Current Surgical Practice for Children Born with a Cleft lip and/or Palate in the United Kingdom.

OBJECTIVE: This study describes primary surgical reconstructions performed for children born with a cleft lip and/or palate (CL ± P) in the United Kingdom (UK). DESIGN: Data forms completed at the time of surgery included details on timing, technique, and adjuncts used during the operative period. Demographic data on participants were validated via parental questionnaires. SETTING: Data were obtained from the Cleft Collective, a national longitudinal cohort study. PATIENTS: Between 2015 and 2021, 1782 Cleft Collective surgical forms were included, relating to the primary reconstructions of 1514 individual children. RESULTS: The median age at primary cheiloplasty was 4.3 months. Unilateral cleft lips (UCL) were reconstructed with an anatomical subunit approximation technique in 53%, whereas bilateral cleft lips (BCL) were reconstructed with a broader range of eponymous techniques. Clefts of the soft palate were reconstructed at a median age of 10.3 months with an intravelar veloplasty in 94% cases. Clefts of the hard palate were reconstructed with a vomer flap in 84% cases in a bimodal age distribution, relating to reconstruction carried out simultaneously with either lip or soft palate reconstruction. Antibiotics were used in 96% of cases, with an at-induction-only regimen used more commonly for cheiloplasties (P < .001) and a 5 to 7-day postoperative regime used more commonly for soft palatoplasties (P < .001). Perioperative steroids were used more commonly in palatoplasties than cheiloplasties (P < .001) but tranexamic acid use was equivalent (P = .73). CONCLUSIONS: This study contributes to our understanding of current cleft surgical pathways in the UK and will provide a baseline for analysis of the effectiveness of utilized protocols.

Tessier 30 Facial Clefts-A Literature Review of 72 Cases (1996-2020), Suggested Treatment Protocol, Outcome Measures, Minimum Dataset for Future Case Reports, and Registries.

INTRODUCTION: Tessier 30 facial cleft is a rare anomaly presenting in the soft and hard tissues over the central lower face. Owing to the rarity of cases and difficulty of treatment, there is no universally accepted surgical management strategy. The last comprehensive literature review of Tessier 30 clefts was in 1996. This report aims to update the literature to inform decision-making on treating Tessier 30 cases. METHODOLOGY: A literature search was performed. PubMed, SCOPUS, and OVID databases were searched. A total of 72 cases in 51 articles were analyzed, looking at demographics, extent of cleft, parent health, family history, procedures, follow-up, existence of other anomalies, and stages of repair. RESULTS: Surgeons are increasingly choosing to repair Tessier 30 defects in one rather than multiple stages. Of the 72 cases studied, only 31 had documented the completed repair of the cleft. All completed soft tissue only defects were repaired in 1 stage of repair (n = 11). Where both soft tissue and mandible was involved (n = 20), 55% (n = 11) had undergone 1-stage repair to address the Tessier 30 cleft. DISCUSSION: We argue that a single-stage approach is preferable to multistage. Primary mucogingivoperiosteoplasty should be undertaken in children at the time of management of the soft tissue cleft. The timing of this procedure should be in the latter half of the first year of life, as this is when mandibular symphyseal fusion normally occurs. We have suggested a treatment protocol and we hope that future case reports use our minimum data set.

Exploring the Relationship Between Palatal Cleft Type and Width With the Use of Relieving Incisions in Primary Repair.

OBJECTIVE: The mainstay of palatal repair in the United Kingdom is the intravelar veloplasty (IVVP). It is not always possible to align the oral mucosa in the midline to achieve tension-free repair. The addition of lateral relieving incisions may aid transposition of the oral mucosa to allow closure. The aim of this study was to explore cleft features that may predispose to a requirement for relieving incisions in order to allow palate closure. DESIGN: We performed a national multiinstitutional retrospective study using data from the UK Cleft Collective cohort study. PATIENTS: The study sample consisted of 474 patients who had undergone IVVP at the time of palatal closure across all 16 of the UK cleft units. RESULTS: We found strong evidence for the requirement for relieving incisions in patients with an increased degree of clefting per the Veau classification (P < .001), increasing palatal soft-edge width (P < .001) and moderate evidence of an associated use in patients with Pierre Robin sequence (P = .015). Insufficient data were available to explore the relationship between intertuberosity distance and the presence of fistula formation with the use of relieving incisions. CONCLUSIONS: The results of this study identify cleft features that increase the likelihood for requiring lateral relieving incisions to allow palatal closure. The degree to which the addition of relieving incisions to IVVP affects maxillary growth and speech outcomes is unknown. Further study is required to answer this important question.

The impact of changing cigarette smoking habits and smoke-free legislation on orofacial cleft incidence in the United Kingdom: Evidence from two time-series studies.

BACKGROUND: Both active and passive cigarette smoking have previously been associated with orofacial cleft aetiology. We aimed to analyse the impact of declining active smoking prevalence and the implementation of smoke-free legislation on the incidence of children born with a cleft lip and/or palate within the United Kingdom. METHODS AND FINDINGS: We conducted regression analysis using national administrative data in the United Kingdom between 2000-2018. The main outcome measure was orofacial cleft incidence, reported annually for England, Wales and Northern Ireland and separately for Scotland. First, we conducted an ecological study with longitudinal time-series analysis using smoking prevalence data for females over 16 years of age. Second, we used a natural experiment design with interrupted time-series analysis to assess the impact of smoke-free legislation. Over the study period, the annual incidence of orofacial cleft per 10,000 live births ranged from 14.2-16.2 in England, Wales and Northern Ireland and 13.4-18.8 in Scotland. The proportion of active smokers amongst females in the United Kingdom declined by 37% during the study period. Adjusted regression analysis did not show a correlation between the proportion of active smokers and orofacial cleft incidence in either dataset, although we were unable to exclude a modest effect of the magnitude seen in individual-level observational studies. The data in England, Wales and Northern Ireland suggested an 8% reduction in orofacial cleft incidence (RR 0.92, 95%CI 0.85 to 0.99; P = 0.024) following the implementation of smoke-free legislation. In Scotland, there was weak evidence for an increase in orofacial cleft incidence following smoke-free legislation (RR 1.16, 95%CI 0.94 to 1.44; P = 0.173). CONCLUSIONS: These two ecological studies offer a novel insight into the influence of smoking in orofacial cleft aetiology, adding to the evidence base from individual-level studies. Our results suggest that smoke-free legislation may have reduced orofacial cleft incidence in England, Wales and Northern Ireland.

Elite and Professional sports facial injuries management - a consensus report.

Whilst there have been great improvements in the management of elite and professional athletes with the formalisation of the clinical specialty of Sports and Exercise medicine, the management of facial injuries has perhaps lagged in this group. Professional athletic careers can be put in jeopardy due to unnecessarily long absences from training and competition after facial injuries. Professional and elite sports athletes can benefit from different approaches to the management of their facial injuries to reduce time away from competing but maximise their safety. On 6th December 2018, a consensus meeting of interested clinicians involved in the management of facial injuries of elite and professional athletes was held at the Royal Society of Medicine, London, UK to provide a contemporary review of the approaches to conservative, operative and recovery management of facial injuries. National experts with specialist experience of facial injuries presentation and athlete needs from a range of elite and professional sports led the day's programme and the discussions so that guidelines could be formulated. These are presented in this review paper.

Tessier 30 Median Mandibular Hard and Soft Tissue Cleft, One-Stage Reconstruction Using a Template-Guided Resorbable "U"-Shaped Plate.

Midline clefts of the lower lip, tongue, and mandible are a rare type of facial cleft classified as "Tessier 30." We present the case of a female patient with an isolated Tessier 30 facial cleft affecting the tongue, lower lip, and mandibular symphysis with ankyloglossia. This was reconstructed with a template-guided resorbable "U"-shaped plate at 10 months of age. The procedure was carried out in one stage, which avoided the need for a repeat general anesthetic for the patient. We had a successful outcome with normal dental eruption and we believe such an approach could be considered as a relevant treatment modality for future cases.

Aberrant facial flushing following monobloc fronto-facial distraction.

BACKGROUND: Patients with syndromic forms of craniosynostosis may experience functional problems such as raised intracranial pressure, proptosis, obstructive sleep apnoea and failure to thrive. The monobloc fronto-facial advancement with osteogenic distraction is increasingly used to correct these functional problems in one procedure as well as improve appearance. The authors report the phenomenon of post operative aberrant facial flushing - an unusual and previously unreported complication of the procedure. METHODS: The case notes of 80 consecutive patients undergoing fronto-facial advancement by distraction using the rigid external distraction device (RED) were reviewed for features of aberrant facial flushing. RESULTS: Four out of eighty individuals developed facial flushing after monobloc fronto-facial distraction using the rigid external distractor (RED) frame. All were female with Crouzon or Pfeiffer syndromes causing the severe functional problems for which they underwent the surgery. They were aged 6-8 years. Following removal of the frame, they developed intermittent but severe facial flushing. The flushing spontaneously settled in three patients after up to four years but persists in the other child seven years after her surgery. CONCLUSION: Aberrant facial flushing is a rare but significant complication of monobloc fronto-facial surgery. It occurred in 4 of our 80 (5%) patients. The skull base osteotomies essential for the procedure are made anterior to the pterygopalatine ganglion and it is our contention that damage from these was responsible for a neuropraxia of its efferent nerve branches. A review of the autonomic control of the facial vascular system suggests that the phenomenon is due to an unequal process of recovery that leaves the cutaneous vasodilating parasympathetic or beta-adrenergic innervation relatively unopposed - a situation that persists until with time a normal balance of autonomic input is achieved.

Optical diagnostic techniques for use in lesions of the head and neck: review of the latest developments.

Optical biopsy systems are a potential adjunct to the histopathological assessment of tissue specimens; they are not invasive and can give an immediate result. We review the most common optical biopsy techniques used to detect lesions of the head and neck: elastic scattering spectroscopy, microendoscopy, narrow band imaging, fluorescence, and optical coherence tomography, and discuss their clinical use.

The surgical management of Treacher Collins syndrome.

Treacher Collins syndrome (TCS), mandibulofacial dysostosis, or Franceschetti-Zwahlen-Klein syndrome, is a rare genetic disorder characterised by dysgenesis of the hard and soft tissues of the first and second branchial arches. Early operations focus on maintaining the airway, protecting the eyes, and supporting auditory neurological development. Later operations include staged reconstruction of the mouth, face, and external ear. Bimaxillary surgery can improve the maxillomandibular facial projection, but correction of malar, orbital rim, and temporal defects may be more difficult. We present a clinical review of the syndrome with a chronological approach to the operations.

Monobloc and bipartition in craniofacial surgery.

BACKGROUND: The frontofacial monobloc advancement with osteogenic distraction is increasingly used as a surgical treatment for children with complex craniosynostosis-associated syndromes. However, the subfrontal osteotomy cuts to free the facial skeleton from the skull base require extradural retraction of the frontal lobes. The purpose of this study was to determine the frequency and degree of radiologically identifiable frontal lobe changes and whether any such changes affected the patients' outcome. METHODS: The clinical records and preoperative and postoperative computed tomography imaging of all patients undergoing monobloc frontofacial distraction advancement (with or without bipartition) were reviewed. A retrospective medical notes review was undertaken to assess any patient or surgically related factors that might predispose to frontal lobe changes and evaluate outcome from surgery. Where available, magnetic resonance imaging scans were reviewed to compare outcome with that on computed tomography. RESULTS: Fifty cases were identified as suitable for the study. Eighteen patients (36%) had no frontal lobe changes. Thirty-two cases (64%) did have changes that appeared related to the position of maximum retraction during subfrontal osteotomy cuts. There were no changes in the incidence/extent of these changes over time or of any link to the patients' diagnosis, age at surgery, phenotype severity, surgery type, or any surgical or postoperative adverse events. We found no evidence that these changes were responsible for neurologic problems (eg, epilepsy) or reduced cognitive function. CONCLUSIONS: This study reveals a high incidence of frontal lobe changes demonstrable on neuroimaging following the frontofacial monobloc procedure reflecting the retraction points during surgery. Although no postoperative disability was reported, it is clearly important to consider more detailed neuropsychologic testing and review current surgical techniques to ensure that such changes are kept to a minimum.

Lingual cyst lined by respiratory and gastric epithelium in a neonate.

Congenital oral cysts of foregut origin include bronchogenic, enteric, and oesophageal cysts, and they are much rarer than the well described dermoid, epidermoid, and thyroglossal cysts. The exact aetiology is poorly understood, but they are thought to arise from misplaced embryonic rests of the primitive foregut. The presentation of cysts lined by respiratory or gastrointestinal epithelium in the oral cavity is unusual. There have been previous reports of bronchogenic or gastrointestinal epithelium-lined lingual cysts, but few report both features occurring within the same cyst. In view of the scarcity of such reports, we present the case of a lesion on the ventral surface of the tongue of a newborn boy. On removal it was found to be a cyst lined by immature squamous, respiratory, and gastric body epithelium.

The surgical management of severe macroglossia in systemic AL amyloidosis.

Amyloidosis is a disease characterised by the deposition in body tissues of amyloid: abnormal protein in a beta pleated sheet formation. It is a systemic disorder and macroglossia may be seen in all forms. Changes to the normal architecture of the tissues and systemic features of the disease and its underlying cause can complicate the surgical management of the enlarged tongue.

Orbital fractures in children.

In children, differences in the properties and proportions of bone in the craniofacial skeleton and the lack of development of the paranasal sinuses result in orbital fractures that present differently from those in adults. Facial growth may be disturbed by such injuries and also by surgical intervention, which should therefore be as conservative as possible. However, urgent operation is needed to prevent irreversible changes when fractures of the orbital floor involve entrapped muscle. We present an approach to such injuries.

Free groin flap in hemifacial volume reconstruction.

Use of the free groin flap, one of the first microvascular free flaps described, has been neglected recently because it has a short pedicle and varies anatomically. However, we have found its anatomical features and type of tissue ideal for volumetric enhancement in severe hemifacial asymmetry. We present a retrospective review of a consecutive series of 14 patients who had hemifacial augmentation with a free groin flap (mean age at operation 17 years, range 10-42) since 2001, and discuss the surgical technique. The most common cause of asymmetry was hemifacial microsomia (n=6). Anatomical variation of the vessels in the groin did not cause problems. Arterial anastomosis was to the facial artery in 13 patients; 12 patients had simultaneous hard tissue procedures. No flaps failed. The free groin flap is a useful adjunct in the management of hemifacial deficits in volume when free fat grafts will not provide enough bulk. Although the operation can take longer than non-vascularised grafts, little tissue is lost so long-term results may be more predictable. We have found the anatomy fairly consistent and the short pedicle caused no problems.

Pneumocephalus--late cause of neurological deterioration after craniomaxillofacial trauma.

We report a case of pneumocephalus, which is an unusual cause of late deterioration of the level of consciousness after craniomaxillofacial trauma.

Photodynamic therapy in the management of lesions of the head and neck.

Photodynamic therapy (PDT) is a promising and effective treatment for lesions of the head and neck. It uses illumination with light of a specific wavelength, which activates a photosensitising drug in the presence of oxygen. It can be used in combination with other treatments or on its own, and results in the cellular destruction of the lesion through a free-radical process. Photosensitisers can be applied topically or given systemically depending on the lesion being treated. Results indicate that PDT is an effective adjunct to standard conventional treatments. We review its use.