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Neonatal gastric perforation is rare and life-threatening condition period and can be associated with overdistension, gastric intubation, ischemia, or idiopathic causes. Infants with this complication have a high mortality (25–50%). Typically there is a constellation of symptoms which can help identify this condition. These symptoms include fever, sepsis, abdominal distension, hematemesis, pneumoperitoneum, and respiratory failure. Recognition of the symptoms allows preoperative preparation including endotracheal intubation for respiratory failure, volume resuscitation, administration of antibiotics, and possible abdominal decompression. Traditional operative approaches and laparoscopy have been employed to correct this abnormality. Operative principles include control of spillage, debridement of devitalized tissue, and multiple repair techniques. Future investigation includes improvements in the understanding of the underlying causes of idiopathic and spontaneous perforation, improvement in prevention of the perforations of known cause, and advancement of repair techniques and approaches.
KeywordsGastric perforation Pneumoperitoneum Stomach Trauma Ischemia
Gastric perforation in the neonatal period is rare; however, it continues to be associated with significant morbidity and mortality. Spontaneous neonatal gastric perforation is estimated to occur in 1 in 2,900 live births (Rosser et al. 1982) and accounts for approximately 10–15% of all gastrointestinal perforations in neonates and children. Gastrointestinal perforations occur more commonly in males; however, there appears to be no sex predilection for those occurring in the stomach (Bell 1985). Recent series may suggest a male predominance, but this remains inconclusive (Duran et al. 2007). The incidence of gastrointestinal perforation is increasing in some populations; however, the relative incidence of gastric perforation is decreasing (Terui et al. 2012). The terminology used to describe neonatal gastric perforation has been inconsistent, and its etiology remains a topic of debate. Spontaneous or idiopathic gastric perforations refer to those with no identifiable underlying cause and account for the majority of gastric perforations in most reported series (Rosser et al. 1982; Kara et al. 2004). Nevertheless, many pediatric surgeons believe that an underlying cause can be found in most cases of neonatal gastric perforation (Leone and Krasna 2000).
Siebold, in 1826, is credited with the first description of a gastrointestinal perforation with no demonstrable cause, the so-called spontaneous perforation (Siebold 1826). In 1929, Stern et al. (1929) reported attempts at surgical repair. Agerty et al. reported the first successful repair of a neonatal intestinal (ileum) perforation in 1943 (Agerty et al. 1943), and Leger, in 1950, described the first successful repair of a neonatal gastric perforation (Leger et al. 1950). Survival following a neonatal gastric perforation was rare prior to the 1960s. While mortality has improved since that time, it remains significant and ranges from 25% to over 50% in most series (Rosser et al. 1982).
Causes and associations of neonatal gastric perforation
Pyloric atresia (Burnett and Halpert 1947)
Duodenal atresia (Holgersen 1981)
Midgut volvulus (Miller 1957)
Congenital deficiency of gastric muscle
Positive pressure ventilation
Corticosteroids (O’Neil et al. 1992)
Recent studies suggest a deficiency of the tyrosine kinase receptor C-KIT+ mast cells, and a lack of C-KIT+ interstitial cells of Cajal may contribute to idiopathic gastric perforation (Ohshiro et al. 2000). Mice lacking C-KIT+ mast cells develop spontaneous gastric ulceration or perforation. Pathologic specimens from non-necrotic portions of the stomach in six spontaneous gastric perforation patients showed a decreased number of interstitial cells of Cajal (Jactel et al. 2013). In addition, postmortem examination of stomachs of neonates, who died of idiopathic gastric perforation, revealed a deficiency in both C-KIT+ mast cells and interstitial cells of Cajal when compared to controls. The authors suggest that these abnormalities could result in impaired immunity and abnormal motility predisposing to gastric perforation (Yamataka et al. 1999).
The clinical presentation of gastric perforation is variable. The majority of cases present within the first 7 days of life; however, later presentations are reported (Bell 1985). The neonates are often premature or have a history of asphyxia or hypoxia (Holgersen 1981). Neonates may present with feeding intolerance or emesis that may contain blood. Many develop abrupt onset of rapidly progressive abdominal distension from pneumo- or hydroperitoneum (Aydin et al. 2011). These infants progress to respiratory distress, hemodynamic instability, and signs of shock such as hypothermia, cyanosis, poor peripheral perfusion, and low urine output. The abdomen may rapidly become tense and tender with signs of peritoneal irritation. Ventilation may be impaired or ineffective until the abdomen is decompressed. Subcutaneous emphysema in the abdominal wall or pneumoscrotum may be appreciated (Aslan et al. 1999). Infants with posterior perforations into the lesser sac may present with a more insidious course, making the diagnosis difficult.
Infants with perforation secondary to an underlying process often have evidence of the predisposing condition such as findings of tracheoesophageal fistula, duodenal atresia, malrotation, or diaphragmatic hernia (Holgersen 1981). In some instances, a secondary cause is found at the time of operation. In cases of iatrogenic perforation, a history of traumatic naso- or orogastric intubation, prior surgery, corticosteroid or nonsteroidal administration, and aggressive ventilation or cardiopulmonary resuscitation may be obtained (Graivier et al. 1973).
The differential diagnosis is broad and includes conditions that cause sudden deterioration in the newborn and conditions that produce vomiting and abdominal distension. Conditions causing cardiovascular collapse include sepsis, pneumothorax, cardiac dysfunction, intraventricular hemorrhage, electrolyte abnormalities, hypoglycemia, necrotizing enterocolitis, perforated viscus, and malrotation with midgut volvulus. Conditions associated with vomiting and abdominal distension include Hirschsprung’s disease, intestinal atresia, meconium ileus, meconium plug syndrome, imperforate anus, perforated viscus, necrotizing enterocolitis, and midgut volvulus.
Infants with gastric perforation develop septic parameters and need to be resuscitated accordingly. Neonates may become unstable prior to the development of free intra-abdominal air. Infants who develop respiratory distress require intubation, and increased ventilator support is needed as the abdomen becomes more distended. Appropriate laboratory investigations include blood cultures, white blood cell count, hemoglobin, hematocrit, platelet count, electrolyte profile, and blood gas analysis. Broad-spectrum antibiotics should be initiated. Fluid boluses and blood transfusions are given to achieve hemodynamic stability and adequate urine output. An oro- or nasogastric tube should be carefully passed and placed on low intermittent suction. Once free, intra-abdominal air is identified, the patient is stabilized, and a laparotomy should be performed. Aspiration of the peritoneum with an IV cannula when an overly distended abdomen is impeding ventilation can be a life-saving measure (Touloukian 1973). In select cases, peritoneal drainage has been reported with resolution of the peritonitis and healing of the perforation (Aydin et al. 2015).
Following repair of the perforation, the abdomen is lavaged with warm saline. Peritoneal drainage is not needed for most primary repairs and has not been shown to reduce postoperative complications but is used routinely by some surgeons. The fascia and skin are closed in standard fashions. Postoperatively, supportive and resuscitative care is continued. The child is maintained on broad spectrum antibiotics, gastric acid suppression therapy, and total parenteral nutrition. The stomach should be decompressed. Feedings are held until the infant has stabilized. Many surgeons obtain a contrast study prior to initiating enteral feeds.
The surgical management of gastric perforation in neonates has remained largely unchanged. However, the overall mortality is changing. Yang et al. (Yang et al. 2018) reported results in the largest cohort of neonatal perforations in 68 patients and found that the overall mortality had decreased from 100% during 1980–1989 to 16% during 2010–2016. Similarly, a Japanese study showed a mortality rate of 14% among 42 neonates with gastric perforations (Sato et al. 2017). Chen et al. (Chen et al. 2018) reviewed 168 cases of gastric perforation in the newborn and reported that the single most prognostic factor was prematurity, with the mortality being significantly higher in preterm neonates.
Conclusion and Future Directions
Gastric perforation in the newborn is a rare event, but with early recognition, appropriate care and adherence to principles of surgical management survival can be maximized. Causes of gastric perforation may be multiple including idiopathic and spontaneous perforation. Recognition of the patterns of symptoms related to gastric perforation will aid in identification of this rare clinical condition. Careful attention to preoperative preparation will aid in a successful repair. Operative principles include control of spill of the luminal contents, debridement of devitalized tissue, and closure of the defect. Postoperative care includes supportive measures, broad spectrum antibiotics, and gastric acid suppression. Nutrition plays a key role in healing with TPN until the stomach is healed. Contrast study of the stomach may be considered prior to initiation of intestinal feeds.
Gastric perforation remains a poorly understood and rare event in the neonatal period. Associated risk factors are poorly characterized, and future investigation may help define which patients are at the highest risk (Yang et al. 2015). These patients may benefit from gastric acid suppression or other trophic factors to support the stomach. Gastric perforation may be in the same spectrum as necrotizing enterocolitis or spontaneous intestinal perforation. Investigation related to improved understanding of those processes may provide improvement in the understanding of gastric perforation.
Previous studies have shown the relationship of the C-KIT+ mast cells and the interstitial cell of Cajal to gastric perforation. These underlying cellular and molecular deficiencies are poorly characterized and may represent future targets for therapy or possible preventive strategies.
Finally, technology has assisted in the development of minimal access techniques and minimal access instrumentation. Future instrumentation may be even smaller, and this micro-access approach may allow even more technically complex procedures in the neonate. Advancements also allow endoscopic procedures in the neonate which have been limited up to this point by the size of endoscope and the available instrumentation. In humans and animal models, gastric perforation has been successfully approached using endoscopic clips (Maekawa et al. 2015) and suturing techniques (Halvax et al. 2015).
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