Laskimoepämuodostumat : erotusdiagnostiikka, hyytymishäiriöt ja hoidon turvallisuus

Venous malformations (VMs) are congenital vascular anomalies. They cause variable symptoms and are associated with blood coagulation disorders. Sclerotherapy has recently become the primary treatment for most VMs. This thesis aimed to investigate histology and imaging of VMs operated on for unsatisfactory sclerotherapy response, blood coagulation and fibrinolysis activity in pediatric VM patients, and safety aspects of sclerotherapy for trunk and extremity and head and neck VMs. We studied VM patients undergone sclerotherapy at Helsinki University Hospital between 2007 and 2013 and paediatric VM patients collected from the Helsinki Children's hospital outpatient registry. We analyzed histology and imaging findings of extremity VMs operated on for poor response to sclerotherapy (n=102), sclerotherapy complications of all sclerotherapy-treated patients (n=202), and coagulation abnormalities of the paediatric VM patients (n=62). Of the sclerotherapy-treated extremity VMs, 19% were operated on for insufficient sclerotherapy response. The histology of most intramuscular VMs was not consistent with common VM, corresponding instead to angiomatosis of soft tissue (AST). The imaging findings for common VM and AST were overlapping. Pediatric VM patients had significant abnormalities in leukocyte, antithrombin, FVII, FVIII, and FXIII levels, in addition to previously reported elevated D-dimer levels. Disseminated intravascular coagulation did not occur and platelets were generally normal. The sclerotherapy complication rate per procedure was 13% for trunk and extremity VMs and 10% for head and neck VMs. Superficial location and use of ethanol increased the risk for local complications. Blood coagulopathy was a predisposing factor for severe complications. Histology has an important role in the differential diagnostics of intramuscular VMs, as different histological entities require different treatment approaches. VMs are associated with specific abnormalities in coagulation biomarkers, implying a close interrelation between coagulation and angiogenesis. Sclerotherapy for both peripheral and head and neck VMs is generally safe, but entails a risk for severe complications.Laskimoepämuodostumat ovat verisuonten paikallisia kehityshäiriöitä, jotka syntyvät sikiökehityksen aikana. Ne ovat synnynnäisistä suonipoikkeavuuksista tavallisimpia. Laajuudeltaan, sijainniltaan ja oirekuvaltaan laskimoepämuodostumat ovat hyvin vaihtelevia. Tavallisimpien kipu- ja turvotusoireiden lisäksi etenkin pinnalliset ja kookkaat epämuodostumat voivat aiheuttaa merkittävän ulkonäköhaitan. Laskimoepämuodostumiin liittyy myös veren hyytymishäiriöitä, jotka altistavat sekä tukos- että vuotokomplikaatioille. Laskimoepämuodostumien hoito suunnitellaan niistä aiheutuvan haitan mukaan. Nykyisistä hoitovaihtoehdoista skleroterapia eli pistoshoito on useimmissa tapauksissa ensisijainen. Tässä väitöskirjassa tutkittiin tekijöitä laskimoepämuodostumien huonon hoitovasteen taustalla analysoimalla kudosnäytteitä ja kuvantamistutkimuksia. Lisäksi tutkittiin laskimoepämuodostumiin liittyvien veren hyytymishäiriöiden tarkempaa luonnetta lapsipotilailla sekä laskimoepämuodostumien skleroterapiahoidon turvallisuutta ja komplikaatioille altistavia tekijöitä. Tutkimuksen aineistona oli Meilahden sairaalassa vuosina 2007-2013 skleroterapialla hoidetut (n = 202) sekä Lastenklinikalla vuosina 2002-2015 hoitoarviossa olleet laskimoepämuodostumapotilaat (n = 62). Merkittävä osa huonon skleroterapiavasteen vuoksi leikattujen laskimoepämuodostumien kudosnäytteistä sopi paremmin ns. pehmytkudosangiomatoosiin (angiomatosis of soft tissue) kuin tavalliseen laskimoepämuodostumaan. Näiden kahden muutoksen kuvantamislöydökset todettiin kuitenkin varsin samankaltaisiksi, mikä korostaa kudosnäytteiden merkitystä lihaksen sisäisten laskimoepämuodostumien erotusdiagnostiikassa. Poikkeavuudet veren hyytymisarvoissa osoittautuivat aikaisemmin raportoitua monimuotoisemmiksi, mikä vahvistaa käsitystä yhteisestä geneettisestä taustasta hyytymishäiriöiden ja laskimoepämuodostumien taustalla. Skleroterapiaan liittyvien komplikaatioiden raportoinnissa otettiin käyttöön systemaattinen Clavien–Dindo-luokittelu. Komplikaatioista valtaosa oli paikallisia ja itsestään paranevia. Etanolin käytön sklerosoivana aineena ja epämuodostuman pinnallisen sijainnin todettiin altistavan komplikaatioille. Vakavat haittavaikutukset olivat harvinaisia ja liittyivät usein veren hyytymishäiriöihin

Plunging ranula - patient characteristics, treatment, and comparison between different populations

Objectives: To review our clinical experience and characteristics of Finnish patients with plunging ranula and compare our results with reports from other populations. Design: A retrospective study from the electronic hospital records between 2005 and 2016. Setting: The Department of Otorhinolaryngology and Head and Neck Surgery of Helsinki University Hospital, Finland. Results: We describe the characteristics and treatment of 41 patients with MRI-confirmed plunging ranula. Most of our patients were young adults and 88% of them were male. Surgery and sclerotherapy were used for treatment. Conclusions: The vast majority of Finnish plunging ranula patients in our cohort were male, suggesting significant population-related differences in plunging ranula gender distribution. Transoral surgery seemed to result in lowest recurrence rate and was the most common treatment in our clinic.Peer reviewe

Sclerotherapy complications of peripheral venous malformations

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A case of congenital tuberculosis with a favorable outcome in a full term neonate

This case of congenital tuberculosis (TB) emphasizes that TB should be suspected in newborns whose parents are from areas with high incidence of TB or who present with symptoms of an infection unresponsive to wide-spectrum antibiotics.Peer reviewe

Complications of sclerotherapy for 75 head and neck venous malformations

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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 patientsPeer reviewe

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.Peer reviewe

Lasten suonipoikkeavuuksien diagnostiikan on oltava tarkkaa

Vertaisarvioitu.• Lasten yleisin hyvänlaatuinen kasvain on infantiili hemangiooma eli mansikkaluomi. Valtaosa häviää itsestään, mutta lisätutkimuksia ja hoitoa vaativat on tärkeää tunnistaa. • Suoniepämuodostumat ovat suonten synnynnäisiä kehityshäiriöitä. Ne luokitellaan suonityypin mukaan. • Jos lapsella todetaan suonipoikkeavuuden lisäksi raajojen eriparisuus, poikkeava kasvu tai muita anomalioita, on syytä epäillä oireyhtymää.Peer reviewe

Outcomes at a Mean of 13 Years After Proximal Humeral Fracture During Adolescence

Background:The long-term outcomes of a proximal humeral fracture during adolescence are not well known. We investigated the course of primary treatment for these injuries and the long-term outcomes in adulthood, comparing the outcomes with those from age-matched controls. We also compared outcomes after operative and nonoperative treatment via propensity score matching.Methods:We included children who sustained a proximal humeral fracture between the ages of 10.0 and 16.0 years and underwent treatment between 1995 and 2005. Data from primary treatment episodes were extracted from patient files. The patients were invited to a follow-up visit with outcome assessment and radiographs or to a telephone interview if unable to attend. The primary outcome was the Disabilities of the Arm, Shoulder and Hand (DASH) questionnaire. The secondary outcomes were the Simple Shoulder Test (SST), pain at rest and with strenuous use, shoulder range of motion, strength measurements, health-related quality of life (15D), and harms. Participant results were compared with the normal values of an age-matched population. The effect of operative treatment was assessed using propensity score matching and the average treatment effect was calculated.Results:This study included 209 patients (210 fractures). The mean follow-up (and standard deviation) was 13.1 +/- 3.2 years. Outcome data were obtained from 152 participants (153 fractures); 78 participants attended the follow-up visit. The primary treatment episodes were uneventful. The mean scores were 2.5 (95% confidence interval [CI], 1.8 to 3.3 [range, 0 to 13]) for the DASH and 11.7 (95% CI, 11.5 to 11.8 [range, 8 to 12]) for the SST. Other outcomes were similarly good. There were no differences in function compared with the normal population values. Propensity matching showed no treatment effect for operative treatment compared with nonoperative treatment.Conclusions:Proximal humeral fractures of adolescents heal well and rarely result in impairments whether treated operatively or nonoperatively.Peer reviewe