Bilateral Choanal Atresia and Endoscopic Surgery: A Chance for CHARGE Patients.

Bilateral choanal atresia (CA) is a rare congenital malformation frequently associated with other anomalies. CHARGE association is closely linked to bilateral CA. The aim of this study was to describe the outcomes of the endoscopic repair in bilateral CA, and to assess the role of postoperative nasal stenting in two cohorts of CHARGE-associated and non-syndromic CA. Thirty-nine children were retrospectively analyzed (16 patients had CHARGE-associated CA). The rate of postoperative neochoanal restenosis was 31.3% in the CHARGE population, and 47.8% in the non-syndromic CA cohort. Data on postoperative synechiae and granulation tissue formation, need for endonasal toilette and dilation procedures, and number of procedures per patient were presented. Stent positioning led to a higher number of postoperative dilation procedures per patient in the non-syndromic cohort (p = 0.018), and to a higher rate of restenosis both in the CHARGE-associated, and non-syndromic CA populations. Children with CHARGE-associated and non-syndromic bilateral CA benefitted from endonasal endoscopic CA correction. The postoperative application of an endonasal stent should be carefully evaluated.

High prevalence of human papillomavirus infection in sinonasal inverted papilloma: a single-institution cohort of patients.

BACKGROUND: Both the prevalence of sinonasal inverted papilloma (IP) and the causal association with alpha-human papillomaviruses (alpha-HPVs) are controversial. In this study we aimed to determine HPV status in histologically selected, microdissected, formalin-fixed, and paraffin-embedded tissue samples of IP. METHODS: HPV status was assessed retrospectively by polymerase chain reaction (PCR)-bead-based multiplex genotyping on tissue samples of patients diagnosed with IP and consecutively treated with endoscopic resection. Forty-one HPV genotypes were considered, distinguishing between high risk and low risk. HPV status was correlated with demographics and clinical variables. Sixty sinonasal IP tissue samples were initially considered. After exclusion of 5 cases due to insufficient quality/quantity of the samples, 55 patients were included for analysis. RESULTS: HPV-DNA sequences were identified in 34 of 55 (61.8%) IPs, with a higher prevalence of high-risk than low-risk HPV genotypes (19 [55.9%] and 15 cases [44.1%], respectively). HPV16 strongly prevailed among the high-risk HPV cases (84.2%), and HPV54 prevailed among the low-risk HPV cases (53.3%). IPs with origin within the maxillary sinus were significantly associated with high-risk HPV (p = 0.019). No significant associations emerged between HPV status and demographics or clinical variables. CONCLUSION: In a series of 55 IP tissue samples, HPV-DNA sequences were identified in 61.8% of cases, which differs from the data of previous investigations. Further case-control studies are advocated to confirm this prevalence in the Italian population addressed, and also to clarify any pathogenic involvement of HPV in the natural history of IPs.

Successful Surgery Management of Giant Periorbital Malignant Schwannoma in a Low-Income Setting.

Schwannoma can arise from any cranial, peripheral or autonomic nerve, except the olfactory and optic. About 25-45% of extracranial schwannomas lie in the head and neck. Data on malignant schwannoma from low-income settings are inconsistent. We reported a case of giant periorbital malignant schwannoma successfully treated in a low-income setting. The strength of our case is given not only by the rarity and the size of the disease but also for highlighting the weakness of health system in low-resource settings. It is mandatory to strengthen the health system with particular attention to physical, psychologic and social aspects and to promote comprehensive programs including all these aspects.

Malignant Versus Benign Tumors of the Sinonasal Cavity: A Case-Control Study on Occupational Etiology.

Case-control studies on malignant sinonasal tumors and occupational risk factors are generally weakened by non-occupational confounders and the selection of suitable controls. This study aimed to confirm the association between sinonasal malignant tumors and patients' occupations with consideration for sinonasal inverted papillomas (SNIPs) as a control group. Thirty-two patients affected by adenocarcinoma (ADC) and 21 non-adenocarcinoma epithelial tumors (NAETs) were compared to 65 patients diagnosed with SNIPs. All patients were recruited in the same clinical setting between 2004 and 2016. A questionnaire was used to collect information on non-occupational factors (age, sex, smoking, allergies, and chronic sinusitis) and occupations (wood- and leather-related occupations, textile industry, metal working). Odds ratios (OR) with 95% confidence intervals (CI) associated with selected occupations were obtained by a multinomial and exact logistic regression. Between the three groups of patients, SNIP patients were significantly younger than ADC patients (p = 0.026). The risk of NAET increased in woodworkers (OR = 9.42; CI = 1.94⁻45.6) and metal workers (OR = 5.65; CI = 1.12⁻28.6). The risk of ADC increased in wood (OR = 86.3; CI = 15.2⁻488) and leather workers (OR = 119.4; CI = 11.3⁻1258). On the exact logistic regression, the OR associated to the textile industry was 9.32 (95%CI = 1.10⁻Inf) for ADC, and 7.21 (95%CI = 0.55⁻Inf) for NAET. Comparing sinonasal malignant tumors with controls recruited from the same clinical setting allowed demonstrating an increased risk associated with multiple occupations. Well-matched samples of cases and controls reduced the confounding bias and increased the strength of the association.

Endoscopic Transsphenoidal Salvage Surgery for Symptomatic Residual Cystic Craniopharyngioma after Radiotherapy.

Objectives Ideal treatment of craniopharyngiomas is still controversial. Radiotherapy (RT) is considered effective for recurrences or after subtotal tumor removal (STR). About 40 to 50% of patients may experience tumor cyst expansion soon after RT; in these cases, the role of salvage surgery is debated. Design Operative video. Setting Tertiary care center. Participants An 11-year-old boy diagnosed with persistent craniopharyngioma. In 2015, the patient underwent right frontotemporal craniotomy for STR at another center, complicated by panhypopituitarism. Two years later, fractionated 54-Gy RT was performed on growing residual tumor. After 3 months, he was admitted to our hospital due to persistent malaise, vomiting, pulsating headache, and epistaxis. Ophthalmologic evaluation evidenced left homonymous hemianopsia. Results A contrast-enhanced magnetic resonance imaging (MRI) showed a 27-mm cystic component enlarging from the cranial end of the persistent craniopharyngioma lesion, extending into the third ventricle. Biventricular hydrocephalus and brain midline shift to the right were present. Compared with the early post-RT MRI, the cystic component of the tumor demonstrated growth. The patient underwent external ventricular drainage placement for emergent treatment of hydrocephalus and endoscopic transsphenoidal surgery. After cystic content drainage, the lesion was completely removed with its capsule. A "gasket seal" technique was performed for skull base reconstruction, with autologous fascia lata, septal bone, and mucoperiosteum from inferior turbinate. Histologic examination confirmed the craniopharyngioma diagnosis. Postoperative MRI showed resolution of the hydrocephalus and complete tumor removal. Conclusion Although shrinkage of cystic components of craniopharyngioma residuals may occur within 5 to 6 months after RT, salvage surgery is indicated in symptomatic patients. The link to the video can be found at https://youtu.be/4x6Qe76bf60 .

Olfaction deterioration in cognitive disorders in the elderly.

BACKGROUND: Parkinson's and Alzheimer's diseases are widespread neurodegenerative pathologies. Parkinson's disease affects about 1 % of the population over the age of 65 years, while Alzheimer is considered the most common cause of dementia, with an annual incidence of 1 % in persons aged 65 years. It has been demonstrated that both these neurodegenerative diseases are associated with smell dysfunction. AIM: The aim of the present review is to describe briefly modern olfactory evaluation tools as well as the importance of olfactory sensitivity screening in the elderly, especially where cognitive disorders, such as Alzheimer's or Parkinson's diseases, are suspected. METHODS: A brief literature review focusing on the basic principle of smell tests is illustrated together with their application in elderly patients affected by cognitive disorders, in particular Parkinson's and Alzheimer's diseases. RESULTS/CONCLUSIONS: Alzheimer's and Parkinson's diseases are both neurodegenerative disorders typically found in the elderly. As both diseases are characterized by the early presence of dysosmia, simple validated smell tests could very well help clinicians in the early diagnosis of these neuropathological conditions. Elderly patients complaining of smell loss and found to be dysosmic, by means of validated olfactory tests, should be neurologically evaluated as early as possible to detect slight motor abnormalities in an at-risk population.

Olfactory threshold for bourgeonal and sexual desire in young adult males.

Olfactory receptors were found to be expressed also in human sperm giving rise to the hypothesis that they might play a role in fertility and sexual behavior. For instance, bourgeonal was demonstrated to be an agonist of sperm cells olfactory receptor, OR1D2. OR1D2 has been found to be expressed in human olfactory epithelium and to play a critical role in human sperm chemotaxis. Recent preliminary evidence showed that olfaction sensitivity (determined by n-butanol olfactory threshold) and sexual desire were associated in young adult males. It is reasonable to hypothesize that bourgeonal olfactory threshold could be related with human sexual behavior and desire. In 37 healthy young adult male volunteers (age range 20-36 years), the bourgeonal odor threshold and the intensity of sexual desire [the latter using the International Index of Erectile Function (IIEF) scale] were examined. In addition, samples of DNA were collected. Allele and genotype frequency of the OR1D2 single nucleotide polymorphisms (SNPs) were then evaluated in order to study the relationship between sexual desire and OR1D2 SNPs expression. The olfactory threshold was categorized as <10, 10⩽threshold<15, 15⩽threshold<20, ⩾20. IIEF 1 and IIEF 2 scores were significantly associated. IIEF1 scores, but not IIEF2 scores were significantly associated with olfactory threshold. No statistically significant associations were found neither between genotypes frequency and sexual desire (IIEF1 and IIEF2), nor between genotypes frequency and olfactory threshold. Hypothesizing for the first time the relationship between bourgeonal olfactory sensitivity and sexual desire in a group of young adult males, the present study found a significant association between lower olfactory threshold for bourgeonal and stronger sexual desire, in terms of IIEF1.

Peak nasal inspiratory flow: a useful and handy tool for the diagnosis of nasal obstruction in the elderly.

The global population is aging, with the over-65 age group expected to double in the USA by 2030. Three subcategories of elderly people have been identified: "young old" (from 65 to 74 years), "older old" (from 75 to 84) and "oldest old" (85 or more). Rhinosinusitis is a common disease that affects more than 31 million people in the USA every year. Nasal obstruction is one of the most common symptoms in patients affected by rhinosinusitis. An accurate nasal obstruction evaluation in the elderly is becoming of increasing interest for medical doctors, especially for geriatricians. Peak nasal inspiratory flow (PNIF) is a cheap and easy method for assessing nasal patency. The purpose of the study was to compare young old normal PNIF values with older old normal PNIF values. Charts relating PNIF normal values in the elderly with various explanatory variables have been provided. PNIF measurements were performed in 113 volunteers aged 65-84 years. One hundred and five of them fulfilled the study criteria and were self-reported healthy elderly. None of them complained of nasal symptoms. Data were statistically analyzed and figures and tables were produced relating PNIF to height, sex and age. PNIF values decreased with age (p = 0.0053) and were significantly lower in the "older old" sub-cohort than in the "young old" group (p = 0.007). Nasal obstruction in the elderly is a common problem and appropriate diagnosis and treatment are important for improving their quality of life. The measurement of PNIF could be useful in evaluating elderly patients who complain of nasal obstruction.

Olfactory sensitivity and sexual desire in young adult and elderly men: an introductory investigation.

BACKGROUND: It has been suggested that olfaction could influence human sexual behavior. Age is negatively associated with many aspects of sexuality and, with increasing age, people tend to show a declining sexual desire. The present pilot study investigated the relationship between sexual desire and olfactory sensitivity in healthy men of two age groups, young adult and elderly (≥65 years old), to ascertain whether their sense of smell could determine sexual desire and whether an age-related weaker olfactory sensitivity could correlate with the decline in sexual appetite in elderly patients. METHODS: Sixty-three volunteers were recruited and divided into two groups, one consisting of 48 healthy young adult men, the other of 15 healthy elderly men. All participants were tested to ascertain their odor threshold for n-butanol (Sniffin' Sticks) and their sexual desire using the International Index of Erectile Function (IIEF), presenting only the questions relating to frequency (IIEF1) and level (IIEF2) of sexual desire. RESULTS: Sexual desire and mean olfactory thresholds were higher in the younger adults than in the elderly men (p = 0.001 and p = 0.02, respectively). There was a significant association between butanol threshold and sexual desire for the young adult group (p = 0.02), but not for the elderly group (p = 0.35). CONCLUSION: This study found a preliminary association between olfaction and sexual behavior, in young adults at least. More studies are needed to improve our knowledge in this intriguing field, possibly using electrophysiological olfactory methods.