A Study on the Distinctive Clinical Profile and Thrombophilia in Pediatric Cerebral Venous Sinus Thrombosis.

Aim: The objective of the study was to systematically bring out the clinical presentations, neuro-imaging features, treatment given, and long-term outcomes of children with cerebral venous sinus thrombosis (CVST). Materials and Methods: Data were obtained by retrospective analysis of electronic records spanning 13 years, of children (<18 years) with a confirmed diagnosis of CVST based on magnetic resonance imaging of the brain and managed at a tertiary care children's hospital in the UK. Results: Seventeen patients with pediatric CVST were identified over a 13-year study period, highlighting the uncommon prevalence of this entity. This study comprised 10 males and seven females. The age range at presentation was between 2 days and 17 years with a median age of 5.5 years. Headache was the commonest presenting symptom in 10 of 17 children and focal neurological signs were seen in 11 of 17 patients. Among risk factors, six patients had an antecedent infection of the ear/mastoid, three children had acute leukemia, and two patients had central venous catheters. Para-infectious CVST (seven of 17 patients) responded well to appropriate antibiotic therapy. Thrombophilia screens were available in 10 of 17 patients with noninfectious CVST and returned abnormal in four patients (two with Factor V Leiden mutations and one each with deficiency of protein C and anti-thrombin III). Anticoagulants were used in only six of 17 cases and were generally well tolerated. Follow-up data revealed, 11 of 17 patients had a complete recovery and four of 17 patients had residual neurological deficits. Two children died in the entire cohort. Conclusion: Pediatric CVST is uncommon and has a different spectrum from adults, with unique clinical triggers and thrombophilic states. Management varies significantly among clinicians, due to the paucity of trial evidence and also due to the heterogeneity of this condition in children.

Oral ranula in an HIV-positive patient: case report and literature review.

We describe the presentation and treatment of an HIV-positive patient with an oral ranula, and review the literature. Ranulas are mucoceles or retention cysts formed by the extravasation of mucus from the sublingual gland, presumably due to continued production of saliva in the presence of ductal obstruction. Oral ranulas in children are rare and the overall prevalence of mucoceles has been reported as 0.08% in children aged 0-12 years. However, there has been a documented increased occurrence in HIV-positive patients. This has been attributed to a blockage of the salivary gland by inflammation and peri-ductal fibrosis following HIV-associated salivary gland disease. Oral lesions may indicate infection with HIV and can also predict progression of HIV to AIDS. The most common oral manifestation is oral candidiasis occurring in 67% of children with HIV. Following this salivary gland disease, periodontal and gingival disease and herpes simplex are the next most common. The exact prevalence of ranulas in an HIV population is not known but the occurrence of a paediatric patient with HIV having at least one oral lesion has been documented as high as 63% and salivary gland disease at 50%. The true extent of the relationship between HIV and ranula is as yet unknown. This represents the only reported case of oral ranula in an HIV-positive patient in the UK.

Tonsillectomy: a cost-effective option for childhood sore throat? Further analysis of a randomized controlled trial.

OBJECTIVE: To compare the estimated cost-effectiveness of childhood (adeno)tonsillectomy vs medical therapy for recurrent sore throats from the intention-to-treat (ITT) analysis of a randomized controlled trial (RCT) with that modeled on the recorded timing of surgical interventions as observed in all participants irrespective of their original group allocation. STUDY DESIGN: A pragmatic RCT (trial) with a parallel nonrandomized patient preference group (cohort) of (adeno)tonsillectomy vs medical therapy. SETTING: Five secondary care UK otolaryngology departments. SUBJECTS AND METHODS: Eligible children, aged 4 to 15 years, were enrolled to the trial (268) or cohort (461) groups. Outcomes included sore throat diaries, quality of life, and general practice consultations. The RCT protocol ITT analysis was compared with an as-treated analysis incorporating the cohort group, modeled to reflect the timing of tonsillectomy and the differential switch rates among the original groups. RESULTS: In the RCT ITT analysis, tonsillectomy saved 3.5 sore throats, whereas the as-treated model suggested an average reduction of more than 8 sore throats in 2 years for surgery within 10 weeks of consultation, falling to only 3.5 twelve months later due to the spontaneous improvement in the medical therapy group. CONCLUSION: In eligible UK school-age children, tonsillectomy can save up to 8 sore throats at a reasonable cost, if performed promptly. Further prospective data collection, accounting for baseline and per-trial preferences and choice, is urgently needed.

Childhood tonsillectomy: who is referred and what treatment choices are made? Baseline findings from the North of England and Scotland Study of Tonsillectomy and Adenotonsillectomy in Children (NESSTAC).

BACKGROUND: Tonsillectomies are frequently performed, yet variations exist in tonsillectomy rates. Clinicians use guidelines, but complex psychosocial influences on childhood tonsillectomy include anecdotal evidence of parental enthusiasm. Studies indicate that undergoing preferred treatment improves outcome. Despite the enthusiasm with which tonsillectomy is offered and sought, there is little evidence of efficacy. This resulted in a randomised controlled trial to evaluate the cost-effectiveness of (adeno)tonsillectomy in children with recurrent sore throats. OBJECTIVE: To compare characteristics of children entering the randomised trial with those recruited to a parallel, non-randomised study, to establish trends in referral and patient preferences for treatment. DESIGN: Baseline data from a randomised controlled trial with parallel non-randomised preference study, comparing surgical intervention with medical treatment in children aged 4-15 years with recurrent sore throat referred to five secondary care otolaryngology departments located in the north of England or west central Scotland. RESULTS: Centres assessed 1546 children; 21% were not eligible for tonsillectomy. Among older children (8-15 years), girls were significantly more likely to be referred to secondary care. Of 1015 eligible children, 268 (28.2%) agreed to be randomised, while 461 (45.4%) agreed to the parallel, non-randomised preference study, with a strong preference for tonsillectomy. Participants reporting that progress at school had been impeded or with more experience of persistent sore throat were more likely to seek tonsillectomy. Referred boys were more likely than girls to opt for medical treatment. Socio-economic data showed no effect. CONCLUSION: Preference for tonsillectomy reflects educational impact and recent experience, rather than age or socio-economic status.

Gut overgrowth of vancomycin-resistant enterococci (VRE) results in linezolid-resistant mutation in a child with severe congenital neutropenia: a case report.

A child with severe congenital neutropenia was monitored with microbiologic surveillance cultures for 3 years. He had recurrent bacterial infections and carriage of vancomycin-resistant enterococci. Resistance to linezolid emerged in the colonizing vancomycin-resistant enterococci after each course of this antibiotic when enterococci were present in overgrowth in the gut before treatment. The child was successfully treated for his congenital neutropenia by unrelated donor stem cell transplantation.

Protocol for north of England and Scotland study of tonsillectomy and adeno-tonsillectomy in children (NESSTAC). A pragmatic randomised controlled trial comparing surgical intervention with conventional medical treatment in children with recurrent sore throats.

BACKGROUND: Uncertainties surrounding the effectiveness and cost-effectiveness of childhood tonsillectomy for recurrent sore throat led the NHS Health Technology Assessment Programme to commission this research to evaluate the effectiveness and cost-effectiveness of tonsillectomy and adeno-tonsillectomy in comparison with standard non-surgical management in children aged under 16 with recurrent throat infections. The aim is to evaluate if tonsillectomy and adeno-tonsillectomy reduces the number of episodes of sore throats among children to a clinically significant extent. METHODS/DESIGN: A simple prospective pragmatic randomised controlled trial with economic analysis and prospective cohort study of non-trial participants comparing surgical intervention with conventional medical treatment. The treatment arm will receive tonsillectomy and adeno-tonsillectomy while in the control arm non-surgical conventional medical treatment only will be used. The primary outcome measure will be reported number of episodes of sore throat over two years with secondary outcomes measures of reported number of episodes of sore throat, otitis media and upper respiratory tract infection which invoke a GP consultation; reported number of symptom-free days; reported severity of sore throats and surgical and anaesthetic morbidity. The study will take place in five hospitals in the UK. The trial population will be 406 children aged 4-15 on their last birthday with recurrent sore throat referred by primary care to the 5 otolaryngology departments. The duration of the study is seven years (July 2001-July 2008). DISCUSSION: As with all pragmatic randomised controlled trials it is impossible to control the external environment in which the research is taking place. Since this trial began a number of factors have arisen which could affect the outcome including; a reduction in the incidence of respiratory tract infections, marked socio-economic differences in consultation rates, the results from the National Prospective Tonsillectomy Audit and the Government's waiting list initiatives.

British Association of Paediatric Otorhinolaryngology members experience with recurrent respiratory papillomatosis.

OBJECTIVES: To establish current practice in the treatment of recurrent respiratory papillomatosis (RRP) in the UK. METHODS: Consultant members of the British Association of Paediatric Otorhinolaryngology (BAPO) were sent a questionnaire on current practice concerning the management of recurrent respiratory papillomatosis in the paediatric population. RESULTS: The response rate was 39.4% perhaps reflecting the relatively small number of otolaryngologists who treat this condition. Data were analysed from 41 respondents representing 27 departments for a total of 103 patients. A total of 13 patients (12.6%) received adjuvant medical therapies with the antiviral agent cidofovir accounting for 10 patients. Distal spread of RRP has occurred in 27 (26.2%) patients. There were six reported deaths due to progressive RRP. Various lasers (CO(2), KTP, and pulsed dye) are the preferred method of surgical removal of RRP in children. Spontaneous ventilation (65.3%) is the preferred method of anaesthesia. Half of the consultants do not routinely send for HPV subtyping and 75% send lesions for histological examination if there is a change in growth pattern. Two thirds of respondents do not routinely treat their patients with antireflux medication. CONCLUSION: There is a need for the establishment of a centralized national base to which all treating consultants can report their cases. The time has come for national multicenter controlled trials on the use of adjuvant interventions for the treatment of both severe and less severe RRP disease. Both of the above can be organized under the umbrella of BAPO.