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Some cases of Coffin-Lowry syndrome recognized episodic drops and it tended to be intractable for medical treatment. We reported here a patient with the Coffin-Lowry syndrome associated with obstructive sleep apnea syndrome (OSAS). The patient had epileptic seizures and drop attacks only during night-time and it was not recognized during the daytime. His sleep-induced electroencephalogram was normal. At 12-years old of his age, his OSAS was worse, so we performed a tracheotomy. Notably after the operation, his epileptic episodes were disappeared. [ABSTRACT FROM AUTHOR]

The mutant allele frequency of the Pyruvate kinase (PK) gene has been investigated mostly in pure breed cats. We investigated the PK mutant gene in stray and animal hoarding mongrel cats in Hokkaido, Japan. We also investigated the kinship of individuals carrying the mutant gene. Genotyping was conducted using the previously reported real-time PCR method. Fourteen microsatellite markers were used to identify the parents and offspring of cats carrying the PK mutant gene, and some kinship such as parent-offspring and siblings was observed. Some stray and animal hoarding cats carried the PK mutation gene and that consanguinity was confirmed among these cats indicated that the PK mutation gene was spread by unregulated interbreeding.

The cranial cruciate ligament (CCL) rupture is a common orthopedic disease in dogs that is usually managed with tibial plateau leveling osteotomy (TPLO) or extracapsular lateral suture (ECLS). Osteotomy is generally associated with some complications, including nonunion. The periosteum plays an important role in bone growth and remodeling. Osteocrin (OSTN), which was recently identified and is involved in bone formation and differentiation, is produced in the periosteum and osteoblasts. The aimed to investigate whether the concentrations of serum OSTN change before and after stifle surgery in dogs and compare the OSTN concentrations in the two surgical techniques (TPLO: n = 20 vs. ECLS: n = 36). The postoperative serum OSTN concentration in the TPLO group was significantly lower than the preoperative value (p < 0.05), while serum OSTN concentrations differed statistically between the preoperative and suture-removal periods. In contrast, no significant differences were observed in the ECLS group. In conclusion, osteotomy affects serum OSTN concentrations during the perioperative period in dogs, which may be related to periosteal injury., Competing Interests: Declaration of Competing Interest None of the authors have any financial or personal relationships that could inappropriately influence or bias the content of this paper., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Background: A staging system is essential for determining the optimal surgical approach and predicting postoperative outcomes for inverted papilloma (IP). Although staging systems based on the extent to which the location is occupied by an IP have been widely used, an origin site-based classification of IP using unsupervised machine learning algorithms has recently been reported., Objective: To determine the most appropriate of five staging systems for sinonasal IP by comparing recurrence rates for each stage according to each of those systems., Methods: Eighty-seven patients with sinonasal IP were enrolled in the study. Their tumors were retrospectively categorized according to the Krouse, Oikawa, Cannady, and Han staging systems, which are based on the extent of IP, and the Meng system, which is based on the site of origin. The rates of recurrence for each stage of the five systems were compared., Results: Seven of the 87 patients (8.0%) had recurrences during an average 45.5 months (12-138 months) of follow-up. There were significant differences in disease-free survival between the stages specified by Han and Meng (p = 0.027 and p < 0.001, respectively), but not between the stages specified by Krouse, Oikawa, and Cannady (p = 0.236, 0.062, and 0.130, respectively). Cox proportional hazard models revealed that Meng system (adjusted hazard ratio [aHR] 4.32, 95% confidence interval [CI] 1.10-17.04) and presence of dysplasia (aHR 7.42, 95% CI 1.15-47.85) were significantly associated with recurrence., Conclusion: The staging systems proposed by Han and Meng were found to be accurate in terms of tumor recurrence. We recommend use of the Han staging system before surgery and the Meng system after intraoperative identification of the origin of the tumor.

Objective: We employed a steroid-eluting, sinus-bioabsorbable device for local treatment after surgery for eosinophilic chronic rhinosinusitis (ECRS). One year later, we investigated its efficacy in suppressing recurrence and reducing the use of oral steroids., Methods: At one year after ECRS surgery, both 18 cases treated with a postoperative steroid-eluting, sinus-bioabsorbable device (Post-ST group) and 25 cases receiving conventional postoperative therapy (Post-Con group) showed significant improvement in the nasal symptoms (nasal obstruction, nasal discharge and olfactory dysfunction), CT score and threshold test (discrimination test)., Results: The olfactory dysfunction, CT score and threshold test were significantly improved in the Post-ST group compared with the Post-Con group, but the polyp score was not. The mean total number of oral steroid tablets ingested during one year after surgery was 24.3±2.8 tablets in the Post-ST group, which was significant lower than the 36.3±3.7 tablets used in the Post-Con group., Conclusion: The above results indicate that insertion of a steroid-eluting, sinus-bioabsorbable device after ECRS surgery can reduce the oral steroid intake while maintaining long-term suppression of disease recurrence., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: The C-reactive protein/albumin (CRP/Alb) ratio has been recently established as a prognostic indicator in various cancer types. However, few reports regarding the prognostic value of the CRP/Alb ratio in head and neck cancer exist. This study aimed to investigate the significance of the CRP/Alb ratio in clinical outcomes after invasive surgery involving laryngectomy for hypopharyngeal and laryngeal cancer., Methods: We evaluated 56 patients who underwent total laryngectomy or total pharyngolaryngectomy between 2003 and 2012. Univariate and multivariate analyses were retrospectively performed to examine the prognostic value of the CRP/Alb ratio in these patients., Results: The optimal cutoff value of the CRP/Alb ratio was 0.32. Multivariate analysis showed that the CRP/Alb ratio was a significant and independent predictor of poor overall and disease-free survival., Conclusion: The CRP/Alb ratio may be a novel and useful indicator for predicting postoperative outcomes in patients with hypopharyngeal and laryngeal cancer., (© 2018 Wiley Periodicals, Inc.)

A 33-year-old woman presented with an unusual subglottic bulging mass accompanied by prolonged cough and wheeze. Laryngeal endoscopy revealed a bilateral, symmetrical mass immediately below the vocal cords with marked airway obstruction. Chronic subglottic laryngitis with inflammation or another condition such as amyloidosis was initially suspected. Cervicothoracic computed tomography revealed an obvious reduction of laryngeal caliber caused by an engulfing mass extending from just under the vocal cords to the cricoid ring, which was associated with thyroid, arytenoid, and cricoid cartilage destruction. Histopathological diagnosis of a biopsy specimen collected via a tracheotomy revealed that the lesion was a cT4aN0M0 adenoid cystic carcinoma (ACC) originating from the laryngeal minor salivary glands. The patient was treated by total laryngectomy with elective bilateral neck dissection under general anesthesia. Gross inspection of resected tissue confirmed yellowish-white, solid tumor mainly circumferentially encompassing the lumina of the cricoid ring. The histopathological findings confirmed typical ACC accompanied by a predominant cribriform appearance with no evidence of lymph node metastasis. The patient remains well and free of recurrence or metastasis. We herein describe laryngeal ACC and discuss radiological images and the surgical pathology., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

The fibrous variant of Hashimoto thyroiditis is uncommon, accounting for approximately 10% of all cases of Hashimoto thyroiditis. We report a case of this variant that behaved like a malignant neoplasm. The patient was a 69-year-old man who presented with a right-sided anterior neck mass that had been rapidly growing for 2 weeks. Fine-needle aspiration cytology revealed clusters of large multinucleated cells suggestive of an anaplastic carcinoma. A week after presentation, we ruled out that possibility when the mass had shrunk slightly. Instead, we diagnosed the patient with an acute exacerbation of Hashimoto thyroiditis on the basis of laboratory findings. We performed a right thyroid lobectomy, including removal of the isthmus, to clarify the pathology and alleviate pressure symptoms. The final diagnosis was the fibrous variant of Hashimoto thyroiditis, with no evidence of malignant changes. Physicians should keep in mind that on rare occasions, Hashimoto thyroiditis mimics a malignant neoplasm.

We encountered a 38-years-old female patient who was complaining of an unpleasant sensation of the left cervical area due to a recurrent tumor originating from the middle pharynx. She had a history of surgically resected neurofibroma and lipoma from the anterior oropharyngeal wall respectively 5 years and 11 years previously. The preoperative diagnosis of a benign, non-epithelial neoplasm was made based on the imaging studies and surgical treatment was scheduled. An extended surgical resection of the middle pharynx including normal mucosa and a part of the tongue base was successfully accomplished. To cover the pharyngeal defect, a right antero-lateral thigh musculo-cutaneous flap was used for reconstruction. Microscopically, the surgically resected tissue showed a mixed condition of mature cartilaginous, bony and fibroadipose tissue without atypia. The final diagnosis was a benign mesenchymoma which was thought to have developed from pluripotential mesenchymal cells. We considered that the past tumorous lesions had possibly originated in those cells. Because pluripotential mesenchymal cells cannot easily be identified with ordinary histopathological examination, the determination of optimal surgical margins is difficult. In the case of mesenchymoma, substantial marginal tissue should be resected in order to prevent recurrence even in the case of a pathologically-proven benign tumor.

Objective: Thyroid surgery is a common basic procedure in otorhinolaryngology. The purpose of this study was to assess the efficiency of using a new LigaSure(®) vessel sealing system (LigaSure(®) Small Jaw Instrument; Covidien, Boulder, CO, USA)., Methods: We evaluated 83 patients who underwent thyroid surgery between July 2009 and June 2012. The patients were allocated to two groups, which underwent thyroid surgery using either the LigaSure(®) Small Jaw Instrument or conventional techniques. We investigated the duration of operation, estimated blood loss, pathological characteristics, postoperative complications, and length of hospital stay. As for duration of operation and estimated blood loss, we also performed analyses by subgroups according to the extent of thyroid resection, as either total thyroidectomy or hemithyroidectomy., Results: The study groups showed no significant differences in age, sex, indications, extent of operation and estimated blood loss. Complication rates for recurrent laryngeal nerve palsy, hypoparathyroidism, hematoma, and wound infection did not differ between groups, except for temporary hypoparathyroidism. Operation time was significantly shorter in the LigaSure(®) Small Jaw Instrument group than in the conventional group for all extents of thyroid resection., Conclusion: Use of the LigaSure(®) Small Jaw Instrument seems likely to reduce the operating time for thyroid surgery without increasing estimated blood loss or the frequency of postoperative complications compared to conventional techniques., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)

Two cases of orbital tumor are presented, and the surgical technique by means of an endoscopic transnasal approach is discussed. In Case 1, a non-vascular, benign tumor was located inside the orbit, and it was able to be completely removed, without any complications. The tumor in Case 2 was also located inside the orbit, but it was determined to be a hemangioma based on the intraoperative pathology. To avoid eye complications arising from performance of the surgery in the presence of bleeding, it was decided to widely open the orbital lamina papyracea and periosteum, and perform only orbital decompression. The eye symptoms disappeared in both patients. Endoscopic transnasal surgery is indicated for benign orbital tumors that are medially located in the orbit. It can be thought that the eye symptoms can be alleviated by avoiding orbital pressure, regardless of whether-based on the intraoperative pathology-the tumor is completely removed or not., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

Objective: Long-term retrospective evaluation was performed of computed tomography (CT) images and endoscopic findings after endoscopic sinus surgery for 88 cases of chronic pediatric sinusitis with nasal polyps. The objective was to determine the appropriate duration of such postoperative evaluation for children., Methods: Fifty-one patients had both sinusitis and nasal polyps (BSP group), and the surgical procedure was decided in consideration of each patient's age (for less than 10 years of age, polypectomy (n=12); for 10-13 years old, anterior ethmoidectomy plus opening of the fontanelle and nasofrontal duct (n=20); and for serious cases older than 13 years, total sinusectomy (n=19)). On the other hand, for cases of unilateral sinusitis with antrochoanal polyps (USP group), anterior ethmoidectomy plus opening of the fontanelle was performed regardless of the patient's age (n=37, 5-15 years old)., Results: The postoperative endoscopic findings indicated that the polyps had been eliminated in approximately 91% of total patients. Good postoperative findings of CT images in most patients of USP group are observed at one year after the operation like the same of the postoperative course of adult chronic sinusitis. On the other hand CT images in BSP group evaluated one year after the operation were rated as unchanged or worsened in approximately half of the patients. However, at 4 years after the operation nearly all the patients were rated as improved or better. In addition, comparison of the age at final observation and the postoperative course found a striking decrease in the proportion of unchanged and worsened patients aged 12 and above., Conclusion: Accordingly, it was concluded that there is difference of healing process after the operation between USP and BSP group. Postoperative evaluation of BSP group should be performed for 4 years and up to an age of at least 12 years although post-ESS following of USP group is similar to that of adult sinusitis., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

A 14-year-old girl was referred for evaluation and management of progressive, painful swelling of the right cheek. Swelling had been present since 3-year old and had gradually increased in size. Magnetic resonance imaging (MRI) revealed a well-circumscribed, brighter mass in the right masseter muscle with numerous rounded areas of signal hypointensity. Preoperative diagnosis was intramuscular hemangioma of the masseter muscle and surgery was performed. The tumor was completely removed except for a few, small phleboliths. Pathological examination of resected tissue led to a high suspicion of cavernous hemangioma with phlebolithiasis. Nine months postoperatively, the patient developed another painful mass in the right masseter muscle. MRI indicated recurrent hemangioma and further surgery was performed. Careful exploration resulted in completely removal of residual phleboliths accompanied with fibroadipose tissue. Part of the buccal branch of the facial nerve was excised to achieve complete resection of the lesion. Histological examination revealed distinct venous formation in phleboliths adjacent to fibroadipose tissue, demonstrating that both phleboliths and feeding vessels had been left by the previous operation. The present report reviews the literature on intramuscular hemangiomas of the masseter muscle, and discusses diagnostic methods and optimal surgical treatment.

Sarcoidosis is a multiorgan granulomatous disease of unknown cause, named after the characteristic histopathological findings of the disease. In this article, we shall report a case in which biopsy needed to be repeated three times until a final diagnosis of laryngeal sarcoidosis could be made. Since sarcoidosis associated with only a laryngeal pathology may not be associated with any laboratory abnormalities in many cases, first priority should be attached in suspected cases to detect the presence of noncaseating epithelioid cell granulomas. In this case, the results of the first and second laryngeal biopsies did not show non-caseating epithelioid cell granulomas, while the third biopsy showed noncaseating epithelioid cell granulomas with giant cells. Finally, the diagnosis of laryngeal sarcoidosis was made after the results of whole-body imaging became available. Laryngeal sarcoidosis must be borne in mind as a suspected diagnosis in cases where the larynx shows yellowish-white diffuse swelling.