Surgically Managed Acquired Vulvar Lymphangioma Circumscriptum.

Lymphangioma circumscriptum is a rare benign disorder of lymphatic channels in deep dermal and subcutaneous layers. It can occur either as a congenital abnormality or as acquired damage to previously normal lymphatic channels. It occurs in different parts of the body, and the vulva is one of the parts which is commonly affected. Here, we presented a 39 years old para 2 who presented with vulvar swelling. She was diagnosed with acquired lymphangioma circumscriptum of the vulva and superficial vulvectomy was done.

Multifocal lymphangioendotheliomatosis without thrombocytopenia or clinical signs of systemic bleeding.

Multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT) is an extremely rare recently described disorder characterized by diffuse congenital skin and gastrointestinal vascular lesions that may be associated with gastrointestinal bleeding and thrombocytopenia. We herein present a case report of multifocal lymphangioendotheliomatosis without thrombocytopenia or extensive extracutaneous involvement (gastrointestinal bleeding). Given the high morbidity and mortality associated with this disease, it is important for clinicians to recognize this disorder in order to select the most appropriate therapeutic approach.

Congenital lymphangioma circumscriptum of the vulva.

Lymphangioma circumscriptum is a rare benign disorder of unknown etiology affecting lymph vessels in the deep dermal and subcutaneous layers. The commonest sites of presentation are the axilla, shoulders, groins, tongue and oral cavity. Lymphangioma circumscriptum of the vulva is very rare can be either congenital or secondary to lymphadenectomy or pelvic radiation in patients with genital cancer, genital tuberculosis, Crohn's disease, cellulitis, morbid obesity and immobilization. We report the case of congenital lymphangioma circumscriptum of the vulva in an adolescent with congenital lymphedema of the leg.

Congenital lymphangioma circumscriptum of vulva presenting as multiple giant mass lesions: a case report and literature review.

A G2L2 33-year-old woman presented to our clinic with large verrucous warty masses in labia major, perinea and the end portion of her spine measuring about 15 × 7, 9 × 7 and 8 × 8 cm, which had been enlarged following puberty and pregnancy. Her right upper and left lower limbs had gross congenital lymphedema. The masses were removed by superficial partial vulvectomy with a qualified margin and repaired without skin graft. Pathology report showed lymphangioma circumscriptum. Several months of follow up revealed normal healing and no recurrence. In conclusion, congenital vulvar lymphangioma, which is a rare disorder, can be highly triggered by hormonal-stimulating situations like puberty and pregnancy. Thus, it is better to visit the affected cases in a timely manner in order to excise these lesions before massive enlargement. In our experience, a superficial partial vulvectomy without skin graft can be a sufficient procedure. Obviously, an appropriate approach during and after surgery would be very important for obtaining a desirable healing.

Microbubbles in macrocysts - Contrast-enhanced ultrasound assisted sclerosant therapy of a congenital macrocystic lymphangioma: a case report.

BACKGROUND: Congenital cystic lymphangiomas are benign malformations due to a developmental disorder of lymphatic vessels. Besides surgical excision, sclerosant therapy of these lesions by intracavitary injection of OK-432 (Picibanil®), a lyophilized mixture of group A Streptococcus pyogenes, is a common therapeutical option. For an appropriate application of OK-432, a detailed knowledge about the structure and composition of the congenital cystic lymphangioma is essential. SonoVue® is a commercially available contrast agent commonly used in sonography by intravenous and intracavitary application. CASE PRESENTATION: Here we report the case of 2 month old male patient with a large thoracic congenital cystic lymphangioma. Preinterventional imaging of the malformation was performed by contrast-enhanced ultrasound after intracavitary application of SonoVue® immediately followed by a successful sclerotherapy with OK-432. CONCLUSIONS: Contrast agent-enhanced ultrasound imaging offers a valuable option to preinterventionally clarify the anatomic specifications of a congenital cystic lymphangioma in more detail than by single conventional sonography. By the exact knowledge about the composition and especially about the intercystic communications of the lymphangioma sclerosant therapy becomes safer and more efficient.

Fetal neck tumors - antenatal and intrapartum management.

In this review article we describe the ante- and perinatal management of fetal neck tumors. Although there are rare congenital anomalies, the clinical consequences for the fetus may be fatal and include airways obstruction, heart insufficiency, pulmonary hypoplasia and cosmetic effect. The right management allows to decrease the fetal and neonatal mortality and morbidity associated with the disease. It includes intrauterine therapy in some cases, mostly in a goitrus hypothyroidism of the fetus, but firstly, an assessment of the fetal airways patency with a subsequent, eventual Ex-Utero Intrapartum Treatment (EXIT).

Diffuse, encasing lymphangioma of the supraglottis.

BACKGROUND: Lymphangioma of the head and neck is considered a rare congenital tumor with the vast majority presenting before two years of age. Surgical excision and sclerosing therapy using OK-432 are recognized as effective treatment options for the majority of these lesions; however, treatment options of laryngeal lesions are less straightforward due to the risk of airway compromise and the desire to maintain the integrity of a functional larynx. We present the case of a four month old male who presented with chief complaint of inspiratory stridor after a single episode of pneumonia. His clinical presentation, flexible fiberoptic laryngoscopic examination, and operative evaluation were consistent with a lymphangioma completely encasing the epiglottis and arytenoids and isolated to the supraglottis. This report outlines our approach to workup and treatment of this rare lesion. METHOD: Case report of presentation, diagnosis and treatment is presented. RESULTS: A four-month old male presented to ENT clinic with inspiratory stridor, worsened with crying, without frank respiratory distress. History and initial examination was consistent with airway obstruction. Flexible fiberoptic laryngoscopy showed laryngomalacia as well as diffuse and symmetric supraglottic edema. Surgical evaluation identified edema limited to the supraglottis, notably along the ventral surface of the epiglottis and dorsal surface of bilateral arytenoids. Biopsies were taken and immunohistochemical staining was performed with strong positivity for D2-40 and CD31, supporting the diagnosis of isolated lymphangioma of the supraglottis. Treatment was performed through multiple point spot welding with a fiber equipped CO2 LASER (OmniGuide TM) at 5 watt continuous power. CONCLUSION: Isolated supraglottic lymphangioma was diagnosed via direct laryngoscopy, with pathologic and immunohistochemical confirmation. Carbon dioxide laser spot welding technique was used with excellent clinical improvement in stridor. Clinical improvement is sustained after 6 months follow-up.

[CONGENITAL CHYLOUS ASCITES: A CASE REPORT].

Congenital chylous ascites is a rare polietiologic entity, requiring close study of the infant's organism by visualization methods in order to diagnose the bening or malignant underlying pathology. In the article is given a report on case of congenital chylous ascites in infant, caused by lymphangioma in the peritoneal cavity. Atypical clinic and the lack of diagnostic standards led to the later detection of the ascite's origin.

Intraorbital Cystic Lesions: An Imaging Spectrum.

Presence of a cyst or a cystic component in an intraorbital mass often narrows the list of differential diagnoses to specific entities. Such a lesion in the orbit may arise from structures within the orbit, globe, and lacrimal system or from neighboring paranasal sinuses or meninges. Common congenital and developmental lesions encountered within the orbit include dermoids and epidermoids, and infrequently coloboma. Parasitic cysts (cysticercus), orbital abscess, mucocele, and vascular lesions are the most common acquired pathologies giving rise to fluid-containing lesions within the orbit. The role of a radiologist is crucial in expediting the diagnosis of orbital lesions with the help of characteristic imaging features on ultrasound, computed tomography, or magnetic resonance imaging. It also helps in identifying complications in others where formulation of an early and effective management strategy is vital for preserving vision.

[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.

A rare case of lymphangioma in the gluteal region: surgical treatment combined with sclerotherapy and laser therapy.

BACKGROUND: Lymphangioma, a rare benign malformation of lymphatic channels, is among the surgically treatable diseases of the lymphatic vessels. However, damage to surrounding structures together with incomplete resection due to adjacent organ infiltration and a high recurrence rate have encouraged the search for alternative treatments. This study aimed to identify the best therapeutic management for this difficult-to-treat disease. METHODS: This report describes a case of congenital lymphangioma in the gluteal region in a young woman. First, sclerotherapy of the lymphangioma was performed and then surgical excision of residual lesions. Finally, as a third therapeutic step, the patient also was subjected to a cycle of laser therapy with the aim of further improving the appearance of the gluteal region. Pulsed dye laser was used to remove cutaneous dyschromias, and an erbium:yttrium-aluminum-garnet laser was used to remove surface irregularities and obtain better skin smoothness. The outcome assessments included standardized photography, the physician's global assessment, the patient's satisfaction scores, and magnetic resonance imaging of the gluteal region. RESULTS: All the lesions of lymphangioma were removed. After the last laser treatment, the skin of the entire treated region was eutrophic and uniform in color, and it appeared to be smooth and soft when palpated. The patient was satisfied with the clinical and aesthetic results achieved. CONCLUSIONS: The combination of sclerotherapy with surgery can be a good solution for the treatment of lymphangioma. The authors also propose laser therapy as an additional therapeutic approach that may follow surgery or sclerotherapy with the aim of improving the skin trophism and the appearance of the affected region.

Fetal magnetic resonance imaging of lymphangiomas.

OBJECTIVES: To evaluate the fetal magnetic resonance imaging findings of lymphangiomas. METHODS: The magnetic resonance scans of eight fetuses with lymphangiomas were evaluated. Magnetic resonance evaluation included: number; size; signal intensities of the lesions; thickness of the septae; configuration of the margins; presence of blood breakdown products; change in size or signal intensity (in four patients with multiple examinations); exact expansion of the lesions to the adjacent anatomical structures; and concomitant pathological findings. Results were compared with postpartum clinical assessment and imaging in seven patients and with autopsy in one patient. RESULTS: Two retroperitoneal, three thoracic, and three cervical lymphangiomas (diameters between 3.3 and 15.6 cm) were included. All lesions consisted of macrocysts, and additional microcystic parts were found in three lymphangiomas. Blood breakdown products were found in one lesion. Agreement with postpartum imaging was excellent. One patient received intrauterine drainage for chylothorax, and one pregnancy was terminated. CONCLUSIONS: Fetal lymphangiomas display the same magnetic resonance imaging features as postnatal lymphangiomas. Intrauterine magnetic resonance characterization of lymphangiomas provides the exact delineation, detection of associated and/or concomitant pathologies, and differential diagnosis among other cystic pathologies. Patient management may be altered with respect to the type and/or time of treatment, and with regard to the continuation or termination of pregnancy.

Looking at appearance of urine before performing a renal biopsy in nephrotic syndrome.

Chyluria results from an abnormal connection between lymphatic bed and urinary tract, causing lymph leakage into the urine. The clinical picture often begins with the appearance of cloudy, milky urines accompanied by monolateral flank pain, malnutrition, weight loss and weakness. We report a case of chyluria that occurred in a young woman who was referred to our unit for nephrotic-range proteinuria. Before performing a renal biopsy, we found that urine analysis demonstrated a massive lipiduria. Therefore, we collected urine samples from each kidney with a selective ureteral catheterization, demonstrating a monolateral source of lipids and proteins. We suspended the renal biopsy and performed a lymphography that showed an inherited lymphangioma on the left lumbar lymphatic bed. Sclerosing solution instillation, renal pedicle lymphatic disconnection or laser therapy are invasive therapeutical options that may cause severe adverse effects. Instead of these procedures, a conservative therapy based on a low-fat diet supplemented with medium-chain triglycerides was chosen. This dietetic schedule was followed by complete resolution of proteinuria and lipiduria. The patient progressively gained body weight and improved quality of life. No relapses were observed after 3 years of follow-up. This case emphasizes the possible role of a noninvasive therapeutical option for patients with chyluria.

[Congenital intrapulmonary lymphangioma and pulmonary hemangioma in 5 infants and young children].

OBJECTIVE: To analyze the clinical, imaging and pathological findings of congenital intrapulmonary lymphangioma and hemangioma in 5 infants and young children. METHOD: Data of 3 cases with congenital intrapulmonary lymphangioma and 2 cases with haemangioma were analyzed. RESULT: All the 5 cases had cough, difficulty in breathing, cyanosis of lips, and shortness of breath. Imaging study indicated cystic and space occupying changes of partial lung. In the two cases of hemangioma, the blood vessels passing through the hemangioma were observed on CT films. Histochemical studies showed that the cystic pockets from removed tissues were different in size and there was a line of flat endothelial tissue around these pockets. Immunochemical studies indicated D2-40 positive, factor VIII weak positive for lymphangioma cases; while in hemangioma cases, factor VIII was positive, D2-40 was negative or weakly positive, and both of cytokeratin was negative. CONCLUSION: It is very important to consider and identify congenital intrapulmonary lymphangioma or hemangioma when a patient has cystic pockets and space occupying change in their lung by imaging and pathological studies.