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Copyright (c) 2019 Carlos Arturo Paniagua-Coahuila, Crescencio Martínez-Martínez , José Luis Arellano-Hernández , Dulce Alejandra Alonso-Lozano , Freddy Mendoza-Hernández , Hugo Mendieta Zerón
This work is licensed under a Creative Commons Attribution 4.0 International License.
Klippel Trenaunay Weber syndrome in pregnancy. Case report and literature review
Corresponding Author(s) : Carlos Arturo Paniagua-Coahuila
Ciencia e Innovación en Salud,
2019
Abstract
Background: Klippel-Trenaunay syndrome (SKT) is a congenital disorder characterized by the presence of vascular cutaneous nevus, venous malformations, and soft tissue and bone hypertrophy affecting one or more extremities.
Case report: A 20-year-old female, primiparous, diagnosed with SKT since childhood, was attended at 34 weeks of gestation with multiple cervical and vulvar varices and the presence of a vasculocutaneous nevus in the right pelvic limb; Contrast angioresonance was performed, where multiple arteriovenous malformations were confirmed at the level of the right pelvic limb, compromising the inguinal, vulvar, femoral region and discreetly the uterine segment. Treatment with low molecular weight heparin was started until it was suspended 24 hours prior to the scheduling of caesarean section at 39.1 weeks of gestation.
Discussion: An uncomplicated newborn was obtained with transsurgical bleeding of 450 ml. She went home 48 hours post-surgery without evidence of complications and subsequent follow-up in an outpatient clinic for angiology and obstetric critical medicine.
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- Estrada MM, Guerrero AGML, Enríquez GR, González de la CJ. Síndrome de Klippel Trenaunay. Hallazgos clínicos y de imagen. An. Radiol. Méx. 2006;5(3):245-251.
- Velázquez Chacón W, Matos Batista Y. Enfoque Interdisciplinario en la Gestante con Síndrome de Klippel-Trenaunay-Weber. Revista Científica Hallazgos21, 2016;1(1):1-9 https://revistas.pucese.edu.ec/hallazgos21/article/view/10
- Pérez M, Hernández H, Vázquez LA. Síndrome de Klippel-Trenaunay-Weber complicado con trombosis venosa cerebral. CorSalud, 2016;8(4):265-269. https://www.medigraphic.com/cgi-bin/new/resumen.cgi?IDARTICULO=73879
- Lindenauer SM. The klippel-trenaunay syndrome: varicosity, hypertrophy and hemangioma with no arteriovenous fistula. Ann Surg. 1965;162(2):303-14.
- Clasificación Internacional de las Enfermedades. Disponible en: https://eciemaps.mscbs.gob.es/ecieMaps/browser/index_10_mc.htm
- Angiogenic factor with G-patch and FHA domains 1; AGGF1. Disponible en: https://www.omim.org/entry/608464
- Barajas TJ, Delgado EG, Urióstegui LC, López V, Luna U. Síndrome de Klippel Trenaunay. Rev Cubana Med Gen Integr, 2016;32(3):1-7. http://scielo.sld.cu/scielo.php script=sci_abstract&pid=S0864-21252016000300020&lng=es&nrm=iso
- Ceballos-Quintal JM, Pinto-Escalante D, Castillo-Zapata I. A new case of Klippel-Trenaunay-Weber (KTW) syndrome: evidence of autosomal dominant inheritance. Am J Med Genet. 1996;63:426–427. https://pubmed.ncbi.nlm.nih.gov/8737646/
- Lorda-Sánchez I, Prieto L, Rodríguez-Pinilla E, Martínez-Frías ML. Increased parental age and number of pregnancies in Klippel-Trenaunay-Weber syndrome. Ann Hum Genet. 1998;62:235–239. https://pubmed.ncbi.nlm.nih.gov/9803268/
- Whelan AJ, Watson MS, Porter FD, Steiner RD. Klippel-Trenaunay-Weber syndrome associated with a 5:11 balanced translocation. Am J Med Genet. 1995;59:492–494. https://pubmed.ncbi.nlm.nih.gov/8585570/
- Wang Q, Timur AA, Szafranski P, Sadgephour A, Jurecic V, Cowell J, et al. Identification and molecular characterization of de novo translocation t(8;14) (q22.3;q13) associated with a vascular and tissue overgrowth syndrome. Cytogenet Cell Genet. 2001;95:183–188. https://
- pubmed.ncbi.nlm.nih.gov/12063397/
- Timur AA, Sadgephour A, Graf M, Schwartz S, Libby ED, Driscoll DJ, et al. Identification and molecular characterization of a de novo supernumerary ring chromosome 18 in a patient with Klippel-Trenaunay syndrome. Ann Hum Genet. 2004;68:353–361. https://pubmed.ncbi.nlm.nih.gov/15225160/
- González E, Blasco M, Andérica J. Klippel-Trenaunay syndrome complicating pregnancy. BMJ Case Reports. 2012:1-4. https://pubmed.ncbi.nlm.nih.gov/22854239/
- Redondo SCA, Yaber GY, Gutiérrez DR, Garavito GP, Charris M, Sogamoso H, Cantillo Z, Ordóñez HJ, Parra G, Denubilla E. Síndrome de Klippel Trenaunay Weber y asplenia. Revista Científica Salud Uninorte, 1998;13(1):31-33. http://rcientificas.uninorte.edu.co/index.php/salud/article/ viewArticle/4221/5876
- Torres E, Torres LG, Burciaga AS. Síndrome de Klippel-Trenaunay y
- embarazo. Comunicación de un caso. Ginecol Obstet Mex, 2010;78(5):287-290. https://www.medigraphic.com/cgi-bin/new/resumen.cgi?IDARTICULO=27245
- Chenbhanich J, Leelayuwatanakul N, Phowthongkum P. Klippel-Trenaunay-Weber syndrome as a cause of chronic thromboembolic pulmonary hypertension, BMJ Case Rep. 2018 Mar 22;2018. pii: bcr-2018-224621. https://
- pubmed.ncbi.nlm.nih.gov/29572375/
- Méndez TJ, Otero IC, García R, Pérez B. Síndrome de Klippel- Trenaunay-Weber: presentación de un caso. Rev Cubana Oftalmol 2001;14(1):47-52.
- Acacio Y, Gutiérrez M, Morantes J, Roye R, Zamora M, Fernández V, Robles
- Y. Angioqueratoma asociado a Síndrome de Klippel-Trénaunay: a propósito de un caso. Dermatología Venezolana. 2006;44 (3):22-26.
- Corder B, Fishman SJ, Fox VL, Greene AK, Han L. Clinical Practice Guidelines for Klippel-Trenaunay Syndrome, Boston Children's Hospital KTS Working Group Vascular Anomalies Center. Updated 1.6.2016. https://k-t.org/assets/images/content/BCH-Klippel-Trenaunay-Syndrome-Management-Guidelines-1-6-2016.pdf
- Gontero R, Ortiz A, Roverano S, Paira S. Síndrome Klippel-Trenaunay: comunicación de dos casos. Rev Arg Reumatol. 2017;28(1):42-47. https:// pesquisa.bvsalud.org/portal/resource/pt/biblio-958077
- Boló H, Ortiz LM, Samudio GC, Ruiz J. Síndrome de Klippel-Trenaunay asociado a litiasis renal. Reporte de un caso pediátrico y revisión de la literatura. Pediatr. (Asunción), 2013;40(3):247-251. https://www.revistaspp.org/index.php/pediatria/article/view/117
- Vallés J, Roy V. Síndrome de Klippel-Trenaunay y analgesia epidural Obstétrica. Rev Argent Anestesiol. 2016;74(2):76-79.
- Nápoles D, Rodríguez LY, Nápoles D. Síndrome de Klippel-Trenaunay-Weber en una gestante. Medisan 2016;20(1):77-83.
- De la Paz S, Rojas L, Remond RH. Neurofibromatosis tipo I y Síndrome de Klippel-Trenaunay. Revista Electrónica Dr. Zoilo E. Marinello Vidaurreta, 2016;41(10). http://www.revzoilomarinello.sld.cu/index.php/zmv/article/view/854
- Moguel MA, Pérez FJ, Morgado I, Morales J, Torres LM. Analgesia epidural para el parto en gestante con síndrome de Klippel-Trenaunay.Rev. Soc. Esp. Dolor, 2005;12 (3):181-182. http://scielo.isciii.es/scielo.php? script=sci_arttext&pid=S1134-80462005000300007
References
Estrada MM, Guerrero AGML, Enríquez GR, González de la CJ. Síndrome de Klippel Trenaunay. Hallazgos clínicos y de imagen. An. Radiol. Méx. 2006;5(3):245-251.
Velázquez Chacón W, Matos Batista Y. Enfoque Interdisciplinario en la Gestante con Síndrome de Klippel-Trenaunay-Weber. Revista Científica Hallazgos21, 2016;1(1):1-9 https://revistas.pucese.edu.ec/hallazgos21/article/view/10
Pérez M, Hernández H, Vázquez LA. Síndrome de Klippel-Trenaunay-Weber complicado con trombosis venosa cerebral. CorSalud, 2016;8(4):265-269. https://www.medigraphic.com/cgi-bin/new/resumen.cgi?IDARTICULO=73879
Lindenauer SM. The klippel-trenaunay syndrome: varicosity, hypertrophy and hemangioma with no arteriovenous fistula. Ann Surg. 1965;162(2):303-14.
Clasificación Internacional de las Enfermedades. Disponible en: https://eciemaps.mscbs.gob.es/ecieMaps/browser/index_10_mc.htm
Angiogenic factor with G-patch and FHA domains 1; AGGF1. Disponible en: https://www.omim.org/entry/608464
Barajas TJ, Delgado EG, Urióstegui LC, López V, Luna U. Síndrome de Klippel Trenaunay. Rev Cubana Med Gen Integr, 2016;32(3):1-7. http://scielo.sld.cu/scielo.php script=sci_abstract&pid=S0864-21252016000300020&lng=es&nrm=iso
Ceballos-Quintal JM, Pinto-Escalante D, Castillo-Zapata I. A new case of Klippel-Trenaunay-Weber (KTW) syndrome: evidence of autosomal dominant inheritance. Am J Med Genet. 1996;63:426–427. https://pubmed.ncbi.nlm.nih.gov/8737646/
Lorda-Sánchez I, Prieto L, Rodríguez-Pinilla E, Martínez-Frías ML. Increased parental age and number of pregnancies in Klippel-Trenaunay-Weber syndrome. Ann Hum Genet. 1998;62:235–239. https://pubmed.ncbi.nlm.nih.gov/9803268/
Whelan AJ, Watson MS, Porter FD, Steiner RD. Klippel-Trenaunay-Weber syndrome associated with a 5:11 balanced translocation. Am J Med Genet. 1995;59:492–494. https://pubmed.ncbi.nlm.nih.gov/8585570/
Wang Q, Timur AA, Szafranski P, Sadgephour A, Jurecic V, Cowell J, et al. Identification and molecular characterization of de novo translocation t(8;14) (q22.3;q13) associated with a vascular and tissue overgrowth syndrome. Cytogenet Cell Genet. 2001;95:183–188. https://
pubmed.ncbi.nlm.nih.gov/12063397/
Timur AA, Sadgephour A, Graf M, Schwartz S, Libby ED, Driscoll DJ, et al. Identification and molecular characterization of a de novo supernumerary ring chromosome 18 in a patient with Klippel-Trenaunay syndrome. Ann Hum Genet. 2004;68:353–361. https://pubmed.ncbi.nlm.nih.gov/15225160/
González E, Blasco M, Andérica J. Klippel-Trenaunay syndrome complicating pregnancy. BMJ Case Reports. 2012:1-4. https://pubmed.ncbi.nlm.nih.gov/22854239/
Redondo SCA, Yaber GY, Gutiérrez DR, Garavito GP, Charris M, Sogamoso H, Cantillo Z, Ordóñez HJ, Parra G, Denubilla E. Síndrome de Klippel Trenaunay Weber y asplenia. Revista Científica Salud Uninorte, 1998;13(1):31-33. http://rcientificas.uninorte.edu.co/index.php/salud/article/ viewArticle/4221/5876
Torres E, Torres LG, Burciaga AS. Síndrome de Klippel-Trenaunay y
embarazo. Comunicación de un caso. Ginecol Obstet Mex, 2010;78(5):287-290. https://www.medigraphic.com/cgi-bin/new/resumen.cgi?IDARTICULO=27245
Chenbhanich J, Leelayuwatanakul N, Phowthongkum P. Klippel-Trenaunay-Weber syndrome as a cause of chronic thromboembolic pulmonary hypertension, BMJ Case Rep. 2018 Mar 22;2018. pii: bcr-2018-224621. https://
pubmed.ncbi.nlm.nih.gov/29572375/
Méndez TJ, Otero IC, García R, Pérez B. Síndrome de Klippel- Trenaunay-Weber: presentación de un caso. Rev Cubana Oftalmol 2001;14(1):47-52.
Acacio Y, Gutiérrez M, Morantes J, Roye R, Zamora M, Fernández V, Robles
Y. Angioqueratoma asociado a Síndrome de Klippel-Trénaunay: a propósito de un caso. Dermatología Venezolana. 2006;44 (3):22-26.
Corder B, Fishman SJ, Fox VL, Greene AK, Han L. Clinical Practice Guidelines for Klippel-Trenaunay Syndrome, Boston Children's Hospital KTS Working Group Vascular Anomalies Center. Updated 1.6.2016. https://k-t.org/assets/images/content/BCH-Klippel-Trenaunay-Syndrome-Management-Guidelines-1-6-2016.pdf
Gontero R, Ortiz A, Roverano S, Paira S. Síndrome Klippel-Trenaunay: comunicación de dos casos. Rev Arg Reumatol. 2017;28(1):42-47. https:// pesquisa.bvsalud.org/portal/resource/pt/biblio-958077
Boló H, Ortiz LM, Samudio GC, Ruiz J. Síndrome de Klippel-Trenaunay asociado a litiasis renal. Reporte de un caso pediátrico y revisión de la literatura. Pediatr. (Asunción), 2013;40(3):247-251. https://www.revistaspp.org/index.php/pediatria/article/view/117
Vallés J, Roy V. Síndrome de Klippel-Trenaunay y analgesia epidural Obstétrica. Rev Argent Anestesiol. 2016;74(2):76-79.
Nápoles D, Rodríguez LY, Nápoles D. Síndrome de Klippel-Trenaunay-Weber en una gestante. Medisan 2016;20(1):77-83.
De la Paz S, Rojas L, Remond RH. Neurofibromatosis tipo I y Síndrome de Klippel-Trenaunay. Revista Electrónica Dr. Zoilo E. Marinello Vidaurreta, 2016;41(10). http://www.revzoilomarinello.sld.cu/index.php/zmv/article/view/854
Moguel MA, Pérez FJ, Morgado I, Morales J, Torres LM. Analgesia epidural para el parto en gestante con síndrome de Klippel-Trenaunay.Rev. Soc. Esp. Dolor, 2005;12 (3):181-182. http://scielo.isciii.es/scielo.php? script=sci_arttext&pid=S1134-80462005000300007