Diagnosis and Treatment of Venous Malformations. Consensus Document of the International Union of Phlebology (IUP): updated 2013.

Venous malformations (VMs) are the most common vascular developmental anomalies (birth defects) . These defects are caused by developmental arrest of the venous system during various stages of embryogenesis. VMs remain a difficult diagnostic and therapeutic challenge due to the wide range of clinical presentations, unpredictable clinical course, erratic response to the treatment with high recurrence/ persistence rates, high morbidity following non-specific conventional treatment, and confusing terminology. The Consensus Panel reviewed the recent scientific literature up to the year 2013 to update a previous IUP Consensus (2009) on the same subject. ISSVA Classification with special merits for the differentiation between the congenital vascular malformation (CVM) and vascular tumors was reinforced with an additional review on syndrome-based classification. A "modified" Hamburg classification was adopted to emphasize the importance of extratruncular vs. truncular sub-types of VMs. This incorporated the embryological ongm, morphological differences, unique characteristics, prognosis and recurrence rates of VMs based on this embryological classification. The definition and classification of VMs were strengthened with the addition of angiographic data that determines the hemodynamic characteristics, the anatomical pattern of draining veins and hence the risk of complication following sclerotherapy. The hemolymphatic malformations, a combined condition incorporating LMs and other CVMs, were illustrated as a separate topic to differentiate from isolated VMs and to rectify the existing confusion with name-based eponyms such as Klippei-Trenaunay syndrome. Contemporary concepts on VMs were updated with new data including genetic findings linked to the etiology of CVMs and chronic cerebrospinal venous insufficiency. Besides, newly established information on coagulopathy including the role of D-Dimer was thoroughly reviewed to provide guidelines on investigations and anticoagulation therapy in the management of VMs. Congenital vascular bone syndrome resulting in angio-osteo-hyper/hypotrophy and (lateral) marginal vein was separately reviewed. Background data on arterio-venous malformations was included to differentiate this anomaly from syndromebased VMs. For the treatment, a new section on laser therapy and also a practical guideline for follow up assessment were added to strengthen the management principle of the multidisciplinary approach. All other therapeutic modalities were thoroughly updated to accommodate a changing concept through the years.

Guideline. Diagnosis and treatment of venous malformations. consensus document of the international union of phlebology (iup): updated-2013.

Venous malformations (VMs) are the most common vascular developmental anomalies (birth defects). These defects are caused by developmental arrest of the venous system during various stages of embryogenesis. VMs remain a difficult diagnostic and therapeutic challenge due to the wide range of clinical presentations, unpredictable clinical course, erratic response to the treatment with high recurrence/persistence rates, high morbidity following nonspecific conventional treatment, and confusing terminology. The Consensus Panel reviewed the recent scientific literature up to the year 2013 to update a previous IUP Consensus (2009) on the same subject. ISSVA Classification with special merits for the differentiation between the congenital vascular malformation (CVM) and vascular tumors was reinforced with an additional review on syndrome-based classification. A "modified" Hamburg classification was adopted to emphasize the importance of extratruncular vs. truncular subtypes of VMs. This incorporated the embryological origin, morphological differences, unique characteristics, prognosis and recurrence rates of VMs based on this embryological classification. The definition and classification of VMs were strengthened with the addition of angiographic data that determines the hemodynamic characteristics, the anatomical pattern of draining veins and hence the risk of complication following sclerotherapy. The hemolymphatic malformations, a combined condition incorporating LMs and other CVMs, were illustratedas a separate topic to differentiate from isolated VMs and to rectify the existing confusion with namebased eponyms such as Klippel-Trenaunay syndrome. Contemporary concepts on VMs were updated with new data including genetic findings linked to the etiology of CVMs and chronic cerebrospinal venous insufficiency. Besides, newly established information on coagulopathy including the role of D-Dimer was thoroughly reviewed to provide guidelines on investigations and anticoagulation therapy in the management of VMs. Congenital vascular bone syndrome resulting in angio-osteo-hyper/hypotrophy and (lateral) marginal vein was separately reviewed. Background data on arterio-venous malformations was included to differentiate this anomaly from syndrome-based VMs. For the treatment, a new section on laser therapy and also a practical guideline for follow up assessment were added to strengthen the management principle of the multidisciplinary approach. All other therapeutic modalities were thoroughly updated to accommodate a changing concept through the years.

[Technical procedure in the use of lyophilized dura for closure of extensive abdominal wall defects]. / Technisches Vorgehen bei der Anwendung von lyophilisierter Dura zum Verschluss grosser Körperwanddefekte.

In the Department of Pediatric Surgery in the Steglitz Hospital of Berlin Free University, major defects in the thoracic and abdominal wall and other sites were treated in 122 children by implantation of lyophilised dura. Surgery was performed 49 times at the abdominal wall, 26 times at the diaphragm, 14 times at the thoracic wall, and 33 times in various other regions. Lyophilised dura, after it has been desantigenised and sterilised, is very versatile in application. However, it is necessary to avoid any area of contact with the lumen of the digestive tract, the tracheo-bronchial system and the body surface. If the implanted dura can be covered with endogenous soft parts and skin, complete incorporation is achieved with the formation of a solid cicatricial plate which provides satisfactory stabilisation of the wall even in extensive defects. Another essential prerequisite is safe anchoring of the dura implants, which can always be achieved if an appropriate suturing technique is employed.

[Megaduodenum in the newborn (author's transl)]. / Das Megaduodenum im Neugeborenenalter.

Megaduodenum presents a functional obstruction which is difficult to correct surgically. Various serious complications have been reported and repeat laparotomy is frequently necessary. 6 newborns with megaduodenum are reported and a sub-total longitudinal resection of the duodenum is advocated. The remainder of the duodenal wall is reconstituted over a tube and this procedure has been used by us 4 times. 3 infants have survived. It may be possible to avoid repeat laparotomy by the method described above.

[The syndrome of the fifth postoperative day after appendicectomy (author's transl)]. / Das Syndrom des 5. postoperativen Tages nach Appendektomie.

41 children were subjected to re-laparotomy because of obstruction after appendectomy. The causes were entero-colitis, occlusive ileus, rupture of the bowel and suture insufficiency. In this paper we report 6 children who had none of these complications. In these 6 children a "5-day syndrome" consisted usually of a fatal outcome with severe general peritonitis without a leakage of the appendix stump. Infection caused the endogenous spread of Bacteroides, following paralytic ileus and septicaemia. Early therapy with antibiotics and eventual re-laparotomy is then necessary.

[Treatment of complications of pulmonary anomalies in childhood (author's transl)]. / Die Behandlung von Komplikationen bei Lungenfehlbildungen im Kindesalter.

Congenital anomalies of the lungs very often remain asymptomatic and are discovered accidentally during routine examinations. Only one-third of 24 patients with congenital pulmonary abnormalities showed acute symptoms; in a further one-third a chronic recurrent symptom complex required surgical intervention as the only possible mode of treatment. The complications arising from these anomalies varied considerably. The most serious was the "acute thorax" in newborns; in a number of cases it needed emergency thoracotomy. The various types and their symptoms are described and their treatment is reviewed.

Causes of death in operated neonates with diaphragmatic defects.

The causes for the high mortality in neonates with diaphragmatic hernia are manifold. These cases have to be classified as being in the "fatal zone" of Boix-Ochoa. Analysis of our patients showed that 27 patients belonged to this group, 15 of whom died. The causes of death were hypoplasia of both lungs in 1 case and intracranial haemorrhage in 1. In 6 neonates there were additional complex cardiac malformations and in 1 other severe malformations. The foetal circulation persisted in 3 infants and a tension pneumothorax was the cause in 2, an intestinal obstruction the cause of death in 1 infant. In these 5 last-mentioned children, improvements in treatment might have possibly prevented death.