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.

Maternal Cigarette Smoking and Cleft Lip and Palate: A Systematic Review and Meta-Analysis.

A systematic review and meta-analysis to determine the association between active maternal smoking and cleft lip and palate etiology.Medline, Embase, Web of Science and the Cochrane Library from inception to November, 2020.Observational studies of cigarette smoking habits in pregnant women. Outcomes included cleft lip and/or palate, cleft lip ± palate and cleft palate only.Publication bias analyses were performed and the Newcastle Ottawa scales were used to assess study quality. Fixed or random effect models were used in the meta-analysis, dependent on risk of statistical heterogeneity.Forty-five studies were eligible for inclusion of which 11 were cohort and 34 were case-control studies. Sixteen studies were of sufficient standard for inclusion in the meta-analysis. The summary odds ratio for the association between smoking and cleft lip and/or palate was 1.42 (95%CI 1.27-1.59) with a population attributable fraction of 4% (95%CI 3%-5%). There was limited evidence to show a dose-response effect of smoking.This review reports a moderate association between maternal smoking and orofacial cleft but the overall quality of the conventional observational studies included was poor. There is a need for high quality and novel research strategies to further define the role of smoking in the etiology of cleft lip and palate.

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 Management of Nonsyndromic, Isolated Sagittal Synostosis.

AIM: Isolated sagittal synostosis is the commonest form of craniosynostosis. The reasons for surgery are to normalize the head shape and to increase the cranial volume, thus reducing the risk of raised intracranial pressure and allowing for normal brain development. It has been suggested that sagittal synostosis may impair neuropsychological development. This systematic review appraised the literature on the management of sagittal synostosis. METHODS: A literature search was performed with the assistance of a professional librarian. Studies selected had to satisfy the criteria set by PICO (patients, intervention, comparison, and outcome). Cranial index and neuropsychological outcome were used as outcome measures. MINORS was used to assess the methodological quality of the selected articles. A score of 75% was deemed to be of satisfactory quality, and the quality of the evidence from the selected studies was graded using the GRADE system. RESULTS: One hundred forty-eight articles were initially identified. Only 6 articles fulfilled the PICO criteria and scored a minimum of 75% on MINORS. Four studies compared 1 technique to another with documented cranial indices. Two studies compared 1 group to another and assessed the neuropsychological development. According to GRADE, the quality of evidence was deemed to be very low. CONCLUSIONS: This systematic review assessed cranial index and neuropsychological outcome following surgery for isolated, nonsyndromic sagittal synostosis. The quality of the evidence in the published literature was noted to be of very low quality. There is a need for better-designed, prospective studies to guide surgeons involved in management of sagittal synostosis.

The Craniofacial and Upper Limb Management of Nager Syndrome.

INTRODUCTION: Nager syndrome is a rare condition characterized by craniofacial and upper limb abnormalities. It is commonly mistaken for Treacher Collins syndrome, with which it shares the same craniofacial phenotype. However, patients with Treacher Collins do not exhibit hand anomalies, which are seen in patients with Nager syndrome. This paper reviews the multidisciplinary management of patients with Nager syndrome who were treated at the Australian Craniofacial Unit, Adelaide and the Erasmus Medical Centre, Rotterdam. METHODS: The database of both units was scrutinized and the case-notes of the patients with Nager syndrome were reviewed. Data was collected on patient demographics, surgical management, complications, and outcome. RESULTS: Nine patients (6 M:3 F) were identified with Nager syndrome, with a mean age at presentation of 3.7 years (range 8 days to 11.8 years). The mean follow-up time was 2.2 years (2 months to 19 years). SF3B4 mutation was noted in 2 patients and 1 patient had an X:9 translocation. Seven (77.8%) had obstructive sleep apnoea, with 5 patients diagnosed as severe obstructive sleep apnoea. Four patients had pollicization of their index, 2 patients had excision of extra radial digits and 1 patient underwent thumb duplication correction. Craniofacial surgery included mandibular advancement in 5 patients, temporo-mandibular joint reconstruction in 2 patients, and a genioplasty in 1 patient. CONCLUSION: Nager syndrome is a rare acrofacial dysostosis syndrome that is best managed within the realms of a multidisciplinary team. The authors would advocate early pollicization in patients with thumb anomalies to prevent any impairment in manual dexterity.

Association of Perioperative Antibiotics with the Prevention of Postoperative Fistula after Cleft Palate Repair.

Background: There is debate amongst surgeons regarding the use of antibiotics to prevent fistulae after palatoplasty. Prescribing should be evidence based, as antibiotic stewardship is integral to reducing antibiotic resistance. Our aim was to determine whether differing perioperative regimens affect the prevalence of postoperative fistulae. Methods: The sample comprised participants from the Cleft Collective who had undergone palatoplasty. Participants were recruited across all 16 UK cleft centers between 2013 and 2021. The exposure was perioperative antibiotic regimen prescribed at the time of palatoplasty. The primary outcome was the presence of palatal fistula. Results: Fistula data were available for 167 participants when exploring antibiotic regimen and for 159 when exploring antibiotic agent. There was no evidence to suggest a difference in fistula rate between those receiving antibiotics on induction only versus as an inpatient or up to 7 days postoperatively (χ2 = 4.57; P = 0.10). There was no evidence to suggest a difference in fistula rate between those who received co-amoxiclav and those who had an alternative antibiotic (χ2 = 0.16; P = 0.69). Postoperative fistulae increased with the extent of the cleft (χ2 = 20.39; P < 0.001). When adjusting for cleft type, no evidence of an association between antibiotic regimen and fistulae was found (inpatient antibiotics: OR 1.36; 95% confidence interval, 0.53-3.51; antibiotics up to 7 days postoperatively: OR 0.68; 95% confidence interval, 0.26-1.80). Conclusions: The choice of antibiotic and dosing regimen does not influence the formation of postoperative fistulae. These results should be supported by interventional trials.

Poly implant prothèse (PIP) breast implants: our experience.

INTRODUCTION: This study describes our experience on the management of patients with PIP (Poly Implant Prothèse) breast implants between 2000 and 2008. MATERIALS AND METHODS: The medical records of patients were reviewed. Data was collected on clinical presentation, investigations, management and outcome. RESULTS: 44 patients, with bilateral breast implants, and a median age of 33 years (18-54 years), were reviewed, and of these, 31 patients were asymptomatic. Symptoms at presentation included lymphadenopathy, capsule formation, breast lump, seroma and breast pain. Patients underwent mammography, ultrasound and MRI scanning of the breasts as part of the imaging investigations. 5 patients declined explantation. Reasons for explantation included patient anxiety, silent rupture, aesthetic breast change, palpable nodes and breast lump. 17 out of a total of 78 implants (21.8%) were noted to have ruptured; 2 had a simple tear and 15 were totally disintegrated. 1 patient underwent removal of the implants, 18 underwent exchange of implants, and 20 patients had a capsulotomy and exchange of implants. Postoperative complications included wound infection, seroma, axillary lymphadenopathy, hypersensitive scar and overgranulation of the wound. CONCLUSION: Our series confirms the high rate of PIP implant rupture (21.8%), the majority of which were asymptomatic. The main reasons for explantation were patient anxiety and silent rupture of implants. It is imperative that patients should be appropriately counselled, prior to surgery with regards to removal of the implants, given the increased rupture rates noted.

Syndromes associated with Robin sequence: a national prospective cohort study.

OBJECTIVES: To determine the prevalence of syndromic Robin sequence (RS) in the UK and if this group of patients had an increased need for airway and feeding management compared with a non-syndromic RS cohort. DESIGN: A prospective national multicentre study of cases submitted to the Cleft Collective cohort studies. SETTING: Specialist cleft services in the UK. PATIENTS: 259 participants who fulfilled the diagnosis of RS. This group was compared with 548 participants with cleft palate only (CPO). MAIN OUTCOME MEASURES: The primary outcome measure was the presence of a syndrome in patients with RS and CPO. Secondary outcome measures included the use of airway and feeding adjuncts. RESULTS: An associated syndrome was seen in 28% of patients with RS and 14% of patients with CPO. The most common syndrome for the RS group was Stickler syndrome (27%). Syndromic status was significantly higher among patients with RS compared with those with CPO (OR 2.36, 95% CI 1.65 to 3.39; p<0.001). Patients with syndromic RS have an increased reliance on airway adjuncts compared with the patients without syndromic RS (OR 2.02, 95% CI 1.13 to 3.64; p=0.018). There was no evidence of a difference in the use of feeding adjuncts between syndromic and non-syndromic RS groups (OR 2.43, 95% CI 0.78 to 7.58; p=0.126). CONCLUSION: The presence of a syndrome has implications for management of patients with RS. Early identification of a syndrome may help prevent the consequences of a missed syndromic diagnosis. Routine ophthalmological and genetic screening for Stickler syndrome should be mandatory for all patients with RS.

Binder syndrome.

UNLABELLED: Patients with chondrodysplasia punctata (CDP) usually present with Binder-type features, and often CDP is misdiagnosed as Binder syndrome. This study reviewed the management and outcome of patients with Binder syndrome and CDP in a multidisciplinary setting. METHODS: The notes and radiographs of the patients managed at the Australian Craniofacial Unit with a multidisciplinary setting since 1976 were reviewed, and data were collected on patient demographics, associated medical and surgical problems, subsequent management, and complications. RESULTS: Seventy-seven patients were treated over the 30-year period (5 patients were lost to follow-up); of the remaining 72 patients, 60 (83%) had Binder syndrome, and 12 (17%) were patients with CDP. Forty were males, and 32 were females, with an age range of 6 months to 47 years. Thirteen patients (18%) had a strong family history, and 65 patients (90%) have so far undergone surgical correction, and of those, 35 (54%) have completed their treatment, the longest follow-up time being 18 years. The mean number of surgical procedures was 2.4, and 18 patients (28%) had postoperative complications, which included partial necrosis of the maxilla, osteomyelitis of the mandible, facial nerve and inferior alveolar nerve neuropraxia, nasal bone graft exposure, and cellulitis. DISCUSSION: Because of the phenotypic characteristics shared by both Binder syndrome and CDP, it is most likely that Binder syndrome is not a syndrome, nor is it an entity, but most likely to be an "association." We would advocate that these patients should be managed in a multidisciplinary setting.

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.

Management of the exposed total knee prosthesis, a six-year review.

INTRODUCTION: An exposed knee prosthesis is a limb threatening condition. Our unit manages such cases according to a multidisciplinary orthoplastic protocol. Whilst early prosthetic joint infection with dehiscence may be managed by Debridement, Antibiotics and Implant Retention (DAIR) and soft tissue coverage, the majority of these cases are chronic and in our unit are managed by Debridement, Explantation, Antibiotics (spacer and systemic) and Flap (DEAF). PATIENTS AND METHODS: We report our experience of managing 17 of these challenging cases, 16 DEAFs and one DAIR and flap. Outcomes were assessed clinically and using the 36-item Short Form Health Survey (SF-36). RESULTS: The mean time from arthroplasty to presentation in our unit was 19months (range: 0.5-80). Whilst an open knee replacement is by definition 'infected', significant microbiological growth from deep tissue/fluid samples was only detected in 14 patients (82%). Five patients (29.4%) subsequently underwent an amputation. Of these five, three patients were extensor deficient at presentation. At follow-up, health-related quality of life scoring using the Short Form-36 demonstrated poor physical function and highlighted differences in emotional function and pain levels between patients whose limbs were salvaged and patients who underwent amputation. CONCLUSION: An exposed total knee prosthesis is a devastating complication, which despite our multidisciplinary salvage approach, has a high rate (5/17=29%) of amputation in this series. Quality of life in this patient group is poor irrespective of limb salvage. Salvage surgery was associated with worse pain, but better emotional profile than patients with an above knee amputation.

Outcome of the management of open ankle fractures in an ortho-plastic specialist centre.

INTRODUCTION: We reviewed the functional outcome of 68 patients with open ankle fractures managed in an orthoplastic specialist centre. MATERIALS AND METHODS: Patients managed at Frenchay Hospital over a 6 year period were divided into 3 groups: group P were patients initially seen and managed at Frenchay Hospital (an orthoplastic specialist centre), group S were patients stabilised at a different unit and referred for definitive management, while group R were patients managed in a different unit and referred following complications. Injuries were graded using the AO score and outcome was measured using the Enneking score (both validated). RESULTS: 19 patients (group P, mean age: 43 years), 26 patients (group S, mean age: 41 years) and 23 patients (group R, mean age: 41.9 years) made the cohort. 82.4% patients required free tissue transfer. Mean AO scores of groups P, S and R were 11.5, 12.3 and 9.7 (p+0.03). Mean number of procedures for P, S and R were 2.6, 3.5 and 4.2 (p = 0.0006). Mean follow up time was 55.5, 61.0 and 57.0 weeks respectively (p = 0.72). Mean Enneking scores for groups P, S and R were 63.3, 74.8 and 73.5 (p = 0.16). CONCLUSION: Patients from groups S and R underwent more procedures. However, a similar outcome can be achieved, highlighting the importance of managing such injuries in an orthoplastic specialist centre.

Proximally pedicled medial plantar flap based on superficial venous system alone for venous drainage.

The proximally pedicled medial plantar flap is well described for coverage of wounds around the ankle and heel. This flap is usually based on the deep venae comitantes for venous drainage, with the superficial veins divided during dissection. Usually any disruption of the deep venous system of the flap would result in abandoning this choice of flap. Venous congestion is a recognised complication of medial plantar flaps. The patient described in this case report had a medial ankle defect with exposed bone, for which a proximally pedicled medial plantar flap was used. As we raised the flap, both venae comitantes of the medial planter artery were found to be disrupted. The flap was raised based on the superficial veins draining into the great saphenous, as the only system for venous drainage, with no evidence of venous congestion. The flap was successfully transposed into the defect and healed with no complications. The proximally pedicled medial plantar flap can safely rely on the superficial venous system alone for drainage. In addition, preserving the superficial veins minimise the risk of venous congestion in this flap. We recommend preservation of superficial venous system when possible.

A long-term evaluation of 150 costochondral nasal grafts.

INTRODUCTION: This paper reviewed the outcome of cantilevered costochondral grafts used for dorsal nasal augmentation in the management of patients treated at the Australian Craniofacial Unit (ACFU), Adelaide over a 29-year period. MATERIALS AND METHODS: All patients undergoing dorsal nasal augmentation with costochondral grafts as part of their craniofacial management between 1981 and 2009 were identified using the ACFU database, and their medical notes were reviewed. RESULTS: 107 patients (50 M, 57 F), with a mean age of 12.3 years (range: 2-62 years) and requiring a total of 150 costochondral grafts, were identified from the departmental database. Mean follow-up after nasal augmentation was 5.6 years (1 month-31.5 years). 46% of the patients were diagnosed with Binder syndrome/Chondrodysplasia punctata; other diagnoses included Tessier midline clefts, cleft lip and palate and frontonasal dysplasia. A dorsal midline incision (49%) was the commonest method of access, with 84% of patients having mini-screw fixation for graft stabilisation. Complications included screw palpability, infection, skin necrosis and graft fracture. The commonest reasons for a replacement graft were graft atrophy and fracture, infection and persistent deformity. CONCLUSION: Cantilevered nasal costochondral grafting is an excellent technique for improving nasal contour and function in a wide variety of clinical situations.

Autologous reconstruction of the inguinal ligament using pedicled fascia lata flap: A new technique.

INTRODUCTION: A technique of reconstructing the inguinal ligament using a pedicled fascia lata flap is described. PRESENTATION OF CASE: A 62-year-old man was referred with massive bilateral abdominal wall hernias, following numerous attempts at repair and subsequent recurrences. There was complete absence of the right inguinal ligament. The inguinal ligament was reconstructed using a strip of fascia lata, pedicled on the anterior superior iliac spine. This was transposed to cover the external iliac vessels, and sutured to the pubic tubercle. The musculoaponeurotic abdominal wall was reconstructed with two 20cm×20cm sheets of porcine acellular dermal matrix and an overlying sheet of polypropylene mesh, sutured to the remaining abdominal wall muscles laterally, and to both inguinal ligaments. The cutaneous abdominal wall was closed with an abdominoplasty technique. The reconstruction has remained intact nine months following surgery. DISCUSSION: Complete destruction of the inguinal ligament is rare but can occur following multiple operative procedures or trauma. To date, the only published reports of inguinal ligament reconstruction have been performed using synthetic mesh. The use of autologous tissue should reduce the risk of erosion into the neurovascular bundle, seroma formation, and enhance integration into surrounding tissues. CONCLUSION: This new technique for autologous reconstruction of the inguinal ligament provides a safe alternative to the use of synthetic mesh in the operative armamentarium of plastic and hernia surgeons.

Does vascular injury affect the outcome of open tibial fractures?

BACKGROUND: Consensus on the definition of a Gustilo grade IIIC injury was sought, and the influence of vascular injuries on outcome of severe open tibial fractures was investigated. METHODS: Three thousand three hundred fifty-one plastic and orthopedic surgeons were sent questionnaires to determine their interpretation of Gustilo grade IIIC injuries. Notes of patients with severe open tibial injuries reconstructed at Frenchay orthoplastic center with free tissue transfer between 2006 and 2010 were reviewed. Data were collected on patient demographics and vascular integrity. Outcome was measured using the Enneking score. RESULTS: Four hundred seventy-six plastic surgeons and 2875 orthopedic surgeons were contacted. Seven hundred fifty-three responses (22.5 percent) were received; 46.2 percent thought a grade IIIC injury was a devascularized limb, 24.2 percent felt it was a one- or two-vessel injury, 6.9 percent thought it represented any vascular injury, and 22.7 percent had no definite answer. Sixty-eight patients (50 men and 18 women; mean age, 42.7 years) were identified. Fifty had normal angiograms and 18 sustained vascular injuries. Forty-two percent of vascular injuries were to the anterior tibial artery, 37 percent were to the posterior tibial, and 27 percent were to the peroneal. Mean follow-up was 11.2 months. Mean Enneking score for patients without and with vascular injury was 29.8 and 24.4, respectively (p = 0.004). CONCLUSIONS: Vascular injury independently influences long-term limb function. The authors suggest a modification to the current classification to improve communication among surgeons, and advocate the use of preoperative angiography before free soft-tissue reconstruction of severe open tibial fractures.

The osmotic tissue expander: a 5-year experience.

Tissue expansion is a valuable technique in soft tissue reconstruction. Osmotic expanders are self-inflating and obviate the need for repeated injections. In doing so, they eliminate port site problems and may reduce the potential to introduce infection. The use of such expanders has become more common in recent years. We report on our experience with the Osmed™ osmotic expanders over the last 5-years.

Desmoid tumour of the breast.

Desmoid tumour of the breast is a rare fibroblastic tumour whose spectrum ranges from being locally inert to aggressive and destructive, and represents 0.2% of all breast tumours. A 22-year-old woman, 2 years post-cosmetic augmentation mammoplasty with silicone implants, presented with a 6-month history of an enlarging mass in her left breast. Ultrasound showed a well-defined, hypoechoic mass arising within the muscles of the anterior chest, deep beneath the implant and not involving the underlying rib. Fine needle aspiration (FNA) showed features in keeping with changes associated with silicone implants. A provisional diagnosis of scarring and fibroblastic proliferation was made. The lesion was excised and the implant, noted to be intact, was exchanged. Immunochemistry showed strong positivity for vimentin and variable positivity for SMA and desmin, and patchy positivity for beta catenin. A final diagnosis of aggressive fibromatosis was made. Although the association of desmoid tumour and breast implants has been described, this case is unique as the FNA was highly suggestive of a silicone granuloma and the diagnosis of desmoid tumour was made on definitive pathology. The aetiology of desmoid tumours is reviewed and current treatment modalities discussed.