[Etiology, pathogenesis and surgical treatment of atresia of the small intestine].
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Animals↗
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[The assessment of risk factors of the atresia of the gastro-intestinal tract].
The rate of gastro-intestinal tract atresia is around 0.3 percent in Georgia. In common cases delayed surgical treatment of these malformations is the reason of perinatal mortality. So, it is very important to concretize the risk factors, which cause the development of gastro-intestinal tract atresia. Our research was performed on the basis of examination of 161 probands with Gastro-Intestinal tract atresia and 155 control probands from the common population of Georgia, and also their relatives of I, II, III degrees of kinship. The methods of investigation were: clinical, paraclinical, genealogical, genetical-epidemiological. The analysis of the clinical material was done by the multiple-statistical method with the alternative analysis. It was shown, that an elderly age of parents, spontaneous abortions, or a hazard of spontaneous abortions, acute infections, emotional stress and the action of other teratogenic factors during the I trimester of pregnancy, hormonal misbalance of mother and some of the professional factors (especially agricultural) are most important etiologic and pathogenetic factors of the gastro-intestinal tract atresia.
Esophageal Atresia↗
Emergency operations in the newborn.
With the present-day development and understanding of anesthetic methods, fluid and electrolyte therapy, antibiotic medications and pediatric care, many congenital anomalies once uniformly fatal are now being successfully treated by emergency operations in the neonatal period. The eight most common of these which demand emergency operation in the immediate postnatal period are esophageal atresia and tracheoesophageal fistula, diaphragmatic hernia with dislocation of the abdominal viscera into the chest, malrotation of the intestine with obstruction, intestinal atresia, meconium ileus, imperforate anus, omphalocele and myelomeningocele. Although infants born with any of these serious problems often are born prematurely and often have more than one congenital anomaly, survival rates in the surgical treatment of these conditions are steadily improving. Early diagnosis and prompt treatment are the most important factors in the continued improvement of these survival rates.
Anus, Imperforate↗
An unusual variant of a jejunoileal atresia.
This is a report of a patient with an unusual variant of intestinal atresia associated with third trimester bleeding that required an extensive small bowel resection. The patient had a 40 cm segment of atretic intestine removed, and continues to do well at age 11 mo despite a short-bowel syndrome and lack of ileocecal valve. No lumen was present in the atretic segment, but numerous foamy histiocytes were present. She weighs 8.1 kg, and is receiving a combination of parenteral nutrition and oral feedings.
Appendix↗
Multiple gastrointestinal atresias with cystic dilatation of the biliary duct.
A term newborn developed signs and symptoms of an upper-intestinal obstruction. A palpable transverse upper-abdominal mass was identified. An ultrasound examination demonstrated a cystic mass with massive dilatation of the biliary tree. At laparotomy, dozens of segmental intestinal atresias (IA) from the jejunum to the rectum were identified. The cystic mass was the duodenum, obstructed distally from the atresias and proximally from an antral web. The biliary dilatation was due to normal drainage into a closed-loop duodenal obstruction, and was relieved by division of the web. The multiple atresias were explored, but establishment of intestinal continuity was impossible. The entity of multiple segmental IAs in association with cystic biliary dilation has been previously described, but there are no recorded survivors; 35 patients have been reported in the world literature. Our patient was the longest survivor; she finally died after 2 years of gastrostomy-tube drainage and total parenteral nutrition while awaiting intestinal transplantation. Only 5 patients other than ours had documented immune-system abnormalities. We review the findings and management of this rare syndrome.
Bile Duct Diseases↗
Congenital atresia of the colon combined with segmental dilatation of the ileum: a case report.
Atresia of the colon is a rare form of congenital intestinal atresia. Other gastrointestinal anomalies have been reported in these patients, but to our knowledge, the association between colonic atresia and segmental dilatation of the ileum has not been reported.
Anastomosis, Surgical↗
Experimental small bowel obstruction in chick embryos: Effects on the developing enteric nervous system.
BACKGROUND/PURPOSE: After surgical repair of congenital small bowel atresias, intestinal motility disorders often are observed. These may be caused by changes in the enteric nervous system (ENS) secondary to obstruction. To assess these changes, small bowel atresias were induced experimentally in chick embryos. METHODS: On day 11, the small intestines of 90 chicken embryos were ligated microsurgically in ovo. Breeding of the eggs was continued until day 19. The small bowel was removed, fixed, and embedded for silver-staining, semithin serial sections, and transmission electron microscopy. Additionally, acetyl-cholinesterase (AChE)-staining was performed. Normal chick embryos of the same age served as controls. RESULTS: Macroscopically, experimentally induced small bowel atresias had the same characteristics as human newborns. Microscopically, the wall structure was preserved; however, the ENS differed markedly from controls. Both proximal and distal to the obstruction, the submucosal plexus was almost completely absent, whereas the myententeric plexus was diminished only in the proximal dilated blind pouch. The axonal net was disrupted additionally. Ganglion cells of the myenteric plexus in the proximal segment were arranged in longitudinal clusters of densely packed cells. In the distal segment ganglion cells formed round clusters. The cells of Cajal, which normally surround the myenteric ganglia, were absent in the proximal and distal segments. CONCLUSIONS: In our experiments, structural changes in the ENS could be observed secondary to experimentally induced small bowel atresias in the chick. Because of the lack of ischemia in this model, the main cause of these ENS changes seems to be the dilatation oft the proximal gut. Dilatations are common features in intestinal atresias, anorectal malformations, and Hirschsprung's disease. Our observations, thus, explain motility disorders after the surgical repair of these diseases.
Animals↗
Duodenal and biliary atresia associated with facial, thyroid and auditory apparatus abnormalities: a new mandibulofacial dysostosis syndrome?
We report a female child born at 36 weeks of gestation with multiple abnormalities including dysmorphic and coarse facial features with features of mandibulofacial dysostosis that include bilateral microtia with the absence of external auditory meati and Mondini dysplasia as well as, duodenal atresia, intestinal malrotation, anterior displacement of the anus, left hemiaplasia of the thyroid and biliary atresia in sibs. The associations of duodenal atresia with intrahepatic and extrahepatic biliary atresia in sibs have been reported, suggesting an autosomal recessive syndrome. However, the associated external, middle and internal ear anomalies and the thyroid malformation, however, have not been reported in this condition. To the best of our knowledge, this is a hitherto new syndrome with an unknown inheritance.
Abnormalities, Multiple↗
The association of jejunal atresia and cystic lymphangioma in the same location.
Cystic lymphangiomas, which are localized on the mesentery of the intestine, are rarely seen. The presence of intestinal atresia together with cystic lymphangioma in the same location has never been described before. In this study, a premature girl weighing 2,630 g who had jejunal atresia, cystic lymphangioma localized in the mesentery of the distal atretic jejunal segment, and multiple rib anomalies is presented. The association of jejunal atresia and cystic lymphangioma may be coincidental, or jejunal atresia may be caused by an intrauterine vascular compression of volvuled cystic lymphangioma. Such an association is being reported for the first time in the literature.
Abnormalities, Multiple↗
[Our experience in neonatal surgery in a 15-year period. Follow-up of 1,003 cases].
From 1982 to 1994, 1.003 cases of surgical newborns have been retrospectively studied. Frequency and epidemiology have been evaluated, so as associated malformations. Prenatal diagnosis was made in 72 cases (7.1%). The most frequent entity was intestinal atresia, with 122 cases (12.1%), followed by necrotizing enterocolitis in 80 cases (7.9%), nevertheless this last one was the most frequent finding in preterm newborns. Esophageal atresia was found in 57 cases (5.6%). Association with other malformations was found in 127 cases (12.6%), and 32 cases of them (3.1%) constituted congenital malformations syndromes. The number of surgical newborns has increased in the last years, mainly due to ambulatory surgery. Global mortality of operated patients was 62 cases (5.9%); the highest mortality index was for congenital diaphragmatic hernia (33.5%), followed by necrotizing enterocolitis (28%). Mortality rate has decreased in recent years, due to improving in management and treatment of these patients, and the consolidation of surgical newborn units. Future efforts should be aimed for decreasing in mortality and improving the standard of life of these patients.
Enterocolitis, Pseudomembranous↗
Tapering enteroplasty in infants with bowel atresia and short gut.
Antimesenteric tapering enteroplasty was evaluated as an alternative to resection in 16 babies. Tapering enteroplasty was done for jejunal atresia (11), ileal atresia (three), total colonic aganglionosis (one), and colonic atresia (one). Most had short gut with proximal bowel dilation. There were no anastomotic leaks but slight functional delay (eight to 14 days). One patient with 14 jejunoileal atresias received continuous-drip feeding while the remainder took regular diet. Mean weight gain was in the 25th percentile at 1 year of age, and the 35th percentile at 2 years of age. There were two deaths from liver failure and brain damage. Antimesenteric tapering enteroplasty is particularly useful in selected instances of intestinal atresia associated with short gut, where preservation of bowel length may be essential for survival. Gastrointestinal function, bowel movement patterns and growth and development have been gratifying.
Colon↗
Jejunal atresia with 'apple peel' deformity. A report of eight survivors.
Jejunal atresia with an "apple peel" deformity is one of the most severe forms of intestinal atresia. Until quite recently, neonatal mortality rates as a result of this anomaly were high. Early diagnosis, appropriate surgery, and prolonged parenteral nutrition have led to a spectacular improvement of the survival rates. We are reporting eight cases of apple peel atresia--all eight patients survived. The pathophysiology, diagnostic aspects, surgical approaches, and postoperative management are discussed.
Female↗
Familial apple peel jejunal atresia: surgical, genetic, and radiographic aspects.
Fifty-seven cases of apple peel jejunal atresia have been reported in the English literature. Patients with this anomaly have a high incidence of prematurity (70%), malrotation (54%), short gut syndrome (74%), multiple atresias (15%), complications (63%), and mortality (54%). Mortality has decreased from 63% to 47% since 1970. We report three new cases of apple peel jejunal atresia, including two from one family. Radiographic evidence of high small bowel obstruction and a malrotated microcolon on preoperative roentgenogram with barium enema should suggest this diagnosis. Five families, including ours, have been reported in which more than one child was affected, and it has been suggested that this disorder is transmitted by an autosomal recessive gene. However, the occurrence of conventional intestinal atresia in other siblings, the association with multiple atresias, and discordance in a set of apparently monozygotic twins indicate that there may be a more complex spectrum of genetic transmission. Subsequent siblings are at increased risk for apple peel atresia or related malformations. Prenatal ultrasound can facilitate early diagnosis and treatment.
Anastomosis, Surgical↗
[Perioperative management of a neonate with aglossia-adactylia syndrome].
Aglossia-adactylia syndrome is a rare disorder characterized by aglossia and deformity of the limbs of variable degree. We managed a 2-day-old neonate with ileojejunal atresia with aglossia-adactylia syndrome. He was scheduled for the repair of the intestinal atresia, gastrostomy and palatal impression taking, under general anesthesia with sevoflurane supplemented with fentanyl. In this case we could achieve tracheal intubation by direct laryngoscopy with cricoid pressure applied, and could manage without any complications. But many of the patients with this syndrome are complicated with cleft palate and micrognathia, and we should be careful of the difficult airway at the induction of general anesthesia and aspiration during perioperative period.
Abnormalities, Multiple↗
Familial combined duodenal and jejunal atresia.
Combined duodenal and jejunal atresia is extremely uncommon. The familial occurrence of congenital duodenal and small bowel atresia is even more unusual. To the authors' knowledge, this is the first report of two siblings with simultaneous duodenal and jejunal atresia who underwent successful surgical repair. The report may support the genetic origin of some forms of high intestinal atresia.
Abnormalities, Multiple↗
[Surgical treatment of atresia of the small intestine].
Three cases of atresia of the intestine, one of which with multiple atresia of the small intestine, are reported. All three cases recovered. The surgical procedures applied for correcting atresia are discussed. In all the cases end-to-back anastomosis in an extramucous layer was performed, extramucous suture being considered as sufficiently resistant and having the advantage of not stenozing the intestinal luman. This type of suture proved efficient not only in the case of multiple atresia but also in the presence of peritonitis lesions.
Humans↗
Polysplenia complex and duodenal atresia. A case report.
The association between splenic abnormalities, congenital heart defects, and abnormalities in the location and shape of other viscera is well established in the literature. Intestinal atresia is rarely found with either the polysplenia or asplenia syndromes. We studied a case of polysplenia syndrome accompanied by duodenal atresia; we believe that duodenal atresia is a rare but predictable complication of the polysplenia complex.
Adult↗
Surgical repair of atresia jejuni in two calves.
Two Holstein calves with similar histories of not defecating since birth were determined to have atresia jejuni. Resection of the distended blind end of the jejunum and the remaining jejunum and ileum followed by a jejunocecostomy was performed. One calf died 7 months after surgery of unknown causes. The second calf has survived for greater than 1 year, and has maintained a growth rate similar to other calves its age. Atresia jejuni has been reported to be a universally fatal disorder of unknown pathogenesis. It has been postulated that intestinal atresia is caused by palpation of the amniotic vesicle at the time of pregnancy diagnosis, between days 36 and 42 of gestation.
Anastomosis, Surgical↗