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.

Long-term stability, survival, and tolerability of a novel osseointegrated implant for bone conduction hearing: 3-year data from a multicenter, randomized, controlled, clinical investigation.

OBJECTIVE: To compare the 3-year stability, survival, and tolerability of 2 osseointegrated implants for bone conduction hearing: a wide 4.5-mm-diameter moderately roughened implant with a rounded 6-mm abutment (test) and a 3.75-mm diameter as-machined implant with a conically shaped 5.5-mm abutment (control). STUDY DESIGN: In this randomized, prospective, controlled, multicenter clinical study, 77 adult patients were included. Test and control implants were randomly assigned in proportions of 2:1. The implants were loaded with the sound processor from 6 weeks postimplantation. Follow-up after surgery was conducted at 10 days; at 4, 6, 8, and 12 weeks; and at 6, 12, 24, and 36 months after surgery. At every visit, implant stability quotient (ISQ) values were recorded by means of resonance frequency analysis (RFA), and skin reactions were evaluated according to Holgers' classification. RESULTS: Statistically significantly higher mean ISQ values were recorded for the test implant compared with the control implant at each evaluation time point. Between 2 and 3 years after surgery, ISQ values decreased but remained above baseline values. Implant survival was high for both implants: 96.2% of the test implants and 100% of the control implants survived these 3 years. Statistically significantly improved soft tissue outcomes were observed in the test implant group. CONCLUSION: This extensive long-term clinical investigation demonstrated that the test implant is more stable in terms of ISQ-values and provides high tolerability for the soft tissue. The results show that implant loading at 6 weeks is safe.

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.

Tracheostomy in mucopolysaccharidosis type II (Hunter's Syndrome).

OBJECTIVE: Patients with mucopolysaccharidosis type II (MPS II) may develop progressive multi-level upper airway obstruction. Despite the unique challenges presented by these complex patients, tracheostomy remains an important intervention to safeguard the airway when other interventions have failed or when the airway obstruction involves multiple sites. Airway involvement is largely responsible for the significant anaesthetic risk seen in MPS II. We reviewed our tertiary unit's experience of tracheostomies in patients with MPS II. STUDY DESIGN: Retrospective study. METHODS: Case note review of MPS II patients requiring tracheostomy at our tertiary institution. The primary outcome measure used for this study was complications following tracheostomy. RESULTS: We identified 10 MPS II patients requiring tracheostomy to manage upper airway obstruction. Mean age at which tracheostomy was 11 years 2 months (range 4 years 6 months to 28 years 10 months). Tracheostomy insertion was indicated in 3 scenarios: (1) to safeguard an anticipated difficult airway prior to a planned non-ENT surgical procedure, (2) to treat refractory progressive upper airway obstruction and (3) emergency airway management. Complications recorded included infratip and suprastomal granulations, local wound infection and skin ulceration from mechanical trauma. There were no immediate postoperative complications. CONCLUSIONS: Progressive upper airway obstruction is common in children with MPS II. Tracheostomy is an effective way of managing airway obstruction when less invasive interventions are no longer adequate. Tracheostomy in these patients can be technically difficult and although the complications of tracheostomy in MPS II do not significantly differ from other patient groups, the implications and management complexity vary considerably. The impact of ERT on airway obstruction is not yet fully understood, with tracheostomies likely to remain an important airway adjunct in some patients who fail to respond to ERT, or in those patients surviving into adulthood. It is vital that a multidisciplinary team, comprising clinicians with experience in managing such patients, are involved in airway management of patients with MPS II to enable the best standard of care to be given. The significant additional implications of a tracheostomy in a patient with MPS II, in terms of safety, aftercare and potentially life-threatening complications must be discussed in detail with the patient's family and/or carers. LEVEL OF EVIDENCE: 2c.

Potential risk factors associated with the use of cidofovir to treat benign human papillomavirus-related disease.

BACKGROUND: Cidofovir is currently being used off-licence to treat different viral infections, such as benign low-risk human papillomavirus (HPV)-related recurrent respiratory papillomatosis (RRP). There are concerns over the safety of this practice as rat studies demonstrated a high malignant transformation rate. As yet, there are no clinical reports of cidofovir-induced malignant changes in humans. METHODS: Telomerase immortalised human keratinocytes (hTert) stably expressing E6 proteins from either low-risk HPV6b or high-risk HPV16 and vector control cells were treated with either low-dose (5 microg/ml) or higher dose (30 microg/ml) cidofovir for 2 days and the effects evaluated by clonogenic survival assays. Based on these results, gene expression microarray analysis was performed on cidofovir-treated low-risk E6 and vector cells before, during and after drug treatment, and the results verified by real-time PCR. RESULTS: Both low-risk and high-risk E6-expressing cells show significantly improved long-term survival compared with vector control cells when exposed to 5 microg/ml cidofovir for 2 days, (hTert T6E6 P=0.0007, hTert T16E6 P=0.00023 and hTert vector control P=0.62). Microarray and real-time PCR analyses of low-dose cidofovir-treated low-risk E6-expressing cells revealed changes in gene expression that are known to be associated with malignant progression, which were not observed in drug-treated vector control cells. CONCLUSIONS: This is the first report that cidofovir can both increase cell survival and induce alterations in gene expression that are known to be associated with malignant transformation in cells transduced only with the E6 gene from low-risk HPV. It is our belief that these data provide cause for concern over the off-license use of this drug to treat RRP.

A survey of parent's attitudes towards viewing intraoperative photographs used as an educational tool.

OBJECTIVES: To assess the usefulness of intraoperative photographs taken during paediatric upper airway endoscopy used as an educational tool for parents and to define their attitudes towards seeing these photographs. DESIGN AND SETTING: Questionnaire based survey of 50 parents at a tertiary referral centre for paediatric otolaryngology in North West England. RESULTS: The response rate was 82%. All parents wanted to see the intraoperative images and reported improved understanding of their child's condition. No parents reported lasting anxiety. 79.2% of parents seeing photographs for the first time found the images to be informative; 82.4% of parents reported reassurance from being able to see progress in treatment; 17.6% found it easier to accept further intervention from seeing the photographs. CONCLUSION: Intraoperative photographs are useful as an educational tool for parents; they provide reassurance and help parents accept clinical management.

Cidofovir induces an increase in levels of low-risk and high-risk HPV E6.

BACKGROUND: Cidofovir is a nucleoside analogue that is used off-license to treat recurrent respiratory papillomatosis (RRP) caused by HPV6/11. However, the effect of this drug upon low-risk HPV 6/11 gene expression is unknown. METHODS: The expression of E6 was evaluated by RT-PCR in HPV-ve C33A cervical carcinoma cells stably transfected with both low- and high-risk HPV E6 cDNA's and in SiHa (HPV16+ve) cervical carcinoma cells after treatment with 2 doses and durations of exposure to cidofovir. RESULTS: Compared to the vector only transcript, E6 RNA levels showed an 8-fold increase in low-risk and 20-fold increase in high-risk E6-expressing cells. High-risk E6 protein levels were also detected by Western blot in cidofovir-treated C33A Type16 E6-transfected cells. CONCLUSION: These data may indicate a potential rationale for increased risk of genetic instability and thus transformation due to drug-induced increase in the level of E6.

Acquired paediatric subglottic cysts: a series from Manchester.

UNLABELLED: Subglottic cysts (SGC) have long been regarded as a rare cause of airway obstruction but through increased awareness an increase in the number of cases of SGC has been reported. OBJECTIVE: This paper describes the pathogenesis and management of SGC. DESIGN: Retrospective case series. Ethical approval not sought. SETTING: Royal Manchester Children's Hospital. PATIENTS: Two hundred and six new referrals for direct laryngotracheobronchoscopy (DLTB) were identified from records between September 2003 and September 2005. MAIN OUTCOMES MEASURED: Age at birth, sex, length of intubation, presenting symptoms, age at presentation, DLTB findings, interventional procedures, and follow-up DLTBs. RESULTS: Fourteen out of 206 (6.8%) infants were diagnosed as with subglottic cysts. This represented the fourth most common cause of upper airway pathology. Thirteen out of 14 (93%) infants were preterm (26.8 weeks S.D. 25.3-28.3 weeks). All infants had been intubated ranging from 1 to 180 days (median 42 days). The onset of symptoms ranged from 1 to 13 months (median 4.25 months). Initially, 8/14 (57.2%) infants had SGC cysts marsupialised with microforceps. A further six cysts (50%) were decapped between 2 and 4 months and one between 6 and 12 months. CONCLUSION: The number of cases of SGC has been increasing over the last three decades and represents the fourth most common causes of airway obstruction in our series. There is a delay in onset of symptoms and high rate of recurrence in the first 4 months. It is therefore prudent to reschedule further endoscopic evaluation between 2 and 4 months and after 6 months should the clinical need arise.

Effects of cidofovir on a novel cell-based test system for recurrent respiratory papillomatosis.

BACKGROUND: Cidofovir has been reported to have activity against human papillomavirus (HPV) type 16, but no laboratory studies have been performed on HPV type 6, the main cause of recurrent respiratory papillomatosis (RRP). METHODS: HPV6b E6 cDNA-based C33A (non-HPV cervical carcinoma) cell line was produced. Two different doses of cidofovir were applied to parent C33A, C33AT6E6, and C33AT16E6 (HPV 16). Growth and flow cytometry analysis were performed. RESULTS: Polymerase chain reaction confirmed HPV6 E6 expression in C33AT6E6 cells. High-dose cidofovir was found to be toxic to all cell lines. Low-dose exposure was found to be toxic to C33AT16E6 cells at 3 days, whereas C33A and C33AT6E6 showed minimal toxicity at 6 days and earlier recovery following drug withdrawal. CONCLUSIONS: Cidofovir showed nonspecific toxicity against all 3 cell lines tested. HPV16 E6 expressing cells were more sensitive than parent or HPV6 E6 expressing cells. Cidofovir has no selective advantage for the RRP-related HPV6 E6 expressing cell line.