Multiseptate gallbladder with gallstones in a pediatric patient

Abstract

Multiseptate gallbladder is a rare congenital anomaly characterized by multiple intraluminal septa. While often asymptomatic, it can present with abdominal pain or complications, such as gallstones. Cases of multiseptate gallbladder with gallstones have been rarely reported. We describe the case of a 15-year-old girl with multiseptate gallbladder complicated by cholelithiasis. No other biliary ductal anomalies were identified. Laparoscopic cholecystectomy was performed and the gallstones were identified as bilirubin stones. Complete symptom relief was achieved postoperatively. This case highlights that multiseptate gallbladder may contribute to bile stasis and pigment stone formation, even in the absence of other congenital anomalies. Early imaging is essential to confirm the diagnosis and guide management. Magnetic resonance cholangiopancreatography enabled exclusion of associated anomalies, guiding management.

Introduction

Multiseptate gallbladder (MSG) is an exceptionally rare congenital malformation, first described by Simon and Tandon in 1963 [1]. It is characterized by multiple thin septa traversing the gallbladder lumen, creating a honeycomb-like appearance on imaging. Fewer than 60 cases have been reported, with pediatric presentations being particularly uncommon [[2], [3], [4]]. Although MSG is often asymptomatic, reported symptoms include intermittent abdominal pain, nausea, and, less commonly, complications, such as gallstones or biliary sludge [4,5]. The pathogenesis remains uncertain; proposed embryologic mechanisms include excessive mucosal infolding during development (epithelial proliferation theory) or rapid elongation of the gallbladder, leading to redundant folding and twisting of the wall [2,3]. These developmental anomalies may also reflect broader disturbances in the coordinated formation of the hepatobiliary system, explaining occasional associations with other congenital biliary malformations [6]. Herein, we report a case of MSG in a symptomatic adolescent with coexisting cholelithiasis.

Case report

A 15-year-old girl presented with intermittent upper abdominal pain persisting for over 3 years. The pain was dull, localized in the epigastrium and right upper quadrant, and was not associated with nausea, vomiting, weight loss, or jaundice. Her medical and surgical histories were unremarkable and physical growth and development were age-appropriate. On presentation, her vital signs were within normal limits, with a body temperature of 36.8°C, heart rate of 73 beats/min, and blood pressure of 112/60 mmHg. There was no clinical jaundice. The abdomen was soft with mild epigastric tenderness, and no hepatosplenomegaly was palpable. Before presenting to our hospital, an upper gastrointestinal endoscopy performed elsewhere suggested possible superficial gastritis, and she received a brief course of antacids without symptom resolution. Laboratory investigations, including complete blood count and liver function tests, were within normal limits (Table 1).

Table 1.

Laboratory data on admission.

Parameter	Result	Reference rangea
CBC
WBC (/µL)	7,300	3,500–9,000
RBC (×10⁶/µL)	5.01	3.80–4.90
Hb (g/dL)	12.8	11.5–15.0
Ht (%)	39.9	34.0–45.0
PLT (×10³/µL)	243	150–350
Liver function tests
AST (U/L)	18	13–33
ALT (U/L)	11	6–27
ALP (U/L)	232	115–359
LDH (U/L)	168	119–229
T-Bil (mg/dL)	0.9	0.2–1.2
Alb (g/dL)	4.6	3.9–5.1
γ-GTP (U/L)	10	9–32
Inflammatory markers
CRP (mg/dL)	0.13	<0.30
Tumor markers
CEA (ng/mL)	2.01	<5.0
CA19-9 (U/mL)	<2.00	<37

^aReference ranges are for general adult populations in our institution. All laboratory parameters were within normal or near-normal limits.

AST, aspartate aminotransferase; ALT, alanine aminotransferase; ALP, alkaline phosphatase; Alb, albumin; CRP, C-reactive protein; CEA, carcinoembryonic antigen; CA19-9, carbohydrate antigen 19-9; Hb, hemoglobin; Ht, hematocrit; LDH, lactate dehydrogenase; PLT, platelet count; T-Bil, total bilirubin; γ-GTP, gamma-glutamyl transpeptidase; RBC, red blood cell count; WBC, white blood cell count.

Abdominal ultrasonography (US) revealed a normal-sized gallbladder containing multiple linear echogenic septations running transversely and longitudinally within the lumen, producing a distinct honeycomb-like appearance (Fig. 1). These septations lacked posterior acoustic shadowing, and the gallbladder wall thickness was normal. Several hyperechoic foci with shadowing were also observed in the dependent portion of the lumen, consistent with the presence of gallstones.

Fig. 1.

Ultrasonographic findings. (A) Transverse ultrasonographic view of the gallbladder demonstrating multiple fine internal echogenic septations crossing the lumen, forming a honeycomb-like appearance (arrow) and (B) Dependent portion of the lumen with multiple echogenic foci exhibiting posterior acoustic shadowing, consistent with gallstones (arrow).

Magnetic resonance cholangiopancreatography (MRCP) confirmed the presence of multiple fine septa arranged in various planes within the gallbladder lumen. The intrahepatic and extrahepatic bile ducts were normal in caliber and configuration. No choledochal cyst or pancreaticobiliary maljunction was evident (Fig. 2).

Fig. 2.

Magnetic resonance cholangiopancreatography (MRCP) findings. (A) MRCP image showing multiple thin intraluminal septations within the gallbladder lumen (arrow), consistent with multiseptate gallbladder (MSG) and (B) T2-weighted image showing no evidence of bile duct dilatation, choledochal cyst, or pancreaticobiliary maljunction.

Laparoscopic cholecystectomy was performed for symptom relief and to remove the gallstones. Intraoperatively, the gallbladder was externally unremarkable. Bile analysis within the gallbladder showed a pancreatic amylase level of 4 IU/L, providing biochemical support against pancreaticobiliary maljunction (PBM).

Gross examination of the resected specimen revealed thin internal septations dividing the lumen into multiple compartments (Fig. 3). The gallstones were dark and pigmented and were identified as predominantly bilirubin stones by chemical analysis. Histology confirmed the presence of fibromuscular septa lined with biliary epithelium, consistent with congenital MSG.

Fig. 3.

Gross and histopathological findings. (A) Gross specimen of the resected gallbladder showing multiple thin membranous septa dividing the lumen into compartments and (B) Histological section revealing septa composed of fibromuscular tissue lined with biliary epithelium, consistent with congenital multiseptate gallbladder. MSG, multiseptate gallbladder; MRCP, magnetic resonance cholangiopancreatography; PBM, pancreaticobiliary maljunction.

The patient’s postoperative recovery was uneventful, with complete resolution of abdominal pain reported at the 5-month follow-up.

Discussion

MSG is a rare congenital anomaly with an uncertain pathogenesis. Several hypotheses have been proposed, including the epithelial proliferation theory, which suggests that redundant mucosal infolding during gallbladder development leads to septal formation [4]. Another theory posits that rapid elongation of the gallbladder during the fetal period causes redundant folding and twisting of the wall, resulting in a multilocular appearance [5]. These theories may reflect broader disturbances in the coordinated development of the hepatobiliary system, which originates from the endodermal hepatic diverticulum.

MSG may present with abdominal pain or other nonspecific gastrointestinal symptoms. Pain is thought to result from transient bile flow obstruction through septal orifices or from uncoordinated gallbladder contractions causing compartmental pressure elevation [6]. MSG has been reported with other disorders in children, including gallstones and PBM, with or without choledochal cysts [7,8].

In a review by Amgad et al. [9], gallstones or biliary sludge were reported in 3 of 37 (8.1%) cases, whereas PBM with or without choledochal cysts was reported in 4 of 37 (10.8%) cases. Although this review had limitations such as publication bias, these comorbidity rates appear higher than expected in the general pediatric population, justifying careful assessment when MSG is identified. Gallstone formation may be related to bile stasis within the MSG. Conversely, MSG with concomitant PBM can be explained by fetal development of the hepatobiliary system. The gallbladder, cystic duct, and extrahepatic bile ducts all originate from the same endodermal hepatic diverticulum. According to Lemaigre, coordinated molecular signaling is crucial for proper morphogenesis of the biliary tract, and disruptions in this process may result in multiple concurrent structural anomalies [10]. Similar to our experience, a recently reported pediatric case of MSG with gallstones demonstrated the value of preoperative MRCP in confirming the diagnosis and excluding PBM, thereby guiding appropriate surgical management [11].

US remains the first-line modality for gallbladder evaluation. In the present case, US clearly demonstrated gallstones and multiple septa within the gallbladder; however, PBM could not be ruled out. Nondilated PBM, which lacks choledochal cyst dilatation, has been reported in association with MSG in pediatric patients [10]. The clinical importance of nondilated PBM is well established, as it may manifest symptoms and develop into malignancy over time [12,13]. MRCP clearly demonstrated a biliary tree and pancreatic duct without radiation exposure and excluded PBM in our case. Therefore, even in asymptomatic cases, a comprehensive evaluation of the biliary system using MRCP is warranted [9].

In addition to PBM and choledochal cysts, the differential diagnosis of gallbladder anomalies includes hyperplastic cholecystosis, such as adenomyomatosis, chronic cholecystitis, and other structural variants. Adenomyomatosis is characterized by gallbladder wall thickening with intramural Rokitansky–Aschoff sinuses, which may produce comet-tail artifacts on US and the “pearl necklace” sign on MRCP, features that help distinguish it from MSG, which shows fine septa without inflammatory wall thickening or sinuses. MRCP, in conjunction with US, provides complementary information to differentiate these entities and to evaluate the entire biliary tree [14]. Although surgical intervention is recommended if comorbidities, such as gallstones, are present, asymptomatic patients can be safely observed [15]. It is important to recognize the potential risk of gallstone formation with nonoperative management of MSG.

Conclusions

We report a rare pediatric case of MSG with gallstones. Although typically considered a benign anomaly, MSG can contribute to bile stasis and pigment stone formation. This case highlights the importance of recognizing structural gallbladder anomalies in children presenting with unexplained upper abdominal pain.

Availability of data and materials

The authors declare that all data in this manuscript are available within the article.

Ethics approval and consent to participate

Not applicable.

Patient consent

Written informed consent was obtained from the patient and her legal guardian for publication of this case report and accompanying images.