Splenic cystic lymphangiomatosis in association with omental varices and portal hypertension: A case report.

RATIONAL: Lymphangiomatosis is rare and benign, and slowly proliferating lymphatic vessels of unknown etiology and visceral lymphangiomatosis involving the spleen is rare. Since lymphangiomatosis may be asymptomatic or present as a sense of fullness, splenic cystic lymphangiomatosis is a disease of little concern. PATIENT CONCERNS: A 34-year-old woman suffering from progressive epigastric fullness after oral intake for two weeks. DIAGNOSES: Physical examination showed a palpable mass which was more than 10 cm in size over the left hypochondrium. An abdominal computed tomography disclosed marked splenomegaly with multiple cystic lesions in the spleen, causing external compression with right-sided deviation of the adjacent organs and varices in the upper abdomen. Esophagogastroduodenoscopy revealed portal hypertensive gastropathy. INTERVENTIONS: Conventional total splenectomy was performed in this patient because of an enlarged spleen and unknown etiology, preoperatively. Upon surgery, splenomegaly with polycystic content and varicose vessels over the omentum were noted. Autologous spleen transplantation was not performed because of limited orthotopic and vascularized spleen. OUTCOMES: The patient is doing well 18 months after splenectomy. LESSONS: This was a rare case presenting with splenic cystic lymphangiomatosis in association with omental varices and portal hypertension. Splenic cystic lymphangiomatosis should be considered in the differential diagnosis of patients with a palpable painless mass over the left hypochondrium.

[Extracranial vascular anomalies (hemangiomas and vascular malformations) in children and adolescents--diagnosis, clinic, and therapy]. / Extrakranielle vaskuläre Fehlbildungen (Hämangiome und vaskuläre Malformationen) im Kindes- und Jugendalter--Diagnostik, Klinik und Therapie.

The field of extracranial vascular anomalies is considered as special focus of pediatric otolaryngology and it has shown a rapid development during the last years. The reason for this interest is finally also due to the global acceptance of the classification introduced by the ISSVA (International Society for the Study of Vascular Anomalies). Hemangiomas are the most frequently observed vascular tumors. Today the systemic propranolol therapy is mostly used for therapy of hemangiomas requiring treatment. Increasingly, the topical application of beta blocker is discussed while the benefit in the head and neck seems to be limited. Vascular malformations are classified according to the morphology of the affected part of the vascular system in arterial, venous, arterio-venous, lymphatic, capillary, and combined vascular malformations. Conventional surgery, sclerosing therapy, and laser treatment are invasive options for the treatment of lymphatic malformations. The options for the treatment of venous malformations could be significantly improved during the last years. In this context, the use of Nd:YAG laser, the conservative treatment of the localized disseminated intravascular coagulation with low-molecular weight heparin, the re-discovery of bleomycin as effective sclerosing agent, and the improvement of alcohol-based embolization agents must be mentioned. Today the treatment with dye laser is the preferred therapy for capillary malformations and it is superior to other therapeutic options as for example photodynamic therapy. Arterio-venous malformations as representatives for high-flow lesions are the high-risk lesions. Frequently they are compared to malignant head and neck tumors, in particular when a curative treatment can no longer be assured because of diffuse or multifocal extent and when the disease shows a progressive course. The combined treatment of embolization and surgical resection and if necessary consecutive defect reconstruction have turned out to be appropriate for arterio-venous malformations. Incurable findings are still a major challenge. Despite the introduction of antiangiogenetic drugs in oncology, the medicamentous therapeutic approach could not be established for arterio-venous malformations up to now.

[Lymphedema: anatomy, physiology and pathophysiology of lymphedema, definition and classification of lymphedema and lymphatic vascular malformations]. / Das Lymphödem - Anatomie, Physiologie und Pathophysiologie des Lymphödems, Definition und Klassifizierung des Lymphödems und lymphatischer Gefäßmalformationen.

Lymphedema is a chronic disease associated with a congenital or acquired disorder of the lymphatic vessels or lymph nodes. Untreated lymphedema can lead to complications and disability. Clinical Lymphology deals not only with lymphedema of the extremities but also of the head, the genitals and the internal organs (lymphostatic enteropathy, chylaskos, chylothorax, chylopericard etc). Symptoms of this disorder are often misdiagnosed or not recognized. Ignorance and trivialization of lymphedema causes insufficient treatment, which then is not carried out to the extent as it is possible today by scientific findings. Even today delayed or not optimal treatment causes a long ordeal for many patients.The fact that lymphedema for those affected is a major psychological and social burden, which is limiting the quality of life, has also often been unregarded. The knowledge of anatomy, physiology and pathophysiology as well as the knowledge of causes are necessary for diagnosis, so that early treatment can be initiated.

Classification of vascular disorders in the skin and selected data on new evaluation and treatment.

Cutaneous vascular disorders are common. They include arteries, veins, and lymphatic vessels, or a mixture of them. In this review, we discuss classification, new developments in understanding and treatment of vascular diseases. We focus on infantile hemangiomas and drug therapy, vasculitides with new vasculitic syndromes, yellow nail syndrome and localized lymphatic malformations. Benign cutaneous vascular lesions may be a sign of severe internal diseases. In many cases multidisciplinary treatment is important. The dermatologist can often act as a pilot for these patients.

Orbital lymphangiomas: a review of management strategies.

PURPOSE OF REVIEW: Orbital lymphangiomas present a difficult management problem for the ophthalmologist. This review offers a strategy for managing the condition. RECENT FINDINGS: There have been recent publications discussing the use of intralesional fibrin glue to aid dissection and intralesional sclerosant to shrink the lesion. We discuss the evidence for these treatments and present a case in which these treatments were combined. SUMMARY: This review highlights the classification and presents management options for orbital lymphangioma. We encourage a holistic and individualized approach to the patients, recognizing the variety of clinical manifestations that the condition can cause. In the event of surgical excision, we present the evidence for the use of intralesional fibrin glue and intralesional sclerosant, and describe using both in the same case.

[Vascular tumors of the orbit]. / Tumeurs vasculaires de l'orbite.

Vascular tumors of the orbit include capillary hemangioma, cavernous hemangioma, hemolymphangioma, hemangiopericytoma and a few rare tumors. Capillary hemangioma and hemolymphangioma, occurring mainly in children, are covered in the chapter devoted to childhood tumors. In this chapter, cavernous hemangioma and hemangiopericytoma are discussed as well as rare vascular tumors. Although orbital varix is not a tumor, it is also considered because of the diagnostic problems and the close correlation of orbital varix with a true tumor: hemolymphangioma.

[Vascular lesions of the orbit in children]. / Pathologies vasculaires de l'orbite de l'enfant.

Vascular lesions of the orbit in children are most often hemangiomas and lymphangiomas. Hemangioma is a tumor that tends toward spontaneous regression. Lymphangioma is a malformation that tends to increase in size with growth spurts that can occur until adulthood. The treatment is decided according to the functional impact.

[Differential diagnosis of vascular malformations of the upper aerodigestive tract]. / Zur Differenzialdiagnostik vaskulärer Malformationen im oberen Aerodigestivtrakt.

The differential diagnosis of vascular malformations of the upper aero-digestive tract may represent a diagnostic challenge and become a hindrance for therapy planning. The terminus "extracranial vascular anomalies" of the Head and Neck integrates hemangiomas and vascular malformations. The differential diagnosis contains benign and malign neoplasms, naevi, pigmentations and purpura.

[Vascular malformations (I). Concept, classification, pathogenesis and clinical features]. / Malformaciones vasculares (I). Concepto, clasificación, fisiopatogenia y manifestaciones clínicas.

Vascular malformations are anomalies always present at birth that, contrary to hemangiomas, never regress and may grow during lifetime. Clinical presentation of vascular malformations is extremely variable and ranges from asymptomatic spots of mere aesthetic concern to lesions with high blood flow or located in critical sites that may be life-threatening. Given the low incidence of these disorders it is difficult to establish therapeutic guidelines. In addition to a correct classification of vascular anomalies, it is necessary a multidisciplinary approach for the follow-up and management of these patients. The first part of this review focuses on the different classifications of vascular anomalies, maintaining as reference the one proposed by the International Society for the Study of Vascular Anomalies (ISSVA). Additionally, clinical features of the different subtypes of vascular anomalies as well as their association in certain syndromes are reviewed.

[Vascular tumours and malformation of the orbit]. / Les lésions vasculaires de l'orbite.

Vascular diseases are an important part of orbital pathology. We describe vascular tumours of the orbit and vascular diseases with repercussion on the orbit, from intra or extra orbital origin. The classification of these abnormalities is difficult and several terms are used to describe the same histological entity. The objective of this work is, using the current classification, to illustrate the different imaging aspects of the most frequent vascular diseases of the orbit.

[Management of lymphatic malformations in oral and maxillofacial regions: the rationale according to the new classification].

According to the classification of ISSVA, Waner and Suen, the traditionally called lymphangiomas are now referred to as lymphatic malformations, including both macrocystic and microcystic lesions. They are commonly seen vascular anomalies, and most frequently diagnosed at birth and most often occur in the head and neck area. The etiology of lymphatic malformations is still unknown, resulting in a variety of treatment modalities. This paper reviewed the recent literatures with respect to the indications, contraindications, advantages and disadvantages of surgical excision, sclerotherapy and laser ablation of lymphatic malformations of the head and neck, with detailed discussion of the advances in molecular biology and clinical treatment prospects. It is concluded that although lymphatic malformations are benign lesions, they seldom involute spontaneously. Of all vascular malformations, lymphatic malformations are the most difficult to be eradicated. Their infiltrating nature coupled with the difficulty in distinguishing involved important structures of the head and neck from adjacent normal tissues makes complete surgical extirpation even more difficult. The likelihood of postsurgical recurrence and complications is thus higher than other vascular lesions. Although many treatment protocols are available in clinic, indications, contraindications, advantages and disadvantages exist in each modality. The selection of treatment modalities should depend on the patients' status and techniques available. The treatment protocol should be individualized and comprehensive as well as sequential, in order to obtain the best treatment outcomes. In general, treatment will vary according to the depth and the extent of the lesions. Superficial mucosal microcystic lesions and cervicofacial macrocystic lesions are amenable to ablation with sclerotherapy using Bleomycin and OK-432 with the advantages of the absence of a surgical scar. The sclerosing agent OK-432 is effective for macrocystic lymphatic malformations but showed less promise for microcystic lesions, mixed lesions, and lesions outside the head and neck region. In addition, superficial mucosal microcystic lesions are also amenable to CO2 laser therapy. Deeper microcystic lesions are still challenging head and neck surgeons, which are usually extensive and sometimes need to be resected in stages, and even may be impossible to be completely removed. Somnoplasty shows promise for reduction of tongue lymphatic malformations. Surgical excision, staged when necessary, continues to be integral to management in many cases, but should not be overused without consideration of the histologic types and extent. Localized mucosal microcystic lesions and major cervicofacial macrocystic lesions are amenable to primary excision. Care should be exercised in identifying and preserving important cervical and facial structures because anatomical planes are often distorted. Combined sequential approach is recommended for mixed lesions as well as extensive lesions involving both the mucosa and soft tissues.

Lymphangioma colli--a new classification contributing to prognosis.

The aim of the study was to introduce a classification of lymphangioma colli, which allows us to predict the expected morbidity and prognosis with surgical treatment. We defined 4 types, dependent on the volume of the lymphangioma as assessed by prenatal ultrasound or clinically: type I included tumors which had no or only a minimal effect on the contour of the neck; type II lymphangiomas were smaller than a line drawn at the lateral border of the head; type III tumors exceeded this line; in type IV the lymphangioma extended beyond the midline of the body. We report on the pre- and postoperative findings in 36 cases operated on between 1988 and 2000, mean follow-up was 4.2 years. 25 % of the cases were classified as type I, 36 % as type II, 22 % as type III, and 17 % as type IV. Cesarean section was carried out for 79 % of type III and IV lymphangiomas following prenatal diagnosis (71 %) and for 14 % of type I and II. MRT was indicated in 38 % of type II compared to 86 % in type III and IV lymphangiomas. Endoscopy was necessary in 25 % of type III and in 66 % of type IV lymphangiomas. In 2 cases (type I and IV), imaging revealed an intrathoracic portion. In 64 % of type I and II cases, resection was complete compared to 21 % in infants with more extended tumors. In all type IV cases, at least one further operation was required, likewise in 25 % of type III lymphangiomas. Postoperative effusion and infection occurred significantly more often in group IV than in groups I to III. 5 nerval lesions remained in children with operated type IV lymphangioma and 2 in type III cases. A visible lack of facial symmetry was evident at the follow-up examination in 83 % of type IV cases and moderate asymmetry was found in 19 % of all type I and II cases. Tracheostomy and gastrostomy were required only in type IV. The introduced classification of lymphangioma colli enables predictive statements to be made concerning the morbidity and prognosis with surgical therapy. The prognosis in type I is excellent; in type II, a cosmetically significant facial asymmetry remains in only 15 %. In type III lymphangiomas postoperative complications occurred in 38 % of cases, the necessity for reoperation with definitive lesions occurred in only 25 %. Morbidity gets significantly higher and the prognosis is worse if the lymphangioma exceeds the midline of the body.

Quiste hidatídico primario de cuello en un paciente pediátrico / Primary hydatidic cyst of the neck

Presentamos un paciente de sexo masculino, de 11 años de edad, con masa palpable en región lateral izquierda de cuello de 2 años de evolución: por US quística, adherida a la glándula submaxilar. Se realizó exéresis de la tumoración con diagnóstico histopatológico de hidatidosis. La hidatidosis de localización cervical es muy poco frecuente y su diseminación es arterial. Estudios posteriores demostraron la ausencia de otras localizaciones. Se revee la fisiopatología, diagnósticos diferenciales y literatura de esta inusual presentación (AU)

New treatment options for lymphangioma in infants and children.

Lymphangiomas are congenital malformations of the lymphatic system. These lesions occur most often in the head and neck area, and their treatment continues to be a challenge. Fortunately, a number of advances have occurred in the diagnosis and management of lymphatic malformations in the past decade. The purpose of this article is to clarify the embryology, pathogenesis, histopathology, and classification of these lesions, as well as to describe their various forms of clinical presentation. We provide a complete review of the diagnostic measures available and thoroughly discuss new therapeutic interventions proposed to treat lymphangiomas.

[Haemangiomas and congenital vascular malformations: their classification and diagnosis]. / Hemangiomen en congenitale vasculaire malformaties; classificatie en diagnostiek.

Mulliken and Glowacki's classification of peripheral blood- and lymph-vessel abnormalities is based on their clinical course and cellular characteristics, and is therefore clear to and readily usable by the practising physician. In order to make the diagnostic process more accessible, the Haemangiomas and Congenital Vascular Malformations Nijmegen working group has developed a system of diagnostic guidelines on the basis of this classification. The anamnesis should be directed at the following six distinguishing characteristics: presence of the anomaly at birth, growth, involution, change in volume, pain and outflow. The physical examination is directed at the following five characteristics: the possibility of emptying or pushing aside the anomaly, changes in volume during engorgement, murmur/'thrill'/pulsation, phleboliths, and hyper- or hypotrophy. If a diagnosis still cannot be made, then additional investigations may be carried out. Duplex scanning is usually sufficient for this purpose, after which the nature and extent of the malformation can be determined with MRI. On the basis of the results, the persons involved can be informed as to the prognosis of the malformation and a plan of treatment can be proposed.

[Not all vascular birthmarks are angiomas]. / No todas las manchas vasculares son angiomas.

Vascular anomalies are extremely common but for many years have been poorly understood, partly due to the complex and colorful terminology used, which is full of descriptive terms tinged with folklore. While many lesions look the same, they have a very different prognosis and require very different treatment. Therefore, it is essential to be precise when using the nomenclature. This article reviews the current classification of vascular anomalies and describes recent advances in their diagnosis and management. Our aim is to replace the old confusing terminology and contribute to the unification of concepts and terms. At the same time we stress the importance of interdisciplinary collaboration in order to offer patients accessible and integral treatment.

Linfangioma en territorio de cabeza y cuello. A propósito de siete casos / Lynfhangioma in the head and neck area. A report of seven cases

Los linfangiomas son raras tumoraciones de carácter benigno derivadas de los vasos linfáticos. Se caracterizan por ser quistes y canales linfáticos de pared fina y constituidos por células endoteliales alineadas agrupadas de forma heterogénea y con un tamaño que varía desde unos pocos milímetros hasta más de 20 centímetros. En este artículo serían comentados los distintos aspectos de esta lesión, se valorarán las clasificaciones, aspectos diagnósticos así como las posibilidades terapéuticas. Se analizarán distintos aspectos del tratamiento quirúrgico, así como las posibilidades terapéuticas. Se analizarán distintos aspectos del tratamiento quirúrgico, así como las nuevas terapias con sustancias esclerosantes que están haciendo, dadas las limitaciones del tratamiento quirúrgico en algunos casos, que se replanteen algunas indicaciones terapéuticas (AU)