Sirenomelia is extremely rare congenital malformation that characterized by the complete or incomplete fusion of the low extremities and associated by the various organs’ disorders, such as urinary system, which determined the poor outcome in these infants that died during antenatal or early neonatal period. Prenatal ultrasonography, especially during first-second trimester, permits to establish the presence of sirenomelia. Due to the rarity of this malformation, the own observation of first case of sirenomelia in one infant from twins is presented, which borne in Ukraine. Diagnosis of sirenomelia was established at the planned ultrasonographic screening of multigravida mother. After the birth, neonate with sirenomelia was transferred at the intensive care unit of Lviv regional children’s clinical hospital «OXMATDYT». At the first examination, the throughout fusion of lower extremities, the absent of one kidney, bladder, and external genitalia was revealed. With increasing signs of renal and respiratory failure baby died at 9 days after birth. Conclusions. Sirenomelia is extremely rare congenital malformation that associated with the high mortality during early neonatal period due to the presence of underlying congenital malformations, such as urinary system. Ultrasonography during the prenatal period is the screening methods for the diagnosis of this malformation. No conflict of interest was declared by the authors. Key words: sirenomelia, diagnostic, twins.
The diagnosis of adnexal torsion is challenging due to the absence of specific symptoms that can determine the postponing of surgery, and, as the consequence, the development of necrotic changes at adnexa. Today, the surgical treatment of patients with this pathology shifting to the ovary-sparing surgery (detorsion) despite to the presence of necrotic changes during surgery. By that, the question of the choice of the method of treatment in case of adnexal torsion still under debate between pediatric surgeons and gynecologists. Purpose - to summarize the own experience in the treatment of patients with adnexal torsion. Materials and methods. This study based on the results of the treatment of 73 patients with adnexal torsion, which were operated at 2010-2022 years. Diagnosis was established on the results of clinical investigation and ultrasonographic (US) data. It was performed 74 surgical procedures (one patient was operated on twice), among them 57 (77%) by laparoscopic and 17 (23%) open approach (laparotomy). It was 49 (66.2%) ovary-sparing procedures (detorsion with or without cystectomy) and 25 (33.8%) - adnexectomy. Results of the study were evaluated by the statistical program StatPlus: mac, AnalystSoft Inc. (version v8). Results. Mean age of patients was 11.50.5 years. Abdominal pain syndrome (in 100%) and vomiting (in 49.3%) were the main clinical appearances. Unilateral ovaria enlargement and peripheral displacement of follicles were the most frequent US findings. The absence of the blood flow was revealed in 39.5% and the whirlpool sign in 11.6% of patients. Among of all patients, simple torsion confirmed in 24.3%, while the torsion caused by cyst or dermoid tumors, revealed in 75.7% of patients. Detorsion only was performed in 24.3% of patients and in 42.5% of patients it was supplemented by cystectomy. Adnexectomy was performed in 32.2% of patients. Conclusions. The diagnosis of adnexal torsion should be considered in all females with acute abdominal pain syndrome. Ultrasonography with or without Doppler is the first-line imaging modality. Laparoscopic detorsion, simple or supplemented by cystectomy, is the method of choice in the treatment of patients with adnexal torsion. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of all participating institutions. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.
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