Surgical Management of Submucous Cleft Palate by Radical Muscle Dissection Veloplasty: Speech Outcomes in Patients with 22q11.2 Deletion Syndrome.

OBJECTIVE: To evaluate the benefit of anatomical muscle dissection repair for velopharyngeal insufficiency (VPI) in patients with submucous cleft palate (SMCP) with 22q11.2 deletion syndrome. DESIGN: Retrospective blinded randomised analysis of a surgeon's management over 10 years. SETTING: The study was performed at a specialised Paediatric hospital in the United Kingdom. PATIENTS: Children with SMCP and 22q11.2 deletion syndrome. INTERVENTIONS: All participants underwent radical muscle dissection veloplasty. OUTCOMES MEASURED: Pre- and post- operative measurements included severity of anatomical defect, speech samples and lateral images which were digitised, randomised then externally and blindly analysed using validated techniques. Stata software was used to perform statistical analysis. RESULTS: 57 children with 22q11.2 deletion syndrome were included in this analysis. Intra-operatively, the majority of cases were identified as SMCP Grade I anomalies. Post-operatively, a statistically significant improvement in hypernasality, resting palate length, palate length at maximum closure, palate excursion and gap size at maximum closure was observed. Secondary surgery was performed for 59% of patients by ten years. CONCLUSION: Muscle dissection repair improves hypernasality, palate closure function and the closure gap in patients with 22q11.2 deletion syndrome. Although over 50% of patients may require further surgery, muscle dissection repair should be a first step due to its utility at a younger age, when invasive investigations are impossible, its lower morbidity, speech and language benefits or altering the plans for less obstructive secondary surgery when it lead to reduced velo-pharyngeal gap and improved palate mobility even when adequate velo-pharyngeal closure was not achieved.

Circulating MicroRNA Biomarkers in Melanoma: Tools and Challenges in Personalised Medicine.

Effective management of melanoma depends heavily on early diagnosis. When detected in early non-metastatic stages, melanoma is almost 100% curable by surgical resection, however when detected in late metastatic stages III and IV, 5-year survival rates drop to ~50% and 10⁻25%, respectively, due to limited efficacy of current treatment options. This presents a pressing need to identify biomarkers that can detect patients at high risk of recurrence and progression to metastatic disease, which will allow for early intervention and survival benefit. Accumulating evidence over the past few decades has highlighted the potential use of circulating molecular biomarkers for melanoma diagnosis and prognosis, including lactate dehydrogenase (LDH), S100 calcium-binding protein B (S100B) and circulating tumor DNA (ctDNA) fragments. Since 2010, circulating microRNAs (miRNAs) have been increasingly recognised as more robust non-invasive biomarkers for melanoma due to their structural stability under the harsh conditions of the blood and different conditions of sample processing and isolation. Several pre-analytical and analytical variables challenge the accurate quantification of relative miRNA levels between serum samples or plasma samples, leading to conflicting findings between studies on circulating miRNA biomarkers for melanoma. In this review, we provide a critical summary of the circulating miRNA biomarkers for melanoma published to date.

Submucous cleft palate: a systematic review of surgical management based on perceptual and instrumental analysis.

Objective : Submucous cleft palate (SMCP) is a congenital condition associated with abnormal development of the soft palate musculature. In a proportion of cases, this results in velopharyngeal insufficiency (VPI), the treatment for which includes pharyngeal flap surgery, pharyngoplasty, and palate reconstruction. The aim of this paper is to determine whether there is superiority of one or more types of surgical procedure over the others in improving speech in patients with VPI secondary to SMCP. Methodology : Nine databases, including MEDLINE and EMBASE, were searched between inception and January 2013 to identify articles published relating to the surgical management of SMCP. Only studies that reported outcome measures for postoperative speech were included in the systematic review. Results : Twenty-six studies analyzing the outcomes of surgery for VPI in patients with SMCP met the inclusion criteria. In these studies, speech outcomes were measured either in a binary fashion (i.e., normal speech or evidence of VPI) or using scales of VPI severity. Of the 26 studies, only two utilized blinded speech assessment, and 12 included both preoperative and postoperative speech assessment. Conclusions : The review found little evidence to support any specific surgical intervention. This is in large part due to the inclusion of mixed etiologies within study populations and the lack of unbiased validated preoperative and postoperative speech assessment. Further methodologically rigorous studies need to be conducted to provide a secure evidence base for the surgical management of SMCP.

Successful delayed nose replantation following a dogbite: arterial and venous microanastomosis using interpositional vein grafts.

The nose is one of the most important aesthetic units of the face. Following traumatic amputation, although technically very challenging, replantation is undoubtedly the procedure of choice. We present the first successful replantation of a partially amputated nose subjected to an ischaemic time of over 12 h. The injury was sustained following a dog-bite and inter-positional vein grafts were used to re-establish both arterial and venous blood flow.

Submucous Cleft Palate: A Systematic Review of Surgical Management Based on Perceptual and Instrumental Analysis.

Objective: Submucous cleft palate (SMCP) is a congenital condition associated with abnormal development of the soft palate musculature. In a proportion of cases, this results in velopharyngeal insufficiency (VPI), the treatment for which includes pharyngeal flap surgery, pharyngoplasty, and palate reconstruction. The aim of this paper is to determine whether there is superiority of one or more types of surgical procedure over the others in improving speech in patients with VPI secondary to SMCP. Methodology: Nine databases, including MEDLINE and EMBASE, were searched between inception and January 2013 to identify articles published relating to the surgical management of SMCP. Only studies that reported outcome measures for postoperative speech were included in the systematic review. Results: Twenty-six studies analyzing the outcomes of surgery for VPI in patients with SMCP met the inclusion criteria. In these studies, speech outcomes were measured either in a binary fashion (i.e., normal speech or evidence of VPI) or using scales of VPI severity. Of the 26 studies, only two utilized blinded speech assessment, and 12 included both preoperative and postoperative speech assessment. Conclusions: The review found little evidence to support any specific surgical intervention. This is in large part due to the inclusion of mixed etiologies within study populations and the lack of unbiased validated preoperative and postoperative speech assessment. Further methodologically rigorous studies need to be conducted to provide a secure evidence base for the surgical management of SMCP.

Experience of ReCell in Skin Cancer Reconstruction.

The ReCell system (Avita Medical) is a cell culture product that allows the immediate processing of a small split-thickness skin biopsy to produce a complete population of cells including keratinocytes, melanocytes, Langerhans cells and fibroblasts. This series is the first to highlight the reconstructive applications of ReCell following ablative skin cancer surgery. The ReCell system was utilized for three patients following skin cancer excision. In two cases, the cells were applied to forehead flap donor sites following nasal reconstruction. In one case, the cells were applied to the calvarial periosteum following wide local excision of a melanoma scar. Assessment of the treated area was performed using the patient and observer scar assessment scale after 1 year. The Patient and Observer Scar Assessment Scale (POSAS) scores for the 2 patients treated with ReCell following forehead flap surgery were 22 and 32. The score for the patient that underwent wide local excision of a melanoma scar was 45. The absence of a donor site, accelerated healing and the satisfactory aesthetic appearance of the mature scars in this series suggest that ReCell may play a useful role in reconstruction following skin cancer excision.

Nasendoscopy: an analysis of measurement uncertainties.

Objective : The purpose of this study was to analyze the optical characteristics of two different nasendoscopes used to assess velopharyngeal insufficiency and to quantify the measurement uncertainties that will occur in a typical set of clinical data. Design : The magnification and barrel distortion associated with nasendoscopy was estimated by using computer software to analyze the apparent dimensions of a spatially calibrated test object at varying object-lens distances. In addition, a method of semiquantitative analysis of velopharyngeal closure using nasendoscopy and computer software is described. To calculate the reliability of this method, 10 nasendoscopy examinations were analyzed two times by three separate operators. The measure of intraoperator and interoperator agreement was evaluated using Pearson's r correlation coefficient. Results : Over an object lens distance of 9 mm, magnification caused the visualized dimensions of the test object to increase by 80%. In addition, dimensions of objects visualized in the far-peripheral field of the nasendoscopic examinations appeared approximately 40% smaller than those visualized in the central field. Using computer software to analyze velopharyngeal closure, the mean correlation coefficient for intrarater reliability was .94 and for interrater reliability was .90. Conclusion : Using a custom-designed apparatus, the effect object-lens distance has on the magnification of nasendoscopic images has been quantified. Barrel distortion has also been quantified and was found to be independent of object-lens distance. Using computer software to analyze clinical images, the intraoperator and interoperator correlation appears to show that ratio-metric measurements are reliable.

Gluteal necrosis following pelvic fracture and bilateral internal iliac embolization: Reconstruction using a transposition flap based on the lumbar artery perforators.

INTRODUCTION: The use of bilateral internal iliac artery embolization to control hemorrhage associated with pelvic fractures is a life saving intervention. Gluteal necrosis is a rare but potentially fatal complication of this procedure. Following debridement, reconstruction can present a considerable challenge due to the compromised vascularity of local tissue. PRESENTATION OF CASE: A 17 year old girl suffered an open book pelvic fracture following a road traffic accident. In order to stop profuse bleeding, bilateral internal iliac artery embolization was performed. This procedure was complicated by the development of right sided gluteal necrosis. Following extensive debridement, a transposition flap based on the lumbar artery perforators was performed to cover the soft tissue defect. DISCUSSION: Gluteal necrosis occurs in approximately 3% of cases following internal iliac artery embolization. Following complete excision of the devitalised tissue reconstructive surgery is necessary. Local flaps are suboptimal options when the integument supplied by branches of the internal iliac arteries has been compromised following embolization. Furthermore, the use of a free flap is restricted by the lack of a readily accessible undamaged recipient vessel. In the present case a transposition flap based on the lumbar artery perforators facilitated robust reconstruction of the buttock region. CONCLUSION: To avoid sepsis, it is imperative that gluteal necrosis following internal iliac artery embolization is recognized and promptly debrided. A transposition flap based on the lumbar artery perforators is a good option for subsequent soft tissue coverage, which avoids use of tissue supplied by the branches of the internal iliac arteries.

The significance of the Van Nuys prognostic index in the management of ductal carcinoma in situ.

BACKGROUND: Debate regarding the benefit of radiotherapy after local excision of ductal carcinoma in situ (DCIS) continues. The Van Nuys Prognostic Index (VNPI) is thought to be a useful aid in deciding which patients are at increased risk of local recurrence and who may benefit from adjuvant radiotherapy (RT). Recently published interim data from the Sloane project has showed that the VNPI score did significantly affect the chances of getting planned radiotherapy in the UK, suggesting that British clinicians may already be using this scoring system to assist in decision making. This paper independently assesses the prognostic validity of the VNPI in a British population. PATIENTS AND METHODS: A retrospective review was conducted of all patients (n = 215) who underwent breast conserving surgery for DCIS at a single institution between 1997-2006. No patients included in the study received additional radiotherapy or hormonal treatment. Kaplan Meier survival curves were calculated, to determine disease free survival, for the total sample and a series of univariate analyses were performed to examine the value of various prognostic factors including the VNPI. The log-rank test was used to determine statistical significance of differential survival rates. Multivariate Cox regression analysis was performed to analyze the significance of the individual components of the VNPI. All analyses were conducted using SPSS software, version 14.5. RESULTS: The mean follow-up period was 53 months (range 12-97, SD19.9). Ninety five tumours were high grade (44%) and 84 tumours exhibited comedo necrosis (39%). The closest mean initial excision margin was 2.4 mm (range 0-22 mm, standard deviation 2.8) and a total of 72 tumours (33%) underwent further re-excision. The observed and the actuarial 8 year disease-free survival rates in this study were 91% and 83% respectively. The VNPI score and the presence of comedo necrosis were the only statistically significant prognostic indicators (P < 0.05). CONCLUSION: This follow-up study of 215 patients with DCIS treated with local excision and observation alone is one of the largest series in which rates of recurrence are unaffected by radiation therapy, hormone manipulation or chemotherapy. It has afforded us the opportunity to assess the prognostic impact of patient and tumour characteristics free of any potentially confounding treatment related influences. The results suggest that the VNPI can be used to identify a subset of patients who are at risk of local recurrence and who may potentially benefit from RT.