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Background and Purpose The aim of this study is to describe the prevalence of obstructive sleep apnea (OSA) and its level of severity associated with Orticochea pharyngoplasty in patients with velopharyngeal insufficiency after at least 1 year of the surgical procedure. Design Case series prospective descriptive study Main Outcome Measures At FISULAB, a rehabilitation center for patients with cleft palate, we studied 37 patients who were treated elsewhere with Orticochea pharyngoplasty for velopharyngeal insufficiency; these patients may or may not have had clinical symptoms related to OSA. All participants underwent a polysomnography sleep study, which was also done in different institutions. We applied the Epworth Sleepiness Scales during the clinical investigation because it is an effective instrument used to measure average daytime sleepiness. Another questionnaire to identify cases of OSA was used. Among other variables studied, the apnea/hypopnea index was the main outcome, while age and type of cleft were secondary variables. Results From 37 patients who were studied (100%), we obtained the following results: normal apnea/hypopnea index: seven patients (18.9%); mild apnea/hypopnea index: 14 patients (37.8%); moderate apnea/hypopnea index: eight patients (21.6%); and severe apnea/hypopnea index: eight patients (21.6%) Conclusions In this study, we found that more than three quarters (81%) of the patients who were treated for velopharyngeal insufficiency with Orticochea pharyngoplasty presented obstructive sleep apnea when analyzing the apnea/hypopnea index in the polysomnography sleep study.

Objective The aim of this study is to compare patients treated with pharyngoplasty and those treated with palatoplasty for velopharyngeal insufficiency to establish what surgical procedure poses the highest risk for developing sleep apnea. The hypothesis tested in this study is that the incidence of obstructive sleep apnea syndrome associated with pharyngoplasty is greater than that associated with palatoplasty for velopharyngeal insufficiency. Subjects Twenty patients were taken from the Institution FISULAB. Design Observational cohort analytic study. Main Outcome Measures An overnight polysomnographic study was used to determine the incidence and severity of obstructive sleep apnea syndrome. Results The incidence of obstructive sleep apnea syndrome following pharyngoplasty was shown to be significantly higher than after palatoplasty. The apnea-hypopnea index, also called the respiratory disturbance index, was 12.7 in the pharyngoplasty group and 1.35 in the palatoplasty group (p < .001). When obstructive sleep apnea syndrome was stratified into different levels of severity according to the values of respiratory disturbance index, there were noticeable differences between these two groups. In the palatoplasty group, one patient had mild obstructive sleep apnea syndrome. In the pharyngoplasty group, two patients had mild obstructive sleep apnea syndrome, one patient had moderate obstructive sleep apnea syndrome, and two patients had severe obstructive sleep apnea syndrome. Conclusions When comparing the apnea-hypopnea index (i.e., respiratory disturbance index) of patients treated for velopharyngeal insufficiency with palatoplasty versus pharyngoplasty, we observed an important difference between the groups, with the highest indices in the pharyngoplasty group.

Abstract Context The reproductive axis is controlled by a network of gonadotropin-releasing hormone (GnRH) neurons born in the primitive nose that migrate to the hypothalamus alongside axons of the olfactory system. The observation that congenital anosmia (inability to smell) is often associated with GnRH deficiency in humans led to the prevailing view that GnRH neurons depend on olfactory structures to reach the brain, but this hypothesis has not been confirmed. Objective The objective of this work is to determine the potential for normal reproductive function in the setting of completely absent internal and external olfactory structures. Methods We conducted comprehensive phenotyping studies in 11 patients with congenital arhinia. These studies were augmented by review of medical records and study questionnaires in another 40 international patients. Results All male patients demonstrated clinical and/or biochemical signs of GnRH deficiency, and the 5 men studied in person had no luteinizing hormone (LH) pulses, suggesting absent GnRH activity. The 6 women studied in person also had apulsatile LH profiles, yet 3 had spontaneous breast development and 2 women (studied from afar) had normal breast development and menstrual cycles, suggesting a fully intact reproductive axis. Administration of pulsatile GnRH to 2 GnRH-deficient patients revealed normal pituitary responsiveness but gonadal failure in the male patient. Conclusions Patients with arhinia teach us that the GnRH neuron, a key gatekeeper of the reproductive axis, is associated with but may not depend on olfactory structures for normal migration and function, and more broadly, illustrate the power of extreme human phenotypes in answering fundamental questions about human embryology.

To evaluate if timing of chemotherapy with Temozolomide (TMZ) was able to modify the outcome of glioblastoma (GBM), we analyzed two comparable series of supratentorial GBM patients, treated with surgery and radiotherapy, in which the administration of TMZ has been performed in the first group at first relapse and in the second group in newly diagnosed cases. The end-points were the median survival, the time tumor progression (TTP) and also the Karnofsky (KPS) scale and the Mini Mental State Examination (MMSE) scale at follow-up. From December 1999 to December 2001 30 patients with recurrent GBM received TMZ until progression. From January 2002 to January 2004 38 newly diagnosed patients received a first cycle of TMZ immediately after surgery, and additional cycles after completing radiotherapy until recurrence. In order to obtain a greater drug exposure we adopted a once-daily 10 days schedule of TMZ every 28 days as follows: 150 mg/m(2)/day (day 1-5) and 75 mg/m(2)/day (day 6-10). The first group had a median overall survival of 14 months and a median TTP of 6. The second group had a median survival of 16 months and a median TTP of 10. The difference of TTP was statistically significant (P < 0.001), while the overall survival was not. The values of KPS and MMSE at 12 months demonstrated a better quality of life in the second group (P < 0.01). Our regimen permitted to cover the therapeutic \"window\" between surgery and the beginning of radiotherapy in newly diagnosed cases and is well tolerated by the patients with limited side effects. We will propose as alternative option when the concomitant radio-chemotherapic protocol is not feasible.