Neurectomy of the Masseteric Nerve Using an Extra-Oral Approach to Treat Masseter Hypertrophy: Case Report and Literature Review.

Masticatory muscle hypertrophy is a benign clinical anomaly which leads to facial asymmetry or a squared face appearance. We report a case of masticatory muscle hypertrophy, particularly on the right side, that was successfully treated by neurectomy of the right masseteric nerve through an extra-oral approach. Clinical examination showed significant aesthetic improvement of the facial symmetry with complete paralysis and atrophy of the right masseter muscle. The impaired postoperative function of the frontal branch of the right facial nerve was fully restored 10 weeks postoperatively. The patient no longer experiences headaches or discomfort with eating or sleeping on her right side.

Ultrasound-guided core needle biopsy and incisional biopsy of the parotid gland are comparable in diagnosis of primary Sjögren's syndrome.

OBJECTIVES: Salivary gland lymphocytic infiltrates are a hallmark of primary SS (pSS), but traditional biopsy techniques hold several disadvantages. Ultrasound-guided core needle (US-guided CN) parotid gland biopsy is minimally invasive and reliable for diagnosis of lymphoma in pSS. This proof-of-concept study aimed to explore this technique in the diagnostic work-up of pSS and is the first to address its value in a consecutive cohort independently of the presence of salivary gland swelling. METHODS: Combined incisional and US-guided CN parotid biopsy was performed in 20 patients with suspected or confirmed pSS from the Belgian Sjögren's Syndrome Transition Trial (BeSSTT). Surface area and presence of a focus score (FS) of at least one, germinal centres and lymphoepithelial lesions were recorded. RESULTS: Salivary gland tissue was interpretable in 19 patients. Fourteen patients had ≥4 mm2 salivary gland tissue by both techniques, in four US-guided CN biopsies salivary gland tissue was <4 mm2. Paired biopsies ≥4 mm2 displayed a concordance of 90% for FS ≥ 1. Presence of lymphoepithelial lesions and germinal centres showed absolute concordance. Of four US-guided CN biopsies <4 mm2, three interpretable incisional biopsies were available, 2/3 with perfect concordance. When including biopsies of <4 mm2 salivary gland tissue, presence of FS ≥ 1 or germinal centres gave a sensitivity of 70% in incisional and of 69% in US-guided CN biopsy. CONCLUSIONS: US-guided CN biopsy of the parotid gland is at least equivalent to incisional biopsy of the parotid gland in the diagnostic work-up of pSS.

The value of separate detection of anti-Ro52, anti-Ro60 and anti-SSB/La reactivities in relation to diagnosis and phenotypes in primary Sjögren's syndrome.

OBJECTIVES: Autoantibody detection is an essential step in pSS diagnosis. However, the value of separate anti-Ro52, anti-Ro60 and anti-SSB/La detection in pSS diagnosis and phenotyping has not been extensively studied. This study aimed to explore disease characteristics of anti-SSA/Ro positive, suspected and definite pSS patients, in relation to serological profiles based on anti-Ro52, anti-Ro60 and anti-SSB/La reactivity. METHODS: Of 187 anti-SSA/Ro positive patients included in the Belgian Sjögren's Syndrome Transition Trial (BeSSTT), 155 were considered definite pSS patients, due to fulfilment of the 2016 ACR-EULAR classification criteria, and 32 suspected, due to reactivity against SSA/Ro without presence of other criteria. None of the patients met any of the ACR-EULAR exclusion criteria for pSS. Patients were grouped based on the presence of anti-Ro52, anti-Ro60 and anti-SSB/La antibodies. RESULTS: Mono-reactivity against Ro60 or Ro52, double reactivity against Ro52/Ro60 and triple reactivity against Ro52/Ro60 and SSB was detected in respectively 30, 23, 70 and 60 patients. Mono-anti-Ro60 positive patients showed the least pSS features. Mono-anti-Ro52 positive patients reported a significantly higher dryness burden (p=0.016) and tended toward more salivary gland ultrasound (SGUS) abnormalities (p=0.054) than mono-anti-Ro60 positives. Double positive patients showed similar characteristics as mono-anti-Ro52 positive patients, whereas triple positive patients showed lowest unstimulated salivary flow rates (p=0.002) and Schirmer tests (p=0.002), highest ocular staining scores (p<0.001), most positive labial salivary gland biopsies (p=0.039), most laboratory abnormalities compatible with B-cell hyperactivity and highest SGUS scores (p<0.001) compared to other patient groups. CONCLUSIONS: These data indicate that separate detection of anti-Ro52, anti-Ro60 and anti-SSB/La reactivity is not only relevant towards pSS diagnosis, but markedly aids in patient stratification and evaluation of disease burden. Our results suggest a stepwise model in which mono-reactivity against Ro60 displayed the least objective and subjective glandular pSS features, whereas glandular abnormalities and signs of B-cell hyperactivity were most present in patients showing triple reactivity against Ro60, Ro52 and SSB/La.

Discriminative power of salivary gland ultrasound in relation to symptom-based endotypes in suspected and definite primary Sjögren's Syndrome.

OBJECTIVES: Salivary gland ultrasound (SGUS) is emerging as essential tool in primary Sjögren's Syndrome (pSS), but its link to symptom-based endotypes is unknown. Therefore, we explored SGUS outcomes in relation to endotypes in patients with definite and suspected pSS. METHODS: Definite pSS patients (n = 171) fulfilling the 2016 ACR/EULAR classification criteria, and suspected pSS patients (n = 119), positive for at least one criterion, were included in the Belgian Sjögren's Syndrome Transition Trial (BeSSTT). Stratification into endotypes according to the Newcastle Sjögren's Stratification Tool resulted in low symptom burden (LSB), pain dominant with fatigue (PDF), dryness dominant with fatigue (DDF) and high symptom burden (HSB). SGUS was assessed with Hocevar score (0-48). The dataset was randomly divided into a discovery (n = 203) and replication (n = 87) cohort. RESULTS: SGUS had strong discriminative power for pSS classification (AUC=0.74), especially in DDF (AUC=0.89). In definite pSS, Hocevar scores in DDF were high compared to other endotypes (38 (20-44) versus 18 (9-33); p < 0.001). Patients with highest SGUS-scores showed more sicca and laboratory abnormalities. Moreover, a subset of young, anti-SSA/Ro positive patients not fulfilling classification criteria showed clear SGUS abnormalities. Replication showed similar results. CONCLUSIONS: SGUS-scores were significantly higher in definite pSS with DDF endotype, providing the first evidence of imaging abnormalities in salivary glands matching distinct biological profiles ascribed to pSS endotypes. Additionally, a subset of patients with potential early disease was detected based on presence of anti-SSA antibodies and high SGUS-scores. These results underscore the role of SGUS as powerful tool both in pSS classification and stratification.

OAB supraspinal control network, transition with age, and effect of treatment: A systematic review.

OBJECTIVE: In light of a better understanding of supraspinal control of nonneurogenic overactive bladder (OAB), the prevalence of which increases with age, functional imaging has gained significant momentum. The objective of this study was to perform a systematic review on the transition of supraspinal control of OAB with age, the effect of therapeutic modalities, and a coordinate-based meta-analysis of all neuroimaging evidence on supraspinal OAB control in response to bladder filling. METHODOLOGY: We performed a systematic literature search of all relevant libraries in November 2021. The coordinates of brain activity were extracted from eligible neuroimaging studies to perform an activation likelihood estimation (ALE) meta-analysis. RESULTS: A total of 16 studies out of 241 were selected for our systematic review. Coordinates were extracted from five experiments involving 70 patients. ALE meta-analysis showed activation of the insula, supplementary motor area, dorsolateral prefrontal cortex, anterior cingulate gyrus, and temporal gyrus with a transition of activation patterns with age, using a threshold of uncorrected p < 0.001. Among young patients, neuroplasticity allows the activation of accessory circuits to maintain continence, as in the cerebellum and temporoparietal lobes. Anticholinergics, pelvic floor muscle training, sacral neuromodulation, and hypnotherapy are correlated with supraspinal changes attributed to adaptability and possibly a substratum of an intrinsic supraspinal component. The latter is better demonstrated by a resting-state functional connectivity analysis, a promising tool to phenotype OAB with recent successful models of predicting severity and response to behavioral treatments. CONCLUSION: Future neuroimaging studies are necessary to better define an OAB neurosignature to allocate patients to successful treatments.

Congenital bilateral dacryocystocoele: A neonatal emergency.

INTRODUCTION AND IMPORTANCE: Bilateral congenital dacryocystocoele with intranasal extension is very rare and may lead to mild to severe respiratory distress, depending on the degree of obstruction, in an otherwise healthy newborn. If severe, an urgent surgical intervention may be life saving. Our aim is to alert paediatric surgeons to this rare condition. Early detection and early treatment with a minimally invasive surgical procedure may be life saving and prevent severe sequelae due to respiratory distress. CASE PRESENTATION: We present a healthy newborn girl who was admitted to neonatal intensive care with progressive respiratory distress. After a full work-up, she was diagnosed with bilateral dacryocystocoele with intranasal extension and complete obstruction of the anterior nasal cavity. Emergency bilateral endoscopic marsupialization of the cysts with probing of the nasolacrimal duct was performed. The girl recovered without sequelae. CLINICAL DISCUSSION: In congenital dacryocystocoele, coexistent obstruction of the valve of Rosenmüller and the valve of Hasner is observed. Subsequent intranasal protrusion with obstruction of the inferior nasal cavity may occur. The incidence is unknown, but if bilateral, it is very rare. In most cases, a dacryocystocoele is uncomplicated and may be treated conservatively. However, if intranasal protrusion occurs, and especially if the involvement is bilateral, it is a surgical emergency. CONCLUSION: Early diagnosis with subsequent minimally invasive surgical treatment of bilateral congenital dacryocystocoele with intranasal protrusion may prevent serious complications due to respiratory distress.

Idiopathic spontaneous sublingual hematoma: a case report and literature review.

PURPOSE: Sublingual hematoma is a rare complication seen in bleeding disorders, trauma, dental procedures, and the use of anticoagulants. Only a few publications have described idiopathic spontaneous sublingual hematoma, and all of them suspected severe hypertension as etiology. Our objective is to present a case of idiopathic spontaneous sublingual hematoma in a 48-year-old patient without any medical history of hypertension and to perform a literature review on this subject. METHODS: Case report presentation based on information extracted from the electronic patient database of Ghent University Hospital including a literature review of sublingual hematoma. RESULTS: This case report describes a 48-year-old male patient presenting with a spontaneous sublingual hematoma, which needed to be treated with an urgent tracheotomy. Drainage of the old hematoma followed 11 days later. He was not known with hypertension or any bleeding disorder. Clinical examination using fiberoptic nasolaryngoscopy, a CT scan, MRI scan, and biopsy of the sublingual salivary gland showed no focus for etiology. CONCLUSIONS: Clinical work-up is necessary to look for a cause of a spontaneous sublingual hematoma. Idiopathic spontaneous hematoma is only a rare entity. Aggressive airway management should be the first step in treatment. To date, there is no consensus about the management regarding the hematoma itself. Mostly, clinicians start with observation for spontaneous resolution of the hematoma and when possible to treat causative factors. Surgical drainage is performed when conservative treatment is not sufficient.

Vestibular schwannoma: natural growth and possible predictive factors.

Background: Prediction of vestibular schwannoma (VS) growth would allow for a more differentiated follow-up protocol. Objectives: The natural course of a VS and predictive factors of growth are investigated. Methods: Sixty-two sporadic VS cases diagnosed between 2003 and 2015 were included in this retrospective cohort study. After initial surveillance, active therapy was initiated in 31/62 patients. Regular magnetic resonance images (MRIs) were performed. Two mm/year linear difference was the cut-off value for significant growth. The STROBE guidelines have been implemented. Results: Growth of the tumor was detected in 56% of patients and mainly observed in the first three years of follow-up. Tumor size remained stable in 34% and decreased in 10% of patients. No baseline information, symptom, or sign was found to be predictive for growth. Conclusions: In 56% of the initially conservatively managed VSs growth was observed and active treatment was initiated. Eighty-seven percent of the growing VSs were identified during the first three years of follow-up. The initially larger VSs seemed to grow faster and needed active treatment earlier during follow-up. No predicting factors for growth identified from the literature could be confirmed in the present study. Serial MRI remains the appropriate method to detect tumor growth.

Hydroxyapatite cranioplasty: a retrospective evaluation of osteointegration in 17 cases.

BACKGROUND: Cranial reconstruction with autologous bone is still the gold standard although several biomaterials are available to re-establish the integrity of the cranial vault. Due to their biological and morphological characteristics, hydroxyapatite implants show promising results in small clinical cohort studies, especially within the paediatric population. Its biocompatibility and osteoconductivity should allow the formation of osseous bridging at the skull-prosthesis interface. OBJECTIVE: To examine the possible occurrence of osteointegration and to quantify it. METHODS: A retrospective study of patients with a hydroxyapatite implant from 2010 to 2014 at our neurosurgical department was conducted. Demographic, surgical and radiological data were studied. A senior neuroradiologist, a staff member neurosurgeon and a resident neurosurgeon independently performed the radiological evaluation. A new software analysis technique was developed to objectively quantify the degree of osteointegration. RESULTS: Seventeen implants were evaluated with an average patient age of 39 years and a mean follow-up of 155 weeks. Through radiologic evaluation, osseous bridging was deemed higher than 50% in six prostheses and higher than 75% in three. In five patients, no osteointegration could be seen. The remaining patients exhibited sparse signs of osteointegration, estimated between 10 and 50%. Software analysis showed an average osteointegration ratio of 37.4% with a 400-HU filter and 27.3% with a 700-HU filter. CONCLUSION: In this small retrospective study of cranial hydroxyapatite implants, osteointegration did occur and to a degree of more than 50% in 1/3 of the patients.

Late mucosal ulcers in dose-escalated adaptive dose-painting treatments for head-and-neck cancer.

BACKGROUND: To identify predictive factors for the development of late grade 4 mucosal ulcers in adaptive dose-escalated treatments for head-and-neck cancer. MATERIAL AND METHODS: Patient data of four dose-escalated three-phase adaptive dose-painting by numbers (DPBN) clinical trials were analyzed in this study. Correlations between the development of late grade 4 ulcers and factors related with the treatment, disease characteristics and the patient were investigated. Dosimetrical thresholds were searched among the highest doses received by 1.75 cm3 (D1.75cc) of the primary gross tumor volume (GTVT) and the corresponding normalized isoeffective dose (NID21.75cc, with a reference dose of 2Gy/fraction and α/ß of 3 Gy). RESULTS: From 39 studied patients, nine developed late grade 4 mucosal ulcers. The continuation to either smoke or drink alcohol after therapy was the factor that showed a strong (eight out of nine patients) association with the occurrence of grade 4 ulcers. Six of the patients who continued to smoke or/and drink had D1.75cc and NID21.75cc above 84 Gy and 95.5 Gy, respectively. Seven of the patients with grade 4 had the dose levels above these thresholds, but even if the D1.75cc threshold was significant in the prediction of late grade 4 ulcers, it could not be considered as the only contributing factor. CONCLUSIONS: The search for patterns provided strong reasons to apply a dosimetrical threshold for the peak-dose volume of 1.75 cm3 as a preventive measure for late grade 4 mucosal ulcers. Also, patients that continue to smoke or drink alcohol after therapy have increased risk to develop late mucosal ulcers.

Omalizumab is effective in allergic and nonallergic patients with nasal polyps and asthma.

BACKGROUND: Adult patients with nasal polyps often have comorbid asthma, adding to the serious effect on the quality of life of these patients. Nasal polyps and asthma might represent a therapeutic challenge; inflammation in both diseases shares many features, such as airway eosinophilia, local IgE formation, and a T(H)2 cytokine profile. Omalizumab is a human anti-IgE mAb with proved efficacy in patients with severe allergic asthma. Omalizumab could be a treatment option for patients with nasal polyps and asthma. OBJECTIVE: The goal of this study was to investigate the clinical efficacy of omalizumab in patients with nasal polyps and comorbid asthma. METHODS: A randomized, double-blind, placebo-controlled study of allergic and nonallergic patients with nasal polyps and comorbid asthma (n = 24) was conducted. Subjects received 4 to 8 (subcutaneous) doses of omalizumab (n = 16) or placebo (n = 8). The primary end point was reduction in total nasal endoscopic polyp scores after 16 weeks. Secondary end points included a change in sinus computed tomographic scans, nasal and asthma symptoms, results of validated questionnaires (Short-Form Health Questionnaire, 31-item Rhinosinusitis Outcome Measuring Instrument, and Asthma Quality of Life Questionnaire), and serum/nasal secretion biomarker levels. RESULTS: There was a significant decrease in total nasal endoscopic polyp scores after 16 weeks in the omalizumab-treated group (-2.67, P = .001), which was confirmed by means of computed tomographic scanning (Lund-Mackay score). Omalizumab had a beneficial effect on airway symptoms (nasal congestion, anterior rhinorrhea, loss of sense of smell, wheezing, and dyspnea) and on quality-of-life scores, irrespective of the presence of allergy. CONCLUSION: Omalizumab demonstrated clinical efficacy in the treatment of nasal polyps with comorbid asthma, supporting the importance and functionality of local IgE formation in the airways.

Insights into levator muscle dysfunction in a cohort of patients with molecularly confirmed blepharophimosis-ptosis-epicanthus inversus syndrome using high-resolution imaging, anatomic examination, and histopathologic examination.

OBJECTIVE: To study the basis of defective levator palpebrae superioris (LPS) function in blepharophimosis-ptosis-epicanthus inversus syndrome (BPES), an autosomal dominant eyelid malformation sometimes associated with ovarian dysfunction. METHODS: Eight patients with molecularly proved BPES underwent high-resolution surface-coil 3-T magnetic resonance imaging before surgical intervention. The features of LPS muscle and adjoining connective tissue were compared with an age-matched control subject. During LPS resection for ptosis repair, detailed anatomic examination of the LPS was performed. Histopathologic characteristics were compared with normal control samples from a cadaver and a patient with simple severe congenital ptosis. RESULTS: The most striking feature shown on magnetic resonance imaging was the thin, long anterior part of the LPS. During the operation, this consisted of a disorganized, thin, long aponeurosis. However, in the posterior part of the LPS, there was an organized thick structure suggestive of a muscle belly. Histopathologic examination revealed posteriorly well-formed striated muscle fibers in all patients with BPES but not in the control sample from the patient with simple severe congenital ptosis. These striated muscle fibers were comparable to those of the normal control tissue but were more intermixed with collagenous tissue and little fatty degeneration. CONCLUSIONS: The presence of striated muscle fibers in LPS of patients with BPES contrasts with the fatty degeneration in patients with simple severe congenital ptosis. To our knowledge, this is the first study providing novel insights into the pathogenesis of the eyelid malformation in BPES through extensive imaging, anatomic study, and histopathologic testing in a unique cohort of patients with molecularly proved BPES.

Increased levator muscle function by supramaximal resection in patients with blepharophimosis-ptosis-epicanthus inversus syndrome.

OBJECTIVE: To study the efficacy and clinical and anatomical results of supramaximal levator resection in patients with blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) with severe congenital ptosis with poor levator function (LF). METHODS: Eleven patients with molecularly proven BPES underwent supramaximal levator resection. Palpebral fissure height and LF were measured preoperatively and postoperatively. RESULTS: All patients showed an excellent reduction in ptosis with a single intervention resulting in a clear visual axis. Palpebral fissure height improved from mean (SD) 3.3 (0.7) mm preoperatively to 7.1 (0.9) mm postoperatively (P value <.001). Four patients underwent additional surgery because of cosmetic issues with eyelid height asymmetry. All patients showed a marked, consistent, and lasting improvement in LF, going from mean (SD) 1.9 (0.9) mm preoperatively to 7.4 (1.1) mm postoperatively (P value <.001). This improvement could be attributed to the presence of a very long and thin tendon, as well as a striated muscle belly. This elongated aponeurosis inhibits the levator muscle from having sufficient impact on the vertical eyelid excursion. CONCLUSIONS: We demonstrated that supramaximal levator resection performed in patients with BPES not only results in good cosmetic appearance in terms of ptosis reduction in the majority of cases but also in a significant increase of the levator palpebrae superioris function. An anatomical substrate was found to explain these findings. To our knowledge, this is the first study to provide evidence of a marked increase in LF in BPES due to resection of the elongated tendon with reinsertion of the muscle belly.

Positron emission tomography-guided, focal-dose escalation using intensity-modulated radiotherapy for head and neck cancer.

PURPOSE: To assess the feasibility of intensity-modulated radiotherapy (IMRT) using positron emission tomography (PET)-guided dose escalation, and to determine the maximum tolerated dose in head and neck cancer. METHODS AND MATERIALS: A Phase I clinical trial was designed to escalate the dose limited to the [(18)-F]fluoro-2-deoxy-D-glucose positron emission tomography ((18)F-FDG-PET)-delineated subvolume within the gross tumor volume. Positron emission tomography scanning was performed in the treatment position. Intensity-modulated radiotherapy with an upfront simultaneously integrated boost was employed. Two dose levels were planned: 25 Gy (level I) and 30 Gy (level II), delivered in 10 fractions. Standard IMRT was applied for the remaining 22 fractions of 2.16 Gy. RESULTS: Between 2003 and 2005, 41 patients were enrolled, with 23 at dose level I, and 18 at dose level II; 39 patients completed the planned therapy. The median follow-up for surviving patients was 14 months. Two cases of dose-limiting toxicity occurred at dose level I (Grade 4 dermitis and Grade 4 dysphagia). One treatment-related death at dose level II halted the study. Complete response was observed in 18 of 21 (86%) and 13 of 16 (81%) evaluated patients at dose levels I and II (p < 0.7), respectively, with actuarial 1-year local control at 85% and 87% (p = n.s.), and 1-year overall survival at 82% and 54% (p = 0.06), at dose levels I and II, respectively. In 4 of 9 patients, the site of relapse was in the boosted (18)F-FDG-PET-delineated region. CONCLUSIONS: For head and neck cancer, PET-guided dose escalation appears to be well-tolerated. The maximum tolerated dose was not reached at the investigated dose levels.