Operative Management of Intra-abdominal Lymphatic Malformations in Children: A Single Tertiary Center Experience.

BACKGROUND: Intra-abdominal lymphatic malformations (LMs) are relatively rare congenital anomalies and can be divided into intra- and retroperitoneal subgroups. This study aims to evaluate the outcomes after surgical resection of intraperitoneal LMs in children. METHODS: The records of all patients ≤16 years of age with intraperitoneal LMs managed at our tertiary center between 2007-2022 were reviewed. Patients with retroperitoneal LMs were excluded. Our preferred approach is surgical exploration for large (>5 cm), symptomatic lesions if they are potentially resectable on imaging. RESULTS: A total of 12 patients (10 males) were diagnosed with intraperitoneal LMs located in the small bowel mesentery or omentum at a median age of 6.6 (range, 0.6-14.4) years. Ten (83%) patients presented with acute symptoms, including abdominal pain (n = 8; 67%) and vomiting (n = 3; 25%). Two (17%) intraperitoneal LMs were found on imaging performed for other reasons. Surgical exploration was performed in 11/12 (92%) cases. Intestinal volvulus around a mesenteric macrocyst had occurred in 4 (36%) patients. Complete macroscopic resection was achieved in 10/11 (91%) patients. Most lesions were localized to a short intestinal segment. One mixed type LM with an extensive mesenteric involvement was biopsied as the lesion was deemed to be unresectable in preoperative imaging and the diagnosis was initially unclear. There was one recurrence (8%) during postoperative follow-up on imaging (median 3.6 (range, 1.5-6.9) years) but the patient has remained asymptomatic and not undergone interventions. 6/8 (75%) of screened intraperitoneal LMs were positive for a somatic PIK3CA mutation. CONCLUSIONS: Most large, pedunculated macrocystic LMs in the small bowel mesentery or omental location were amenable to macroscopic resection. Intraperitoneal LMs tend to present with acute symptoms including a risk for intestinal volvulus based on anatomical location. Our results suggest low recurrence rates or need for further interventions over follow-up. LEVEL OF EVIDENCE: III.

On a collision course: fatal motorcycle and bicycle accidents of adolescents in Finland from 2008 to 2019.

BACKGROUND: Transport injuries are a major cause of mortality among adolescents. Our aim was to evaluate the circumstances and trauma associated with fatal accidents involving adolescents and two-wheeled vehicles. METHODS: We analyzed retrospective data from the Finnish Crash Data Institute from 2008 to 2019 involving 10- to 24-year-old victims of fatal traffic accidents who were injured while riding a bicycle, moped or motorcycle. We collected data on patient characteristics, accident circumstances and possible treatment. These fatalities were compared with national mortality rates among the respective age groups. RESULTS: We identified 147 fatalities over the 12-year period involving 20 bicycle, 50 moped and 77 motorcycle riders. Most accidents involved males (n = 121, 82%). Less than half of vehicles were in good condition (46%); motorized vehicles were often illegally tuned (37%) or had tire problems (31%). Most of the accidents were collisions with another vehicle (n = 99, 67%) or other objects (n = 35, 24%). In 94% of cases, the Injury Severity Score was >25. Head injury was the most common cause of death (62%). Among 15-year-olds, every fifth death was due to accidents on two-wheeled vehicles. CONCLUSIONS: Fatal transport accidents among adolescents comprise several elements that should be incorporated into driver's education and in case of minors, also communicated to parents. These include the condition of the vehicle, proper helmet use and effects of speed on both control of the vehicle and the consequences of a possible collision.

The VASCERN-VASCA Working Group Diagnostic and Management Pathways for Venous Malformations.

To elaborate expert consensus patient pathways to guide patients and physicians toward efficient diagnostics and management of patients with venous malformations. Methods: VASCERN-VASCA (https://vascern.eu/) is a European network of multidisciplinary centers for Vascular Anomalies. The Nominal Group Technique was used to establish the pathways. Two facilitators were identified: one to propose initial discussion points and draw the pathways, and another to chair the discussion. A dermatologist (AD) was chosen as first facilitator due to her specific clinical and research experience. The draft was subsequently discussed within VASCERN-VASCA monthly virtual meetings and annual face-to-face meetings. Results: The Pathway starts from the clinical suspicion of a venous type malformation (VM) and lists the clinical characteristics to look for to support this suspicion. Strategies for subsequent imaging and histopathology are suggested. These aim to inform on the diagnosis and to separate the patients into 4 subtypes: (1) sporadic single VMs or (2) multifocal, (3) familial, multifocal, and (4) combined and/or syndromic VMs. The management of each type is detailed in subsequent pages of the pathway, which are color coded to identify sections on (1) clinical evaluations, (2) investigations, (3) treatments, and (4) associated genes. Actions relevant to all types are marked in separate boxes, including when imaging is recommended. When definite diagnoses have been reached, the pathway also points toward disease-specific additional investigations and recommendations for follow up. Options for management are discussed for each subtype, including conservative and invasive treatments, as well as novel molecular therapies. Conclusion: The collaborative efforts of VASCERN-VASCA, a network of the 9 Expert Centers, has led to a consensus Diagnostic and Management Pathways for VMs to assist clinicians and patients. It also emphasizes the role of multidisciplinary expert centers in the management of VM patients. This pathway will become available on the VASCERN website (http://vascern.eu/).

ErbB signaling is a potential therapeutic target for vascular lesions with fibrous component.

Background: Sporadic venous malformation (VM) and angiomatosis of soft tissue (AST) are benign, congenital vascular anomalies affecting venous vasculature. Depending on the size and location of the lesion, symptoms vary from motility disturbances to pain and disfigurement. Due to the high recurrence of the lesions, more effective therapies are needed. Methods: As targeting stromal cells has been an emerging concept in anti-angiogenic therapies, here, by using VM/AST patient samples, RNA-sequencing, cell culture techniques, and a xenograft mouse model, we investigated the crosstalk of endothelial cells (EC) and fibroblasts and its effect on vascular lesion growth. Results: We report, for the first time, the expression and secretion of transforming growth factor A (TGFA) in ECs or intervascular stromal cells in AST and VM lesions. TGFA induced secretion of vascular endothelial growth factor (VEGF-A) in paracrine fashion, and regulated EC proliferation. Oncogenic PIK3CA variant in p.H1047R, a common somatic mutation found in these lesions, increased TGFA expression, enrichment of hallmark hypoxia, and in a mouse xenograft model, lesion size, and vascularization. Treatment with afatinib, a pan-ErbB tyrosine-kinase inhibitor, decreased vascularization and lesion size in a mouse xenograft model with ECs expressing oncogenic PIK3CA p.H1047R variant and fibroblasts. Conclusions: Based on the data, we suggest that targeting of both intervascular stromal cells and ECs is a potential treatment strategy for vascular lesions having a fibrous component. Funding: Academy of Finland, Ella and Georg Ehnrooth foundation, the ERC grants, Sigrid Jusélius Foundation, Finnish Foundation for Cardiovascular Research, Jane and Aatos Erkko Foundation, GeneCellNano Flagship program, and Department of Musculoskeletal and Plastic Surgery, Helsinki University Hospital.

The VASCERN-VASCA working group diagnostic and management pathways for lymphatic malformations.

Lymphatic malformations (LMs) are developmental defects of lymphatic vessels. LMs are histologically benign lesions, however, due to localization, size, and unexpected swelling, they may cause serious complications that threaten vital functions such as compression of the airways. A large swelling of the face or neck may also be disfiguring and thus constitute a psychological strain for patients and their families. LMs are also highly immunologically reactive, and are prone to recurrent infections and inflammation causing pain as well as chronic oozing wounds. The European Reference Network on Rare Multisystemic Vascular Diseases (VASCERN) is dedicated to gathering the best expertise in Europe. There are only few available guidelines on management and follow up of LMs, which commonly focus on very specific situations, such as head and neck LM (Zhou et al., 2011). It is still unclear, what constitutes an indication for treatment of LMs and how to follow up the patients. The Vascular Anomalies Working Group (VASCA-WG) of VASCERN decided to develop a diagnostic and management pathway for the management of LMs with a Nominal Group Technique (NGT), a well-established, structured, multistep, facilitated group meeting technique used to generate consensus statements. The pathway was drawn following 2 face-to-face meetings and multiple web meetings to facilitate discussion, and by mail to avoid the influence of most authoritative members. The VASCA-WG has produced this opinion statement reflecting strategies developed by experts and patient representatives on how to approach patients with lymphatic malformations in a practical manner; we present an algorithmic view of the results of our work.

Long-term outcomes of lymphatic malformations in children: An 11-year experience from a tertiary referral center.

BACKGROUND: Lymphatic malformations (LMs) are benign, congenital lesions that display considerable heterogeneity in terms of size, location and characteristics. This study aims to describe the long-term outcomes of current management strategies for patients with simple (cystic) LMs. METHODS: The case records of all patients (age ≤16 years) with simple (cystic) LMs at our tertiary institution between 2008 and 2019 were assessed for clinical features, imaging and details of management, including complications. RESULTS: Of a total of 164 patients (60% male), 66% were diagnosed aged <2 years. The median follow-up was 5 (0.3-16) years from diagnosis. LMs were located in the head and neck (40%), extremities (27%), trunk (23%), mediastinum (4%), or intra-abdominally (6%). Types were macrocystic in 47%, microcystic in 21% and mixed in 32%. Sclerotherapy was the most common intervention (38%). Primary surgery had been performed in 12%. Symptomatic improvement, reduction in size, or complete regression were observed in 82/102 (80%) of LMs after interventions; complications from treatment were uncommon (Clavien-Dindo grade I-II: 6%; grade III-IIId: 1%). Sixty-two patients (38%; median age 0.5 (range, 0-12) years) had not required interventions to date; spontaneous regression of the LM occurred in 16 (26%) of these expectantly followed-up cases. CONCLUSIONS: Most studies to date have focused on LMs in selected anatomical locations. Herein the outcomes of an entire population from a single tertiary unit of patients are presented, demonstrating the wide heterogeneity of simple (cystic) LMs and highlighting the importance of individualized, multidisciplinary approaches to care in achieving optimal outcomes.

The VASCERN-VASCA working group diagnostic and management pathways for severe and/or rare infantile hemangiomas.

The European Reference Network on Rare Multisystemic Vascular Diseases (VASCERN), is dedicated to gathering the best expertise in Europe and provide accessible cross-border healthcare to patients with rare vascular diseases. Infantile Hemangiomas (IH) are benign vascular tumors of infancy that rapidly growth in the first weeks of life, followed by stabilization and spontaneous regression. In rare cases the extent, the localization or the number of lesions may cause severe complications that need specific and careful management. Severe IH may be life-threatening due to airway obstruction, liver or cardiac failure or may harbor a risk of functional impairment, severe pain, and/or significant and permanent disfigurement. Rare IHs include syndromic variants associated with extracutaneous abnormalities (PHACE and LUMBAR syndromes), and large segmental hemangiomas. There are publications that focus on evidence-based medicine on propranolol treatment for IH and consensus statements on the management of rare infantile hemangiomas mostly focused on PHACES syndrome. The Vascular Anomalies Working Group (VASCA-WG) decided to develop a diagnostic and management pathway for severe and rare IHs with a Nominal Group Technique (NGT), a well-established, structured, multistep, facilitated group meeting technique used to generate consensus statements. The pathway was drawn following two face-to-face meetings and in multiple web meetings to facilitate discussion, and by mail to avoid the influence of most authoritative members. The VASCA-WG has produced this opinion statement reflecting strategies developed by experts and patient representatives on how to approach patients with severe and rare IH in a practical manner; we present an algorithmic view of the results of our work.

Efficacy of Sirolimus in Patients Requiring Tracheostomy for Life-Threatening Lymphatic Malformation of the Head and Neck: A Report From the European Reference Network.

Extensive lymphatic malformations (LMs) of the head and neck region may require tracheostomy to secure the airway. Treatment of these life-threatening LMs is usually multimodal and includes sclerotherapy and surgery, among others. Recently, systemic therapy with sirolimus has been introduced as an effective treatment for venous and lymphatic malformations; its efficacy and safety profile in patients with extensive LM requiring tracheostomy are, however, as yet not fully known. We performed a retrospective, multicenter review and identified 13 patients with an extensive LM of the head and neck region, who previously underwent placement of tracheostomy and subsequently received sirolimus treatment with the aim to improve the local respiratory situation and remove the tracheostomy. Under sirolimus therapy, tracheostomy could be reversed in 8/13 (62%) patients, a further 2/13 (15%) patients improved markedly, and removal of the tracheostomy was planned at the time of writing, while 3/13 (23%) patients showed insufficient or absent response to sirolimus, rendering tracheostomy reversal not feasible. The median duration of sirolimus treatment until removal of tracheostomy was 18 months (range, 8 months to 5.6 years). Adverse events of sirolimus therapy were common [10/13 (77%) patients], yet the majority of these were mild [9/10 (90%) patients] and only one severe adverse event was recorded, with ulceration and necrosis at a catheter insertion site. In conclusion, sirolimus can be considered an effective and safe salvage treatment in patients with extensive LM even after placement of a tracheostomy, as closure of the latter was possible in the majority of patients (62%) of our retrospective cohort. A better understanding of when to start sirolimus therapy, of the duration of treatment, and of factors allowing the prediction of treatment response will require further investigation.

Pierre Robin sequence causes position-dependent obstructive sleep apnoea in infants.

INTRODUCTION: Obstructive sleep apnoea (OSA) and feeding difficulties are key problems for Pierre Robin sequence (PRS) infants. OSA management varies between treatment centres. Sleep positioning represents the traditional OSA treatment, although its effectiveness remains insufficiently evaluated. DESIGN: To complete a polysomnographic (PSG) evaluation of effect of sleep position on OSA in PRS infants less than 3 months of age. We analysed a 10-year national reference centre dataset of 76 PRS infants. PSG was performed as daytime recordings for 67 in the supine, side and prone sleeping position when possible. In most cases, recording included one cycle of non-rapid eye movement (NREM) and rapid eye movement (REM) sleep in each position. RESULTS: One-third of infants (9/76, 12%) had severe OSA needing treatment intervention prior to PSG. During PSG, OSA with an obstructive apnoea and hypopnoea index (OAHI) >5 per hour was noted in 82% (55/67) of infants. OSA was most severe in the supine and mildest in the side or in the prone positions. The median OAHI in the supine, side and prone positions were 31, 16 and 19 per hour of sleep (p=0.003). For 68% (52/67) of the infants, either no treatment or positional treatment alone was considered sufficient. CONCLUSIONS: The incidence of OSA was 84% (64/76) including the nine infants with severe OSA diagnosed prior to PSG. For the most infants, the OSA was sleep position dependent. Our study results support the use of PSG in the evaluation of OSA and the use of sleep positioning as a part of OSA treatment.

Venous Malformations and Blood Coagulation in Children.

INTRODUCTION: Venous malformations (VMs) are congenital low-flow lesions with a wide spectrum of clinical manifestations. An increasing number of studies link VMs to coagulation abnormalities, especially to elevated D-dimer and decreased fibrinogen. This condition, termed localized intravascular coagulopathy (LIC), may pose a risk for hemostatic complications. However, detailed data on the laboratory variables for coagulation and fibrinolytic activity in VM patients are limited. We addressed this question by systematically analyzing the coagulation parameters in pediatric VM patients. METHODS: We included 62 patients (median age 11.9 years) with detailed laboratory tests for coagulation and fibrinolytic activity at a clinically steady phase. We assessed clinical and imaging features of VMs and their correlations with coagulation and fibrinolysis variables using patient records and MRI. RESULTS: D-dimer was elevated in 39% and FXIII decreased in 20% of the patients, as a sign of LIC. Elevated D-dimer and decreased FXIII were associated with large size, deep location, and diffuse and multifocal VMs. FVIII was elevated in 17% of the patients and was associated with small VM size, superficial and confined location, discrete morphology, and less pain. Surprisingly, antithrombin was elevated in 55% of the patients but without associations with clinical or other laboratory variables. CONCLUSIONS: LIC was common in pediatric patients with VMs. Our results provide a basis for when evaluating the risks of hemostatic complications in children with VMs. Further research is warranted to explore the mechanisms behind coagulation disturbances and their relation to clinical complications.

Teens on Wheels and Consequences: A Six-Year Population-Based Study of Bicycle and Moped Injuries.

INTRODUCTION: Road traffic accidents are a major contributor to morbidity and mortality in the pediatric and adolescent population. Among adolescents, bicycles and light motorized two wheelers are popular means of transportation and increase adolescents' autonomy. Most previous studies on injury risk and incidence have pooled different vehicles and age groups together but more distinct data are required to guide policy. MATERIALS AND METHODS: We gathered data on all 1,432 children and adolescents (age 7-15) who had been treated for injuries from bicycle(n = 841) or moped/motorized scooter (n = 591) accidents at our study centers during a 6-year period (2008-2013). In addition to clinical data, we reviewed Injury Severity Scores (ISS) and calculated incidence estimates for the population of 15-year-olds in the study area. RESULTS: Most bicyclists were injured after a fall (72%), whereas most moped/scooter riders were injured in a collision (51%), most often with a heavier motorized vehicle. Internal injuries, multiple injuries, and severe injuries (ISS >15) were more common among moped/scooter riders than bicyclists (p < 0·001 for all). Moped/scooter riders were more often hospitalized and underwent more operations than bicyclists (p < 0·001 for both). The annual estimated incidence rates of injury were roughly eightfold for 15-year-old moped/scooter riders compared to bicyclists of the same age. CONCLUSION: Cycling is in general a safe mode of transportation and rider safety could be further increased with the proper use of helmets. Although no patient deaths occurred in this study population, mopeds and motorized scooters led to significant morbidity.

Intra-articular venous malformation of the knee in children: magnetic resonance imaging findings and significance of synovial involvement.

BACKGROUND: Intra-articular venous malformations of the knee are an uncommon cause of unilateral knee pain in children. Timely diagnosis is important because lesions with intrasynovial involvement can lead to joint space hemorrhage and secondary cartilage damage. OBJECTIVE: To describe our tertiary center's experience of diagnostics and typical magnetic resonance imaging (MRI) findings. MATERIALS AND METHODS: A retrospective review of all patients ≤16 years of age managed for intra-articular venous malformations of the knee at our institution between 2002 and 2018. RESULTS: Of 14 patients (8 male), the mean age at presentation was 6 years (range: 0-14 years). The most common clinical findings were unilateral knee pain (93%), joint swelling (79%), quadriceps atrophy (50%) and a limited range of motion (29%). Cutaneous manifestations were present in four patients (29%). Contrast-enhanced MRI was available in all cases. After initial MRI, a vascular anomaly etiology had been identified in 11 cases (79%), and correctly reported as a venous malformation in 6 (55%). Three patients received entirely different diagnoses (arthritis, tumor or pigmented villonodular synovitis). Three of seven patients with intrasynovial lesions had established chondropathy at diagnosis. Two patients with lesions of the suprapatellar fat pad had intrasynovial involvement that was not visualised on MRI. CONCLUSION: Although MRI usually permits the diagnosis, clinical awareness of these lesions is important for optimal imaging, accurate interpretation and timely diagnosis. Involvement of the intrasynovial cavity carries a risk of hemarthrosis and progressive chondropathy that may be underestimated by MRI.

Angiomatosis of soft tissue as an important differential diagnosis for intramuscular venous malformations.

Background We aimed to improve management of extremity low-flow vascular malformations by analyzing the histology and imaging of venous malformations (VMs) not responsive to sclerotherapy. Method We reviewed patient records of 102 consecutive patients treated with sclerotherapy for extremity VM in our institution to identify patients who had undergone surgery due to insufficient response. We semi-quantitatively analysed the tissue specimens and compared histological findings to those in preoperative imaging. Result The number of patients operated on was 19 (18.6%); 15 of them had lower-extremity intramuscular lesions. The histological pattern of 13 of these 15 lesions corresponded to angiomatosis of soft tissue (AST). All other lesions treated surgically were VMs. The histology of AST was distinctive but magnetic resonance imaging findings often overlapped with those of VM. Conclusion AST is easily mixed with intramuscular VM. The differentiation of these two entities has therapeutic importance. We emphasize the role of histology in the differential diagnostics of intramuscular slow-flow vascular malformations.

Inheritance Patterns of Infantile Hemangioma.

BACKGROUND AND OBJECTIVE: Infantile hemangioma (IH) includes, among its other risk factors, familial clustering, but a definitive understanding of IH's inheritance model and genetic basis is lacking. Our objective was to collect IH pedigrees in Finland, to study the inheritance patterns of IH within these families, and to analyze the characteristics of familial IHs. METHODS: We identified 185 patients with IH who visited our vascular anomaly clinic between 2004 and 2007. Based on hospital records and a questionnaire sent to these patients and their families, IH characteristics and family history of IH were studied. We compared characteristics between patients with positive (familial) and negative (sporadic) IH family history. Families with positive IH family history were further interviewed for extended pedigree data. RESULTS: One-third of our IH cohort's families reported a family history positive for IH, with IH characteristics and perinatal data between the familial and sporadic cases being similar. IH patients with affected first-degree relatives reported higher long-term discomfort rates than the sporadic cases. Of the 40 families interviewed, 11 included ≥4 IH-affected family members; these were most commonly first-degree relatives (63%). Segregation patterns match with autosomal dominant inheritance with an incomplete penetrance or maternal transmission. We also present a case of monozygotic twins that manifest identical IHs. CONCLUSIONS: Based on this large number of IH pedigrees, we suggest at least 2 possible mechanisms of inheritance: autosomal dominant and maternal transmission. This study highlights the need for additional genetic studies to define inheritance of this common disease.

Risk factors and morbidity of infantile haemangioma: preterm birth promotes ulceration.

AIM: We identified the characteristics of an infantile haemangioma (IH) that predispose children to complications, interventions and long-term morbidity and examined perinatal risk factors for IH. METHODS: We studied children with IHs admitted to Helsinki University Hospital's paediatric vascular anomaly clinic in Finland in 2004-2007 and registered perinatal records, IH characteristics, complications and interventions. These patients received a questionnaire on perinatal data and long-term morbidity resulting from IH. We analysed factors related to complications, interventions and morbidity and compared our cohort's perinatal data to the Finnish Medical Birth Register (FMBR) figures. RESULTS: We approached 185 families, of which 136 replied to the questionnaire. Children with facial, segmental and indeterminate IHs showed more complications, interventions and higher long-term morbidity. Preterm birth predisposed infants to ulceration of IHs, with a 95% confidence interval (CI) of 1.02-5.14 and odds ratio (OR) of 2.29. In addition to earlier known risks, maternal gestational diabetes mellitus rate was higher in our IH cohort than the rate in the FMBR (95% CI 1.39-4.95, OR 2.62). CONCLUSION: Physicians treating IHs should consider the elevated ulceration risk in preterm infants. The association between gestational diabetes mellitus and child's risk for an IH is uncertain and requires further research.

Sclerotherapy complications of peripheral venous malformations.

Background Sclerotherapy is often the primary treatment for peripheral venous malformations. It is mostly sufficient alone, but can be combined with other endovascular techniques. Despite its mini-invasiveness, it is not without potentially severe complications. Here, we systematically report sclerotherapy complications in trunk and extremity venous malformations. Methods We retrospectively assessed the complications of 127 consecutive patients who had received sclerotherapy for peripheral venous malformation in our tertiary care unit (January 2007-August 2013). We applied the Clavien-Dindo classification to grade the severity of complications. We mostly used detergent sclerosants (85.7%), and less often ethanol (5.7%) or bleomycin (4.2%). In 4.2% of the procedures, we combined glue, coils, endovascular laser or particles to sclerotherapy. Results The overall complication rate per procedure was 12.5%. Most complications (83.3%) were local and managed conservatively. We encountered four severe complications, all related to blood coagulopathy. Subcutaneous lesion location and use of ethanol significantly increased the risk of local complications. Conclusion Sclerotherapy alone or combined with other endovascular techniques is a safe method for local venous malformations with moderate risk for conservatively manageable complications. Blood coagulopathy constitutes a risk for, otherwise rare, severe complications.

Driver's education may reduce annual incidence and severity of moped and scooter accidents. A population-based study.

BACKGROUND AND AIMS: In our previous study, the annual number of adolescents treated at Helsinki Children's Hospital and Töölö Trauma Centre for injuries from moped and scooter accidents increased five-fold between 2002 and 2007. In June 2011, the requirements for a moped/scooter license changed to include driver's education and a vehicle handling evaluation. The aim of this retrospective study was to assess the influence of legislative changes on moped and scooter related serious injuries in adolescents. PATIENTS AND METHODS: Data from 520 patients (age 15-16) treated for trauma from moped and scooter accidents at our institutions between January 2008 and December 2013 were included. Case numbers were compared with population data from national databases. Overall incidence, trauma mechanism, injury profile, and proportion of patients requiring hospital admission were calculated for time periods before and after the law amendment. RESULTS: After the law change in 2011, the annual incidence of moped/scooter injuries among 15-year-olds in our area decreased from 0.8% in 2011 to 0.3% in 2013 (p<0.001), and estimated incidence of injuries per new moped/scooter license declined from 1.8% in 2011 to 1.0% in 2013 (p=0.001). Simultaneously, proportions of patients injured in collisions, diagnosed with multiple trauma or requiring in-patient care reduced. CONCLUSIONS: A change in moped/scooter license requirements may have a causal relationship with both reduced number and severity of moped/scooter related injuries in adolescents.

Complications of sclerotherapy for 75 head and neck venous malformations.

Sclerotherapy is one treatment option for head and neck venous malformations (VMs). Evaluation of complication risks is, however, essential to improve its safety. We aimed to systematically report sclerotherapy complications by means of the Clavien-Dindo classification and to distinguish factors predisposing to complications. We identified our institution's head and neck VM patients who received sclerotherapy between 1 January 2007 and 31 August 2013, analyzed patient reports retrospectively, and applied to them the Clavien-Dindo classification. Our 75 VM patients underwent a total of 150 sclerotherapy sessions. The most common sclerosants were 3 % sodium tetradecyl sulfate and polidocanol. Complications occurred in 13 patients (17.3 %) and in 15 sessions (10.0 %); 3 complications required extensive postprocedural treatment and caused permanent morbidity, whereas 12 received conservative treatment. Patients with sclerotherapy complications underwent more treatments (p = 0.009) and more often needed further surgery (p = 0.007). We thus consider sclerotherapy a relatively safe treatment modality for head and neck VMs. To avoid complications, evaluation of VM characteristics and optimal treatment technique in a multidisciplinary team is vital.

An interdisciplinary specialist team leads to improved diagnostics and treatment for paediatric patients with vascular anomalies.

AIM: Patients with vascular anomalies are often misdiagnosed, leading to delayed or improper treatment. The aim of this study was to evaluate the impact of an interdisciplinary team on the diagnosis and treatment of paediatric patients with vascular anomalies. METHODS: We reviewed the paediatric patients evaluated by our interdisciplinary team between 2002 and 2012, analysing the referral diagnosis, final diagnosis, patient age, sex, clinical history, laboratory tests, imaging studies and treatments. RESULTS: Of the 480 patients who were evaluated, 435 (90.6%) had a vascular anomaly: 30.7% of all patients had a tumour and 55.2% had a malformation. Haemangiomas comprised 93.2% of all tumours, while malformations included capillary (9.8%), lymphatic (30.1%), venous (36.8%), arteriovenous (3.8%) and combined slow-flow (7.9%) malformations. Tumours were initially diagnosed correctly in 89.2% of the patients, but only 38.0% of the malformations were diagnosed correctly. Improper treatment was given to 1.4%, due to incorrect diagnoses. CONCLUSION: This study showed that haemangiomas were likely to be diagnosed correctly, but other tumours and vascular malformations were likely to be misdiagnosed. Misdiagnosis seldom led to improper treatment, but probably led to delayed treatment in many cases. The interdisciplinary approach led to improved diagnostics and treatment.

Second branchial cleft fistulae: patient characteristics and surgical outcome.

BACKGROUNDS: Second branchial cleft anomalies predispose to recurrent infections, and surgical resection is recommended as the treatment of choice. There is no clear consensus regarding the timing or surgical technique in the operative treatment of these anomalies. Our aim was to compare the effect of age and operative techniques to patient characteristics and treatment outcome. METHODS: A retrospective study of pediatric patients treated for second branchial sinuses or fistulae during 1998-2012 at two departments in our academic tertiary care referral center. Comparison of patient characteristics, preoperative investigations, surgical techniques and postoperative sequelae. RESULTS: Our data is based on 68 patients, the largest series in the literature. One-fourth (24%) of patients had any infectious symptoms prior to operative treatment. Patient demographics, preoperative investigations, use of methylene blue, or tonsillectomy had no effect on the surgical outcome. There were no re-operations due to residual disease. Three complications were observed postoperatively. CONCLUSIONS: Our patient series of second branchial cleft sinuses/fistulae is the largest so far and enables analyses of patient characteristics and surgical outcomes more reliably than previously. Preoperative symptoms are infrequent and mild. There was no difference in clinical outcome between the observed departments. Performing ipsilateral tonsillectomy gave no outcome benefits. The operation may be delayed to an age of approximately three years when anesthesiological risks are and possible harms are best avoided. Considering postoperative pain and risk of postoperative hemorrhage a routine tonsillectomy should not be included to the operative treatment of second branchial cleft fistulae.