Recurrent congenital chylothorax

King, Patricia A.; Ghosh, Anupam; Tang, Mónica; Lam, S. K.

doi:10.1002/pd.1970111010

Cited by 22 publications

(17 citation statements)

References 8 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Most cases of chylothorax are sporadic, however Reece et al [2] suggested an X-linked recessive transmission and another report [1] described a congenital chylothorax in two consecutive female siblings suggesting autosomal recessive inheritance. We believe that the association of congenital chylothorax with trisomy X is worthy of notice in order to counsel parents after the appearance of an aected ®rst child.…”

mentioning

confidence: 99%

Congenital chylothorax associated with trisorny X

et al. 2001

View full text Add to dashboard Cite

The association of congenital chylothorax and trisomy X is important for counselling families at risk.Recently we treated a female newborn with congenital chylothorax who was thought initially to have a bilateral fetal hydrothorax.The baby was born by caesarean section at 35 weeks gestation due to fetal distress and hydrops fetalis. The mother was 42 years of age, her pregnancy was complicated by gestational diabetes and because of her age, an amniocentesis was performed in the 19th week. The fetal karyotype was 47XXX or trisomy X. This was con®rmed by karyotyping the baby's lymphocytes and then the ®broblasts when the diagnosis of chylothorax was established. The congenital chylothorax was treated according to the numerous articles found in the literature. The baby is seen regularly at the outpatient clinic and her clinical condition and development are both good. However, we could not ®nd any article in the literature concerning the association of congenital chylothorax and trisomy X.In an attempt to explain this case, we suggest that the fetal karyotype was a mosaic 47XXX/45X. Therefore in early fetal life there must have been a loss of one cell line, the 45X. This line, not detected later in chromosomal studies done in the amniocytes and the baby's cultured skin ®broblasts, was related to problems in lymphatic development resulting in the chylothorax.Most cases of chylothorax are sporadic, however Reece et al. [2] suggested an X-linked recessive transmission and another report [1] described a congenital chylothorax in two consecutive female siblings suggesting autosomal recessive inheritance. We believe that the association of congenital chylothorax with trisomy X is worthy of notice in order to counsel parents after the appearance of an aected ®rst child. References 1. King PA, Ghosh A, Tang MHY, Lam SK (1991) Recurrent congenital chylothorax. Prenat Diagn 11: 809±811 2. Reece EA, Lockwood CJ, Rizzo N, Pilu G, Bivicelli L, Hobbins JC (1987) Intrinsic intrathoracic malformations of the fetus: sonographic detection and clinical presentation. Obstet Gynecol 70: 627±632 Eur J Pediatr (2001) 160: 743

show abstract

mentioning

confidence: 99%

Congenital chylothorax associated with trisorny X

et al. 2001

View full text Add to dashboard Cite

show abstract

“…There is a 2:1 male to female ratio, leading some authors to postulate an X-linked recessive gene. While most cases of this condition are sporadic, definite autosomal recessive inheritance has been reported in one case (two affected females) [King et al, 1991]. Consanguinity was not documented.…”

Section: Family Onementioning

confidence: 95%

Unusual autosomal recessive lymphatic anomalies in two unrelated Amish families

Williams

Josephson

1997

Am. J. Med. Genet.

View full text Add to dashboard Cite

We report on two unrelated Amish families with familial occurrence of unusual lymphatic anomalies. The first family had two children, a boy and a girl, with congenital chylothorax both of whom died as a consequence of this condition (one prenatally and one neonatally). The second family has two brothers with isolated cystic hygroma. Neither family has any other individuals affected with any type of lymphatic anomaly. Differential diagnosis and presumed autosomal recessive inheritance pattern will be discussed. Familial cystic hygroma not associated with hydrops fetalis and neonatal death has not been reported previously.

show abstract

“…Chylothorax is a very rare condition of pleural infusion in young children, occurring in approximately 1 in 10,000–15,000 pregnancies which is associated with a high overall mortality rate of 25%–50% [1], [2]. It is not presented commonly however; it is a very common cause of pleural effusion in the fetus and neonatal age [1], [2].…”

Section: Introductionmentioning

confidence: 99%

“…It is not presented commonly however; it is a very common cause of pleural effusion in the fetus and neonatal age [1], [2]. It has been observed that males are most commonly affected than females.…”

Section: Introductionmentioning

confidence: 99%

“…Chylothorax is usually induced spontaneously due to lymphatic malformations or may be caused during birth, due to a trauma in the thoracic duct following difficult labor [4]. It may cause nutritional failure, respiratory compromise or even immune system deficiency, which subsequently can cause septic complications [1]. Herein, we present a case of familial congenital chylothorax which was definitely treated with an open thoracic duct ligation after failure of the conservative treatment.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Successful treatment of familial congenital chylothorax by ligation of the thoracic duct: A case report

Dori

Smaropoulos

Tagarakis

et al. 2017

Respiratory Medicine Case Reports

View full text Add to dashboard Cite

A full term boy was admitted with respiratory distress in the fourth week of his life due to spontaneous chylothorax in his right hemithorax. Spontaneous chylothorax occurred previously in a first cousin of the neonate establishing that way the final diagnosis of familial idiopathic congenital pneumothorax. Failure of the conservative treatment consisting of chest tube drainage, discontinuation of oral diet and administration of total parenteral nutrition in combination with octreotide for one month was followed by the successful ligation of the thoracic duct through a right thoracotomy. The boy still remains free of symptoms and without recurrence of the chylothorax two years later.

show abstract

Recurrent congenital chylothorax

Cited by 22 publications

References 8 publications

Congenital chylothorax associated with trisorny X

Congenital chylothorax associated with trisorny X

Unusual autosomal recessive lymphatic anomalies in two unrelated Amish families

Successful treatment of familial congenital chylothorax by ligation of the thoracic duct: A case report

Contact Info

Product

Resources

About