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Introduction. Neonatal ovarian cysts develop in case of hormonal imbalance in the mother-placenta-fetus system. Cystic transformation in the ovary may cause appendage torsion which leads to follicular necrosis and loss of ovarian reserve. Most often, torsion occurs in the utero, but in premature girls- due to the specific hormonal status - the risk of cyst growth and its torsion remains in the postnatal period. Currently, a unified approach to the surgical treatment of neonatal ovarian cysts is absent.Material and methods. In the department of pediatric surgery for malformations in the Perinatal Center of the Amazov National Medical Research Center, 34 girls with ovarian cysts were examined during 2012-2020; 9 of them (27%) were premature. In the presented observation, we faced an ovarian cyst in the fetus of 30 week gestation.Results. The cyst looked uncomplicated, but had the enormous size, so we discussed a possibility to perform an intrauterine puncture. However, due to severe hemolytic disease of the fetus and premature delivery, the intervention was not carried out. By the third week of life, torsion of the cystic-transformed ovary developed; necrosis and self-amputation of the right uterine appendage were revealed intraoperatively. By the age of three months, cystic transformation of the only ovary developed. Timely performed laparoscopic fenestration was organ-sparing. Further follow-up revealed preserved and normally growing single ovary what confirmed the right choice of surgical tactics.Conclusion. Dynamic ultrasound examination of the pelvic organs is indicated to all premature girls, at least once every two weeks (in case of revealed ovarian cyst - weekly). We consider it reasonable to make the laparoscopic fenestration of uncomplicated cysts that have size of 3 cm and more. Newborn girls with ovarian cysts should be under the joint control of pediatrician and pediatric gynecologist for developing an individual follow-up plan.

Introduction. Penile agenesis (aphallia) is an extremely rare congenital defect characterized by the complete absence of the penis in a child with a male karyotype 46XY.Material. The analysis of the literature on keywords in the Pubmed and Medline databases was carried out.Results. The incidence of aphallia is estimated 1 out of 10-40 million newborn boys. Aphallia, in spite of its rarity, is a congenital defect which is thoroughly described in the medical literature. The combination of agenesis of the penis with other congenital anomalies often leads to death in such children. Currently, it is believed that these patients should be left with their genetic male sex, therefore, there is a difficult dilemma of choosing the optimal method and age for creating the neophallus and neourethra.Conclusions. The rarity of the aphallia determines the lack of publications covering the long-term results of phalloplasty in both children and adults.

Introduction. This article describes a clinical case of a pregnant woman with fetal meconium peritonitis and further observation and treatment of the child. Meconium peritonitis, being aseptic intrauterine peritonitis, is a serious disease that requires an integrated approach. Tactics of management the fetus, intrauterine risks and prognosis of survival in this pathology as well as choice of the technique for correcting meconium peritonitis and its complications during pregnancy and after childbirth are discussed.Material and methods. Prenatal ultrasound examination revealed in a male fetus signs of low intestinal obstruction, distortion of bowel loops, calcifications, ascites and polyhydramnios. This case shows that depending on the clinical course of peritonitis the timely performed diagnostics reduces risks of life-threatening complications.Results. Due to the newly developed ultrasound criteria which assess the pathology severity (meconium ascites, compression of the fetal chest cavity, edema and polyhydramnios), it became possible to predict the course of meconium peritonitis. Management of pregnancy, childbirth and neonatal period as well as therapeutic and surgical correction of this severe pathology with further early rehabilitation are described in details.Conclusion. Meconium peritonitis is a pathology in which a timely established prenatal diagnosis is extremely important because it significantly improves prognosis and allows to take adequate steps prenatally for stabilizing the condition of both a fetus and a pregnant woman.

Introduction. Recently, achievements in molecular genetics, imaging techniques (PET/CT), medicamentous therapy as well as in surgical treatment have promoted a better control of hypoglycemia and, consequently, better outcomes in children with congenital hyperinsulinism.Purpose. To specify indications, volume and outcomes of surgical treatment in patients with congenital hyperinsulinism depending on pathology form, differential diagnostics with PET / CT and intraoperative express biopsy.Material and methods. 41 children with congenital hyperinsulinism were operated in the department of pediatric surgery in Almazov National Medical Research Centre (Saint-Petersburg) during 2011 – September 2020. In Group 1, there were 6 children who had standard treatment with subtotal resection of the pancreas (95%). In Group 2, there were 35 patients who had PET tomography with 18-F-DOPA before surgery and intraoperative express biopsy of pancreas tissue. The analyzed patients were operated on in 2017-September 2020. PET tomography with 18-F-DOPA findings revealed that these children had 10 diffuse forms and 22 focal forms; the other 3 children had a disputable picture.Results. After surgery, diffuse form was confirmed in 10 (29%) children; focal form - in 21 (60%) children; 4 (11%) patients had the atypical form which was diagnosed intraoperatively. Of 35 children from Group 2, 33 (94%) had complete hyperinsulinism reversal, significant improvement in their psycho-motor function; however, 10 (29%) of them require insulin replacement therapy with minimal dosages - 8 children with the diffuse form of hyperinsulinism and 2 children with the atypical one. There were no intra- and postoperative complications. 20 (95%) out of 21 children with focal forms recovered completely. The authors also describe problems and peculiarities of urgent histological examination.Conclusion. Thus, partial pancreatectomy in focal forms, subtotal in atypical ones and almost total in diffuse forms allows to cope with hypoglycemia caused by congenital hyperinsulinism.

Introduction. 30-60% of all patients with congenital hyperinsulinism have a delayed psychomotor development, and 15-25% of them have a severe organic brain damage, including epilepsy. The timely diagnostics and intensive care can prevent severe neurological complications. If the conservative therapy is not effective, then the surgical treatment is a need. Objective: To demonstrate features of surgical correction of atypical forms of congenital hyperinsulinism. Material and methods. 11 children with atypical forms of congenital hyperinsulinism were operated on in the department of pediatric surgery in the V.A. Almazov National Medical Center in 2017-03.2019. Results. 2 children demonstrated a complete relief of hyperinsulinism in 9 months (1.5 years) of follow-up; one patient had a significant improvement of his psycho-motor development. Conclusion. Some children with presumably focal forms of hyperinsulinism, by PET-CT findings, may have histologically atypical forms. In the atypical lesion of the pancreas, an intraoperative biopsy dramatically changes the planned volume of pancreatectomy up to almost total removal of the gland.

Introduction. 30-60% of all patients with congenital hyperinsulinism have a delayed psychomotor development, and 15-25% of them have severe organic brain damage, including epilepsy. Timely diagnostics and intensive treatment can prevent severe neurological complications. With the ineffectiveness of conservative therapy, surgical treatment is a necessary alternative. Objective. To define indications for various options of surgical correction of focal forms of congenital hyperinsulinism. Material and Methods. In the Department of Pediatric Surgery of V.A. Almazova National Medical Research Centre, in 2017-03.2019, 14 children with focal forms of congenital hyperinsulinism were operated after analyzing their PET-CT findings; in 2 patients out of them they were doubtful. Results. 13 (93%) patients had a complete relief of hyperinsulinism and a significant improvement in their psycho-motor development. 10 (71%) of 14 children had a complete recovery; 1 (7%) - a significant positive trend. 3 (21%) patients needed insulin therapy with minimal dosages. They all were children with an atypical (mixed adenomatous-diffuse) form of hyperinsulinism. Conclusions. In case of focal lesions in the pancreas, partial pancreatectomy with maximal preservation of healthy tissue, defined by the express biopsy, and with any lesion localization is indicated. For atypical forms, the volume for pancreatectomy dissection is defined individually.

Introduction. Advances in molecular genetics, imaging techniques (PET/CT), medicamentous therapy and surgical treatment in the recent decades have improved hypoglycemia control and , thus, improved treatment outcomes in children with congenital hyperinsulinism. Purpose. To define indications for different techniques of surgical correction in patients with congenital hyperinsulinism depending on the disease form. Material and methods. 23 children were operated on for congenital hyperinsulinism in the department of pediatric surgery in the Almazov Medical Research Center from 2011 till 2018. In 2011-2016, 5 children were operated on by the standard approach which included subtotal pancreas resection (95%). Since 2017, PET tomography with 18-F-DOPA and intraoperative rapid biopsy of the pancreas have been introduced into the curative algorithm. In 2017-2018, 18 children were operated: 6 patients with the diffuse form and 10 - with the focal one. Two more children had controversial outcomes. Results. Fifteen children (83%) had complete hyperinsulinism control and significant improvement in their psycho-motor function. Out of 18 children, 10 (56%) had a complete recovery: among them - 8 (89%) out of 9 children with the focal form of hyperinsulinism and 2 (22%) out of 9 children with the diffuse form. Two children (11%) had a marked positive dynamics. Six patients (33%) needed insulin therapy with minimal dosages - all were children with the diffuse form of hyperinsulinism (6 (67% ) out of 9). Conclusion. Partial pancreatectomy in patients with focal forms or subtotal pancreatectomy in patients with diffuse and atypical forms allow to eliminate hypoglycemia caused by the congenital hyperinsulinism and to prevent damage to the central nervous system in newborns and infants.