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2020, Number 1

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Finlay 2020; 10 (1)

Complete Morris syndrome. Case presentation

Brito VM, Brito GÁB, Batista GD
Full text How to cite this article

Language: Spanish
References: 10
Page: 56-61
PDF size: 191.97 Kb.


Key words:

sexual differentiation, inguinal hernia, case presentation.

ABSTRACT

Androgen insensitivity syndrome, Morris syndrome or testicular feminization is a disorder in sexual differentiation, in which the individual is phenotypically feminine, but with a man's genetic characteristics. The case of a 9-year-old schoolgirl with bilateral inguinal hernia is presented as a form of presentation of Morris syndrome. It is assessed in consultation confirming the presence of testicles in the surgery, corroborated by pathological anatomy. A karyotype was described describing chromosomally 46XY. In prepubertal age it is reevaluated with bilateral inguinal hernias and external genitals of female appearance. It is verified by imaging examinations, the absence of uterine body and annexes, with presence of permeable vagina until its middle third, which ends in a blind sac inside the pelvis, performing orchiectomy by minimal access without vaginoplasty. Bilateral inguinal hernia in childhood was the form of presentation of Morris syndrome in this patient. The case is presented due to the low frequency of occurrence of this condition.


REFERENCES

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  2. Souhail R, Amine S, Nadia A, Tarik K, Khalid EK, Abdellatif K, et al. Complete androgen insensitivity syndrome or testicular feminization: review of literature based on a case report. Pan Afr Med J. 2016;25(1):199

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  4. CemiK, Serdar S, Gonul C, Husseyin O, Bumin N. A Novel Mutation in Human Androgen Receptor Gene Causing Partial Androgen Insensitivity Syndrome in a Patient Presenting with Gynecomastia at Puberty. J Clin Res Pediatr Endocrinol. 2016;8(2):232-5

  5. Batista Rl, Costa EMF, Rodríguez AS, Gómez NL, Faria Jr, Nishi MY, et al. Androgen insensitivity syndrome: a review. Arch Endocrinol Metab. 2018;62(2):227-35

  6. Touzon MS, Pérez N, Marino R, Ramirez P, Constanzo M, Guercio G, et al. Androgen Insensitivity Syndrome: Clinical Phenotype and Molecular Analysis in a Single Tertiary Center Cohort. J Clin Res Pediatr Endocrinol. 2019;11(1):24-33

  7. Baca JE, Leuchter IJ, Domínguez CLG, Mayagoitia GJC. Testicular torsion in the inguinal canal with recurrent hernia, in a patient with complete androgenic insensitivity syndrome («feminizing testicle»). Rev Mex Cir Endosc. 2018;19(2):82-6

  8. Borrego JA, Varona JA, Areces G, Formoso LE. Síndrome de Morris. Rev Cubana Obst Ginecol [revista en Internet]. 2012 [citado 17 Mar 2018];38(3):[aprox. 10p]. Disponible en: http://scielo.sld.cu/scielo.php?script=sci_abstract &pid=S0138-600X2012000300014&lng=es&nrm =iso

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  10. Samy AM. Androgen insensitivity syndrome with inguinal testes: MRI diagnosis. Egyp J Radiol Nuc Med. 2016;47(1):607-9




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