To present the first reported case of Haemophilus influenzae type b epiglottitis leading to necrotising fasciitis.

Case report and review of the literature regarding the association of necrotising fasciitis with Haemophilus influenzae infection and with epiglottitis.

A previously well, 64-year-old woman presented with epiglottitis, and subsequently developed necrotising fasciitis of her chest wall. The cause of both infections was Haemophilus influenzae serotype b. This organism has frequently been implicated in epiglottitis, but has not previously been reported to cause simultaneous necrotising fasciitis. The patient became critically ill, requiring intensive care management, but following surgical debridement and antibiotic treatment she made a full recovery.

Although increasingly uncommon, clinicians must continue to be proficient in the diagnosis and management of epiglottitis, and to be aware of its full range of possible complications. This case report highlights a previously unknown and potentially fatal complication of Haemophilus influenzae type b epiglottitis.

We present a unique case in which closure of a large tracheoesophageal fistula was achieved with planned conservative management.

The literature was reviewed for other documented cases of spontaneous closure of traumatic tracheoesophageal fistula.

Acquired tracheoesophageal fistula may result secondary to a chemical burn from an alkaline disc battery impacted in the oesophagus, particularly when the presentation, and thus diagnosis, are delayed. This condition is rare. The majority of such cases occur in children, and are conventionally managed with surgical repair. We found only three previously reported cases in which conservative management was attempted.

Non-interventional management should be tried initially for the management of paediatric acquired tracheoesophageal fistula, to permit closure by secondary intention.

This study aimed to analyse vocal performance and to investigate the nature of the neoglottal sound source in patients who had undergone supracricoid laryngectomy with cricohyoidoepiglottopexy, using a high-speed digital imaging system.

High-speed digital imaging analysis of neoglottal kinetics was performed in two patients who had undergone supracricoid laryngectomy with cricohyoidoepiglottopexy; laryngotopography, inverse filtering analysis and multiline kymography were also undertaken.

In case one, laryngotopography demonstrated two vibrating areas: one matched with the primary (i.e. fundamental) frequency (75 Hz) and the other with the secondary frequency (150 Hz) at the neoglottis. In case two, laryngotopography showed two vibrating areas matched with the fundamental frequency (172 Hz) at the neoglottis. The interaction between the two areas was considered to be the sound source in both patients. The waveform of the estimated volume flow at the neoglottis, obtained by inverse filtering analysis, corresponded well to the neoglottal vibration patterns derived by multiline kymography. These findings indicated that the specific sites identified at the neoglottis by the present method were likely to be the sound source in each patient.

High-speed digital imaging analysis is effective in locating the sites responsible for voice production in patients who have undergone supracricoid laryngectomy with cricohyoidoepiglottopexy. This is the first study to clearly identify the neoglottal sound source in such patients, using a high-speed digital imaging system.

We report two cases of branchial cleft abnormalities investigated using magnetic resonance imaging.

There appears to be no clear imaging technique that is universally recommended for imaging branchial cleft abnormalities. Options include fistulography, computed tomography, magnetic resonance imaging and ultrasonography.

Case reports and literature review.

Two cases, although not unusual in themselves, are described to illustrate the use of magnetic resonance imaging to define the anatomy and to assist surgical planning.

Magnetic resonance imaging is able to accurately depict the extent and course of branchial cleft abnormalities, and in the current cases could have been relied upon to determine the necessary surgical procedure. Branchial cleft abnormalities are sufficiently rare for magnetic resonance imaging to be recommended as the first-line imaging modality.

To report an unusual observation whereby hearing was improved by insertion of cotton wool onto a retracted tympanic membrane, and to explore potential explanations for this.

We report the case of a 56-year-old woman with a severe mixed hearing loss who was noted to have a significant improvement in her hearing using a technique shown to her many years ago by a Russian physician. Upon application of a moist piece of cotton wool, gains of up to 40 dB were observed. This was associated with a significant subjective improvement in hearing. We discuss potential mechanisms for this effect, including amplification, a baffle effect and phase differences.

The mechanism of the hearing improvement observed in this case is unknown, but is likely to be related to sound amplification from the relatively large surface area of the cotton wool to the smaller oval window.

To report an unusual case of pseudoaneurysm formation following ingestion of a thin wire.

Case report, including management, and review of the world literature concerning pseudoaneurysm and its management.

A 15-year-old boy presented as an emergency with a two-week history of painful swallowing, and a one-week history of a progressively enlarging, right-sided swelling of the lower neck. A foreign body had been ingested two weeks previously. Radiological investigation showed a pseudoaneurysm of the right common carotid artery. The neck was explored, the foreign body removed and the common carotid artery repaired. The patient was discharged on the 10th post-operative day without neurovascular complication.

This is an unusual presentation of pseudoaneurysm of the common carotid artery following accidental ingestion of a foreign body, reported for its rarity and management.

Neck lumps in young adults are not uncommon, and most represent benign, reactive lymphadenopathy. Cystic swellings are less common. Spontaneous cervical lymphocoeles are very rare, and present as fluctuant, asymptomatic, cystic swellings in the neck in otherwise fit individuals.

We report two healthy young women who presented with spontaneous cervical lymphocoeles and who were treated successfully with surgical excision. The timing of surgical intervention was influenced in both cases by their imminent wedding celebrations.

Whilst sclerotherapy has been advocated by some authors, we found it unhelpful; however, surgery provided definitive treatment.

We report a case of internal carotid arterial damage following blunt neck trauma. This rare mechanism of injury demands a high index of suspicion to enable prompt specialist management.

A 22-year-old man presented to hospital after sustaining blunt neck trauma. Rapid onset of stridor necessitated an emergency tracheostomy. Computed tomography angiography demonstrated a tear of the right internal carotid artery, which was repaired surgically.

Blunt carotid vessel injury, although rare, has a high mortality rate. Mechanisms of injury include hyperextension and contralateral neck rotation, a direct blow to the vessel, and laceration by adjacent bony structures. The ‘gold standard’ investigation for suspected blunt carotid vessel injury is catheter angiography, although this carries a small risk of stroke. Computed tomography angiography is a less invasive, alternative investigation which has almost equivalent accuracy. The extent of damage to the vessel wall will dictate treatment. In our literature review, we discuss the presentation, investigation and different treatment modalities available.

This case highlights an unusual mechanism of carotid artery injury, with a delayed, potentially fatal presentation. Such injury demands a high index of suspicion, and confirmation with specific investigations. Management is hazardous and requires experienced personnel in all aspects of care.

We report a rare case of internal jugular vein duplications, in order to raise the level of awareness of this anomaly amongst ENT surgeons, radiologists and intensive care practitioners. We briefly review and discuss the related literature.

Duplicated internal jugular veins are a rare anatomical finding. They may be subclinical, or may present with neck swellings that may be mistaken for laryngocoeles or branchial cysts. We present a case of bilateral internal jugular vein duplication in a young adult. The referral was made on the basis of intermittent neck swelling, dyspnoea and dysphagia. Conservative treatment was instigated, and symptoms improved without surgical intervention.

Only a handful of cases of duplicated internal jugular veins have been reported. The current case is unique, as no previously reported cases have presented with dyspnoea and dysphagia. We suggest a conservative approach, as there is currently no evidence that duplicated internal jugular veins cause any adverse health outcomes.

We report a case of an elderly man receiving treatment with perindopril, who presented with angioedema of the left side of the tongue, floor of the mouth and upper neck. This affected his speech and swallowing, and occurred one day after a burr hole and evacuation procedure undertaken to treat a subdural haematoma. The patient was kept under close observation and treated with intravenous hydrocortisone. The angioedema resolved completely in two days. This is the third reported case of unilateral tongue angioedema occurring secondary to angiotensin-converting enzyme inhibitor use.

Case report and literature review concerning angiotensin-converting enzyme inhibitor induced angioedema.

Unilateral angioedema of the tongue is a rare adverse reaction to angiotensin-converting enzyme inhibitors. Early recognition may prevent unnecessary surgical intervention and complications.

Ossifying fibromyxoid tumour is a recently described, rare but morphologically distinctive soft tissue neoplasm characterised by a combination of myxoid and/or fibrous stroma with areas of ossification. Although most authors postulate a neuroectodermal origin for this peculiar tumour, there is no agreement in the literature regarding its histopathogenesis. To our knowledge, this is the first reported case of ossifying fibromyxoid tumour involving the sphenoid sinus.

Tumour of low cell density, composed of small, spindle-shaped or stellate cells with small, irregular nuclei set in a fibromyxoid stroma.

Following discussion at the skull base multidisciplinary team meeting, a combined surgical team including an otorhinolaryngologist and a neurosurgeon carried out resection of the lesion, using an endoscopic transnasal approach, followed by reconstruction of the defect.

An awareness of the distinctive histopathological features of ossifying fibromyxoid tumour, and of its clinical effects, is crucial to establishing a definitive diagnosis and thereby instituting appropriate management. This case report also reinforces the evolving role of the endoscopic transnasal approach in the management of inflammatory and neoplastic disease involving the skull base. This is increasingly being made possible by close collaboration between multiple surgical specialties, including otorhinolaryngology and neurosurgery.

Description of two siblings with unexplained, progressive, perceptive hearing loss after head trauma.

Case report.

Two siblings aged six and eight years old with bilateral, intermittent but progressive hearing loss.

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.

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.

We report the first documented case in the world literature of primary extra-pulmonary small cell carcinoma occurring in the neopharynx following laryngectomy.

We present a case report and a review of the world literature regarding the management of tumours of the neopharynx and extra-pulmonary small cell carcinoma.

The paucity of cases of extra-pulmonary small cell carcinoma has resulted in many departments managing this neoplasm similarly to pulmonary small cell cancer. However, the site of the primary can have an impact on disease survival and treatment options.

Neopharyngeal small cell carcinoma has not previously been reported. It should be managed in the same way as other extra-pulmonary small cell carcinomas occurring within the pharynx or larynx, with combined multi-drug chemotherapy and radiotherapy. Surgery has a limited role due to the aggressiveness of the disease and the high risk of local and distant spread at presentation.

To demonstrate the importance of thorough investigation of patients with Horner syndrome, and to explain the relevant anatomy.

A 46-year-old man presented with right-sided Horner syndrome. No other abnormality was found. Magnetic resonance imaging showed calcification of the stylohyoid ligament, with a pseudoarthrosis in the mid-portion of the ligament. This pseudoarthrosis was displacing and compressing the internal carotid artery and the adjacent sympathetic chain, causing Horner syndrome.

In this case, magnetic resonance imaging was invaluable in elucidating the cause of the Horner syndrome. This is the first described case of pseudoarthrosis of a calcified stylohyoid ligament causing Horner syndrome.

We report an extremely rare presentation of concomitant tuberculous cervical lymphadenitis in a patient with carcinoma of the larynx.

A 66-year-old man presented with a nine-month history of hoarseness. He was found to have an exophytic lesion over the posterior half of the right vocal fold. He also had a palpable right jugulodigastric node. Biopsy of the vocal fold lesion revealed invasive squamous cell carcinoma. Histological analysis of an excision biopsy specimen from the lymph node was highly suggestive of tuberculosis. On further assessment of the slides, one acid-fast bacillus was seen. The patient was treated with radiotherapy to the larynx and concomitant anti-tuberculosis chemotherapy. Five months following treatment, there was no sign of laryngeal cancer recurrence; however, the patient continued to have a productive cough and night sweats.

To our knowledge, this is the first report of a laryngeal carcinoma with concurrent tuberculous cervical lymphadenitis. In the face of an unhelpful fine needle aspiration cytology examination, an assumption of metastatic neck disease could have been made. Subsequent surgical and/or oncological intervention would have been highly inappropriate, with potentially catastrophic effects. This case highlights the importance of proper diagnosis, and emphasises the fact that tuberculosis should always be borne in mind in the differential diagnosis.

To describe a case of superficial temporal artery pseudoaneurysm occurring as a rare complication of parotid surgery (to the authors' knowledge, the first such case reported), to review the aetiology of superficial temporal artery pseudoaneurysm, and to highlight pseudoaneurysm as a cause of facial swelling after parotidectomy.

A literature search of the PubMed and EMBASE databases was carried out using the Medical Subject Heading key words ‘superficial temporal artery’, ‘aneurysm’, ‘pseudoaneurysm’ and ‘parotid’.

No previously reported cases of superficial temporal artery aneurysm or pseudoaneurysm following parotid surgery were identified.

Pseudoaneurysm of the superficial temporal artery is a rare complication of parotid surgery which has not previously been reported.

We report an extremely rare case of congenital cholesteatoma of the mastoid region, presenting as acute mastoiditis. We also review the 16 previously reported cases of congenital cholesteatoma of the mastoid region.

A 65-year-old man presented with left-sided, post-auricular swelling and pain. Acute mastoiditis was diagnosed, with computed tomography demonstrating destruction of the bony plates of the posterior cranial fossa and sigmoid sinus. Initial surgery revealed a cholesteatoma in the mastoid, with no extension into the aditus ad antrum or attic. These findings were confirmed by pathological and immunohistochemical analysis of the surgical specimen, the latter using involucrin. The cholesteatoma matrix was completely removed in a second operation.

Including this case, only four of the 17 reported cases of congenital cholesteatoma of the mastoid region showed post-auricular pain or swelling, indicating acute mastoiditis. Clinicians should bear in mind that congenital cholesteatoma may be present in patients presenting with mastoiditis, particularly adults.

Rosai–Dorfman disease is a rare, idiopathic, histiocytic proliferative disorder with a distinctive microscopic appearance, which was formerly thought to be a disease process limited to lymph nodes. However, extranodal involvement has been documented in less than half of the reported patients, but rarely without associated lymphadenopathy.

We report the case of a 43-year-old Senegalese woman who presented with a polypoid, intranasal mass caused by Rosai–Dorfman disease. A diagnosis of a granulomatous process, including rhinoscleroma, was initially discussed. The correct diagnosis was made histologically by demonstrating aggregates of histiocytes with large amounts of cytoplasm, emperipolesis and protein S100 antigen expression. Despite using ancillary methods (molecular biology and electron microscopy), we failed to demonstrate any associated pathogen.

Diagnosis of Rosai–Dorfman disease can be very difficult, in particular in adults from Africa with pure, isolated, intranasal localisation, in whom clinical and radiological features may mimic other infectious or neoplastic disorders. The diagnosis is made based on the histological presence of large histiocytes with lymphophagocytosis. Moreover, immunohistochemical analysis of these histiocytes using anti-protein S100 antibody shows strong positivity.

We report an extremely rare case of rhinosporidiosis with involvement of both larynx and trachea, together with coexisting nasal, nasopharyngeal and oropharyngeal lesions, in a 32-year-old man.

Case report and review of the world literature concerning laryngotracheal and disseminated rhinosporidiosis.

A 32-year-old, South Indian man presented with a nasal mass of four years' duration, with progressive hoarseness for one year. Strawberry-like rhinosporidial masses were seen in both nasal cavities. Fibre-optic laryngoscopic examination revealed reddish masses with whitish surface specks, involving the glottis, subglottis and trachea. Computed tomography revealed soft tissue opacities involving both nasal cavities and the nasopharynx and extending to the oropharynx, with involvement of the larynx and trachea. Direct laryngoscopy and rigid bronchoscopy guided excision of the laryngeal and tracheal lesions was performed.

Rhinosporidiosis is a chronic, granulomatous disease which usually affects the mucous membranes of the nose and nasopharynx. Lower dissemination into the trachea is extremely rare. Laryngotracheal involvement poses many diagnostic and therapeutic challenges, due to the potential risk of bleeding and aspiration. In the presented case, local spread of rhinosporidiosis, due to direct spillage of spores from the nasopharynx into the larynx during episodes of bleeding or previous surgery, may have resulted in laryngotracheal involvement, although systemic spread cannot be excluded.