Патофізіологічні основи розвитку захворювання Гіршспрунга (Morbus Hirsсhsprung)

Abstract

На сьогоднішній день захворювання Гіршспрунга (ЗГ) у новонароджених та грудних дітей – це важка та складна проблема у розвитку сучасної дитячої хірургії. Це пов’язано із широким спектром клінічних проявів, складності діагностики та вибору методики лікування. Зауважуємо, що неадекватна тактика хірургічного втручання в ранньому віці є основною причиною смертності пацієнта. В статті подано інформацію про перші згадки захворювання Гіршспрунга (ЗГ). Представлено декілька яскравих випадків лікування аномалії, а також класифікацію хвороби, котрою користуються лікарі протягом багатьох років. Автори докладно пояснюють сутність проблеми ЗГ, складність в діагностиці та лікуванні, а також причини можливого ускладнення; На сегодняшний день заболевание Гиршспрунга (ЗГ) у новорожденных и грудных детей – это тяжелая и сложная проблема в развитии современной детской хирургии. Это связано с широким спектром клинических проявлений, сложности диагностики и выбора методики лечения. Замечаем, что неадекватная тактика хирургического вмешательства в раннем возрасте является основной причиной смертности пациента. В статье представлена информация о первых упоминаниях o ЗГ. Представлено несколько ярких случаев лечения аномалии, а также классификацию болезни, которой пользуются врачи на протяжении многих лет. Авторы подробно объясняют сущность проблемы ЗГ, сложность в диагностике и лечении, а также возможного осложнения; Today, Hirschsprung`s disease (HD) in newborns and infants is a difficult and complicate problem for development of modern pediatric surgery. This is due to a wide range of clinical symptoms, the complexity of diagnosis and choice of treatment methods. Its need to replaced, that inadequate tactics of surgical intervention at an early age is the main cause of death of the patient. Aganglionic megacolon or Hirschsprung’s disease is an anomaly of the colon development, which is represented by a violation of the innervation of a specific area of the body. The anomaly is particularly characteristic of the sigmoid colon and rectum and is more common in boys than in girls. Characteristic symptoms of agangliosis are persistent constipation, which are accompanied by acute intestinal obstruction. There are early and late symptoms of disease, the first of which is the delay or failure of defecation and flatulence, which arise from the moment of birth. The symptoms are aggravated by the introduction of solid food. Physical methods of research make it possible to successfully differentiate the flaw among others. The late symptoms are anemia, fecal intoxication and asymmetry of the chest and abdomen. The Scientists point out the existence of several anatomical forms of the disease – rectal (25%), rectosigmoidal in proportion, which accounts for 70%, segmental (1.5%), subtotal (3%) and total, accounting for 0.5% of cases. Also, there are known phases of HD – compensatory, subcompensatory and decompensatory stages. The main methods of HD investigation are irrigoscopy, anorectal manometry, and biopsy of the rectum mucosa for histochemical and immunological examination. In particular, when conducting a complete X-ray, it is possible to detect pneumotization and a sharp increase in intestinal loops, especially in the region of the sigmoid colon. It is proved, that with complication of HD enterocolitis, there is a toxic organ distribution. A characteristical symptoms of agangliosis in irrigoscopy is the narrowing of the distal intestinal tract, as well as the appearance of acute enterocolitis. The sense of the aganglionical megacolon is in the absence or reduction in the muscle fiber of the Auerbach plexus and the sympathetic nerve component of the Meisner plexus. Therefore, the large intestine is constantly in tone, that is spasm, which is not desirable for the digestive system. So, it explains the problem of the complexity of passing feces by a natural way. This disease is observed in different age groups, ranging from newborns to elderly people. Despite on it such number, the disease is manifested by several characteristical symptoms, that helps to differentiate the anomaly. Thus, the task of pediatricians and neonatologists is to monitor the fetus of a pregnant woman and identify pathology in the early stages of development, and for surgeons, its the creation of conditions for the failure of possible complications. The problem requires a further detailed investigation, since this anomaly can take place both in mild form and in severe, even leading to mortality. If the etiology of HD has already been studied, but there is no specific solution to this situation; it remains only to use operational methods that partly help the patient. The article provides information about first mentions of the Hirschsprung`s disease (HD). There are presented several bright cases of abnormalities treatment, a classification of the disease, that doctors have been using for many years. The authors detaily explain the origin of the HD problem, the complication of diagnosis and treatment.