Functional optimisation in chondrosarcoma of the subglottis: a novel surgical approach.

Chondrosarcoma of the larynx is rare accounting for approximately 1% of laryngeal cancers; clear cell subtype is a rare variant. Given the low risk of occult nodal disease, they present a unique opportunity to maximise tissue preservation in order to optimise both recovery and long-term functional outcomes. We present a case of laryngeal clear cell chondrosarcoma causing critical airway obstruction. An emergency tracheostomy was performed and mapping biopsies were taken. The tumour originated from the cricoid and extended into both arytenoid superstructures precluding cricotracheal resection. A modified narrow-field laryngectomy was performed, preserving all pharyngeal mucosa and neurovascularly intact infrahyoids. Organ preservation surgery is preferred in the management of laryngeal chondrosarcoma. If laryngectomy is required, the surgeon must ensure that all uninvolved, functional tissue is preserved carefully to improve swallow and voice outcomes postoperatively. We describe a novel technique used to achieve this outcome.

Is OperaVOX a clinically useful tool for the assessment of voice in a general ENT clinic?

BACKGROUND: Objective acoustic analysis is a key component of multidimensional voice assessment. OperaVOX is an iOS app which has been shown to be comparable to Multi Dimensional Voice Program for most principal measures of vocal function. As a relatively cheap, portable and easily accessible form of acoustic analysis, OperaVOX may be more clinically useful than laboratory-based software in many situations. This study aims to determine whether correlation exists between acoustic measurements obtained using OperaVOX, and perceptual evaluation of voice. METHODS: Forty-four voices from the multidisciplinary voice clinic were examined. Each voice was assessed blindly by a single experienced voice therapist using the GRBAS scale, and analysed using OperaVOX. The Spearman rank correlation co-efficient was calculated between each element of the GRBAS scale and acoustic measurements obtained by OperaVOX. RESULTS: Significant correlations were identified between GRBAS scores and OperaVOX parameters. Grade correlated significantly with jitter (ρ = 0.495, p < 0.05), shimmer (ρ = 0.385, p < 0.05), noise-to-harmonic ratio (NHR; ρ = 0.526, p < 0.05) and maximum phonation time (MPT; ρ = -0.415, p < 0.05). Roughness did not correlate with any of the measured variables. Breathiness correlated significantly with jitter (ρ = 0.342, p < 0.05), NHR (ρ = 0.344, p < 0.05) and MPT (ρ = -0.336, p < 0.05). Aesthenia correlated with NHR (ρ = 0.413, p < 0.05) and MPT (ρ = -0.399, p < 0.05). Strain correlated with Jitter (ρ = 0.560, p < 0.05), NHR (ρ = 0.600, p < 0.05) and MPT (ρ = -0.356, p < 0.05). CONCLUSIONS: OperaVOX provides objective acoustic analysis which has shown statistically significant correlation to perceptual evaluation using the GRBAS scale. The accessibility of the software package makes it possible for a wide range of health practitioners, e.g. general ENT surgeons, vascular surgeons, thyroid surgeons and cardiothoracic surgeons to objectively monitor outcomes and complications of surgical procedures that may affect vocal function. Given the increasing requirement for surgeons to monitor their outcomes as part of the move towards 'surgeon reported outcomes' this may become an invaluable tool towards that goal.

Laryngeal Trauma Following an Inhalation Injury: A Review and Case Report.

The primary concern when managing a patient with inhalation injury is security of the airway. Airflow may be impeded by both edema of the upper airway and reduction of oxygen delivery to the lower respiratory tract. Although there has been much discussion regarding management of the latter, the focus of this article is the management of the former. This review aimed to determine the optimum management in burn victims with upper airway inhalation injury as an attempt to prevent laryngeal trauma leading to long-term voice disorders and upper airway dyspnea. We describe the case of a 57-year-old woman with significant inhalation injury and discuss the natural progression of her injuries and the laryngeal controversies surrounding her care. We conclude with advice on the optimal management of this condition based on our experience, combined with current best evidence.

Parathyroid carcinoma.

PURPOSE OF REVIEW: This article highlights recent advances in our understanding of the incidence, epidemiology, clinical presentation, evaluation, diagnosis, treatment, and prognosis of parathyroid carcinoma. RECENT FINDINGS: The prevalence of parathyroid carcinoma is approximately 0.005% of all cancers. Therefore, parathyroid carcinoma is one of the rarest malignancies known. Patients with parathyroid carcinoma present with clinical symptoms of hypercalcaemia as these cancers are usually hormonally functional. It is not uncommon that patients present with complications of profound hypercalcaemia because of an elevated parathyroid hormone. Parathyroid carcinoma is difficult to diagnose preoperatively unless patients present with metastatic disease. Serum calcium often exceeds 14 mg/dl and serum parathyroid hormone is significantly elevated commonly between three and 10 times of the upper limit. Fine needle aspiration is not recommended because of the risk of parathyromatosis. Treatment includes surgery as a primary form of therapy and this usually follows with postoperative radiotherapy, although its use remains controversial. SUMMARY: Patients with parathyroid carcinoma should undergo adequate surgical excision with an attempt of preserving vital structures such as the recurrent laryngeal nerve. Often en bloc resection of the ipsilateral thyroid lobe with comprehensive level VI dissection is required. Postoperative radiotherapy should be considered in most cases.

Serious neonatal airway obstruction with massive congenital sublingual ranula and contralateral occurrence.

BACKGROUND: Congenital ranulas seldom occur, with bilateral presentation and prenatal diagnosis reported very rarely. We believe this is the first reported case of a neonate with a antenally diagnosed massive congenital ranula, who went on to develop a non-contiguous contralateral ranula, both contributing to obstruction in a complex paediatric airway. CASE REPORT: A female neonate was born to a non-primagravid mother via a planned elective caesarean section due to a lower facial defect and oral cyst. Antenatal aspiration of the pseudocyst was performed under ultrasound guidance with limited success. In the immediate post-natal period a poor airway was observed and the cyst was subsequently marsupialised. With the development of macroglossia secondary to oedema and tongue base collapse the airway was secured through surgical tracheostomy. A subsequent ultrasound scan revealed the presence of a second solitary cystic mass on the contralateral side. After careful excision of the contralateral pseudocyst, tongue function improved, with the resolution of a safe airway which permitted successful decannulation. A planned definitive procedure antenatally did not result in the anticipated improvement in function. However the subsequent development of a second non-contiguous pseudocyst and further surgical management resulted in a safe airway, improved masticator function and the ability to thrive. CONCLUSIONS: The prenatal diagnosis of congenital ranulas have been seldom reported, with no reported cases of contralateral occurrence and airway obstruction from an intraoral ranula. This rare case highlights the need for a well considered contingency plan when surgery is required for a neonatal airway at risk.

Infiltrative mass of the skull base and nasopharynx: A diagnostic conundrum.

Inflammatory skull base masses are enigmatic and often behaviourally unpredictable. We present a case of idiopathic hypertrophic pachymeningitis (IHP) forming a central skull base mass to illustrate the process required when one investigates such skull base lesions. This is the first description of mass forming or tumefactive IHP extending into the nasopharynx. A 32-year old woman presented with frontal headaches and nasal discharge. She then deteriorated and was admitted with worsening headaches, serosanguinous nasal discharge and bilateral ophthalmoplegia. Multimodality imaging confirmed a destructive central skull base soft tissue mass involving the posterior clivus, floor of sphenoid sinus, nasopharynx and extending into both cavernous sinuses. Unfortunately, the patient continued to deteriorate despite treatment with broad-spectrum antibiotics. Cerebrospinal fluid, blood tests and transnasal biopsies for histology and microbiology did not reveal a diagnosis. Further neuroimaging revealed extension of the mass. Early corticosteroid treatment demonstrated radical improvement although an initial reducing regime resulted in significant rebound deterioration. She was stable on discharge with slowly reducing low dose oral prednisolone and azathioprine. We discuss the complexity of this case paying special attention to the process followed in order to arrive at a diagnosis of idiopathic hypertrophic pachymeningitis based on both the clinical progression and the detailed analysis of serial skull base imaging. Knowledge of the potential underlying aetiologies, characteristic radiological features, common pathogens and the impact on blood serology can narrow the potential differentials and may avoid the morbidity associated with extensive resective procedures.

Embolic suppurative thyroiditis with concurrent carcinoma in pregnancy: lessons in management through a case report.

BACKGROUND: The thyroid undergoes a variety of physiological changes during pregnancy. The relatively low iodine levels seen in pregnancy have been implicated in thyroid growth during this time. Management of thyroid cancer in pregnancy is not immediately apparent. Furthermore, acute suppurative thyroiditis is rare and this is attributed to the glands innate immunity. We thoroughly review the evidence regarding management of thyroid abscess and thyroid malignancy during pregnancy and illustrate it via an extremely rare case of an embolic thyroid abscess highlighting an underlying carcinoma in a pregnant woman. CASE: A 29-year old female was found to have a thyroid mass during an antenatal assessment. Following a wound infection from Caesarian section she developed a rapidly progressive thyroid abscess. Incision and drainage of the abscess, and subsequent histology revealed papillary carcinoma. She subsequently underwent both total thyroidectomy with level 6 dissection and radio-iodine ablation post-natally. CONCLUSION: The literature is inconsistent regarding pregnancy as a risk factor for thyroid cancer, but overall it has been suggested as equally or slightly more frequent than in the non-pregnant population. Thyroid mass investigation should be as for the non-pregnant population. In the first trimester any endocrine surgery is associated with miscarriage, whereas these risks are reduced in second trimester. Importantly, there is no survival benefit in undergoing papillary carcinoma surgery in the third trimester versus early post partum and the risks of premature labour may outweigh any benefit gained by operating early. Most importantly, acute suppurative thyroiditis is rare entity and clinicians should have a low threshold for suspicion of underlying malignancy in these patients. This is especially true in the pregnant population who may be especially susceptible whilst undergoing hypertrophic thyroid changes.

Kasabach-Merritt syndrome in a child with upper airway compromise and spontaneous periorbital bruising.

A hemangioma that rapidly increases in size has the potential to trap platelets and cause a consumptive coagulopathy. We describe the case of an 18-week-old boy who was brought to a local emergency department with ecchymosis on his nasal bridge and medial epicanthi, as well as a subconjunctival hemorrhage. He was noted to be anemic and thrombocytopenic. Packed red blood cells and platelets were transfused. However, despite hematologic correction, the ecchymosis and petechiae worsened, and a mass became evident in the right posterior triangle of the patient's neck. Computed tomography demonstrated a lobular soft-tissue-density mass in the right posterior triangle that extended to the level of the skull base. Histologic analysis of a biopsy specimen revealed that the lesion was a giant kaposiform hemangioma. The patient was diagnosed with Kasabach-Merritt syndrome, and prednisolone was commenced as a first-line treatment. However, the mass continued to grow, resulting in inspiratory stridor. Magnetic resonance imaging revealed encroachment into the thecal sac and compression of the spinal cord. The lesion was embolized, and vincristine therapy was commenced. Following a second embolization, the size of the lesion decreased and no further blood products were required. The hemangioma was deemed to be unresectable. The successful treatment in this case was dependent on the maintenance of hemostasis, the initial medical treatment with a corticosteroid, repeat embolization, and longer-term control with vincristine.

Cochlear implantation in a patient with sensori-neural deafness secondary to Charcot-Marie-Tooth disease.

OBJECTIVE AND IMPORTANCE: Charcot-Marie-Tooth (CMT) disease is the most common hereditary motor and sensory neuropathy and can result in profound sensori-neural hearing loss with deficiency in speech perception out of proportion to that which would be expected if the loss was cochlear in origin. This study investigates whether the reintroduction of auditory synchrony by means of cochlear implantation will improve speech perception in those with dys-synchrony related to impairment of temporal processing abilities secondary to CMT. CLINICAL PRESENTATION: A 67-year-old male presented with a gradual but significant decrease in his hearing as part of a slowly progressing demyelinating peripheral neuropathy. On open-set speech discrimination he scored 0%. INTERVENTION: A Med-el Flex(SOFT) cochlear implant (CI) was fully inserted into the left ear with no surgical complications. The CI speech processor was fitted 1 month post-implantation and standardized speech assessments conducted at 1 week, 3 months, 9 months, and 21 months following initial fitting, gave open-set speech discrimination scores of 0, 0, 53, and 54%, respectively. CONCLUSION: This report demonstrates that cochlear implantation is an option to rehabilitate severe-to-profound hearing loss in adults with auditory dys-synchrony secondary to CMT disease. Progress post-implantation is likely to be slower than for the average CI user.