Abstract. Pediatric sinonasal rhabdomyosarcoma (PSR) is a rare condition, with a limited number of previously reported cases. It is an aggressive malignancy associated with poor outcomes, and no consensus has been reached on the optimal therapeutic strategy. The present study reported the case of embryonal PSR in a 2-year old girl, presenting with unilateral nasal obstruction and a polypoid mass protruding from the left nasal cavity. The pediatric patient was initially treated with surgical resection, followed by adjuvant chemotherapy containing vincristine (1.5 mg/m 2 , weekly) and actinomycin-D (1.5 mg/m 2 , three times weekly). On the 10th month of follow-up, tumor recurrence was detected and a salvage surgery was performed, while the same chemotherapy regimen was resumed. Following the first cycle of chemotherapy, the patient developed a fungal bronchopneumonia and succumbed due to disease progression, acute respiratory distress syndrome and septic shock 12 months after diagnosis.
The functional resemblance between kidney proximal tubular and inner ear epithelial cells which has often been pointed out in the literature led us to hypothesize that nephrotoxic agents that cause renal tubular injury might also impair the function of inner ear cells. As one of the most toxic environmental nephrotoxic agents is cadmium, we aimed to study its effects on hearing experimentally in rats. In this study, increased blood and renal cortical cadmium levels were associated with high cadmium accumulation in ear ossicles and labyrinth in rats exposed to cadmium. The changes in auditory brainstem response (ABR) and otoacoustic emission in 2-month-old male rats exposed to drinking water containing 5 and 15 ppm CdCl2 for 30 days showed that cadmium-induced nephrotoxicity was associated with signs of defective hearing at a concentration of 15 ppm CdCl2 but that 5 ppm CdCl2 caused hearing loss without affecting kidney function. The mean latency of ABR wave 1, which indicates the function of the cochlea, was 1.335 +/- 0.31 ms in the control group and 1.641 +/- 0.052 and 1.74 +/- 0.88 ms in the rats subjected to 5 and 15 ppm CdCl2, respectively (p < 0.001). In the cadmium-treated groups short interpeak wave I-III latencies (p < 0.01) indicated cochlear dysfunction and this was also supported by the distortion product otoacoustic emission results (p < 0.001). Non-significant changes in wave III and V latencies were accepted as evidence of unaltered function of the other parts of the auditory system. These results suggest that hair cells are more sensitive to cadmium than kidney tubule cells and that the cochlear component of hearing is more vulnerable to cadmium toxicity than other parts of the auditory system.
The aim of this study was to evaluate the effects of modified tongue base suspension (mTBS) procedure on continuous positive airway pressure (CPAP) levels in patients with severe obstructive sleep apnea (OSA). From November 2011 to December 2012, a total of 31 patients with severe OSA who underwent mTBS were included into this prospective case series with planned data collection. Prior to surgery, all the patients were subjected to a polysomnography (PSG) and CPAP titration on two separate nights. Following the surgery, patients were subjected to a control PSG and CPAP titration at the sixth month of follow-up period. The preoperative and postoperative mean apnea hypopnea index (AHI), CPAP titration values, AHI during CPAP use and amount of sleeping time with CPAP were compared. Median age was 48 years (range 31-66), and most patients were male (87.0 %). Postoperative mean AHI (44.73 ± 17.05 vs. 19.96 ± 19.52), optimal CPAP value (12.64 ± 1.60 vs. 8.00 ± 1.77) and AHI during CPAP use (3.79 ± 1.78 vs. 2.25 ± 1.81) were decreased, and the amount of sleeping time with CPAP (5.29 ± 0.84 vs. 6.52 ± 0.89) was increased significantly (p < 0.001 for all parameters). The surgery was considered to be successful when 50 % reduction in the mean AHI and/or the decrease of AHI below 20/h were obtained. A total of 24 patients (77.4 %) met the surgical success criteria. The mTBS is a safe and feasible procedure with favorable effects on CPAP levels in patients with severe OSA.
We aimed to evaluate existing research into the effectiveness and safety of two tongue base suspension (TBS) techniques (Repose(®) system and modified TBS) with or without uvulopalatopharyngoplasty (UPPP) in obstructive sleep apnea. The literature review was performed using PubMed database. Searched terms used included the keywords "sleep apnea", "surgery", "tongue", "tongue base", "suspension", "Repose", "uvulopalatopharyngoplasty", and "hypopharynx". Levels of evidences and grades of recommendations were determined according to the hierarchy proposed by Oxford Centre for Evidence-based Medicine. Seven studies including 113 patients met the eligibility criteria for TBS as a stand-alone procedure. Four of seven studies including 62 patients used the Repose(®), and three studies including 51 patients used the modified TBS. The success rates were higher in the studies that used modified technique (74.5 %) than those that used the Repose(®) (25.8 %), (p < 0.001). Ten studies including 300 patients met the eligibility criteria for TBS combined with UPPP. Seven of ten studies including 176 patients used the Repose(®), and three studies including 124 patients used the modified TBS. The success rates in this group were similar between the modified TBS (73.4 %) and Repose(®) (67.6 %), (p = 0.341). When aggregate data of 413 patients were compared, the modified TBS was found to be associated with significantly higher success rates (73.7 vs. 56.7 %, p < 0.001). The evidence supports primarily grade C recommendations for the benefits of both techniques with or without UPPP; although there is a trend toward improved outcome with modified technique.
These results suggest that simultaneous and bilateral occurrence of SPP is an indicator of an advanced lung pathology with a poor prognosis.
The aim of this study was to evaluate the influence of multilevel upper airway surgery on subsequent continuous positive airway pressure (CPAP) use and tolerance in patients with moderate to severe obstructive sleep apnea (OSA). The study cohort enrolled 67 consecutive patients, who underwent septoplasty plus modified uvulopharyngopalatoplasty (mUPPP) with or without modified tongue base suspension (mTBS) due to CPAP intolerance, and who had residual OSA requiring CPAP therapy [non-responders to surgery, apnea-hypopnea index (AHI) >15 events/h] that had been confirmed by control polysomnography at the sixth month postoperatively. A questionnaire including questions on postoperative CPAP use, problems faced during CPAP use after the surgery, change in OSA symptoms, and satisfaction with the surgery was designed, and filled through interviews. Seventeen (25.4 %) patients had septoplasty plus mUPPP and 50 (74.6 %) had septoplasty plus mUPPP combined with mTBS. Postoperatively, mean AHI (45.00 ± 19.76 vs. 36.60 ± 18.34), Epworth sleepiness scale (ESS) score (18.00 ± 4.45 vs. 13.00 ± 4.72), oxygen desaturation index (ODI) (48.98 ± 16.73 vs. 37.81 ± 17.03), and optimal CPAP level (11.80 ± 1.40 vs. 8.96 ± 1.20) were decreased (p < 0.001 for all parameters). Fifty-nine percent of patients reported that they fairly satisfied with the surgery and 49.2 % reported that their symptoms were completely resolved. While none of the cases could tolerate CPAP before surgery, almost half (47.8 %) of the cases used CPAP without problems postoperatively. Postoperative CPAP users had significantly higher postoperative AHI (p = 0.001), supine AHI (p = 0.009), ESS (p = 0.019), and ODI (p = 0.014), and significantly lower postoperative minimum O2 saturation (p = 0.001) compared with non-users. Multilevel upper airway surgery with less invasive techniques may improve CPAP tolerance in well-selected patients.
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