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. Magnetic resonance imaging (MRI) is a crucial examination for confirming venous malformations because it can accurately identify different features of the lesions. OBJECTIVE: The aim of our study was to compare MRI and histopathological findings of venous malformations in children to assess the possibilities and limitations of MRI. MATERIALS AND METHODS: In a retrospective study, two observers independently evaluated the contrast-enhanced MRI of 26 children with venous malformations. Several radiological parameters were considered and compared with histopathological findings. The agreement between the interobserver radiological evaluation and between histopathological and radiological diagnosis was verified using Cohen's kappa. RESULTS: MRI interobserver agreement was excellent for micro-shunts and good for the remaining findings. The radiological-pathological agreement was perfect for the presence/absence of phleboliths and of macro-shunts and almost perfect for the presence of intralesional adipose tissue, lymphatic component, and micro-shunts. CONCLUSION: MRI in venous malformations can detect the presence of phleboliths, adipose tissue, and lymphatic components with excellent accuracy and good to excellent interobserver agreement. Furthermore, MR angiography can detect micro-shunts in simple and combined venous malformations with substantial agreement with histopathological findings.

Giant congenital fibroblastic connective tissue nevus associated with vascular anomalies.

We described an unusual combination of fibroblastic connective nevus (FCTN) already present at birth with underlying vascular anomalies. Overall, the lesion appeared as a large purplish-brown mass in the groin region up to the third of the right thigh, with partial spontaneous regression during the first three months of life. The FCTN observed exhibited several unusual characteristics: it was congenital, large in size, and located in the lower limbs. Finally, it represented the first case described in which an FCTN arose in association with vascular anomalies.

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. The ISSVA Classification that has its roots in the previous Classification of Mulliken and Glowacky distinguishes vascular anomalies in two main groups: vascular tumors and vascular malformations. In head and neck, where vascular anomalies are the most common benign lesions of infancy and childhood, correct diagnosis with the use of unequivocal terminology is more crucial for treatment considering the relevance of structures that can be involved. The aim of this work has been to clarify information and knowledges currently available in the field of vascular anomalies. Referring to ISSVA Classification, clinico- histopathological aspects of each entity have been elucidated.

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 (25.8%) were in the paediatric age group, 8 to 16 years (mean, 13 years). No sex prevalence was found. The presenting symptoms were neurologic in 2 patients (25%) and secondary to venous flow impairment in 6 (75%). At phlebography, venous thrombosis was seen in 2 cases, and functional intermittent obstruction was seen in 4. Seven patients underwent decompressive surgical partial resection of the first rib with transaxillary or supraclavicular access. One patient was treated conservatively. RESULTS: There were no major postoperative complications. Mean hospital stay was 2.7 days. In no patient were there signs of recurrence after a mean follow-up of 18 months (range, 3 to 36 months). CONCLUSIONS: In the authors' experience TOS in paediatric patients occurs with the same symptoms and thrombotic complications as in adults. The same surgical strategy adopted in adult patients is advisable for affected children.

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, 10.3 years). The clinical findings are presented. Surgical treatments included stripping of persistent marginal vein (n = 16), multiple legation of bulky varicosities (n = 10), complementary sclerotherapy (n = 14) and laser photocoagulation (n = 13), and excision of associated lymphatic malformations (n = 5). No mortality or major postoperative morbidity occurred. Follow-up period ranged from 6 months to 2 years. CONCLUSION: These preliminary results suggest that early surgical management of DVH in KTS is safe and could be effective in preventing or minimising the long-term haemodynamic effects of DVH in absence of associated deep venous system anomalies.

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. All major operations carried out on each patient since birth were evaluated. Patients for whom a diagnosis was not reached were excluded. A bibliographic database (MEDLINE) was used to search for the articles published between January 1986 and December 1995 on the analysed operations. The type of evidence supporting the operations was classified as follows: I=evidence from randomised controlled trials (RCTs); II=self-evident intervention (obvious effectiveness not requiring RCTs); III=evidence from prospective and/or comparative studies; IV=evidence from follow-up studies and/or retrospective case series; and V=intervention without substantial evidence for or against results of randomised trials. Seventy operations (32 individual types) were performed on 49 patients (1-5 operations/patient); 18 (26%) were supported by RCTs (type of evidence I). Two patients (3%) received a self-evident intervention (type II); 48 operations (68%) were based on non-randomised prospective or retrospective studies (type III=13%; type IV=55%). Two patients (3%) received an operation not supported by or against convincing scientific evidence (type V). A significant proportion of operations in paediatric surgery is supported by RCTs. However, the vast majority of these trials were conducted on adult patients. Sixty-eight per cent of the operations were based on prospective follow-up studies or retrospective case series, which may not represent solid scientific evidence. More RCTs are needed in paediatric surgery.

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. The intestinal permeability was also studied by injecting Escherichia coli labeled with indium-111 oxine in the proximal ostomy. Translocation of radiolabeled bacteria was quantitated in extraintestinal tissues by radionuclide counts and number of viable organisms and in vivo by scintigraphic imaging. One day after transplant, significant graft damage (score 17.2 +/- 4.2) was observed when compared with the pretransplant value (7.3 +/- 2.6). The degree of tissue injury was similar on days 3 (15.8 +/- 3.5) and 5 (16.1 +/- 3.9) after transplant and remained high on day 7 (11.8 +/- 2.8). The lactulose to mannitol ratio showed a significantly increased permeability on day 1 (17.5) versus pretransplant values (2.6), remained high on day 3 (8.6), and returned to normal values on day 5 (2.8). Translocation of bacteria to distant organs, as measured by both radionuclide counts and number of viable organisms, was strikingly enhanced on day 1 after transplantation, compared with control animals, but returned to the pretransplant value on day 3. A good qualitative and quantitative correlation was observed between radionuclide counts in the extraintestinal organs and in vivo images obtained by scintigraphic scanning. In conclusion, in this model, timing and degree of bacterial translocation do not seem to correlate well, with the exception of the acute post-transplantation phase, with morphologic and permeability changes of the graft. Evaluation of translocation by scintigraphic imaging appears a suitable approach to study in vivo the kinetics and distribution of this process.