Keywords
Intestinal Lymphangiectasis
Fetal Ultrasonography
Children
How to Cite
Abstract
Background. Chylous ascites is an infrequent entity that is rarely diagnosed in utero. Although intestinal lymphangiectasia is mentioned as one of the causes of chylous ascites, actually it is difficult to detect this relation.
Clinical case. We report a case of an infant diagnosed in utero by ultrasonography of fetal ascites and hydrocele. Physical examination after birth was significant for generalized oedema, ascites, and hydrocele. Aparacentesis yielded liquid of chylous characteristics. Duodenal biopsy showed dilated lymphatics and villous atrophy. Aformula containing most lipids as medium chain triglycerides resulted in a paulatine cessation of ascites and hydrocele.
Conclusions. Primary intestinal lymphangiectasia usually manifests as a protein-losing enteropathy, but it is also considered as part of a congenital disorder of the lymphatic system as a whole. This may explain its presentation as congenital chylous ascites.
References
Ruhra J. Richard Morton, M.D., 1637-1698. A note on the history of chylous ascites. Am J Dis Child 1934; 47:629-631. https://doi.org/10.1001/archpedi.1934.01960100155019
Cochran WJ, Klish WJ, Brown MR, Lyons JM, Curtis T. Chylous ascites in infants and children: a case report and literature review. J Ped Gastroenterol Nutr 1985; 4:668-673. https://doi.org/10.1002/j.1536-4801.1985.tb08928.x
Vardy PA, Lebenthal E, Shwachman H. Intestinal lymphangiectasia: a reappraisal. Pediatrics 1975; 55:842-851. https://doi.org/10.1542/peds.55.6.842
Schmider A, Henrich W, Reles A, Vogel M, Dudenhausen JW. Isolated fetal ascites caused by primary lymphangiectasia: a case report. Am J Obstet Gynecol 2001; 184:227-228. https://doi.org/10.1067/mob.2001.106756
Munck A, Foucaud P, Walti H, Dumez Y, Vaudour G, Navarro J. Lymphangiectasies intestinales primitives a revelation antenatale. Arch Fr Pediatr 1986; 43:195-6.
Machmouhci M, Amin A, Lanjaoui I, Jacobs A, Hatoum C, Al Zahrani D. Congenital chylous ascites: report of four cases and review of the literature. Ann Saudi Med 2000; 436-439. https://doi.org/10.5144/0256-4947.2000.436
Pomerantz M, Waldmann TA. Systemic lymphatic abnormalities associated with gastrointestinal protein loss secondary to intestinal lymphangiectasia. Gastroenterology 1963; 45:703-711. https://doi.org/10.1016/S0016-5085(19)34802-4
Molina M, Romero A, Antón S, Sarriá J, Prieto G, Polanco I. Linfangiectasia intestinal primaria: evolución a largo plazo. An Esp Pediatr 2001; 54(Supl 3): 33-35.
Takahashi H, Kohzoh I. What are the objectives of treatment for intestinal lymphangiectasia? J Gastroenterol 2001; 36:137-138. https://doi.org/10.1007/s005350170144
Caty MG, Hilfiker MI, Azizkhan RG, Glick PL. Successful treatment of congenital chylous ascites with a somatostatine analog. Pediatr Surg Int 1996; 11:396-397. https://doi.org/10.1007/BF00497824
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Copyright (c) 2007 Boletín de Pediatría