CleftED: A National Collaborative Study of Undergraduate Education and Exposure to Cleft lip and Palate Within the United Kingdom.

INTRODUCTION: Cleft Lip and/or Palate (CLP) are the most common congenital orofacial anomalies. Those involved in CLP care may extend beyond the core members of the Cleft multidisciplinary team (MDT) with a variety of medical healthcare professionals destined to contribute to the management of CLP patients at some point during their respective careers. Therefore, it seems essential that a basic understanding of CLP, CLP-associated problems, and potential avenues for direct or indirect involvement in CLP care be introduced at undergraduate level. AIMS: To investigate penultimate and final year undergraduate medical student CLP knowledge and exposure obtained whilst at medical school. METHODOLOGY: An online questionnaire was distributed to penultimate and final year medical students throughout the UK. RESULTS: A total of 3102 responses were received from 35 medical schools. 44.3% (n = 1374) of respondents had no exposure to CLP teaching up until their current year of education. 61.3% (n = 1903) of respondents had never been involved in the care of a CLP patient. 53.6% (n = 1662) of respondents were not confident in their current knowledge of CLP. 78.5% (n = 2257) of respondents indicated a desire to be involved in the care of CLP patients in their future career. CONCLUSION: More than half of the medical student survey respondents were not confident in their current knowledge of CLP and had limited involvement in CLP care. This may translate to a delay in diagnosis when students qualify. Improving CLP education and exposure during undergraduate training can help achieve national CLP standards for early diagnosis.

Single Suture Synostosis and Isolated Cleft Palate in Non-Apert Syndrome Patients.

INTRODUCTION: Single suture craniosynostosis (SSC) and isolated cleft palate (ICP) in non-Apert syndrome patients rarely occur together. Management includes airway optimization, timing surgery appropriately, and assessing both cranial vault aesthetics and speech outcomes. The aim of this study was to compare treatment pathways and outcomes in patients with both conditions to standard treatment for these conditions in isolation. METHODS: Patient hospital medical records were retrospectively reviewed for demographic data, timing of surgery, aesthetic outcome (using the Whitaker grading system for head shape), and speech outcome (from speech therapy records of general development and speech assessment). RESULTS: Six patients with SSC and ICP were identified over an 18-year period. Cranial surgery was performed between 4 and 16 months and cleft surgery between 6 and 34 months of age in all cases. Documentation of cleft surgery and genetic testing was not available for 1 patient. One patient with hemi-palatal absence had an obturator inserted at 34 months. Two patients were found to have Fragile X and Emanuel syndrome, respectively.No increased perioperative airway risk was highlighted in any case. Four cases were designated Whitaker grade 1, 1 case was designated grade 2, and 1 case was not graded as no cranial surgery was performed. Half of the patients had general and speech developmental delay, while the other half demonstrated no signs of developmental delay. DISCUSSION/CONCLUSION: Examination of 6 patients with SSC and ICP suggests the presence of both conditions does not adversely impact management or outcome for each condition, or increase perioperative risk.

What We Do: Greater Palatine Medializing Foraminal Osteotomy for Repair of the Wide Cleft Palate.

Wide cleft palates (>15-mm gap) present a number of challenges to the surgeon tasked with their repair ( Bardach, 1999 ). Eliminating the need for secondary surgery due to fistula formation can reduce additional anesthetic and scarring risks and optimize early speech development. Greater palatine foraminal osteotomy is a useful surgical adjunct that allows additional medial movement of oral mucoperiosteal flaps to aid in tension-free closure of the oral layer. We use a technique similar to that described by Seibert in 1995 with a few modifications. Closure of the nasal layer in these wide clefts can be achieved using a sphenoid flap, a technique recently published by our unit ( Khan et al, 2018 ).

In an in vitro model of human tuberculosis, monocyte-microglial networks regulate matrix metalloproteinase-1 and -3 gene expression and secretion via a p38 mitogen activated protein kinase-dependent pathway.

BACKGROUND: Tuberculosis (TB) of the central nervous system (CNS) is characterized by extensive tissue inflammation, driven by molecules that cleave extracellular matrix such as matrix metalloproteinase (MMP)-1 and MMP-3. However, relatively little is known about the regulation of these MMPs in the CNS. METHODS: Using a cellular model of CNS TB, we stimulated a human microglial cell line (CHME3) with conditioned medium from Mycobacterium tuberculosis-infected primary human monocytes (CoMTb). MMP-1 and MMP-3 secretion was detected using ELISAs confirmed with casein zymography or western blotting. Key results of a phospho-array profile that detects a wide range of kinase activity were confirmed with phospho-Western blotting. Chemical inhibition (SB203580) of microglial cells allowed investigation of expression and secretion of MMP-1 and MMP-3. Finally we used promoter reporter assays employing full length and MMP-3 promoter deletion constructs. Student's t-test was used for comparison of continuous variables and multiple intervention experiments were compared by one-way ANOVA with Tukey's correction for multiple pairwise comparisons. RESULTS: CoMTb up-regulated microglial MMP-1 and MMP-3 secretion in a dose- and time-dependent manner. The phospho-array profiling showed that the major increase in kinase activity due to CoMTb stimulation was in p38 mitogen activated protein kinase (MAPK), principally the α and γ subunits. p38 phosphorylation was detected at 15 minutes, with a second peak of activity at 120 minutes. High basal extracellular signal-regulated kinase activity was further increased by CoMTb. Secretion and expression of MMP-1 and MMP-3 were both p38 dependent. CoMTb stimulation of full length and MMP-3 promoter deletion constructs demonstrated up-regulation of activity in the wild type but a suppression site between -2183 and -1612 bp. CONCLUSIONS: Monocyte-microglial network-dependent MMP-1 and MMP-3 gene expression and secretion are dependent upon p38 MAPK in tuberculosis. p38 is therefore a potential target for adjuvant therapy in CNS TB.

Faciocervical Group B Streptococcal Necrotising Fasciitis: a Multidisciplinary Approach Management.

Neonatal necrotising fasciitis secondary to Streptococcus agalactiae, also known as Group B Streptococcus (GBS), is a rare, life-threatening entity with approximately 40 cases reported in the literature.1 GBS soft tissue infection in infancy most commonly affects the face, likely originating from the colonised oral cavity.2 In cases unresponsive to medical management alone, early surgical debridement can be life-saving. We present a case of faciocervical GBS necrotising fasciitis in a male neonate requiring multiple surgical debridements. The resultant soft tissue defect healed with topical negative pressure therapy and eventual placement of a double-layer dermal substitute. Due to his prematurity, the patient was not skin grafted to limit donor site morbidity. After recovering from his life-threatening infection, the patient had intensive scar therapy leading to a favourable cosmetic result with no evidence of function-limiting contracture. Our report draws focus to the need for a multidisciplinary approach incorporating therapy-led scar management early in the postsurgical recovery plan.

Isolated post laparoscopic DIEA injury in bilateral DIEP breast reconstruction.

Scarring from previous open abdominal surgery in patients undergoing autologous deep inferior epigastric perforator (DIEP) breast reconstruction has been reported to increase overall flap and donor site complication rates. The evidence to date demonstrates that it can be performed safely although with significantly higher postoperative donor site morbidity. It would seem logical that minimal access laparoscopic surgery is less likely to be associated with increased risks to flap vascularity or donor-site complications; however, there is little evidence available in the literature about the impact of previous laparoscopic surgery to the DIEP harvest site. The typical positions for port placement in standard laparoscopic procedures are usually distant from ideal perforator locations reducing the risk of perforator damage. We present a case of unilateral isolated injury to the proximal deep inferior epigastric artery (DIEA) following previous laparoscopic abdominal surgery in a patient undergoing bilateral mastectomy and breast reconstruction with bilateral free DIEP flaps.

Palmaris profundus in the carpal tunnel.

Carpal tunnel release is a routinely performed operation to relieve pressure caused by compression on the median nerve. In the majority of cases, the causation of the compression will be idiopathic. Among the secondary causes of median nerve compression is the palmaris profundus, a rare anatomical variant separate to the palmaris longus tendon. It has been suggested that it may cause carpal tunnel syndrome as it courses underneath the flexor retinaculum with the contents of the carpal tunnel reducing the space available to the median nerve. Several cases have found it intimately associated with the median nerve within the carpal tunnel. Raising awareness of this anatomical variant is therefore important for those undertaking carpal tunnel decompression in order to avoid unintended damage.

The Wire Study-a protocol for a multi-stage feasibility study evaluating K-wire fixation of hand fractures in the UK.

BACKGROUND: Hand fractures are common and sometimes require surgery to restore function. Placement of Kirschner wires (K-wires) is the most common form of surgical fixation. After placement, a key decision is whether to bury the end of a K-wire or leave it protruding from the skin (exposed). A recent systematic review found no evidence to support either approach and a national clinician and surgeon survey demonstrated further uncertainty. We aim to determine the design of a definitive randomised controlled trial assessing the cost and clinical effectiveness of buried versus exposed Kirschner wires for adults with metacarpal or phalangeal fractures. METHODS: We will employ three methodologies: a national service evaluation of current clinical practice, patient and surgeon focus groups and a consensus meeting to finalise the protocol for a randomised controlled trial. For the service evaluation, all outcomes will be summarised using descriptive statistics overall and split by group (buried versus exposed K-wires). Information collected in the patient focus groups will be analysed thematically. The surgeon consensus meeting will address each part of the design in turn and through discussion agree a final protocol. DISCUSSION: The study may be monitored, or audited in accordance with the current approved protocol, Good Clinical Practice (GCP), relevant regulations and standard operating procedures. The Chief Investigator will submit and, where necessary, obtain approval from the above parties for all substantial amendments to the original approved documents. A feasibility study report will be published by the Wire Study Steering committee. Additional members of the steering group and citable collaborators will be listed within the manuscript and their roles identified.