Ptosis, facial numbness and parotid gland mass as the first symptoms of an extranodal NK/T cell lymphoma, nasal type.

We present a case of a patient visiting the ear, nose and throat department with a parotid gland mass, ptosis and facial numbness. CT imaging confirmed a mass in the parotid gland; however, it also revealed a mass in the left maxillary sinus. MRI, positron emission tomography combined with CT and nasal biopsy confirmed the diagnosis of a extranodal natural killer/T cell lymphoma, nasal type. Because this is a rare clinical entity in Western society, patients are typically diagnosed in an advanced stage; symptoms resemble chronic rhinosinusitis and histopathological analysis is challenging. In this atypical case, the patient presented with symptoms of ptosis, parotid gland mass and facial numbness instead of nasal symptoms. In this case, we want to emphasise that diagnosing a sinonasal NK/T-cell lymphoma is often challenging.

Outcome of adenotonsillectomy in children with Down syndrome and obstructive sleep apnoea.

OBJECTIVE: To evaluate the outcome of adenotonsillectomy (AT) in a cohort of children with Down syndrome (DS) and obstructive sleep apnoea (OSA). DESIGN: Retrospective, cross-sectional study. SETTING: Tertiary care centre. PATIENTS: Children with DS and OSA, without previous upper airway (UA) surgery. INTERVENTIONS: AT and full overnight polysomnography. MAIN OUTCOME RESULTS: A significant improvement of the obstructive apnoea-hypopnoea index (oAHI) after AT was obtained. No differences in sleep efficiency or sleep fragmentation were found postoperatively. Almost half of the children had persistent OSA (oAHI ≥5/hour). RESULTS: Data are presented as median (lower-upper quartile). Thirty-four children were included, median age 4.0 years (2.7-5.8), body mass index (BMI) z-score 0.81 (-0.46-1.76), and oAHI 11.4/hour (6.5-22.7). The majority presented with severe OSA (58.9%). AT was performed in 22 children, tonsillectomy in 10 and adenoidectomy in two. Postoperatively, a significant improvement of the oAHI was measured from 11.4/hour (6.5-22.7) to 3.6/hour (2.1-9.5) (p=0.001), with a parallel increase of the minimum oxygen saturation (p=0.008). Children with initially more severe OSA had significantly more improvement after UA surgery (p=0.001). Persistent OSA was found in 47.1% of the children. CONCLUSIONS: AT results in a significant improvement of OSA in children with DS without a change in sleep efficiency or sleep stage distribution. Severe OSA was associated with a larger reduction of OSA severity. Almost half of the children had persistent OSA, which was not correlated to age, gender or BMI z-score.

Drug-induced sedation endoscopy in surgically naive children with Down syndrome and obstructive sleep apnea.

OBJECTIVE: To describe the pattern of upper airway (UA) obstruction in surgically naive children with Down syndrome and obstructive sleep apnea (OSA), and to evaluate the outcome of drug-induced sedation endoscopy (DISE)-directed treatment. METHODS: A prospective study of DISE in surgically naive children with Down syndrome and OSA was performed. Treatment was individually tailored based on the DISE findings and was evaluated by control polysomnography (PGS). Results are presented as median (lower-upper quartile) unless otherwise stated. RESULTS: In 41 children, aged 4.2 years (range, 2.8-6.0) with a body mass z score of 1.04 (-0.55 to 1.82) and obstructive apnea-hypopnea index (oAHI) of 10.1/h (range, 6.3-23.0), DISE was performed. Adeno-/tonsillar obstruction was found in 75.6% of the patients, and these patients subsequently underwent UA surgery. Seven patients were non-surgically treated, and three received a combined treatment. A multilevel collapse was present in 85.4%. Tongue base obstruction was present in ten patients (24.4%) and epiglottic collapse in 48.8%. Pre- and postoperative PSG data were available for 25 children (adenotonsillectomy, n = 16; tonsillectomy, n = 7; adenoidectomy, n = 2). A significant improvement in oAHI from 11.4/h (range, 7.7-27.0) to 5.5/h (range, 2.1-7.6) was found. Persistent OSA was present in 52% of the children. No significant association between different DISE findings and persistent OSA could be found. CONCLUSION: Most patients with Down syndrome and OSA present with multilevel collapse on DISE. Adenotonsillectomy results in a significant improvement of the oAHI; however more than half of the patients had persistent OSA, probably due to multilevel collapse. Upper airway evaluation may provide more insights into the pattern of UA obstruction in patients with persistent OSA.

Sleep problems and obstructive sleep apnea in children with down syndrome, an overwiew.

INTRODUCTION: Children with Down syndrome (DS) have a high prevalence of sleep problems, including behavioural sleep disturbances and obstructive sleep apnea. Sleep problems are associated with a wide range of adverse health effects. Since children with DS are already known to have many comorbidities, they are particularly susceptible for the negative impact of sleep problems. Aim of this study is (1) to evaluate the prevalence of sleep problems in children with DS, (2) compare the prevalence of sleep problems in children with DS with a community sample of typical developing school-aged children, and (3) to correlate the existence of sleep problems in children with DS and OSA. METHODS: Children enrolled at the multidisciplinary Down team of the University Hospital Antwerp and seen at the ENT department were eligible for this study. The prevalence of sleep problems was evaluated by the use of the Child Sleep Habits Questionnaire (CSHQ) and a full overnight polysomnography was performed to screen for obstructive sleep apnea. RESULTS: Parents of fifty-four children with DS, aged 7.5 years (5.4-11.6), completed the CSHQ and an overall prevalence of sleep problems was found in 74.1%. In 57.1% of the children OSA was diagnosed with a median obstructive apnea-hypopnea index (oAHI) 7.25/h (5.7-9.8). Overall sleep problems were not age-or gender related, however boys suffer more from daytime sleepiness. Symptoms of sleep disordered breathing correlate with parasomnias, a longer sleep duration and more daytime sleepiness. No correlation was found between sleep problems and underlying OSA. CONCLUSION: Children with Down syndrome have a significantly higher prevalence of sleep problems, compared to normal developing healthy school-aged children. We didn't find any correlation between the parental report of sleep problems and underlying OSA, or OSA severity.

Prevalence of Obstructive Sleep Apnea in Children with Down Syndrome.

STUDY OBJECTIVES: To evaluate the prevalence of obstructive sleep apnea (OSA) in a large cohort of children with Down syndrome (DS), and to investigate which patient- related factors correlate with disease severity. METHODS: We performed a retrospective, cross-sectional study in children with DS referred for full overnight polysomnography in a tertiary care center. RESULTS: Polysomnographic data are available for 122 children (70 boys), age 5.0 y (2.8-10.5), and body mass index (BMI) z-score 0.7 (-0.3 to 1.7). The overall prevalence of OSA was 66.4%. In almost half of these children severe OSA was diagnosed (obstructive AHI [oAHI] ≥ 10/h). In children with parental reports of snoring or witnessed apneas (group A), OSA was significantly more common (75.7%) than in those without these symptoms (group B) 53.8% (P = 0.019). Children in group A had more severe OSA, oAHI 5.7/h (1.7-13.8) compared to those in group B 2.2/h (0.8-8.0) (P = 0.018). A significant inverse correlation between age and oAHI (P = 0.028) was found. Sex and BMI z-score were not significantly correlated to oAHI. CONCLUSIONS: Based upon full night polysomnography, an overall 66.4% prevalence of OSA was found in children with Down syndrome. Even in those with a negative history for OSA, the prevalence was 53.8%. Younger age was associated with more severe disease.

Immunohistochemical characterisation of dendritic cells in human atherosclerotic lesions: possible pitfalls.

BACKGROUND: Previously we demonstrated decreased blood myeloid (m) and plasmacytoid (p) dendritic cell (DC) counts in atherosclerotic patients. Therefore, we examined whether DCs, in particular DC precursors, accumulate in human plaques. METHODS: Blood DC antigen (BDCA)-1, CD11c (mDCs), BDCA-2, CD123 (pDCs), langerin, fascin, S-100 (immature/mature DCs), and CD1a and CD83 (mature DCs) were investigated by immunohistochemistry of carotid arteries obtained by endarterectomy (EAS, frozen n = 11, fixed n = 11) or autopsy (fixed, n = 87). RESULTS: Fascin and S-100 required formaldehyde fixation, other markers needed cryo-preservation. BDCA-1, BDCA-2, langerin, and S-100 appeared specific for intimal DCs, unlike CD123 and fascin (staining endothelial cells), CD11c and CD1a (staining monocytes, foam cells) or CD83 (staining lymphocytes). BDCA-1 and BDCA-2 cells were detected in EAS, preferentially near microvessels. S-100 cells increased successively from intimal thickening, via pathological intimal thickening, fibrous cap atheroma and finally complicated plaques. Fascin cells followed the same pattern, but were more abundant. However, in lesions containing microvessels (complicated plaques, plaque shoulders and most EAS) this was partly explained by fascin positive endothelial cells. Even complicated plaques contained relatively few mature CD83 DCs. CONCLUSIONS: Accumulation of BDCA-1 and BDCA-2 around neovessels showed that mDCs and pDCs are recruited to advanced plaques, which is in line with the previously described decline of circulating blood DCs in patients with coronary artery disease. Unexpectedly, several DC markers yielded false positive signals. Hence, some accounts on numbers, trafficking and activation of DCs in atherosclerotic plaques may require re-evaluation.