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.

Piezoelectric instrumentation in secondary cleft rhinoplasty: Techniques and clinical experience.

INTRODUCTION: Cleft rhinoplasty is a challenging procedure, with precision required to achieve good results. Cases often present with more complex structural and soft tissue asymmetries than non-cleft cases. Piezoelectric instrumentation uses ultrasonic vibrations to cut bone. At certain frequency, it will only cut bone, sparing soft tissue and is reported to decrease postoperative pain, oedema and echymosis. It allows nasal bony work to be performed under direct vision without losing stability of the bony fragments by preserving the underlying periosteum. There is good evidence on the use of piezoelectric instrumentation in cosmetic rhinoplasty; however, none to date has focused exclusively on cleft rhinoplasty. We present a single surgeon experience using piezoelectric instrumentation in cleft rhinoplasty. METHODS AND PATIENTS: We reviewed the case histories of 21 consecutive patients who had Piezo-assisted cleft rhinoplasty surgery between 2017 and 2021. We present our operative techniques and results of piezoelectric cleft rhinoplasty and compare it with 19 patients undergoing cleft rhinoplasty with conventional instrumentation over the same time period, by the same surgeon. RESULTS: Piezo-assisted rhinoplasty steps included bony osteotomies, dorsal hump removal, modification of composite cartilage/ ethmoid grafts and instrumentation of the anterior nasal spine. There were no significant complications and no revision surgeries. There was no difference in operative time compared to conventional instruments. CONCLUSION: Piezoelectric instrumentation is a valuable and efficient tool in cleft rhinoplasty. It offers potentially significant advantages in terms of the precision of bony work, whilst minimising trauma to the surrounding soft tissues.

Educational impact of a novel cleft palate surgical simulator: Improvement in surgical trainees' knowledge and confidence.

BACKGROUND: Trainees' experience in cleft surgery is limited due to the high-risk nature of the surgery and centralization of cleft care. Simulation training allows trainees to learn complex surgical tasks whilst ensuring patient safety. Existing cleft surgical simulators are over-simplified or prohibitively expensive. In this article, we show the development and application of a high-fidelity yet cost-effective simulator for cleft palate repair. METHODS: Skeletal elements were obtained through high-resolution scanning of a pathologic specimen, 3-dimensional printed, and then molded in plastic. Soft tissue components were formed through molding layers of silicone. The simulator was tested by 26 United Kingdom (UK) specialty trainees who performed a vomerine mucosal flap and intra-velar veloplasty in a 1-hour workshop. Pre- and post-simulation questionnaires assessing cleft knowledge and surgical confidence were compared for statistical significance. RESULTS: The simulator had high acceptability: 23/26 participants confirmed that the simulation training was a valuable learning experience. Baseline experience of cleft palate surgery was low: 24 participants had never performed any part of the procedure before. Following the workshop, mean knowledge score increased by 38%, and confidence by 53%. The paired T-test demonstrated that observed improvements in both knowledge and confidence were statistically significant (p<0.001). CONCLUSIONS: Simulation training is increasingly recognized as a crucial component of improving patient safety. Our new cost-effective cleft palate simulator has high acceptability and is a powerful educational tool that is effective in improving cleft palate surgical knowledge and confidence across all grades of surgical trainees.

The importance of neurology and genetic testing in the patient with non-cleft velopharyngeal dysfunction.

OBJECTIVE: A significant proportion of the referrals made to a speech investigation clinic in a cleft unit include patients with non-cleft velopharyngeal dysfunction (VPD). This study aims to quantify the underlying diagnoses of these patients and describe the investigative pathway and diagnostic information subsequent to presentation in our clinic. MATERIALS AND METHODS: The case notes of 136 consecutive patients with non-cleft VPD who attended our Velopharyngeal Investigation (VPI) clinic from July 2014-December 2019 were reviewed. RESULTS: In the paediatric group (n = 118) the most common cause was 22q11 chromosomal anomalies (n = 46), while post palatal tumour resection was the commonest cause of acquired non-cleft VPD in adults (n = 8). Fifty-nine patients were referred to the clinic with a known underlying pathology such as a syndromic diagnosis. Of those presenting without a known aetiology, fifty-eight were referred onto our genetics and/or neurology colleagues. Although a genetic or neurological cause could not be identified in some of those patients, thirty-one patients received a new diagnosis, with subsequent implications for ongoing care. CONCLUSION: There are a wide range of diagnoses resulting in non-cleft VPD, but there are very few large-scale studies focusing on investigating these patients for an underlying aetiology. This study highlights the role of genetics and neurology in the diagnosis and management plan for this cohort of patients.

Robin Sequence: 5-Year Speech Outcomes-A Case-Control Study.

BACKGROUND: Pierre Robin sequence (Robin sequence) is defined as the triad of micrognathia, glossoptosis, and airway obstruction. It is frequently associated with palatal clefting. In recent years, increased interest in speech outcomes of cleft patients diagnosed with Robin sequence has been shown. METHODS: Speech outcomes of cleft patients with Robin sequence were assessed at age 5 in comparison with a cleft palate-only cohort. Speech parameters were evaluated according to the Cleft Audit Protocol for Speech-Augmented and analyzed using the National Audit Standards for Speech (United Kingdom). All patients were treated in the same institution during the same period (2005 to 2012). Subjects who needed nasopharyngeal airway support and those whose airway was managed by positioning only were eligible. RESULTS: Fifty-one cleft patients diagnosed with Robin sequence were included in this study. Outcomes were compared to those of 128 nonsyndromic cleft palate-only patients.Patients with Robin sequence were shown to present with a significantly higher rate of cleft speech characteristics in comparison to the reference cohort (p = 0.001). Furthermore, it was shown that Robin sequence is associated with a significantly higher rate of secondary speech surgery for velopharyngeal dysfunction before the age of 5 (p = 0.016). Robin sequence patients with a nasopharyngeal airway presented with a higher rate of cleft speech characteristics compared to Robin sequence patients managed with positioning only. CONCLUSION: Cleft patients with Robin sequence are more likely to need further surgery to correct velopharyngeal dysfunction before the age of 5 and are more prone to present with cleft speech characteristics at the age of 5. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

A Cadaveric Study of the Buccal Fat Pad: Implications for Closure of Palatal Fistulae and Donor-Site Morbidity.

BACKGROUND: For the cleft surgeon, palatal fistulae after cleft palate repair remain a difficult problem, with a paucity of local tissue options to aid closure. Small clinical series have described the use of the buccal fat pad flap to repair palatal fistulae; however, there is no literature detailing the anatomical coverage of the flap. This study delineates the anatomy of the buccal fat pad flap to guide surgeons in patient selection and examines the residual buccal fat after flap harvest to provide new information with regard to possible effects on the donor site. METHODS: Buccal fat pad flaps were raised in 30 hemicadavers. The reach of the flap across the midline, anteriorly and posteriorly, was recorded. In 18 hemicadavers, the entire buccal fat pad was then exposed to determine the effects of flap harvest on movement and volume of the residual fat. RESULTS: All buccal fat pad flaps provided coverage from the soft palate to the posterior third of the hard palate and all across the midline. Approximately three-fourths of flaps would cover the mid hard palate. The flap constitutes 36 percent of the total buccal fat pad on average, and a series of retaining ligaments were identified that may prevent overresection. CONCLUSIONS: The buccal fat pad flap is a useful tool for coverage of fistulae in the soft palate to the posterior third of the hard palate. In most cases, it will also reach the middle third; however, it is not suitable for more anterior defects. On average, two-thirds of the buccal fat pad remains within the cheek after flap harvest, which may protect against unwanted alteration in aesthetics.

Use of Preoperative Cervical Vascular Imaging in Patients With Velocardiofacial Syndrome and Velopharyngeal Dysfunction in the United Kingdom.

OBJECTIVE: In patients with velocardiofacial syndrome (VCFS), medial displacement of the internal carotid arteries (ICAs) may increase the risk of vascular injury during the surgical correction of velopharyngeal dysfunction (VPD). Some surgeons advocate the use of vascular imaging studies prior to surgery. Nevertheless, the role of preoperative imaging is still controversial. This study aimed to review the current practice of the UK cleft units and also examine our own practice at the Evelina London Children's Hospital in relation to children with VCFS undergoing speech surgery over the previous 7 years. DESIGN: A questionnaire was sent to all UK cleft surgeons to enquire about the management and use of preoperative vascular imaging in patients with VPD and VCFS. A retrospective study was also conducted of the unit's 7-year series of patients with VPD and VCFS. RESULTS: Thirty-four completed questionnaires were returned (response rate 100%). Most UK surgeons (73.5%) do not regularly order preoperative vascular imaging for patients with VCFS although some reportedly would consider it if a posterior pharyngeal wall pulsation was visible. In our unit, between 2013 and 2019, a total of 40 patients affected by VCFS have been assessed for VPD. A magnetic resonance angiography (MRA) was performed for 23 patients. Medial deviation of the ICAs was identified in 7 (30%) patients. CONCLUSIONS: The results of the national survey showed no consensus on routine use of preoperative vascular imaging. Our retrospective study showed a 30% prevalence of medialized ICAs in our patient cohort. In these patients, the MRA findings influenced the choice of speech surgery.

Use of a purified collagen membrane to aid closure of palatal fistulae.

INTRODUCTION: Fistula formation following closure of palatal clefts remains a difficult clinical complication. A significant recurrence rate has also been reported following attempted closure. We present our results of fistula closure augmented with Bio-Gide(®), a purified porcine collagen membrane designed to promote guided tissue regeneration. METHODS: We reviewed the records of 263 patients operated between 1993 and 2011 for closure of palatal fistula. The patients selected comprised 61 who underwent fistula closure augmented with Bio-Gide and 202 with other techniques in the absence of Bio-Gide. We reviewed the age at surgery, sex, location of fistula, cleft type and outcome. Operation success was defined as an asymptomatic patient along with visible confirmation of closure of the fistula. RESULTS: The overall fistula closure rate was 75% in the Bio-Gide group and 63% in the non-Bio-Gide group (p = 0.070) and 86% versus 61% in the unilateral cleft palate patients (p = 0.027). DISCUSSION: Bio-Gide has improved the success rate in fistula closure in this study. Using this technique, fistula closure can be performed as a day case procedure and does not need to transgress any original suture lines; an additional advantage is that this procedure does not require harvesting of any autologous tissue to augment the repair.

Salvage of the lower limb after a full thickness burn with loss of the knee extensor mechanism: a case report.

We report on a 79-year-old woman who underwent salvage of the knee and lower leg using a Whichita Fusion Nail for knee arthrodesis, combined with a medial gastrocnemius muscle flap for a 3% contact burn that resulted in loss of the extensor mechanism. This provided an alternative to above-knee amputation when extensor mechanism reconstruction was not feasible.

Reconstruction of pharyngolaryngectomy defects using the jejunal free flap: a 10-year experience from a single reconstructive center.

BACKGROUND: Reconstruction following pharyngolaryngectomy presents a complex reconstructive challenge, and a single-stage, reliable reconstruction allowing prompt discharge from the hospital and return of swallowing and speech function is required. The authors present their 10-year experience of 43 jejunal free flaps for pharyngolaryngectomy reconstruction by a single team and outline their operative algorithm to minimize postoperative morbidity. METHODS: The data for patients who underwent jejunal free flap reconstruction of circumferential pharyngoesophageal defects between March of 2000 and September of 2009 were reviewed retrospectively. All cases were included for analysis. RESULTS: There were 31 male patients and 12 female patients, with 100 percent acute flap survival. The authors' overall benign pharyngocutaneous fistula rate was two of 43 (5 percent), with two of 29 (7 percent) occurring in the group without a prophylactic pectoralis muscle flap and zero of 14 occurring in the group that had a prophylactic pectoralis muscle flap. No fistulas occurred when the anastomosis was performed with the gastrointestinal stapler (zero of 48). The authors' overall benign stricture rate was six of 43 (14 percent). Thirty-six patients received either a primary or secondary tracheoesophageal puncture; of these, 28 of 36 (78 percent) used their tracheoesophageal puncture as their primary mode of communication. CONCLUSION: The authors' recommendations for minimizing fistulas and stricture rate, following free jejunal reconstruction, include the gastrointestinal stapler for bowel anastomosis whenever possible, and the use of a prophylactic pedicled pectoralis major muscle flap for patients exposed to previous radiotherapy.

Augmentation of temporal fossa hollowing with Mersilene mesh.

PURPOSE: Temporal fossa hollowing can represent a serious cosmetic concern to patients', whether congenital; part of a post-traumatic deformity; following oncological resection; or following orbital decompression procedures. Various methods have been described to augment the defect including high density polyethylene (HDPE) implants, methyl methylmethacrolate and lipotransfer, but all have their relative drawbacks. METHODS: We describe 13 temporal defects in 10 patients whose defects have been reconstructed with Mersilene mesh (Ethicon, Livingstone UK). When the temporalis muscle is still adequately suspended; folded mesh can be placed in a submuscular/subperiosteal plane. The mesh can be folded as many times as necessary to recreate the appropriate volume, thereby allowing fine control of temple width. If the temporalis is attenuated or displaced inferiorly by previous surgery, its insertion can be recreated using titanium screws to attach the mesh superiorly to the appropriate site on the cranium; the temporalis resuspended and the fascia sutured to the mesh. RESULTS: We report very pleasing cosmetic results with no associated complications such as implant, extrusion or exposure. One patient was complicated by infection requiring removal of the mesh as well as titanium cranioplasty, screws and plates. CONCLUSION: Mersilene mesh is easy to use, contours naturally to the defect and is considerably cheaper than alternatives like HDPE implants.

Contact Heat Evoked Potentials Using Simultaneous Eeg And Fmri And Their Correlation With Evoked Pain.

BACKGROUND: The Contact Heat Evoked Potential Stimulator (CHEPS) utilises rapidly delivered heat pulses with adjustable peak temperatures to stimulate the differential warm/heat thresholds of receptors expressed by Adelta and C fibres. The resulting evoked potentials can be recorded and measured, providing a useful clinical tool for the study of thermal and nociceptive pathways. Concurrent recording of contact heat evoked potentials using electroencephalogram (EEG) and functional magnetic resonance imaging (fMRI) has not previously been reported with CHEPS. Developing simultaneous EEG and fMRI with CHEPS is highly desirable, as it provides an opportunity to exploit the high temporal resolution of EEG and the high spatial resolution of fMRI to study the reaction of the human brain to thermal and nociceptive stimuli. METHODS: In this study we have recorded evoked potentials stimulated by 51° C contact heat pulses from CHEPS using EEG, under normal conditions (baseline), and during continuous and simultaneous acquisition of fMRI images in ten healthy volunteers, during two sessions. The pain evoked by CHEPS was recorded on a Visual Analogue Scale (VAS). RESULTS: Analysis of EEG data revealed that the latencies and amplitudes of evoked potentials recorded during continuous fMRI did not differ significantly from baseline recordings. fMRI results were consistent with previous thermal pain studies, and showed Blood Oxygen Level Dependent (BOLD) changes in the insula, post-central gyrus, supplementary motor area (SMA), middle cingulate cortex and pre-central gyrus. There was a significant positive correlation between the evoked potential amplitude (EEG) and the psychophysical perception of pain on the VAS. CONCLUSION: The results of this study demonstrate the feasibility of recording contact heat evoked potentials with EEG during continuous and simultaneous fMRI. The combined use of the two methods can lead to identification of distinct patterns of brain activity indicative of pain and pro-nociceptive sensitisation in healthy subjects and chronic pain patients. Further studies are required for the technique to progress as a useful tool in clinical trials of novel analgesics.

Relocation of painful neuromas in Zone III of the hand and forearm.

Painful nerves are a difficult and complex clinical problem. We describe the result of treatment by proximal relocation of 51 painful end neuromas and scarred nerves of the forearm in 33 patients. The relocated nerves included 29 superficial radial nerves, 16 lateral antebrachial cutaneous nerves, two medial cutaneous nerves and four posterior cutaneous nerves. These relocations achieved no, or only mild, pain in 100% of nerves at the original site and 94% of nerves at the relocation site. It also achieved no, or only mild, hypersensitivity in 96% of nerves at the original site and 98% of nerves at the relocation site. The technical difficulties encountered in this region, in particular on the radial aspect of the wrist, are discussed.

Age-dependent development of chronic neuropathic pain, allodynia and sensory recovery after upper limb nerve injury in children.

Forty-nine children with distal upper limb nerve injury were studied at a mean follow-up of 2 years 3 months. Patients who were aged 5 years or younger at the time of nerve injury (15/49) had no chronic neuropathic pain symptoms or allodynia. Patients with allodynia on quantitative sensory testing but no spontaneous pain (8/49) were all older than 5 years and those reporting spontaneous chronic neuropathic pain (5/49) were all older than 12 years at the time of injury. Previous studies of adults with similar nerve injuries report chronic hyperaesthesia in up to 40% of cases. Semmes-Weinstein monofilament testing showed a positive correlation between age at injury and abnormal sensory threshold (r = 0.60, P<0.0001). These findings indicate that young children show better sensory recovery and are less likely to develop long-term chronic neuropathic pain syndromes than adults following nerve injury.

Use of the novel Contact Heat Evoked Potential Stimulator (CHEPS) for the assessment of small fibre neuropathy: correlations with skin flare responses and intra-epidermal nerve fibre counts.

BACKGROUND: The Contact Heat Evoked Potential Stimulator (CHEPS) rapidly stimulates cutaneous small nerve fibres, and resulting evoked potentials can be recorded from the scalp. We have studied patients with symptoms of sensory neuropathy and controls using CHEPS, and validated the findings using other objective measures of small nerve fibres i.e. the histamine-induced skin flare response and intra-epidermal fibres (IEF), and also quantitative sensory testing (QST), a subjective measure. METHODS: In patients with symptoms of sensory neuropathy (n = 41) and healthy controls (n = 9) we performed clinical examination, QST (monofilament, vibration and thermal perception thresholds), nerve conduction studies, histamine-induced skin flares and CHEPS. Skin punch biopsies were immunostained using standard ABC immunoperoxidase for the nerve marker PGP 9.5 or the heat and capsaicin receptor TRPV1. Immunoreactive IEF were counted per length of tissue section and epidermal thickness recorded. RESULTS: Amplitudes of Adelta evoked potentials (muV) following face, arm or leg stimulation were reduced in patients (e.g. for the leg: mean +/- SEM - controls 11.7 +/- 1.95, patients 3.63 +/- 0.85, p = 0.0032). Patients showed reduced leg skin flare responses, which correlated with Adelta amplitudes (rs = 0.40, p = 0.010). In patient leg skin biopsies, PGP 9.5- and TRPV1-immunoreactive IEF were reduced and correlated with Adelta amplitudes (PGP 9.5, rs = 0.51, p = 0.0006; TRPV1, rs = 0.48, p = 0.0012). CONCLUSION: CHEPS appears a sensitive measure, with abnormalities observed in some symptomatic patients who did not have significant IEF loss and/or QST abnormalities. Some of the latter patients may have early small fibre dysfunction or ion channelopathy. CHEPS provides a clinically practical, non-invasive and objective measure, and can be a useful additional tool for the assessment of sensory small fibre neuropathy. Although further evaluation is required, the technique shows potential clinical utility to differentiate neuropathy from other chronic pain states, and provide a biomarker for analgesic development.