deviation.

OBJECTIVE: To investigate the effects of septoplasty on pulmonary function tests (PFTs) and bronchial hyperresponsiveness (BHR) in patients who had no previous pulmonary diseases. DESIGN: Prospective, controlled clinical trial. PATIENTS: Fourteen patients with nasal septal deviation (NSD) and 20 controls were enrolled in the study. MAIN OUTCOME MEASURES: All patients were evaluated with anterior rhinoscopy, nasal endoscopy, acoustic rhinometry (AR), visual analogue scale (VAS), PFTs, and bronchoprovocation test (BPT) on the day before surgery and 8 to 12 weeks after surgery. RESULTS: The postoperative values of forced expiratory volume in 1 second, forced vital capacity, and peak expiratory flow percentages were higher than preoperative ones, and these results were statistically significant (p = .007, p = .04, and p = .007, respectively). Nine patients had preoperative BHR, and three of them had negative BPT postoperatively. When compared with the preoperative values, there was a borderline decrease in the rate of BHR in six patients. There were significant improvements in the symptoms of left and right nasal congestion, headache, postnasal drip, and olfactory function after surgery. We found important improvement in the values of AR at right minimal cross-sectional area 1 (p = .02), left distance 1 (p = .04), and left distance 2 (p = .04) postoperatively. CONCLUSION: An improvement in both nasal symptoms and PFT values was seen after surgical treatment for NSD. We concluded that septoplasty may be considered as having favourable effects on BHR.

Simultaneous myringoplasty and septoplasty, and the use of nasal septal perichondrium.

To investigate the more ideal graft for optimal repair of tympanic membrane perforation, we examined the use of septal perichondrium in myringoplasty. Twenty-five patients with ages ranging between 18 and 54 were included in this study. All had a persistent, symptomatic tympanic membrane perforation, and nasal obstruction. All patients were scheduled for myringoplasty by a transmeatal approach in combination with septoplasty under general anaesthesia. Twenty-three patients had intact graft material and had more than 5 dB HL improvement in conductive hearing thresholds at least three frequencies in the follow up period. An overall success rate of 92 and 92% was recorded in terms of hearing improvement and perforation closure, respectively. Nasal septal perichondrium is easily accessible, cost-effective, time saving, sufficiently large, and patient friendly as a graft material in myringoplasty and has a good chance of postoperative survival.

Acute actinic cheilitis-like chemical irritant reaction following accidental contact with ethylene glycol-favorable response to topical 1% pimecrolimus cream: a case report.

Ethylene glycol is a colorless, odorless, nonvolatile, water-soluble fluid, mainly used as automobile antifreeze and coolant. This substance is a frequent culprit in accidental and intentional poisonings. Although potentially fatal systemic consequences of ethylene glycol ingestion are well known, local adverse effects through brief skin and mucosa contact with ethylene glycol have been rarely reported. Herein we report a patient with accidental ethylene glycol contact to the lower lip, who presented with acute, manifest, actinic cheilitis-like chemical irritant reaction and favorably responded to topical pimecrolimus 1% cream.

[The effect of suturing technique and nasal packing on oxidative stress in septoplasty operations]. / Septoplasti ameliyatlarinda dikis teknigi ve nazal tampon uygulamasinin oksidatif stres üzerine etkisi.

OBJECTIVES: We investigated the effect of nasal packing or suturing of the nasal septum after septoplasty on oxidative stress. PATIENTS AND METHODS: The study included 37 patients who underwent septoplasty for isolated septal deviation. At the end of the operation, intranasal packing was used in 18 patients (5 women, 13 men; mean age 30+/-10 years; range 18 to 47 years), and nasal septal suturing was used in 19 patients (5 women, 14 men; mean age 31+/-12 years; range 18 to 54 years). Nasal packs were removed after 48 hours. Plasma levels of malondialdehyde (MDH), total sulphydryl content (SH), and nitric oxide (NO) were measured at four sequential times to evaluate oxidative stress. RESULTS: There were no significant changes in plasma levels of MDH, SH, and NO in both treatment groups (p>0.05). Levels of MDH and NO did not differ significantly at four sequential times between the two treatment groups, whereas postoperative SH levels were always significantly higher following septal suturing (p<0.05). CONCLUSION: High levels of SH observed in the suturing group favor this technique over nasal packing application in terms of antioxidant systems and oxidative stress.

High-dose ropivacaine versus bupivacaine for posttonsillectomy pain relief in adults.

OBJECTIVE: To compare the efficacy and safety of preincisional high-dose ropivacaine with bupivacaine in relieving posttonsillectomy pain. DESIGN: A prospective, randomized, double blind, placebo-controlled clinical trial. SETTING: University hospital. PATIENTS: Before the incision, 58 subjects were randomly assigned to receive 10 mL of 10 mg/mL ropivacaine hydrochloride with epinephrine (n=19, group 1), 10 mL of 2.5 mg/mL bupivacaine with epinephrine (n=20, group 2), or saline with epinephrine (n=19, group 3) as a placebo. MAIN OUTCOMES MEASURES: Postoperative pain, additional analgesic drug consumption, otalgia, operating time, amount of intraoperative blood loss, and possible complications were assessed. The intensity of pain was scored on a visual analogue scale. The patients were followed up for 10 days after surgery. RESULTS: There were no statistically significant differences between the demographic and operational characteristics of the patient groups. Statistically significant differences were determined between in the mean intensity of constant and swallowing pain values of group 1 and group 2 or 3, at 4, 8, and 12 hours, and on days 1, 2, 3, and 4, postoperatively. However, no statistical differences were determined between the groups on days 5, 7, and 10. A lower amount of additional analgesic drug was consumed by the ropivacaine group than by the bupivacaine group, and by the bupivacaine group than by the control group, and the differences were statistically significant. CONCLUSION: Preincisional infiltration of the tonsils with high-dose ropivacaine markedly decreased the intensity of pain after tonsillectomy when compared with bupivacaine or placebo, especially until postoperative day 4 in adults.

[The effect of nasal packing and suture technique on systemic oxygen saturation and patient comfort after septoplasty]. / Septoplasti sonrasi nazal tampon veya dikis tekniginin sistemik oksijen satürasyonu ve hasta konforu üzerine etkisi.

OBJECTIVES: We investigated the effect of intranasal packing placed after septoplasty or alternatively suturing the septum without inserting a packing on systemic oxygen saturation and patient comfort. PATIENTS AND METHODS: The study involved 37 patients who underwent septoplasty for isolated septum deviation. Intranasal packing was used in 18 patients (5 women, 13 men; mean age 30+/-10 years; range 18 to 47 years), while 19 patients (5 women, 14 men; mean age 31+/-12 years; range 18 to 54 years) underwent nasal septal suturing. To evaluate systemic oxygen saturations, partial oxygen saturations were measured pre- and postoperatively by pulse oximetry. The effect of intranasal packing and suturing on patient comfort was assessed by a visual analog scale before and 48 hours after surgery, five days after removal of nasal packing, and 30 days after surgery. RESULTS: Postoperative systemic oxygen saturations exhibited higher levels in the sutured group, but this did not reach significance (p>0.05). The incidence of nasal obstruction was significantly higher with nasal packing (p<0.05). In general, headache and facial pain were more common with nasal packing, while suturing was associated with a higher frequency of nasal crusting, postnasal discharge, and reduced sense of smell (p>0.05). CONCLUSION: Considering that, albeit not significantly, intranasal packing used after septoplasty decreases systemic oxygen saturation more than suturing and thus, has more adverse effects on patient comfort, suturing the septum may be a better alternative, particularly in elderly patients with chronic systemic diseases.

Effect of musical training on musical perception and hearing sensitivity: conventional and high-frequency audiometric comparison.

This prospective study was designed to investigate the role of musical training on musical perception and hearing acuity and to determine probable hearing loss. Thirty students, aged 17 to 23 years, were evaluated for hearing sensitivity within conventional and high-frequency audiometric ranges. The hearing thresholds of the controls were compared with those of the students. To evaluate the effect of musical training on musical perception, students were given an examination consisting of single-note, harmonic hearing; multiple sounds (chords with two, three, and four sounds), horizontal hearing; melody, and rhythm. Musical perception and the average hearing level of the students on admission to the faculty were compared with the data from students after a 2-year musical education program. The hearing sensitivity of the students at the initial and final evaluations was not similar. The average hearing acuity increased for the whole conventional audiometric range (p < .05). There was worsening for 12, 14, and 16 kHz for the high-frequency audiometric range (p < .05). The decrease in average hearing acuity at these frequencies was statistically significant, as indicated by Student's t-test (p < .05). Although the average musical hearing sensitivity increased for horizontal hearing (p < .05), it did not change for harmonic hearing (p > .05). Musical training might increase the spontaneous attention to the sound heard and the ability to discriminate. Hearing reduction at the high frequencies might be attributed to continuous noise exposure.

Preincisional infiltration of tonsils with ropivacaine in post-tonsillectomy pain relief: double-blind, randomized, placebo-controlled intraindividual study.

OBJECTIVE: To determine whether pre-emptive ropivacaine has an influence on postoperative pain in adult patients undergoing tonsillectomy. DESIGN: A prospective, randomized, double-blind, placebo-controlled clinical trial. SETTING: University hospital. PATIENTS: The study included 20 adult patients undergoing elective tonsillectomy. Anesthetic induction and maintenance, dissection tonsillectomy, hemostasis techniques, and postoperative analgesic treatment were standardized for all patients. Before the onset of incision, one tonsillar fossa was administered 5 mL of 2% ropivacaine hydrochloride with epinephrine, whereas the other side received 5 mL of 0.9% saline with epinephrine and was designated as the control side. MAIN OUTCOMES MEASURES: For each side, postoperative pain, otalgia, operating time, amount of intraoperative blood loss, and postoperative hemorrhage were assessed. The intensity of postoperative pain was measured at rest and when the patient was drinking and was scored on a visual analogue scale. The patients were followed up for 10 days after surgery. RESULTS: There was no statistically significant difference in the amount of intraoperative hemorrhage and operation time between sides (p > .05). The constant postoperative pain in the ropivacaine side at rest was significantly less than in the placebo side on days 1, 2, 5, and 6 (p < .05). The post-tonsillectomy pain experienced in the ropivacaine side when swallowing was significantly less than that in the placebo side throughout the study period except on day 10 (p < .05). CONCLUSION: Based on the present findings, preincisional infiltration of ropivacaine 2% appears to be effective against both early and late postoperative pain, especially on swallowing, following tonsillectomy in adults.

Anatomic variations of the sphenoid sinus on computed tomography.

Anatomic variations of the vital structures adjacent to the sphenoid sinus can be jeopardized during functional endoscopic sinus surgery (FESS). The knowledge of the size and extent of pneumatization of the sphenoid sinus (SS) is an important condition for adequate surgical treatment of its disease. The bony anatomic variations of SS as well as its relationship with adjacent vital structures were reviewed in this paper. The study was performed on 267 patients with a complaint of chronic or recurrent sinusitis. Computed tomographic (CT) scans were obtained upon completion of therapy. The evaluations of the sphenoid sinuses were regarded separately, so as 534 sides were examined. Especially bony anatomic variations as well as mucosal abnormalities of the sphenoid sinuses were examined. Pneumatization of the pterygoid process and anterior clinoid process were found in 39.7% and 17.2% of the patients respectively. Vidian canal protrusion was found in a total of 158 sides of which 60 were bilateral. These entities were encountered usually when pneumatization of the pterygoid process occurred. Carotid canal and optic canal protrusions were found in 5.2% and 4.1% of the patients respectively. Mucosal thickening, and polyps or cysts of sphenoid sinuses were detected in 20.6% and 4.5% of the patients respectively. There was a statistically significant correlation between pterygoid pneumatization and vidian canal protrusion (p < 0.001), and vs. foramen rotundum protusion (p = 0.004). While the optic canal protrusion was found significantly associated with the anterior clinoid pneumatization (p < 0.001), there was no statistically significant correlation between a carotid canal protrusion and anterior clinoid pneumatization (p = 0.250). Sphenoid sinus surgery is very risky, because of changing variations of the cavity. We are in the opinion that detailed data from CT scans of SS will enable the surgeon to interpret any anatomic variations and pathological conditions before initiation of the surgical therapy.

The analysis of anterior skull base from two different perspectives: coronal and reconstructed sagittal computed tomography.

The aim of the study was to determine the heights of the anterior skull base and the distances between the anterior nasal spine and the skull base at three levels by means of coronal and reformatted sagittal images of computed tomography. The present study was performed on coronal and reformatted sagittal CT scans of 30 patients with sinonasal complaints. On the coronal view, the heights of the cribriform plate, the roof of ethmoid, and lateral lamella and the medial take-off angle between the ethmoid roof and cribriform plate were measured at different levels. On the reformatted sagittal images the distances from the nasal spine to the anterior cranial base at three different levels were measured. Then, the side-to-side variability of these measurements was statistically compared. The variations with normal distribution and abnormal distribution were analysed by paired t test and Wilcoxon paired-signed rank test, respectively. A statistically significant difference was detected only between the right and left sides in the height of the lateral lamella at the crista galli level (p < 0.05). The lateral lamella at the crista galli level on the left side was higher than on the right side. No statistically significant differences between the left and the right sides were noted in the heights and the distances of other data (p > 0.05). The normal anatomy of the anterior skull base has been described in detail on coronal and reconstructed sagittal computed tomography. These measurements may be helpful in the presurgical evaluation of patients undergoing endoscopic sinus surgery to optimize surgical safety.

Microscopic anatomy of the infraorbital canal, nerve, and foramen.

OBJECTIVE: We performed this study to investigate the morphologic and topographic anatomy, and variations of the infraorbital canal (IOC), infraorbital nerve (ION), and infraorbital foramen (IOF). STUDY DESIGN AND SETTING: This study was performed at the Anatomy Department of Ankara University School of Medicine between 1999 and 2000. The morphometry of these anatomic areas on cadaver heads were studied microscopically. The morphologic and topograpic anatomies of IOC, ION, and IOF were carried out microscopically with x 25 to x 40 magnification on 10 cadaver heads (20 sides). RESULTS: We found the shape of the IOF as oval in 30% (6 sides), round in 40% (8 sides), and semilunar in 30% (6 sides) of the cadaver heads. We detected the IOF as single in 90% (18 sides), double in 5% (1 side), and triple in 5% (1 side) of the cases. We determined a complete roof (we called it as "canal") in 50% of cases and groove plus canal in 50% of the heads. The mediolateral relationship of the vessels to the nerve while emerging from the IOF was investigated, and the artery was always found in a mediosuperior position and the vein was inferior to it. In all specimens, the ION was found to be consisting of 3 to 8 fascicles interwoven with the artery and the vein in the loose connective tissue sheath. CONCLUSION: A detailed knowledge of the anatomic morphometry of this area is necessary for a surgeon while performing maxillofacial surgery and regional block anesthesia. Anatomic variations on this area may take place and a surgeon must take this into consideration so as to increase the surgical success.

Immunologic abnormalities and surgical experiences in recurrent facial nerve paralysis.

OBJECTIVE: To document immunologic findings in patients with recurrent facial paralysis (RFP) and to compare the results of the surgery with the results of medical treatment. STUDY DESIGN: Retrospective case review. SETTING: Tertiary care referral center. PATIENTS: Nine patients with RFP were reviewed. INTERVENTION: Patients underwent nonspecific antibody detection, protein electrophoresis (in blood and cerebrospinal fluid [CSF]) and oligoclonal band determination for immunoglobulin G, A, and M (in CSF). The extended subtotal facial nerve decompression via the transmastoid and transattic route was performed in four patients. Five patients received medical treatment only (steroids, vitamin B). RESULTS: Two patients had the complete and four patients had the oligosymptomatic form of Melkersson-Rosenthal syndrome. The other three patients were diagnosed with idiopathic RFP. Serum immunoglobulin G was high in seven of nine patients (77%). CSF protein electrophoresis demonstrated an elevated albumin fraction in six of nine patients (66%). CSF immunoglobulin G was high in four of nine patients (44%). The oligoclonal band in CSF was negative in all patients. Mean follow-up time was 5.2 +/- 2.6 years and 3 +/- 1.5 years for surgically treated patients and medically treated patients, respectively. None of the patients who underwent the surgery demonstrated recurrence. Although marked recovery was observed in patients who had received medical treatment, three of them had recurrence during the follow-up period. CONCLUSION: Serologic test results have demonstrated immune system involvement in cases of idiopathic RFP and in cases of Melkersson-Rosenthal syndrome, providing no distinction between the two. There was no sign substantiating local antibody production in CSF, which implies that the elevated antibodies in CSF were peripheral in origin. Although the serologic test results were not conclusive for a specific diagnosis, they support an immune-mediated pathogenesis. Despite the small number of patients who underwent the extended transmastoid facial nerve decompression, our follow-up data were suggestive for the prevention of recurrences.

Acoustic impedance study of peritubal myoclonus.

An audible clicking sound coexistent with the contractions of the peritubal muscles is thought to be an isolated form of palatal myoclonus that presents with myoclonal contractions of the soft palate, larynx, pharynx and, sometimes, cervicofacial area. Acoustic impedance measurements, by demonstrating the relation between the muscle contractions and the clicking noise, represent one of the ways in which the diagnosis can be confirmed. This paper reports the impedance changes following various maneuvers and simultaneous electromyography recordings in four patients with peritubal myoclonus and confirms the accuracy and simplicity of these tests. The pathology of peritubal myoclonus and its treatment options are discussed.