https://doi.org/10.53453/ms.2026.7.10
Management of acute cholangitis in a 3-year-old with hereditary
spherocytosis: challenges of percutaneous intervention following
failed ERCP
Joris Ribikauskas
1
, Dalius Malcius
2
1
Medical Academy, Faculty of Medicine, Lithuanian University of Health Sciences, Kaunas, Lithuania
2
Department of Children Surgery, Medical Academy, Lithuanian University of Health Sciences, Kaunas, Lithuania
Abstract
Introduction. Hereditary spherocytosis (HS) is a congenital haemolytic anaemia caused by a defect in the red blood
cell membrane proteins, leading to chronic haemolysis in the spleen. The excessive production of bilirubin during
haemolysis significantly increases the risk of developing pigment gallstones. In paediatric patients, these stones can
lead to complications such as cholecystitis, acute cholangitis, or obstructive jaundice. Standard management involves
symptom control, with major surgeries like cholecystectomy and splenectomy typically delayed until the child reaches
five years of age to minimize infection risks.
Case report. A 3-year-old female patient was admitted with HS and diagnosed with biliary obstruction. Due to the
patient's small paediatric anatomy, specifically the narrow major duodenal papilla (typically 2-5 mm in this age group),
a standard Endoscopic Retrograde Cholangiopancreatography (ERCP) was technically unfeasible. From a
methodological standpoint, a percutaneous transhepatic biliary drainage was selected and justified as the safest
minimally invasive alternative for urgent biliary decompression. The patient’s condition was complicated by the
development of pancreatitis and later acute cholangitis caused by the bacterium Enterobacter ludwigii. After
stabilization with specific antibiotic therapy (ceftazidime and metronidazole), the patient successfully underwent a
laparoscopic cholecystectomy. The cholangiostoma was eventually removed, and the planned splenectomy was
postponed until the patient reaches five years of age to minimize the risk of Overwhelming Post-Splenectomy Infection
(OPSI).
Conclusion. This case highlights that HS is a major risk factor for early-onset gallstones and acute cholangitis in
children. When ERCP is unfeasible due to age and anatomical constraints, percutaneous transhepatic drainage serves
as a safe and effective alternative. Successful management relies on targeted antibiotic therapy, timely surgical
intervention and multidisciplinary collaboration.
Keywords: hereditary spherocytosis, haemolysis, cholelithiasis, acute cholangitis, percutaneous transhepatic
cholangiostoma, laparoscopic cholecystectomy, paediatric splenectomy.
Journal of Medical Sciences. 30 Jul, 2026 - Volume 14 | Issue 5. Electronic - ISSN: 2345-0592
Medical Sciences 2026 Vol. 14 (5), p. 83-88, https://doi.org/10.53453/ms.2026.7.10
83
1. Introduction
Hereditary spherocytosis is a congenital haemolytic
anaemia caused by a defect in the red blood cell
membrane protein: these erythrocytes become sphere-
shaped and are destroyed in the spleen (1). This
condition, due to the excessive production of bilirubin
during haemolysis, significantly increases patients'
predisposition to both gallbladder and bile duct stone
disease (cholelithiasis). Gallstones in patients with
hereditary spherocytosis, which are mostly composed
of bilirubin, can cause complications such as
cholecystitis, cholangitis, and obstructive jaundice (1).
The incidence of biliary stone disease in children has
increased significantly in recent decades, paralleling
trends in obesity and metabolic syndrome. Studies
have estimated a prevalence between 1.9 % and 4 %
(2). Retrospective cholecystography studies with
patients aged 1.4 to 22 years with hereditary
spherocytosis (HS) have reported gallstones in 37-
43% of cases (3).
Treatment for cholelithiasis in patients with hereditary
spherocytosis is tailored to the severity of symptoms
and the extent of haemolysis. Splenectomy is
frequently the primary treatment method, as it
significantly reduces haemolysis and bilirubin
production, thereby indirectly lowering the risk of
gallstone formation. However, splenectomy increases
the risk of infections and, in certain cases, can lead to
severe sepsis. It is more commonly known as
Overwhelming Post-Splenectomy Infection (OPSI).
This complication despite intensive treatment carries
high mortality rate of up to 50%. To decrease this risk,
strategies include partial splenectomy and delaying the
surgery until five years of age (4,5).
Recent research indicates that even after a
splenectomy, persistent haemolysis may still
contribute to the formation of gallstones. Therefore, it
is essential to monitor patient’s post-splenectomy (1).
Prophylactic cholecystectomy is considered a
preventive method for patients with significant
hyperbilirubinemia, even in the absence of gallstone
disease, especially in those with hereditary
spherocytosis (6).
This article presents the case of a a 3-year-old girl with
hereditary spherocytosis who was treated for biliary
duct obstruction. The case illustrates the complications
of this rare genetic disease, specifically acute
cholangitis and pancreatitis, and highlights the unique
diagnostic and treatment challenges. These challenges
required the collaboration of a multidisciplinary team
(paediatric surgeons, radiologists, gastroenterologists,
and onco-haematologists) to plan and monitor the
patient’s follow-up care
2. Case report
A 3-year-old patient with a congenital diagnosis of
hereditary spherocytosis was first hospitalized in the
paediatric surgery department for an initial six-week
period. On the second day of hospitalization, an
attempt was made to perform retrograde
cholangiopancreatography (ERCP) to address the
biliary obstruction. However, the procedure proved
technically unfeasible due to the patient’s age and the
specific anatomical constraints inherent in a three-
year-old child. The patient was subsequently started
on a course of Unasyn. The following day, a
transhepatic bile duct clearance was performed, which
was followed by the development of pancreatitis as a
complication. Ten days later, patient underwent
percutaneous transhepatic cholangiography with the
placement of a 6 Fr drain (Image 1). Following a
cholangioscopy two weeks after the initial drainage
placement drain was replaced with a larger 8.5 Fr
cholangiostoma. Two days later a multidisciplinary
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consultation, identified the management of the
patient's pancreatitis as a clinical priority. The
management plan specified that a minimally invasive
intervention for bile duct clearance and a subsequent
cholecystectomy would be performed only after the
patient’s condition stabilized. Furthermore, it was
recommended that the splenectomy be deferred until
the patient reaches approximately five years of age to
decrease the risk of overwhelming post-splenectomy
infection (OPSI). A follow-up cholangiography
performed one day before discharge, revealed that all
biliary stones and sludge had spontaneously migrated
into the intestinal tract. The patient was subsequently
discharged with the cholangiostoma in situ. and
elective surgery was scheduled for early January.
However, twelve days after discharge due to a
worsened clinical status, the patient was readmitted to
the Department of Paediatric Surgery at the LSMU
Kaunas Clinics, presenting with episodes of
paroxysmal abdominal pain. According to the mother,
the patient experienced two acute pain episodes
requiring analgesic intervention. Objective clinical
findings included a low-grade fever of up to 37.5°C
and a visible intensification of jaundice in both the
sclera and skin. Laboratory investigations confirmed a
severe inflammatory response and significant
cholestasis. C-reactive protein (CRP) was markedly
elevated at 199.5 mg/l, and the white blood cell count
(WBC) reached 26.43 x 10⁹/l, indicating acute
systemic infection. Despite the clinical jaundice, liver
transaminases (ALT 32 IU/l, AST 45 IU/l) remained
within normal ranges, while total bilirubin was
severely increased to 126.48 μmol/l (Table 1). The
patient was hospitalized following a diagnosis of acute
cholangitis. An emergency abdominal ultrasound
revealed a completely contracted gallbladder
containing sludge and several small calculi.
Additionally, the spleen was enlarged for her age,
measuring approximately 10.0 cm. Bile cultures
afterwards yielded Enterobacter ludwigii, prompting
the initiation of intravenous ceftazidime and
metronidazole. A follow-up ultrasound on the fifth day
of the second hospitalization visualized a biliary stent
extending into the duodenum. A laparoscopic
cholecystectomy was successfully performed the next
day. Postoperatively, a subhepatic drain and the
cholangiostoma were left in place. One week after the
surgery, a control cholangiography confirmed that the
contrast medium flowed easily into the intestinal tract.
Consequently, the cholangiostoma was removed, and
the drainage tract within the hepatic parenchyma was
sealed using Embocube particles. A final follow-up
ultrasound showed that the biliary tree was not dilated.
Eight days after the surgery, the patient was
discharged in stable condition (Table 2). Her discharge
recommendations included continued folic acid
therapy until the scheduled splenectomy and
adherence to a specialized post-cholecystectomy
dietary plan.
Image 1 Percutaneous Transhepatic Cholangiogram
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Table 1 Laboratory Data Dynamics: Acute Phase vs. Discharge
Parameter
Reference Range
On Admission
At Discharge
C-reactive protein (CRP)
0 – 5 mg/l
199.5 mg/l
31.8 mg/l
White Blood Cell (WBC)
4.92 – 11.8 x 10⁹/l
26.43 x 10⁹/l
9.74 x 10⁹/l
Total Bilirubin
1.7 – 15.4 μmol/l
126.48 μmol/l
75.86 μmol/l
Hemoglobin (Hb)
111 – 141 g/l
89 g/l
94 g/l
ALT (GPT)
7 – 45 IU/l
32 IU/l
140 IU/l
AST (GOT)
8 – 50 IU/l
45 IU/l
130 IU/l
MCV
72.1 – 87 fl
93.3 fl
87.5 fl
Table 2 Chronological Timeline of the Case
Date
Event / Stage
Description
2022-09-21
Birth and Diagnosis
Patient diagnosed with hereditary spherocytosis.
2025-11-04
First Hospitalization
Admitted due to bile duct stone disease with obstruction.
2025-11-06
Failed Intervention
Attempted ERCP failed due to the patient's small/fine anatomy; initiated
antibiotic treatment with Unasyn.
2025-11-07
Complication
Transhepatic bile duct clearance performed; followed by postoperative
pancreatitis.
2025-11-17
Drainage
Percutaneous transhepatic cholangiography performed and a 6 Fr drain was
inserted.
2025-12-03
Multidisciplinary
Decision
8.5 Fr cholangiostomy inserted. Medical board decided to treat pancreatitis
first and postpone surgery.
2025-12-18
Clinical
Improvement
Follow-up cholangiography showed all stones and sludge had spontaneously
flushed into the intestine.
2025-12-19
Discharge
Patient discharged with a functioning 8.5 Fr cholangiostomy; elective surgery
scheduled for early January.
2026-01-01
Emergency
Readmission
Onset of abdominal pain, fever (up to 37.5°C), and jaundice. Diagnosed with
acute cholangitis.
2026-01-02
Infection
Identification
Bile culture grew Enterobacter ludwigii; targeted IV antibiotic therapy with
Ceftazidime and Metronidazole initiated.
2026-01-07
Surgical Treatment
Laparoscopic cholecystectomy performed.
2026-01-14
Drain Removal
Follow-up cholangiography confirmed free flow to the intestine;
cholangiostomy removed and the tract sealed with "Embocube" particles.
2026-01-15
Final Discharge
Patient discharged in stable condition; continued folic acid treatment and
planned splenectomy at age five.
3. Discussion
This case corroborates documented findings in
medical literature regarding the increased risk of early
onset cholelithiasis and choledocholithiasis in patients
with hereditary spherocytosis. Even in early childhood
due to chronic haemolysis, excessive bilirubin
production leads to the formation of pigment stones,
which can cause severe complications such as biliary
obstruction and acute cholangitis. In this instance, the
standard approach for managing bile duct
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obstruction—Endoscopic Retrograde Cholangio-
pancreatography (ERCP)—was technically unfeasible
due to the patient’s paediatric anatomy. Gastroscopes
that are specifically utilized for ERCP, specialized
pediatric slim duodenoscopes ('Olympus PJF'-7.5,
7.5–8.5 mm) and ultra-slim gastroscopes ('ELUXEO
EG-740N'), were incompatible with the narrow major
duodenal papilla. Anatomical data indicates that in
female patients of this age group, the major papilla
typically measures only 2–5 mm in diameter (7).
When endoscopic access fails, as a safe alternative,
percutaneous transhepatic biliary (PTBD) drainage is
performed. The PTBD procedure relies on using 22G
Chiba needles and 5-8 Fr catheters. PTBD is rarely
performed in children aged 3 years and younger
outside specialized contexts like liver transplantation
or neonatal cholestasis, with no population-level
incidence data due to its infrequent use in high-risk
cases only (8). However, PTBD provides effective
biliary decompression with technical success (89-
96%) and low complication rates (minor 10.8%, major
1.7%) in pediatric series (8). This case highlights the
vital necessity of alternative safest minimally invasive
interventions, such as percutaneous transhepatic
cholangiography and drainage, as safe and effective
management strategies.
Paediatric acute cholangitis due to Enterobacter
ludwigii is a rare clinical finding. Reports of this
specific opportunistic pathogen in children remain
infrequent. In paediatric cholangitis, more typical
organisms are usually Enterobacterales such as E.
coli, Klebsiella, and Enterobacter spp (9). Therefore,
this appears to be an unusual ethology in our patient
necessitating targeted antibacterial therapy with
ceftazidime and metronidazole. Additionally, biliary
duct drainage via a cholangiostoma was required until
the primary focus of infection in the gallbladder could
be definitively eliminated.
The acute conditions of cholangitis and pancreatitis
demanded urgent collaboration from a multidisc-
ciplinary team—including paediatric surgeons,
radiologists, gastroenterologists, and onco-
haematologists—to achieve rapid clinical
stabilization. This multidisciplinary approach was also
essential for planning the subsequent cholecystectomy
and scheduling the splenectomy to be postponed until
the patient reaches five years of age.
Splenectomy was deferred until age 5 to reduce the
risk of overwhelming post-splenectomy infection,
since OPSI is more common in young children and
many recommendations advise postponing elective
splenectomy until after early childhood when
feasible(10).
4. Conclusion
1. Hereditary spherocytosis is a significant risk
factor for the development of choledocholithiasis and
acute cholangitis in paediatric patients.
2. When Endoscopic Retrograde
Cholangiopancreatography is unsuccessful due to the
patient's age and specific anatomical constraints,
percutaneous transhepatic drainage serves as a safe
and effective alternative.
3. Successful management of acute cholangitis
relies on targeted, specific antibiotic therapy and
timely surgical intervention.
4. A multidisciplinary team approach is
essential for managing complex cases of paediatric
biliary obstruction.
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