N.R. AKRAMOV^1,2, M.R. SHAYDULLINA^1,2, A.I. GALLYAMOVA²

¹Children’s Republican Clinical Hospital, 140 Orenburgskiy Trakt, Kazan, Russian Federation, 420138

²Kazan State Medical University, 49 Butlerov Str., Kazan, Russian Federation, 420012

Akramov N.R. — D. Med. Sc., Professor, doctor of Urology Department, Professor of Pediatric Surgery Department, tel. +7-917-253-77-20, e-mail: aknail@rambler.ru

Shaydullina M.R. — PhD (Medicine), doctor of the Endocrinology Department, Assistant Lecturer of the Endocrinology Department, tel. +7-960-045-67-79, e-mail: zizi97@mail.ru

Galliamova A.I. — post-graduate student of the Department of Pediatric Surgery, tel. +7-960-040-68-58, e-mail: abachtiarova@yandex.ru

The paper presents a rare case of diagnosis and surgical treatment of children aged 1 year to 46-XX-male syndrome (de la Chapelle syndrome). Feasibility of a team approach is demonstrated when a combination of urogenital diseases with the pathology of processus vaginalis of peritoneum (inguinal hernias, hydrocele), cryptorchidism and developmental defects of the penis is detected. The authors believe this will prevent the unreasonable manipulation on children with rare anomalies. The gonadectomy with single-trocar laparoscopic access and simultaneous laparoscopic extraperitoneal herniorrhaphy, conducted by the authors, has confirmed its advantages over classical surgical methods.

Key words: 46-XX-male syndrome, de la Chapelle syndrome, children, diagnosis and surgical treatment.

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