Using three-dimensional geometric morphometry for facial analysis in patients with the oculo-auriculo-vertebral spectrum.

AIM: To utilize three-dimensional (3D) geometric morphometry for visualization of the level of facial asymmetry in patients with the oculo-auriculo-vertebral spectrum (OAVS). MATERIALS AND METHODS: Three-dimensional facial scans of 25 Czech patients with OAVS were processed. The patients were divided into subgroups according to Pruzansky classification. For 13 of them, second 3D facial scans were obtained. The 3D facial scans were processed using geometric morphometry. Soft tissue facial asymmetry in the sagittal plane and its changes in two time spots were visualized using colour-coded maps with a thermometre-like scale. RESULTS: Individual facial asymmetry was visualized in all patients as well as the mean facial asymmetry for every Pruzansky subgroup. The mean colour-coded maps of type I and type IIA subgroups showed no differences in facial asymmetry, more pronounced asymmetry in the middle and the lower facial third was found between type IIA and type IIB (maximum 1.5 mm) and between type IIB and type III (maximum 2 mm). The degree of intensity facial asymmetry in affected middle and lower facial thirds did not change distinctly during the two time spots in all subgroups. CONCLUSIONS: The 3D geometric morphometry in OAVS patients could be a useful tool for objective facial asymmetry assessment in patients with OAVS. The calculated colour-coded maps are illustrative and useful for clinical evaluation.

Necessity of flow cytometry assessment of circulating plasma cells and its connection with clinical characteristics of primary and secondary plasma cell leukaemia.

Plasma cell leukaemia (PCL) is a rare and very aggressive plasma cell disorder. Preventing a dismal outcome of PCL requires early diagnosis with appropriate analytical tools. Therefore, the investigation of 33 patients with primary and secondary PCL was done when the quantity of circulating plasma cells (PCs) using flow cytometry (FC) and morphology assessment was evaluated. The phenotypic profile of the PCs was also analysed to determine if there is an association with clinical outcomes and to evaluate the prognostic value of analysed markers. Our results revealed that FC is an excellent method for identifying circulating PCs as a significantly higher number was identified by FC than by morphology (26·7% vs. 13·5%, P = 0·02). None of secondary PCL cases expressed CD19 or CD20. A low level of expression with similar positivity of CD27, CD28, CD81 and CD117 was found in both PCL groups. A decrease of CD44 expression was detected only in secondary PCL. Expression of CD56 was present in more than half of PCL cases as well as cytoplasmic nestin. A decreased level of platelets, Eastern Cooperative Oncology Group score of 2-3 and lack of CD20+ PC were associated with a higher risk of death. FC could be incorporated in PCL diagnostics not only to determine the number of circulating PCs, but also to assess their phenotype profile and this information should be useful in patients' diagnosis and possible prognosis.

MAMAS (mastopexy-augmentation made applicable and safer): A standardized template of pre-operative marking and step-by-step surgical procedure.

Simultaneous breast augmentation with mastopexy is growing in popularity. It is a complex procedure that can lead to post-operative complications, patient dissatisfaction, and increased risk of litigation. The aim of this study is to describe an approach for the inverted-T augmentation-mastopexy technique, which limits intraoperative modifications, minimizes errors, and decreases post-operative complications and patient dissatisfaction. The study included 107 patients with Regnault's grade I and II ptosis and severe pseudoptosis. All patients were marked according to our novel technique, Mastopexy Augmentation Made Applicable and Safer (MAMAS), and operated by a single surgeon. All patients underwent simultaneous breast augmentation with Siltex Mentor Round Silicone Gel breast implants and mastopexy. Pre-operatively and post-operatively, patients filled the BREAST-Q. The mean follow-up was 24 months. Hundred and seven women received treatment in this study. Sixteen presented with post-operative complications, eleven in the early stage of recovery, and five in the late stage. There were eight cases of minor wound healing complications, all treated conservatively. Two cases of infection were noted, both were treated with oral antibiotics. One patient experienced post-operative bleeding after 13 days, which required surgical revision. In the late stage of recovery, five cases of implant displacement occurred and required revision surgery. No cases of capsular contracture and seromas were reported. According to Breast-Q, all patients were satisfied. MAMAS surgical technique, focusing on precise pre-operative marking for augmentation-mastopexy, is simple and easily reproducible. The procedure has a low complication rate and high patient satisfaction. It provides predictable and stable results over time.

The Slavcleft: A Three-Center Study of the Outcome of Treatment of Cleft Lip and Palate Considering Palatal Shape.

The degree of deviation of palatal shape from the norm may reflect facial growth disturbance in cleft lip and palate (CLP). The objective of this study was to compare the palatal morphology in children treated with different surgical protocols. Palatal shape was assessed with geometric morphometrics (GM) including Procrustes superimposition, principal component analysis (PCA), and permutation tests with 10,000 permutations, in 24 children treated with two-stage repair with a late palatoplasty (Prague group; mean age at assessment 8.9 years), 16 children after two-stage repair with early palatoplasty (Bratislava group; mean age 8.2 years), and 53 children treated with a one-stage repair (Warsaw group, mean age 10.3 years). The non-cleft control group comprised 60 children at 8.6 years. The first five principal components (PCs) accounted for a minimum of 5% of the total shape variability (65.9% in total). The Procrustes distance was largest for the Prague vs. Control pair and smallest for the Prague vs. Bratislava pair. Nonetheless, all intergroup differences were statistically significant (p < 0.01). One can conclude that variations in palatal shape roughly correspond to cephalometric and dental arch relationship findings from prior research. Among the children who underwent a one-stage repair of the complete cleft, their palatal morphology most closely resembled that of the non-cleft controls. Conversely, children who received late palatoplasty exhibited the greatest degree of deviation.

More Than 2% of Circulating Tumor Plasma Cells Defines Plasma Cell Leukemia-Like Multiple Myeloma.

PURPOSE: Primary plasma cell leukemia (PCL) is the most aggressive monoclonal gammopathy. It was formerly characterized by ≥ 20% circulating plasma cells (CTCs) until 2021, when this threshold was decreased to ≥ 5%. We hypothesized that primary PCL is not a separate clinical entity, but rather that it represents ultra-high-risk multiple myeloma (MM) characterized by elevated CTC levels. METHODS: We assessed the levels of CTCs by multiparameter flow cytometry in 395 patients with newly diagnosed transplant-ineligible MM to establish a cutoff for CTCs that identifies the patients with ultra-high-risk PCL-like MM. We tested the cutoff on 185 transplant-eligible patients with MM and further validated on an independent cohort of 280 transplant-ineligible patients treated in the GEM-CLARIDEX trial. The largest published real-world cohort of patients with primary PCL was used for comparison of survival. Finally, we challenged the current 5% threshold for primary PCL diagnosis. RESULTS: Newly diagnosed transplant-ineligible patients with MM with 2%-20% CTCs had significantly shorter progression-free survival (3.1 v 15.6 months; P < .001) and overall survival (14.6 v 33.6 months; P = .023) than patients with < 2%. The 2% cutoff proved to be applicable also in transplant-eligible patients with MM and was successfully validated on an independent cohort of patients from the GEM-CLARIDEX trial. Most importantly, patients with 2%-20% CTCs had comparable dismal outcomes with primary PCL. Moreover, after revealing a low mean difference between flow cytometric and morphologic evaluation of CTCs, we showed that patients with 2%-5% CTCs have similar outcomes as those with 5%-20% CTCs. CONCLUSION: Our study uncovers that ≥ 2% CTCs is a biomarker of hidden primary PCL and supports the assessment of CTCs by flow cytometry during the diagnostic workup of MM.

The Slavcleft: a three-center study of the outcome of treatment of cleft lip and palate. Nasolabial appearance.

BACKGROUND: There is a multitude of protocols of treatment of cleft lip and palate (CLP) worldwide differing in number of operations, surgical techniques, and timings of surgeries. Despite, facial appearance in subjects with CLP is rarely ideal and residual stigmata are easy to notice in many patients irrespective of the protocol. The prospective controlled investigations are optimal for comparing effectiveness of treatment protocols. Because prospective studies are very challenging to perform in CLP field, it is reasonable to retrospectively assess different surgical protocols to identify the promising ones and then to test them in a prospective way. METHODS: Our objective was to assess the nasolabial appearance in a preadolescent Slavic population with unilateral cleft lip and palate (UCLP) by using the 0-200 numeric scale with reference photographs. Patients treated in Warsaw, Poland (n = 32), Prague, Czech Republic (n = 26) and Bratislava, Slovakia (n = 17) were included in this retrospective study. Each cleft center used a unique surgical protocol. Two panels of professional raters (n = 7) and laypeople (n = 10) scored blindly the nasolabial esthetics on cropped frontal and profile images with cropped reference photograph present on the same slide. Intra- and inter-rater agreement was assessed with Cronbach's alpha, intraclass correlation coefficients, t-tests, and Bland-Altman plots. Inter-group differences were evaluated with one-way ANOVA and regression analysis. RESULTS: The agreement within and between raters was acceptable. We found that patients treated in Warsaw, Prague, and Bratislava showed comparable nasolabial appearance on frontal and profile photographs when judged by both professional raters (p > 0.05) and laypeople (p > 0.05). Regression analysis did not identify influence of gender, group (i.e., Warsaw, Prague, and Bratislava), age at lip repair, surgeon, and age at photographic assessment on esthetic outcome (p > 0.05). CONCLUSION: This study showed that none of the surgical protocols showed superiority to produce good nasolabial appearance.

The Slavcleft: A three-center study of the outcome of treatment of cleft lip and palate. Part 2: Dental arch relationships.

PURPOSE: Our aim was to evaluate the dental arch relationship in a preadolescent Slavic population with unilateral cleft lip and palate (UCLP) by using the Goslon Yardstick. MATERIALS AND METHODS: Patients treated in Warsaw, Poland (n = 32), Prague, Czech Republic (n = 33) and Bratislava, Slovakia (n = 30) were included in this retrospective study. Each cleft center used a unique surgical protocol. Three raters scored blindly the dental arch relationship on plaster models. Intra- and inter-rater agreement were assessed with kappa statistics, and differences between the groups were evaluated with one-way analysis of variance. Intra-rater agreement was very good (k > 0.825), while inter-rater agreement was either good or very good (kappa >0.703). RESULTS: We found that patients treated in Warsaw showed a more favorable dental arch relationship (Goslon score = 2.58, SD = 0.77) than patients treated in Prague (Goslon score = 3.21, SD = 1.04). Patients treated in Bratislava showed an intermediate Goslon score (3.07; SD = 0.99). CONCLUSION: This study showed that the dental arch relationships in patients treated in Warsaw with a one-stage repair were more favorable than in patients treated in Prague and Bratislava with a two-stage protocol and comparable to the best results obtained in the Eurocleft and Americleft studies.

The Slav-cleft: A three-center study of the outcome of treatment of cleft lip and palate. Part 1: Craniofacial morphology.

Results of a comparison of the outcomes of treatment of cleft lip and palate can be affected by growth characteristics of populations from which subjects with the clefts are derived. Moreover, conventional cephalometric techniques used in cleft studies for analysis of facial morphology provide only a partial description of shape and are confounded by biases regarding the reference structures. In this retrospective comparison, craniofacial morphology of preadolescent patients with unilateral cleft lip and palate treated in Warsaw (n = 35, age = 10.6 years, SD = 1.2), Prague (n = 38, age = 11.6 years, SD = 1.4), and Bratislava (n = 26, age = 10.5 years, SD = 1.6) were evaluated on cephalograms with the cephalometric method used in the Eurocleft study and geometric morphometrics. We found that patients treated in Warsaw showed slightly more favorable outcomes than in Prague and Bratislava. The differences were related primarily to the position of maxillary alveolar process, cranial base, mandibular angle, and soft tissues. Although no association between a component of treatment protocol and the outcome was found, it is possible that organizational factors such as participation of high-volume, experienced surgeons contributed to these results.