Esthesioneuroblastoma: Endonasal Endoscopic Treatment

Objectives: To illustrate endoscopic techniques used in the treatment of esthesioneuroblastoma and to advocate this method of management followed by radiation therapy in selected cases. Methods: A retrospective case series of 10 patients with esthesioneuroblastomas treated between 1999 and 2004 at a tertiary referral center using endonasal techniques. Results: Mean age of patients at presentation was 56.2 years (median 57 years). Seven patients were female and 3 patients were male. Kadish staging at presentation was: A – 3 patients, B – 5 patients, C – 2 patients. Dulguerov stage at presentation was: T1 – 4 patients, T2 – 4 patients, T3 – 2 patients. The period of follow-up ranged from 11 months to 74 months (mean 38.1 months, median 39 months). To date no recurrence has developed in any patient. Conclusions: Our experience suggests that endoscopic surgery can be an effective method of management. Strict attention to selection criteria, careful surgical techniques, and regular follow-up are key elements for success

Nasal endoscopy in asthmatic children: clinical role in the diagnosis of rhinosinusitis.

The aim of the study was to determine the role of rigid nasal endoscopy in the diagnosis of rhinosinusitis and adenoiditis in asthmatic children. Hundred-forty-five asthmatic children (aged 2-15 years) with recurrent upper respiratory symptoms were evaluated with complete ENT examination and nasal endoscopy by rigid endoscope during local anaesthesia. A step by step endoscopic procedure is described. Endoscopy was successfully performed in 128 patients (88.3%). Purulent rhinosinusitis was diagnosed in 61 subjects (47.6%) and adenoiditis in 45 subjects (35.1%). Rhinosinusitis was associated with adenoiditis in 35 subjects (27.3%), more frequently in younger children (i.e. 2-5 years). Nasal bacteria occurred in 90% of rhinosinusitis patients. Numerous anatomical anomalies were identified. Endoscopy of nasal cavity and rhinopharynx is less traumatic and more readily accepted than other methods. Nasal endoscopy may be proposed as an appropriate routine diagnostic tool in children since it is well tolerated, easily and quickly performed, cost-efficient, and useful in diagnosing rhinosinusitis

Short-term effect of rhGH treatment in children with Prader-Labhart-Willi sindrome on respiratory function: a polysomnographic and ENT endoscopic study

Background: Although significant improvement of respiratory function has been reported during rhGH treatment of Prader-Labhart-Willi Syndrome (PLWS), in the last five years several cases of sudden death during the first 6 months of GH therapy were reported in literature. Objectives and hypotheses: to establish if a short-term GH treatment causes obstructive apnea in PWLS patients with normal upper airways patency. Population and/or methods: 12 patients (6 boys , aged 3.9±1.9 years) were studied by one night polysomnography (PSG) monitoring (Grass 4.2) and ENT video endoscopic before and after six weeks of rhGH treatment (0.03mg/kg b.w./day). All patients underwent auxologic assessment including height, weight, BIA and plicometry, fasting blood glucose, insulin and IGF1 evaluation. The main PSG parameter considered was total apnea hypopnea index (AHI), resulting from two components: central apnea hypopnea index (CI) and obstructive apnea hypopnea index (OI). All patients were normal weight and free of severe or moderate upper airways obstruction (such as adenoid/tonsil hypertrophy, nasal obstruction with or without respiratory tract infection) when started GH treatment. Results: Statistically unsignificant changes of PSG indexes were observed after six weeks of GH therapy (see Table). We didn’t find any statistically significantly correlation between body fat, IGF1 and insulin and PSG changing during GH treatment. Video endoscopic ENT evaluation didn’t showed any significant increase of tonsillar and adenoid size after GH treatment. Precise conclusions: Our data confirm previous studies (Miller et al., JCEM, 2006; Festen et al. JCEM 2006) showing that GH treatment doesn’t cause obstructive apnea in PWLS patients with normal upper airways patency

Relation between polysomnography and ENT endoscopic findings in children with Prader-Labhart-Willi syndrome

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The effect of twelve months rhGH treatment on upper airways of non severely obese children with Prader-Willi syndrome.

In a previous study we showed in 16 non-severely obese and obstructive apnoea free children with Prader-Willi Syndrome that a six weeks rhGH treatment does not significantly affect airways patency. We report here the auxological and polisomnographic results of twelve among the above children who attained 12 month of rhGH treatment.Patients and Methods: Twelve children (9 boys, aged from 1.6 to 7.7 median 2.25 yrs) with genetically confirmed Prader-Willi syndrome were studied before and after 6 weeks and 12 months of rhGH treatment. In each of the three evaluations the patients were studied by anthropometry, one night 16 channels polysomnography and ENT examination of upper airways by flexible fibreoptic endoscope. The polysomnographic parameters considered were respiratory disturbance index (RDI), obstructive apnea index (OAI) and minimal SaO2. The Katz criteria were used for polysomnography evaluation. Tonsils hypertrophy was scored from 0 to +4 according to the Brodsky criteria and adenoid hypertrophy was classified as Wang criteria. SDS BMI was calculated according to CDC standards. Non parametric Friedman test and Fisher's exact test were used for statistical analysis.Results: The main results are reported in the table. In particular we found a statistically significant reduction of RDI after 12 months of rhGH treatment and any reduction of upper airways patency in all but in one patient who required adenotonsillectomy. SDS BMI showed a slight, statistically unsignificant increase.Conclusions: The significant reduction of RDI is in agreement with previous studies showing improvement of respiratory function during GH treatment of Prader-Willi children. The important adenoids and tonsils hypertrophy observed in one of our patients during medium-term GH treatment, does not prove a GH dependency of lymphatic tissue hypetrophy, however points out the importance of a close ENT follow-up in these patients

Chirurgia endoscopica naso-sinusale in pazienti affetti da fibrosi cistica: nostra esperienza

Comunicazione al XX Congresso Nazionale SIOP 2009. Contributi Scientific