Initial Presentation of a Pediatric Intestinal Pseudo-Obstruction Episode After SARS-CoV-2 Virus (COVID-19) Infection

INTRODUCTION Pediatric intestinal pseudo-obstruction (PIPO) is an umbrella term describing disorders characterized by gastrointestinal (GI) dysmotility with variable course, severity, and onset. PIPO may account for up to 15% of pediatric intestinal failure cases, which can lead to use of parenteral nutrition (PN) supplementation, intestinal transplantation, and sometimes intestinal resection or the creation of ostomies (1,2). Patients present with symptoms of bowel obstruction in the absence of a mechanical etiology (3). Children with PIPO may also have involvement of smooth muscle viscera, including megacystis with polyhydramnios. The ESPGHAN consensus statement on diagnosis of PIPO requires at least 2 of the following 4 criteria: objective measure of small intestinal neuromuscular involvement, recurrent or persistently dilated loops of small intestine with air-fluid levels, genetic abnormalities, and the inability to maintain adequate nutrition or growth with enteral feeding (4,5). Recent advances in whole-exome sequencing have identified several mutations that can be pathogenic, commonly including the ACTG2 gene (1,3). Viral infections may alter the integrity of the enteric neuromuscular system and play a role in the development of pseudo-obstruction (6). Several case reports over the last 2 decades have reported EpsteinBarr, cytomegalovirus, varicella-zoster, and John Cunningham viral illnesses to be the causal agent of PIPO episodes, possibly due to acquired hypoganglionosis, myopathy, or neuropathy (7,8). While it has been suggested that infections, general anesthesia, psychologic stress, and poor nutritional status may serve as precipitating factors to exacerbations, triggers are largely unpredictable and unknown (9). The novel severe acute respiratory syndrome coronavirus 2 or COVID-19, is a single-stranded RNA virus with emerging evidence for significant GI involvement. Several recent publications have found that 9%–20% of COVID-19 patients experience nausea, vomiting, diarrhea, anorexia, hepatic inflammation, or abdominal pain (10). We believe this virus may also represent a potential trigger for PIPO episodes. CASE REPORT We present the case of a 7-month-old male with history of megacystis, bilateral hydronephrosis, and constipation who presented with acute onset of abdominal distension and bilious emesis. Due to concern for bowel obstruction, he underwent an exploratory laparotomy which did not demonstrate a mechanical etiology. Abdominal radiograph comparison with previous imaging showed persistently dilated bowel loops (Figs. 1 and 2). Barium enema was normal and without evidence of microcolon. Upper GI with small bowel followthrough demonstrated significant delayed emptying of contrast from the small bowel. Rectal biopsy was negative for Hirschsprung’s disease. There were no prenatal anatomic abnormalities or polyhydramnios. There was no history of delayed passage of meconium. He developed constipation at 4 months of age, requiring 2 admissions for bowel clean-out in the setting of abdominal distention. Work up for diabetes and thyroid disease were unremarkable. Approximately 1 month before this presentation, the patient and his family tested PCR positive for COVID-19. Repeat PCR testing at time of admission was also positive. The patient did not develop any extraintestinal symptoms while his family members experienced fever, anosmia, nausea, vomiting, and abdominal pain.

[1]  Changzhen Zhu,et al.  Latest developments in chronic intestinal pseudo-obstruction , 2020, World journal of clinical cases.

[2]  D. Sandhu,et al.  Gastrointestinal and hepatic manifestations of COVID-19: A comprehensive review , 2020, World journal of gastroenterology.

[3]  M. Sood,et al.  Pediatric Intestinal Pseudo-obstruction in the Era of Genetic Sequencing , 2019, Current Gastroenterology Reports.

[4]  B. Barth,et al.  ACTG2-Associated Visceral Myopathy With Chronic Intestinal Pseudoobstruction, Intestinal Malrotation, Hypertrophic Pyloric Stenosis, Choledochal Cyst, and a Novel Missense Mutation , 2018, International journal of surgical pathology.

[5]  Wei Cai,et al.  Causes and prognosis of chronic intestinal pseudo-obstruction in 48 subjects , 2018, Medicine.

[6]  C. Di Lorenzo,et al.  Paediatric Intestinal Pseudo-obstruction: Evidence and Consensus-based Recommendations From an ESPGHAN-Led Expert Group , 2018, Journal of pediatric gastroenterology and nutrition.

[7]  J. Milunsky,et al.  Diagnosis of Chronic Intestinal Pseudo-obstruction and Megacystis by Sequencing the ACTG2 Gene , 2017, Journal of pediatric gastroenterology and nutrition.

[8]  C. Faure,et al.  Pediatric Chronic Intestinal Pseudo-obstruction , 2017 .

[9]  M. Selgrad,et al.  Chronic intestinal pseudo-obstruction related to viral infections. , 2010, Transplantation proceedings.

[10]  M. Besnard,et al.  Intestinal pseudo-obstruction and acute pandysautonomia associated with epstein-barr virus infection , 2000, American Journal of Gastroenterology.