Toledo LM, Tellez GAL, Licourt OD

Language: Spanish
References: 10
Page: 148-154
PDF size: 326.39 Kb.

ABSTRACT

Introduction: Ivemark Syndrome is a rare congenital condition that affects multiple organs of the body; it is classified as a disorder of heterotaxia or laterally disorder. It is characterized by anomalies of the spleen and other abdominal organs as well as malformations of the heart.
Case report: a 15-month-old boy who underwent fetal echocardiography in the 28th week of his fetal development, in the left heart cavity an axis of more than 650 with increase of right cavities, at the aortic valve level a slight decrease in its caliber was observed. Diagnostic impressions are presented as inverse situs, interruption of inferior vena cava and continuation with azygos vein, aortic valvular stenosis and left isomerism. Cesarean delivery was performed at 39 weeks of pregnancy, weight 5080 gr, apgar scoring 9-9. In the abdominal ultrasound the spleen was not defined, the right kidney was malrotated observing also intestinal malrotation. Ivemark Syndrome was the definitive diagnosis. The infant is periodically monitored by a multidisciplinary medical team.
Conclusions: at present, it is possible to formulate an early diagnosis of the cardinal signs that involve internal organs such as cardiovascular system, liver, and spleen during prenatal stage to confirm the Ivemark Syndrome, which contributes to the genetic counseling and the multidisciplinary approach in the neonatal stage, avoiding fatal complications and therefore to increase in quality and life expectancy.

REFERENCES

1. Aljure Reales VJ, Álvarez Gallego GC, Ávila Espitia NC, Arrieta Coley A, Ángel Suárez OG. Situs inversus totalis: revisión de tema con aproximación a la Genética y reporte de casos. Rev Colomb Cardiol [Internet]. 2017 [citado 17 Sep 2018]; 24(1): 40-47. Disponible en: https://www.sciencedirect.com/science/article/pii/S0120563316301929.

2. Jacobs JP, Anderson RH, Weinberg PM, Walters HL, Tchervenkov CI, et al. The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy. Cardiol Young [Internet]. 2007 [citado 17 Sep 2018]; 17(2): 1-28. Disponible en: https://www.cambridge.org/core/journals/cardiology-in-the-young/article/nomenclature-definitionand- classification-of-cardiac-structures-in-the-setting-ofheterotaxy/ 294F737F1C3BDA127BDBC8FBA9525E68/core-reader

3. Forenza N, Albuquerque H, Vallejo S, Muzzo G. Síndrome de Ivemark: vólvulo gástrico asociado con aplenia. Rev de Cir Infantil [Internet]. 2001 [citado 18 Oct 2018]; 11(4): 243-245. Disponible en: http://www.acacip.org.ar/revista/110411.pdf.

4. Florescu R, Radulescu A, Ghita A, Acs B, Kozma A, et all. Numerical abnormalities of the spleen: immunohematological and anatomic consequences. Proc. Rom. Acad [Internet]. 2016 [citado 20 Oct 2018]; 18(3): 223-226. Disponible en: https://papers.ssrn.com/sol3/papers.cfm?abstract_id=3005511

5. Hrusca A, Rachisan Al, Lucian B, Oprita S, Manole S, Cainap S. Ivemark syndrome-a rare entity with specific anatomical features. Rev Med Chile [Internet]. 2015 [citado 20 Oct 2018]; 143:383-386. Disponible en: http://dx.doi.org/10.4067/S0034-98872015000300014

6. EL Fane M, Sodqi M, Marih L, Chakib A, Marhoum K. Congenital Asplenia Revealed by Streptococcus oralis septicemia: Case Report. J Antimicrob Agents [Internet]. 2017 [citado 20 Oct 2018]; 3(2): 1-3. Disponible en: https://www.researchgate.net/profile/M_Fane/publication/318391042_Congenital_Asplenia_Revealed_ by_Streptococcus_oralis_septicemia_Case_Report/links/59bfc0520f7e9b48a29b85bc/Congenital- Asplenia-Revealed-by-Streptococcus-oralis-septicemia-Case-Report.pdf.

7. Masiwal P, Chenthil KS, Priyadarsini B, Gnanaprakasam J, Srihari I. Ivemark Syndrome. Journal of The Association of Physicians of India [Internet]. 2016 [citado 20 Oct 2018]; 64: 73-75. Disponible en: http://www.japi.org/may_2016/16_cr_ivemark_syndrome.pdf.

8. Kutsal A, Koc M. Ivemark syndrome with cor triatriatum, primum ASD, cleft mitrale and pulmonary stenosis. EJCM [Internet]. 2015 [citado 20 Oct 2018]; 3(1): 11-14. Disponible en: http://www.ejcvsmed.org/articles/case-report/ivemark-syndrome-cor-triatriatum-primum-asd-cleftmitrale- pulmonary-stenosis/.

9. Jain D, Chavan B, Manoj A. Syndrome of Right Isomerism: Ivemark Syndrome. Journal of Mahatma Gandhi Institute of Medical Sciences [Internet]. 2018 [citado 20 Oct 2018]; 23(2): 92-94. Disponible en: http://www.jmgims.co.in/article.asp?issn=0971- 9903;year=2018;volume=23;issue=2;spage=92;epage=94;aulast=Jain

10. Gort Hernández M, Cayetano Choque C. Situs inversus totalis en hospital boliviano español Patacamaya, Bolivia. Rev. Ciencias Médicas de Pinar del Río [Internet]. 2016 [citado 20 Oct 2018]; 20(3): 364-368. Disponible en: http://scielo.sld.cu/scielo.php?pid=S1561- 31942016000300016&script=sci_arttext&tlng=en.