Four-dimensional computed tomography as first-line imaging in primary hyperparathyroidism, a retrospective comparison to conventional imaging in a predominantly single adenoma population.

BACKGROUND: To determine the use of four-dimensional CT as first-line imaging compared to the traditional combination of ultrasound and [99mTc]Tc-Sestamibi SPECT. MATERIALS AND METHODS: Retrospective review of preoperative imaging in patients with primary hyperparathyroidism, who underwent parathyroidectomy between 2012 and 2021. In one group, the combination ultrasound and [99mTc]Tc-Sestamibi SPECT was used as first-line imaging (n = 54), in the other group four-dimensional CT was the first-line imaging modality (n = 51). Sensitivity and positive predictive value were calculated on patient, lateralisation and localisation level. The need for additional imaging was also assessed for both groups. RESULTS: Four-dimensional CT had a significantly higher sensitivity compared to the combination of ultrasound/[99mTc]Tc-Sestamibi SPECT on patient and localisation level (70.6% vs. 51.9%, p = 0.049 and 60.8% vs. 35.2%, p = 0.009 respectively). Sensitivity for lateralisation also appeared higher, but did not reach significance (62.7% vs. 44.4%, p = 0.060). Positive predictive value was not significantly higher for four-dimensional CT compared to ultrasound and [99mTc]Tc-Sestamibi SPECT (88.9% vs. 85.7% for lateralisation and 86.1% vs. 67.9% for localisation respectively). Additional imaging was required in 14 patients with four-dimensional CT as first-line imaging (27.4%) consisting of 2 ultrasound/[99mTc]Tc-Sestamibi SPECT and 13 [18F]fluorocholine PET/CT, compared to 24 patients with ultrasound/[99mTc]Tc-Sestamibi SPECT as first-line imaging (44.4%), requiring 22 four-dimensional CT and 9 [18F]fluorocholine PET/CT. CONCLUSIONS: Four-dimensional CT as the sole first-line parathyroid imaging modality had higher sensitivity than the combination of ultrasound and [99mTc]Tc-Sestamibi SPECT, therefore requiring fewer additional procedures. Although the most costly, [18F]fluorocholine PET/CT was the most effective technique to localise parathyroid adenoma in case all other imaging was negative.

Preoperative imaging in primary hyperparathyroidism patients using 4DCT subtraction maps, a report of three cases.

Four-dimensional computed tomography (4DCT) is one of the preoperative imaging modalities that can be used to localize a parathyroid adenoma in primary hyperparathyroidism patients however, sensitivity differs in literature and could be improved especially for multiglandular hyperplasia or double adenomas. The most robust feature on the 4DCT for the differentiation between parathyroid adenoma and thyroid gland tissue is arterial enhancement. To make this better visible, we have developed a subtraction map that shows arterial enhancement as a color scale to increase sensitivity for 4DCT. In this report of 3 cases, we present the usefulness of this subtraction map in a 54-year-old male, a 57-year-old female and a 51-year-old male. Subtraction maps may increase sensitivity for 4DCT, especially for multiglandular hyperplasia or double adenomas.

Phenotype description of a Dutch otosclerosis family with suggestive linkage to OTSC7.

This study reports on a clinical investigation of a Dutch family that shows suggestive linkage to OTSC7. Cross-sectional as well as longitudinal analyses of audiometric data were performed. Also, high-resolution computed tomography (CT) images of the temporal bones from genetically affected family members were obtained to study the incidence and extent of otospongiotic foci. Audiometric data showed a considerable degree of phenotypic variability. Cross-sectional regression analysis did not show age-dependent progression of bone conduction (BC), air conduction (AC), and air-bone gap (ABG) levels. Longitudinal analysis of audiometric follow-up data of one family member showed age-dependent progression of AC, BC, and ABG levels. High-resolution CT images revealed an otospongiotic focus in six of six (100%) clinically affected individuals that carried the disease haplotype. In none of the clinically unaffected family members that showed linkage to OTSC7, an otospongiotic focus was detected by CT. In conclusion, hearing impairment in the present otosclerosis family seems to be variable in terms of onset age and level of progression. Long-term audiometric data of one patient proved to be valuable in understanding progression of hearing impairment in this individual. The detection rate of otospongiotic foci in our study group is similar compared to previous reports on CT data in consecutive otosclerosis patients who had stapes replacing surgery.

The phenotype of the first otosclerosis family linked to OTSC5.

OBJECTIVE: To report the audiometric and radiologic findings in the first otosclerosis family linked to OTSC5. STUDY DESIGN: A clinical investigation of a family linked to OTSC5, including analyses of audiometric data and of high-resolution computed tomography (CT) images of the temporal bones from genetically affected family members. SETTING: Tertiary referral center. PATIENTS: Family members from a four-generation pedigree with otosclerosis segregating as an autosomal dominant trait. MAIN OUTCOME MEASURE(S): Pre-surgery pure tone audiometric data. Classification of otosclerotic foci on high-resolution spiral CT images of the temporal bones of genetically affected individuals. RESULTS: Audiometric data showed a considerable degree of phenotypic variability. Cross-sectional regression analysis did not disclose any clear age dependence of threshold-related data. Systematic differences between mean parameter values relating to the thresholds in the best or the worst ear were found. High-resolution CT images revealed a fenestral otosclerotic focus in seven of nine (78%) clinically affected individuals and cochlear foci in one of these seven patients. CONCLUSION: The phenotype of OTSC5 seems to be variable. Additional long-term audiometric data are needed to construct age-related typical audiograms, which may also facilitate the comparison between phenotypes of the different otosclerosis loci. The detection rate of otospongiotic foci in our study group is similar or lower compared with previous reports on CT data in consecutive otosclerosis patients who had stapes replacing surgery.

Lack of efficacy of two consecutive treatments of radioimmunotherapy with 131I-cG250 in patients with metastasized clear cell renal cell carcinoma.

PURPOSE: A previous activity dose-escalation study using 131I-labeled chimeric monoclonal antibody cG250 in patients with progressive metastatic renal cell carcinoma (RCC) resulted in occasional therapeutic responses. The present study was designed to determine the safety and therapeutic efficacy of two sequential high-dose treatments with 131I-cG250. PATIENTS AND METHODS: Patients (n = 29) with progressive metastatic RCC received a low dose of (131)I-cG250 for assessment of preferential targeting of metastatic lesions, followed by the first radioimmunotherapy (RIT) with 2220 MBq/m2 131I-cG250 (n = 27) 1 week later. If no grade 4 hematologic toxicity was observed, a second low-dose 131I-cG250 (n = 20) was given 3 months later. When blood clearance was not accelerated, a second RIT of 131I-cG250 was administered at an activity-dose of 1110 MBq/m2 (n = 3) or 1665 MBq/m2 (n = 16). Patients were monitored weekly for toxicity, and tumor size was evaluated by computed tomography once every 3 months intervals. RESULTS: The maximum-tolerated dose (MTD) of the second RIT was 1,665 MBq/m2 because of dose-limiting hematological toxicity. Based on an intention-to-treat analysis, after two RIT treatments, the disease stabilized in five of 29 patients, whereas it remained progressive in 14 of 29 patients. Two patients received no RIT, and eight of 29 received only one 131I-cG250 RIT because of grade 4 hematologic toxicity, formation of human antichimeric antibodies, or disease progression. CONCLUSION: In patients with progressive end-stage RCC, the MTD of the second treatment was 75% of the MTD of the first RIT. In the majority of patients, two cycles of 131I-cG250 could be safely administered without severe toxicity. No objective responses were observed, but occasionally two RIT doses resulted in stabilization of previously progressive disease.

Evidence of progression and fluctuation of hearing impairment in branchio-oto-renal syndrome.

We retrospectively analysed long-term serial audiometry data from patients with branchio-oto-renal (BOR) syndrome to show the features of progression and fluctuation in hearing impairment and relate the findings to age and magnetic resonance imaging (MRI) findings in the petrosal bones. Thirty-two clinically affected BOR patients from six Dutch families (A-F) were included. Audiograms were available in 24 cases, covering followup intervals of between 3 and 30 years, and suitable for individual statistical analysis in 16 cases: 14 cases also had MRI findings. Significant progression in hearing impairment was found in 10 cases, while findings of significant fluctuation were made in seven cases. These findings did not clearly correlate with MRI findings. Substantial fluctuation occurred only in cases followed at a relatively young age. Patients with an enlarged endolymphatic duct and/or sac showed significantly higher sensorineural hearing thresholds than those with either normal MRI findings or cochlear/labyrinthine hypoplasia with or without enlarged duct or sac. We conclude that progressive, fluctuant hearing loss occurred in some BOR patients; however, only young patients showed substantial threshold fluctuation. BOR patients with an enlarged endolymphatic duct and/or sac on MRI seemed to be predisposed to developing more severe hearing impairment.

Evaluation of the effects of primary myotomy in total laryngectomy on the neoglottis with the use of quantitative videofluoroscopy.

OBJECTIVE: To evaluate the influence of primary myotomy on characteristics of the neoglottis in patients after laryngectomy. DESIGN: Patient survey. SETTING: University Medical Center St Radboud, Nijmegen, the Netherlands. PATIENTS: Nineteen consecutive patients who underwent laryngectomy (12 with primary lateral myotomy of the upper esophageal sphincter [marked by metal clips]; 7 not requiring myotomy [according to intraoperative palpation]). INTERVENTIONS: Videofluoroscopy. MAIN OUTCOME MEASURES: Visual assessments and quantitative measures of the neoglottis were used to study the relationships between myotomy, and anatomic and morphologic characteristics of the neoglottis. RESULTS: Quantitative measurements showed no difference between the neoglottic characteristics of the patients with (n = 12) and without (n = 7) myotomy, who were all judged to have moderate (n = 4) or good (n = 15) voice quality. Results for the entire patient group during phonation showed only 1 single neoglottic bar, no hypertonicity of the neoglottis, and a significant shortening of the neoglottic bar (P =.007). Results for the myotomy group during phonation showed elevation of the caudal clip (P =.046), shortening of the myotomy (P =.01), and decreased overlap of the cranial clip and the neoglottic bar (P =.007). Furthermore, significant relationships were found between the various quantitative measures of the neoglottis and those of the myotomy. CONCLUSIONS: Quantitative videofluoroscopy enables study of the influence of myotomy on the anatomic and morphologic characteristics of the neoglottis. Our results suggest that a planned myotomy of the upper esophageal sphincter is beneficial when prosthetic voice rehabilitation is applied after total laryngectomy.

Screening for second primary lung cancer after treatment of laryngeal cancer.

OBJECTIVES/HYPOTHESIS: As a result of smoking, patients who have received curative treatment for laryngeal cancer run a high risk of developing lung cancer. Therefore, these patients enter a screening program that aims to detect lung cancer at an asymptomatic stage. The study evaluated whether screening for lung cancer by means of regular chest x-ray examinations contributed to prolonging survival. STUDY DESIGN: A longitudinal follow-up study was performed to analyze the survival of patients who had received curative treatment for squamous cell laryngeal cancer and developed lung cancer during the follow-up period. METHODS: Patients with lung cancer were divided into two groups: 1) patients with asymptomatic screen-detected lung cancer and 2) patients with complaints indicating lung cancer, whose tumor was detected in the interval between screening examinations by chest x-ray films. RESULTS: In the complete group of patients with laryngeal cancer, no prognostic factors could be identified for developing lung cancer. There was no prolongation of survival in the screen-detected asymptomatic lung cancer patients. The median survival of both groups was 56 months (P =.57). The date of detection of the lung cancer was clearly brought forward by screening; a difference of 8 months was found between the median detection date of the two groups (P <.001). There was no difference in tumor-specific mortality between the two groups. CONCLUSION: Screening by chest x-ray examination to detect lung cancer in an asymptomatic stage after curative treatment for squamous cell laryngeal cancer does not improve survival for patients who develop lung cancer.

Inner ear anomalies are frequent but nonobligatory features of the branchio-oto-renal syndrome.

OBJECTIVE: To summarize the syndromic features and evaluate the presence of inner ear anomalies in 35 patients with branchio-oto-renal (BOR) syndrome from 6 families. DESIGN: Retrospective evaluation of magnetic resonance imaging of the temporal bones and clinical features in patients with BOR syndrome. SETTING: Tertiary referral center. PATIENTS: The study population comprised 35 clinically affected patients with BOR syndrome from 6 families. Most of these families were followed for over 25 years. MAIN OUTCOME MEASURES: Twenty-four patients underwent high-resolution, heavily T2-weighted 3-dimensional magnetic resonance imaging of the temporal bones for evaluation of inner ear anomalies. Special attention was paid to the endolymphatic duct and sac. RESULTS: A total of 7 enlarged endolymphatic ducts and sacs (3 bilaterally and 4 unilaterally) and 5 enlarged endolymphatic ducts only (2 bilaterally and 3 unilaterally) were observed. Eight hypoplastic cochleas and 6 hypoplastic labyrinths were seen bilaterally. Seven family members had normal inner ears. CONCLUSION: These findings suggest that inner ear anomalies are frequent but nonobligatory features of BOR syndrome.

Effects of breathing a hyperoxic hypercapnic gas mixture on blood oxygenation and vascularity of head-and-neck tumors as measured by magnetic resonance imaging.

PURPOSE: For head-and-neck tumors, breathing a hyperoxic hypercapnic gas mixture and administration of nicotinamide has been shown to result in a significantly improved tumor response to accelerated radiotherapy (ARCON, Accelerated Radiotherapy with CarbOgen and Nicotinamide). This may be caused by improved tumor oxygenation, possibly mediated by vascular effects. In this study, both blood oxygenation and vascular effects of breathing a hyperoxic hypercapnic gas mixture (98% O2 + 2% CO2) were assessed by magnetic resonance imaging (MRI) in patients with head-and-neck tumors. METHODS AND MATERIALS: Tumor vascularity and oxygenation were investigated by dynamic gadolinium contrast-enhanced MRI and blood oxygen level dependent (BOLD) MRI, respectively. Eleven patients with primary head-and-neck tumors were each measured twice; with and without breathing the hyperoxic hypercapnic gas mixture. RESULTS: BOLD MR imaging revealed a significant increase of the MRI time constant of transverse magnetization decay (T2*) in the tumor during hypercapnic hyperoxygenation, which correlates to a decrease of the deoxyhemoglobin concentration. No changes in overall tumor vascularity were observed, as measured by the gadolinium contrast uptake rate in the tumor. CONCLUSION: Breathing a hyperoxic hypercapnic gas mixture improves tumor blood oxygenation in patients with head-and-neck tumors, which may contribute to the success of the ARCON therapy.