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Systemic congenital lymphangiomatosis

Souza, Ligia Maria Suppo de; Bentlin, Maria Regina; Abreu, Eliane Souto de; Bacchi, Carlos Eduardo
Fonte: Associação Paulista de Medicina (APM) Publicador: Associação Paulista de Medicina (APM)
Tipo: Artigo de Revista Científica Formato: 1278-1281
ENG
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26.29%
A linfoangiomatose é uma doença rara, caracterizada pela exarcebação da proliferação dos canais linfáticos, ocorrendo em crianças e adultos jovens. Nós descrevemos um caso extremamente raro de linfoangiomatose sistêmica congênita, em um recém-nascido que apresentava ascite e insuficiência respiratória, desenvolvidos imediatamente após o nascimento. O óbito ocorreu nas primeiras horas de vida. Achados de autópsia demonstraram numerosos cistos em tecido mole da região cervical, mediastino, diafragma, e em diversos outros órgãos incluindo: fígado, baço, tireóide e rins. O grave e difuso acometimento de cistos nos pulmões pela linfoangiomatose foi associado ao mau prognóstico e morte no caso relatado.; Systemic lymphangiomatosis is a rare disease characterized by the exageration of lymphatic channel proliferation, occurring in children and young adults. We describe an extremely rare case of congenital systemic lymphangiomatosis in a newborn who had ascitis and respiratory failure develop immediately after delivery. Death occurred during the first hour of life. Autopsy findings showed numerous cysts in soft tissues of the cervical area, mediastinum and diaphragm, and several other organs including the liver, spleen...

Systemic congenital lymphangiomatosis

Souza,Ligia Maria Suppo de; Bentlin,Maria Regina; Abreu,Eliana Souto de; Bacchi,Carlos Eduardo
Fonte: Associação Paulista de Medicina - APM Publicador: Associação Paulista de Medicina - APM
Tipo: Artigo de Revista Científica Formato: text/html
Publicado em 01/10/1996 EN
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26.13%
Systemic lymphangiomatosis is a rare disease characterized by the exageration of lymphatic channel proliferation, occurring in children and young adults. We describe an extremely rare case of congenital systemic lymphangiomatosis in a newborn who had ascitis and respiratory failure develop immediately after delivery. Death occurred during the first hour of life. Autopsy findings showed numerous cysts in soft tissues of the cervical area, mediastinum and diaphragm, and several other organs including the liver, spleen, thyroid and kidneys. The severe and diffuse involvement with cysts in both lungs by lymphangiomatosis was associated with poor prognosis and death in our case.

Cystic Hygroma in an Adult; a Case Report

Veeraraghavan, G; Denny, C; Lingappa, A
Fonte: CoAction Publishing Publicador: CoAction Publishing
Tipo: Artigo de Revista Científica
Publicado em 01/12/2009 EN
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16.7%
Lymphangioma is a benign infiltrative malformation of the lymphatic channels. Cystic lymphangioma or cystic hygroma is a subtype of lymphangioma which exhibits large macroscopic cystic space histologically. The cause of cystic hygroma is believed to be developmental defect or primary Multilocular cystic malformation of dilated lymphatic channels. Cystic hygroma is a common and distinct entity that is not manifested in the oral cavity but occurs in the neck as a large, deep diffuse swelling. They are usually found in the posterior triangle of the neck. They often cross the midline, reaching axilla and mediastinum. Such localization verifies the complexity and extent of the lymphatic system in the cervical region when compared to other regions of the body. The five main locations where cystic hygroma can occur are, cervical (75–90%), axillary (20%), inguinal, retroperitoneal and thoracic. They usually appear as solitary lesions. They are usually infiltrative, often separating fascial planes and incorporating nerves, muscles, and blood vessels. They are fluctuant, freely mobile, compressible, painless and transilluminate well. The skin overlying the lesion is normal and usually there is no associated lymphadenopathy. Various treatment modalities have been tried. Surgery has been the main form of treatment...

Cervical cystic lymphangioma in an adult, diagnosed on FNAC

Shahi, Mohit; Bagga, Permeet K; Mahajan, Nanak C
Fonte: Medknow Publications Pvt Ltd Publicador: Medknow Publications Pvt Ltd
Tipo: Artigo de Revista Científica
Publicado em //2009 EN
Relevância na Pesquisa
16.13%

Vulval Metastasis from Squamous Cell Carcinoma of the Cervix Clinically Presenting as Lymphangioma Circumscriptum

Kim, Won-Jeong; Park, Hyun-Je; Kim, Hoon-Soo; Kim, Su-Han; Ko, Hyun-Chang; Kim, Byung-Soo; Kim, Moon-Bum
Fonte: Korean Dermatological Association; The Korean Society for Investigative Dermatology Publicador: Korean Dermatological Association; The Korean Society for Investigative Dermatology
Tipo: Artigo de Revista Científica
EN
Relevância na Pesquisa
26.29%
Patients with cervical cancer may develop local recurrence or distant metastasis, and the rate of these events is increased in proportion to the clinical stage. Cutaneous metastasis of cervical cancer is very rare and only a few cases have been reported in Korean literature. It is common at the abdominal wall, vulva, and anterior chest wall and mainly presents as an asymptomatic dermal or subcutaneous nodule, ulcer or plaque. We herein report on an interesting case of vulval metastasis from squamous cell carcinoma of the cervix with an unusual clinical manifestation resembling lymphagioma circumscriptum.

Iniencephaly clausus: A case report with review of literature

Kulkarni, Padmaja R.; Rao, Ravikala V.; Alur, Mohan B.; Joshi, S. K.
Fonte: Medknow Publications & Media Pvt Ltd Publicador: Medknow Publications & Media Pvt Ltd
Tipo: Artigo de Revista Científica
Publicado em //2011 EN
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16.13%
Iniencephaly is a rare neural tube defect characterized by extreme retroflexion of the head with the absence of neck due to spinal deformities. The important features that help us to diagnose a case of iniencephaly are occipital bone deficit leading to enlarged foramen magnum, fusion of malformed cervical and thoracic vertebrae, and upward turned face with chin continuous with chest because of the absence of neck. The differential diagnoses include anencephaly with spinal retroflexion, Klippel–Fiel syndrome, nuchal tumors such as teratoma, goiter, and lymphangioma and Jarcho–Levin syndrome. Previously many case reports on radiological features of iniencephaly are published, but there are very few articles on necropsy findings and differential diagnosis. In the present case we have discussed in detail the necropsy findings of iniencephaly clausus with special reference to differential diagnosis.

Cervical Cystic Hygroma

Guruprasad, Yadavalli; Chauhan, Dinesh Singh
Fonte: Springer-Verlag Publicador: Springer-Verlag
Tipo: Artigo de Revista Científica
EN
Relevância na Pesquisa
26.37%
Cystic hygroma or cystic lymphangioma is a congenital malformation of the lymphatic system that manifests itself as a soft, benign, and painless mass. It is widely accepted that they arise from the remnants of embryonic lymphatic tissue which retains the potential for proliferation. They grow in the fashion of sprouting and are capable of transgressing anatomical boundaries. They can occur almost at any anatomical site. However, 75–80% cystic hygromas are located in the head and neck region. In the neck, they are typically located within the posterior cervical triangle. The majority of cases (80–90%) are diagnosed under the age of two. We present a case of cervical cystic hygroma in a 6 year old male child which was surgically treated.

Dumbbell–shaped lymphangioma of neck and thorax

Kumar, Shailendra; Kumar, Sanjeev; Kumar, Surender; Prakash, Ved; Kumar, Vijay
Fonte: Medknow Publications & Media Pvt Ltd Publicador: Medknow Publications & Media Pvt Ltd
Tipo: Artigo de Revista Científica
Publicado em //2014 EN
Relevância na Pesquisa
26.4%
Vascular malformations consist of a spectrum of lesions involving all parts of the body. They have different terminologies like vascular tumors, vascular malformations, vascular anomalies, and so on, which create a lot of confusion in understanding and treating these pathologies. Of late, classification on the basis of cellular kinetics and clinical behavior has been devised. Hemangioma is the most common vascular tumor. Vascular malformations are of either lymphatic or capillary and venous origin. Sometimes they are of a mixed origin. Lymphangiomas are common in the face and neck area. They are also not unusual in the mediastinum. We present a case where a huge lymphangioma occupied the right supraclavicular area of the neck, extending to nearly the entire right thoracic cavity, compressing the whole lung. The patient had occasional symptoms of cough. Ultrasonography (US) and computed tomography (CT) images clearly described the lesion as cystic, occupying both the supraclavicular and thoracic cavity compressing the lung parenchyma. Surgical excision was planned and we approached the lesion via both cervical and thoracotomy incisions. Complete excision was done, and the lung expended fully after the surgery.

Cervical Cystic Hygroma in an Adult

Derin, Serhan; Şahan, Murat; Dere, Yelda; Çullu, Neşat; Şahan, Leyla
Fonte: Hindawi Publishing Corporation Publicador: Hindawi Publishing Corporation
Tipo: Artigo de Revista Científica
EN
Relevância na Pesquisa
26.29%
Cystic hygromas/lymphangiomas are extremely rare malformations in adults. They are usually seen in infants and children under 2 years of age. En bloc resection is difficult due to the adhesive characteristics of the tumors. Inadequate surgical intervention often leads to recurrent disease. We report herein the case of a cystic hygroma/lymphangioma that presented as an uncommon mass on the cervical region in an adult, together with its histopathological, radiologic, and operative features.

Neonatal tumors.; Tumores no período neonatal.

Costa, Carla; Serviço de Neonatologia, Hospital de São João, Porto.; Rocha, Gustavo; Grilo, Marta; Bianchi, Ricardo; Sotto Mayor, Tânia; Monteiro, Joaquim; Guimarães, Hercília
Fonte: Ordem dos Médicos Publicador: Ordem dos Médicos
Tipo: info:eu-repo/semantics/article; article; article; info:eu-repo/semantics/publishedVersion Formato: application/pdf
Publicado em 14/06/2010 POR
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16.98%
Tumors affecting the fetus and newborn differ from those found in older children and adults, leading to new diagnostic and therapeutic challenges.To evaluate the main clinical aspects related to neonatal tumors.Retrospective analysis of clinical data from newborn patients admitted to the Service of Neonatology of São João Hospital between 1996 and 2006, with the diagnosis of tumor or neoplasia.Total = 32 cases, 16M/16F, birth weight: 3146 g (965-4590), gestational age 38 weeks (28-41), seven (22%) preterm, C-section rate 75% (n = 24), two with EXIT procedure.Teratoma (n = 8); lymphangioma (n = 7), neuroblastoma (n = 6), haemangioma (n = 5), other solid tumors (n = 6); acute lymphoblastic leukemia (n = 1). Prenatal diagnosis 50% (n = 16). Teratoma: immature (n = 3); mature (n = 5), sacrococcygeal location (n = 5); cervical (n = 3); total macroscopic resection (n = 8). Cystic lymphangioma: cervical location (n = 5); cervicothoracic location (n = 1); thoracoabdominal location (n = 1); total macroscopic resection (n = 7). NEUROBLASTOMA: abdominal location (n = 5); cervical location (n = 1); deletion 1p (n = 0); oncogene n-myc amplification (n = 0); stage I (n = 1); IIB (n = 1); III (n = 3); IV (n = 1). Chemotherapy (n = 5), according to the (n = 2)...

Kaposiform hemangioendothelioma in tonsil of a child associated with cervical lymphangioma: a rare case report

Rekhi, Bharat; Sethi, Shweta; Kulkarni, Suyash S; Jambhekar, Nirmala A
Fonte: BioMed Central Publicador: BioMed Central
Tipo: Artigo de Revista Científica
Publicado em 23/05/2011 EN
Relevância na Pesquisa
36.54%
Kaposiform hemangioendothelioma (KHE) is an uncommon vascular tumor of intermediate malignant potential, usually occurs in the extremities and retroperitoneum of infants and is characterized by its association with lymphangiomatosis and Kasabach-Merritt phenomenenon (KMP) in certain cases. It has rarely been observed in the head and neck region and at times, can present without KMP. Herein, we present an extremely uncommon case of KHE occurring in tonsil of a child, associated with a neck swelling, but unassociated with KMP. A 2-year-old male child referred to us with history of sore throat, dyspnoea and right-sided neck swelling off and on, since birth, was clinicoradiologically diagnosed with recurrent tonsillitis, including right sided peritonsillar abscess, for which he underwent right-sided tonsillectomy, elsewhere. Histopathological sections from the excised tonsillar mass were reviewed and showed a tumor composed of irregular, infiltrating lobules of spindle cells arranged in kaposiform architecture with slit-like, crescentic vessels. The cells displayed focal lumen formation containing red blood cells (RBCs), along with platelet thrombi and eosinophilic hyaline bodies. In addition, there were discrete foci of several dilated lymphatic vessels containing lymph and lymphocytes. On immunohistochemistry (IHC)...

Anesthesia for Ex Utero Intrapartum Treatment: Renewed Insight on a Rare Procedure

Vieira Marques, M; Carneiro, J; Adriano, M; Lança, F
Fonte: Elsevier Publicador: Elsevier
Tipo: Artigo de Revista Científica
Publicado em //2015 ENG
Relevância na Pesquisa
46.56%
The ex utero intrapartum treatment is a rare surgical procedure performed in cases of expected postpartum fetal airway obstruction. The technique lies on a safe establishment of a patent airway during labor in anticipation of a critical respiratory event, without interrupting maternal-fetal circulation. Anesthetic management is substantially different from that regarding standard cesarean delivery and its main goals include uterine relaxation, fetal anesthesia and preservation of placental blood flow. We present the case of an ex utero intrapartum treatment procedure performed on a fetus with a large cervical lymphangioma and prenatal evidence of airway compromise. Modifications to the classic ex utero intrapartum treatment management strategies were successfully adopted and will be discussed in the following report.