2023, Number 2
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Rev Mex Anest 2023; 46 (2)
Primary intestinal lymphagiectasia in Klippel-Trénaunay syndrome. Anesthetic management. Case report
Mora-de LBJ, Téllez-Martínez SE
Language: Spanish
References: 24
Page: 144-148
PDF size: 382.47 Kb.
ABSTRACT
Klippel-Trénaunay syndrome with associated primary intestinal lymphangiectasia is a rare entity in pediatrics. It presents with edema due to hypoproteinemia, and in some cases with ascites. The objective is to present a clinical case whose diagnosis was certified by intestinal esdoscopy and biopsy. For refractary ascities, the treatment was surgical, performing peritoneal venous bypass. The anesthetic management of this type of patients has been described in limited reports, so in the present case we emphasize supportive therapy with albumin infusion as the proposed symptomatic treatment for patients with massive drainage of ascities.
REFERENCES
Klippel M, Trénaunay P. Du naevus variqueux osteohypertrophique. Arch Gen Med. 1900; 185: 641-672.
Pereda Marin RM, Garcia Collada JC, Garrote Martinez AI, Miralles Serrano EM, Morales Aguilar JL. Anesthetic management of Klippel-Trenaunay syndrome and attendant gastrointestinal hemorrhage. A case report. Minerva Anestesiol. 2007; 73: 187-190.
Waldmann TA, Steinfeld JL, Dutcher TF, Davidson JD, Gordon RS. The role of the gastrointestinal system in idiopathic hypoproteinemia. Gastroenterology. 1961; 41: 197-207.
Waldmann TA. Protein-losing enteropathy. Gastroenterology. 1966; 50: 422-443.
Hennekam RC, Geerdink RA, Hamel BC, Hennekam FA, Kraus P, Rammeloo JA, Tillemans AA. Autosomal recessive intestinal lymphangiectasia and lymphedema, with facial anomalies and mental retardation. Am J Med Genet. 1989; 34: 593-600.
Hokari R, Kitagawa N, Watanabe C, Komoto S, Kurihara C, Okada Y, Kawaguchi A, Nagao S, Hibi T, Miura S. Changes in regulatory molecules for lymphangiogenesis in intestinal lymphangiectasia with enteric protein loss. J Gastroenterol Hepatol. 2008; 23: 88-95.
Martínez CLF, Casas LM, Martínez NY, Cubero MO, García PW. Ascitis en ausencia de edema: forma rara de inicio de la linfangiectasia intestinal congénita. Rev Cubana Pediatr. 2005; 77 (2).
Mazzie JP, Maslin PI, Moy L, Price AP, Katz DS. Congenital intestinal lymphangiectasia: CT demonstration in a young child. Clin Imaging. 2003; 27: 330-332.
Rivet C, Lapalus MG, Dumortier J, Le Gall C, Budin C, Bouvier R, Ponchon T, Lachaux A. Use of capsule endoscopy in children with primary intestinal lymphangiectasia. Gastrointest Endosc. 2006; 64: 649-650.
Lymphoedema Framework: Best practice for the management of lymphoedema. International consensus. London: MEP Ltd; 2006.
David B, Wilson J. Anesthesia for Surgery Related to Klippel–Trenaunay Syndrome: A Review of 136 Anesthetics. Anesth Analg. 2011; 113: 98-102.
Fontanarosa PB, Goldman GE, Polsky SS, Shuckman HA, Poyle M. Sitting oral-tracheal intubation. Ann Emerg Med. 1988; 17: 336-338.
De Leon-Casasola OA, Lema MJ. Epidural anesthesia in patients with Klippel-Trenaunay syndrome. Anesth Analg. 1992; 74: 470-471.
Holak EJ, Pagel PS. Successful use of spinal anesthesia in a patient with severe Klippel-Trenaunay syndrome associated with upper airway abnormalities and chronic Kasabach-Merritt coagulopathy. J Anesth. 2010; 24: 134-138.
Bernardi M, Carceni P, Navickis RJ, Wilkes MM. Albumin infusion in patients undergoing large volume paracentesis: a meta-analysis of randomized trials. Hepatology. 2012; 55: 1172-1181.
Ginés P, Angeli P, Lenz K et al. EASL clinical practice guidelines on the management of ascites, spontaneous bacterial peritonitis, and hepatorenal syndrome in cirrhosis, J. Hepatol. 2010; 53: 397-417.
Mantz J. A quelle concentration l'albumine doit-elle etre prescrite? Ann Fr Anesth Réanim. 1996; 15: 543-546.
Tjoeng MM, Bartelink AK, Thijs LG. Exploding the albumin myth. Pharm World Sci. 1999; 21: 17-20.
Roberts I, Blackhall K, Alderson P, Bunn F, Schierhout G. Human albumin solution for resuscitation and volume expansion in critically ill patients. Cochrane Database Syst Rev. 2011; 2011: CD001208.
Wilkes MM, Navickis RJ. Patient survival after human albumin administration: a meta-analysis of randomized, controlled trials. Ann Intern Med. 2001; 135: 149-164.
Finfer S, Bellomo R, Boyce N, French J, Myburgh J, Norton R; SAFE Study Investigators. A comparison of albumin and saline for fluid resuscitation in the intensive care unit. N Engl J Med. 2004; 350: 2247-2256.
Delaney AP, Dan A, McCaffrey J, Finfer S. The role of albumin as a resuscitation fluid for patients with sepsis: a systematic review and meta-analysis. Crit Care Med. 2011; 39: 386-391.
The SAFE Study Investigators. Saline or albumin for fluid resuscitation in patients with traumatic brain injury. N Engl J Med. 2007; 357: 874-884.
Quaranta JF. Complication immédiates et retardées liées a l'administration d'albumine. Ann Fr Anesth Réanim. 1996; 15: 553-559.