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Ear, Nose, & Throat Journal Jun 2024We aimed to summarize the surgical treatment for pharyngolaryngeal stenosis and discuss prognosis in patients with Behcet's disease. Six cases of pharyngolaryngeal...
We aimed to summarize the surgical treatment for pharyngolaryngeal stenosis and discuss prognosis in patients with Behcet's disease. Six cases of pharyngolaryngeal stenosis caused by Behcet's disease were analyzed retrospectively. All underwent surgical treatment for pharyngolaryngeal stenosis after systematic medical treatment. The follow-up time for the 6 patients was between 1 and 12 years. Four of the 6 patients underwent adhesiolysis as their first procedure. Two of these 4 experienced recurrence of stenosis within 6 months and underwent flap repair as their second procedure. The remaining two patients underwent flap reconstruction as their first procedure and maintained good swallowing function. Three of the 6 patients underwent preoperative tracheotomy because of dyspnea. Tracheal decannulation was successful in all patients. None of the patients experienced recurrence after their final surgical procedure and all recovered to a near-normal condition. Pharyngolaryngeal stenosis caused by Behcet's disease is a rare but severe complication; surgical intervention should be considered in patients with dysphagia after systematic medical treatment.
Topics: Humans; Behcet Syndrome; Male; Adult; Retrospective Studies; Female; Pharyngeal Diseases; Laryngostenosis; Constriction, Pathologic; Middle Aged; Surgical Flaps; Deglutition Disorders; Recurrence; Treatment Outcome; Young Adult; Tracheotomy
PubMed: 34814770
DOI: 10.1177/01455613211053427 -
PloS One 2019Brachycephalic syndrome (BS) is a pathophysiological disorder caused by excessive soft tissue within the upper airways of short-nosed dog breeds, causing obstruction of...
Brachycephalic syndrome (BS) is a pathophysiological disorder caused by excessive soft tissue within the upper airways of short-nosed dog breeds, causing obstruction of the nasal, pharyngeal and laryngeal lumen, resulting in severe respiratory distress. As the prevalence of BS appears to be high among some of the affected breeds, there is an urgent need for breeding efforts to improve the health status of those dogs. In the present study, we evaluated correlations between morphometric and other phenotypic characteristics and BS in a population of 69 French bulldogs from Denmark to identify parameters that could serve as a basis for breeding against BS. Furthermore, the genetic variation was monitored to determine whether it would be possible to breed based on these characteristics without simultaneously causing a critical reduction in genetic variation. Six phenotypic characteristics were correlated with the Brachycephalic Syndrome Functional (BSF) score. Among the morphometric risk factors, nostril stenosis (NS) and neck girth (NG) had the highest impact on the BSF score, accounting for 32% and 4% of the variation, respectively. The genetic variation in the population was comparable to other pure breeds, i.e. estimated and observed heterozygosity was 0.60 and the average inbreeding coefficient was 0.01. If only dogs with Grades 1 and 2 NS (no or only mild NS) were selected for breeding the mean BSF score would be reduced significantly. However, it would result in the exclusion of 81% of the population for breeding and this is not prudent. Excluding only dogs with severe stenosis (Grade 4) would exclude 50% of the population without any adverse impact on genetic variation within the population. Although exclusion of dogs with Grade 4 would result in an apparent reduction in the mean BSF score, this reduction is not significant. As NS accounts for 32% of the variation in BSF score, a possible long term strategy to reduce the prevalence of the BS in French bulldogs would seem to be a selection scheme that first excluded dogs with the most severe NS from breeding, gradually moving towards selecting dogs with lower NS grades. According to our findings there is no viable short term solution for reducing the prevalence of BS in the French bulldog population.
Topics: Airway Obstruction; Animals; Breeding; Craniosynostoses; Dog Diseases; Dogs; Exercise Test; Female; Male; Nasal Cavity; Phenotype; Respiration; Risk Factors; Selection, Genetic; Syndrome
PubMed: 31841527
DOI: 10.1371/journal.pone.0226280 -
Developmental Dynamics : An Official... Nov 201614-3-3ε plays an important role in the maturation of the compact ventricular myocardium by modulating the cardiomyocyte cell cycle via p27 . However, additional cardiac...
BACKGROUND
14-3-3ε plays an important role in the maturation of the compact ventricular myocardium by modulating the cardiomyocyte cell cycle via p27 . However, additional cardiac defects are possible given the ubiquitous expression pattern of this protein.
RESULTS
Germ line deletion of 14-3-3ε led to malalignment of both the outflow tract (OFT) and atrioventricular (AV) cushions, with resulting tricuspid stenosis and atresia, mitral valve abnormalities, and perimembranous ventricular septal defects (VSDs). We confirmed myocardial non-compaction and detected a spongy septum with muscular VSDs and blebbing of the epicardium. These defects were associated with abnormal patterning of p27 expression in the subendocardial and possibly the epicardial cell populations. In addition to abnormal pharyngeal arch artery patterning, we found deep endocardial recesses and paucity of intramyocardial coronary vasculature as a result of defective coronary plexus remodeling.
CONCLUSIONS
The malalignment of both endocardial cushions provides a new explanation for tricuspid and mitral valve defects, while myocardial non-compaction provides the basis for the abnormal coronary vasculature patterning. These abnormalities might arise from p27 dysregulation and a resulting defect in epithelial-to-mesenchymal transformation. These data suggest that 14-3-3ε, in addition to left ventricular non-compaction (LVNC), might be linked to different forms of congenital heart disease (CHD). Developmental Dynamics 245:1107-1123, 2016. © 2016 Wiley Periodicals, Inc.
Topics: 14-3-3 Proteins; Animals; Coronary Artery Disease; Cyclin-Dependent Kinase Inhibitor p27; Endocardium; Gene Expression Regulation, Developmental; Heart Defects, Congenital; Heart Ventricles; Mice; Myocardium
PubMed: 27580238
DOI: 10.1002/dvdy.24440 -
The Laryngoscope Jun 2021Long-term functional outcomes are poorly characterized for salvage laryngectomy. We identified predictors of esophageal stricture and swallowing function after salvage...
BACKGROUND
Long-term functional outcomes are poorly characterized for salvage laryngectomy. We identified predictors of esophageal stricture and swallowing function after salvage laryngectomy in a large cohort.
METHODS
A retrospective study of 233 patients who underwent salvage total laryngectomy for recurrent/persistent squamous cell carcinoma of the larynx or hypopharynx after radiation (XRT) or chemoradiation (CRT) was performed. Primary outcomes were esophageal dilation within 1 year, time to dilation, and gastrostomy tube dependence. Multivariate logistic and Cox regressions were used for statistical analysis.
RESULTS
Dilation was performed in 29.9% of patients. Dilation was twice as likely in patients with post-operative fistula compared to those without (Hazard Ratio (HR) 2.10, 95% Confidence Interval (CI) 1.06-4.13, P = .03). Every year between XRT/CRT and salvage was associated with 10% increase in dilation (HR 1.09, 95% CI 1.03-1.17, P = .01). No factors were associated with dilation by 1 year. About 10% of patients were at least partially gastrostomy tube-dependent 1 year post-operatively. At last follow-up (median 29 months), this rate was 13%. Patients with supraglottic recurrence had an increased risk of gastrostomy tube dependence at 1 year compared to glottic (OR 16.7, 95% CI 1.73-160, P = .02). For every 10 pack years pre-salvage, the OR of requiring tube feeds at last follow-up was 1.24 (95% CI 1.04-1.48, P = .02).
CONCLUSIONS
Fistula and pre-salvage smoking were associated with stricture post-salvage laryngectomy. No factors were associated with dilation by 1 year. Supraglottic recurrence and smoking were associated with gastrostomy tube dependence. These findings are important for pre-operative counseling prior to salvage laryngectomy.
LEVEL OF EVIDENCE
Level 4 Laryngoscope, 131:1229-1234, 2021.
Topics: Aged; Carcinoma, Squamous Cell; Constriction, Pathologic; Deglutition; Esophageal Fistula; Esophageal Stenosis; Female; Gastrostomy; Humans; Hypopharyngeal Neoplasms; Hypopharynx; Laryngeal Neoplasms; Laryngectomy; Larynx; Logistic Models; Male; Middle Aged; Neoplasm Recurrence, Local; Odds Ratio; Postoperative Complications; Postoperative Period; Proportional Hazards Models; Retrospective Studies; Salvage Therapy; Smoking; Time Factors; Treatment Outcome
PubMed: 33152117
DOI: 10.1002/lary.29215 -
Srpski Arhiv Za Celokupno Lekarstvo 2008The American otolaryngologist Eagle was the first to describe styloid syndrome in 1937. Stylohyoid complex is composed of styloid process, stylohyoid ligament and a... (Review)
Review
The American otolaryngologist Eagle was the first to describe styloid syndrome in 1937. Stylohyoid complex is composed of styloid process, stylohyoid ligament and a lesser horn of the hyoid bone. Embriologicaly, these anatomical structures originate from Reichert's cartilage of the second brachial arch. In the general population, the frequency of the elongated styloid process is estimated to be 4%, of which only 4% show clinical manifestations suggesting that the incidence of styloid syndrome is 0.16% (about 16,000 persons in Serbia). The styloid process deviation causes external or internal carotid impingement and pains which radiate along the arterial trunk. Classical stylohyoid syndrome is found after tonsillectomy and is characterised by pharyngeal, cervical, facial pain and headache. Stylo-carotid syndrome is the consequence of the pericarotid sympathetic fibres irritation and compression on the carotid artery. Clinical manifestations are found most frequently after head turning and neck compression. The diagnostic golden standard for styloid syndrome is 3D CT reconstruction. Sagital CT angiography has a leading role in the radiological diagnosis of the stylo-carotid syndrome. Differential diagnosis requires the differentiation of the styloid syndrome from numerous cranio-facio-cervical painful syndromes. If conservative treatment (analgesics, anticonvulsants, antidepressants, and local infiltration with steroids or anaesthetic agents) has no effect, surgical treatment is applied. Styloid syndrome is underrepresented in neurological literature. The syndrome is considered important, because it is clinically similar to many other painful cranio-facial syndromes; it is difficult to be recognized, and the patient should be treated adequately.
Topics: Carotid Stenosis; Humans; Hyoid Bone; Syndrome
PubMed: 19177834
DOI: 10.2298/sarh0812667p -
Ear, Nose, & Throat Journal Feb 2022Acquired choanal atresia is a rare complication of pharyngeal surgical interventions. Here, we report the case of a 72-year-old woman who was treated in our institution...
Acquired choanal atresia is a rare complication of pharyngeal surgical interventions. Here, we report the case of a 72-year-old woman who was treated in our institution for acquired choanal atresia after failed previous treatments. We have excised the obstructing fibrotic scar tissue and then reconstructed the area using a mucosal local flap and free radial forearm flap transfer. Choanal stenosis caused by abnormal granulation was identified in the months after surgery; however, this was treated with endoscopic dilatation and steroid injections in the outpatient setting. No restenosis was identified 1 year after the operation. Postoperative evaluation of speech was satisfactory. Free flap transfer could be an option for refractory choanal atresia, and endoscopic dilatation with local steroid injections may be an effective method to treat postoperative restenosis.
PubMed: 35176899
DOI: 10.1177/01455613211070894 -
International Journal of Surgery Case... 2016Chemoradiotherapy plays an important role in preserving function and morphology in head and neck cancer. However, in a few cases, chemoradiotherapy has been shown to...
INTRODUCTION
Chemoradiotherapy plays an important role in preserving function and morphology in head and neck cancer. However, in a few cases, chemoradiotherapy has been shown to result in late complications, such as hypopharyngeal perforation, which is very rare.
PRESENTATION OF CASE
A 65-year-old man, who had undergone chemoradiotherapy for hypopharyngeal cancer 30 months previously, presented with high fever and neck pain. He subsequently developed hypopharyngeal stenosis, hypopharyngeal perforation, and a retropharyngeal abscess followed by pyogenic spondylitis. He underwent surgical treatment (resection with reconstruction) and was administered an antibacterial agent and steroids for an extended period. This treatment regimen was successful, and the patient has survived disease-free without symptoms.
DISCUSSION
Chemoradiotherapy-induced hypopharyngeal perforation is an extremely rare condition. In the present case, the perforation was large (2cm), and the hypopharyngeal cavity was originally constricted. Pharyngeal reconstruction with a jejunal autograft was therefore necessary. Through the present case, we reconfirmed that although the primary purpose of chemoradiotherapy is organ preservation, it can also lead to organ destruction and fatal complications. It is important that physicians be aware of the possibility of hypopharyngeal perforation so as to avoid delayed diagnosis and treatment of similar rare cases.
CONCLUSION
Hypopharyngeal perforation can sometimes be fatal because it can lead to pyogenic spondylitis. Suitable surgical techniques and appropriate doses of antibacterial agents for long-term use were appropriate treatments for the patient in this case.
PubMed: 26829460
DOI: 10.1016/j.ijscr.2016.01.020 -
The Journal of International Medical... Jul 2020Branchial cleft abnormality is a common congenital neck malformation in children, which is caused by the abnormal development of the gill sac or gill groove. It is... (Review)
Review
Branchial cleft abnormality is a common congenital neck malformation in children, which is caused by the abnormal development of the gill sac or gill groove. It is mainly manifested as a cyst in the sinus tract and fistula in the neck, as well as branchio-oto-renal syndrome (BORS). As a rare autosomal dominant genetic disease, the typical manifestations of BORS are hearing loss, abnormal branchial cleft development and renal dysplasia. In this paper, a patient was admitted to the hospital for bilateral branchial cleft fistulas combined with bilateral anterior auricular fistulas, auricular appendix, auricle dysplasia, external auditory canal stenosis, and hearing loss. The patient was diagnosed with BORS, and underwent fistulectomy of the neck and anterior ear, external auditory canal formation, and tympanoplasty. The aim of this report is to strengthen clinicians' understanding of BORS and reduce the rate of clinical missed diagnosis through our case report and literature review.
Topics: Branchial Region; Branchio-Oto-Renal Syndrome; Child; Craniofacial Abnormalities; Fistula; Humans; Pharyngeal Diseases
PubMed: 32689865
DOI: 10.1177/0300060520926363 -
Diagnostics (Basel, Switzerland) Mar 2022Wellens' syndrome (WS) is a preinfarction state caused by a sub-occlusion of the proximal left anterior descending coronary artery (LAD). In this case report, for the...
Wellens' syndrome (WS) is a preinfarction state caused by a sub-occlusion of the proximal left anterior descending coronary artery (LAD). In this case report, for the first time, we describe how this syndrome can be caused by COVID-19 infection and, most importantly, that it can be assessed bedside by enhanced transthoracic coronary echo Doppler (E-Doppler TTE). This seasoned technique allows blood flow Doppler to be recorded in the coronaries and at the stenosis site but has never been tested in an acute setting. Two weeks after clinical recovery from bronchitis allegedly caused by COVID-19 infection on the basis of epidemiologic criteria (no swab performed during the acute phase but only during recovery, at which time it was negative), our patient developed typical angina for the first time, mainly during effort but also at rest. He was admitted to our tertiary center, where pharyngeal swabs tested positive for COVID-19. A typical EKG finding supporting WS prompted an assessment of the left main coronary artery (LMCA) and the whole LAD blood flow velocity by E-Doppler TTE. Localized high velocity (transtenotic velocity) (100 cm/s) was recorded in the proximal LAD, with the reference velocity being 20 cm/s. This indicated severe stenosis with 90% area narrowing according to the continuity equation, as confirmed by coronary angiography. During follow-up after successful stenting, E-Doppler TTE showed a decrease in the transtenotic acceleration, confirming stent patency and a normal coronary flow reserve (3.2) and illustrating a normal microcirculatory function. Conclusion: COVID infection can trigger a coronary syndrome like WS. E-Doppler TTE, an ionizing radiation-free method, allows safe and rapid bedside management of the syndrome. This new strategy can be pivotal in distinguishing true WS from pseudo-WS. In cases of pseudo-WS, coronary angiography can be avoided. If E-Doppler TTE confirms the stenosis and PCI (percutaneous coronary intervention) is performed, the same method can allow assessment over time of the precise residual stenosis after stenting and verify the microvasculature status by evaluating coronary flow reserve.
PubMed: 35453852
DOI: 10.3390/diagnostics12040804 -
BMC Anesthesiology Jun 2023Goldenhar syndrome is a congenital disease that involves an absence or underdevelopment of structures that arise from the first and second pharyngeal arches and more or...
BACKGROUND
Goldenhar syndrome is a congenital disease that involves an absence or underdevelopment of structures that arise from the first and second pharyngeal arches and more or less severe extracranial anomalies. A variety of supraglottic malformations may be observed, including mandibular hypoplasia, mandibular asymmetry and micrognathia. Subglottic airway stenosis (SGS), which can cause difficulties in airway management during the perioperative period, is seldom emphasized in literature descriptions of Goldenhar syndrome, but can be clinically significant.
CASE PRESENTATION
An 18-year-old female with a history of Goldenhar syndrome presented for placement of a right mandibular distractor, right retroauricular dilator, and stage I transfer of a prefabricated expanded flap under general anesthesia. During tracheal intubation, the endotracheal tube (ETT) met resistance unexpectantly when attempting to pass through the glottis. Subsequently, we attempted the procedure with a smaller size ETT but again met resistance. With fiberoptic bronchoscope, we found that the whole segment of the trachea and bilateral bronchi were obvious narrow. Given the finding of unexpected severe airway stenosis and the associated risks with proceeding with the surgery, the operation was cancelled. We removed the ETT once the patient was fully awake.
CONCLUSIONS
Anesthesiologists should be aware of this clinical finding when evaluating the airway of a patient with Goldenhar syndrome. Coronal and sagittal measurements on computerized tomography (CT) and three-dimensional image reconstruction can be used to evaluate the degree of subglottic airway stenosis and measure the diameter of the trachea.
Topics: Female; Humans; Adolescent; Goldenhar Syndrome; Constriction, Pathologic; Intubation, Intratracheal; Trachea; Glottis
PubMed: 37328815
DOI: 10.1186/s12871-023-02179-w