RESUMEN
BACKGROUND: Complete 46XY gonadal dysgenesis (Swyer syndrome) is a rare and challenging diagnosis among prepubertal girls, as estrogen insufficiency becomes evident only during adolescence, with nonspecific symptoms such as primary amenorrhea and/or delayed puberty. Unfortunately, girls with Swyer syndrome are at high risk for malignancies in the dysgenetic gonads, which can be prevented only by performing prophylactic bilateral gonadectomy. CASE: We present a 9-year-old patient with Swyer syndrome diagnosed with dysgerminoma in the right gonad and gonadoblastoma in the left gonad after prophylactic bilateral gonadectomy. SUMMARY AND CONCLUSION: Concerning the high risk of early gonadoblastoma and its malignant transformation, we recommend performing prophylactic bilateral gonadectomy at the time of diagnosis, even if the patient is prepubertal.
Asunto(s)
Disgerminoma/genética , Disgenesia Gonadal 46 XY/complicaciones , Gonadoblastoma/genética , Neoplasias Ováricas/genética , Castración , Niño , Disgerminoma/patología , Disgerminoma/prevención & control , Femenino , Disgenesia Gonadal 46 XY/diagnóstico , Disgenesia Gonadal 46 XY/cirugía , Gonadoblastoma/patología , Gonadoblastoma/prevención & control , Humanos , Neoplasias Ováricas/patología , Neoplasias Ováricas/prevención & control , Procedimientos Quirúrgicos ProfilácticosRESUMEN
BACKGROUND: The aim of this study was to evaluate the efficiency of the preservation of ovarian tissue in cases of ovarian torsion. MATERIALS AND METHODS: A retrospective study was performed of patients treated at our hospital for ovarian torsion from January 2007 to December 2017. This research does not include patients with antenatal ovarian torsion and 1 girl with an immature teratoma, in whom the twisted ovary was removed during the initial operation. Follow-up ultrasonography of all patients was performed after 4-6â¯weeks and again after more than 12â¯weeks. Volume, blood flow and folliculogenesis of the ovary were measured and assessed. RESULTS: All 42 ovaries (39 patients) preserved their normal anatomy and folliculogenesis after detorsion. All patients had an enlarged ovary at the time of detorsion. In all cases of ovarian torsion, enlargement of the ovary up to an average of 58.14⯱â¯52.86 (17.37-86.83) ml was detected. After 4-6â¯weeks, all untwisted ovaries decreased in volume by an average of 9.01⯱â¯13.69 (2.33-9.30) times, and 59.5% of them became normal in size. In 3 girls, enlarged ovaries were still observed after 12â¯weeks. Teratoma was diagnosed for these patients and ovarian sparing operations were performed. CONCLUSIONS: It is safe to perform detorsion regardless of the level of ischemia or volume of the affected ovary. The follow-up is essential, especially for the further diagnostics of potential pathological structures or tumors; therefore, the normalization of blood flow and the volume of the ovary must be monitored. TYPE OF STUDY: Prognosis retrospective study. LEVEL OF EVIDENCE: II.
Asunto(s)
Neoplasias Ováricas/diagnóstico , Ovario/diagnóstico por imagen , Ovario/patología , Teratoma/diagnóstico , Anomalía Torsional/cirugía , Adolescente , Niño , Preescolar , Femenino , Preservación de la Fertilidad , Estudios de Seguimiento , Humanos , Lactante , Tamaño de los Órganos , Tratamientos Conservadores del Órgano , Neoplasias Ováricas/cirugía , Ovario/irrigación sanguínea , Ovario/fisiología , Embarazo , Pronóstico , Estudios Retrospectivos , Teratoma/cirugía , Anomalía Torsional/patología , UltrasonografíaRESUMEN
BACKGROUND: The purpose of this study is to evaluate the clinical symptoms, diagnosis, management, and outcomes in children with ovarian torsion. METHODS: The charts of 50 patients with 53 cases of ovarian torsion treated between January 1989 and March 2012 were reviewed retrospectively. Long term follow up was available for 20 girls who had their ovaries left in the abdominal cavity after detorsion. RESULTS: In 22 cases ovaries were removed, and in 31 cases the torsion was relieved and the ovaries left in the abdominal cavity. Twenty-five of the salvaged ovaries were black-bluish and 10 bluish in color. Since 2005, after a change in preferred treatment, all ovaries treated by detorsion were left in the abdominal cavity. The long term results were observed clinically and by ultrasound in 20 girls. Multifollicular ovaries were found in 17 girls. One girl had a normal size paucifollicular ovary, a one-year-old girl had a normal size ovary with microfollicles, and one girl had no ovarian material detectable by ultrasound. CONCLUSIONS: Long term analysis of the treatment of ovarian torsion revealed that ovaries treated by detorsion and left in the abdominal cavity preserved their normal anatomy and function. Conservative surgical treatment proved to be safe. None of the girls had thromboembolism or peritonitis, and no malignant tumors were found in the operated ovaries.