Manifestations of pott's disease in the head and neck

Tuberculosis (TB) of the spine, or Pott's disease, is a rarely encountered clinical disorder in the United States today. However, Pott's disease should be strongly suspected in a person who presents with a destructive lesion of the spine involving adjacent vertebrae or a retropharyngeal mass which extends across the midline. The most common presenting signs and symptoms are pain and spinal deformity. The PPD skin test is usually positive, but unlike TB of the larynx, the chest roentgenogrom is almost always negative. Diagnosis may be confirmed by needle aspiration or incisional biopsy and culture. The otolaryngologic literature has not previously addressed this clinical entity, thus, our experience with a patient with a retropharyngeal mass and quadraplegia diagnosed as Pott's disease will be presented. Subsequent medical therapy, stabilization, and odontoidectomy resulted in resolution of his neurological deficit. The incidence, pathogenesis, clinical features, and management of Pott's disease will be discussed.     

Crohn's supraglottitis – The presenting feature of otherwise asymptomatic systemic disease

We present the unique case of a 15 year old boy with upper airway obstruction found to have Crohn's disease in the absence of gastrointestinal symptoms. Laryngeal manifestations of Crohn's disease are extremely rare. Only 11 cases have been reported in the literature, of which only one is in a child. Laryngeal Crohn's disease usually occurs accompanied by gastrointestinal symptoms or in patients with a prior diagnosis of Crohn's disease. The literature on the clinical features and management of laryngeal Crohn's is discussed along with a summary of the reported otolaryngological manifestations and associations with Crohn's disease.     

Diagnostic and therapeutic strategies for Meniere's disease of the Japan Society for Equilibrium Research

ObjectiveWe provided diagnostic and therapeutic strategies for Meniere's disease in accordance with Japanese Clinical Practice Guideline of Meniere's disease and delayed endolymphatic hydrops 2nd ed. Tokyo: Kanehara Shuppan; 2020 edited by the Japan Society for Equilibrium Research.MethodsThe Committee for Clinical Practice Guidelines was entrusted with a review of the scientific literature on the above topic. Clinical Questions (CQs) concerning the treatment for Meniere's disease were produced, and the literature according to each of them including CQ was searched. The recommendations are based on the literature review and the expert opinion of a subcommittee.ResultsDiagnosis criteria of Meniere's disease are classified into Meniere's disease with typical cochlear and vestibular symptoms, and atypical Meniere's disease with either cochlear symptoms or vestibular symptoms. Treatment of Meniere's disease was composed of lifestyle changes, medications such as anti-vertigo drugs and diuretics, middle ear positive pressure treatment, and selective destruction of the vestibule.ConclusionMeniere's disease is diagnosed based on clinical histories and examination findings after processes of differential diagnosis. Treatment option of the disease should be selected in order of invasiveness, according to the severity of the disease and the response to each treatment.     

Clinical features and management of Luc's abscess: Case report and systematic review of the literature

ObjectivesLuc's abscess is a rare complication of acute otitis media, with a challenging diagnosis and a controversial surgical treatment. The aim of the present study was to review the published literature in order to clarify the clinical features and the surgical management of those patients.MethodsA systematic review of the literature was carried out for published reports or case series in English language, describing a temporo-zygomatic (or Luc's) abscess which complicated an acute or chronic otitis media and/or mastoiditis, confirmed through CT scan or MRI of the petrous bone. The collected clinical and radiological data were merged and critically appraised.ResultsEighteen reports of Luc's abscess were included. Adding our case report, a total of 21 cases were included in the analysis. Abscess drainage plus myringotomy alone vs. abscess drainage plus myringotomy and mastoidectomy were the two surgical management approaches described in the literature. Patients undergoing first line mastoidectomy were successfully treated in all cases, while among those undergoing a more conservative approach, one failure required subsequent mastoidectomy.ConclusionsThe clinical features of Luc's abscess are rather constant and help in rising the suspicion before the radiological diagnosis. Although cases with associated intra-cranic complications have been reported, the limited existing data do not permit to advocate the mastoidectomy over a more conservative surgical approach. However, the decision to avoid mastoidectomy as the first line surgical treatment should be based on the clinical and radiologic assessment, after an accurate counseling, particularly in the case of a pediatric patient.     

Update on Intratympanic Gentamicin for Meniere's Disease

Objective: To review the literature relating to intratympanic gentamicin injection therapy for Meniere's disease to detect consistencies and differences that might suggest optimal technique. Design: Retrospective literature review. Methods: Eighteen papers from the literature regarding clinical experience with intratympanic gentamicin injections for treatment of Meniere's disease were reviewed and tabulated. Results: All papers reported high success rates in treating episodic vertigo of Meniere's disease, but technique, dose, duration, and treatment philosophy varied considerably. Hearing loss was typically reported in about 30% of patients. Conclusion: No single technique of gentamicin injection has a significant medical advantage over the others. Until controlled studies indicate otherwise, nonmedical needs such as convenience and safety may be considered when choosing a technique.     

Castleman's Disease: A rare finding in a pediatric neck

Castleman's Disease is a rare lymphoproliferative disorder. In the literature, only 29 cases, associated with the neck presentation in children, have been reported. This is another case report regarding a 5-year old child who presented with a persistent cervical lymphadenopathy. Final pathology, after undergoing exploratory neck dissection and surgical excision, revealed Castleman's Disease. This report, augmented with a literature review of all the 29 cases, compares the clinical course of this patient with the other cases.     

Congenital laryngeal hamartoma with ectopic thymus causing airway compromise in a neonate

To describe an interesting clinical and histopathologic case of a neonate presenting with respiratory distress and a laryngeal hamartoma with ectopic thymus and parathyroid tissue. Case report and PubMed review of the English literature. A full term, newborn male who presented immediately after birth with postitional, inspiratory stridor and airway distress. During operative microlaryngoscopy, a well-delineated, non-cystic, stalk-like mass was found in the endolarynx. Biopsy revealed thymic tissue with Hassall's corpuscles, parathyroid tissue and a central core of mature fibroadipose tissue and skeletal muscle. The mass was removed endoscopically with cold instrumentation. At 4 months postoperatively, the patient was doing well, without any respiratory symptoms, feeding difficulties, or endoscopic findings of recurrence. Neonatal laryngeal hamartomas are extremely rare. In general, an excellent prognosis is associated with these lesions, and the treatment of choice is endoscopic, surgical excision. This case is histopathologically unique because it is the first to encounter the presence of thymic and parathyroid tissue within a glandular hamartoma.     

Extraskeletal Ewing's Sarcoma of parapharyngeal space

Extraskeletal Ewing's Sarcoma (EES) of head and neck region, is a rare soft tissue tumour morphologically similar to the commoner Ewing's Sarcoma arising from bone. We report a case of EES in parapharyngeal space in a 6-year-old male; who presented with stridor. CT scan of neck showed soft tissue mass in right parapharyngeal space with intraorbital and intracranial extention. Histopathological examination showed round cell tumour and immunohistochemistry was positive for CD99 and Vimentin. The patient responded very well to Concurrent Chemoradiation. An extensive review of English literature, to the best of our knowledge, did not reveal any previous case of EES in parapharyngeal space in children; although one similar case in a 53-year male is reported.     

Otosclerosis and meniere's syndrome: Diagnosis and treatment

Occasionally a patient with otosclcrosis and a conductive hearing loss will develop typical findings of Meniere's syndrome in the involved car, years later. A review of clinical and pathological studies in the literature and in our laboratory and clinic indicates a likely cause-and-effect relationship for these cases. The pathology and pathogenesis of the syndrome of otosclcrosis and Meniere's syndrome is discussed. A stapedectomy/sacculotomy was used to treat 17 patients, 13 of whom acquired a satisfactory result in terms of improvement of hearing and control of vertigo. This technique and findings are described and discussed.     

Embryonal rhabdomyosarcoma of the neck masquerading as a congenital lymphangioma in a pediatric patient

The diagnosis of lymphangioma is typically based on history, exam, and imaging. Biopsy is rarely sought due to the accuracy of clinical diagnosis. We report a 4-year-old male with Gorlin syndrome, who presented with a neck mass that was clinically diagnosed as a lymphangioma. Sclerotherapy was performed at the family's request. After the procedure, necrosis developed over the surface of the mass. Surgical excision was then performed, and pathology interpreted as embryonal rhabdomyosarcoma. The presentation allowed this rhabdomyosarcoma to masquerade as a lymphangioma. This unusual presentation brings into question the use of fine needle aspiration in suspected lymphangiomas.     

Developmental language impairment through the lens of the ICF: An integrated account of children's functioning

The conceptual framework of the World Health Organization's International Classification of Functioning, Disability and Health (ICF) has the potential to advance understanding of developmental language impairment (LI) and enhance clinical practice. The framework provides a systematic way of unifying numerous lines of research, which have linked a wide array of factors to the functioning of children with LI. The result is an integrated account of LI where children's functioning emerges from the complex interaction of core linguistic processes, the ability to use them in social interactions, and a variety of environmental and personal factors. This account is well-suited to the clinical context because it focuses clinical attention on how such factors may be interacting to maintain a child's functional limitations, and on how they might work together to facilitate optimal everyday functioning, the ultimate goal of intervention. In this paper, the ICF's conceptual framework is described, and the nature of the relationships among its components explained. We explore how the integrated view of LI inspired by this conceptual framework differs from the prevailing impairment-driven account, provide examples from the literature that are consistent with the former view, and discuss its implications for clinical decision-making.Learning outcomes: As a result of this activity, the reader will be able to: (1) describe the nature of the relationships that exist among components of the ICF; (2) describe how interactions among components may shape the functioning of children with LI; (3) identify ways in which the integrated account of LI engendered by the ICF may improve clinical service.     

A rare case of primary Ewings sarcoma of the nasal bone

Primary Ewing’s sarcoma of the nasal bone has not been previously described. This case presented as a mass in the left ala of the nose in a five year old female child. The clinical, radiological, microscopic features are described and a review of literature is presented. The case was treated with neoadjuvant chemotherapy and local electron beam radiation therapy. The child was free of disease when she reported for follow up in July 1997. Although wide excision is part of the treatment approach in Ewing’s sarcoma, in sites where surgery is not suitable local radiotherapy and chemotherapy adequately controls primary disease.     

Rathke's cleft cyst marsupialization and repair with a free mucosal graft – Video case report and literature review

Rathke's cleft cysts (RCCs) are sellar or suprasellar cystic lesions arising from the remnants of the embryological Rathke's pouch. When symptomatic, RCCs are usually treated surgically via marsupialization. Free mucosal graft (FMG) repair has shown promise in decreasing recurrence versus marsupialization alone. The authors present a case report with operative video of a patient with visual and endocrinological symptoms with a RCC treated with FMG following marsupialization. A search of the PubMed database from July 1997 through April 2022 was conducted using the terms ‘Rathke's cleft cyst’, ‘Rathke's cleft cyst management’, ‘Rathke's cleft cyst repair’, ‘mucosal graft’, ‘mucosal coupling’, ‘transsphenoidal’, and ‘endoscopic endonasal’. Clinical and pathological aspects of the case presented were compared with information obtained from literature review. A 25-year-old female presented with a six-year history of amenorrhea, one-year history of anemia, headaches, and progressive visual loss. Imaging revealed an RCC. The patient underwent surgical treatment via an endoscopic endonasal approach. Marsupialization was achieved with placement of an FMG to help prevent restenosis. Post-operative examination revealed that the patient's OD vision returned to normal, although her OS vision was only slightly improved. Our review of the English literature resulted in 35 full-length articles that were published between 1997 and 2022. The literature suggests that FMG prevents scar formation by allowing for adequate epithelialization, thus decreasing the recurrence rate and being well tolerated by patients. Our findings support the utility of the FMG as a superior surgical treatment option for RCC management.     

Schwannoma of the external auditory canal: An exceptional site

IntroductionSchwannomas are benign solitary neural tumours that are only exceptional located in the external auditory canal, as only a few cases have been reported in the literature.Case reportWe report a case of schwannoma of the external auditory canal in an 18-year-old man admitted for an isolated mass of the initial segment of the right external auditory canal visible to the naked eye, obstructing all of the external auditory meatus. Computed tomography of the temporal bone showed an isolated mass of the external auditory canal. Management of this patient consisted of biopsy-excision of the mass, histological examination of which confirmed a schwannoma of the external auditory canal.DiscussionAlthough rare, the possibility of a nerve tumour of the external auditory canal should always be considered. These tumours may be isolated or may occur in the context of von Recklinghausen's disease. The clinical presentation in the external auditory canal may correspond to recurrent otitis externa secondary to obstruction of the canal by the tumour, as in the case reported here. The definitive diagnosis must be based on the results of histological and immunohistochemical examination.     

Wegener's granulomatosis,acute laryngotracheal airway obstruction and death in a 17-year-old female: case report and review of the literature

Objective: To alert practitioners to the risk of sudden airway obstruction and death in patients with Wegener's granulomatosis. Design: Case report and literature review. Settings: University and Community hospitals. Patient: A 17-year-old white female. Intervention: (1) evaluation and treatment for mental status changes over 2 months. (2) Evaluation and surgical biopsy of nasal septal perforation under general anesthesia at a university children's hospital. (3) Evaluation and observation at a community hospital 2 days later. (4) autopsy. Results: Serology performed 3 days ante-mortem revealed (at 2 days post-mortem) cytoplasmic anti-neutrophil cytoplasmic antibody positive at 1:128. Autopsy was significant for microscopic fibrosis and granulomas in the kidneys and essentially total obstruction of the subglottis and upper trachea by a 3.5 × 1 × 1 cm mass of fibrosis and granulomas overlying circumferentially necrotic mucosa. This mass was centered on the crico-tracheal junction. Conclusions: Wegener's granulomatosis can lead to proliferative tissue growth with acute airway obstruction in the larynx and trachea, and death. Any patient with WG under age 20, as well as patients with WG and laryngotracheal symptoms (e.g. stridor, hoarseness, wheezing) would benefit from evaluation of the airway.     

Immune-mediated audiovestibular disorders in the paediatric population: A review

Recent studies show that several audiovestibular pathologies in the paediatric population may be immune-mediated. This is even more probable if the pathology is associated with a coexisting systemic autoimmune disorder. At this time, however, the current literature is limited to a few case reports, and little is known with regard to prevalence, diagnosis, and management of immune-mediated inner-ear disorders in children. This review aims to shed some light on clinical presentation, diagnosis, and treatment of paediatric immune-mediated inner-ear disorders. Sudden and progressive sensorineural hearing loss is discussed, in addition to some of the systemic autoimmune disorders commonly associated with immune-mediated audiovestibular pathology such as Cogan's syndrome, systemic lupus erythematosus, Behçet's disease, Sjögren's syndrome, and juvenile idiopathic arthritis.     

Old and New in MÉniÈre Disease

The clinical experience is increasing, but still there is no uniform understanding in the substrate of Ménière's disease. The criteria of the diagnosis is loose and great controversy exists in the therapeutic efforts. A bewildering number of concepts and methods have been suggested and praised as the best answers in solving the problem until the next claim of success shatters the popularity of earlier allegations. The array of articles in the world literature for the past 25 years treats the entity of Ménière's disease in general and from the viewpoint of etiology, pathology, histology, clinical diagnosis and treatment. This review is concerned primarily with the treatment aspect of the literature. All the published ideas, regimens and techniques have one significant feature in common. They all claim success but not in 100% of the cases. Recovery varies from about 60% to 80%. Those cases considered “improved” are 20% to 30% and the rate of failure is between 10% and 25%. The diagnostic tools and capabilities have improved considerably. For treatment, except for reasonable medical or surgical palliation, nothing more can be offered than was offered a half century ago.     

Pott's puffy tumor in a 23-month-old: Youngest known case of a rare disease

This case report describes a child who developed Pott's puffy tumor and was treated at the Children's Mercy Hospital in Kansas City. In addition to a discussion of a case, a review of the literature was completed on this topic describing the typical embryology and development of the frontal sinus, and the epidemiology, diagnosis, and treatment of Pott's puffy tumor. The patient was a 23-month-old boy who developed Pott's puffy tumor after recovery from influenza. The patient presented to the hospital with progressing edema of the unilateral eye that spread bilaterally within a few days. A CT scan demonstrated pansinusitis, developed frontal sinuses, right periorbital cellulitis, and medial forehead subperiosteal abscess. Functional endoscopic sinus surgery and transcutaneous abscess drainage were urgently performed and the patient made a full recovery following a course of ertapenem and levofloxacin. The presence of a developed frontal sinus in a 23-month-old is an unexpected radiologic finding. This case represents the youngest patient reported in the literature to develop this rare complication of frontal sinusitis.     

Laryngeal metastasis from colloid adenocarcinoma of the colon: report of a case

Metastatic involvement of the laryngeal is very rare, with around 150 cases reported to the literature. In eight of these cases, the primary tumor was a colon adenocarcinoma. We report the case of a 80 year-old woman treated of a colloid adenocarcinoma of 7 years earlier, referred to us for chronic and progressive dyspnea. Endoscopic examination showed a subglottic spherical mass, which caused an important compromise of the respiratory airway. Tomographic studies revealed also a thyroid mass. The patient was treated with a tracheostomy, resection of the subglottic mass (with intraoperative diagnosis of "mucin producing tumor"), and total thyroidectomy. The final pathologic diagnosis of the subglottic mass was a metastasis of colloid adenocarcinoma of the colon. In the literature reviewed there are no previous reports of metastatic involvement of the larynx with this type of colon adenocarcinoma. We discuss the clinical and radiological findings, and therapeutic options for metastasis to the larynx, as well as pathological differential diagnosis.     

鼻腔鼻窦孤立性纤维性肿瘤1例报道及文献复习

目的 探讨发生于鼻腔鼻窦的孤立性纤维性肿瘤(SFT)的临床诊治经验.方法 结合1例鼻腔及鼻窦SFT患者的病例资料,并回顾性分析国内外文献,对SFT的临床特点、诊断与治疗进行讨论.本例患者术前CT提示:左侧鼻腔鼻窦肿物性质不明,鼻腔息肉可能.予以鼻腔新生物活检后鼻腔出血量大,随即全麻下行鼻内镜左侧鼻腔鼻窦新生物切除术,并...