The Effect of Midface Advancement Surgery on Obstructive Sleep Apnoea in Syndromic Craniosynostosis.

BACKGROUND: Children with syndromic craniosynostosis frequently suffer from obstructive sleep apnoea (OSA). The aim of the authors' study was to investigate if midface advancement surgery for patients with SC improved the severity of OSA by examining the results of sleep studies before and after surgery. METHODS: A retrospective comparison of the pre and postoperative sleep study data of children undergoing midface advancement surgery at Great Ormond Street Hospital between 2007 and 2016. RESULTS: A total of 65 children underwent midface advancement surgery between 2007 and 2016 at Great Ormond Street Hospital and had recorded pre- and postoperative sleep studies. Thirteen patients were excluded from the analysis as their sleep study techniques before and after surgery were not comparable (e.g., different conditions with prong/continuous positive airway pressure use). Fifty-six percent of the patients were treated by monobloc surgery and the remainder with bipartition surgery. A greater proportion of patients had a normal OSA grading following midface advancement (42.3% postoperatively vs. 23.1% preoperatively, P = 0.059) although no statistically significant categorical changes in OSA grade were observed. Seventy-one percent of the patients had a decrease in Apnoea-Hypopnoea Index after surgery (21 patients 2011 onward). Similarly, there was no significant change in median oxygen desaturation index or in oxygen saturation nadir following surgery. CONCLUSION: The authors report one of the largest reviews of the effects of midface advancement surgery on sleep study parameters. Most patients showed improvements in Apnoea-Hypopnoea Index and OSA grading, although measures of oxygenation showed no consistent change.

Head and neck teratomas in children--A series of 23 cases at Great Ormond Street Hospital.

OBJECTIVE: Head and neck teratomas are rare and malignant change is rarer still. This is a report detailing all of the teratomas treated by the ear, nose and throat or craniofacial teams over the last 28 years at our institution. Examining the common presenting and radiological features as well as our success rates compared to the current literature. METHOD: A retrospective review of all cases presenting to our institution with a head and neck teratoma were analysed. Data regarding the following characteristics were collected: antenatal history, clinical features, biological serum makers, radiological and pathological characteristics. Surgical treatment, rates of reoccurrence and the degree of post-operative follow up were also analysed. RESULTS: 23 cases in total were included: 8 cervical, 6 nasopharyngeal, 5 thyroid, 2 thymus, 2 temporal. One had malignant change. The majority of children presented at birth with respiratory distress, 5 cases were picked up antenatally and one case presented at 10 years of age. All were treated surgically; with complete excision in 20 patients. No clinical recurrence occurred but further surgery was performed for radiologically suspected residual disease in one case. DISCUSSION: This is the largest detailed case series in literature in regard to head and neck teratomas. Illustrating that this is frequently a benign disease process disease in the head and neck region and has an excellent long term prognosis following surgery. In the situation of incomplete resection careful meticulous follow up with radiological imaging and a multidisciplinary team approach is a safe and viable alternative.

Primary cervical ganglioneuroblastoma.

OBJECTIVES: Ganglioneuroblastomas represent a histological subgroup of the rare neuroblastic tumours with intermediate malignant potential arising from neural crest progenitor cells of sympathetic nerves. Diagnosis can often be difficult based on imaging alone. We describe 4 cases of children presenting with a solitary neck mass with histology ultimately revealing ganglioneuroblastoma. METHODS: A retrospective case note review was carried out of all patients with cervical ganglioneuroblastoma seen at Great Ormond Street Hospital, UK. RESULTS: Mean age at presentation was 5 years. Based on imaging, the initial diagnoses for three of the cases were: lymphatic malformation, carotid body tumour, paraganglioma, respectively, whilst the remaining case had an immediate incisional biopsy revealing the correct diagnosis. All cases were managed by surgical excision with no evidence of recurrence after a median follow up of 6 years. CONCLUSION: Otolaryngologists should be aware of ganglioneuroblastoma when establishing the differential diagnosis of a child presenting with a neck mass. Biopsy is recommended as the gold standard investigation to avoid an incorrect diagnosis.

Are randomised controlled trials involving adenotonsillectomy well reported?

INTRODUCTION: Evidence-based medicine guides clinical practice. Currently, the evidence base on adenotonsillectomy is under scrutiny to establish clinical guidelines. It is therefore important that reports of clinical trials are of high quality. Guidance on reporting of randomised controlled trials (RCTs) are available in the Consolidated Standards for Reporting Trials (CONSORT) statement first published in 1996 and revised in 2001 and 2010. METHODS: A review of randomised controlled trials on adenotonsillectomy published after 2001 was undertaken. Each report was systematically assessed using the checklist of items from the CONSORT statement. RESULTS: Twenty-five trials were identified. All trials, except one, were identified as a randomised controlled trial by title or abstract. Twenty percent of trials reported a sample size calculation. A third of trials reported their method of generating a random allocation sequence. Similarly, a third stated the method of implementing the random allocation. A fifth of trials reported a clear flow of trial participants, with only a single trial reporting this with the aid of a diagram. CONCLUSION: This review shows the quality of reporting needs to be improved. Critical appraisal of poorly reported trials may result in erroneous conclusions, even though these trials may have been carried out with rigorous adherence to a protocol of high standard. Authors of clinical trial reports should be encouraged to consult the CONSORT statement.

Kikuchi-Fujimoto Disease: A case report and review of the literature.

Kikuchi-Fujimoto Disease is a rare benign, condition of necrotising histiocytic lymphadenitis. A case of a 55 year old gentleman is described here. He presented with fevers, weight loss and tender cervical lymph nodes. Kikuchi-Fujimoto disease was diagnosed after cervical lymph node biopsy. Symptomatic treatment was provided and an uneventful full recovery was made. Of significant note, this patient's daughter had Kikuchi-Fujimoto disease almost a decade earlier. Although Kikuchi-Fujimoto disease has been reported widely, we believe this is the first familial report in the literature of a case of Kikuchi-Fujimoto disease occurring in a parent and child.

Perception by the public and medical profession of oral and maxillofacial surgery--has it changed after 10 years?

Departments of oral and maxillofacial surgery (OMFS) aim to deliver an efficient and effective service. Clear understanding of what the speciality offers is central to appropriate referrals. Studies published 10 years ago showed there was limited awareness of the specialty. We present the results of a simple questionnaire study and compare the results with those of a previous survey. There has been some improvement in the perception of the role of OMFS, but only 34/100 of the public had heard of OMFS and none expected OMFS to treat patients who were unhappy with their facial appearance.