Head and Neck Medicine by Alexandros G.Sfakianakis

Publication date: September 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 100
Author(s): Susan P. Jacups, Denise Newman, Deborah Dean, Ann Richards, Kate M. McConnon
IntroductionOn a background of high rates of severe otitis media (OM) with associated hearing loss, children from the Torres Strait and Cape York region requiring ear, nose and throat (ENT) surgery, faced waiting times exceeding three years. After numerous clinical safety incidents were raised, indicating a failure of the current system to deliver appropriate care, the governing Hospital and Health service opted to deliver surgical care through an alternate process. ENT surgeries were performed on 16 consented children from two remote locations via the private health care system, funded by a health provider partnership.MethodsWe examined the collaboration processes alongside clinical findings from this ENT surgery. Collated patient data, included patient demographics, clinical and audiometry presentation features were reviewed and compared pre and post-operatively. Cost savings associated with the use of TeleHealth post-operatively were briefly examined.ResultsSurgeries were successfully completed in all 16 children. The reported mean waitlist time for ENT surgery was 1.2 years. Pre-surgery pure-tone average hearing thresholds were reported at left: 30.9 dB, right: 38.2 dB. The majority of presentations were for bilateral OM with Effusion (69%). Post-surgical follow up indicated successful clinical outcomes in 80% of patients and successful hearing outcomes in 88% of patients. Mean difference pure-tone average hearing thresholds, left: 8.4 dB and right: 11.2 dB. Furthermore, the majority of patients reported improved hearing and breathing. The use of TeleHealth for post-operative review enabled a minimum cost saving of AUD$21,664 for these 16 children. Overall, a high level of staffing resources was required to successfully coordinate this intense surgical activity.ConclusionThis innovative approach to a health system crisis enabled successful ENT surgical and hearing outcomes in 16 children, whose waitlisted time grossly exceeded state health recommendations. Using private health facilities funded by a health partnership, while unlikely to be a suitable model of care for routine service delivery; may be applied as an adjunct service model when blockages and delays lead to sub-standard service provision. This approach may be applicable to other health care facilities when facing extended elective surgery wait times in ENT or other specialty areas.

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The multivariate analysis of indications of rigid bronchoscopy in suspected foreign body aspiration

Publication date: September 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 100
Author(s): E. Divarci, B. Toker, Z. Dokumcu, A. Musayev, C. Ozcan, A. Erdener
ObjectiveForeign body aspiration (FBA) could be a serious life-threatening condition in children. Patients usually underwent bronchoscopy with suspicious of FBA alone. In this study, we aimed to determine which patients need to go to bronchoscopy based on pre-operative findings.MethodsRetrospective analysis of patients underwent bronchoscopy between 1999 and 2015 was performed. Clinical symptoms, witnessed aspiration event (WAE), physical examination findings (PEFs) and radiological findings (RFs) were analyzed by multivariate analysis to evaluate the indications of bronchoscopy.Results431 patients (266M, 165F) underwent bronchoscopy with a median age of 2 years (7 months-16 years). A foreign body was detected in 68% of the patients. Univariate analysis demonstrated that wheeze was the sole distinctive clinical symptom for detection of FBA (p<0.001). The rates of positive WAE, PEFs and RFs were 83%, 71.7% and 36.9%, respectively. All of them were identified as independent predictive parameters in the detection of FBA by univariate analysis (p = 0.003&p<0.001&p = 0.015). Multivariate analysis was performed with considering the association between them. The rate of positive bronchoscopy was 91.3% in patients with positive WAE, PEFs and RFs together(84/92). In patients with a positive WAE alone who had not got PEFs and RFs, the rate of positive bronchoscopy was 34.2% (25/73). A foreign body was detected in 84% of the patients who had not got a WAE but positive PEFs and RFs together(21/25). Bronchial laceration was occurred in one patient during bronchoscopy. Pneumothorax was not seen in any of the other patients. The rate of mortality was 0.4% in the overall group (2 patients).ConclusionThe indications of bronchoscopy in suspected FBA are usually based on clinical suspicious. The definition of " suspicous" could be a WAE or positive PEFs and RFs. The association of these factors increase the rate of positive bronchoscopies. In the light of our study, the classical indication for suspected FBA is still valid as "suspicious requires bronchoscopy".

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Πέμπτη 20 Ιουλίου 2017

Concurrent Chondrodysplasia Punctata Type 2 (Conradi–Hunermann–Happle Syndrome) and Ichthyosis Vulgaris in Teenaged Twin Girls

Abstract

We present concurrent X-linked chondrodysplasia punctata and ichthyosis vulgaris in adolescent fraternal twin girls, notable for initial presentation with dry skin in adolescence, characterized by dark-brown scale typical of ichthyosis vulgaris and blaschkolinear, atrophic, scaly plaques. This constellation of findings prompted further genetic investigation. Using a multigene approach to examine 39 genes associated with congenital ichthyosis, next-generation sequencing revealed a novel heterozygous missense mutation at a mutational hotspot in the EBP gene c.439C>T (p.R147C) in conjunction with a single nonsense mutation in the FLG gene (p.R501X) in both sisters. These individuals highlight the clinical variability of Conradi–Hunermann–Happle syndrome, illustrate the possibility of co-occurrence of rare and common forms of ichthyosis, and demonstrate the utility of multigene analysis.

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Methotrexate for Severe Childhood Atopic Dermatitis: Clinical Experience in a Tertiary Center

Abstract

Background/Objectives

Atopic dermatitis (AD) affects up to 20% of children. Although the majority of patients are adequately controlled using emollients, topical corticosteroids, topical calcineurin inhibitors, or phototherapy, children with moderate to severe AD may require systemic treatment for control. The objective of the current study was to evaluate the efficacy and safety of methotrexate in children with severe AD attending a tertiary referral center.

Methods

A retrospective chart review was undertaken of all children who received methotrexate for severe AD at our tertiary referral center from November 2010 to August 2015.

Results

Forty-seven children were started on methotrexate for AD during this period. The mean Investigator Global Assessment (IGA) at the 3- to 5-month follow-up improved from 4.25 to 2.8, with further improvement to 1.9 in the patients that continued therapy beyond 10 months. Changes in the Children's Dermatology Life Quality Index (CDLQI) mirrored changes in the IGA, with improvement in the mean CDLQI from 14.4 at the start of the treatment to 7.5 at the 3- to 5-month follow-up. Further improvement in the CDLQI to 6.6 in patients who continued methotrexate beyond 10 months confirmed continued improvement in disease control beyond medium-term therapy. The treatment was well tolerated.

Conclusions

Methotrexate appears to be an effective, safe treatment for severe pediatric AD. Its therapeutic effects continue beyond the medium-term treatment period, as reflected by further improvement in IGA and CDLQI scores in patients who continued methotrexate therapy beyond 10 months.

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