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Pathology - Gastrointestinal anomalies
Oesophageal atresia occurs in 1 in 3,500 live births. • Outcomes from improper segmentation of the foregut into the tracheal and esophageal pathways during the initial month of embryonic development. • In most instances, there exists a link between the distal esophagus and the trachea referred to as a tracheoesophageal fistula. • Neonates exhibit coughing and choking during feeding. Approximately fifty percent of impacted infants exhibit other congenital anomalies, with heart problems constituting the predominant cause of mortality in infants diagnosed with oesophageal atresia.
Duodenal atresia is less prevalent than esophageal atresia. • Correlated with Down's syndrome in 30% of instances. • Resulting from the failure of epithelial apoptosis and inadequate canalization of the intestinal lumen by eight weeks of gestation. • The obstruction typically occurs distal to the ampulla of Vater. • Prenatal ultrasonography reveals dilatation of the proximal duodenum and stomach accompanied by polyhydramnios.
Exomphalos • A defect in the anterior abdominal wall at the umbilicus, resulting in the protrusion of abdominal contents through the umbilicus. • The protrusion is encased in a fragile, transparent sac formed by amniotic membrane and peritoneum. • Occurs due to the failure of the midgut to retract into the abdomen from the umbilical coelom during embryonic development.
Gastroschisis • A defect in the front abdominal wall located adjacent to the umbilicus, through which intestinal loops extrude. • In contrast to exomphalos, there is an absence of a protective covering sac.
Malrotation • Abnormal placement of the intestine and mesentery resulting from inadequate rotation of the growing gut as it retracts from the umbilical coelom to the abdomen during development. A malrotated bowel typically exhibits a constricted mesenteric base, increasing the risk of volvulus around the superior mesenteric artery. • Impaired arterial blood flow results in ischemic necrosis of the complete midgut, from the duodenum to the transverse colon. • Necrosis induces hemorrhage within the intestine and significantly increases the risk of perforation. • In the absence of immediate surgical intervention, the situation may be lethal.
Meckel's diverticulum • A remnant of the vitellointestinal duct, which connects the primitive gut to the yolk sac. • Estimated prevalence in the normal population • The mucosa of the diverticulum may exhibit regions of gastric or pancreatic tissue (heterotopia). • The majority of children with Meckel’s diverticulum are asymptomatic. • The predominant symptom is painless rectal hemorrhage resulting from ulceration in a diverticulum harboring acid-secreting gastric mucosa. • Small bowel obstruction may furthermore arise, associated with intussusception or incarceration.
Imperforate anus • Comprehensive designation for any atretic ailment affecting the rectum or anus. • Lesions vary in severity from a stenosed anal canal to anorectal agenesis. • Surgically, they are classified as either high or low anomalies, based on the termination level of the bowel relative to the pelvic floor. • Low defects are simpler to rectify, and post-operative function is favorable. Higher abnormalities are more challenging to rectify, as they are more prone to being linked with fistulae between the rectum and the genitourinary tract, in addition to a compromised pelvic floor.
Hirschsprung disease • Not precisely a deformity, but a congenital gastrointestinal disorder characterized by the absence of ganglion cells from a variable length of the intestinal wall. • Outcomes resulting from the inability of neuroblasts to move from the esophagus to the anal canal during gestational weeks 5 to 12. • The lack of ganglion cells results in spasms within the aganglionic segment. • It manifests as intestinal obstruction and an inability to pass meconium within 24 hours post-birth. The rectal suction biopsy is the definitive method for diagnosing Hirschsprung's disease. The primary characteristic is the lack of ganglion cells in the submucosa and the presence of unusually thick nerve fibers in the mucosal layer.
Oesophageal atresia occurs in 1 in 3,500 live births. • Outcomes from improper segmentation of the foregut into the tracheal and esophageal pathways during the initial month of embryonic development. • In most instances, there exists a link between the distal esophagus and the trachea referred to as a tracheoesophageal fistula. • Neonates exhibit coughing and choking during feeding. Approximately fifty percent of impacted infants exhibit other congenital anomalies, with heart problems constituting the predominant cause of mortality in infants diagnosed with oesophageal atresia.
Duodenal atresia is less prevalent than esophageal atresia. • Correlated with Down's syndrome in 30% of instances. • Resulting from the failure of epithelial apoptosis and inadequate canalization of the intestinal lumen by eight weeks of gestation. • The obstruction typically occurs distal to the ampulla of Vater. • Prenatal ultrasonography reveals dilatation of the proximal duodenum and stomach accompanied by polyhydramnios.
Exomphalos • A defect in the anterior abdominal wall at the umbilicus, resulting in the protrusion of abdominal contents through the umbilicus. • The protrusion is encased in a fragile, transparent sac formed by amniotic membrane and peritoneum. • Occurs due to the failure of the midgut to retract into the abdomen from the umbilical coelom during embryonic development.
Gastroschisis • A defect in the front abdominal wall located adjacent to the umbilicus, through which intestinal loops extrude. • In contrast to exomphalos, there is an absence of a protective covering sac.
Malrotation • Abnormal placement of the intestine and mesentery resulting from inadequate rotation of the growing gut as it retracts from the umbilical coelom to the abdomen during development. A malrotated bowel typically exhibits a constricted mesenteric base, increasing the risk of volvulus around the superior mesenteric artery. • Impaired arterial blood flow results in ischemic necrosis of the complete midgut, from the duodenum to the transverse colon. • Necrosis induces hemorrhage within the intestine and significantly increases the risk of perforation. • In the absence of immediate surgical intervention, the situation may be lethal.
Meckel's diverticulum • A remnant of the vitellointestinal duct, which connects the primitive gut to the yolk sac. • Estimated prevalence in the normal population • The mucosa of the diverticulum may exhibit regions of gastric or pancreatic tissue (heterotopia). • The majority of children with Meckel’s diverticulum are asymptomatic. • The predominant symptom is painless rectal hemorrhage resulting from ulceration in a diverticulum harboring acid-secreting gastric mucosa. • Small bowel obstruction may furthermore arise, associated with intussusception or incarceration.
Imperforate anus • Comprehensive designation for any atretic ailment affecting the rectum or anus. • Lesions vary in severity from a stenosed anal canal to anorectal agenesis. • Surgically, they are classified as either high or low anomalies, based on the termination level of the bowel relative to the pelvic floor. • Low defects are simpler to rectify, and post-operative function is favorable. Higher abnormalities are more challenging to rectify, as they are more prone to being linked with fistulae between the rectum and the genitourinary tract, in addition to a compromised pelvic floor.
Hirschsprung disease • Not precisely a deformity, but a congenital gastrointestinal disorder characterized by the absence of ganglion cells from a variable length of the intestinal wall. • Outcomes resulting from the inability of neuroblasts to move from the esophagus to the anal canal during gestational weeks 5 to 12. • The lack of ganglion cells results in spasms within the aganglionic segment. • It manifests as intestinal obstruction and an inability to pass meconium within 24 hours post-birth. The rectal suction biopsy is the definitive method for diagnosing Hirschsprung's disease. The primary characteristic is the lack of ganglion cells in the submucosa and the presence of unusually thick nerve fibers in the mucosal layer.
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