The prevalence of gastroesophageal reflux disease and laryngopharyngeal reflux in patients with dysphagia after radiotherapy for nasopharyngeal carcinoma.

BACKGROUND: The prevalence of gastroesophageal reflux disease (GERD) and laryngopharyngeal reflux (LPR) in post-irradiated patients with nasopharyngeal carcinoma (NPC) is unknown. MATERIALS AND METHODS: In a cross-sectional study, 31 NPC and 12 control patients completed questionnaires for GERD/LPR before esophageal manometry and 24-h pH monitoring. The DeMeester score and reflux finding score (RFS) were used to define GERD and LPR, respectively. Risk factors were identified. RESULTS: 51.6% of NPC and 8.3% of control patients, and 77.4% of NPC and 33% of control patients, were GERD-positive and LPR-positive, respectively. The GERD/LPR questionnaire failed to identify either condition in patients with NPC. No parameter differences in esophageal manometry or pneumonia incidence were noted between GERD/LPR-positive and GERD/LPR-negative patients. Post radiotherapy duration, high BMI, lack of chemotherapy, and dysphagia were positive risk factors for GERD/LPR. CONCLUSIONS: A high prevalence of GERD/LPR in patients with post-irradiated NPC exists, but reflux symptoms are inadequate for diagnosis.

Oro-pharyngo-esophageal radionuclide scintigraphy predicts aspiration pneumonia risk and associated survival in post-irradiated nasopharyngeal carcinoma patients.

OBJECTIVE: To demonstrate that oro-pharyngo-esophageal radionuclide scintigraphy (OPERS) not only detects tracheobronchial aspiration after swallowing, but also quantifies the amount of aspiration and subsequent clearance. METHODS: Data collected between 2014 and 2019 were reviewed for aspiration pneumonia at 12 and 24-months after OPERS. The predictive value for aspiration pneumonia on flexible endoscopic evaluation of swallowing (FEES), videofluoroscopic swallowing study (VFSS), and OPERS, and the overall survival of patients with or without aspiration were determined. RESULTS: Thirty-seven patients treated with radiotherapy for nasopharyngeal carcinoma (NPC) were reviewed. The incidence of aspiration detected on FEES, VFSS, and OPERS was 78.4%, 66.7%, and 44.4%, respectively. Using VFSS as a gold standard, the sensitivity and specificity of OPERS for aspiration was 73.7% and 100%. The positive and negative predictive values for aspiration were 100% and 66.7%, respectively, with an overall accuracy of 82.8%. A history of aspiration pneumonia was one factor associated with a higher chance of subsequent aspiration pneumonia within 12 months (odds ratio: 15.5, 95% CI 1.67-145.8, p < .05) and 24 months (odds ratio: 23.8, 95% CI 3.69-152.89, p < .01) of the swallowing assessment. Aspiration detected by OPERS was a significant risk factor for future aspiration pneumonia at 12 and 24 months respectively. Significantly, better survival was associated with an absence of aspiration on OPERS only, but not on FEES or VFSS. CONCLUSION: OPERS predicts the safety of swallowing, the incidence of subsequent aspiration pneumonia, and the survival prognosis in post-irradiated NPC dysphagia patients. LEVEL OF EVIDENCE: 3.

Assessment of pharyngeal motor function using a novel velopharyngeal squeeze maneuver and a novel endoscopic pharyngeal contraction grade scale in patients with dysphagia after radiotherapy for nasopharyngeal carcinoma.

BACKGROUND: To investigate a novel velopharyngeal squeeze maneuver (VPSM) and novel endoscopic pharyngeal contraction grade (EPCG) scale for the evaluation of pharyngeal motor function. METHODS: During endoscopic examination of 77 post-irradiated nasopharyngeal carcinoma patients and control subjects, VPSM was rated and lateral pharyngeal wall movement graded with EPCG scale during swallowing. Pharyngeal constriction ratio (PCR) measured by videofluoroscopy was used for correlation. RESULTS: VPSM and EPCG scale showed almost perfect intra-rater and inter-rater reliability (Kappa: >0.90). VPSM was present in 61% of patients suggesting good pharyngeal motor function. VPSM was predictive of EPCG scale (Wald statistic = 29.99, p < 0.001). EPCG scale also correlated strongly with PCR (r: 0.812) and was predictive for aspiration (odds ratio: 22.14 [95% CI 5.01-97.89, p < 0.001]). CONCLUSIONS: VPSM and EPCG scale are two novel tools to assess pharyngeal motor function, and both correlate well with pharyngeal contractility and aspiration.

Screening for Fabry Disease in patients with unexplained left ventricular hypertrophy.

Fabry Disease (FD) is a systemic disorder that can result in cardiovascular, renal, and neurovascular disease leading to reduced life expectancy. FD should be considered in the differential of all patients with unexplained left ventricular hypertrophy (LVH). We therefore performed a prospective screening study in Edmonton and Hong Kong using Dried Blood Spot (DBS) testing on patients with undiagnosed LVH. Participants found to have unexplained LVH on echocardiography were invited to participate and subsequently subjected to DBS testing. DBS testing was used to measure α-galactosidase (α-GAL) enzyme activity and for mutation analysis of the α-galactosidase (GLA) gene, both of which are required to make a diagnosis of FD. DBS testing was performed as a screening tool on patients (n = 266) in Edmonton and Hong Kong, allowing for detection of five patients with FD (2% prevalence of FD) and one patient with hydroxychloroquine-induced phenocopy. Left ventricular mass index (LVMI) by GLA genotype showed a higher LVMI in patients with IVS4 + 919G > A mutations compared to those without the mutation. Two patients were initiated on ERT and hydroxychloroquine was discontinued in the patient with a phenocopy of FD. Overall, we detected FD in 2% of our screening cohort using DBS testing as an effective and easy to administer screening tool in patients with unexplained LVH. Utilizing DBS testing to screen for FD in patients with otherwise undiagnosed LVH is clinically important due to the availability of effective therapies and the value of cascade screening in extended families.

Pretreatment and early intratreatment prediction of clinicopathologic response of head and neck cancer to chemoradiotherapy using 1H-MRS.

PURPOSE: To determine if choline (cho) identified by proton magnetic resonance spectroscopy ((1)H-MRS) performed pretreatment and early in the course of treatment predicts clinicopathologic response of head and neck squamous cell carcinoma (HNSCC). MATERIALS AND METHODS: In all, 60 patients with HNSCC scheduled to undergo concurrent chemoradiotherapy or radiotherapy alone were recruited. (1)H-MRS was performed pretreatment and early intratreatment (2 weeks after start of treatment). Cho:creatine and cho:water ratios at each timepoint and change in the ratios between the two timepoints were correlated with locoregional failure, distant metastases, overall survival, and cancer-related death. Statistical analysis was performed using logistic regression and chi-square and a P-value of < 0.05 was considered statistically significant. RESULTS: Cho was identified in 47/49 successful pretreatment spectra and 42 of these 47 underwent successful (1)H-MRS early intratreatment, of which 21 showed persistent cho. Locoregional failure occurred in 15, distant metastases in 6, and death in 15 patients; the follow-up period in survivors ranged from 13-64 months (mean, 39 months). No statistically significant correlation was found between (1)H-MRS parameters and clinical endpoints. CONCLUSION: The pretreatment cho and change in cho early during a course of treatment did not predict clinical outcome.

Squamous cell carcinoma of the head and neck: diffusion-weighted MR imaging for prediction and monitoring of treatment response.

OBJECTIVE: To investigate the role of diffusion-weighted imaging (DWI) in predicting and monitoring chemoradiotherapy response in head and neck squamous cell carcinoma (HNSCC). METHODS: Diffusion-weighted imaging was performed pre-treatment (n = 50), intra-treatment (n = 41) and post-treatment (n = 20). Apparent diffusion coefficient (ADC) values were correlated with locoregional failure (LF). RESULTS: Locoregional failure occurred in 20/50 (40%) patients. A significant correlation was found between LF and post-treatment ADC (p = 0.02) but not pre- or intra-treatment ADC. Serial change in ADC was even more significant (p = 0.00001), using a fall in ADC early (pre- to intra-treatment) or late (intra- to post-treatment) to indicate LF, achieved 100% specificity, 80% sensitivity and 90% accuracy. CONCLUSIONS: Single ADC measurements pre- or intra-treatment did not predict response, but ADC post-treatment was a marker for LF. Serial change in ADC was an even stronger marker, when using an early or late treatment fall in ADC to identify LF.

Monitoring of treatment response after chemoradiotherapy for head and neck cancer using in vivo 1H MR spectroscopy.

Elevated choline (Cho) level has been documented on proton magnetic resonance spectroscopy ((1)H MRS) in head and neck squamous cell carcinoma and therefore percentage changes in Cho levels after chemoradiotherapy may serve as a marker of residual cancer in a post-treatment mass (PTM). Forty-six patients underwent (1)H MRS before treatment and the 30 patients with a PTM underwent repeat (1)H MRS at 6 weeks post-treatment. The percentage change in Cho/creatine and Cho/water ratios were correlated with residual cancer. The mean pretreatment Cho/creatine and Cho/water ratios were 2.24 and 1.20 x 10(-3), respectively. Cho persisted in four out of nine PTMs with residual cancer. Cho was absent in five out of nine PTMs with residual cancer and 21/21 PTMs without cancer. The number of PTMs with persistent Cho was too small to allow analysis of percentage change in ratios but the presence of Cho in a PTM showed significant correlation with residual cancer (p = 0.0046), producing a sensitivity, specificity, positive predictive value and negative predictive value of 44%, 100%, 100% and 81%, respectively. Therefore, the presence of Cho in a PTM may serve as a marker of residual cancer. Furthermore since so few PTMs contain Cho, a percentage change in Cho ratios may not be a useful method for monitoring treatment response.

MRI of radiation-induced tumors of the head and neck in post-radiation nasopharyngeal carcinoma.

The aim of this study was to document the sites and MRI features of radiation-induced tumors (RITs) in the head and neck following treatment for nasopharyngeal carcinoma (NPC). The MRI examinations and clinical records of 20 patients with 21 RITs were reviewed retrospectively. RITs developed 3-30 years after radiotherapy and included eleven squamous cell carcinomas, six sarcomas, two neuroendocrine carcinomas, one mucoepidermoid carcinoma and one meningioma. RITs arose in the maxillary region (9), oro/hypopharynx and oral cavity (5), external auditory canal (4), nasopharynx and sphenoid sinus (2) and brain (1). Radiation-induced carcinoma and sarcoma had MRI features that were useful to distinguish them from recurrent NPC. To improve early detection of RITs, the check areas on an MRI of a patient with previous NPC treated by radiation should always include the maxillary region, tongue, and external auditory canal/temporal bone.

Hemangioblastoma of filum terminale associated with arteriovenous shunting.

BACKGROUND: Spinal arteriovenous shunt typically presents in middle age or in the elderly with a strong male predilection. The clinical presentation is usually progressive neurological deficits such as paraparesis or incontinence due to cord edema, although back pain is also a common presentation. Progress of neurological deficit is typically stopped by occlusion of the shunt (surgically or endovascularly), but the return of loss of function may be found in less than half of these patients. In contrast, spinal hemangioblastomas usually occur in adults, and the most common presentation is pain with radiculopathy. Location in the filum terminale is very rare. CASE DESCRIPTION: After a review of the medical literature, we identified 7 cases of hemangioblastomas arising from the filum terminale (Am J Neuroradiol. 2005;26:936-945; Acta Neurochir [Wien]. 2000;142:1059-1062; J Neurosurg Sci. 2001;45:58-62; J Clin Neurosci. 2006;13:285-288; Neurosurgery. 1999;44:220-223; Clin Neurol Neurosurg. 1985;87:55-59). We report an additional case of a filum terminale hemangioblastoma occurring in a 64-year-old man with 1 month exacerbation of chronic low back pain. Preoperatively, it was misdiagnosed as filum terminale arteriovenous fistula. CONCLUSION: Even with modern imaging, preoperative diagnosis can still be difficult.

Endovascular coiling versus neurosurgical clipping for ruptured intracranial aneurysms: significant benefits in clinical outcome and reduced consumption of hospital resources in Hong Kong Chinese patients.

OBJECTIVES: Using specific selection criteria to determine whether endovascular coiling as compared to neurosurgical clipping is associated with significant benefits, in terms of 1-year clinical outcomes and consumption of hospital resources, for the treatment of ruptured intracranial aneurysms in Hong Kong Chinese patients. DESIGN: Retrospective study. SETTING: University teaching hospital, Hong Kong. PATIENTS: Records of outcomes of 169 consecutive Chinese patients, who were treated with endovascular coiling (n=80) or surgical clipping (n=89), were reviewed. All patients were followed up clinically for a mean of 55 (standard deviation, 201) months and radiologically with sequential digital subtraction angiography at 6 and 18 months after treatment. RESULTS: The mean ages of patients were 56 (standard deviation, 13) years for the coiling group, and 57 (standard deviation, 13) years for the clipping group (P=0.575). The median aneurysm size was 4 mm in both groups (P=0.898). The severity of subarachnoid haemorrhage in the two groups did not differ (P=0.619). The rate of death or permanent disability leading to dependency (Glasgow Outcome Scale, 1-3) at 1 year was significantly lower in the coiling group (12/80, 15%) as compared to the clipping group (30/89, 34%) [P=0.005], resulting in a risk reduction of 19% (95% confidence interval, 6-32%). There were significantly more frequent admissions into the intensive care unit in the clipping group (P<0.001); the median duration of intensive care unit stay was 2 days (vs 0 days in the coiling group). The incidence of subsequent treatment procedures for residual or recurrent aneurysm was more common in the coiling group (13/80 vs 3/89; P=0.004). CONCLUSION: Endovascular coiling as compared to neurosurgical clipping for treatment of patients with ruptured intracranial aneurysms is associated with significant benefits in terms of a reduced need for intensive care unit admissions and better general clinical outcomes in Hong Kong Chinese patients.

Nasopharyngeal cancers: which method should be used to measure these irregularly shaped tumors on cross-sectional imaging?

PURPOSE: To determine whether the standard techniques of measuring tumor size and change in size after treatment could be applied to the measurement of nasopharyngeal cancers, which are often irregular in shape. METHODS AND MATERIALS: The standard measurements of bidimensional (BDM) (World Health Organization criteria) and unidimensional (UDM) (Response Evaluation Criteria in Solid Tumors [RECIST] criteria), together with the maximum depth of the tumor perpendicular to the pharyngeal wall (DM), were acquired from axial magnetic resonance images of primary nasopharyngeal carcinoma in 44 patients at diagnosis and in 29 of these patients after treatment. Tumor volume measurements (VM), acquired from the summation of areas from the axial magnetic resonance images, were used as the reference standard. RESULTS: There was a significant association between VM and BDM with respect to tumor size at diagnosis (p = 0.002), absolute change in tumor size after treatment (p < 0.001), and percentage change in tumor size after treatment (p = 0.044), but not between VM and UDM. There was also a significant association between VM and DM with respect to percentage change in tumor size after treatment (p = <0.0001) but not absolute change (p = 0.222). CONCLUSION: When using simple measurements to assess irregularly shaped nasopharyngeal cancers, the BDM should be used to measure size at diagnosis and the BDM and percentage change in size with treatment. Unidimensional measurement does not reflect size or change in size, and therefore the RECIST criteria may not be applicable to all tumor shapes. The use of DM requires further evaluation.

Intracranial aneurysms treated with Guglielmi detachable coils: midterm clinical and radiological outcome in 97 consecutive Chinese patients in Hong Kong.

BACKGROUND AND PURPOSE: Use of Guglielmi detachable coils (GDCs) has proved to be a promising endovascular treatment for intracranial aneurysms. This study aimed to evaluate midterm clinical and radiologic outcomes of this treatment in Hong Kong Chinese patients, 68% of whom had small aneurysms (< or =5 mm). METHODS: We included 97 consecutive patients in whom GDCs were placed with curative intent. The patients presented with subarachnoid hemorrhage (n = 80) or mass effect (n = 17). The aneurysms measured 5 cm +/- 2.8 mm; 68% were < or =5 mm. All patients were followed up clinically for an average of 54.5 +/- 20.9 months and radiologically with sequential digital subtraction angiography at 6 and 18 months. RESULTS: Total occlusion of the aneurysm was successfully achieved in 71.1% of patients after the initial treatment and in 82.5% after subsequent treatments. The retreatment rate was 17.5%. Procedure-related complication and mortality rates were 11.3% and 0%, respectively. The overall mortality was 5%, including mortality due to treatment failure in 1%. Neurologic outcomes were excellent in 77% of patients. Improved neurologic status, unchanged status, and deteriorated status was noted in 61.5%, 22%, and 16.5% of patients, respectively, at the end of the follow-up period. Intrinsic differences existed between Chinese and Western patients regarding the size of the aneurysm at presentation, periprocedural complications, and progression patterns of anatomic outcomes. CONCLUSION: Endovascular coiling with GDCs is a reasonably effective and safe treatment for intracranial aneurysms in this group of Hong Kong Chinese patients, with favorable clinical and radiologic outcomes.