Vol 13, No 1 (2023)

This editorial presents a report on the work of the Chief Pediatric Surgeon of the Ministry of Health of Russia for 2022. From the very beginning of his activity, he established regular interaction with the chief supernumerary pediatric surgeons and executive and legislative authorities of the regions of the Russian Federation, both remotely and by visiting surgical clinics in 15 cities. To prepare a program for the creation of interregional centers for specialized surgical care in the most popular medical specialties, much attention was given to the improvement of pediatric surgical care in the Far East with repeated trips to this region. As a result of the joint work with leading specialists of the Russian Association of Pediatric Surgeons and chief pediatric surgeons of the Russian Federation, the first “Regulations on Interregional Centers for Neonatal Surgery” was created. Chief pediatric surgeons organized surgical care for children in the special military operation zone and border regions with business trips to Taganrog, Rostov-on-Don, and Lugansk. Throughout the year, he coordinated round-the-clock assistance to injured children, and dozens of telemedicine consultations were held. Based on the result of a systematic survey of the chief supernumerary pediatric surgeons of the Russian Federation, a consolidated report on the activities of the pediatric surgical service of the Russian Federation for 2021 was formed, which was important to develop prospects for its further development.

The work plans of the chief pediatric surgeon for 2023 include the completion of important documents for the specialty: “The procedure for providing surgical care to children” and the “Professional standard for a pediatric surgeon”. There was also a task of implementing interregional neonatal surgery centers in the Far Eastern Federal District, and expert, educational work, and audit of children’s surgical clinics will be continued.

BACKGROUND: In recent years, the number of children with pectus carinatum tended to increase. The literature describes only a few cases of a combination of keeled chest deformity with a more serious pathology of the spine, i.e., Scheuermann–Mau disease.

AIM: This study aimed to examine the frequency, clinical manifestations, and timely diagnosis of combined structural lesions of the thoracic spine in children with keeled chest deformity.

MATERIALS AND METHODS: This observational single-center cross-sectional study included patients aged 5–17 years with pectus carinatum. Categorical values were described by indicating absolute values and percentages in the sample, and quantitative indicators with normal distribution were described using arithmetic means and standard deviations and 95% confidence intervals. Quantitative indicators without normal distribution were described using the median and interquartile range.

RESULTS: Scheuermann–Mau disease was detected in 11 (9.3%) of 118 children with pectus carinatum. “Pterygoid scapulae” was noted in 97 (82.2%) children with pectus carinatum, increased cervical lordosis in 93 (79.7%), and sloping, anteriorly adducted shoulders in 99 (83.9%), which significantly hampered the clinical assessment of the extent of thoracic kyphosis. At the time of examination, a rigid thoracic kyphosis was formed in a 16-year-old boy. In younger children (5–14 years old), Scheuermann–Mau disease had no clinical manifestations and was detected only during screening X-ray examination, whereas in the older age group (15–16 years), 3 of 4 adolescents complained of back pain.

CONCLUSIONS: The frequency of Scheuermann–Mau disease in children with pectus carinatum exceeds the average prevalence in the population. In patients with keeled chest pterygoid scapulae, excess cervical lordosis, and rounded shoulders are associated with the difficulty of the assessment of the magnitude of thoracic kyphosis. Asymptomatic progression of Scheuermann–Mau disease is typical in younger children with pectus carinatum, and pain syndrome and the clinical picture of the disease develop only by the age of 15–16 years. Thus, all patients with pectus carinatum and posture disturbance should undergo a screening X-ray examination of the thoracic and lumbar spine to detect Scheuermann–Mau disease and initiate treatment in time.

In children with extrahepatic portal hypertension (EPHT), the most frequent and life-threatening complication is bleeding from varicose veins of the esophagus and stomach. Therefore, the main task of HSV treatment is to prevent bleeding from the veins of the upper gastrointestinal tract. The most effective treatment is portosystemic bypass surgery, with effectiveness of 94%–97%. However, the use of these operations is limited precisely by a decrease in portal liver perfusion and development of portosystemic encephalopathy. This study aimed to analyze domestic and foreign publications devoted to the surgical treatment of portal hypertension and the development of portosystemic encephalopathy during the postoperative period. A systematic search was carried out in PubMed, Web of Science, Scopus, MEDLINE, eLibrary, RSCI, and Cyberleninka; 345 references were analyzed, 110 articles were reviewed, and 97 publications on the surgical treatment of portal hypertension were selected for the review. The development of portosystemic encephalopathy during the postoperative period often occurs after the imposition of total portosystemic bypass surgery. To reduce the risk of developing portosystemic encephalopathy, selective shunts have been developed, which to some extent preserve the PPP. With distal splenorenal anastomosis, the frequency of portosystemic encephalopathy is 10%–15%. An intermediate position is occupied by a side-to-side splenorenal anastomosis, which has signs of selective surgery. The results of the analysis of literature sources showed that discussions are still underway regarding the choice of the optimal treatment strategy for patients with EPHT, place and role of endoscopic methods for the prevention of gastrointestinal bleeding, and mesoportal shunt in the treatment of patients with EPHT. Nevertheless, the majority of world experts consider mesoportal shunt to be the most optimal operation for the primary and secondary prevention of varicose bleeding and other HSV complications. If it is impossible to perform, selective bypass surgery of the distal splenorenal anastomosis can be an alternative to a mesoportal shunt.

Pediatric urologists rarely encounter bladder diverticulum in children. If the bladder diverticulum has clinical manifestations such as pain, urinary tract infection, hematuria, voiding dysfunction, vesicoureteral reflux, or obstruction of the ureterovesical anastomosis, then surgical treatment is indicated. Recently, numerous publications began to appear on the removal of the bladder diverticulum using laparoscopic or vesicoscopic access. Several foreign publications have reported on the removal of a bladder diverticulum in children using a robotic approach. A 9-year-old boy who was diagnosed with a bladder diverticulum underwent robot-assisted bladder diverticulectomy in January 2020 at the Almazov National Medical Research Centre. Ports of da Vinci 12 and two 8-mm ports, as well as an assistant 5-mm port, were used, which were installed in typical places for lower abdominal surgery. The duration of the operation was 135 min, and the console time was 75 min. The blood loss volume was 20 mL. The patient was in the intensive care unit for 1 day. The urethral catheter was removed on day 7 after the operation. Urination independence, delays, and incontinence were not noted. The patient was discharged on postoperative day 9 in satisfactory condition. At the control examination after a year at the patient’s residence, she had no complaints, urination was not disrupted, and there were no pathological changes in the ultrasound of the kidneys and bladder. Robot-assisted bladder diverticulectomy in children is a feasible, effective, and safe treatment option in expert centers with extensive experience in robotic surgery. The described clinical case of bladder diverticulectomy is the first in Russia, which was performed using a robotic system in pediatric practice.

BACKGROUND: Obstructive variants of chronic duodenal obstruction may be caused by congenital malformations, such as rare periampullary duodenal duplication cysts. This localization of gastrointestinal duplications is the rarest and is not described in the domestic literature.

AIM: This study aimed to present the experience of the management of periampullary duodenal duplication cysts in children.

CASES REPORT: Seven children with periampullary duodenal duplication cysts underwent surgery between 2007 and 2022. All patients suffered from prolonged abdominal pain, vomiting of food, and bile. Two children had recurrent pancreatitis, and one child underwent repeated operations for high intestinal obstruction and biliary obstruction in other hospitals. An abdominal cyst was suspected in one patient at another hospital, and the child underwent exploratory laparoscopy, but no formation was found. Ultrasound, gastroduodenoscopy, computed tomography, and magnetic resonance imaging were performed on these children. Ultrasound examination showed a peristaltic cystic formation up to 4 cm in diameter with a double wall. Gastroduodenoscopy showed formation in the second portion of the duodenum in the region of the major duodenal papilla. This formation covers the intestinal lumen by two-thirds and up to nearly complete obturation. The relationship between duodenal duplication and the bile and pancreatic ducts was clarified on magnetic resonance cholangiopancreatography: the relationship was suspected in two children. All patients underwent wide transduodenal excision of periampullary duodenal duplication cyst; in four cases, laparoscopic access was used. No intra- and postoperative complications occurred. Histological examination revealed true duplications in all cases. The children were discharged on days 11–12. In the follow-up after 3 months and 10 years, no complications were noted.

CONCLUSIONS: Periampullary duodenal duplication cysts can be a cause of chronic duodenal obstruction. Transduodenal excision of periampullary duodenal duplication cysts is the optimal treatment, and this operation can be performed laparoscopically.

Cross-dystopia is a rare type of congenital anomaly and is characterized by a displacement of the kidney to the opposite side; as a result, they are both located on the same side, and in approximately 85% of such cases, a fusion of the parenchyma of two kidneys can be observed. We have not found reports of cases in which a nonfunctioning orthotopic cystic dysplastic kidney associated with ureterovesical segment pathology (ureterocele) was fused with the lower pole of a cross-dystopian normally formed kidney in the literature. The patient, 8 days old, had a preliminary diagnosis of agenesis of the right kidney, doubling of the left kidney, cystic dysplasia, doubled left kidney with an ureterohydronephrosis in the lower half and ureterocele. To restore the urine outflow and function of the lower half of the presumably doubled left kidney, a cystourethroscopy was performed. In a typical place on the right, there was a correctly formed ureteral orifice, i.e., an ureterocele on the left, which occupies half of the bladder volume. With a holmium laser, an artificial orifice was formed in the ureterocele to restore the urine passage. At 9 months old, tomography urinary tract was performed, which revealed cross-dystopia of the right kidney with a fusion of the lower pole and a cystic dysplastic orthotopic left kidney. Laparoscopic nephroureterectomy of a non-functioning orthotopic kidney was performed.

The control examination indicated complete clinical remission and social adaptation of the patient after discharge. Cross-dystopia of the kidney with fusion, cystic dysplastic kidney, and a ureterocele are rare congenital anomalies that require timely examination in a specialized clinic and drawing up an individual treatment plan. The surgical method should focus on a symptomatic urological problem, emphasizing preserving kidney function.

Commentary on the article Morozov KD, Sharkov SM, Kozlov MYu, Mordvin PA, Ayrapetyan MI, Morozov DA “Primary intestinal anastomosis in a child with perforation of Meckel’s diverticulum and peritonitis” published in the Russian Journal of Pediatric Surgery, Anesthesia and Intensive Care. 2023;13(1):105–112. (In Russ.) DOI: https://doi.org/10.17816/psaic1498

After reading the article under discussion, serious doubts were raised about the presence of diffuse peritonitis (there was no typical response of the body to a severe inflammatory process in the abdominal cavity), and the mild course of diffuse peritonitis in surgical practice is unknown. Primary intestinal anastomosis in peritonitis cannot be recommended for pediatric surgeons to present its wide introduction in clinical practice.

Information about the life path of the founder of pediatric surgery in the Far East, Professor Alina P. Shapkina-Pilipenko, who died in March 2023.