卷 26, 编号 2 (2022)
ORIGINAL ARTICLES
Diffusion tensor imaging in trauma of the cervical spine in children
摘要
Introduction: The technique of diffusion tensor imaging (DTI) is widely used in brain research. However, in studies of the spinal cord, especially in pediatrics, the use of DTI is difficult: the small size of the spinal cord, which is characterized by a low signal-to-noise ratio, artifacts from respiration and pulsation of the heart and large vessels, and swallowing movements. Also, the EPI sequences used to obtain diffusion indices cause eddy current distortion.
Objective: to investigate changes in the parameters of DTI of the spinal cord in children with cervical spinal cord trauma.
Material and methods: 56 children aged 2 to 17 years with cervical spine injury (CSI) and 20 children who did not have CSI were examined using a Phillips Achieva 3 T magnetic resonance scanner. The protocol consisted of sagittal STIR, sagittal and axial T1- and T2WI SE, and axial DTI. For DTI, apparent diffusion coefficient (ADC), fractional anisotropy (FA), axial (AD) and radial (RD) diffusion coefficients were calculated.
Results: According to the ASIA criteria for injuries, out of 56 children, 29 (51%) had CSI with neurological complications, and 27 (49%) had no complications. The degree of neurological dysfunction A was in 13 patients, B - 3, C - 9, D - 4, E - 27 patients. The average values of the group of patients with were: ADC=0.74 ± 0.12*10−3 mm2/s−1, FA = 0.36 ± 0.07, BP=1.15 + 0.28*10−3 mm2/s−1, RD=0.52 + 0.32*10−3 mm2/s−1
Conclusion: DTI can detect changes beyond the limits of conventional MRI. Low ADC values in acute injury may indicate spinal cord injury and predict a negative functional outcome.
EDITORIAL ARTICLE
He always respected the pain of another. Dedicated to the 100th anniversary of the birth of Professor, Corresponding Member of the Russian Academy of Sciences and the USSR Academy of Medical Sciences Giray Alievich Bayirov
ORIGINAL STUDY
A comparative analysis and choice of surgical tactics in pediatric Crohn’s disease
摘要
Introduction. Currently, surgical complications in Crohn’s disease is still one of the most serious problems in pediatric surgery. An intensive development of video endoscopic surgery contributes to the active introduction of this technology in pediatric coloproctological practice. Crohn’s disease is still one of the most complex pathologies in pediatric gastroenterology, and surgical treatment of its complications is a disputable issue among pediatric surgeons. Up to now, there are no clear indications to surgery in the refractory form of Crohn’s disease, no standardized terms and types for surgical intervention, as well as no optimal variants of surgical access.
Material and methods. A retrospective analysis of patients who had been treated surgically at departments of abdominal and emergency surgery in two pediatric hospitals in Moscow (Morozovskaya and Izmailovskaya ) was carried out. 39 children with Crohn’s disease, aged 4–17 year, were included in the study. All patients had standard clinical examination: clinical examination with anamnesis, laboratory and instrumental diagnostics.
Results. The obtained results have shown that laparoscopic interventions have a number of advantages, such as less traumatic surgery, reduced exposure to anesthesia and shorter intestinal stimulation, less stay in ICU, shorter hospitalization as well as more rapid rehabilitation period.
Conclusion. The performed assessment of outcomes after surgical treatment of children with complicated Crohn’s disease helped to develop indications for the selection of surgical technique in the ileocecal form. Thus, the obtained results improved outcomes in children with complicated Crohn’s disease because of the outlined indications for surgery and surgical tactics when minimally invasive techniques are more preferable.
Diffusion tensor imaging in injuries of the cervical spine in children
摘要
Introduction. The technique of diffusion tensor imaging (DTI) is widely used in brain examination. However, DTI application for examining the spinal cord, especially in children, is not easy: small dimensions of the spinal cord with a typical low signal-to-noise ratio, artifacts from the respiration and pulsation of the heart and large vessels, as well as from swallowing movements. EPI sequences used to obtain diffusion indices cause eddy current distortions.
Objective. To study changes in DTI parameters of the spinal cord in children with cervical spinal cord injury.
Material and methods. 56 children aged 2–17 years with cervical spine injury (CSI) and 20 children without CSI were examined with Phillips Achieva3 T magnetic resonance scanner. The protocol consisted of sagittal STIR, sagittal and axial T1- and T2WI SE and axial DTI. To assess DTI, the following parameters were calculated: apparent diffusion coefficient (ADC), fractional anisotropy (FA), axial (AD) and radial (RD) diffusion coefficients.
Results. By ASIA criteria, 29 (51%) children out of 56 had CSI with neurological complications; 27 (49%) had no complications. Neurological dysfunction of degree A was in 13 patients; B – in 3; C – in 9; D – in 4; E – in 27. Average values of diffusion in patients were: ADC = 0.74 ± 0.12 • 10−3 mm2/s−1, FA = 0.36 ± 0.07, BP = 1.15 + 0.28 • 10−3 mm2/s−1, RD = 0.52 + 0.32 • 10−3 mm2/s−1.
Conclusion. DTI can detect changes which cannot be detected by conventional MRI. Low ADC values in the acute injury may indicate spinal cord injury and predict a negative functional outcome.
Effectiveness of ultrasonic energy in introitoplasty
摘要
Introduction. Currently, the application of ultrasonic energy for tissue dissection is a routine practice in surgery. Its effectiveness and safety has been proven in proctology, pediatric coloproctology, surgeries in the endocrine organs and in the abdominal organs.
Material and methods. Given the features of the endocrine system, the anatomy of virilized genitalia and positive experience in applying ultrasonic energy for surgical interventions, the authors present a comparative trial on the effectiveness of ultrasonic knife (USK) and traditional electrocoagulation in vaginoplasty in patients with disorders of sex development.
Results. The obtained findings demonstrate that USK in comparison to traditional electrocoagulation significantly reduces vaginoplasty duration by 30%: median and quartiles of this index with USK were 57.5 [50; 65] min, while in the electrocoagulation group – 82.5 [75; 90] min. Difference is statistically significant (U-test 83.5, p <0.001) too. Difference between medians and its 95% confidence interval was 25 [20; 35] min.
Conclusion. USK has better effects due to less tissue bleeding and, as a consequence, better visualization of the surgical field. An additional advantage is a unique ability to gently mobilize and preserve anatomical structures of the pelvis.
REVIEWS
Laparoscopic kidney resections in children with nephroblastoma (а literature review)
摘要
Introduction. Nephroblastoma is the third most common solid tumor in childhood, accounting for 7–8% of the total number of solid tumors in children. Currently, there is no unequivocal position of oncosurgeons regarding laparoscopic nephrectomy in patients with nephroblastomas; the cases described in the world literature are rare.
Purpose. To find out the effectiveness of laparoscopic kidney resection in children with nephroblastoma.
Materials and methods. The authors describe two clinical cases of laparoscopic nephrectomy in patients with nephroblastoma, including one-stage bilateral laparoscopic nephrectomy for bilateral nephroblastoma.
Results. At the time of article writing, the two patients were in clinical and instrumental remission for 6 and 11 months, respectively.
Conclusion. Indications for laparoscopic kidney resections in nephroblastoma: stage I, peripheral location of the tumor node with predominant extrarenal growth, response to chemotherapy, absence of the cystic component of the tumor.
Extraction of foreign bodies from the upper gastrointestinal tract (а literature review)
摘要
Introduction. Swallowing foreign bodies is a common case in children which can cause a large number of complications. The statistics informs that 80% of cases are met in children aged from 6 months to 6 years. Late detection of foreign bodies is associated with a high risk of life-threatening complications. Timely detection and removal of foreign bodies is a relevant problem in modern medicine.
Material and methods. A literature review was made by keywords on the diagnostics and treatment of foreign bodies in the gastrointestinal tract in databases Scholar, Pubmed, Medline, Google.
Results. On analyzing the obtained literature data, the authors describe in details types of the approach to diagnose and remove foreign bodies in the gastrointestinal tract. Two large options were identified: non-endoscopic and endoscopic. Besides, the authors make a review of other unique techniques for removing foreign bodies proposed by various authors.
Conclusions. The obtained and analyzed literature data demonstrate that even the authors who offer different curative options recognize that endoscopy is the most safe and effective one for removing various objects. However, despite the obvious advantages of endoscopy in removing foreign bodies, it should be borne in mind that this technique is possible to apply only under certain conditions: namely, proper equipments and hospital setting, as well as experienced and trained personnel.
Лапароскопические резекции почек у детей с нефробластомой
摘要
Нефробластома – третья по распространенности солидная опухоль детского возраста, ее доля составляет 7-8% от общего числа солидных опухолей у детей. Хирургическое лечение, как правило, консервативно и заключается в нефрэктомии. В последнее время широкое распространение при небольших опухолях почек получила нефрон-сохраняющая хирургия (NSS- Nephron sparing surgery) - удаление опухоли почки путем резекции в переделах здоровых тканей, как альтернатива нефрэктомии. В современных протоколах лечения детей с нефробластомой существуют четкие показания для проведения лапароскопической нефрэтомии и для резекции почки. В настоящее время не существует однозначной позиции онкохирургов относительно проведения лапароскопической резекции почки при нефробластоме, а описанные в мировой литературе случаи - единичны. В данной статье представлен обзор литературы, посвященный данной проблеме и собственный опыт в проведении лапароскопических резекций почки при нефробластоме, включая уникальный опыт в проведении одномоментной двухсторонней лапароскопической резекции почек при билатеральной нефробластоме.
Ключевые слова: нефробластома, лапароскопические резекции, почка, малоинвазивная хирургия
УДАЛЕНИЕ ИНОРОДНЫХ ТЕЛ ЖЕЛУДОЧНО-КИШЕЧНОГО ТРАКТА. ОБЗОР ЛИТЕРАТУРЫ.
摘要
Введение. Проглатывание инородного тела - частое явление среди детей, которое может привести к большому количеству осложнений. По данным статистики, данная проблема в 80% случаев встречается у детей в возрасте от 6 месяцев до 6 лет. Позднее выявление инородных тел сопряжено с высоким риском развития грозных осложнений. Своевременное обнаружение и удаление инородных тел является актуальной проблемой современной медицины. Материалы и методы. Был проведен анализ литературы, найденной по ключевым словам, в базах данных Pubmed, Medline, Google Scholar по теме диагностики и лечения инородных тел желудочно-кишечного тракта. Результаты. По результатам обзора литературы подробно описаны методы диагностики и лечения инородных тел желудочно-кишечного тракта. Выделено две большие группы методов лечения: неэндоскопические и эндоскопический, а также рассмотрены уникальные методики удаления инородных тел, предлагаемые различными авторами. Выводы. На основе литературного обзора можно сделать вывод, что авторы, предлагающие различные методы удаления инородных тел желудочно-кишечного тракта, признают, что эндоскопия является наиболее безопасным и эффективным методом извлечения различных объектов. Однако, не смотря на очевидные преимущества использования эндоскопии с целью удаления инородных тел, стоит учитывать, что применение данной методики возможно лишь при определенном уровне оснащения и возможностей стационара, а также, при наличии обученного персонала.
CASE REPORT
Segmental necrosis of the outlet of the stomach and duodenum in a premature newborn baby
摘要
Introduction. Gastric perforation in newborns is a polyetiological, severe disease that combines a number of destructive conditions, such as: isolated pinpoint perforations, wall necrosis and gastric rupture. The first description of this pathology in a premature infant was made by Siebold in 1825, and only in 1968, G. Reams reported a case of successful diagnosis and surgical correction of this disease. Currently, the frequency of gastric perforation occurs from 1 to 6 cases per 30 thousand live births, and the mortality rate is within 40–70%.
In all infants, disorders in the integrity of stomach wall can be divided into iatrogenic and “idiopathic” which are caused, in most cases, by intrauterine hypoxia. Therefore, while examining such patients, differential diagnostics between gastric perforation and necrotizing enterocolitis is often made.
Clinical case. The authors present their experience in diagnosing and treating segmental necrosis of the stomach and duodenum outlet in a child with an extremely low body weight of 800 gr. Predisposing factors in the antenatal period were considered to be manifestations of hereditary thrombophilia, severe preeclampsia, placental insufficiency, and intrauterine growth retardation. As far as the discussed pathology had a subacute course, clinical changes were registered only on the 21st day of infant’s life, when pneumoperitoneum was detected at the plain X-ray image. When the patient’s condition was stabilized, median laparotomy was performed, at which radial necrosis with detachment in the gastroduodenal junction was seen. An end-to-end gastroduodenoanastomosis was put at the defect zone. In the postoperative period, the course was complicated with bronchopulmonary dysplasia, oblique femoral fracture with angular fragment displacement because of premature osteopenia as well as cholestatic hepatitis. The patient was discharged from the hospital in satisfactory condition in 80 days.
Conclusions. Thus, better understanding of specific features in the clinical picture of the discussed pathology and of the mechanisms of perforation development in hollow organs of the abdominal cavity will reduce the number of complications and adverse outcomes in patients with extremely low body weight.
Clinical case: segmental testicular infarction as a complication of varicocele in a 17-year-old teenager
摘要
Introduction. Segmental testicular infarction in childhood and adolescent practice is a rare condition that develops most often as a complication of other andrological or other diseases. This pathology is extremely difficult to diagnose. At the same time, in order to increase the probability of a favorable outcome, it requires a decision to carry out appropriate therapeutic measures as soon as possible.
Description of the observation. The patient was admitted with a picture of acute scrotum syndrome 3 hours after the onset of the disease with suspected testicular torsion? Testicular vein thrombosis on the background of varicocele? Previously, I had not been observed about varicocele, I did not know about the disease.
By organs without gross changes. In the local status, there is an expansion of the testicular veins on the left to the III degree. The testicle on the left is painful, dense, taut. Ultrasound examination and dopplerography of the scrotum organs were performed on an emergency basis. According to the radiation methods, segmental thrombosis was detected in the middle part of the testicle, however, the low frequency of segmental thrombosis and a greater focus on testicular torsion did not allow us to be sure of the absolute accuracy of these additional methods. An emergency revision of the scrotum was performed, in which the diagnosis of segmental thrombosis was confirmed. Subsequently, during the examination after 3 months, a partial restoration of blood flow in the infarction zone was noted without pronounced signs of testicular atrophy.
Conclusion. Segmental testicular infarction is an extremely rare complication of varicocele in childhood and adolescence. An adequate assessment of the clinical situation and the use of ultrasound imaging methods make it possible to establish the true diagnosis of segmental testicular infarction. Not in all cases, segmental testicular infarction should be considered as an irreversible condition that leads to sclerosis in its outcome.
ЛЕВОСТОРОННЕЕ РАСПОЛОЖЕНИЕ ГРЫЖИ АМИАНДА У РЕБЕНКА.
摘要
Грыжа Амианда или паховая грыжа, содержащая червеобразный отросток. Среди ученых до сих пор ведутся споры о том, что же считать истинной грыжей Амианда - ущемление червеобразного отростка в грыжевом мешке паховой или послеоперационной грыжи с развитием гангренозного аппендицита или паховая грыжа, содержащая невоспаленный, или воспалительно-измененный, или перфорированный червеобразный отросток. Спорными являются также вопросы хирургического лечения, целесообразность выполнения аппендэктомии при неизмененном червеобразном отростке в грыжевом мешке и выбор способа грыжепластики. Однако, к сожалению, принимать решения часто приходится экспромтом во время операции, так как определение червеобразного отростка в содержимом грыжевого мешка до оперативного вмешательства проблематично.
В литературных данных описаны случаю подобных грыж, как у взрослых, так и у детей, но с правосторонней локализацией. Частота встречаемости «грыжи Амианда» составляет, по данным разных авторов от 0,07-0,13 до 2-4 %. В доступной медицинской литературе нам не удалось найти описание грыжи Амианда с левосторонним расположением, поэтому сочли целесообразным опубликовать данный редкий клинический случай: левосторонней невправимой пахово-мошоночной грыжи с наличием в грыжевом мешке гангренозно измененного червеобразного отростка. У ребенка от беременности, протекавшей на фоне умеренной преэклампсии; хронической артериальной гипертензии IIст, риск 2; пролапса митрального клапана II –III ст.; анемии легкой степени; плацентарных нарушений; угрозы прерывания. Родившегося на сроке 27-28 недель беременности путем кесарева сечения с экстремально низкой массой тела (610 грамм), бронхолегочной дисплазией, врожденной пневмонией. Обратившимся в возрасте 2 года 8 месяцев по экстренным показаниям с клиникой ущемленной пахово - мошоночной грыжи слева, сопутствующим острым фарингитом. Особенности клиники, анамнеза жизни и заболевания, сложности диагностики, тактические ошибки, допущенные на этапах оказания помощи, проведенное оперативное и консервативное лечение описаны в данной статье.
КЛИНИЧЕСКИЙ СЛУЧАЙ ТЯЖЕЛОГО ТЕЧЕНИЯ НЕКРОТИЗИРУЮЩЕГО ЭНТЕРОКОЛИТА ОСЛОЖНЕННОГО ОТРЫВОМ ЖЕЛУДКА У НОВОРОЖДЕННОГО С ЭКСТРЕМАЛЬНО НИЗКОЙ МАССОЙ ТЕЛА
摘要
Некротизирующий энтероколит (НЭК) одно из наиболее тяжелых хирургических заболеваний желудочно-кишечного тракта у новорожденных, первое упоминание о котором выполнено K. Schmid и K. Quasier в 1953 г. Ключевым патогенетическим звеном является нарушение кровообращения в кишечной стенке. Морфофункциональная незрелость обусловливает динамические изменения, снижение иммунной защиты и эффективности эпителиального барьера желудочно-кишечного тракта. Известно, что в патологическом процессе наиболее уязвимы подвздошный отдел тонкой и толстой кишки. Однако, к редким и тяжелым заболеваниям с высоким уровнем смертности относят перфорации в верхних отделах пищеварительного тракта. Несмотря на раннюю диагностику, летальность в этой группе пациентов может достигать 80%.
Представленный клинический случай демонстрирует тяжелое течение НЭК осложненное отрывом желудка у новорожденного мальчика с экстремально низкой массой тела (ЭНМТ), составившей 800г. В антенатальном периоде были отмечены проявления наследственной тромбофилии, тяжелой преэклампсии, фетоплацентарной недостаточности и задержки внутриутробного развития (ЗВУР). В связи с подострым течением НЭК у ребенка клинические изменения диагностированы на 21 сутки жизни, на обзорной рентгенограмме выявлен пневмоперитонеум. При стабилизации состояния выполнена срединная лапаротомия, на которой обнаружен радиальный некроз с отрывом в области гастродуоденального перехода. На зону дефекта наложен гастродуоденоанастомоз по типу «конец в конец». В послеоперационном периоде констатированы осложнения в виде: бронхолегочной дисплазии, косого перелома бедра с угловым смещением отломков на фоне остеопении недоношенных, явления холестатического гепатита. В результате проведенного лечения пациент выписан в удовлетворительном состоянии из стационара на 80 койко-день.
Таким образом, знания специфики клинической картины и особенностей развития НЭК у пациентов с ЭНМТ позволят сократить количество осложнений и неблагоприятных исходов в данной группе больных.
CLINICAL OBSERVATIONS
Extended necrotic intestinal lesions in a 12-year-old child with thrombophilia
摘要
Introduction. The hereditary tendency to thrombosis in children has been studied for the past 20 years due to molecular genetics. Currently, about 40 point mutations are known.
Purpose. The article presents a clinical case taken from the authors’ practice which demonstrates thrombophilia complications in a child.
Material and methods. A 12-year old girl was admitted to the registration department of the Regional Children’s Clinical Hospital (Chita) with abdominal pain and multiple vomitings. The complete blood count (CBC) revealed a marked hemoconcentration. Thrombophilia was diagnosed before the patients was hospitalized.
Results. The performed surgical treatment was made by stages because of the extended necrotic lesion of the intestine. The testing of genetic polymorphism associated with thrombophilia risks revealed risk alleles FGB*455GA in the patient
Conclusion. The given article describes specific manifestations of gastrointestinal tract lesion, difficulties in diagnostics and care of complications in a patient with thrombophilia.
Segmental testicular infarction in a 17-year-old teenager
摘要
EDITORIAL COMMENT. According to clinical data, segmental testicular infarction occurred due to transient testicular torsion with impaired arterial blood supply. Thus, in the presented clinical case, segmental testicular infarction should not be considered as a complication of varicocele.
Introduction. Segmental testicular infarction in children and adolescents is a rare case that is mostly met as a complication of andrological or other diseases. This pathology is extremely difficult to diagnose. At the same time, in order to increase the chance for favorable outcomes, decision on the proper way of care must be taken as soon as possible.
Purpose. To demonstrate a case of successful diagnostics and treatment of segmental testicular infarction in a teenager with varicocele.
Material and methods. The patient was admitted with a picture of acute scrotum syndrome three hours later after the onset of the disease with preliminary diagnosis "testicular torsion and thrombosis of testicular veins in a patient with varicocele". Four days before, the patient consulted a urologist about pain in the left testicle. Varicocele grade III on the left without orchopathy was diagnosed. The pain syndrome was relieved by non-steroidal anti-inflammatory drugs. Previously, this pathology had not been diagnosed in this patient, and he did not suspect it. Examination of organs: no marked changes; locally, on the left, there is expansion of testicular veins of degree III; testicle on the left is painful, dense. Ultrasound examination and dopplerography of the scrotum organs were urgently made. Findings of radial examinations revealed segmental thrombosis in the middle part of the testicle; however, because of low occurrence of segmental thrombosis and greater focus on the testicular torsion the surgeons were not sure of absolute accuracy of the obtained additional findings. An emergency revision of the scrotum was performed at which the diagnosis of segmental thrombosis was confirmed. Three month later, at a follow-up examination partial restoration of blood flow in the infarction zone without pronounced signs of testicular atrophy was seen.
Conclusion. Segmental testicular infarction is an extremely rare complication of varicocele in children and adolescents. A proper assessment of the clinical picture and ultrasound imaging allow to put the well-timed and correct diagnosis of segmental testicular infarction. Not in all cases, segmental testicular infarction should be considered as an irreversible condition that leads to sclerosis in its outcome.
The left-sided location of Amyand hernia in a 2-year-old child
摘要
EDITORIAL COMMENT. The symptom complex presented in the article (a syndrome of edematous hyperemic scrotum in a child with an irreducible left-sided hernia) was undoubtedly an indication for emergency surgical treatment. The cause of the left-sided localization of Amiand hernia requires additional examination – irrigography.
Introduction. Amyand hernia or inguinal hernia is the hernia in which the vermiform appendix is located within the hernial sac. There are still debates among researchers what is a true Amyand hernia – strangulation of the vermiform process in the hernial sac of an inguinal or postoperative hernia with its development into gangrenous appendicitis or an inguinal hernia with non-inflamed, or inflamed-altered, or perforated vermiform process. Debates on the surgical treatment, reasonability of appendectomy with an unchanged appendix in the hernial sac and ways of hernia repair are still controversial too. Unfortunately, decisions often have to be made right during surgery since the revealing of vermiform process and its morphology in the hernial sac before surgery is a problematic issue.
Literature describes cases of similar hernias, both in adults and in children, but with the right-sided location. Amyand hernias are seen, by different authors, in 0.07–0.13 to 2–4%.
Relevance. In the available medical literature, there is no any description of such hernia with the left-sided location, so the authors have considered it appropriate to publish a rare clinical case.
Objective. To analyze a rare case of the left-sided inguinal-scrotal hernia with a destructive vermiform process in the hernial sac and to identify possible diagnostic and therapeutic errors occurred during medical care.
Material and methods. Clinical case: a 2-year and 8 months old child was admitted to the hospital having emergency symptoms of a left-sided strangulated inguinal-scrotal hernia with a gangrenous-altered vermiform process in the hernial sac and concomitant acute pharyngitis. Anamnesis: child from the first pregnancy which was accompanied by moderate preeclampsia; chronic arterial hypertension of degree II, risk 2; mitral valve prolapse of degree II–III; mild anemia; placental disorders; threats of pregnancy termination. The child was born at 27–28 week gestation with the Caesarean section. It had an extremely low body weight (610 grams), bronchopulmonary dysplasia, congenital pneumonia.
Conclusion. Amyand hernia is an insidious pathology. It has specific clinical manifestations, specific anamnesis and child’s life; it is difficult for diagnostics and can lead to tactical mistakes which are described in the given clinical case as an example.
Leiomyomatosis of the esophagus and rectum in a 16-year-old patient
摘要
Introduction. Leiomyoma is a benign tumor, the source of which is smooth muscles of the internal organs. This neoplasm is rare in children. Leiomyomatosis of the gastrointestinal tract is most often asymptomatic; it is clinically manifested when the size of the formation reaches more than 4 cm. The most common symptoms are abdominal pain and a palpable formation in the abdominal cavity. Surgical removal is the method of choice for most leiomyomas.
Description of clinical case. This article describes a clinical case of leiomyomatosis of esophagus and rectum in a child who had suffered of abdominal pain, constant constipation and dysphagia since early childhood. The correct diagnosis was put several years later after the onset of the disease. Upon examination, a solid formation was found in the anal area. The examination also revealed a volumetric formation in the submucosal layer of the rectum, causing a narrowing of its lumen, as well as a large-sized formation in the thoracic cavity, causing esophageal stenosis. The first stage of surgical intervention was to place colostomy and to take biopsy of the rectal tumor. The second stage one month later was extirpation of the esophagus affected by the tumor, plastic surgery of the esophagus by the stomach. The morphological picture corresponded to leiomyomatosis. The third stage – extirpation of the rectum which was performed at A.N. Ryzhykh State Medical Center.
Conclusion. This clinical case is one of few syndromic variants of leiomyomatosis described in the literature. Surgical intervention is the basic option for treating patients with non-epithelial neoplasms of the gastrointestinal tract. Active surgical tactics in benign tumors are due to high risk of developing or developed life-threatening complications.