Ortega-Salgado JA, Ramírez-Reséndiz A, Carrazco-Daza D, Rodríguez-Jurado R

Language: Spanish
References: 14
Page: 138-142
PDF size: 317.84 Kb.

ABSTRACT

Introduction: The generalized lymphangiomatosis is a not very frequent entity that is due to a flaw in the development of the lymphatic system, characterized by aberrant proliferation of the same ones, in soft tissues, bone or parenchymatous organs. The poor understanding of this illness, must invited us to report the cases to increase the literature reports and this way later on to analyze the accumulated experience in relation to diagnosis, treatment and prognosis of the illness.
Material and Methods: The data of the clinical file of each one of the patients with histology diagnoses of generalized lymphangiomatosis, the relating data to age, sex, diagnose age, histology description of the biopsies, clinical evolution, follow time, treatment, morbidity, and mortality of each case were registered.
Results: Four patients with age range of 9months to 5 years, at the moment of the diagnosis were included. 3 or 5 organs were involved. In all the cases, pleura and peritoneum were involved. Three patients died, one of them went to 12 years and 9months and one only 2 months. In all medical and surgical treatment was given.
Conclusions: The diagnosis and treatment of these patients is difficult. The treatment persists being palliative, and what we have left is to insist in the study of the physiopathology of the illness to give alternative of treatment.

REFERENCES

1. Noonan JA, Walters LR, Reeves JT. Congenital pulmonary lymphangiectasia. Am J Dis Child 1970; 20: 314.

2. Shah AR, Dinwiddle R, Woolf D, Ramani R, Higgins JNP, Matthew DY. Pediatr Pulmonol. 1992; 14:126-130.

3. Eugine H. Cavernous lymphangioma. J Pediatr, 2001; 138(1)

4. Tazelaar HD, Kerr, D, Yousemem SA, Saldana MJ, Langston, Colby TUV, Difuse pulmonary Linphangiomatosis, Human Patology 24,12; 1993 1313-1322.

5. Groves LK, Effler DB. Primary chylopericardium. N Engl J Med 1954; 250:520-3.

6. Higgins JNP, Shah ARR, Dicks-Mereaux, Conry Vg. Case report: computed tomography of generalized lymphangiomatosis and chylothorax, the british Journal of Radiology 66, 1993; 1189-1192.

7. Asch JM, Cohen AH, Moore TC. Hepatic and splenic lymphangiomatosis with skeletal involment report of a case and review of the literature, Surgery 76, 2; august 1974, 334-339.

8. Barrier A, Lacaide F, Callard P, Huguier M, Lymphangiomatosis of the spleen and 2 accesory spleens Surgery 131, (1), 2002, 114-116.

9. Freudlinch I, the role of lymphangiography in chylotorax. A report of six nontraumatic cases. American Journal Radiology 125, 617-627.

10. Morphis LG, Arcinue EL, Krause JR. Pediatrics 1970; 46: 566-575.

11. Shah AR, Dinwiddie R, Ramani R, Higgins JNP, Mathew DJ, Generalized Lymphangiomatosis and Chylothorax in the Pediatric Age Group; Pediatric Pulmonology 14, 1992; 126-130.

12. Kirkland I Chylothorax in infancy and childhood. A method of treatment. Arch Dis Child 1965; 40:186.

13. Dajee H, Woodhouse. Lymphangiomatosis of the mediastinium with chylhotorax and chylhopericardium: Role of Radiation treatment, The Journal of Thoracic and Cardiovascular Surgery 1994; 3: 594.

14. Mertsching H, Walles T, Wildfang I, Macchiarini. Expresion of epidermalgrowth-factor receptor in lymphangiomatosis: a new therapeutic target ? The Lancet Oncology 2004; 5: 6.