A pitfall in diagnosing fetal abdominal lymphangioma: A report of two cases.

Typical ultrasound findings of fetal abdominal lymphangioma include thick-walled, multiseptated anechoic masses. Although a majority of cases can be suspected promptly by ultrasound examination, the two cases presented herein did not meet the standard criteria and were misleading. Both cases involved unilocular cysts without clear septations, but in retrospect were atypical findings of fetal abdominal lymphangioma. A few reports of misleading cases have been described previously; however, the precise characteristics have not been reported in detail. Therefore, in this case report, we focused predominantly upon the difficulties encountered in the prenatal diagnosis of abdominal lymphangioma based on ultrasound morphology alone.

Natural Course of Fetal Axillary Lymphangioma Based on Prenatal Ultrasound Studies.

This series and literature review aimed to prenatally characterize the nature of axillary lymphangioma. A total of 30 cases, including our 5 cases, were analyzed. Insights gained from this review are as follows: Septate and nonseptate cysts seem to be different entities. The nonseptate type tends to be small and transient but more highly associated with aneuploidies. Septate cysts are very rarely associated with other abnormalities and hydrops fetalis, unlike cystic hygroma colli, but are more progressive with gestational age and associated with adverse outcomes. The cases with high flow have a higher risk of intralesional hemorrhage. Prenatal diagnosis is important for the route of timely delivery and possibly prenatal interventions. Shoulder dystocia is common and should always be taken into account for decisions on the route of delivery.

[Iconographic imaging (ultrasound/MRI) in prenatal evaluation of cervical cystic lymphatic malformations]. / Bilan iconographique (échographie/IRM) dans l'évaluation anténatale des malformations lymphatiques kystiques cervicales.

OBJECTIVES: Assess imaging (ultrasound±MRI) in the diagnosis of cervical cystic lymphangioma. METHODS: Retrospective, descriptive study of the patients who underwent reference ultrasound screening for fetal anterior cervival masses in multidisciplinary prenatal diagnosis center of the Lille Regional University Hospital from 1997 to 2014. RESULTS: Seventeen lymphangiomas were identified. Seventy-three percent of lymphangiomas (n=12) were diagnosed in the baseline ultrasound and 85% (n=11) in MRI. An extra-cervical extension was identified in 10 (62%) with ultrasound and 11 (85%) with MRI. Main sites of mass extension objectived by ultrasound and MRI were respectively the face (4,23%/6,46%), the base of tongue (3, 18%/2, 15%) and buccal floor (3, 18%/3, 15%). Pharyngeal disease (5,38%) and mandible (4,31%) were detected by MRI and not found on ultrasound. Associated malformations detected with ultrasound were 2 (12%) urogenital malformation, 1 (6%) heart defect and 1 (6%) facial anomalies. Lymphangiomas remained stable in volume in 12 (71%) cases and disappeared before birth in 2 (12%) cases. CONCLUSION: MRI and ultrasound seem inseparable to assess and optimally manage fetal cervical masses. Detection of associated lesions seems to help in the final lymphangioma diagnosis. Most of the time, lymphangiomas seem to be stable lesion during pregnancy and a good prognosis in cases of uncomplicated cervical lymphatic mass and without pejorative extension.

Ultrasonic findings of fetal axillary lymphangioma with intralesional hemorrhage.

We report a case of an axillary lymphangioma in a fetus delivered at 30 weeks' gestation with suspected intralesional hemorrhage based on the ultrasonic findings. In the ultrasonic examination at 15 weeks' gestation, the fetus was found to have a multilocular mass spreading from the axilla to the chest wall, which was diagnosed as an axillary lymphangioma. Chromosome analysis by amniocentesis showed a normal karyotype, and no other malformations were observed. At 29 weeks, the mass had increased in size, and color Doppler ultrasound examination revealed that the middle cerebral artery peak systolic velocity (MCA-PSV) reached 80.2 cm/s [1.86 MoM (multiples of the median)]. Intralesional bleeding was suspected because of the multiple images of hemorrhage in which sites of blood spouting in a pulsatile fashion were detected within the mass. Cordocentesis at 30 weeks revealed that fetal hemoglobin concentration was 5.1 g/dL. An emergency Cesarean section was performed. A female weighing 2810 g, including the mass, was delivered, and the blood hemoglobin level was 5.9 g/dL at birth. Blood transfusion, fine-needle aspiration of the fluid in the mass, intralesional injection of OK-432, and partial excision of the lymphangioma were performed after birth. Ultrasonic examination proved useful in the diagnosis of intralesional bleeding in this lymphangioma.

Giant fetal lymphangioma at chest wall and prognosis: case report and literature review.

OBJECTIVE: To report a rare liveborn case with a giant, septated, chest wall lymphangioma that underwent prenatal expectation treatment. CASE REPORT: A case of giant fetal chest wall cystic lymphangioma was diagnosed prenatally at 19 weeks gestation. Expectation treatment was performed, carefully after prenatal counseling, ruling out other structural abnormalities. At 38 weeks gestation, ultrasound showed a multilocular, subcutaneous cystic mass of 12.3 cm × 9.2 cm × 11.0 cm located on the left chest wall and left upper arm. The tumor was surgically removed 4 days after birth, and no recurrence was observed in the following 18 months. CONCLUSION: Our experience suggests that a large, septated fetal lymphangioma may still merit prenatal expectation treatment if there is no evidence for chromosomal and structural abnormality.

Foetal axillary lymphangioma with ipsilateral pes equinovarus: pitfalls in sonographic differential diagnosis (axillary lymphangioma & pes equinovarus).

Lymphangiomas are rare congenital malformations of the lymphatic system. Despite the benign histology, they are likely to grow rapidly and invade the surrounding tissues. In contrast to the cystic hygromas, lymphangiomas at the axillary region tend to have normal karyotype. However, associated hydrops makes the prognosis poor. Due to isolated few cases in the literature, the true incidence of foetal axillary lymphangiomas is not known. We present here a pre-natal ultrasonographic diagnosis of a 15-week foetus with rapidly growing axillary lymphangioma with ipsilateral foot abnormality which had normal karyotype.

[Therapy for prenatally diagnosed lymphangioma--multimodal procedure and interdisciplinary challenge]. / Die Therapie von pränatal diagnostizierten Lymphangiomen--Multimodales Vorgehen und eine interdisziplinäre Herausforderung.

INTRODUCTION: Ultrasound diagnostics enable the prenatal diagnosis of extensive lymphatic malformations. The diagnosis is made prenatally or directly after birth in 50-60% of the cases, in about 80% of the afflicted in the first year of life and up to 90% of the cases in the second year of life. PRENATAL DIAGNOSTICS AND POSSIBILITIES OF PRE- AND PERINATAL INTERVENTIONS: The so called hygroma colli is a primary symptom, which is detected during the routine ultrasound examination. Lymphatic malformations, which are diagnosed antenatally are often associated with chromosome abnormalities. The EXIT procedure allows invasive therapeutic or diagnostic interventions. Hereby the airway safety of Newborns with extended cervical or zerviko-facial lymphangiomas can be warranted. The survival chance and the prognosis may be significantly improved. MANIFESTATION AND ENT-CLINICS: 72 patients (mean age 12.3 yrs) with lymphatic malformations of the Head and Neck presented for the first time during a 5-years period until 31.12.2008 at the Department of Otolaryngology of the University of Marburg. Therapeutic interventions consisted in conventional surgery, laser assisted therapy, sclerosing therapy or a combined therapeutic approach. CONCLUSION: Extensive lymphatic malformations of the Head and Neck challenge treating physicians and affected families. Advancements of prenatal diagnostics and opportunities of peri- and postnatal interventions contribute to an improved survival chance and prognosis. Intensive interdisciplinary cooperation in specialised centers is of outstanding importance for an optimal care of these patients.

Congenital neck masses.

Congenital neck lesions reflect abnormal embryogenesis in head and neck development. A thorough knowledge of embryology and anatomy is critical in the diagnosis and treatment of these lesions. The appropriate diagnosis of these lesions is necessary to provide appropriate treatment and long-term follow up, because some of these lesions may undergo malignant transformation or be harbingers of malignant disease.

Tumores fetales: I parte / Fetal tumors: Part I

Se presenta una revisión sistemática y resumida de los diferentes tumores fetales, haciendo énfasis en su diagnóstico prenatal y posibles tratamientos intrauterinos. En esta primera parte se introduce el tema y se analizan tumores del sistema nervioso central, cara y cuello.

[Lymphangioma--prenatal and perinatal aspects--Part one]. / Naczyniak limfatyczny--aspekty prenatalne i perinatalne--czesc pierwsza.

Lymphangioma or cystic hygroma is a congenital malformation of the lymphatic system which has been commonly associated with fetal aneuploidy, hydrops, structural malformations and intrauterine death. In this paper we would like to report two cases of lymphangioma diagnosed prenatally in the third trimester in the fetuses with normal karyotype, normal NT in the first trimester and without other structural anomalies and with good perinatal outcome.

Prenatal diagnosis of a giant foetal lymphangioma and haemangiolymphoma in the second trimester using 2D and 3D ultrasound.

Lymphangiomas are benign tumours of the lymphatic system. Early prenatal diagnosis is important to permit a planned delivery and provide adequate postnatal care. It thereby improves prognosis and allows the option of terminating the pregnancy if poor outcome is predicted. We report two cases, a giant haemangiolymphoma and a lymphangioma. 2D and 3D US findings are presented and differential diagnosis, therapeutic options and prognosis are discussed.

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.

Management of fetal airway obstruction.

Fetal airway obstruction can make it difficult if not impossible to secure the airway at birth, before hypoxia, brain injury, or death results. Fetal airway obstruction can result from an intrinsic defect in the airway, such as the congenital high airway obstruction syndrome or extrinsic compression of the airway caused by a cervical mass, most commonly a cervical teratoma or lymphangioma. As fetuses with fetal airway obstruction reach viability, they should be monitored closely for the development or progression of hydrops in intrinsic obstruction cases or polyhydramnios in extrinsic obstruction cases. The fetus should be delivered by using the ex utero intrapartum treatment procedure, with maintenance of uteroplacental circulation and gas exchange. This approach provides time to perform procedures such as direct laryngoscopy, bronchoscopy, or tracheostomy to secure the fetal airway, thereby converting an emergent airway crisis into a controlled situation.

Congenital malformations of the cervicothoracic lymphatic system: embryology and pathogenesis.

Familiarity with the embryology of the lymphatic system is helpful in understanding the pathogenesis and radiologic appearance of lymphangiomas of the cervicothoracic region. By considering anatomic location and radiologic appearance, one can predict the type of lymphangioma present, the primordial lymph sac from which the malformation arose, and when it formed in embryonic life. Cystic hygromas are composed of large, dilated lymphatic spaces. They form when a primordial lymph sac fails to reestablish communication with the central venous system from which it arose. These lesions may also result from an aberrant bud arising from a primordial lymph sac. Cavernous and capillary lymphangiomas are composed of smaller lymphatic channels. They form from abnormally sequestered buds of the developing lymphatic mesenchyme responsible for the fine meshwork of terminal branches in the periphery of the embryo. Their growth may be inhibited by the relatively tougher tissues in the periphery (eg, skin and muscle) compared with the relatively loose fatty connective tissue in which cystic hygromas form. Not only can all types of lymphangioma occur in one lesion, but lymphatic and vascular malformations may also coexist.

Fetal nuchal cystic hygromata: associated malformations and chromosomal defects.

During a 6-year period (1985-1990), blood karyotyping was performed in 44 fetuses with septated, bilateral, dorsal, cervical cystic hygromata. This condition constitutes a different entity from nuchal oedema. There were 33 (75%) chromosomal abnormalities, including Turner's syndrome (n = 31), trisomy 18 (n = 1) and trisomy 21 (n = 1). Congenital heart defects (CHD), mainly coarctation of the aorta, were present in 15 of the fetuses with Turner's and in 1 of the chromosomally normal fetuses. The incidence of CHD was higher in the fetuses with ultrasonographic evidence of moderate/severe hydrops (41%; 13 of 32 cases) than in those with mild or no hydrops (25%; 3 of 12 cases). Although both the biparietal diameter (BPD) and femur length (FL) were reduced in all fetuses, the FL to BPD ratio was below the 5th percentile in 29 of the 33 (88%) chromosomally abnormal fetuses, but in only 4 of the 11 (36%) chromosomally normal ones. In the chromosomally normal group, 3 of the fetuses had multiple pterygium syndromes, and in such cases the risk of recurrence may be high. In contrast, in the group of mutant chromosomal disorders with monosomy or trisomy, the risk of recurrence is in the order of 1%.

Cervical, cervicomediastinal and intrathoracic lymphangioma.

Lymphangiomas result from abnormal development of the lymphatic system, with obstruction to lymph drainage from the affected area. The neck is the most common site (25%). In this study, we review the literature of lymphangioma in the neck and thorax and have undertaken detailed analysis of 52 children with cervical lymphangioma treated during the 20 years 1969-1988. Cervicomediastinal lymphangioma is uncommon (4%) and lesions confined to the thorax are rare, with none in our series. Neck lymphangiomas occur in early childhood with half being diagnosed at birth and almost 90% before school age. All have a mass. Two-thirds are asymptomatic; sudden enlargement, inflammation, infection, feeding difficulties and respiratory symptoms occur in the remainder. Pharyngeal and laryngeal involvement, usually associated with large infiltrating lesions, results in acute airways obstruction. The respiratory symptoms caused by mediastinal extensions are usually less dramatic. Lymphangiomas have a characteristic appearance on ultrasound examination and CT scan. These investigations are mandatory for an undiagnosed intrathoracic mass and when there is clinical suspicion of mediastinal extension of cervical lymphangioma but should be obtained for neck swellings only when the clinical diagnosis is in doubt. The recommended treatment is surgical excision which can be achieved with no mortality and little morbidity. An initial period of observation is justified for asymptomatic cervical lesions because there is a small incidence (6%) of spontaneous regression. Cervicomediastinal lymphangiomas can be removed at one operation using a neck incision combined with median sternotomy. The surgeon must preserve vital structures (especially vagus, recurrent laryngeal and phrenic nerves) and should not necessarily attempt total removal of all lymphangiomatous tissue. Massive infiltrating cervical lesions pose a particular challenge and may require multiple operations over many years before a satisfactory result with good-quality survival is attained.

Abdominal cystic lymphangioma. A case report and a review of the literature.

Authors report a case of cystic lymphangioma of the mesenterium. The embryology and the histology are important to explain the radiological findings. To make the differential diagnosis with other cystic abdominal tumors it is very important to compare the radiological findings with other clinical symptoms.

Pharyngeal cleft sinuses and cysts, and other benign neck lesions.

Few pediatricians can expect to acquire great personal experience in dealing with many of the aforementioned lesions. Nevertheless, an appreciation of the usual presentation and natural history of most of the entities will usually permit an accurate diagnosis and guide to management in all but the most obscure conditions.