M Can, M Kutlu, Ö Okur, M Ergin

Abstract

Purpose: Gallbladder perforations in infants are scarce and differ aetiologically and clinically from other biliary perforations and other age groups. In our study, we performed a literature review, including our case, to demonstrate these differences. Case Presentation: A 13-day-old boy was admitted for sudden onset of abdominal distension during treatment for jaundice. Laboratory evolution showed leukocytosis and hyperbilirubinaemia. Ultrasonography findings demonstrated a large volume of ascites. Emergency laparotomy was performed due to bile-stained ascites found on paracentesis. A cholecystectomy was performed. Rapid postoperative recovery was achieved. Conclusions: Gallbladder perforation is challenging to diagnose because it is scarce in infants and has a subacute course. Avoidable acquired factors almost always play a role in the aetiology. Simple cholecystectomy is curative in most patients and has a low mortality rate.

Introduction

Gallbladder perforation is a rare cause of acute abdomen in infants, and only 15 cases have been published.^1-13 To date, gallbladder perforations in infants have been analysed either with other age groups or with other biliary perforations. However, since it has characteristics that deserve to be considered separately from both groups, we aimed to reveal the unique features of this rare clinical entity by reviewing the previous publications with our new case.

Case Presentation

A 3675 g male baby born at term as the mother's second healthy baby of her second pregnancy by normal spontaneous vaginal delivery was admitted to a local hospital with a diagnosis of urinary tract infection on postnatal day six and was referred to our clinic because of sudden abdominal distention on postnatal day 13. On examination, he was found to have jaundice, abdominal distension with mild tenderness, and bilateral greenish communicating hydrocele. Laboratory tests revealed Hb 13 g/dL, WBC 14300/µL, CRP 6.8 mg/L, total bilirubin 14.8 mg/dL, direct bilirubin 4.8 g/dL, GGT 1247 IU/L, ALT 25 IU/L, AST 44 IU/L, ALP 240 IU/L, aPTT 33.8 s, PT 11.3 s, INR 0.99. Urinalysis showed protein 2+, bilirubin 3+, erythrocytes 3+, leukocytes 2+. Antibodies to toxoplasma, cytomegalovirus, and rubella were negative. Ultrasonography (USG) showed diffuse abdominal fluid and bilateral hydrocele. Emergency laparotomy was performed after paracentesis revealed ascites stained with bile (total bilirubin 16 mg/dL in peritoneal aspirate).

The abdomen was entered through a transverse right upper quadrant incision. Approximately 400 cc of biliary ascites was aspirated from the peritoneal cavity. The liver appeared normal. A 2 mm perforation was found in the gallbladder infundibulum (Figure 1). The perforation site was repaired with 5-0 Vicril, and an intraoperative cholangiogram was performed. It was observed that the contrast material filled the gallbladder but did not drain from the cystic duct into the common bile duct (Figure 2). Cholecystectomy was performed due to obstruction in the cystic duct. Before cystic duct ligation, a 5 Fr catheter was advanced into the choledoc and flushed with saline. The cystic duct was ligated after the choledoc was found to be open. No gallstones were observed. After saline irrigation of the peritoneal cavity, a drain was placed in the porta hepatis.

Figure 1 Perforation site at the infundibulum of the gallbladder.

Figure 2 Intraoperative cholangiography demonstrating bile duct obstruction.

Postoperative bilirubin and GGT levels improved rapidly. The biliary stool was observed on the third day, and feeding was started. Histologic examination showed that the gallbladder mucosa has oedematous and congestive changes with a 1-2 mm wall thickness. No congenital structural pathology obstructing the cystic duct was found. He was discharged on the 12th day after completing antibiotherapy for S. haemolyticus grown from the paracentesis material. Written consent was obtained from the family to publish this case.

Discussion

Infantile biliary perforations rarely originate from the gallbladder.^1,5,7,11,14 Only 16 cases, including ours, have been published (Table 1).

The mean age was 28.3 (IQR 6.5-46.5) days. Although low birth weight is typical in patients (n=4, 57%), our study shows this does not cause early disease presentation (p=0.43). Although it was more common in males (M/F=2.2, 2/1), the difference was insignificant (p=0.13). Due to the small number of reported cases, statistical studies provide a general idea about the disease.

The aetiology was unknown in half of the patients (n=8, 50%). In the remaining patients, the most common aetiology was gallstones (n=4, 25%). It has been reported in the literature that biliary perforation can be caused by stone obstruction, congenital obstruction, trauma, sepsis and ischaemia due to perinatal asphyxia or spontaneously (idiopathic).^1,5,7-10 However, no congenital obstruction of the biliary tract was found in any of the patients with gallbladder perforation, distinguishing infantile gallbladder perforation from other perforations of the biliary system. It has been suggested that the most likely etiologic cause for the group called spontaneous (idiopathic) perforation may be a congenital weakness in the gallbladder wall or obstruction caused by bile precipitated in the cystic duct.¹ In our case, an intraoperative cholangiogram showed that the perforation was caused by cystic duct obstruction. The presence of bile in the gallbladder excludes the possibility of congenital cystic duct obstruction. Histologic examination showed no congenital obstruction or inflammatory and ischaemic changes. However, since we did not see gallstones during surgery or on pathologic examination, it seems plausible that bile sediment deposited in the cystic duct caused the obstruction.

The most common findings are non-specific symptoms typical of the neonatal period, including abdominal distension (n=12, 75%), vomiting (n=6, 38%), and jaundice (n=6, 38%). In older children, the acute presentation is characterised by fever, pain, and peritonitis.¹⁴ On the contrary, the mean time from presentation to surgery was 6.2 (IQR 0-4.5) days, indicating that the disease follows a subacute clinical course in infants. As biliary ascites without associated infection are relatively well tolerated.¹ Early diagnosis based on clinical and laboratory findings is not always possible.^7,9 Cholestatic jaundice with regular liver function tests in a healthy newborn, biliary peritonitis with acholic stools, dark urine, and abdominal distention should suggest biliary perforation.^7,14

USG and computed tomography (CT) are not always helpful in identifying the source of free fluid in the abdomen.¹⁴ In children, a defect of the gallbladder wall can be detected by USG in only 38.4% of cases and by CT in 69.2% of cases.¹⁴ As in our case, a greenish discolouration of the abdomen or scrotum may facilitate the diagnosis. In this case, detection of biliary ascites by paracentesis should suggest a biliary perforation despite the high diagnostic specificity of magnetic resonance cholangiopancreatography.¹⁴ Biliary peritonitis is often overlooked due to the need for urgent laparotomy, and a definitive diagnosis is made at surgery.^2,9

Perforations are most common in the fundus (n=7, 50%) and neck (n=4, 28%). The fundus has relatively less blood flow.^9,10,14 In the present case, an infundibular perforation was found, which is rare.

Extrahepatic bile ducts were visualised in three patients (n=5) who underwent intraoperative cholangiography. Preoperative hepatobiliary iminodiacetic acid scanning was preferred in two patients and postoperative in one patient. Perioperative catheterisation and irrigation of the bile ducts are other methods used to rule out obstruction. In patients with extrahepatic bile ducts demonstrated by various methods (n=8, 50%), no obstruction was found, and no complications due to biliary obstruction were seen in any patient during postoperative follow-up. Extrahepatic biliary obstruction was not investigated in the remaining half of the patients, and no obstructive complications occurred during the postoperative period. This suggests that congenital obstructions are not involved in the aetiology of infantile gallbladder perforation. Therefore, the necessity of intraoperative cholangiography in infantile gallbladder perforation is controversial. Catheterisation and irrigation of the extrahepatic bile ducts may be sufficient to relieve secondary obstructions caused by gallstones or precipitated bile.

The most common treatment options were simple cholecystectomy (n=7, 43.8%) and cholecystostomy (n=4, 25%). Simple cholecystectomy is curative for gallbladder perforation after control of choledochal duct patency.¹⁴ Other surgical options include partial cholecystectomy and tube cholecystostomy, depending on the patient's condition.^2,9

Early surgical intervention before bacterial contamination of biliary ascites prevents adverse outcomes.¹⁴ Our review shows that the prognosis after surgical treatment is quite good. In the literature examined prior to our study, the follow-up periods for the documented cases ranged from 3 to 12 months. During these intervals, no occurrences of liver function deterioration, recurrent cholangitis episodes, or biliary obstruction were reported. In our case, the patient was lost to follow-up after 3 years, and no late complications were observed.

No mortality due to gallbladder perforation was reported, except for one patient who died due to multi-organ failure.

Gallbladder perforation in infants is difficult to recognise because it is scarce, and the definitive diagnosis is usually made at laparotomy. The slower clinical course compared to older children may delay diagnosis and lead to biliary peritonitis. It occurs secondary to avoidable acquired pathologies such as cholestasis, stones, and ischaemia rather than congenital obstruction of the extrahepatic bile ducts. Surgical treatment is more accessible than for extrahepatic bile duct perforation, and the prognosis is better. Each new case added to the literature will help us update our knowledge of this rare clinical entity.

Declaration of Interest

The authors declare that they have no conflicts of interest.

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