Etiology, prognosis and management of nuchal cystic hygroma: 25 new cases and literature review

Descamps, P.; Jourdain, O; Paillet, C.; Toutain, Annick; Guichet, Agnès; Pourcelot, D.; Gold, F.; Castiel, Mercedes; Body, G.

doi:10.1016/s0301-2115(96)02590-0

Cited by 70 publications

(65 citation statements)

References 80 publications

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“…Two of 5 live-born cases (40%) resulted in intact survival, both having a normal karyotype without structural malformations except for cystic hygroma. It has been reported that fetuses with neither chromosomal aberration nor additional structural malformations are potentially viable [1,2,20]. Our results coincide well with such reports.…”

Section: Discussionsupporting

confidence: 93%

“…On the other hand, the remaining 3 liveborn cases in this study were handicapped and mentally retarded; 2 having chromosomal aberrations and the other having a metabolic disorder. As cases with both cystic hygroma and chromosomal aberration tend to result in fetal demise or termination of pregnancy [2,12,20], there were to our knowledge no reports on the long-term prognoses of such cases. Our results clearly show that these cases have a high possibility of being handicapped, even if a fetus with a chromosomally abnormal cystic hygroma is born live.…”

Section: Discussionmentioning

confidence: 92%

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In utero Evaluation and the Long-Term Prognosis of Living Infants with Cystic Hygroma

Fujita¹,

Shoji²,

Nakayama³

et al. 2001

Fetal Diagn Ther

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Objective: The aim is to evaluate the natural course and the long-term prognosis of cystic hygroma following live birth. Methods: Of 39 cases, 12 were excluded because of either artificial termination or intrauterine treatment. 27 cases were retrospectively classified into 2 groups based on the outcome, and the clinical profiles were compared. The long-term prognosis was analyzed for live births. Results: 5 (18.5%) and 22 (81.5%) cases resulted in live birth and fetal demise, respectively. Both the frequency of hydrops fetalis and the maximum size of the cyst were more significantly associated with a poor prognosis (p < 0.01). Of 5 live births, 3 cases with chromosomal or structural abnormalities were handicapped. Conclusions: Hydrops fetalis and cyst size are poor prognostic factors, and fetal chromosome and structural malformations should be taken into account when predicting the long-term prognosis.

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Section: Discussionsupporting

confidence: 93%

Section: Discussionmentioning

confidence: 92%

In utero Evaluation and the Long-Term Prognosis of Living Infants with Cystic Hygroma

Fujita¹,

Shoji²,

Nakayama³

et al. 2001

Fetal Diagn Ther

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“…Cystic hygroma is described as multiple congenital cysts due to congenital malformations of the lymphatic system. It has been reported that 40-60% of cystic hygroma cases are associated with Turner syndrome [9] . In this study, prenatal cytogenetic test results were evaluated in 3,595 cases to detect the incidence of Turner syndrome and referral indications of Turner syndrome fetuses in the period between 1998 and 2007.…”

Section: Introductionmentioning

confidence: 99%

Prenatally Diagnosed Turner Syndrome and Cystic Hygroma: Incidence and Reasons for Referrals

et al. 2008

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Objective: The objective of this study was to evaluate the incidence and reasons for referrals for prenatally detected Turner syndrome and cystic hygroma cases among prenatal cases performed between 1998 and 2007. Methods: In this study 3,595 amniocentesis, chorionic villus and cordocenthesis materials obtained between 1998 and 2007 were evaluated. Among prenatal cases, 23 Turner syndrome cases were also evaluated according to their referral reasons. Among the indications of prenatal cases, cystic hygroma was evaluated according to karyotype results. Results: Twenty-three cases were Turner Syndrome in which 7 cases were detected to have mosaic pattern. The indications for prenatal diagnosis for the cases were cystic hygroma in 11 cases, missed abortion in 6 cases, advanced maternal age in 5 cases and positive screening test results in 1 case. Among 18 cases having cystic hygroma detected by ultrasonography, 8 cases (44.4%) were found to have a 45,X karyotype, 3 cases were found to be mosaic Turner syndrome (16.7%), 5 cases (27.7%) had normal karyotype, 1 case (5.6%) 47,XX,+13 and 1 case (5.6%) 47,XX,+21. Conclusion: The present study indicates the importance of cystic hygroma in prenatal diagnosis of Turner Syndrome and other aneuploidies.

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“…Obstruction of the lymphatics leads to enlargement of the cyst, most commonly found in the posterior neck. Fetuses with cystic hygroma frequently suffer from other complications such as chromosomal abnormalities, fetal hydrops, and other structural malformations and tend to have poor outcomes [1][2][3][4]. However, recent investigations suggest that cases without these complications have a sufficient potential for intact survival [3,5].…”

Section: Introductionmentioning

confidence: 99%

“…Fetuses with cystic hygroma frequently suffer from other complications such as chromosomal abnormalities, fetal hydrops, and other structural malformations and tend to have poor outcomes [1][2][3][4]. However, recent investigations suggest that cases without these complications have a sufficient potential for intact survival [3,5]. Although there have been a few reports describing trial cases of intrauterine therapy for fetal cystic hygroma, indications for the therapy have not yet been established [6][7][8].…”

Section: Introductionmentioning

confidence: 99%

Intrauterine Therapy for the Acutely Enlarging Fetal Cystic Hygroma

et al. 2004

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Enlarged fetal cystic hygroma is known to cause life-threatening complications such as fetal hydrops and neonatal respiratory difficulty. A 28-year-old Japanese woman, gravida 0, presented with fetal cystic hygroma at 23 weeks of gestation. There were no other structural malformations or hydrops detected by ultrasonographic examination. In addition, the karyotype was diagnosed as normal through amniotic fluid analysis. The cystic lesion showed acute enlargement and intrauterine sclerotherapy using OK-432 was performed at 26 weeks. The size of the cyst initially decreased, which was followed by a gradual increase. A viable 3,098 g male infant was delivered by cesarean section at 37 weeks without any other complications. The infant had no clinical difficulty during the neonatal period and later underwent a surgical removal of the remaining cystic lesion. Cases of fetal cystic hygroma showing acute enlargement without other complications are considered good candidates for intrauterine therapy to prevent subsequent complications.

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Etiology, prognosis and management of nuchal cystic hygroma: 25 new cases and literature review

Cited by 70 publications

References 80 publications

In utero Evaluation and the Long-Term Prognosis of Living Infants with Cystic Hygroma

In utero Evaluation and the Long-Term Prognosis of Living Infants with Cystic Hygroma

Prenatally Diagnosed Turner Syndrome and Cystic Hygroma: Incidence and Reasons for Referrals

Intrauterine Therapy for the Acutely Enlarging Fetal Cystic Hygroma

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