Reporte de Caso Clínico: Linfangioma quístico de epiplón / Case Report: Cystic lymphangioma of the omentum

INTRODUCCIÓN: El linfangioma mesentérico es un tumor quístico congénito, benigno y raro de los vasos linfáticos, que se presenta en menos del 5% de los casos a nivel abdominal. Se presentan con mayor frecuencia durante la infancia. Esta patología se debe considerar como diagnóstico diferencial de abdomen agudo y masas abdominales pediátricas. CASO CLÍNICO: Niño de 6 años de edad, presentó cuadro de dolor abdominal y peritonismo. Presentó febrícula, signo de rebote positivo, además leucocitosis y neutrofilia. Ecografía evidenció abundante líquido libre en abdomen y pelvis. EVOLUCIÓN: Se realizó laparotomía exploratoria, con hallazgo de masa quística dependiente de epiplón mayor, la misma que se resecó. La histopatología fue negativa para malignidad y compatible con linfangioma quístico, la inmunohistoquímica con marcador D2-40 positi-vo. El paciente evolucionó favorablemente sin complicaciones quirúrgicas y sin evidencia de recurrencia hasta el año de seguimiento. CONCLUSION: El linfangioma quístico mesentérico puede debutar con sintomatología de abdomen agudo, puede también tener una evolución crónica. La resección completa es el tratamiento de elección, actualmente se realizan procedimientos mínimamente invasivos, con resultados favorables. La ecografía es útil para realizar un seguimiento a largo plazo.(AU)

BACKGROUND: Mesenteric lymphangioma is a rare, benign congenital cystic tumor of the lym-phatic vessels, which occurs in less than 5% pf the cases at the abdomen. They appear most often during childhood. This tumor should be considered as a differential diagnosis of acute abdomen and other abdominal masses. CASE REPORT: A 6-year-old boy presented with abdominal pain and peritonism. He presented a low-grade fever, a positive rebound sign, as well as leukocytosis and neutrophilia. Ultrasono-graphy showed abundant free fluid in abdomen and pelvis. EVOLUTION: Exploratory laparotomy was performed, finding a cystic mass dependent on the greater omentum, this mass was resected. Histopathology was negative for malignancy and compatible with cystic lymphangioma, immunohistochemistry was positive for D2-40 marker. The patient evolved favorably without surgical complications and without evidence of recurrence up to one year of follow-up. CONCLUSION: Mesenteric cystic lymphangioma can present with symptoms of acute abdomen, it can also have a chronic evolution. Complete resection is the treatment of choice, currently minimally invasive procedures are performed with favorable results. Ultrasound is useful for long-term follow-up.(AU)

Case report of a chest wall cystic hygroma in a teenager.

Cystic hygroma or cystic lymphangioma is a congenital malformation of lymphatic origin. Their occurrence on the chest wall is very rare, and they progressively grow with age infiltrating into the local tissues, around muscle fibers and nerves, making them difficult and hazardous to remove. There are various treatment modalities of such lesion. Based on the literature surgical excision is the preferred treatment of choice in cystic hygroma because it gives a better cure rate compared to other modalities. We report a case successful excision of anterolateral chest wall cystic hygroma in a teenager in Hospital Serdang.

[Gastrocolic omental cyst in an adult: Case presentation and review of literature]. / Quiste omental gastrocólico en un adulto: presentación de un caso y revisión de la bibliografía.

BACKGROUND: Mesenteric lymphangioma are rare tumours. They usually present early on in life, if congenital, or soon after trauma. The usual sites of presentation of lymphangiomas are in the neck, and axillae. In the abdomen they are more common in the mesentery, primarily of the ileum, or retroperitoneal. OBJECTIVE: A rare case is presented of a mesenteric lymphangioma. CLINICAL CASE: It involves an elderly African-American male, many years after trauma, and characterised with early satiety, causing weight loss, but without gastric outlet obstruction or vomiting. Its diagnosis, management and review of literature are presented. CONCLUSIONS: Mesenteric cysts are rare tumours that should be included as differential diagnosis in elderly patients with a history of previous abdominal trauma.

Linfangioma quístico retroperitoneal: A propósito de 2 casos clínicos / Cystic Lymphangioma: About 2 cases

Los linfangiomas quísticos son malformaciones congénitas hamartomatosas del sistema linfático comunes en niños, muy raros en adultos. Los linfangiomas quísticos afectan, sobre todo a la piel y al tejido subcutáneo. Menos del 1% de los linfangiomas quísticos son de localización retroperitoneal. Se caracterizan por aparecer en la infancia a modo de grandes masas a partes blandas con tendencia a crecer, a no ser que se extirpen de forma completa en sus etapas inciales. El también llamado higroma quístico, constituye una variante de linfangioma. Describimos dos casos de linfangioma quístico, de localización poco frecuente, en pacientes jóvenes

Cystic lymphangiomas are lymphatic congenital malformations rare between adults, but very common in children. These tumors speccially affect skin and subcutaneous tissue. Less than 1 percent have retroperitoneal location. Linphamgiomas are characterised by appearing in childhood as big soft tissue masses with a tendency to grow. The so-called cystic higroma is a variety of lymphamgioma. We describe 2 cases of unusual location, in young patients

Higroma quístico congénito. Presentación de un caso / Congenital cystid hygroma. A case report

Fundamento: Los linfagiomas son malformaciones congénitas del sistema linfático, se clasifican en quísticos, cavernosos y mixtos o macro y microquísticos. Se caracterizan por espacios dilatados limitados por endotelio, que varían en tamaño desde canales microscópicos hasta grandes quistes. El linfangioma quístico es una anomalía congénita del sistema linfático muy infrecuente. Presentación de caso: Se presenta un caso de higroma quístico en un niño de cinco días de nacido que fue remitido al servicio de pediatría del Hospital General de Benguela, Angola, por presentar una tumoración que interesa desde la región lateral izquierda del cuello y se extiende a la axila del mismo lado. Se analizaron algunos datos embriológicos del sistema linfático y también la localización de los quistes y su frecuencia. Conclusiones: En la medida que casos como este se divulguen entre los profesionales de la salud se puede lograr el rápido diagnóstico de esta enfermedad para el tratamiento y profilaxis de las complicaciones.

Background: The lymphangioma are congenital malformations of the lymphatic system which are classified into: Cystid, cavernous, mixed, macro, microcystid, characterized by dilated spaces that are limitated by the endothelium, which vary in size from the microscopic channels to big cysts. Case Presentation: A case of Cystid hygroma is presented in a boy of 5 days of birth and who was sent to the pediatrician in the General Hospital of Benguela, Angola, having a tumor that interests since the lateral left region of the neck to the armpit of the same part. Were analyzed some embryological data of the lymphatic system and also the place of the cyst and its frequency. Conclusions: As far as cases like this are reported among health professionals, a rapid diagnose can be obtained of this disease for a treatment and prophylaxis of the complications.

Higroma quístico congénito / Congenital cystid hygroma

Los linfagiomas son malformaciones congénitas del sistema linfático, se clasifican en quísticos, cavernosos y mixtos o macro y microquísticos. Se caracterizan por espacios dilatados limitados por endotelio, que varían en tamaño desde canales microscópicos hasta grandes quistes. El linfangioma quístico es una anomalía congénita del sistema linfático muy infrecuente. Presentación de caso: Se presenta un caso de higroma quístico en un niño de cinco días de nacido que fue remitido al servicio de pediatría del Hospital General de Benguela, Angola, por presentar una tumoración que interesa desde la región lateral izquierda del cuello y se extiende a la axila del mismo lado. Se analizaron algunos datos embriológicos del sistema linfático y también la localización de los quistes y su frecuencia. Conclusiones: En la medida que casos como este se divulguen entre los profesionales de la salud se puede lograr el rápido diagnóstico de esta enfermedad para el tratamiento y profilaxis de las complicaciones(AU)

The lymphangioma are congenital malformations of the lymphatic system which are classified into: Cystid, cavernous, mixed, macro, microcystid, characterized by dilated spaces that are limitated by the endothelium, which vary in size from the microscopic channels to big cysts.Case Presentation: A case of Cystid hygroma is presented in a boy of 5 days of birth and who was sent to the pediatrician in the General Hospital of Benguela, Angola, having a tumor that interests since the lateral left region of the neck to the armpit of the same part. Were analyzed some embryological data of the lymphatic system and also the place of the cyst and its frequency. Conclusions: As far as cases like this are reported among health professionals, a rapid diagnose can be obtained of this disease for a treatment and prophylaxis of the complications(AU)

Congenital mature cystic teratoma of the lateral neck presenting as cystic hygroma: a rare case report with literature review.

Teratoma of the neck is a rare extra-gonadal teratoma. Although it can be seen in adult, most of the cases present in fetal life or early childhood. Grading and classification of teratoma is important for management and prognosis. Decision making between mature and immature teratoma is sometime challenging if it is not composed of extensive unusual neuroectodermal tissue component. We reported a rare case of huge congenital mature cystic teratoma composted of various neuroectodermal tissues in an 8-month-old boy; it was located at the lateral neck and mimicked congenital cystic hygroma. A literature review was done to grade and classify the tumour.

Congenital cystic neck masses: embryology and imaging appearances, with clinicopathological correlation.

Congenital cystic masses of the neck are uncommon and can present in any age group. Diagnosis of these lesions can be sometimes challenging. Many of these have characteristic locations and imaging findings. The most common of all congenital cystic neck masses is the thyroglossal duct cyst. The other congenital cystic neck masses are branchial cleft cyst, cystic hygroma (lymphangioma), cervical thymic and bronchogenic cysts, and the floor of the mouth lesions including dermoid and epidermoid cysts. In this review, we illustrate the common congenital cystic neck masses including embryology, clinical findings, imaging features, and histopathological findings.

Aggressive giant cystic lymphatic malformation in a newborn.

Lymphatic malformations are uncommon, benign and congenital malformations of the lymphatic system exhibiting lack of development of communication between the lymphatic and venous circulation. We report the unusual case of rapidly expanding giant lymphatic malformation of the torso, bilateral axillae and left upper extremity of a newborn. As the first-line treatment, aspiration and sclerotherapy with bleomycin were performed. The sclerotherapy failed to cause regression of the mass, and rapid expansion of the malformation necessitated surgery. Partial resection of the mass was performed. Clinical symptoms of respiratory distress resolved in the early postoperative period, and the patient became hemodynamically stable. However, intrathoracic invasion of the mass developed, leading to restriction of thoracic expansion, ending in death. In conclusion, surgical treatment of giant lymphatic malformations remains challenging.

The use of the trapdoor incision for access to thoracic inlet pathology in children.

Lesions at the thoracic inlet are difficult to access via a thoracic or cervical approach. The use of the anterior cervico-thoracic trapdoor incision has been reported to give good exposure to the anterior superior mediastinum in adults. We report our experience of four cases where a trapdoor incision was used to gain excellent access and exposure to thoracic inlet pathology in children.

Congenital abdominal cystic lymphangiomas: what is the correct management?

INTRODUCTION: Intraabdominal cystic lymphangioma is a rare benign lesion with a variable clinical presentation. The aim of this study was to evaluate the natural outcome of this lesion and to propose a decision-making protocol for antenatal suspected intraabdominal lymphangioma. We also would like to emphasize the role of laparoscopy in treatment. MATERIALS AND METHODS: This retrospective study examined 14 cases of intraabdominal cystic lymphangioma in children underwent to our observation, at the Pediatric Surgery Department in Lapeyronie Hospital, Montpellier between 1996 and 2007. RESULTS: Of the 14 patients, 12 were operated on (7 by laparoscopy and 5 in open surgery). In 2 patients we didn't perform intervention because the lesion was localized in the mesenteric root: we observed a regression of the lesion on US-scan. CONCLUSION: The intraabdominal cystic lymphangioma is a rare lesion which is now sometimes suspected on antenatal US scan; consequently we observed at the present time an increasing number of lesion potentially asymptomatic: in these case the management has to be established and a risk-benefit balance has to be made before a surgical intervention. Our experience is limited to a small number of cases, and it is too premature to describe any final conclusion; however our suggestion is that clinical course of cystic abdominal lymphangioma is unknown, and we propose that if no complication occurs, a clinical and ultrasonographic monitoring should be done in case of mesenteric root localisation.

Congenital cystic lesions of the head and neck.

This article presents clinical characteristics and radiologic features of congenital cervical cystic masses, among them thyroglossal duct cysts, cystic hygromas, branchial cleft cysts, and the some of the rare congenital cysts, such as thymic and cervical bronchogenic cysts. The imaging options and the value of each for particular masses, as well as present clinical and radiologic images for each, are discussed.

Assisted laparoscopic excision of huge abdominal cysts in newborns and infants using the umbilical laparoscopic port incision.

PURPOSE: The objective of this article was to present our experience in successfully managing a variety of abdominal cysts in newborns and infants using a mini-invasive technique performed in 8 cases using an umbilical laparoscopic port incision. METHODS: Assisted laparoscopic excision using an umbilical port incision was performed in 8 cases. Six were neonates (5 ovarian cysts and one hepatic cyst) and 2 were infants (6-months old with intestinal duplication and 11-months old with lymphatic malformation). RESULTS: The procedure was performed with ease and was successful in all 8 cases. Oral feeding was started early after the surgical intervention. Seven patients were discharged the next day. The last case was discharged on the fifth day after surgery because of intestinal resection. CONCLUSION: This proposed mini-invasive technique using the umbilical port incision proved to be less invasive, easier, and less technically demanding than the classic laparoscopic approach and, hence, may be applied to different abdominal cystic pathologies in newborns and infants.

Neonatal ovarian lymphangioma.

Ovarian cysts are primarily of follicular origin and ovarian lymphangioma is an extremely rare lesion. We report a case of prenatal diagnosis of unilateral ovarian cyst in which pathologic examination revealed an ovarian lymphangioma. Ovarian cystic lymphangioma should be included in the differential diagnosis of an ovarian cyst mass in neonate.

Anesthetic management of a patient undergoing an ex utero intrapartum treatment (EXIT) procedure: a case report.

The ex utero intrapartum treatment (EXIT) procedure involves partial delivery of the fetus with the fetal-placental circulation maintained. This allows for management of the obstructed fetal airway via direct laryngoscopy, bronchoscopy, tracheostomy, or surgical intervention. These complex and often challenging procedures have been performed about 100 times in the United States to date. Recent advances in prenatal diagnosis of fetal congenital malformations, in particular, abnormalities involving the fetal airway, have allowed for the development of the EXIT strategy to convert potentially catastrophic situations during fetal delivery to a controlled environment. Indications for the EXIT procedure have expanded to a variety of congenital abnormalities, including fetal neck masses, lung or mediastinal tumors, congenital high airway obstruction syndrome, conjoined twin separation, and acute respiratory distress syndrome requiring transitioning from EXIT to extracorporeal membrane oxygen transitioning. Various considerations must be managed by the anesthesia provider during the EXIT procedure to ensure positive maternal and fetal outcomes. Careful attention to achieving adequate uterine relaxation, maintaining maternal blood pressure, avoiding placental abruption, prioritizing fetal airway establishment, and providing return of uterine tone when indicated are examples of these considerations. In this case report, a parturient presented for an EXIT procedure secondary to fetal cystic hygroma.

A holistic approach to a rare pathology.

A case of cystic hygroma (CH), a congenital lymph-filled multicystic hamartoma of the neck, with a holistic approach to patient management is presented. The stigma of the CH imposed by attending physicians, dentists, parents, teachers and peers affected the psychological development of this patient since her diagnosis in childhood. Although surgery relieves the threat of airway obstruction and may improve the esthetic appearance of the CH, the patient may be isolated and traumatized by the ignorance and superstitions of the individuals she encounters on a daily basis.

Pattern of congenital neck masses in a Kenyan paediatric population.

BACKGROUND: The distribution of congenital neck masses varies between countries and is important in diagnosis and treatment modalities. Data from Africa is scarce, and altogether absent from Kenya. OBJECTIVE: To describe the pattern of congenital masses in a Kenyan paediatric population. SET UP: Kenyatta National Hospital, Nairobi Kenya. STUDY DESIGN: Prospective descriptive cross-sectional. METHODOLOGY: Children aged 15 years and below with neck masses who presented to various surgical clinics and wards at the Kenyatta National Hospital between December 2006 and April 2007 were included in the study. They were examined for age, gender, type and location of the neck mass. Mode of investigation and final diagnosis were recorded. Data was analyzed by using statistical package for social sciences. Descriptive statistics were applied to determine means, frequencies and modes. Ninety-five percent confidence interval was used and p value of 0.05 taken as significant. Data are presented in form of tables. RESULTS: Two hundred and thirty-five children (129 male) with neck masses were examined. Fifty-one (22%) of these masses were congenital. Thyroglossal duct cysts were the most common (29%) followed by cystic hygromas (21%) and branchial cleft cysts (20%). Fifty-one percent of the masses were present at birth. The midline was the most common location (31.4%) followed by anterior border of sternocleidomastoid (27.5%) and submandibular region (19.6%). Ultrasound was the commonest diagnostic investigation. CONCLUSION: Congenital defects constitute an important differential diagnosis for paediatric neck masses in Kenya. Thyroglossal duct cysts, cystic hygromas and branchial cleft cysts are the most prevalent occurring most commonly in the midline and anterior border of sternocleidomastoid muscle. An understanding of the distribution of these masses improves diagnosis, preoperative decision making and their overall management.