Vol 23, No 4 (2019)

Actuality. The Hirschsprung’s disease is one of the leading pathologies in the digestive tract in children. Despite the variety of diagnostic techniques for this disease, none of them has an absolute certainty when the short form disease is suspected, and only the assessment of findings after complex examination plus clinical picture can result in establishing a reliable diagnosis. The authors analyze outcomes of surgical treatment of the Hirschspring’s short form disease in children when the technique of dosed dissection of the internal anal sphincter under ultrasound control was applied and compare these results with those when conventional surgical techniques were used. Material and methods. 83 children aged 1-17.5 y.o. with the established diagnosis of Hirschsprung’s disease and an ultrashort aganglionic segment were taken into the study. Patients were divided into two groups. Group 1 (41 patient) who had surgical correction by the Lynn technique; Group 2 ( 42 children) who were operated on by the new developed technique. Results. 74% (31 patient) of children who were operated on by the dosed dissection of the internal anal sphincter under ultrasound control had good results in their late postoperative period; 10 patients (24%) had satisfactory results; unsatisfactory outcome was seen in 1 patient (2%). In children , who were operated on by the Lynn technique, good results were in 23 patients (57%); satisfactory results in 15 patients (37%) and unsatisfactory result in 3 patients (6%). Conclusion. The rectal form of Hirschsprung’s disease with a short and ultrashort segment requires surgical treatment in the vast majority of cases. In this case, surgery with dosed dissection of the internal anal sphincter under ultrasound control can be a method of choice.

Introduction. In modern world literature there are no data on changes in the central hemodynamics in children with purulent-septic pathologies. Purpose: to improve diagnostics of cardiovascular disorders in children with appendicular peritonitis and destructive pneumonia using device “Cardiocode”. Material and methods. The trial with “Cardiocode” was conducted in two regional medical centers in Voronezh and Belgorod. Hemodynamic parameters were studied in healthy children (n = 60), in children with destructive pneumonia (n = 83) and in children with appendicular peritonitis (n = 98). Parameters of the central hemodynamics were studied in pre- and postoperative period until recovery. Results. It has been found out that in children with appendicular peritonitis and destructive pneumonia the heart rate exceeds normal limits by 37-58%. The stroke volume in children with appendicular peritonitis did not differ of normal values, while in children with destructive pneumonia it declines by 10-15%. Early diastole was significantly reduced on admission, by 75-85% of normal limits. Reduction of parameters was recorded in the rapid expulsion of left ventricular systole from 80% to 70% of normal limits. In children with destructive pneumonia and appendicular peritonitis, tone of the ascending part of the aorta (Vt.a) was increased as well. conclusion. Apparatus "Cardiocode" is an effective non-invasive tool for early diagnostics and for controlling treatment of hemodynamic disorders in children with appendicular peritonitis and destructive pneumonia.

Introduction. Aglossia / hypoglossia is a rare condition combined with underdeveled lower jaw, lack of mandibular incisors and various degrees of limb anomalies. In domestic and foreign literature, one can find only few such observations in pediatric and adult populations. This malformation is met in the syndrome of limb and oromandibular hypogynesia or aglossia-adactylia syndrome. The incidence of aglossia / hypoglossia is very low (1 / 175,000-1 / 1,000,000 live births, ) and in most cases it is represented by hypoglosia, and not true aglossia. The isolated aglossia without limb malformations is an even more rare condition. The etiology of aglossia / hypoglosia is unknown, since all known cases are sporadic and by present moment no more than 100 observations have been published. Material and methods. In 2015, a girl (1 year 10 mon old ) and a 3- year old boy were examined in hospital and outpatiently because of complaints for the reduction of lower jaw. The children were examined by an orthodontist; additional examinations were prescribed to the girl because of revealed comorbidities. Results. The girl also had vertebrae malformation. Such a pathological combination had not been seen previously in the available literature. The second child was a boy of 3 y.o.; his parents decided to consult an orthodontist for correcting the bite. In both cases, children were well adapted, although there were some deficiencies in verbal activity and difficulties in feeding; after speech therapy and spoon-feeding trainings these problems were solved. Conclusion. Even with a significant degree of tongue underdevelopment, there are practically no functional limitations in patients with aglossia-adactyl syndrome, which is important for determining prognosis as well as for consulting patients and their parents and for making decision about the need for corrective surgery.

The authors describe an extremely rare combination of omphalocele and isolated segments of the small intestine with enetercystomas having their own mesentery. This pathology was manifested by a syndrome of partial intestinal obstruction during 50 days after suturing the anterior abdominal wall because of omphalocele. During relaparotomy, a pair of separated intestinal fragments with hypertension in their inner content was removed. The patient recovered completely.

Choledocholithiasis is a rare pathology in children. Various techniques have been proposed for removing calculi from the common bile duct: percutaneous puncture drainage of bile ducts, endoscopic retrograde cholangiopancreatography with papillosphincterotomy as well as revision of the common bile duct which can be done laparoscopically or in the open abdomen. However at present, there is no any unified approach to managing this pahtology in children. The article describes authors’ experience of laparoscopic revision of the common bile duct in an infant with choledocholithiasis. The authors consider that this technique can be applied in pediatric surgical practice.