Acute acalculous cholecystitis in an infant after gastroschisis closure

Abstract

Gastroschisis is a common type of congenital anterior abdominal wall defect with intraabdominal organs exposed outside the abdominal cavity. With modern neonatology and surgical practices, the overall prognosis for infants with gastroschisis is excellent. However, a subset of infants with gastroschisis will develop complications, requiring repeat surgical interventions. We present a case of a female infant with complicated gastroschisis who developed acute perforated acalculous cholecystitis, which was accurately diagnosed with abdominal ultrasound and successfully treated with medical management and a percutaneous cholecystostomy tube.

Gastroschisis is a congenital defect of the full-thickness periumbilical abdominal wall in which abdominal contents do not return appropriately to the abdomen during embryological development, usually associated with evisceration of bowel and sometimes other abdominal organs. Advances in perinatal care, closure techniques, and postoperative parenteral and enteral nutritional support contribute to a survival rate above 90%, even in cases of complicated gastroschisis.^1,2 Congenital anomalies and postsurgical complications of the biliary system are extremely rare in patients with gastroschisis. Choledochal cyst,³ gallbladder atresia, biliary atresia,⁴ gallbladder evisceration,⁵ choledocholithiasis in an adolescent with gastroschisis closure as neonate, cholestasis, and biliary obstruction in infants who had undergone gastroschisis closure have been reported.^6,7 To our knowledge, there have been no reports of acalculous cholecystitis. Herein, we report a case of acute perforated acalculous cholecystitis in an infant who had undergone gastroschisis closure.

CASE DESCRIPTION

At 24 weeks’ gestation, a female fetus was diagnosed with gastroschisis by routine fetal ultrasound. At 36 weeks and 6 days, she was born small for gestational age (2320 g) by spontaneous vaginal delivery to a 21-year-old G2P1001 mother. Apgar scores were 7 at 1 minute and 9 at 5 minutes of life, and no resuscitation was required. She was managed with gastroschisis protocol (preheated warmer, right side down, occlusive clear plastic bag up to the axilla, nothing by mouth, gastric decompression with a Replogle to low continuous suction, peripherally inserted central catheter access, ampicillin, gentamicin, and intravenous acetaminophen). At birth, the surgical team immediately evaluated the patient and placed a Silastic silo, as the bowel was edematous and could not be reduced into the abdomen.

On day of life (DOL) 5, the patient underwent a partial ileectomy (approximately 8 cm of the ileum was resected) for bowel necrosis with subsequent anastomosis on DOL 55. Postoperatively, dexmedetomidine, fentanyl, and vecuronium infusions were utilized. On DOL 61, return of bowel function occurred and feeds were started at 10 mL/kg/day and advanced slowly. In the following weeks, feeds were paused intermittently due to sporadic bouts of emesis without abnormality on abdominal x-rays and blood tests. On DOL 85, the patient developed fever and generalized abdominal tenderness. An order was given for nothing by mouth, total parenteral nutrition was initiated, and vancomycin and gentamicin were started empirically. Abdominal x-ray showed a nonobstructive bowel pattern, and septic workup was notable for an elevated white blood cell level of 22,000 and a C-reactive protein of 67 mg/L (normal 0.0–3.2 mg/L). Abdominal ultrasound on DOL 87 showed a 4 mm thickened and hyperemic gallbladder wall with sludge within the gallbladder, suspicious for acute cholecystitis (Figure 1). The patient’s symptoms persisted and the C-reactive protein continued to rise to 256 mg/L. Antibiotics were switched to piperacillin/tazobactam and fluconazole for empiric fungal treatment. Repeat ultrasound on DOL 90 showed increased sludge within the gallbladder, persistent gallbladder wall thickening, and a new focal disruption of the gallbladder wall with a 2.1 × 1.4 cm hypoechoic structure adjacent to the gallbladder, consistent with gallbladder perforation with pericholecystic abscess (Figure 1).

Figure 1.

(a) Initial longitudinal and (b) transverse ultrasound images of the gallbladder (GB) demonstrate sludge (S) within the gallbladder with wall thickening (calipers). (c) Follow-up longitudinal ultrasound image 3 days later shows a focal disruption of the gallbladder wall (arrow) with a new adjacent pericholecystic fluid collection (FC). (d) Percutaneous cholangiogram at cholecystostomy tube removal demonstrates contrast freely flowing into the gallbladder (GB), common bile duct (CBD), and duodenum (D) without contrast extravasation.

Due to recent laparotomies and acute decompensation, surgical intervention was deemed to be an unacceptable risk. Interventional radiology proceeded to successfully place a percutaneous cholecystostomy tube under ultrasound and fluoroscopic guidance. Initially, 5 mL of purulent bilious fluid was drained from the cholecystostomy tube. Escherichia coli and Klebsiella pneumonia grew from the cholecystostomy drainage fluid, which were both resistant to piperacillin/tazobactam. Antibiotics were switched to ampicillin and ceftazidime after consultation with pediatric infectious disease. The patient’s clinical signs and symptoms and laboratory values improved quickly.

On DOL 97, ultrasound showed a decrease in the size of the pericholecystic abscess. Ceftazidime was stopped after completion of a 10-day course and ampicillin was continued. One month after cholecystostomy tube placement (DOL 121), percutaneous cholangiogram confirmed the absence of biliary leak and the cholecystostomy tube was removed without complication (Figure 1). Barium enema was later performed and demonstrated intestinal stenosis at a previous anastomosis site, which prompted an exploratory laparotomy, adhesion lysis, and resection of the prior small bowel anastomosis with formation of a new ileoileostomy. Ampicillin was continued for 6 weeks. On DOL 132, oral feed with expressed breast milk was started at 10 mL/kg/day and advanced slowly to reach full feeds at 160 mL/kg/day. The patient was subsequently discharged home on DOL 167 on oral and nasogastric tube feeds with follow-up with her pediatrician and neonatal intensive care unit high risk, pediatric dietary, gastroenterology, and surgery clinics as an outpatient.

DISCUSSION

Acute acalculous cholecystitis (AAC) is defined as an acute inflammatory disease of the gallbladder in the absence of cholelithiasis. Tsakayannis et al⁸ estimated only 1.3 pediatric cases for every 1000 adult cases. AAC is believed to arise by two main mechanisms: ischemic injury from hypoperfusion and chemical injury from bile stasis. Moreover, concomitance and/or superimposition of infection with enteric pathogens including E. coli, Enterococcus faecalis, Klebsiella pneumonia, Pseudomonas, Proteus species, and Bacteroides⁹ contribute to the pathogenesis of AAC. Without immediate treatment, there may be rapid progression to perforation, gangrenous cholecystitis, or bile peritonitis.

Risk factors for AAC include infectious diseases, shock (i.e., sepsis, trauma, burns), multiple transfusions, immune-mediated diseases, immunocompromised states, renal disease, genetic disease, congenital anomalies of the biliary tree with cystic duct obstruction, prolonged periods of fasting, and use of opiates in postoperative or severely ill patients.¹⁰

The diagnosis of AAC is a clinical challenge that requires a high index of clinical suspicion. Abdominal ultrasonography is a noninvasive and easily obtained test that can be helpful in the diagnosis of AAC. The presence of at least two of the following ultrasound criteria, in addition to the absence of gallstones, usually supports the diagnosis of AAC: (1) increased gallbladder wall thickness (>3 mm);¹¹ (2) pericholecystic fluid; (3) presence of echogenic material/mucosal membrane sludge; and (4) gallbladder distension.^11–13

While some children with AAC can be safely managed nonoperatively^13,14 with measures such as suspension of oral feeding, evacuation of gastric contents, administration of parenteral nutrition, effective pain and fever control through nonopioid medications, and antibiotic therapy covering both gram-negative and anaerobic microorganisms,^14,15 procedural interventions such as cholecystectomy or cholecystostomy are often needed for source control in the setting of AAC complicated by infection.

AAC carries high morbidity and mortality and is often seen as a complication of severe illness. The poor predictors of AAC mortality include the presence of thrombocytopenia, anemia, hypofibrinogenemia, gallbladder sludge, presence of pericholecystic fluid, septic shock, and sepsis plus hepatitis.^16,17 The reported mortality in children varies from 10% to 20%.^17,18

In our case, lack of enteral feeding, administration of total parenteral nutrition, opiates, and abdominal surgery predisposed the patient to delayed gallbladder emptying, which led to bile stasis and increased intraluminal pressure. This static environment also increased seeding and growth of enteric pathogens E. coli and K. pneumonia, ultimately leading to the development of gallbladder perforation and pericholecystic abscess. The combination of three of the aforementioned ultrasound criteria (gallbladder distension, wall thickness, and sludge) on the initial ultrasound with progression of findings and development of gallbladder perforation with pericholecystic abscess on the following ultrasound prompted procedural intervention. The selection of percutaneous cholecystostomy using a transhepatic approach led to rapid recovery with normalization of the patient’s ultrasound examination, laboratory tests, and gallbladder function with retention of the gallbladder.

Disclosure statement

The authors report no funding or conflicts of interest. The parent of the patient gave written informed consent for the publication of this case report.