UK Guidelines on the Diagnosis and Treatment of Breast Implant-Associated Anaplastic Large Cell Lymphoma (BIA-ALCL) on behalf of the Medicines and Healthcare products Regulatory Agency (MHRA) Plastic, Reconstructive and Aesthetic Surgery Expert Advisory Group (PRASEAG).

Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is an uncommon T cell Non-Hodgkin Lymphoma (NHL) associated with breast implants. Raising awareness of the possibility of BIA-ALCL in anyone with breast implants and new breast symptoms is crucial to early diagnosis. The tumour begins on the inner aspect of the peri-implant capsule causing an effusion, or less commonly a tissue mass to form within the capsule, which may spread locally or to more distant sites in the body. Diagnosis is usually made by cytological, immunohistochemical and immunophenotypic evaluation of the peri-implant fluid: pleomorphic lymphocytes are characteristically anaplastic lymphoma kinase (ALK) negative and strongly positive for CD30. BIA-ALCL is indolent in most patients but can progress rapidly. Surgical removal of the implant with the intact surrounding capsule (total en-bloc capsulectomy) is usually curative. Late diagnosis may require more radical surgery and systemic therapies and although these are usually successful, poor outcomes and deaths have been reported. By adopting a structured approach, as suggested in these guidelines, early diagnosis and successful treatment will minimize the need for systemic treatments, reduce morbidity and the risk of poor outcomes. These guidelines provide an evidence-based and systematic framework for the assessment and treatment of patients with suspected or proven BIA-ALCL and are aimed at all clinicians involved in the care of people with breast implants.

UK Guidelines on the Diagnosis and Treatment of Breast Implant-Associated Anaplastic Large Cell Lymphoma (BIA-ALCL) on behalf of the Medicines and Healthcare products Regulatory Agency (MHRA) Plastic, Reconstructive and Aesthetic Surgery Expert Advisory Group (PRASEAG).

Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is an uncommon T cell Non-Hodgkin Lymphoma (NHL) associated with breast implants. Raising awareness of the possibility of BIA-ALCL in anyone with breast implants and new breast symptoms is crucial to early diagnosis. The tumour begins on the inner aspect of the peri-implant capsule causing an effusion, or less commonly a tissue mass to form within the capsule, which may spread locally or to more distant sites in the body. Diagnosis is usually made by cytological, immunohistochemical and immunophenotypic evaluation of the aspirated peri-implant fluid: pleomorphic lymphocytes are characteristically anaplastic lymphoma kinase (ALK) negative and strongly positive for CD30. BIA-ALCL is indolent in most patients but can progress rapidly. Surgical removal of the implant with the intact surrounding capsule (total en-bloc capsulectomy) is usually curative. Late diagnosis may require more radical surgery and systemic therapies and although these are usually successful, poor outcomes and deaths have been reported. By adopting a structured approach, as suggested in these guidelines, early diagnosis and successful treatment will minimize the need for systemic treatments, reduce morbidity and the risk of poor outcomes.

UK Guidelines on the Diagnosis and Treatment of Breast Implant-Associated Anaplastic Large Cell Lymphoma on behalf of the Medicines and Healthcare products Regulatory Agency Plastic, Reconstructive and Aesthetic Surgery Expert Advisory Group.

Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is an uncommon T-cell non-Hodgkin Lymphoma (NHL) associated with breast implants. Raising awareness of the possibility of BIA-ALCL in anyone with breast implants and new breast symptoms is crucial to early diagnosis. The tumour begins on the inner aspect of the peri-implant capsule causing an effusion, or less commonly a tissue mass to form within the capsule, which may spread locally or to more distant sites in the body. Diagnosis is usually made by cytological, immunohistochemical and immunophenotypic evaluation of the aspirated peri-implant fluid: pleomorphic lymphocytes are characteristically anaplastic lymphoma kinase (ALK)-negative and strongly positive for CD30. BIA-ALCL is indolent in most patients but can progress rapidly. Surgical removal of the implant with the intact surrounding capsule (total en-bloc capsulectomy) is usually curative. Late diagnosis may require more radical surgery and systemic therapies and although these are usually successful, poor outcomes and deaths have been reported. By adopting a structured approach, as suggested in these guidelines, early diagnosis and successful treatment will minimise the need for systemic treatments, reduce morbidity and the risk of poor outcomes.

Determining postoperative outcomes after cleft palate repair: A systematic review and meta-analysis.

BACKGROUND: A lack of high-level evidence exists on the outcomes of different cleft palate repair techniques. A critical appreciation for the complication rates of common repair techniques is paramount to optimize cleft palate care. METHODS: A literature search was conducted for articles on the measurement of fistula and velopharyngeal insufficiency (VPI) rates following cleft palate repair. Study quality was determined using validated scales. The heterogeneity between studies was evaluated using the I2 statistic. Random-effect model analysis and forest plots were used to report pooled relative risks (RRs) with 95% confidence intervals for treatment effect. P-values of 0.05 were considered statistically significant. RESULTS: Of 2386 studies retrieved, 852 underwent screening and 227 met inclusion criteria (130 studies (57%) on fistulas and 122 studies (54%) on VPI). Meta-analyses were performed using 32 studies. The Furlow technique was associated with less postoperative fistulae than the von Langenbeck and Veau/Wardill/Kilner techniques (RR = 0.56 [0.39-0.79], p < 0.01 and RR = 0.25 [0.12-0.52], p < 0.01, respectively). One-stage repair was associated with less fistulae compared to two-stage repair (RR = 0.42 [0.19-0.96], p = 0.04). The Furlow repair was also associated with a less VPI than the Bardach palatoplasty (RR = 0.41 [0.23, 0.71], p < 0.01), and the one-stage repair was associated with a reduction in VPI rates compared to two-stage repair (RR = 0.55 [0.32, 0.95], p = 0.03). CONCLUSION: The Furlow repair is associated with less risk of fistula formation than the von Langenbeck and Veau/Wardill/Kilner techniques and less VPI compared to the Bardach repair. One-stage repair is associated with less risk of fistula formation and VPI than two-stage repair.

Retraction of the Soft Palate During a Modified Hynes Pharyngoplasty: A Technical Note.

Surgical management of velopharyngeal insufficiency by construction of sphincter pharyngoplasty is well described in the medical literature. Hynes advocated splitting an intact soft palate when it would be helpful for better exposure of the posterior pharyngeal wall for flap inset. We describe a modification to the Hynes pharyngoplasty whereby the soft palate is retracted upward, giving the operator unrestricted surgical access to the salpingopharyngeus muscles and their overlying mucosa. This allows the surgeon to raise and inset the flaps, as described by Hynes, without the need to divide the soft palate. The retraction catheters avoid the need for splitting a soft palate, which has been optimized by either a Furlow or soft palate re-repair in the past, avoiding unnecessary compromise of the integrity and architecture of the soft palate.

Towards a safety net for management of 22q11.2 deletion syndrome: guidelines for our times.

UNLABELLED: The commonest autosomal deletion, 22q11.2 deletion syndrome (22q11DS) is a multisystem disorder varying greatly in severity and age of identification between affected individuals. Holistic care is best served by a multidisciplinary team, with an anticipatory approach. Priorities tend to change with age, from feeding difficulties, infections and surgery of congenital abnormalities particularly of the heart and velopharynx in infancy and early childhood to longer-term communication, learning, behavioural and mental health difficulties best served by evaluation at intervals to consider and initiate management. Regular monitoring of growth, endocrine status, haematological and immune function to enable early intervention helps in maintaining health. CONCLUSION: Guidelines to best practice management of 22q11DS based on a literature review and consensus have been developed by a national group of professionals with consideration of the limitations of available medical and educational resources.

The use of SymNose for quantitative assessment of lip symmetry following repair of complete bilateral cleft lip and palate.

INTRODUCTION: The SymNose computer program has been proposed as an objective method for the quantitative assessment of lip symmetry following unilateral cleft lip repair. This study aims to demonstrate the use of SymNose in patients with complete bilateral cleft lip and palate (BCLP), a group previously excluded from computer-based analysis. METHODS: A retrospective cohort study compared several parameters of lip symmetry between BCLP cases and non-cleft controls. 15 BCLP cases aged 10 (±1 year) who had undergone primary repair were recruited from the patient database at the South West Cleft Unit, Frenchay Hospital. Frontal facial photographs were selected for measurement. 15 age-matched controls were recruited from a local school. Lip symmetry was expressed as: percentage mismatch of left vermillion border and upper lip area over the right, horizontal lip tilt and lateral deviation of the lip. RESULTS: A significant increase in lip asymmetry was found in the BCLP group expressed as upper vermillion border mismatch across computer-defined and user-defined midlines (mean difference was 16.4% (p < 0.01) and 17.5% (p < 0.01) respectively). CONCLUSIONS: The results suggest that a significant degree of lip asymmetry remains in BCLP patients even after primary repair. This challenges previous assumptions that those with bilateral defects would be relatively symmetrical.

Quantitative computer-based assessment of lip symmetry following cleft lip repair.

Objective : To demonstrate an objective method of measuring lip symmetry after cleft lip repair by comparing patients with unilateral cleft lip (UCL) to non-cleft lip controls using the SymNose computer program. Design : Retrospective cohort study comparing several parameters of lip symmetry between UCL cases and controls. Participants were represented by digital photographs that were traced using SymNose. Setting : This study was performed at the South West Cleft Unit, Bristol, U.K. Participants : Forty-four participants aged 10 years (±1 year) were recruited into and completed the study. A consecutive case series of 22 patients with UCL ± palate had previously undergone primary repair. Twenty-two age-matched controls were recruited from a local primary school. Main Outcome Measures : Lip symmetry, expressed as the percentage mismatch of an overlaying of the left upper lip area and upper vermillion border area over the right, horizontal lip tilt, and lateral deviation of the lips. Results : A significant increase in labial asymmetry was seen in the UCL group in the following parameters: upper lip mismatch and upper vermillion border mismatch. No significant differences were observed in horizontal lip tilt or lateral lip deviation. Conclusion : This study supports the use of SymNose for the objective quantitative assessment of lip symmetry as an outcome measure of surgery following cleft lip repair. It allows comparison of surgical techniques and can be used to perform audits. It is a time-efficient process, relatively inexpensive, and straightforward to use.