+ Site Statistics
+ Search Articles
+ PDF Full Text Service
How our service works
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ Translate
+ Recently Requested

Primary lymphatic dysplasia in children chylothorax chylous ascites and generalized lymphatic dysplasia



Primary lymphatic dysplasia in children chylothorax chylous ascites and generalized lymphatic dysplasia



European Journal of Pediatrics 145(4): 286-292



Primary lymphatic "dysplasia", a congenital maldevelopment, interferes with function of the lymphatic system and causes efflusion of chyle or lymph into the limbs and pleural or peritoneal cavity. Between 1955 and 1982, 38 Mayo Clinic patients were found to have a chylous effusion or dysplasia of the lymphatic system. In 22, the condition was secondary to surgery or other medical problems and in 16 it was primary. These cases were separated into three categories: chylothorax, chylous ascites, and generalized lymphatic dysplasia. Conservative therapy, such as a restricted fat diet or total parenteral nutrition with repeated thoracentesis or paracentesis, was effective in the children with isolated abnormalities of the lymphatic system (75% resolution rate, no deaths). All five children with documented generalized dysplasia reported in the literature had died; of the three reported here, one has died and two have become progressively worse.

Please choose payment method:






(PDF emailed within 0-6 h: $19.90)

Accession: 006183496

Download citation: RISBibTeXText

PMID: 3769996

DOI: 10.1007/bf00439402


Related references

Chylous reflux and chylous ascites in lymphatic dysplasia, with the lymphographic demonstration of mediastinal and retroperitoneal lymphatic cysts. Rofo 122(1): 14-19, 1975

Generalized lymphangiectasis associated with chylothorax; a possible dysplasia of the lymphatic system. European Journal of Pediatrics 118(1): 9-24, 1974

Generalized lymphangiectosis associated with chylothorax; a possible dysplasia of the lymphatic system. Zeitschrift für Kinderheilkunde 118(1): 9-24, 1974

Postmenarchal Development Of Chylous Ascites In Acrocephalosyndactyly With Congenital Lymphatic Dysplasia. Obstetrics & Gynecology 97(5): 829-831, 2001

Postmenarchal development of chylous ascites in acrocephalosyndactyly with congenital lymphatic dysplasia. Obstetrics & Gynecology 97(5 Part 2): 829-831, 2001

Congenital lymphatic-vessel dysplasia with intestinal lymphangiectasie and chylous ascites in twins. Monatsschrift für Kinderheilkunde 125(5): 526-529, 1977

Clinical analysis of 2 cases with chylothorax due to primary lymphatic dysplasia and review of literature. Zhonghua Er Ke Za Zhi 52(5): 362-367, 2015

Indocyanine green lymphography and lymphaticovenous anastomosis for generalized lymphatic dysplasia with pleural effusion and ascites in neonates. Annals of Vascular Surgery 29(6): 1111-1122, 2016

Non-traumatic chylothorax associated with diffuse lymphatic dysplasia. Lymphology 17(4): 111-112, 1984

Use of somatostatin analogues to treat chylothorax in a child with Generalised Lymphatic Dysplasia. Respiratory Medicine Case Reports 5: 76-77, 2012

Pleuroperitoneal shunt in the management of chylothorax caused by thoracic lymphatic dysplasia. Journal of Pediatric Surgery 34(9): 1420-1422, 1999

Retroperitoneal nodal aplasia, asplenia, chylous effusion and lymphatic dysplasia: an acquired immunodeficiency syndrome?. Lymphology 18(1): 31-36, 1985

Dysplasia of the retroperitoneal lymphatic system and chyle ascites. Fortschritte auf dem Gebiete der Rontgenstrahlen und der Nuklearmedizin 106(4): 533-541, 1967

Generalized lymphatic dysplasia in a 6-month-old nursing infant. La Pediatria Medica E Chirurgica 13(1): 99-103, 1991

The diagnosis of lymphatic vascular dysplasia and lymphatic fistula formation. Rofo 156(3): 282-285, 1992