Relative expression of hormone receptors by endothelial and smooth muscle cells in proliferative and non-proliferative areas of congenital arteriovenous malformations.

BACKGROUND: Episodic growth due to microvascular proliferations (MVP) has been reported in congenital arteriovenous malformations (AVM), which are normally quiescent lesions composed of mature malformed vessels. Since AVM also may worsen under conditions of hormonal dysregulation, we hypothesized that hormonal influences may stimulate this process of vasoproliferative growth through potential interactions with hormone receptors (HR). METHODS: 13 Cases of AVM tissue with histologically documented vasoproliferative growth were analyzed quantitatively for the presence and tissue localization of estrogen receptor (ER), progesterone receptor (PGR), growth hormone receptor (GHR) and follicle-stimulating hormone receptor (FSHR) in relation to resident cells of interest (endothelial cells (EC), smooth muscle cells (SMC) and mast cells (MC)) by applying multiplex immunohistochemistry (IHC) staining. Expression patterns in lesions with MVP and mature vessels were quantified and compared. Available fresh frozen tissues of 3 AVM samples were used to confirm the presence of HR using Reverse-Transcriptase quantitative Polymerase Chain Reaction (RT-qPCR). RESULTS: All four HR studied were expressed in all cases within EC and SMC in areas of MVP and mature vessels, but not in normal skin tissue. ER, GHR, and FSHR showed more expression in EC of MVP and in SMC of mature vessels. RT-qPCR confirmed presence of all 4 HR in both areas. CONCLUSION: Expression of ER, PGR, GHR, and FSHR in vasoproliferative areas of congenital AVM could explain onset of sudden symptomatic growth, as has observed in a subpopulation of patients. These findings may have implications for eventual anti-hormonal targeted therapy in the lesions involved.

Measurement instruments for the core outcome set of congenital melanocytic naevi and an assessment of the measurement properties according to COSMIN: a systematic review.

Background: Congenital melanocytic naevi (CMN) can impact on patients' lives due to their appearance and the risk they carry of neurological complications or melanoma development. The development of a core outcome set (COS) will allow standardised reporting and enable comparison of outcomes. This will help to improve guidelines. In previous research, relevant stakeholders reached a consensus over which core outcomes should be measured in any future care or research. The next step of the COS development is to select the appropriate measurement instruments. Aim: Step 1: to update a systematic review identifying all core outcomes and measurement instruments available for CMN. Step 2: to evaluate the measurement properties of the instruments for the core outcomes. Methods: This study was registered in PROSPERO and performed according to the PRISMA checklist. Step 1 includes a literature search in EMBASE (Ovid), PubMed and the Cochrane Library to identify core outcomes and instruments previously used in research of CMN. Step 2 yields a systematic search for studies on the measurement properties of instruments that were either developed or validated for CMN, including a methodological quality assessment following the COSMIN methodology. Results: Step 1 included twenty-nine studies. Step 2 yielded two studies, investigating two quality of life measurement instruments. Conclusion: Step 1 provided an overview of outcomes and instruments used for CMN. Step 2 showed that additional research on measurement properties is needed to evaluate which instruments can be used for the COS of CMN. This study informs the instrument selection and/or development of new instruments.

Appearance-related concerns and their impact on health-related quality of life in patients with peripheral vascular malformations.

BACKGROUND: Peripheral vascular malformations (VMs) may lead to disfigurement of the body and face, potentially impairing aesthetic appearance. Yet, data on appearance in this population is limited. This study aimed to examine appearance-related concerns and their impact on health-related quality of life (HR-QoL) in patients with VMs. METHODS: In this cross-sectional study, 384 adults and 194 children with VMs were invited to complete the Outcome Measures for VAscular MAlformations (OVAMA) questionnaire to evaluate potential appearance-related concerns on a five-point verbal-rating scale, with higher scores indicating more appearance-related concerns (e.g., colour-difference, facial-distortion, reduced self-esteem, and dissatisfaction with appearance). HR-QoL was evaluated using Patient-Reported Outcome Measurement Information System (PROMIS). Subgroups of patients reporting more appearance-related concerns were identified using univariate analysis. Associations between appearance-related concerns and various HR-QoL domains (e.g., anxiety and social participation) were assessed. RESULTS: A total of 184 patients (32%) completed the questionnaires; 121 patients (66%) reported that one or more appearance outcome was severely affected (i.e., 4-5 out of 5). The following factors statistically significant associated with more appearance-related concerns: capillary/combined origin, facial localization, subcutaneous tissue involvement, larger size, overgrowth, and diagnosis of a syndrome. In adults, dissatisfaction with appearance and reduced self-esteem due to the appearance of the VM correlated with more anxiety and depression symptoms. Reduced self-esteem correlated with less social participation. In children, bodily distortion and being stared at were correlated with less peer relationships. CONCLUSION: Severe appearance-related concerns were present in two-thirds of patients with VMs, impairing their mental HR-QoL. Clinicians should acknowledge appearance-related aspects, monitor psychological well-being, and offer intervention aimed at improving satisfaction with appearance.

Domains and outcomes of the core outcome set of congenital melanocytic naevi for clinical practice and research (the OCOMEN project): part 2.

BACKGROUND: Congenital melanocytic naevi (CMN) can have a great impact on patients' lives owing to perceived stigmatization, and the risk of melanoma development and neurological complications. Development of a core outcome set (COS) for care and research in CMN will allow standard reporting of outcomes. This will enable comparison of outcomes, allowing professionals to offer advice about the best management options. In previous research, stakeholders (patients, parents and professionals) reached consensus on the core domains of the COS. To select the appropriate measurement instruments, the domains should be specified by outcomes. OBJECTIVES: To reach consensus on the specific core outcomes describing the core domains pertaining to clinical care and research in CMN. METHODS: A list of provisional outcomes (obtained earlier) was critically reviewed by the Outcomes for COngenital MElanocytic Naevi (OCOMEN) research team and by relevant stakeholders through an online questionnaire, to refine this list and provide clear definitions for every outcome. When needed, discussion with individual participants was undertaken over the telephone or by email. During an online consensus meeting, stakeholders discussed the inclusion of potential outcomes. After the meeting, participants voted in two rounds for the inclusion of outcomes. RESULTS: Forty-four stakeholders from 19 countries participated. Nine core outcomes were included in the COS relative to clinical care and 10 core outcomes for research. CONCLUSIONS: These core outcomes will enable standard reporting in future care and research of CMN. This study facilitates the next step of COS development: selecting the appropriate measurement instruments for every outcome.

Development of an international core domain set for medium, large and giant congenital melanocytic naevi as a first step towards a core outcome set for clinical practice and research.

BACKGROUND: Medium, large and giant congenital melanocytic naevi (CMN) can impose a psychosocial burden on patients and families, and are associated with increased risk of developing melanoma or neurological symptoms. Lack of consensus on what outcomes to measure makes it difficult to advise patients and families about treatment and to set up best practice for CMN. OBJECTIVES: Fostering consensus among patient representatives and professionals, we aim to develop a core outcome set, defined as the minimum set of outcomes to measure and report in care and all clinical trials of a specific health condition. We focused on the 'what to measure' aspect, the so-called core domain set (CDS), following the COMET and CS-COUSIN guidelines. METHODS: We conducted a systematic review to identify outcomes reported in the literature. Focus groups with patient representatives identified patient-reported outcomes. All these outcomes were classified into domains. Through e-Delphi surveys, 144 stakeholders from 27 countries iteratively rated the importance of domains and outcomes. An online consensus meeting attended by seven patient representatives and seven professionals finalized the CDS. RESULTS: We reached consensus on six domains, four of which were applied to both care and research: 'quality of life', 'neoplasms', 'nervous system' and 'anatomy of skin'. 'Adverse events' was specific to care and 'pathology' to research. CONCLUSIONS: We have developed a CDS for medium-to-giant CMN. Its application in reporting care and research of CMN will facilitate treatment comparisons. The next step will be to reach consensus on the specific outcomes for each of the domains and what instruments should be used to measure these domains and outcomes.

Outcomes and measurement instruments used in congenital melanocytic naevi research: A systematic review.

BACKGROUND: Congenital melanocytic naevi (CMN) can have a great impact on patients' lives due to the remarkable appearance and the risk of developing melanoma and neurocutaneous melanosis. Comparison of treatment efficacy is currently hindered by the lack of standard and uniform outcome reporting; this impedes guidance on optimal management policy. To address this, we aim to perform the first step in developing a core outcome set. With this systematic review, we identified a list of domains, outcomes (including patient reported outcomes) and outcome measurement instruments used in CMN research. METHODS: The review was registered in PROSPERO, registration number CRD42018095235. A search was conducted in EMBASE (Ovid), PubMed and the Cochrane Library from 2006 to January 2019. Studies with 10 or more patients, with all sizes of CMN and reporting outcomes on interventional and conservative management were included. RESULTS: A total of 1,285 individual studies was found; 63 studies were included. We extracted 57 different outcomes and 34 outcome measurement instruments showing large heterogeneity. Patient-reported outcomes were included in 38% of studies. Few outcome measurement instruments were described. Moreover, none of the studies reported that the used instruments were ever validated in a CMN population. CONCLUSION: Heterogeneity exists in outcomes and instruments used in CMN research. The development of a core outcome set may reduce this heterogeneity in future research, thereby enabling treatment comparison and eventually facilitating guidance on management. Furthermore, this overview demonstrates a need for the use and validation of (patient reported) outcome measurement instruments for CMN.

Protocol for the development of core set of domains of the core outcome set for patients with congenital melanocytic naevi (OCOMEN project).

BACKGROUND: Having large congenital melanocytic naevi (CMN) is associated with a psychosocial burden on patients and their parents because of its remarkable appearance and the extra care it may require. Large CMN also pose an increased risk of malignant melanoma or neurocutaneous melanosis. There is a lack of international consensus on what important outcome domains to measure in relation to treatment. This makes it difficult to compare options, to properly inform patients and their parents, and to set up treatment policy for CMN. Therefore, we aim to develop a core outcome set (COS), i.e. the minimum set of outcomes that are recommended to be measured and reported in all clinical trials of a specific health condition. This COS can be used in the follow-up of CMN patients with or without treatment, in clinical research and practice. METHODS: In the Outcomes for Congenital Melanocytic Nevi (OCOMEN) projects, we follow the recommendations from the Core Outcome Measures in Effectiveness Trials (COMET) initiative and the Cochrane Skin Core Outcomes Set Initiative (CS-COUSIN). This project entails the following: (i) a systematic review to identify the previous reported outcomes in literature; (ii) focus groups with national and international patients and parents to identify patient-important outcomes; (iii) classification of outcomes into outcome domains; (iv) e-Delphi surveys in which stakeholders (patients/parents and professionals) can rate the importance of domains and outcomes; and (v) an online consensus meeting to finalize the core outcome domains of the COS. RESULTS: The results will be disseminated by means of publication in a leading journal and presentations in international meetings or conferences. We engage international experts in CMN, both patients and professionals, to ensure the international utility and applicability of the COS.

Port wine stain treatment outcomes have not improved over the past three decades.

BACKGROUND: Since the early '80s, the pulsed dye laser has been the standard treatment tool for non-invasive port wine stain (PWS) removal. In the last three decades, a considerable amount of research has been conducted to improve clinical outcomes, given that a fraction of PWS patients proved recalcitrant to laser treatment. Whether this research actually led to increased therapeutic efficacy has not been systematically investigated. OBJECTIVE: To analyse therapeutic efficacy in PWS patients globally from 1986 to date. METHODS: PubMed was searched for all available PWS trials. Studies with a quartile percentage improvement scale were included, analysed and plotted chronologically. Treatment and patient characteristics were extracted. A mean clearance per study was calculated and plotted. A 5-study simple moving average was co-plotted to portray the trend in mean clearance over time. The data were separately analysed for multiple treatment sessions in previously untreated patients. RESULTS: Sixty-five studies were included (24.3% of eligible studies) comprising 6207 PWS patients. Of all patients, 21% achieved 75-100% clearance. Although a few studies reported remarkably good outcomes in a subset of carefully selected patients, there was no upward trend over time in mean clearance. CONCLUSION: The efficacy of PWS therapy has not improved in the past decades, despite numerous technical innovations and pharmacological interventions. With an unwavering patient demand for better outcomes, the need for development and implementation of novel therapeutic strategies to clear all PWS is as valid today as it was 30 years ago.

Inter- and intrarater reliability of Hurley staging for hidradenitis suppurativa.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic, inflammatory and recurrent skin disease. Different staging instruments have been suggested, but none has achieved universal acceptance. Despite the fact that Hurley staging is one of the most widely applied HS disease severity staging instruments, it has not been validated. OBJECTIVES: To determine the inter- and intrarater reliability of the Hurley staging system. METHODS: Fifteen raters (five plastic surgeons, five general surgeons and five dermatologists) independently staged 30 photos of patients with HS according to Hurley staging at two time points. Reliability was assessed using kappa (&kgr;) statistics, and multivariable logistic regressions were used to determine independent risk factors for photos with discordant staging. RESULTS: Inter-rater reliability was moderate for the three stages of HS [κ = 0·59, 95% confidence interval (CI) 0·48-0·70]. It was moderate for Hurley stage I (κ = 0·45, 95% CI 0·32-0·55) and stage II (κ = 0·51, 95% CI 0·31-0·71) and it was almost perfect for stage III (κ = 0·81, 95% CI 0·62-1·00). The intrarater reliability was substantial for all stages and all raters (κ = 0·65, 95% CI 0·58-0·72). For stage I it was moderate (κ = 0·50, 95% CI 0·38-0·62), for stage II it was substantial (κ = 0·62, 95% CI 0·51-0·73) and for stage III it was almost perfect (κ = 0·82, 95% CI 0·77-0·87). Hurley stages II and III were less likely to result in discordant staging than Hurley stage I (odds ratios 0·47, 95% CI 0·29-0·77 and 0·21, 95% CI 0·12-0·38, respectively). The mean time spent on staging a photo was 14 s. CONCLUSIONS: Hurley staging is reliable for rapid severity assessment of HS, with moderate inter-rater and substantial intrarater reliability for all stages. It is best for assessing Hurley stage III HS, which is an indication for surgery.

Analysis of instability patterns in acute scaphoid fractures by 4-dimensional computed tomographic imaging - A prospective cohort pilot study protocol.

INTRODUCTION: A scaphoid fracture is the most common carpal fracture. When healing of the fracture fails (nonunion), a specific pattern of osteoarthrosis occurs, resulting in pain, restricted wrist motion and disability. Scaphoid fracture classification systems recognize fragment displacement as an important cause of nonunion. The fracture is considered unstable if the fragments are displaced. However, whether and how displaced bone fragments move with respect to one another has not yet been investigated in vivo. With a four-dimensional (4D) computed tomographic (CT) imaging technique we aim to analyze the interfragmentary motion patterns of displaced and non-displaced scaphoid fragments. Furthermore, the correlation between fragment motion and the development of a scaphoid nonunion is investigated. We hypothesize that fragment displacement is not correlated to fragment instability; and concurrent nonunion is related to fragment instability and not to interfragmentary displacement. METHODS: In a prospective single-center cohort pilot study, patients with a one-sided acute scaphoid fracture and no history of trauma to the contralateral wrist are illegible for inclusion. Twelve patients with a non-displaced scaphoid fracture and 12 patients with a displaced scaphoid fracture are evaluated. Both wrists are scanned with 4D-CT imaging during active flexion-extension and radio-ulnar deviation motion. The contralateral wrist serves as kinematic reference. Relative displacement of the distal scaphoid fragment with respect to the proximal scaphoid fragment, is described by translations and rotations (the kinematic parameters), as a function of the position of the capitate. Non-displaced scaphoid fractures are treated conservatively, displaced scaphoid fractures receive intraoperative screw fixation. Follow-up with CT scans is conducted until consolidation at 1½, 3 and 6 months. This trial is registered in the Dutch Toetsingonline trial registration system, number: NL60680.018.17. ETHICS: This study is approved by the Medical Ethics Committee of the Academic Medical Center, Amsterdam.

Awake Flexible Fiberoptic Laryngoscopy to diagnose glossoptosis in Robin Sequence patients.

BACKGROUND: Robin Sequence (RS) is usually defined as the combination of micrognathia, glossoptosis and upper airway obstruction. No objective criteria to diagnose RS exist. To compare management strategy results, a single RS definition using objective criteria is needed. The most frequently used primary diagnostic tool for glossoptosis is awake Flexible Fiberoptic Laryngoscopy (aFFL). OBJECTIVES: To determine the reliability of the aFFL videos as an independent diagnostic tool itself, rather than on the complete evaluation of a patient. DESIGN, SETTING, PARTICIPANTS: All RS individuals from an existing cohort with an available aFFL video were included retrospectively. Thirty age-matched patients without pathologic findings on aFFL were used as controls. aFFL videos were scored by six otolaryngologists as: a. Marked glossoptosis, b. Mild glossoptosis, c. Severity unknown, d. No glossoptosis, e. Insufficient video quality. Videos were anonymised and rated twice, in altered sequences, after a washout period of minimally 2 weeks. MAIN OUTCOME MEASURES: Inter-rater and intrarater agreement. RESULTS: Twenty-six videos of 16 RS patients and 30 videos of controls were included. Inter-rater agreement was fair in the whole group (κ: 0.320) and RS group (κ: 0.226), and fair to moderate in determining presence of glossoptosis (total group κ: 0.430; RS κ: 0.302; controls κ: 0.212). The intrarater agreement for the presence of glossoptosis in RS was moderate (κ: 0.541). CONCLUSIONS: aFFL offers fair to moderate inter-rater agreement, with moderate intrarater agreement, in evaluating glossoptosis in RS. Using aFFL as the single tool in choosing management strategies in RS seems insufficient. There is need for a more reliable, patient friendly diagnostic tool or an internationally accepted aFFL scoring system, to diagnose glossoptosis in RS.

Education in wrist arthroscopy: past, present and future.

PURPOSE: Arthroscopy has assumed an important place in wrist surgery. It requires specific operative skills that are now mainly acquired in the operating room. In other fields of endoscopic surgery, e-learning and virtual reality (VR) have introduced new perspectives in teaching skills. This leads to the following research question: Could the current way of teaching wrist arthroscopy skills be supported using new educational media, such as e-learning and simulator training? METHOD: The literature was searched for available methods of teaching endoscopic skills. Articles were assessed on the evidence of validity. In addition, a survey was sent to all members of the European Wrist Arthroscopy Society (EWAS) to find out whether hand surgeons express a need to embrace modern educational tools such as e-learning or simulators for training of wrist arthroscopy skills. RESULTS: This study shows that the current way of teaching wrist arthroscopy skills can be supported using new educational media, such as e-learning and simulator training. Literature indicates that e-learning can be a valuable tool for teaching basic knowledge of arthroscopy and supports the hypothesis that the use of virtual reality and simulators in training enhances operative skills in surgical trainees. This survey indicates that 55 out of 65 respondents feel that an e-learning program would be a valuable asset and 62 out of the 65 respondents are positive on the additional value of wrist arthroscopy simulator in training. CONCLUSION: Study results support the need and relevance to strengthen current training of wrist arthroscopy using e-learning and simulator training. LEVEL OF EVIDENCE: V.

Analysis of patient satisfaction and donor-site morbidity after different types of breast reconstruction.

BACKGROUND AND AIMS: Breast reconstruction has been shown to improve quality of life. However, factors involved in long-term patient satisfaction are largely unknown. Our aim was to evaluate patient satisfaction and donor-site morbidity in five types of breast reconstruction. MATERIAL AND METHODS: A prospectively collected database of all breast surgery patients at Hospital Rudolfstiftung, Vienna, Austria, was searched for five types of breast reconstruction (2000-2006): implant, latissimus dorsi-flap, latissimus dorsi-flap with implant, free transverse rectus abdominis musculocutaneous-flap, and deep inferior epigastric perforator-flap. Patients were sent a study-specific questionnaire to assess satisfaction. Short-form 36-item health survey was used to analyze (quality of life), and complication data were retrieved from the database and assessed during a follow-up visit. RESULTS: There were 257 patients identified, of whom, 126 responded to the survey (17 implant, 5 latissimus dorsi + implant, 64 latissimus dorsi, 22 transverse rectus abdominis musculocutaneous, and 18 deep inferior epigastric perforator reconstructions). No statistical differences were found in complication or reoperation rates. Deep inferior epigastric perforator-flap patients were significantly more satisfied compared to patients from the implant group (p = 0.007). However, there was no significant difference regarding quality of life scores among the groups. After logistic regression analysis, only "impairment on daily life" showed to be independently correlated with patient satisfaction. This contrary to both operation type and complication rate which did not correlate with patient satisfaction. CONCLUSIONS: Our results indicate that operation type, complication rate, and revision rate did not independently correlate with patient satisfaction. Therefore, to further improve patient satisfaction, future research should be focused on other pro-operative factors, for example, patient education, expectations, and personality characteristics.

The diagnostic accuracy of wrist cineradiography in diagnosing scapholunate dissociation.

Ruptures of the scapholunate ligament (SLL) may cause carpal instability, also known as scapholunate dissociation (SLD). SLD may lead to osteoarthritis of the radiocarpal and midcarpal joints. The aim of this retrospective study was to determine the diagnostic value of wrist cineradiography in detecting SLD. All cineradiographic studies made during a 24 year period were retrieved. All patients who underwent the confirmation method (arthroscopy and/or arthrotomy) and cineradiography were included. In total, 84 patients met the inclusion criteria. Sensitivity, specificity, likelihood ratio, positive predictive value, negative predictive value, and diagnostic accuracy for detecting SLD were calculated for radiography and cineradiography. Cineradiography had a sensitivity of 90%, a specificity of 97%, and a diagnostic accuracy of 0.93 in detecting SLD. Radiography had a sensitivity of 81%, a specificity of 80%, and a diagnostic accuracy of 0.81. Cineradiography has a high diagnostic value for diagnosing SLDs. A positive cineradiography markedly increases the post-test probability of SLD.

Venous thromboembolism and prothrombotic parameters in Klippel-Trenaunay syndrome.

BACKGROUND: In Klippel-Trenaunay syndrome (KTS), a congenital combined vascular (capillary, venous and lymphatic) malformation with localised disturbed growth, venous thromboembolisms (VTEs) are frequently reported in small cohorts. DESIGN AND METHODS: We quantified the frequency of VTE by screening a large KTS-patient cohort with duplex compression ultrasonography. Additionally, we performed a case-control study to evaluate whether coagulation alterations were related to VTE and magnitude of vascular malformations as quantified by magnetic resonance imaging (MRI). RESULTS: Twenty-nine (39%) of 75 patients had signs of current or previous VTE, including superficial venous thrombosis, six (8%) of whom had a deep venous thrombosis or a pulmonary embolism. Compared with 105 controls, 54 adult patients (both: median age 33 years) had higher plasma levels of D-dimer, medians 266 (IQR 195-366) versus 457 (IQR 270-3840) mg÷l (p.

Evidence-based decisions for local and systemic wound care.

BACKGROUND: Decisions on local and systemic wound treatment vary among surgeons and are frequently based on expert opinion. The aim of this meta-review was to compile best available evidence from systematic reviews in order to formulate conclusions to support evidence-based decisions in clinical practice. METHODS: All Cochrane systematic reviews (CSRs), published by the Cochrane Wounds and Peripheral Vascular Diseases Groups, and that investigated therapeutic and preventive interventions, were searched in the Cochrane Database up to June 2011. Two investigators independently categorized each intervention into five levels of evidence of effect, based on size and homogeneity, and the effect size of the outcomes. RESULTS: After screening 149 CSRs, 44 relevant reviews were included. These contained 109 evidence-based conclusions: 30 on venous ulcers, 30 on acute wounds, 15 on pressure ulcers, 14 on diabetic ulcers, 12 on arterial ulcers and eight on miscellaneous chronic wounds. Strong conclusions could be drawn regarding the effectiveness of: therapeutic ultrasonography, mattresses, cleansing methods, closure of surgical wounds, honey, antibiotic prophylaxis, compression, lidocaine-prilocaine cream, skin grafting, antiseptics, pentoxifylline, debridement, hyperbaric oxygen therapy, granulocyte colony-stimulating factors, prostanoids and spinal cord stimulation. CONCLUSION: For some wound care interventions, robust evidence exists upon which clinical decisions should be based.

A pilot study assessing the effectiveness of postoperative splinting after limited fasciectomy for Dupuytren's disease.

Before surgery for Dupuytren's contracture, 54 patients with a proximal interphalangeal (PIP) joint flexion contractures of at least 30° were randomized to receive either a 3-month splinting protocol together with hand therapy under the direct supervision of hand therapists, or the same hand therapy alone. Extension deficit of the PIP joint (primary outcome measure), global perceived effect, pain intensity, comfort and complications were assessed at baseline and 1 year after surgery. In an intention-to-treat analysis, the group assigned to splint-plus-hand therapy had a mean reduction of 21° in flexion contracture after 1 year, compared with 29° in the group receiving hand therapy alone (p = 0.1). There was no difference between the groups regarding other parameters. After operative release of a Dupuytren's contracture, a postoperative protocol using a splint and hand therapy was no better than hand therapy alone in minimizing postoperative flexion contractures.

Surgical treatment of macroglossia in patients with Beckwith-Wiedemann syndrome: a 20-year experience and review of the literature.

Macroglossia is observed in the majority of paediatric patients diagnosed with Beckwith-Wiedemann syndrome and surgical treatment may be indicated. A 20-year retrospective study was performed to evaluate all patients with Beckwith-Wiedemann syndrome who underwent tongue reduction surgery at the authors' institution. A literature review was performed. Surgical treatment was indicated and carried out in 23 patients with a yearly average varying from 0 to 3 subjects. The mean follow-up time was 7 years. Primary indications for surgical treatment of macroglossia included significant tongue protrusion in 30% (n=7), and a combination of clinical problems in 70% (n=16). In all cases, the procedure was performed by the same surgeon using an anterior, V-shaped, wedge resection technique. Two patients had direct postoperative complications. No recurrence of macroglossia was observed in this study. Reported clinical outcome during follow-up demonstrated a satisfactory resting tongue position in all patients. Review of the literature demonstrated variability in surgical indications and techniques, and postoperative outcome. The results of this study indicate that the anterior wedge resection is a simple, effective and safe technique in the surgical treatment of paediatric patients, diagnosed with Beckwith-Wiedemann syndrome, suffering from macroglossia.

Is an isolated cleft lip an isolated anomaly?

INTRODUCTION: It is well known that patients with cleft lip/palate or cleft palate can have associated anomalies. However, there is a relative paucity of information about the possible anomalies associated with an isolated cleft lip. A recent study (Vallino et al., 2008) showed that children with cleft lip and/or alveolus often develop cleft palate-related issues. This inspired us to investigate our population. METHODS: A questionnaire was sent to the parents of 214 children with cleft lip and/or alveolus; 161 questionnaires were returned (response rate (RR): 75%) and included in our study. The study consisted of 91 boys and 70 girls (0.3-13.1 years: mean 6.8 ± 3.5 years). RESULTS: Speech and/or language problems were reported in 34% and ventilation tube insertion in 21% of children with ≥6 years' follow-up. Of the children in that group, 33% reported to have undergone an episode of acute otitis media and 11% reported five episodes or more. Additional congenital anomalies were found in 4% of children with a cleft lip and in 16% of children with a cleft lip/alveolus. CONCLUSION: Our results demonstrate that an isolated cleft lip can often be described as an isolated anomaly, although children with cleft lip and/or alveolus develop cleft palate-related issues more often than anticipated. Therefore, we suggest an intensive monitoring and treatment of children with these types of clefts.

Normal compartment pressures of the lower leg in children.

Compartment pressures have not previously been studied in healthy children. We compared the pressures in the four lower leg compartments of healthy children with those of healthy adults. We included patients aged between two months and six years, and measured the pressures in 80 compartments of 20 healthy children using simple needle manometry. Measurements were repeated in a control group of 20 healthy adults. The mean compartment pressure in the lower leg in children was significantly higher than in adults (p < 0.001). On average, pressures in the four compartments varied between 13.3 mmHg and 16.6 mmHg in the children and between 5.2 mmHg and 9.7 mmHg in the adults. The latter is in accordance with those recorded in the literature. The mean arterial pressure did not relate to age or to pressure in the compartment. The findings of this study that the normal compartment pressure of the lower leg in healthy children is significantly higher than that in adults may be of considerable significance in clinical decision-making in children of this age.