Cases reported "Lymphangioma"

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1/20. Posterior midline cervical fetal cystic hygroma.

    Posterior midline cervical cystic hygromas (PMC) are frequently found associated with chromosomal aberrations and usually do not survive. The present report illustrates diagnosis of this condition by sonography in an 18 weeks old fetus and an amniocentesis revealed 45 x0 karyotype and increased concentration of alpha-fetoproteins. pregnancy was terminated in view of Turner's syndrome. The etiology and natural history of the condition is reviewed.
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keywords = karyotype
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2/20. Cystic hygroma and congenital diaphragmatic hernia: early prenatal sonographic evaluation of Fryns' syndrome.

    We report a case of cystic hygroma and diffuse lymphangiectasia detected by sonogram at 12 weeks' gestation. Fetal karyotype was normal. At 20 weeks' gestation, herniation of the bowel into the chest was noted. At delivery, the infant was diagnosed as having Fryns' syndrome. This is the first reported case of Fryns' syndrome presenting with cystic hygroma.
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keywords = karyotype
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3/20. prenatal diagnosis of giant cystic hygroma: prognosis, counselling, and management; case presentation and review of the recent literature.

    Cystic hygromas have historically been associated with a grim prognosis when discovered during prenatal sonographic study of the fetus. This same grim prognosis is not observed by the paediatric surgeon who evaluates the neonate or paediatric patient. We present a fetal patient with a massive anterior cystic hygroma discovered in the third trimester prior to 30 weeks. This case and a review of the literature suggest tailoring the prognosis by category when counselling patients: (1) first trimester, normal karyotype--good; (2) first trimester, abnormal karyotype--poor; (3) second trimester and early third--poor to guarded; and (4) mid to late third trimester--good.
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ranking = 2
keywords = karyotype
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4/20. prognosis in fetal cystic hygroma.

    Axillary and lateral cervical fetal cystic hygromas in a fetus with normal karyotype are described. fetal death at 25 weeks' gestation occurred. A literature review revealed that for cystic hygroma 42% of infants are 45XO, 38% have a normal karyotype, and 18% have trisomies. prognosis is grim if the karyotype is abnormal or if hydrops or bilateral pleural effusions are present. survival rate progressively improves with normal karyotype (27%), unilateral pleural effusion (40%), atypical location (56%), and resolution of cystic hygroma (71%). No single feature signifies 100% survival. The overall survival rate for fetal cystic hygroma is 10%. prognosis remains guarded regardless of all other factors until the fetus reaches 26 weeks' gestation, after which time a 67% chance of ultimate survival can be expected. Only 42% of documented survivors were completely normal at follow-up.
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ranking = 4
keywords = karyotype
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5/20. Massive cystic lymphangiomas of a fetus.

    We present a fetus with progressive massive subcutaneous lymphangiomas leading to intrauterine death. A 28-year-old woman was referred to our hospital because of a precordial cystic mass of the fetus. An ultrasound revealed lymphangiomas extending from bilateral axillae to the anterior chest wall. At 18 weeks' gestation, amniocentesis was performed and the karyotype of the fetus was found to be normal 46, XY. Thereafter the lesions increased in size gradually and spread over the body. amniotic fluid decreased, pericardial, and pleural effusion appeared, and cardiomegaly became evident. The fetus died in utero at 25 weeks' gestation. Postmortem examination revealed a male fetus surrounded with multicystic soft masses spreading over the body, and syndactyly (left third and fourth fingers) was present. Histologically, a number of irregularly dilated lymphatics extended through subcutaneous tissues to the skeletal muscles. No communications between the cysts and the thoracic or abdominal cavity existed, and no lymphatic dilations in the viscera were confirmed. As far as we know, such conditions have rarely been reported. Considering that in previous literature, a favorable prognosis of a fetus with an atypically located (lateral cervical or non-cervical) lymphangioma with a normal karyotype has been reported, our case may be included in a distinct pathological entity. When we find a lymphangioma in a fetus, careful follow-up by ultrasound is mandatory.
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ranking = 2
keywords = karyotype
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6/20. Spontaneous resolution of fetal nuchal cystic hygroma.

    Complete resolution of the hygroma occurred in two fetuses with the mid-trimester ultrasound diagnosis of a nuchal cystic hygroma. Cytogenetic studies showed a normal 46,XX karyotype in one fetus, and a 47,XX, 18 in the other. Complete regression of cystic hygroma has been reported in fetuses with normal chromosomes, as well as in those with trisomy 21, and with Turner's syndrome. The incidence of spontaneous in utero resolution of fetal nuchal cystic hygroma is unknown. The natural history of cystic hygroma in utero cannot be correlated with the chromosome complement. An antenatal karyotype determination should be offered to any patient whose fetus has cystic hygroma, even to those with spontaneous resolution.
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ranking = 2
keywords = karyotype
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7/20. Pitfall in the diagnosis of nuchal cystic hygroma.

    We present a case of spontaneous resolution of a nuchal cystic hygroma in a fetus with a normal karyotype. This unusual case is important in the counseling of patients with affected fetuses, since the transitory nature of the disease is not well known.
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ranking = 1
keywords = karyotype
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8/20. Familial cystic hygroma with normal karyotype.

    A patient is described who had three consecutive fetuses with cystic hygroma and hydrops, two of which had documentation of normal karyotype. Some twenty percent of fetuses with cystic hygroma have a normal karyotype, and many of these have other major malformations. An autosomal recessive pattern of inheritance has been postulated, but cystic hygroma may also occur in association with a variety of syndromes, some of which have other patterns of inheritance.
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ranking = 6
keywords = karyotype
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9/20. Spontaneous resolution of cystic hygroma in a 46,XX normal female.

    We report a case of nuchal cystic hygroma with spontaneous resolution detected by ultrasound examination at 13 weeks' gestation. Fetal karyotype and amniotic fluid alpha-fetoprotein levels were normal. Extreme caution in evaluating this situation is stressed.
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ranking = 1
keywords = karyotype
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10/20. A case of renal lymphangioma with a karyotype 45,X,-X,i dic(7q).

    cytogenetic analysis of a polycystic unilateral renal lymphangioma was performed by short-term culture and banding methods. The tumor's cells showed an isochromosome of the long arm of chromosome #7 and monosomy of x chromosome, whereas the peripheral lymphocytes stimulated with phytohemagglutinin showed a normal female karyotype, 46,XX. These karyotypic anomalies suggest that lymphangioma, although clinically benign, may have malignant potential.
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ranking = 5
keywords = karyotype
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