Radiofrequency ablation for autonomously functioning nodules as treatment for hyperthyroidism: subgroup analysis of toxic adenoma and multinodular goitre and predictors for treatment success.

PURPOSE: Treatment of hyperthyroidism caused by autonomously functioning thyroid nodules (AFTN) with 131I often leads to undesirable hypothyroidism. Radiofrequency ablation (RFA) has emerged as a promising alternative. This retrospective analysis aimed to examine the efficacy of, and postprocedural hypothyroidism after, RFA for AFTN. METHODS: Patients with hyperthyroidism caused by AFTN and treated with RFA were included if follow-up of at least 1 year was available. Cure was defined as thyroid medication-free biochemical euthyroidism. To predict cure, patient and treatment factors were analysed. A distinction was made between solitary toxic adenoma (STA) and toxic multinodular goitre (TMG). RESULTS: Forty-eight patients (36 STA, 12 TMG) were included. One year post-RFA cure rate was 72% in STA versus 25% in TMG (p = 0.004). One patient developed hypothyroidism. In 11 patients that remained hyperthyroid, a second RFA was successful in 83% of STA and 40% of TMG patients. At last available follow-up, this amounted to a total cure rate of 81% in STA and 33% in TMG (p = 0.002). In STA, cured patients had a higher baseline TSH and a lower FT3 than non-cured patients (p = 0.026 and 0.031). Cure was observed in 91% of patients when > 2.1 kJ/mL was delivered during RFA, compared to 44% when less energy was administered. CONCLUSION: The efficacy of RFA was nearly 3 times higher in STA patients compared to TMG. Severity of hyperthyroidism and kJ/mL delivered during RFA predicts cure. Direct comparison to the current standard of care is needed to implement RFA in treatment of hyperthyroidism caused by AFTN.

Radiofrequency ablation for symptomatic, non-functioning, thyroid nodules: a single-center learning curve.

Objective: Radiofrequency ablation (RFA) is increasingly considered the prime option for treating symptomatic, benign, non-functioning thyroid nodules (NFTN). However, little is known about the degree of operator experience required to achieve optimal results. This study describes the RFA learning curve of a single-center team. Methods: A retrospective cohort study of the first 103 patients receiving RFA treatment for a single, symptomatic, and benign NFTN, with a follow-up of at least 1 year. The primary outcome measure was technique efficacy, defined as the percentage of patients with a 6-month nodal volume reduction ratio (VRR) >50% after single-session RFA. Optimal treatment efficacy was defined as a 6-month VRR >50% achieved in at least 75% of patients. Secondary outcomes were complications of RFA and indications of secondary interventions. Results: Median nodal volume at baseline was 12.0 mL (range 2.0-58.0 mL). A 6-month VRR >50% was achieved in 45% of the first 20 patients, 75% of the next 20, and 79% of the following 63 patients. Complications included minor bleeding (N = 4), transient hyperthyroidism (N = 4), and transient loss of voice (N = 1). Poor volume reduction or nodular regrowth led to diagnostic lobectomy in 11 patients and a second RFA in 5. Lobectomy revealed a follicular carcinoma (T2N0M0) in 2 patients. In 1 patient, nodule regrowth was caused by an intranodular solitary B-cell lymphoma. Conclusion: About 40 procedures are required to achieve a 6-month VRR >50% in the majority of patients. Appropriate follow-up with re-evaluation is recommended for all patients with a VRR <50% and in those with regrowth to exclude underlying malignancy.

Prediction of total body electrical resistance normal values based on limb muscle thickness assessed by ultrasound.

BACKGROUND: Recently, a new model has been proposed to assess hydration in patients by measurement of total body electrical resistance (TBER), with results expressed in ohm rather than in liter body water. According to this approach, hydration is considered to be normal if TBER is within the normal range. As TBER is inversely related to the size of the limb muscle compartment, this relationship can be used to calculate the patient-specific TBER normal value (TBERnorm). The present study investigates whether the prediction of TBERnorm can be improved by the use of ultrasound (US) instead of anthropometrically derived parameters of limb muscularity. METHODS: In total, 129 healthy subjects (60 men and 69 women) ranging in age from 18 to 75 yr, and in BMI from 17.4 to 52.0 kg/m2 were included in the study. Arm muscle cross-sectional area assessed by anthropometry (AMAcaliper) was compared with mean muscle thickness (MMT) of arm and leg assessed by B-mode US. RESULTS: MMT correlated stronger with TBER than AMA, and reduced the standard error of the estimate (SEE) by 15% in men and by 26% in women. Muscularity was overestimated by AMAcaliper due to a systematic error directly proportional to subcutaneous fat layer thickness. The gender independent relation between MMT and TBERnorm is described by the equation: TBERnorm = 705-75.4⋅MMT (R2 = 0.85, SEE = 22.3 Ω/m, P < 0.001). CONCLUSIONS: US-based measurement of limb muscularity provides a more precise prediction of TBERnorm, in particular in obese subjects, and is recommended as the method of choice.

Hyperactive thyroid nodules treated by radiofrequency ablation: a Dutch single-centre experience.

BACKGROUND: Hyperactive thyroid nodules (HTN) are usually treated with radioactive iodine (RAI). However, as RAI is associated with a 30-60% long-term risk of permanent hypothyroidism, radiofrequency ablation (RFA) may be a good alternative. Primary aim of this study was to assess the percentage of patients achieving euthyroidism after RFA. PATIENTS AND METHODS: Patients with a symptomatic HTN were treated by ultrasound-guided RFA, using the trans-isthmic approach and moving-shot technique, in an outpatient setting under local anaesthesia. RESULTS: Twenty-one patients were included, ranging in age from 37-75 years. Follow-up was at least one year. All patients had a suppressed serum thyroid-stimulating hormone (TSH), with free thyroxine (FT4) and free triiodothyronine (FT3) concentrations mildly elevated in 33% and 43% of cases, respectively. RFA was not associated with clinically meaningful adverse effects. TSH normalisation was achieved in 11/21 patients (52%) after first RFA. A partial response, defined as a normalisation of FT4 and FT3, but incomplete improvement of TSH, was observed in 6/21 patients (29%). Three patients had no response (14%), and one patient developed mild, asymptomatic subclinical hypothyroidism. Five patients underwent a second RFA and this led to TSH normalisation in four, thereby raising the rate of complete remission to 71%. Recurrence of TSH suppression did not occur during the study period. CONCLUSION: These data suggest that RFA is a safe and promising treatment for symptomatic hyperactive thyroid nodules, with a low risk of permanent hypothyroidism. Long-term studies are needed to identify the recurrence risk of hyperthyroidism.

Contrast Enhanced Abdominal Ultrasound in the Assessment of Ileal Inflammation in Crohn's Disease: A Comparison with MR Enterography.

BACKGROUND AND AIMS: To prospectively examine the feasibility and accuracy of Contrast Enhanced Ultrasound (CEUS) in the assessment of Crohn's disease (CD) activity in the terminal ileum in comparison to Magnetic Resonance Enterography (MRE), using endoscopy as a reference standard. METHODS: 105 consecutive patients with alleged clinically active CD were assessed by MRE and CEUS. CEUS of the terminal ileum was performed using an intravenous microbubble contrast enhancer. Accuracy values of CEUS and MRE for the presence of active terminal ileitis were evaluated using the Receiver Operating Characteristic method, using endoscopic findings as a reference standard. Sensitivity and specificity values of MRE and CEUS were compared by the McNemar test. RESULTS: CEUS was feasible in 98% of patients, MRE in all. Optimal diagnostic accuracy in CEUS was obtained at a peak intensity value of 10%, showing 100% sensitivity, 92% specificity and an accuracy of 99% in demonstrating ileal mucosal inflammation. For MRE, overall sensitivity, specificity and accuracy were, 87%, 100%, and 88%, respectively. CEUS and MRE were highly correlated in assessing length and wall thickness of the terminal ileum. CEUS identified 11 of 16 MRE-detected strictures, but no fistulae. CONCLUSION: The accuracy of CEUS is comparable to that of MRE in the assessment of active, uncomplicated terminal ileal CD and therefore a valuable bedside alternative to MRE in the follow-up of these patients.

Two siblings with progressive, fluctuating hearing loss after head trauma, treated with cochlear implantation.

OBJECTIVE: Description of two siblings with unexplained, progressive, perceptive hearing loss after head trauma. DESIGN: Case report. SUBJECTS: Two siblings aged six and eight years old with bilateral, intermittent but progressive hearing loss. RESULTS: These patients had a c.1172G>A (p.Ser391Asn) mutation in the SLC26A4 gene, which has not previously been reported and which caused Pendred or enlarged vestibular aqueduct syndrome. The diagnosis was based on the perceptive hearing loss, computed tomography findings and mutation analysis. The patients were each fitted with a cochlear implant because of their severe, progressive, perceptive hearing loss with deep fluctuations. The results were good. CONCLUSION: Further testing for the presence of an enlarged vestibular aqueduct is recommended when children present with sudden progression in perceptive hearing loss, whether or not in combination with head trauma. Cochlear implantation is indicated in patients with persistent, progressive hearing loss that leads to deafness. Implantation can be undertaken successfully despite cochlear hypoplasia.

Magnetic resonance imaging in patients with meningitis induced hearing loss.

The aim of this multicentre study was to compare T1 with T2 weighted MRI scans of the labyrinth after meningitis and to investigate whether waiting with scanning improved the reliability of diagnosing an ongoing process such as cochlear osteogenesis. Forty-five patients were included who suffered from meningitis induced hearing loss (radiological imaging <1 year after meningitis). Twenty-one gadolinium enhanced T1 and 45 T2 weighted MRI scans were scored by two radiologists regarding the condition of the labyrinth. These radiological observations were compared with the condition of the cochlea as described during cochlear implantation. A higher percentage of agreement with surgery was found for T2 (both radiologists 73%) than for T1 weighted MRI scans (radiologist 1: 62%, radiologist 2: 67%), but this difference is not significant. There was no significant difference between early (0-3 months) and late (>3 months) scanning, showing that radiological imaging soon after meningitis allows early diagnosis without suffering from a lower agreement with surgical findings.

Right middle lobe torsion: evaluation with CT angiography.

A case of right middle lobe torsion occurring after a right upper lobectomy is described. HR-CT angiography was successful in identifying the obstructed right middle lobe bronchus and the avascular nature of the infected and displaced right middle lobe. This detailed information, obtainable from present day multislice HR-CT scanners with the right CT protocols, seems to diminish the need for further confirmation by other methods.

Dysphagia in facioscapulohumeral muscular dystrophy.

Dysphagia is not considered a symptom of facioscapulohumeral muscular dystrophy (FSHD). In this study, the authors found that dysphagia does occur in patients with advanced FSHD showing mild involvement of the jaw and lingual muscles. Dysphagia is seldom life threatening in these patients. The authors conclude that dysphagia should not be considered an exclusion criterion for FSHD.

A half-yearly chest radiograph for early detection of lung cancer following oral cancer.

In a retrospective analysis of 339 patients, treated with a curative intention for a squamous cell carcinoma of the oral cavity between 1988-1996, the value of a half-yearly routine chest radiograph during the first 2 years of follow-up, was evaluated. Metastatic or primary lung malignancies developed in 18 (5.3%) of the 339 patients in the first 2 years of the follow-up. In 11 (3.2%) patients these malignancies were detected on routine chest radiographs while in 7 (2.0%) patients these were detected on chest radiographs taken because of pulmonary complaints. The mean survival after detection of the pulmonary malignancy was 8 months. All patients died within 21 months, independent of pathology or therapy. No correlation was found between stage, location or differentiation of the primary tumour. As a result of this study it can be concluded that there is no benefit for the patient for a 6-month thoracic radiograph in routine follow-up for patients with oral squamous cell carcinoma. It gives false certainty and burdens the health care system.

Clinical report on the L95P mutation in a Dutch family with paraganglioma.

OBJECTIVE: To describe the new L95P mutation of the paraganglioma 1 gene for glomus tumors in a Dutch paraganglioma 1 family with six affected family members and to report the clinical findings and results of treatment in nine glomus tumors with a maximum follow-up of 34 years. SETTING: Tertiary referral centers. RESULTS: Mutation analysis of the SDHD gene of paraganglioma 1 showed the L95P mutation in six affected family members and two nonaffected carriers protected from becoming affected by genomic imprinting. In six affected family members, nine glomus tumors (five glomus caroticum tumors, two glomus vagale tumors, and two glomus jugulare tumors) were traced. The ages at presentation varied from 25 to 61 years. In two of six affected family members with a total of four tumors, all the tumors were traced in the extended family study, using magnetic resonance imaging; at that time these tumors were silent. After radiotherapy in one patient at the age of 34 years, a T4 planocellular carcinoma of the tongue occurred within the previous radiation field 27 years later, when the patient was 61 years old. Volume measurements of three untreated glomus tumors (two glomus vagale tumors, one glomus caroticum tumor) during 25 months showed an increase in two tumors (left glomus caroticum, left glomus vagale tumor) and a decrease in one tumor (right glomus vagale tumor). Surgery to remove two bilateral and one unilateral glomus caroticum tumors was successful. A wait-and-see policy is being applied to two glomus vagale tumors. CONCLUSIONS: In family members of paraganglioma 1 patients, mutation analysis can be used to make an early diagnosis of glomus tumors. Radiotherapy may have induced a carcinoma. Modalities of treatment can include a wait-and-see policy. Long-term follow-up studies on the natural course of glomus tumors are needed to improve decisions about treatment modalities.

Fluctuant, progressive hearing loss associated with Menière like vertigo in three patients with the Pendred syndrome.

OBJECTIVE: To evaluate vestibular and long-term audiometric findings in patients with Pendred syndrome. STUDY DESIGN: Retrospective analysis of long-term clinical data. SETTING: University hospital department. PATIENTS: Three patients with Pendred syndrome caused by a mutation in the SLC26A4 gene. METHODS: Perchlorate discharge test, mutation analysis of the SLC26A4 gene, MR imaging of temporal bones, vestibular function test (in two cases) and serial audiometry. A saturation hyperbola with onset age was fitted to the audiometric threshold-on-age data using a nonlinear regression method. The residues remaining after regression were analyzed in a correlation analysis to detect significant ipsilateral or contralateral cofluctuation. RESULTS: All three patients had a mutation in the SLC26A4 gene and bilateral enlarged vestibular aqueduct; two of them had a positive perchlorate discharge test but in one of two siblings this test was negative. Hearing loss was significantly progressive with significant ipsilateral and contralateral cofluctuation in all evaluable cases, combined with episodes of Menière like vertigo in two cases. The episodes of vertigo are as seen in Menière disease. One case had unilateral caloric areflexia and one had bilateral vestibular hyporeflexia, proven to be progressive in a repeat examination. CONCLUSIONS: Patients with Pendred syndrome may exhibit progressive and fluctuant hearing loss with episodes of vertigo.

Method for quantitative mapping of dynamic MRI contrast agent uptake in human tumors.

A method is presented for the acquisition and analysis of dynamic contrast-enhanced (DCE) MRI data, focused on the characterization of tumors in humans. Gadolinium (Gd) contrast was administered by bolus injection, and its effect was monitored in time by fast T1-weighted MRI. A simple algorithm was developed for automatic extraction of the arterial input function (AIF) from the DCE-MRI data. This AIF was used in the pixelwise pharmacokinetic determination of physiological vascular parameters in normal and tumor tissue. Maps were reconstructed to show the spatial distribution of parameter values. To test the reproducibility of the method 11 patients with different types of tumors were measured twice, and the rate of contrast agent uptake in the tumor was calculated. The results show that normalizing the DCE-MRI data using individual coregistered AIFs, instead of one common AIF for all patients, substantially reduces the variation between successive measurements. It is concluded that the proposed method enables the reproducible assessment of contrast agent uptake rates.

Progressive fluctuant hearing loss, enlarged vestibular aqueduct, and cochlear hypoplasia in branchio-oto-renal syndrome.

OBJECTIVE: To study the results of petrosal bone imaging and audiometric long-term follow-up of two patients with branchio-oto-renal (BOR) syndrome and relate them to the clinical features, including caloric responses. STUDY DESIGN: Longitudinal case study. SETTING: Tertiary referral center. PATIENTS: A father and son with the BOR syndrome. MAIN OUTCOME MEASURES: Both patients underwent imaging studies to detect and evaluate inner ear anomalies. Longitudinal audiometric analysis of the hearing threshold data over the previous 23 years was performed. Caloric tests were performed at various ages. RESULTS: The son had a short, wide internal acoustic canal, a hypoplastic cochlea, a plump vestibule, and a wide vestibular aqueduct on both sides; the semicircular canals and endolymphatic sac were of normal size. He showed progressive fluctuant sensorineural hearing loss. Caloric tests disclosed hyporeflexia on the left side. The father had a plump internal acoustic canal and hypoplastic cochlea on both sides. The left vestibule was hypoplastic, and the left vestibular aqueduct was marginally enlarged. He showed severe hearing impairment, without substantial progression or fluctuation, and caloric areflexia on the left side. CONCLUSION: These findings suggest a correlation between progressive fluctuant sensorineural hearing loss with caloric hypofunction and the presence of an enlarged vestibular aqueduct in the BOR syndrome. Additional longitudinal case studies are needed to further evaluate such a correlation.