Zankl A, Stähelin J, Roth K, Boudny P, and Zeilinger G, Children’s Hospital of Aarau (ZA, SJ, RK, ZG) and Institute of Pathology (BP), Aarau, Switzerland

Published: September 1, 2002

Case presentation
This male infant, twin A, was born to a 34-year-old GIII/PIII by cesarean section at 37 0/7 weeks of gestation after an uneventful pregnancy. The newborn adapted well, with a cord-pH of 7.23 and Apgar scores of 7/9/10. The birth weight was 2470g. Physical examination was unremarkable, except for hypothermia, for which the patient was admitted to our neonatal ward. Adaptation of twin B was uneventful.

Initial routine laboratory studies revealed a low white blood cell count (WBC) of 8500/ mm3 with an absolute neutrophil count of 2590/mm3. Blood sugar, C-reactive protein and capillary blood gas analysis were normal. Body temperature stabilized, the clinical exam remained unremarkable and the patient was discharged back to the maternity ward.

At 72 hours of age the leukocyte count had decreased to 5100/ mm3 with an absolute neutrophil count of 970/ mm3. When the leukocyte count fell to 4600/ mm3 with an absolute neutrophil count of 780/ mm3 at 96 hours of life, with still normal values for CRP and blood sugar, the patient was admitted to the NICU.

Over a period of 6 hours the infant became increasingly irritable, tachycardic and tachypneic. The abdomen was tender and distended. He developed emesis, which subsequently turned bilious. A nasogastric tube was inserted, feedings were discontinued and intravenous fluids and antibiotic therapy were started. Abdominal radiographs were obtained (Fig. 1, 2) which demonstrated extraintestinal air, consistent with the diagnosis of intestinal perforation. Over the next hour, while preparations for laparotomy were under way, acute worsening of the abdominal distension occurred. Gastric aspiration of faecal stained fluids and occurrence of inguinal hernias complicated the clinical situation. Shortly before laparotomy the WBC had diminished further to a value of 1300/ mm3 with an absolute neutrophil count of 380/ mm3, a CRP was 2.5 mg/l and clotting studies were normal.

Fig. 1. Abdominal X-ray in supine position with vertical beam.	Fig. 2. Abdominal X-ray in left lateral decubitus position with horizontal beam.	Fig. 3. H&E stain of gastric specimen showing areas with necrotic gastric mucosa.

At emergency laparotomy an immense necrosis of the stomach with perforation extending from the greater curvature of the stomach to the cardia was observed. Peritoneal fluid cultures grew staphylococcus aureus as a mono–culture. Pathologic examination of the resected stomach tissue showed acute hemorrhagic necrosis (Fig. 3).

The postoperative course was complicated by bilateral small scrotal abscesses, one of which perforated spontaneously. Later on orchidopexie was needed. The patient was discharged after 21 postoperative days of hospitalisation.

Discussion
Gastric perforation is a rare condition that accounts for 10 to 16 % of all gastrointestinal perforations(1). The usual age of presentation is 2 to 7 days in term infants. The incidence is higher in black than in white infants and is at least 4 times higher in males then in females (2). Most cases of gastric perforation were considered spontaneous in the older literature, whereas more recent retrospective reviews reveal several associated gastrointestinal lesions and contributing factors (4).

Deficiencies in the gastric muscle layer have been described by a number of authors, but it is probable that muscle layer deficiencies are a result of perforation rather than the cause. In 1965 Shaw et al (5) experimentally proved the latter theory in dogs by tying off the esophageal and duodenal ends of the stomach and insufflating the organ with air until it perforated. All perforations occurred on the greater curvature, and when examined histologically, all specimens showed lack of musculature near the perforation. He concluded that this was a non-specific finding produced by mechanical rupture of the stomach, as a result of increased intragastric pressure. When distension is sufficient to force muscle bundles to separate from one another, rupture can occur in the weakened area.

Traumatic causes are described, especially secondary to feeding tube placement or vigorous respiratory resuscitation (2). Drug associated gastrointestinal perforation has been noticed under treatment with dexamethasone or indomethacin (5-6). Prematurity, low birth weight, necrotizing enterocolitis and isolated gastric ischemia due to hypoxia are considered to be additional risk factors (3). There seems to be an association with prenatal conditions such as premature rupture of membranes, amnionitis, gestational diabetes or maternal group B streptococcal infection leading to emergency cesarean delivery (2).

In our patient none of the above-mentioned maternal, prenatal or postnatal risk factors were identified. Nonetheless, we believe that our patient must have suffered from isolated gastric ischemia leading to the extended necrosis with neutrophil consumption. Pathologic examination of the resected stomach tissue showed acute hemorrhagic necrosis and was in our case non-contributory to elucidate the cause of the acute necrosis. Early diagnosis and rapid management allowed this infant to survive this rare abdominal catastrophe.

References
1. Holgersen LO. The aetiology of spontaneous gastric perforation of the newborn: reevaluation. J Pediatr Surg 1981;16:608-613 (Abstract)

2. Rosser SB, Clark CH, Elechi EN. Spontaneous neonatal gastric perforation. J Pediatr Surg 1982;17:390-394 (Abstract)

3. Leone RJ, Krasna IH. “Spontaneous” neonatal gastric perforation: is it really spontaneous? J Pediatr Surg 2000;35:1066-1069 (Abstract)

4. Shaw A, Blank WA, Santulli TV et al. Spontaneous rupture of the stomach in a newborn: A clinical and experimental study. Surgery 1965;58:561-571

5. O’Neil EA, Chwals WJ, O’Shea MD, Turner CS: Dexamethasone treatment during ventilator dependency: possible life-threatening gastrointestinal complications. Arch Dis Child 1992;67:10-11 (Abstract)

6. Grossfeld JL, Chaet M, Molinari F et al: Increased risk of nectrotizing enterocolitis in premature infants with patent ductus arteriosus treated with indomethacin. Ann Pdiatr Surg 1996;224:350-357 (Abstract)