An Unusual Origin of Fetal Lymphangioma Filling Right Axilla

Abstract

A 36-year-old multiparous (gravida 4, para 2, dilatation and curettage 1) pregnant woman who admitted to our perinatology outpatient clinic, because of a fetal right-sided septated axillar mass revealed by ultrasonographic investigation at 22 nd week of gestation. The mass had a multilocular structure and well-circumscribed borders and had a measure that was 5x7x7cm in three dimensions. It was originating from the fetal chest wall, and filling the right fetal axilla. The colour Doppler ultrasonographic examination revealed no blood flow through the mass and the detailed fetal anatomic scan revealed no other anomaly. First and second trimester aneuploidy scans were all in normal ranges. The woman was in a non-consanguineous marriage and had no significant medical or gestational history. No familial history of congenital abnormalities was noted in maternal and paternal genetical inquiry. One of her previous two children died of mitochondrial encephalopathy at 13 th years, the gender was female. The first child was a 20-yearold healthy male. After a detailed counseling, the family chose the expectant management until the delivery. Amniocentesis was performed and revealed a normal constitutional karyotyping. Thereafter, serial ultrasound studies demonstrated that the lesion increased in size slowly, it reached the size of 7x8x10cm at 28 week's gestation, 9x10x11cm at 32 weeks' gestation [Table/ Fig-1]. In the follow-up ultrasonographic examinations, it was seen that there were areas of intracystic haemorrhage in some of the locules and the right arm of the fetus was in the position of abduction, continuously [video-1,2].The fetus was delivered via cesarean section at 38 weeks' gestation, when the active labor started. Cesarean section was chosen to avoid possible fetal trauma during vaginal birth. A female baby weighing 3470 grams was delivered with the Apgar scores of 7 and 9 at 1 and 5 min, respectively, in the presence of an expert neonatologist. Neonatal examination revealed that the cystic mass (measured 10x12x14cm in three dimensions) covered the right anterior and lateral chest wall, and extended to the right upper arm through the axillary region. Also, it was related to a smaller cystic lymphangioma at the apex of the posterior axillary region, measured 3cm in three dimensions and also there were small various sites of skin discolouration and millimetric lymphangiomas Fetal lymphangioma is a hamartomatous congenital anomaly of the lymphatic system, which is embracing the fetal skin (sometimes mucous membranes) and the subcutaneous tissue. The general consensus is that it occurs as a result of failure in lymphatic drainage. A 36-year-old pregnant woman was referred to our perinatology clinic at 22 weeks' gestation, because of a fetal right-sided axillary mass revealed by ultrasonography. The mass measuring 5x7x7cm in three dimensions had a multilocular structure without colour Doppler flow and well-circumscribed borders. Amniocentesis revealed a normal constitutional karyotyping. Lymphangioma was considered a...

Treatment of Lymphatic Malformations with the mTOR Inhibitor Sirolimus: A Systematic Review

“…17 In six publications, the initial dose of sirolimus was not reported. 15,18,19,22,25,28 The target blood level of sirolimus was similar (£15 ng/mL), but slightly differed between the studies. The target blood level was 10-15 ng/mL in seven studies, 8,12,18,22,23,26,27 5-15 ng/mL in four studies, 21,24,29,30 10-13 ng/mL in one study, 16 and 4-8 ng/mL in one study.…”

“…Through these methods, 20 articles were identified, containing a total of 71 cases in which sirolimus was used to treat lymphatic malformations, venolymphatic malformations, and capillary-lymphatico-venous malformations. 8,[12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30] After identifying these articles, all text and figures were carefully assessed for relevant data. The following information on study characteristics and clinical treatments was extracted from all included studies: publication metrics (name of first author and year of publication), subject information (age, gender, and localization of malformation), and treatment information (sirolimus dose, planned target trough level, treatment duration, treatment outcomes, additional therapies, and adverse events).…”

“…8,[12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30] Most studies were retrospective case series or case reports, and there was only one phase II trial. In all, 16 publications about 45 patients with lymphatic malformations were identified (Table 1), 8,[12][13][14][15][16][17][18][19][20][21][22][23][24][25][26] five publications about eight patients with venolymphatic malformations (Table 2), 12,21,[27][28][29] and four publications about 18 patients with capillary-lymphatico-venous malformations (Table 3). 8,12,29,30 All patients had extensive malformations, which were distributed about the w...…”

Natural Course of Fetal Axillary Lymphangioma Based on Prenatal Ultrasound Studies

“…We searched PubMed, Scopus, and the Web of Science for all consecutive cases of fetal axillary cystic hygroma or lymphangioma as well as hemangiolymphangioma. Up to May 2017, 25 cases of axillary lymphangioma were published, as presented in Table . The review included only cystic hygromas and lymphangiomas mainly located at the axilla, but they might extend to the chest wall, the neck, or even the thorax.…”

“…Up to May 2017, 25 cases of axillary lymphangioma were published, as presented in Table 1. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] The review included only cystic hygromas and lymphangiomas mainly located at the axilla, but they might extend to the chest wall, the neck, or even the thorax. Together with 5 our own cases, a total of 30 cases were analyzed and summarized as follows: be taken into account for decisions on the route of delivery.…”