Prenatal management, pregnancy and pediatric outcomes in fetuses with septated cystic hygroma

It has been reported that, compared with simple increased nuchal translucency, fetal cases with septated cystic hygroma (CH) are more likely to face perinatal handicaps. However, pediatric outcomes and proper prenatal counseling for this anomaly have not yet been truly defined. We performed this study to determine pregnancy and pediatric outcomes of fetuses with septated CH. We searched records for cases with septated CH and collected data for structural abnormalities, karyotype analysis, and pregnancy outcomes. Fetuses born with septated CH were also evaluated for their pediatric outcomes. Sixty-nine fetuses with septated CH were enrolled in the study. Results showed that chromosomal abnormalities were present in 28 fetuses (40.6%), and the most common aneuploidy was Turner syndrome (n=14, 20.3%); 16 (23.2%) of the remaining cases, in which aneuploidy was not found, had coexistent structural malformations; 25 (36.2%) cases had normal karyotype and morphology. The total number of live births and infants with unfavorable neurologic follow-up were 13 (18.8%) and 2 (2.9%), respectively. Septated CH is associated with poor perinatal outcomes; therefore, karyotype analysis and ultrasonographic anomaly screening should be performed as initial steps, and expectant management should be offered to couples with euploid fetuses that have normal morphology.

Linfangioma mesentérico multiquístico como causa de vólvulo intestinal / Mesenteric lymphangioma causing volvulus: report of one case

Lymphangiomas are rare benign lesions, most commonly seen in children and adolescents. Their intra-abdominal presentation is uncommon. We report a 19 years old male, presenting to the emergency room with abdominal pain associated with fever, vomiting and abdominal distension. There was a history of multiple previous consultations for abdominal pain. A plain abdominal X-ray examination suggested a bowel obstruction. At surgery a volvulus and dilation of the small bowel in relation to a vascular malformation was found, excising 40 cm of jejunum with an end-to-end intestinal anastomosis. The pathology report showed a Mesenteric Multicystic Lymphangioma. Presently, the patient is in good conditions.

Los linfangiomas son lesiones benignas, de baja incidencia, más comunes en niños y adolescentes. Con muy baja frecuencia son intraabdominales y poseen presentación clínica variable, yendo desde el hallazgo clínico hasta pacientes con riesgo vital por abdomen agudo. Presentamos el caso de un paciente de 19 años, sin antecedentes mórbidos que acude a urgencias por dolor abdominal asociado a fiebre, vómitos y distensión abdominal; la radiografía simple es sugerente de obstrucción intestinal. En laparotomía exploradora se encuentra un vólvulo y dilatación del intestino delgado en relación a una malformación vascular y lesiones multilobuladas en mesenterio. Se resecan 40 cm de yeyuno y realiza anastomosis término-terminal. La histología reveló un Linfangioma Multiquístico Mesentérico. El paciente evoluciona en buenas condiciones generales y se mantiene control clínico e imagenológico seriado. Aunque infrecuente, es uno de los diagnósticos diferenciales de abdomen agudo. A pesar de su naturaleza benigna puede llegar a comprometer el pronóstico vital del paciente.

Higroma quístico / Cystic hygroma

En este caso clinico se realizó el control ultrasonográfico del embarazo de una paciente de 15 años, en el que se observó una masa líquida que era compatible con un higroma quístico. Se realizaron controles posteriores, donde se comprobó el crecimiento de la masa líquida, hasta constatar la muerte fetal en la semana 23. Se describen las principales características de esta malformación, y de su diagnóstico diferencial.

Unusual presentation of a retroperitoneal lymphangioma.

A 3 year old child presented with hemorrhagic ascites of short duration. Based on clinical manifestation and investigations a differential diagnosis of peritoneal tuberculosis, pancreatic ascites or malignant ascites was considered. Laparotomy revealed a huge retroperitoneal cystic lymphangioma confirmed by histopathology. The unusual presentation of retroperitoneal tumours as pseudoascites is discussed.

Management of compromised airway due to unusual presentation of cystic hygroma

The successful management of a unique case of huge cystic hygroma in a fourteen month old child who presented in the emergency room with severe respiratory distress as a result of nearly almost complete upper airway obstruction, is presented

Diagnóstico ecográfico de higroma quístico: a propósito de un caso en el Hospital Universitario Dr. José María Vargas en junio de 2003: San Cristóbal Estado Táchira / Ultrasonographic diagnosis of cystic hygroma: a purpose of a case in the Hospital Universitario Dr. José María Vargas in june 2003: in San Cristóbal Táchira State

El higroma quístico es una malformación congénita de los vasos linfáticos yugulares que ocasiona la acumulación de la misma en una o mas cavidades (1). Se presenta en 1,7 de cada 10.000 embarazadas y su diagnóstico se realiza ecográficamente durante el primer trimestre del embarazo (2). Presentamos el caso de una paciente femenina de 39 años, IV Gesta I para II abortos, con gestación de 14 semanas con hallazgos ecográficos compatible con Higroma quístico, se interrumpió la gestación y se obtuvo un feto masculino de 130 gramos, con gran tumoración cervical, que ocupa toda la región nucal, se toma muestra de sangre intracardíaca para la realización de estudios de cariotipo.

Primary Idiopathic Chylopericardium Associated with Cervicomediastinal Cystic Hygroma

Chylopericardium is a rare clinical entity in which chylous fluid accumulates in the pericardial cavity. We report a case of primary idiopathic chylopericardium associated with multiple, small cervicomediastinal cystic hygromas occurring in an asymptomatic 43-year-old woman with no history of trauma, thoracic surgery, malignancy, infection or tuberculosis. Echocardiography showed a large amount of pericardial effusions and pericardial fluid analysis revealed inappropriately elevated triglyceride. We did not demonstrate communication between the thoracic duct and the pericardial sac by lymphangiography and chest computed tomography. She successfully responded to 30 days of continuous pericardial drainage and 15 days of a medium-chain triglyceride diet after 30 days of total parenteral nutrition. Follow-up echocardiography 6 months after treatment commencement showed a minimal reaccumulation of pericardial fluid without symptom. We conclude that if a patient is asymptomatic and can well tolerate daily life, surgery including pericardiectomy or ligation of the thoracic duct is not necessarily required.

Giant cystic lymphangioma of the middle mediastinum.

A middle-aged male presenting with complaint of progressively increasing dysphagia was found to have a large cystic mass lesion in the middle mediastinum on evaluation. A diagnosis of an endo-duplication cyst was considered after exploration, in view of infiltration of the muscular layer of the lower thoracic esophagus, presence of multiple hyperemic nodular lesions on its inner surface and its location in the middle mediastinum. However, the histopathology revealed the lesion to be a cystic lymphangioma.

Retro-peritoneal cystic lymphangioma in association with fetal hydantoin syndrome.

Antiepileptic drugs are known to be teratogenic. Use of phenytoin during pregnancy can cause various congenital malformations leading to 'fetal hydantoin syndrome'. One such case reported is unique in the sense that it occurred with retroperitoneal cystic lymphangioma, itself a rare condition. Such an association is not described elsewhere.

Linfangioma abdominal en el niño: formas de presentación clínica / Abdominal lymphangioma in child: form of clinical symptoms

El linfangioma abdominal (LA) es una malformación infrecuente en pediatría. La clínica es variable, abarcando desde un abdomen agudo hasta un niño asintomático. Entre 1985 y 1995, 7 pacientes con diagnóstico de LA fueron operados por el autor. La edad fluctuó entre 4 meses y 8 años. Dos casos se presentaron como abdomen agudo, dos como dolor abdominal, dos como masa asintomática, y el último fue un hallazgo quirúrgico. Las lesiones se localizaron homogéneamente entre epiplón mayor, mesenterio ileal, y colon ascendente. El tratamiento consistió en quistectomía total en la mayoría de los casos. Sólo un paciente presentó complicaciones postoperatorias

Higroma quístico: diagnóstico antenatal y manejo clínico / Cystic hygroma: prenatal diagnosis and clinical management

Se presenta una serie de veintisiete casos de higroma quístico, cuyo diagnóstico ultrasonográfico prenatal se realizó entre las semanas trece y treinta de gestación. Veintiuno de los veintisiete casos estudiados, presentaban cromosomopatías agregadas, constituyendo el síndrome de Turner el hallazgo más frecuente. El pronóstico de esta alteración depende de su asociación con cromosomopatías, del tamaño y/o tabicamiento de las formaciones quísticas, de la presencia de hidrops fetal y de la existencia de otras malformaciones agregadas. Nuestra experiencia es absolutamente concordante o coincidente con la literatura mundial

Intrathoracic cystic hygroma in an infant with respiratory failure

A 6-month-old infant presenting initially with clinical signs of bronchiolitis developed acute symptoms of large airway obstruction and respiratory failure despite treatment for bronchiolitis. Endotracheal intubation and oxygen therapy resulted in immediate and dramatic improvement of respiratory failure. Plain chest radiograph, computerized tomography and surgical biopsy with subsequent histological examination established that the respiratory failure was due to a mediastinal cystic hygroma.

Splenic cystic lymphangiomatosis: an unusual cause of massive splenomegaly: report of a case.

A 29-year-old woman had a history of an upper quadrant abdominal mass for about 6 years. An ultrasound examination revealed splenomegaly containing several cysts. On exploratory laparotomy, multiple cysts were found in the spleen. Because of the extensive involvement of the spleen, splenic salvage could not be performed, and total splenectomy was done. Both morphological and histological features of the removed spleen were typical of cystic lymphangiomatosis.

Higroma quístico: diagnóstico antenatal y manejo clínico / Cystic hygroma: prenatal diagnosis and clinical management

Se presenta una serie de 9 casos de higroma quístico, cuyo diagnóstico ultrasonográfico perinatal se realizó entre la semana 14 y 28 de gestación. Siete de los nueve casos estudiados, presentaban cromosomopatías agregada, constituyendo el Síndrome de Turner el hallazgo mas frecuente. El pronóstico de ésta alteración depende de su asociación con cromosomopatías, del tamaño y/o tabicamiento de las formaciones quísticas, de la presencia de hidrops fetal y de la existencia de otras malformaciones agregadas. Nuestra experiencia es absolutamente concordante o coincidente con la literatura mundial

Regresión espontánea de un higroma quístico / Spontaneous resolution of a cystic hygroma

Se presenta el caso de un higroma quístico no tabicado, con regresión espontánea in útero. Son discutidos los hallazgos clínicos, ecográficos, morfológicos y pronósticos de los higromas quísticos tabicados y no tabicados