Introduction. Hirschsprung’s disease is a congenital anomaly characterized by the absence of ganglion cells in submucosal and intramuscular layers of intestinal wall that leads to the intestinal obstruction. 70–80 % of cases are isolated malformation but it can be combined with chromosomal abnormalities and other malformations. Coexistence of Hirschsprung’s disease with intestinal atresia is extremely rare. It can cause significant difficulties in diagnostics and treatment.Material and methods.Patient A, a boy, was transferred to the surgical department at the age of two days with a history of intestinal obstruction. He had abdominal X-ray studies: intestinal obstruction. The contrast enema showed microcolon that was coiled in the pelvis. Patient was prepared to the surgical treatment. Intraoperatively colonic atresia was identified at 10 sm from the ileocecal valve. A double colostomy was performed to the child and biopsy on the level of mucous fistula was taken. Histological study showed the aganglionosis of the distal colon. At the age of 4 months, the patient underwent Soave-Swenson endorectal pullthrough procedure with intraoperative extended express-biopsy that confirmed the absence of ganglion cells in whole distal bowel. The aganglionic part was resected, the ileocecal valve with the part of the colon of 10 cm long was mobilized and the endorectal bringing the colon down to the perineum was performed by Soave – Swenson. Postoperative recovery was uneventful.Results.A high index of suspicion is required to promptly diagnose Hirschsprung’s disease in a child with colonic atresia despite the rare combination of these two anomalies. In this case the histological study allowed to recognize association of colonic atresia with Hirschsprung’s disease and helped to avoid complications after further surgery.Conclusions. Early detection of coexisting of these two anomalies helps to prevent the development of serious postoperative complications.
Introduction. Despite the continuous improvement of methods for prenatal diagnosis of fetal malformations, at present, the limitations of intrauterine verifcation of vascular lung anomalies and relevance in postnatal typing of all components of pathological pulmonary hemodynamic, especially in the case of complex, combined vascular malformations, remain. Detailed and expert assessment of the anatomy of the pulmonary vessels, intrapulmonary hemodynamic is necessary to minimize the time for the surgical correction of the defect in order to avoid the development of irreversible pulmonary hypertension. One of the most rare and diffcult to diagnose forms of a combination of vascular abnormalities is congenital pulmonary venolobar syndrome (CPVS), which combines venous, arterial, pulmonary and cardiac pathology, to one degree or another coexisting in one patient.Objective – analysis of clinical material and data of different diagnostic methods in children with CPVS. This everything will help to optimize diagnostics and choose the most safe and informative method to visualize combination of pulmonary and cardiovascular diseases and to determine the specifcity of pulmonary circulation. Furthermore, it will allow to minimize all risks and negative consequences for patient’s body.Material and methods. We reviewed the records of 11 children (age from 14 days to 17 years old) with intraoperative verifcation of CPVS. We systematized all variants of vascular anomalies of lungs in children with CPVS, its possible combinationswith other abnormalities. We performed the comparison of capability and diagnostic signifcance of plain radiography, echocardiography, selective angiography and CTscan in determining the specifcity of pulmonary circulation in children with CPVS and assessment of combination of the malformation components. Results. Antenatal echocardiography and postnatal diagnostics using echocardiography, plain radiography and selective angiography is extremely limited. We suggested the diagnostic algorithm using CTscan with the proof of the great representativeness. Obligatory components of CPVS were identifed: reduction of the right lung volume, anomalous pulmonary venous drainage (scimitar syndrome) and nonobligate components of malformation: aplasia or hypoplasia of the right pulmonary artery, systemic pulmonary arterial supply with differential pulmonary perfusion, pulmonary sequestration, other congenital heart abnormalities.Conclusion. CPVS is a rare congenital malformation. Antenatal diagnosis of all components of CVLS is impossible. MDCTAG is the most effective diagnostic method that allows to identify all components of CPVS in postnatal period. This method does not require additional radiodiagnostics and invasive diagnostic procedures.
Статья поступила в редакцию: 01. 03.2016 Статья принята к печати: 16.05.2016В статье представлены медико-статистические данные в целом о детской специализированной травматоло-гической службе в Санкт-Петербурге, о пролеченных больных в круглосуточном травматологическом отде-лении крупного педиатрического стационара Санкт-Петербурга со всеми травмами, в том числе с травмами костно-мышечной системы (КМС), которые составили 67,3 % от всех детей, госпитализированных в травма-тологическое отделение. Кроме того, выявлены пациенты с травмами КМС, которые лечились в непрофиль-ных отделениях детского стационара (4,1 %). Дана углубленная характеристика детского травматологического стационарного отделения (коечный фонд, штаты и т. д.). Представлены структура госпитализированных детей с травмами КМС, основные показатели работы отделения, методы лечения и диагностики. Большой раздел статьи посвящен проблемам реабилитации детей с травмами КМС. Показаны структура, сроки лечения детей с последствиями травм КМС на этапе реабилитации. Авторы доказывают необходимость увеличения числа реабилитационных коек для удовлетворения потребности в реабилитации всех нуждающихся пациентов.Ключевые слова: травмы, костно-мышечная система, дети, стационар, показатели работы, реабилитация. Актуальность исследованияТравматизм у детей остается одной из веду-щих медико-социальных проблем вследствие вы-сокой распространенности травм и их влияния на уровень смертности и инвалидизации детского населения. Он занимает второе место в структуре общей заболеваемости детей, составляя 6-8 % [1]. Многие исследователи отмечают высокие показа-тели частоты травм у детей в крупных городах, в центрах интенсивного дорожного движения [2][3][4]. В структуре первичной инвалидности детей заболевания и последствия повреждений кост-но-мышечной системы (КМС) составляют 11,2 % от общего количества детей-инвалидов [5, 6].Стационарная специализированная помощь является наиболее ресурсоемким видом медицин-ской помощи, поэтому анализ ее состояния с уче-том территориальных особенностей может по-служить основой для разработки рекомендаций, направленных на совершенствование специализи-рованной помощи детям.Цель работы -на основе изучения дан-ных о состоянии стационарной травматологиче-ской помощи детям при травмах КМС в Санкт-Петербурге разработать рекомендации по ее совершенствованию.В ходе изучения решались следующие задачи.
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.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.