Introduction. Definition " upper respiratory tract stenosis " (URTS) includes a large number of nosologies. The most common are cicatricial stenoses, in particular, post-intubation ones as well as bilateral paralysis of the larynx. An important issue for nowadays is choosing the curative modality for such patients.Purpose. To compare outcomes after treating children with URTS.Material and methods. The authors have assessed results of treatment of 110 patients who had reconstructive surgery in 2010-2019. Patients were divided into two groups depending on the nosology: stenosis of the upper respiratory tract (n = 71) and bilateral paralysis of the larynx (n-39). In each group, four types of surgeries were performed: costal cartilage plasty, plasty with T-tube (TT), a modified version of plasty with TT, plasty with a stent. The choice of treatment technique was determined mostly by the tendency to make this or that type of surgery in authors' departments, as well as by the nosology, severity of the disease and patient's age. Decannulation terms, surgical time, early and late postoperative complications as well as relapse-free period were assessed.Results. 66% patients were decannulated in Group 1; in Group 2 - 69%. Group 1 a statistically reliable comparison was possible between the following techniques: costal cartilage plasty and laryngeal stent plasty (p 0.005) as well as plasty with TT implantation and the proposed modified technique (p 0.005). While using Mann-Whitney test and Log Rank, the authors found out that in Group 1 there were no significant difference in surgical time, relapse-free period and stenosis degree (p 0.005). However, decannulation terms after plastic surgery of the larynx with costal cartilage implantation are longer than after plastic surgery with stent implantation (p 0.005). In Group 2, the authors compared three abovementioned curative modalities, excluding laryngeal plasty with TT implantation. Thus, surgical time for costal cartilage plasty is longer, if to compare to other two techniques (p 0.005). There is also no significant difference in decannulation terms and relapse-free periods between all three techniques (p 0.005).Conclusion. The results obtained have demonstrated the effectiveness of long-term stenting with TT placement in children, mainly with cicatricial stenoses, and in older children. The trial also revealed that laryngoplasty with costal cartilage in children with bilateral laryngeal paralysis is an effectiveness choice too.

Introduction. Stenosis of the upper respiratory tract is a common pathology in childhood. The most common cause is a prolonged intubation resulting in a persistent cicatricial narrowing of the airway. In modern literature, there are many techniques for its surgical treatment. At the first curative stage, tracheostomy is put. Nowadays, reconstructive techniques are widely used. One of the successful curative options in this pathology is laryngeal plasty with T-tube placement followed by the long-term stenting of the lumen.Purpose. To demonstrate potentials of performing reconstructive surgical treatment of upper respiratory tract stenosis in young children.Material. A clinical case of a two-year-old patient with acquired cicatricial stenosis of the larynx of the subglossal space, grade III, was treated at the department of thoracic surgery in Filatov Municipal Children’s Hospital.Conclusion. Our clinical observation has shown that plastic surgery of the upper respiratory tract with placement of T-tube in young children with cicatricial stenosis is an effective technique that forms a satisfactory air pathway, thereby providing the child with adequate breathing through the natural airways.

Purpose . The basic management task of children with extrahepatic portal hypertension (EHPH) is to prevent bleeding from intestinal and gastric protuberant varicosities. Materials and methods . Meso-portal shunting doesn’t only prevent bleedings from varicose veins, but also restores normal anatomical and physiological relations within the basin of the portal vein. The Clinical Hospital No. 13 has the largest experience of treating patients with EHPH. From 1989 to 2017 we observed 789 patients with portal hypertension aged 2.5 months to 17 years. 23.5% of them included children under 3 years old. Since 2000, meso-portal shunting was done in 261 children. 239 (91.6%) patients had an excellent result such as reduction of varicose veins and restored portal hepatic perfusion. Discussion . A clinical case of a 9-year-old child diagnosed with extrahepatic portal hypertension was presented. The girl underwent meso-portal shunting. However, no reduction of intestinal varicose veins was presented during the post-operative period. Meso-portal shunting stenosis was found during an angiographic study. The child had another operation; stenosis of meso-portal shunt was diagnosed, a narrowed section was resected with subsequent restoration of vascular insertion patency. During the postoperative period, varicose veins disappeared completely. Conclusion . The presented clinical example displays modern surgical possibilities of portal hypertension in children even in such complicated cases as stenosis of vascular venous anastomosis occurring following MPS approximately in 10% of cases.

The objective. To analyze early and long-term results of surgical treatment of biliary atresia in children. 120 patients with biliary atresia underwent Kasai procedure from 2000 to 2018. The follow-up course varied from 6 months to 15 years. The authors assessed the survival rate of children with native liver and the survival rate without indications for liver transplantation in different age periods, as well as the frequency of surgical complications, bacterial cholangitis, portal hypertension, etc.The results. The survival rate of children with native liver within 1 year was 82.7%; within 2 years – 57.72%; 3 years – 49.6%; 5 years – 42.1%; over 10 years – 33.25%. In case of effective surgery the authors noted the restoration of stool color, jaundice relief and gradual normalization of bilirubin level during the 1st year. The activity of gamma-glutamyl transferase and transaminases in most children increased in the early postoperative period and, then, gradually decreased. The most frequent postoperative complications were episodes of cholangitis and portal hypertension. During the first year, episodes of cholangitis were detected in 50 (42.3%) of 119 children, regardless of the effectiveness of the Kasai procedure. 56 children (47.75%) had signs of portal hypertension during the first year of life; its frequency increased to 70% by the age of 5–10 years.Conclusion. In most cases, Kasai procedure prolongs life with the native liver, allowing you to postpone transplantation and it is an effective method of treatment of biliary atresia in children. The most frequent complications are cholangitis and portal hypertension, the early detection and timely correction of them is an integral part of the treatment of these patients.

AIM:To present an overall experience of endoscopic operations in children with neurogenic tumors in two medical institutions.MATERIAL AND METHODS:Thoracoscopic excision of tumors was performed in 19 children aged 1 month-7.5 years (mean 1.5 years) in two clinics for the period 2010-2014. In 9 children (47.3%) age did not exceed 1 year. In our study ganglioneuroma was diagnosed in 9 cases, ganglioneuroblastoma--in 2 patients, neuroblastoma stage I--in 8 cases. All patients were under observation and treated according to NB2004 protocol. Tumor's diameter ranged from 1 to 6 cm (mean 4.9±1.9 cm). Mean duration of surgery was 62±22 minutes. There were no any intraoperative complications. Early postoperative period in all patients after endoscopic surgery was more favorable than in those after open operations. Any local recurrences were not observed for the follow-up period.RESULTS:Thoracoscopic operations can become more preferable method in treatment of children with neurogenic tumors if great vessels are not involved into neoplastic process and limited volume of tumor is absent.

At present, endoprosthesis of bones and joints with the use of modular oncology prosthesis is the most common method of organ-preserving treatment. Since the moment of opening of the FCRC of PHOI named after Dmitriy Rogachev, over 20 surgical operations for endoprosthesis replacement have been performed in the department of surgery of children and adolescents. MSTS (Musculoskeletal Tumor Society Score) international scale was used for assessment of the functional result after the surgical therapy. The worst result in accordance with the scale comprised 50 %, the best result comprised 93 %. The average index was 76 %. The possibility of the intra-operational varying of the replacement volume of the postresection defect is a good feature of modular endoprosthesis that saves the surgeon from possible errors of the pre-operational planning and unpleasant findings in the course of the operation. This simplified performance of endoprosthesis replacement in conditions of a small medical center with little clinical experience in this field, particularly in the course of performance of operations for children. Endoprosthesis secures good onclological and functional results as well as favors the most adequate social adaptation of a child.

UNLABELLED:Question of feasibility of laparoscopic Kasai procedure and laparoscopic hepaticojejunostomy for biliary atresia (the BA) and choledochal cysts (the CC) in children has been a subject of discussion for a long time and still remains unsolved. AIM:To improve the results of surgery for biliary tract malformations (the BTM) in children using laparoscopic technique. MATERIALS AND METHODS:The are 55 patients with BA and CC, who were performed endosurgical operations, and 37 patients, who were performed traditional open surgical procedures. During this study we worked out and put into laparoscopic practice some new methods. We've made an analysis of technical features of liver transplantation after laparoscopic and traditional Kasai's operation in BA. RESULTS:The duration of laparoscopic Kasai procedure was significantly longer than open surgery (p < 0.05). In CC the duration was the similar (p > 0.05). There was no any urgent surgical complication after laparoscopic Kasai procedure in contrast to open surgery. We observed significantly fewer complications (40%) after laparoscopic hepaticojejunostomy than after traditional hepaticojejunoduodenostomy (84.6%, p < 0.05). The average length of stay in ICU and duration of analgesia after laparoscopy was significantly lower than after open surgery (p < 0.05). Comparing the efficiency of endosurgery and traditional operations using coloration of stool, decreasing of bilirubin level and the presence of indications to liver transplantation criteria we didn't find any significant difference (p > 0.05). CONCLUSION:At the same efficiency of laparoscopic and open reconstructive surgery for BA and CC the first has a number of incontrovertible advantages. Our experience allows us to recommend laparoscopic procedures to perform for the correction of BTM in children.

In modern neuro-oncology and onco-hematology, intraventricular injection of chemotherapeutic agents (most typically, methotrexate) is an inevitable part of many protocols for treating patients with malignant tumors of the CNS, neuroleukemia, CNS lymphomas and some other disorders. A ventricular catheter system (also known as the Ommaya reservoir) is used to provide repeated injection of chemotherapeutic agents to cerebral ventricles. The use of modern neuronavigation systems allows one to place Ommaya reservoir in patients with narrow and slit-like ventricles. Material and methods. During the period between March 2012 and October 2013, 27 patients underwent stereotactic placement of the Ommaya reservoir using a Cart II optical neuronavigation system (Stryker) at the Dmitry Rogachev Federal Scientific and Clinical Center of Pediatric Hematology, Oncology and Immunology. The patients for stereotactic placement of a ventricular catheter were selected on a subjective basis (small ventricular size was the criterion for selection). In one patient, the surgery for placement of the Ommaya reservoir was combined with stereotactic biopsy. Results. In all patients, a ventricular catheter was placed in the anterior horn of the lateral ventricle on the first try; no intraoperative complications were observed. Conclusions. Frameless navigation is an illustrative, mobile, and multifunctional method. The same device can be used to perform brain, transsphenoidal, ENT, and spine surgeries, as well as orthopedic interventions. Today, neuronavigation systems are used in neurosurgical operating rooms and make it possible to avoid using stereotactic frames in most cases.

The literature review explores surgical diagnostic approaches in pediatric oncology and hematology. Various methods of biopsy, technical aspects, advantages, accuracy, rate of compications and application area are presented.

The Turcot syndrome is a rare disease which is characterized by a combination of a brain tumor with a y at which the neoplasm of the colon. About 150 of such observations are described in world literature. Our own observation and a literature review are presented in this article.

J. Turcot [26] described 2 cases of combination of colon polyposis with a brain tumor in two sick relatives, brother and sister in 1959. In the first case polyposis and adenocarcinoma of the sigmoid colon were combined with medulloblastoma; in the second case — with glioblastoma and pituitary adenoma. Three years later, combination of primary brain tumors and colorectal polyposis was called Turcot syndrome by McKusick, who also suggested an autosomal recessive inheritance mode of the disease. Since then, researchers have begun to pay attention to this rare disorder, and the number of observations has started to grow rapidly. The paper by H. Crail [7] describing the case of polyposis colon cancer, medulloblastoma, and thyroid carcinoma (published in 1949) was found retrospectively. Over 150 cases of patients with the Turcot syndrome have been reported in literature [12, 22]. It became apparent that this is a heterogeneous group of patients having different clinical manifestations of the cancer and heterogeneous genetic changes. Our own observation is presented below. A 12-year-old boy T. was sick for 2 years (since 2009), when the rare episodes of headache and nausea appeared. Since spring 2011, the emergence of bright red blood in the stool was noted. Left-sided hemiparesis accompanied by aggravated headache appeared since December 2011. A cystic tumor in the right parietal region was revealed by CT. The surgery aimed at removing the tumor in the right parietal region was performed at a local hospital of his residence in January 2012. The tumor was considered as glioblastoma. Radiation therapy (TFD ~46 Gy) was applied to the tumor bed during the next two months. The blood emergence in the stool was noted again when the patient was subjected to radiation therapy. Sigmoid colon polyp was revealed with rectromanoscopy and a biopsy was performed. Histological diagnosis was the proliferating tubular adenoma. A recurrent tumor in the right parietal region was revealed with the control CT in April 2012. The child was admitted to the Federal Scientific and Clinical Centre of Pediatric Hematology, Oncology and Immunology named after D. Rogachev in May 2012 for further examination and treatment. The family history had no data on the incidence of brain or colon tumors. No marriages between close relatives occurred. The clinical presentation upon admission included left-sided hemiparesis (up to 3 points) and blood in the stool. MRI showed a cystic tumor in the right parietal region and intensive accumulation of the contrast agent by tumor walls (Fig. 1). The repeated colonoscopy revealed a giant polyp that completely overlapped the lumen of the sigmoid colon, with loose, uneven mucosa with the areas of contact

The objective of the present work was to overview current concepts of epidemiology and clinical picture of a rare benign neoplasm, lipoblastoma, known to occur in the children, to report an original observation of one case of this condition, and to discuss specific features of its surgical treatment in the patient presenting with a rare localization of the tumour in the neck region extending onto certain vitally important anatomical features. Diagnosis of lipoblastoma of rare localization on the neck was verified in a 20-month old child. The necessity of differential diagnostics of lipoblastoma from other tumours (including malignant ones) in the neck region is substantiated along with the approaches to its surgical treatment bearing in mind the difficult of access localization of the tumour. It is concluded that the benign character of this neoplasm predetermines the favourable outcome of its treatment; however, there is a risk of incomplete dissection of the tumour and its relapse.

The aortic arch and its branches anomalia compound 1-3.8% of all vessel inborn diseases. The treatment of such anomalia as tracheal vessel ring aims the liquidation of esophageal and tracheal compression. The authors introduce their experience of thoracoscopic tracheal vessel rings resection in 6 children. The time of the operation was 145±15min. There were no conversions to the traditional open surgery. The result was excellent in 4 of 6 children, who were completely releaed of tracheal obstruction symptoms. The rest 2 children demonstrated mild respiratory symptoms, cuased by the tracheomalation.