Pyloric atresia: authors’ clinical experience and literature review

Introduction. Congenital pyloric atresia is a rare malformation of the stomach outlet which amounts to about 1% of all atresias in the gastrointestinal tract. The gastric outlet atresia may be either isolated or combined with other abnormalities.Material and methods. There are few publications on the surgical treatment of pyloric atresia with extraluminal and intraluminal endoscopic interventions; however, at present information about it is not enough to assess their effectiveness and safety in young children. In literature, one can also find only few detailed reviews on clinical observations of pyloric atresia; most of which describe anatomical type I. Membranectomy and pyloroplasty are used for surgical treatment of pyloric atresia of type I. Surgery for pyloric atresias of types II and III which includes the resection in atresia zone and the formation of anastomosis between the stomach and duodenum causes complications due to the duodeno-gastric reflux in postoperative period. To prevent these complications, there is a surgical technique which was first described by A. Dessanti, et al. and later was modified and detailed by S. Yokoyama. This article is a literature overview on the topic and authors’ own experience in treating pyloric atresia of type II in 2 patients and of type I in one 1 patient.Conclusion. The choice of surgical approach depends on the anatomical type of pyloric atresia. Currently, there is no any unified approach to the surgical treatment of atresia of types II and III. Membranectomy and gastroduodenoanastomosis seem to be the most appropriate surgical techniques to correct this abnormality what has been confirmed by our preliminary findings.

Case Report: Uncommon Association of ITGB4 and KRT10 Gene Mutation in a Case of Epidermolysis Bullosa With Pyloric Atresia and Aplasia Cutis Congenita

Background: Epidermolysis bullosa is a rare form of genodermatosis produced by different gene mutations. The junctional form of the disease (JEB-PA) can associate pyloric atresia, renal abnormalities, and aplasia cutis congenita.Case Description: A case of a male preterm newborn with suspicion of digestive tube malformation at fetal ultrasound and who was born by cesarian section. At birth, he presented extensive cutaneous aplasia on the lower limbs and bilaterally under ears; outer ear agenesis; nasal septum hypoplasia; micrognathia; multiple blisters on the face, trunk, and limbs; lower limb deformities and absence of toe nails. Pathological examination following a surgical procedure with unfavorable outcome showed pyloric atresia, junctional form of epidermolysis bullosa and aplasia cutis congenita. Homozygous variants in two genes were identified: c.3111+1G>A in ITGB4 (class 5) and c.1498G>T in KRT10 (class 3).Conclusion: The particularity of our case is the novel finding of a coincidental occurrence in the context of consaguinity of two mutations in the ITGB4 and KRT10 genes, and clinical characteristics of epidermolysis bullosa.

Atresia of the antral and pyloric parts of the stomach

Introduction. Congenital obstruction of the gastric outlet is one of the rarest surgical diseases in newborns. Its incidence ranges from 0.003% to 1% of all cases of gastrointestinal atresias. This congenital malformation has several anatomical forms and divides into antral and pyloric atresias.Etiology. There is no any unified opinion as to the origin of atresia in the gastric outlet. Several concepts are known to suggest possible causes of the impaired patency of gastric outlet. Among them, there are genetic factors, intrauterine mesenteric ischemia, epidermolysis bullosa.Material and methods. The analysis of 55 literary sources is carried out.Results. The most common systematization of various forms of antral and pyloric atresia divides them into membranous forms (type I), solid forms without diastasis (type II) and forms with diastasis between segments (type III). Gastric outlet atresia can be diagnosed before birth if polyhydramnios and stomach dilatation are revealed at examination. The main diagnostic sign of the abnormality after birth is the radiographic symptom of “a single bubble”. The technique for patency restoration of the stomach distal parts is chosen depending on the anatomical type of obstruction: for pyloric and antral type I atresia, it is recommended to perform membranectomy and pyloroplasty by Heineke–Mikulicz technique; in pyloric atresia of type II and III, gastroduodenal anastomosis is put. Recently, new alternative techniques for restoring the patency of gastric outlet lumen have appeared, namely, laparoscopy and intraluminal endoscopy. Conclusion. Thus, atresia of the gastric antrum and pylorus are fairly rare congenital diseases. Surgical strategies for correcting these abnormalities envisage membranectomy or gastroduodenoanastomosis. A perspective direction in the care of elective cases of congenital gastric outlet obstruction is intraluminal endoscopy and laparoscopy

Laparoscopic treatment of pyloric atresia in a newborn

Introduction. Pyloric atresia is one of the rarest surgical diseases in newborns. Laparotomy is the most common modality for correcting this abnormality. In modern scientific literature, there is only one report on the endosurgical treatment of pyloric atresia. The case of laparoscopic correction of gastric outlet atresia presented in this study is one more reference to the successful care of this condition.Material and methods. The presented clinical case is a newborn boy in whom a prenatal ultrasound examination at the 31st week of gestation revealed polyhydramnios and an enlarged stomach. The baby’s birth weight was 2660 grams. In the first hours of life, the child showed symptoms of high intestinal obstruction in the form of gastric content vomiting. Postnatal ultrasound and X-ray examination confirmed the diagnosis of pyloric atresia. During laparoscopy, performed on the 2nd day of life, the morphological type of anomaly was examined, and the type I atresia of membranous form was established. A laparoscopic membranectomy with Heineke–Mikulicz pyloroplasty was performed. In the final part of this research, early and late outcomes of the performed surgical treatment were analyzed.Results. The surgery lasted for 70 min. Postoperative period was uneventful. Enteral nutrition started on day 3 after the surgery. A complete enteral diet was possible on day 7. The newborn patient was discharged from the hospital on the 9th postoperative day. Histological examination confirmed the pyloric atresia of membranous type. During 12-month follow-up, no complications associated with the surgical procedure were seen.Conclusion. The described case of laparoscopic treatment of type I pyloric atresia in a newborn expands laparoscopy application in pediatric practice.