Syndrome of complete insensitivity to androgens of the 4-years-old child

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Abstract

To demonstrate an interesting and rare case in the clinical practice of testicular feminization syndrome in a child, as an accidental finding when performing a planned surgical intervention. A mother with a child E., born in 2010 (at the time the child was 4 years old) was hospitalized with the mother’s complaints about the presence of protrusions in both inguinal areas from birth, the mother denied cases of infringement. The child was examined on an outpatient basis and the next day a scheduled surgical intervention was prescribed — hernia repair on both sides. At the opening of the hernial sac on the right, the contents were the testicle, 1.6 × 1.6 × 1.0 cm in size, with an appendage and vas deferens. Then an opening of the hernial sac was performed on the left, the contents of the hernial sac was also a testicle measuring 1.8 × 1.2 × 1.0 cm with an appendage and d. deference. Examination was performed: on an ultrasound of the pelvic organs — the bladder is full, the effusion in the pelvis is up to 12–15 mm high, the uterus and ovaries are not located at the time of the examination. In the abdominal cavity (in the iliac regions), ovarian testicles with mediastinum are located on both sides — 19 × 11 mm on the right, 16 × 10 mm on the left. The clinical case that we have provided will be of interest to pediatric surgeons and geneticists in view of the unusual and complex clinical diagnosis of this condition. In the presence of a female phenotype, the child completely lacked the organs of the female reproductive system, this disease was detected only due to the presence of a concomitant pathology in the child, a bilateral inguinal hernia. The early diagnosis of STF is especially important given the risk of malignancy of the gonads in the post-pubertal period.

About the authors

Vasily P. Gavrilyuk

Kursk State Medical University

Email: wvas@mail.ru
ORCID iD: 0000-0003-4792-1862

Dr. Sci. (Med.), associate professor, the head of the department of pediatric surgery and pediatrics of faculty of postdegree education

Russian Federation, Kursk

Stanislav V. Kostin

Kursk State Medical University; Kursk Regional Children’s Hospital No. 2

Email: drkostin@inbox.ru
ORCID iD: 0000-0003-0857-6437

Сand. Sci. (Med.), associate professor, the associate professor of the department of pediatric surgery and pediatrics of faculty of postdegree education; the head of the department of pediatric’s surgery

Russian Federation, Kursk

Maria I. Statina

Kursk State Medical University

Email: mari.statina.94@mail.ru
ORCID iD: 0000-0001-9918-9306

lecture assistant of the department of pediatric surgery and pediatrics of faculty of postdegree education

Russian Federation, Kursk

Dmitry A. Severinov

Kursk State Medical University

Author for correspondence.
Email: dmitriy.severinov.93@mail.ru
ORCID iD: 0000-0003-4460-1353

lecture assistant of the department of pediatric surgery and pediatrics of faculty of postdegree education

Russian Federation, Kursk

Elena O. Ageeva

Kursk Regional Children’s Hospital No. 2

Email: elenrao@mail.ru
ORCID iD: 0000-0001-9918-9306

anesthesiologist-resuscitologist

Russian Federation, Kursk

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Supplementary files

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2. Fig. 1. Testicle in hernia sac on the right side

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3. Fig. 2. Testicle in hernia sac on the left side

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4. Fig. 3. Ultrasonography examination of the pelvic organs (sensor located on right (a) and left (b) in inguinal areas)

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