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      Fetal axillary cystic hygroma: a case report and review

      case-report

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          Abstract

          Cystic hygroma (CH) is a lymphatic malformation occurring different parts of fetal body, typically in the region of the fetal neck and axillary, abdominal wall, mediastinal, inguinal and retroperitoneal areas. CH has been associated with fetal aneuploidy, hydrops fetalis, structural malformations and intrauterine fetal death.

          A 24-year-old gravida 1, para 1 was admitted to our hospital at 28 weeks of gestation. Ultrasonographic examination determined 28 weeks of gestation, singleton, alive fetus who had a mass derived from the right axillary region which was extending to the anterior and posterior thoracic wall with fluid-filled cavities about 12 cm in size. There was no evidence of intrathorasic or intraabdominal extension of mass. Cordocentesis was performed and karyotype examination was normal 46 XY. The fetal demise was found after the first visit. The patient was delivered vaginally after labor induction with oxytocin infusion. The fetal autopsy confirmed the diagnosis of CH.

          The fetal CH carries high risk of aneuploidy and fetal malformations. Patients that have been diagnosed with CH in antenatal follow-ups should be assessed in terms of other anomalies. Fetal karyotyping should be done and the patient should be monitored for fetal hydrops. The birth should be planned in a multidisciplinary hospital and as neonatal resuscitation could be needed, pediatricians should be consulted.

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          Most cited references38

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          First-trimester septated cystic hygroma: prevalence, natural history, and pediatric outcome.

          To estimate prevalence, natural history, and outcome of septated cystic hygroma in the first trimester in the general obstetric population, and to differentiate this finding from simple increased nuchal translucency. Patients at 10.3-13.6 weeks of gestation underwent nuchal translucency sonography as part of a multicenter clinical trial. Septated cystic hygroma cases were offered chorionic villi sampling for karyotype, and targeted fetal anatomical and cardiac evaluations. Survivors were followed up for fetal and long-term pediatric outcome (median 25 months, range 12-50 months). Cases of septated cystic hygroma were also compared with cases of simple increased nuchal translucency. There were 134 cases of cystic hygroma (2 lost to follow-up) among 38,167 screened patients (1 in 285). Chromosomal abnormalities were diagnosed in 67 (51%), including 25 trisomy-21, 19 Turner syndrome, 13 trisomy-18, and 10 others. Major structural fetal malformations (primarily cardiac and skeletal) were diagnosed in 22 of the remaining 65 cases (34%). There were 5 cases (8%) of fetal death and 15 cases of elective pregnancy termination without evidence of abnormality. One of 23 (4%) normal survivors was diagnosed with cerebral palsy and developmental delay. Overall, survival with normal pediatric outcome was confirmed in 17% of cases (22 of 132). Compared with simple increased nuchal translucency, cystic hygroma has 5-fold, 12-fold, and 6-fold increased risk of aneuploidy, cardiac malformation, and perinatal death, respectively. First-trimester cystic hygroma was a frequent finding in a general obstetric screening program. It has the strongest prenatal association with aneuploidy described to date, with significantly worse outcome compared with simple increased nuchal translucency. Most pregnancies with normal evaluation at the completion of the second trimester resulted in a healthy infant with a normal pediatric outcome.
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            OK-432 therapy for lymphangioma in children: why and how does it work?

            Intralesional injection of OK-432 (lyophilized incubation mixture of group A Streptococcus pyogenes of human origin) is safe and effective therapy for lymphangioma. The authors evaluated the mechanism of this therapy in 6 patients who had cystic lymphangioma. The intracystic fluid of the cystic lymphangioma was aspirated before and after (on days 1 and 4) the OK-432 therapy. Changes in cell populations and cytokine productions in each aspirated fluid were analyzed. White blood cells in the intracystic fluid increased markedly in number. Before OK-432 therapy, 96% of the intracystic white blood cells were lymphocytes, and the remaining were neutrophils and macrophages. On day 1, the percentages of neutrophils and macrophages increased to 72% and 21%, respectively. On day 4, the percentage of lymphocytes increased to 72%. Flow cytometry analysis using monoclonal antibodies showed that the number of natural killer cells (CD56+) and T cells (CD3+) had increased. The activity of cytotoxic tumor necrosis factor (TNF) and interleukin-6 increased immediately after OK-432 injection and remained high in titer until day 4. These findings suggest that the white blood cells induced and activated by OK-432, and the cytokines (including TNF) produced by these cells increased the endothelial permeability, and thus the accelerated lymph drainage and increased lymph flow let to shrinkage of the cystic spaces.
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              Cystic lymphangioma in children: report of 32 cases including lesions atrrare sites.

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                Author and article information

                Journal
                Rare Tumors
                RT
                RT
                Rare Tumors
                PAGEPress Publications (Pavia, Italy )
                2036-3605
                2036-3613
                24 October 2011
                21 October 2011
                : 3
                : 4
                : e39
                Affiliations
                [1 ]Department of Obstetrics Gynecology, Sisli Etfal Education and Research Hospital, Istanbul;
                [2 ]Department of Obstetrics Gynecology, Medicana International Ankara Hospital, Ankara;
                [3 ]Department of Obstetrics Gynecology, Zeynep Kamil Education and Research Hospital, Istanbul;
                [4 ]Department of Radiology, Haseki Education and Research Hospital, Istanbul;
                [5 ]Department of Pathology, Zeynep Kamil Education and Research Hospital, Istanbul, Turkey
                Author notes
                Correspondence: Osman Temizkan, Sisli Etfal Education and Research Hospital, Department of Obstetrics Gynecology, 34377 Sisli Istanbul, Turkey. Tel. +90.505.7655176 - Fax: +90.212.2431121. E-mail: mdtemizkan@ 123456yahoo.com

                Conflict of interest: the authors report no conflicts of interest.

                Article
                rt.2011.e39
                10.4081/rt.2011.e39
                3282444
                22355494
                0fb435a9-1900-4968-8f55-cff77004d512
                ©Copyright O. Temizkan et al., 2011

                This work is licensed under a Creative Commons Attribution NonCommercial 3.0 License (CC BY-NC 3.0).

                Licensee PAGEPress, Italy

                History
                : 10 April 2011
                : 27 July 2011
                Categories
                Case Report

                Oncology & Radiotherapy
                cystic hygroma,diagnosis,prognosis.,karyotype,axillary,treatment
                Oncology & Radiotherapy
                cystic hygroma, diagnosis, prognosis., karyotype, axillary, treatment

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