Venous malformations in children: comparison between magnetic resonance imaging and histopathological findings.

Background: Among low-flow vascular malformations, venous malformations are relatively frequent. The pathological patterns vary in severity and are generally characterized by dilated vessels and low-flow blood that over time can organize into phleboliths. Sometimes small capillary and/or lymphatic vessels may be associated, micro- and/or macro-shunts may form alone or in different combinations, and finally adipose tissue may be interposed between the malformed vessels.

Cerebellar dysplasia related to PIK3CA mutation: a three-case series.

The term PROS (PIK3CA-Related Overgrowth Spectrum) indicates a wide spectrum of overgrowth disorders related to somatic mutations in PIK3CA (phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha) pathway. We present three cases with PIK3CA mutation and clinical characteristics encompassing MCAP (megalencephaly-capillary malformation) condition but lacking all criteria to a certain diagnosis, most of all showing prevalent and peculiar involvement of cerebellar structures at MRI (magnetic resonance imaging) mainly consisting in cortical rim thickening and abnormal orientation of folia axis. These cases expand the spectrum of intracranial MRI features in PIK3CA disorders.

CD10 and CD34 as markers in vascular malformations with PIK3CA and TEK mutations.

Aims: Vascular malformations (vMs) encompass a wide range of diseases often associated with somatic or, more rarely, germinal genetic mutations. A mutation in the PIK3Ca/mTOR pathway is more often involved in various vMs. CD10 and CD34 are cellular markers that may play a role in mesenchymal differentiation and proliferation.

Head and neck vascular anomalies. A multidisciplinary approach and diagnostic criteria.

Vascular anomalies represent a heterogeneous group of pathologies of the circulatory system that can affect any type of hematic and /or lymphatic vessel of different diameter or anatomic site. The extreme variability of tissue types and districts involved by these lesions determines a wide heterogeneity of clinical manifestations, resulting in involvement of different medical expertise. In this context, a commonly agreed terminology is crucial for the appropriate evaluation and multidisciplinary management of patients.

Haemostatic squeezing and purse-string sutures: optimising surgical techniques for early excision of critical infantile haemangiomas.

Background/purpose: Surgical excision of critical infantile haemangiomas (HMs) is usually delayed until intralesional blood flow spontaneously decreases, but fibrofatty tissue and exuberant skin invariably remain even after total involution. The aim of this study was to describe 2 surgical techniques used for early excision in 50 selected cases of HM defined critical in site or size.

Methods: Among a total of 952 patients affected by HM observed from 1999 to 2005, 50 children (5.

Thoracic outlet syndrome in paediatrics: clinical presentation, surgical treatment, and outcome in a series of eight children.

Background/purpose: This report reviews the clinical presentation, surgical treatment, and outcome of 8 children treated for the thoracic outlet syndrome (TOS) during the last 3 years.

Methods: From 1998 through 2001 31 patients were admitted to our Vascular Surgery Unit with TOS. Eight of them (25.

Early surgical management of Klippel-Trenaunay syndrome in childhood can prevent long-term haemodynamic effects of distal venous hypertension.

Purpose: The aim of this study was to evaluate the authors' preliminary experience in early surgical treatment of distal venous hypertension (DVH) in children affected by Klippel-Trenaunay syndrome (KTS).

Methods: Clinical assessment, surgical management, and outcome of 29 children (18 girls, 11 boys) affected by KTS observed from October 1998 to October 2000 were reviewed retrospectively.

Results: Patients ranged in age from 8 months to 17 years (median age at surgery, 10.

Evidence-based operations in paediatric surgery.

It has been assumed that only 10% of medical interventions are supported by solid scientific evidence. The aim of this study was to determine the type of research evidence supporting operations in a tertiary referral paediatric surgical unit. All patients admitted over a 4-week period to two surgical firms were enrolled in the study.

In vivo evaluation of timing, degree, and distribution of bacterial translocation following experimental small bowel transplantation.

These studies were designed to evaluate the correlation between morphologic and functional changes after heterotopic auxiliary small bowel isograft with systemic venous drainage and two ostomies in 20 Lewis rats. Morphologic damage of the graft was scored by full-thickness biopsies before surgery and 1, 3, 5, and 7 days after transplant. Functional evaluation of the graft was done, at the same time points, by urinary excretion of lactulose and mannitol injected in the proximal ostomy.