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  1. Pyloric stenosis is a medical and not a surgical emergency managed first by volume repletion. Hypochloremic, hypokalemic metabolic alkalosis is the classic derangement and once corrected, pyloromyotomy is the surgical intervention of choice.

  2. An international randomized multicenter trial did not find any strong evidence that infants exposed to general anesthesia for 1 hour during inguinal herniorrhaphy had any measurable neurocognitive or behavioral deficits at 5 years of age.

  3. Insufflation of the peritoneum with CO2 for laparoscopic procedures leads to increased intra-abdominal pressure (IAP) with sometimes clinically significant cardiorespiratory, neuro, and renal physiological changes.

  4. Intussusception is the most common abdominal emergency in patients less than 2 years of age with classic presentation of abdominal pain, and bloody (currant jelly) stool.

  5. Gastroschisis is usually an isolated abdominal defect, whereas omphaloceles are usually associated with a wide variety of abnormalities (cardiac defects, congenital diaphragmatic hernia, chromosomal anomalies).

  6. Congenital diaphragmatic hernia (CDH) presents at birth with respiratory distress-tachypnea, grunting, use of accessory muscles, and cyanosis. Prognostic indicators include the degree of lung hypoplasia and the presence of pulmonary hypertension.

  7. The classic presentation of appendicitis includes periumbilical abdominal pain with anorexia, nausea, and vomiting. Leukocytosis and fever may be present. Transabdominal ultrasound (US) is the most common diagnostic modality.

  8. Hirschsprung disease or congenital intestinal agangliosis is the most common cause of intestinal obstruction in neonates. Neonates who fail to pass meconium within 48 hours of life should be assessed for this disorder.

A wide variety of gastrointestinal surgeries for various types of clinical disorders are performed in pediatric patients. Since the majority of disorders involve neonates and young infants, a general understanding of the embryological development would be helpful to appreciate the pathology and the clinical presentations of those disorders.

In this chapter, we will provide clinical presentations and treatment options, and brief descriptions of the anesthetic management of several pediatric diseases related to the gastrointestinal system: pyloric stenosis, inguinal hernia, umbilical hernia, laparoscopic surgery, intussusception, gastroschisis, omphalocele, congenital diaphragmatic hernia, appendicitis, aganglionic megacolon (Hirschsprung disease).


Hypertrophic pyloric stenosis is the most common gastrointestinal surgical disorder in neonates. This disorder was first reported in 1717 by Patrick Blair, but a complete description with autopsy findings was then described by Harald Hirschsprung in 1887. The operative procedure of pyloromyotomy for pyloric stenosis was first described by Ramstedt in 1912.

The incidence of pyloric stenosis (PS) is 0.9 to 5.1 per 1000 live births1 and is more common in first born male infants. It usually presents at 3 to 8 weeks of age. The primary pathology is segmental hypertrophy of circular muscle fibers of the pyloric region of the stomach, resulting in gastric outlet obstruction. No definite inheritance pattern is noted, but the possibility of polygenic mode of inheritance pattern influenced by environmental factors has been suggested. Erythromycin, a motilin antagonist,2 and maternal smoking...

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