Necrotizing enterocolitis with gastric pneumatosis

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ESPR434
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History: 


A preterm female infant born by caesarean section at 28 weeks' gestation weighing 850g after failed induction of labor.  The pregnancy was complicated by pre-eclampsia with severe features and ultimately non-reassuring fetal heart rate tracing and failure to progress. Maternal antibiotics, magnesium, nifedipine, labetelol, and betamethasone were given prior to delivery.  Infant received positive pressure ventilation (PPV) via t-piece for poor respiratory effort with a maximum 0.3 FiO2 in the delivery room and was transitioned to continuous positive pressure ventilation (CPAP) of 5cm H2O on 0.21 FiO2.  Apgar scores assigned were 3 and 8 at 1 and 5 minutes of life.   Central access was obtained with umbilical venous and arterial catheter placement.  At approximately 6 hours of life, she was intubated and given surfactant for an increasing oxygen requirement secondary to respiratory distress syndrome (RDS) and placed on mechanical ventilation.   She remained intubated until DOL 3 when she was extubated to non-invasive positive pressure ventilation (NIPPV) and remained stable.  Enteral feeds were initiated on day of life (DOL) 1 with breast milk and by DOL 9 had reached full enteral feeds per tube with human donor or maternal breast milk with human milk derived fortifier at 26 calories per ounce; feeds were tolerated well.  


On the evening of DOL 9 she had an increase in abdominal girth by 2cm, emesis, hypoactive bowel sounds and a dusky appearing abdomen with tenderness on examination. Abdominal x-ray showed a mottled appearance throughout abdomen most consistent with pneumatosis intestinalis and necrotizing enterocolitis (NEC), additionally there was an atypical curvilinear lucency in the left upper quadrant concerning for gastric pneumatosis, there was no portal venous gas or pneumoperitoneum. Patient was made NPO, replogle placed, blood cultures drawn, and antibiotics started. Surgery was called and they came to examine patient. Due to stability of patient there was no indication to emergently perform surgery. The next day patient continued to have stable vitals and did not require increased respiratory support. The patient was stable on NIPPV; however, there were concerns that any increased positive air pressure to the stomach could lead to gastric perforation due to the presence of extensive gastric pneumatosis.


Blood culture grew Klebsiella pneumoniae after 8 hours and antibiotics switched to meropenem for better coverage.  Repeat imaging showed improved but residual pneumatosis and the patient developed severe thrombocytopenia requiring multiple platelet transfusions. On DOL 13 the decision made to operate due to medical intractability with worsening thrombocytopenia and abdominal distension. In the operating room patchy areas of necrotizing enterocolitis were found but were not full thickness and would like recover without removal. The distal ileum was found to perforated 10 cm proximal to ileocecal valve and a large perforation was found in the cecum. Stomach was fully viable and distended with no signs of perforation. The perforated piece of the ileum was removed and the cecal perforation was closed. Terminal ileostomy and mucous fistula were brought out and found to be viable. Patient tolerated surgery well and was transferred back to NICU.  


Patient completed full course of antibiotics. After surgery patient did exceptionally well; she was tolerated advancement of feeds and was brought back to OR on DOL 60 for re-anastomosis and ileostomy closure. After reversal patient advanced to full feeds and was able to be discharged home on DOL 76. 


 

Physical Exam: 

Vitals: T 36.5C (on 36.2C heat), HR 180, BP 54/32 (MAP 41), SpO2 95% on NIPPV FiO2 21%

Cardiac: RRR, S1/S2 appreciated, no murmurs 

Respiratory: Pt on NIPPV, breathing comfortably, clear breath sounds throughout. 

Abdomen: Distended and discoloured. Firm to palpation in lower quadrants. No bowel sounds. 

Extremities: Good perfusion, decreased tone from baseline 


Labs/Imaging:

CBC: WNL (initially) 

BMP: WNL

BCx: Klebsiellla pneumoniae 

X-ray: molted appearance throughout abdomen most consistent with pneumotosis/NEC. There was an atypical curvilinear lucency in the left upper quadrant most likely to signify prominent gastric pneumotosis. No portal gas or pneumoperitoneum


Procedures: 

On day of life 13 decision made to operate due to medical intractability with worsening thrombocytopenia and abdominal distension. In the operating room patchy areas of necrotizing enterocolitis were found but were not full thickness and would like recover without removal. The distal ileum was found to perforated 10 cm proximal to ileocecal valve and a large perforation was found in the cecum. Stomach was fully viable and distended with no signs of perforation. The perforated piece of the ileum was removed and the cecal perforation was closed. Terminal ileostomy and mucous fistula were brought out and found to be viable. Patient tolerated surgery well and was transferred back to NICU.  


Final Diagnosis: Necrotizing enterocolitis with significant gastric pneumatosis 


Discussion: 


What makes this case of NEC interesting was the additional finding of gastric pneumatosis and our decision to change respiratory management.  In review of the literature there is not a great deal of discussion of respiratory management in cases of NEC with gastric pneumatosis, often the decision to intubate is based on clinical status and/or need for surgical intervention. Our patient was stable on NIPPV but we elected to intubate based on the finding of severe gastric pneumatosis rather than respiratory decompensation or need for surgery.  This decision was made to avoid excessive additional pressure from the non-invasive ventilation due to concerns for the integrity of the gastric mucosa, leading to further damage or potential perforation. Our patient had improvement of gastric pneumatosis radiographically as well as no evidence of gastric perforation at the time of surgery.

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Albany Medical Center, Albany NY
Albany Medical Center, Albany NY

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