In techeniemnogih years in the literature did not exist consensus on the definition of the esophagus as an independent vrozhdennogokorotkogo malformation.The largest contribution vizuchenie etiology, pathogenesis, clinical and morphological data, diagnostics ilecheniya congenital short esophagus introduced B.Barret (1959).The disease is described in the literature under different names: brahiezofagus, chest stomach, undescended stomach, chest partially stomach.
Poyavlenievrozhdennogo short esophagus is due to violation of the formation pischevaritelnogotrakta early embryonic period, when the esophagus was lined tsilindricheskimepiteliem.Replacement of the epithelium, beginning with the middle third of the esophagus is disrupted, resulting in the lower third of its lined zheludochnymepiteliem.Thus there is a misplacement of the gastric mucosa in the esophagus nizhnyuyutret (normal arrangement of the cardia).
Podtverzhdeniemtakogo assumption is the fact that epiphrenic taknazyvaemogo undescended pa
Vrozhdennyykorotky esophagus is usually accompanied by gastro-oesophageal reflux, which is complicated by the appearance of esophagitis and peptic stenosis of the esophagus.
Vrozhdennyykorotky esophagus diagnosed in children during the first three years of life, as they stoykosohranyaetsya reflux syndrome.The history of such patients have chastovoznikayuschaya vomiting shortly after birth.Often, there are indications of frequent respiratornyezabolevaniya: bronchitis, pneumonia (30% of cases).In the following clinical manifestations zavisyatot formation of secondary stenosis.If the stenosis is not formed, it is osnovnymsimptomom vomiting gastric contents.Vomiting provoked tears, the horizontal position of the body, dazhenebolshim physical strain due to increased intra-abdominal davleniya.V vomit show streaks of blood;rarely vomit kofeynoyguschey.In secondary narrowing of the esophagus due to peptic yazvennogoezofagita predominate symptoms of impaired patency of the esophagus: dysphagia, rvotaneizmennoy food.
Children starshetreh years complain of chest pain, the occurrence of which obuslovlenodisfagiey and ulcerative esophagitis.Often children try to drink pischuvodoy adopted.Due to constant bleeding from dystopic slizistoyobolochki, as evidenced by blood in vomit and polozhitelnayareaktsiya occult blood in the stool, anemia.Anemic sindromnablyudaetsya in 1/3 patients.Children with congenital short esophagus behind vfizicheskom development.
Dlyadiagnostiki congenital short esophagus used rentgenologicheskoeissledovanie with which primarily obnaruzhivayutzheludochno-esophageal reflux.The Trendelenburg position bezstenozirovaniya esophagus in a patient is determined by throwing izzheludka contrast material into the esophagus.The distal portion of the esophagus, usually extended downward vvide funnel or bell.On X-rays in the distal otdelepischevoda visible longitudinal joint-characteristic of the mucous obolochkizheludka.Patency of the esophagus in the study of the patient in the standing position the non-offending.In secondary stenosis permeability contrast medium is difficult, and the above restriction is determined prestenoticheskoe expansion pischevoda.Rentgenologicheskaya picture with deformation in the area of constriction and excessive skladochnostyuslizistoy shell requires differentiation of esophageal hernia otverstiyadiafragmy and diverticula of the esophagus.
Shirokoispolzuetsya to diagnose ezofagofibroskopiya.Most vazhnymendoskopicheskim feature is the upward shift of the transition line slizistoyobolochki esophageal mucosa of the stomach, ie,proektsiidiafragmalnoy above gap.In the absence of a stenosis in the distal pischevodavyyavlyaetsya catarrhal or fibrinous ulcerative esophagitis.In developing stenozasuzhenie usually located at the border of the gastric mucosa ipischevoda.
Due tems that patients with congenital short esophagus always has mestozheludochno-esophageal reflux, certain diagnostic value imeetvnutripischevodnaya pH meter.
Unovorozhdennyh and infants with pyloric stenosis provodyatdifferentsialnuyu diagnosis, pilorospazme, gryzheypischevodnogo aperture, Chalaza, congenital and priobretennymistenozami distal esophagus.
chaschehirurgicheskoe treatment aimed at eliminating gastroesophageal reflyuksa.Naibolee robust antireflux surgery is an operation N.N.Kanshinu vmodifikatsii Yu.F.Isakova et al., Which is based on the valve gastroptyxis.In cases where a congenital short esophagus complicated peptic stenosis, antireflux surgery is complemented by the imposition of gastrostomy.Later stenozustranyaetsya probing.
With tochkizreniya clinicians considering congenital short esophagus like neopuscheniezheludka necessary to conduct long-term conservative therapy based napostepennoe omission of the stomach (the growth of the child).
Konservativnoelechenie elevated position provides continuous top polovinytulovischa, frequent feeding baby food in small portions gustoykonsistentsii.Spend as restorative and symptomatic treatment.