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Introduction. Minimally invasive procedures used in the treatment of children with Hirschsprung’s disease (HD) have reduced the rate of complications and allowed to perform these surgeries at any age. In literature, one can find much information on surgical treatment, complications, long-term results. However, features of postoperative period and mechanisms of colon function restorations after different pull-through procedures are not well described. Material and methods. From 2008 to 2019, 77 patients with different forms of HD were operated in Children’s Clinical Specialized Center of High Medical Technologies in St-Petersburg. Patients were aged from 14 days till 3 years. They were divided into 3 groups according to the applied endorectal technique- Soave, «Soavson», Swenson. The following parameters were analyzed: age, sex, stoma, surgery duration, length of hospitalization, features of postoperative period, complications.Results. An early postoperative period was similar in all groups. Two children after Soave procedure (9%) had wound infection; two children had anastomotic stricture (1 – Soave, 1 - «Soavson») which was treated with regular dilatations. Enterocolitis was diagnosed in 19 patients (25%). 20 patients (26%) had no self-defecation for 14 days – 6 months after the surgery. Most of the patients had HD of the rectosigmoid form. These disorders developed more often in patients after Soave and «Soavson» procedures, less often- after Svenson one.Discussion. An issue of choosing an optimal technique for rectal dissection is still a debatable one. There has been no any significant difference revealed between the three variants of endorectal dissection during an early postoperative period.Conclusion. A transanal endorectal pull-through procedure in children with HD is a preferable option of choice. It gives good functional outcomes. Features of the postoperative period are caused by the adaptation of new intestine to new defecation conditions.

BACKGROUND: Extrathoracic lung sequestration is a rare variant of a developmental malformation that can be diagnosed prenatally and should be included in the differential diagnosis of abdominal tumor-like formations. CASES REPORT: From 1996 to 2020, 70 children with confirmed lung sequestration were treated at the Children's City Hospital No. 1 in St. Petersburg. Of these, 29 had intralobar sequestration, and 41 had extralobar sequestration. Four of these patients were diagnosed with a rare extrathoracic (intraabdominal) form. All four were born full-term without clinical manifestations of the defect. Ultrasound examination of all children revealed a solid formation up to 3 cm in diameter, located in the upper left quadrant of the abdominal cavity or retroperitoneal space in the projection of the left adrenal gland. Due to the location of the formation and the inability to visualize the aberrant arterial vessel during ultrasound examination, the first two patients were differentially diagnosed with an adrenal tumor (neuroblastoma). The cancer markers were negative, and adrenal cortex hormones were normal. The children underwent multispiral computed tomography-angiography to clarify the diagnosis. In both cases, the diagnosis of intraabdominal extrapulmonary sequestration with a feeding arterial vessel extending from the thoracic aorta was confirmed. Two children, previously treated in the last two years, were diagnosed by the ultrasound examination results that enabled the visualization of an aberrant arterial vessel feeding the sequester. Indications for surgical treatment have been set. The parents of one child refused treatment. Three children underwent sequestration removal (two by laparoscopic access, one had a laparotomy). Histologically, extrathoracic sequestration in combination with cystoadenomatosis was confirmed. The postoperative period proceeded without complications. RESULTS: Long-term treatment results were observed in all operated patients for a period of four to 10 years. The assessment was performed based on complaints and ultrasound data. All patients matured and developed according to their ages. CONCLUSIONS: Extrathoracic lung sequestration is a rare type of defect that is subject to surgical treatment. This pathology should be included on the spectrum of differential diagnosis of tumorous formations of the abdominal cavity and retroperitoneal space in newborns. The main methods for postnatal diagnosis of the defect are ultrasound and multispiral computed tomography-angiography.

Background. The cystic form of biliary atresia is a rare form of atresia of the biliary tract, which is a relatively favorable variant of the defect and can be diagnosed antenatally. In practice, it is important not only to suspect this diagnosis, but also to differentiate this variant of impaired development of the external bile duct from the cyst of the common bile duct. This is due to the difference in approaches and methods of surgical treatment of choledochal cysts and biliary atresia. Obliteration (atresia) of the bile ducts in the absence of timely surgical intervention quickly leads to the progression of cirrhosis and the development of liver failure. The method of choice in the treatment of AD is Kasai surgery, often palliative in nature, but allowing to delay the time until liver transplantation. The cyst of the common bile duct rarely requires early surgical treatment, and the risk of cirrhosis is significantly lower. Surgical intervention is aimed at removing the cyst and restoring the flow of bile by anastomosing the external bile ducts with the intestines, which is a radical method of treatment and leads to the recovery of the child. External similarity in ultrasound examination of the fetus and newborn baby of the cystic form of biliary atresia of the bile ducts with a cyst of the common bile duct does not always allow differentiation of one defect from another, which can lead to untimely correction of the defect and an unfavorable outcome. Aim. Demonstrate a rare type of biliary atresia. Materials and methods. Between 2001 and 2019, 33 patients with biliary atresia were treated in the Childrens City Multidisciplinary Clinical Specialized Center for High Medical Technologies in St. Petersburg, only two patients had a cystic form. Both children were initially treated as patients with bile duct cyst. Children were operated on at the age of 2 and 3.5 months. The first patient underwent surgery Kasai, the second hepaticoyunoanastomosis. Results. During the observation period (9 years and 4 years), the synthetic function of the liver is normal, and there are currently no indications for transplantation. Conclusion. If a fetus or a newborn with neonatal jaundice is detected during ultrasound examination of a cystic formation in the gates of the liver, it is very important to correctly and quickly make a differential diagnosis between the cystic form of biliary atresia of the biliary tract and the common bile duct cyst.

Introduction. Cysts of the common bile duct or choledoch cysts (CC) are congenital cystic dilatation of the extrahepatic and / or intrahepatic bile ducts. The etiology is not fully clear, but it has been proven that abnormal pancreatobiliary anastomosis plays a major role in CC formation. Clinical manifestations range from prolonged jaundice in newborns to non-specific abdominal pains in older patients. Purpose. To study clinical manifestations of common bile duct cysts in children of different age, to define diagnostic criteria as well as to analyze outcomes after surgical treatment. Material and methods. In 2001-2019, 27 patients with chronic obstructive CC, aged from several days to 12 y.o., were treated in the Children’s City Multidisciplinary Clinical Specialized Center for High Medical Technologies of St. Petersburg. Most of them (16-59.2%) had CC of type IA by the Todani classification. Ultrasound examination and MRI cholangiography were used for diagnostics. Children with CC of type V (Caroli disease) required a specific approach to their treatment because of the combined malformations in kidneys (polycystic kidney disease); in 19 patients with CC of types I and IV the cystically altered choledoch was resected and hepaticojejunostomy was put on the loop by the P technique. Results. 19 patients were operated on. Good outcomes were in 15 cases. Underlying disease complications in the preoperative period were noted in 8 patients: pancreatitis in 5 patients, perforation of cysts with biliary peritonitis in 3 patients. In the postoperative period, 4 patients had complications: stenosis of the anastomosis in 1; portal hypertension in 1; 3 children developed cholangitis, 1 of them required surgical correction. Out of 5 patients with Caroli disease, 2 children died in the neonatal period, one because his parents refused of treatment. Two children aged 12 and 17 had liver and kidney transplantation. Conclusion. CC clinical manifestations are diverse; asymptomatic course can develop up to some age. Ultrasound and MRI cholangiography are main diagnostic tools. Resection of the cyst with hepaticojejunostomy on the loop by the P technique is a radical approach to the treatment and has good outcomes. Patients with Caroli disease require an individual approach.

Hirschsprungs disease usually manifests from the first days of life and is diagnosed in the newborn period. In some patients Hirschsprungs disease cant be diagnosed in the newborn period because of different forms of disease and clinical features. From 2008 to 2019 75 patients with Hirschsprungs disease were operated in the City Childrens Hospital No. 1. 21 patients had delayed diagnosis. 11 newborns didnt have very clear clinical symptoms, intestinal obstruction disappeared after decompression. 2 patients with associated chromosomal disorders were diagnosed with Hirschsprungs disease later because of. In some of older patients disease manifested with severe constipations. Also, we presented some clinical cases of major diagnostic errors in patients with Hirschsprungs disease. Conclusion. Diagnostic errors in patients with Hirschsprungs disease are associated with the lack of alertness of neonatologists and inadequate interpretation of clinical manifestations and X-ray study. For many years these children can be treated by different specialists before having surgical consult.

Presented herein is a clinical case report concerning successful simultaneous surgical treatment of a female patient with a complication after transcatheter treatment for chronic dissection of the descending portion of the thoracic aorta. The woman was subjected to elective transcatheter isolation of chronic dissection of the descending thoracic aorta using a stent graft with complete coverage of the zone of the origin of the left subclavian artery. Repeat control imaging studies several months after the intervention revealed residual blood flow through the false channel, directed retrogradely from the distal edge of the stent graft to the left subclavian artery. Besides, the patient was also found to have local dissection of the distal part of the ascending aorta (zone 0). An operative intervention was performed: sternotomy, prosthetic repair of the ascending portion of the aorta and part of the aortic arch, as well as transposition of the left subclavian artery to the left common carotid artery. The control imaging studies confirmed radical removal of the false aneurysm of the ascending aorta and the presence of total thrombosis of the false channel at the level of the stent graft.Представлено клиническое наблюдение успешного одномоментного хирургического лечения больного с осложнением после транскатетерного лечения хронического расслоения нисходящего отдела грудной аорты. Пациентке в плановом порядке выполнена транскатетерная изоляция хронического расслоения нисходящей грудной аорты стент-графтом с полным перекрытием зоны отхождения левой подключичной артерии. При повторных контрольных визуализирующих исследованиях через несколько месяцев после вмешательства выявлен резидуальный кровоток по ложному каналу, направленный ретроградно от дистального края стент-графта в левую подключичную артерию. Кроме того, обнаружено локальное расслоение дистальной части восходящей аорты (зона 0). Выполнено оперативное вмешательство: стернотомия, протезирование восходящего отдела и части дуги аорты, а также транспозиция левой подключичной артерии в левую общую сонную артерию. При контрольных визуализирующих исследованиях подтверждено радикальное устранение ложной аневризмы восходящей аорты и тотальный тромбоз ложного канала на уровне стент-графта.

An analysis of treatment results was made in 216 patients with intussusception of bowels at the period from 2000 to 2015. The authors showed that it would be reasonable to carry out a conservative therapy in the cases of recurrent intussusception in absence of peritonitis symptoms.

An analysis of treatment results was made in 216 patients with intussusception of bowels at the period from 2000 to 2015. The authors showed that it would be reasonable to carry out a conservative therapy in the cases of recurrent intussusception in absence of peritonitis symptoms.

The article presents the experience of treatment of newborn children and infants with congenital malformations of the lung and mediastinum, which required a surgery. Children (138 cases) were treated during recent 18 years. There was a prevalence of full-term infants (73%). Fetal malformations were diagnosed in prenatal period in majority of cases. Computed tomography was the main method of diagnostics after delivery. Children (110 cases) were operated out of 138. Children with extrapulmonary sequestration didn’t undergo surgery in case of absence of clinical manifestations. The authors made a conclusion that malformations of the lung and mediastinum should be included in number of differentiated diseases in case of respiratory distress syndrome in newborn children. The indications to early surgery should be the danger of contamination and malignant transformation, presence of intrathoracic tension syndrome in neonatal period.

The authors share the observations from their cardiosurgical practice showing that one of the causes of sudden death or of rapid worsening of the state of patients with implanted bicuspid artificial valves might be their dysfunction resulting both from breakage of the hinged mechanisms of the valve with its dislocation and migration and from the disturbed mobility of the valve cusp due to a thrombus formed in the atrium. The observations show that the "ideal" construction of the valve is to be created.

Pulse oximetry is widely used to measure oxygen saturation in inpatient and outpatient settings. As with any medical technology, competence in interpretation and application of the data is essential for patient care. We therefore undertook a study to quantify pediatric house staff's knowledge of pulse oximetry and their ability to interpret the information.A 16-item multiple choice questionnaire (6 demographic, 10 knowledge) was developed to assess knowledge of pulse oximetry. The questionnaire was administered to a total of 134 pediatric house staff from five medical school-affiliated residency programs in New York City.The mean test score and standard deviation was 64.3% +/- 1.9, with a range of 10% to 100%. Regarding three questions related to the knowledge of the oxyhemoglobin deviation curve, only 17% of the the respondents answered all three questions correctly. In three questions pertaining to the accuracy of the pulse oximeter, 36% of the respondents answered all questions correctly. Forty-three percent of the house staff believed that training in the use of pulse oximetry was inadequate.The results showed that there is marked variability in pediatric house staff's knowledge of all aspects of pulse oximetry. This could contribute to patient morbidity. We suggest that medical schools and residency programs place more emphasis on teaching the principles and applications of pulse oximetry and the oxyhemoglobin dissociation curve.

Fifty-nine newborn infants with Hirschsprung's disease were treated at the Saint-Petersburg Center of Surgery of Developmental Anomalies from 1978 to 1991. The typical rectosigmoid form was found in 47.5% and a long zone of agangliosis in 35.6% of children. Mortality rate--16.7%. According to the development of enterocolitis, three variants of the course of the disease in the newborns were distinguished. It is shown that the informativeness of the diagnostic methods increases with the growth of the infants, and the final diagnosis of Hirschsprung's disease is best established at the age of over two weeks on the basis of repeated or initially postponed contrast study of the large intestine. Inefficacy of nonoperative treatment conducted for 2-3 days and recurrence of symptoms of intestinal obstruction are indications for operative decompression of the intestine. The performance of one-stage radical operation at the age of about one month is advisable in an uncomplicated course of the disease.