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Journal of Pediatric Surgery (2012) 47, E47–E50

www.elsevier.com/locate/jpedsurg

Surgical treatment of inspissated bile syndrome using a
2-stage pure laparoscopic approach: A case report☆,☆☆
Justus Lieber a , Femke E. Piersma b , Ekkehard Sturm b , Jürgen F. Schäfer c ,
Jörg Fuchs a , Philipp O. Szavay a,⁎
a
University Children's Hospital, Department of Pediatric Surgery and Pediatric Urology, Hoppe-Seyler-Strasse 1,
D-72076 Tuebingen, Germany
b
University Children's Hospital, Department of Pediatric Gastroenterology and Hepatology, Hoppe-Seyler-Strasse 3,
D-72076 Tuebingen, Germany
c
University Hospital, Department of Diagnostic and Interventional Radiology, Hoppe-Seyler-Strasse 1,
D-72076 Tuebingen, Germany

Received 13 June 2012; revised 3 September 2012; accepted 6 September 2012

Key words:
Abstract We describe a 99-day old girl with inspissated bile syndrome (IBS) unresponsive to treatment with
Inspissated bile syndrome;
oral ursodeoxycholic acid. We performed a pure laparoscopic 2-stage procedure, consisting of
Laparoscopy;
cholecystostomy and insertion of an indwelling balloon catheter for local ursodeoxycholic acid flushing
Cholestasis;
for 13 consecutive days. Subsequently, the cholecystostomy was removed, preserving the gallbladder using
Extrahepatic bile duct
the same laparoscopical approach when bilirubin values returned to normal and bile duct obstruction was no
obstruction;
longer detectable radiologically. This is the first report of an exclusively laparoscopic management of IBS.
Ursodeoxycholic acid
© 2012 Elsevier Inc. All rights reserved.

In 1935 Ladd reported inspissated bile syndrome (IBS), a parenteral nutrition in preterm infants, administration of
condition causing partial or complete biliary tract obstruction diuretic medication, and with conditions leading to bowel
with an incidence of 1 in 175,000 live births that accounted dysfunction [3]. Oral ursodeoxycholic acid can resolve bile
for about 8% of all surgeries for jaundice during infancy stasis, however, if ineffective, therapy requires escalation with
[1,2]. IBS has been described after blood transfusions, antegrade flushing of the bile ducts at the time of percutaneous
transhepatic cholangiography or even laparotomy [4,5].
Interventional procedures may be technically challenging
☆ due to the small body proportions in infants, whereas
Financial disclosure: none of the authors.
☆☆
Funding: none. laparotomy is significantly more invasive for the patient.
⁎ Corresponding author. Tel.: +49 7071 2986621; fax: +49 7071 Minimal invasive surgery has various advantages such as
294046. rapid postoperative recovery and less pain, shorter hospital
E-mail addresses: justus.lieber@med.uni-tuebingen.de (J. Lieber), stay and fewer complications [6]. Here, we report a purely
femke.piersma@med.uni-tuebingen.de (F.E. Piersma), laparoscopic two stage approach for IBS preserving the
ekkehard.sturm@med.uni-tuebingen.de (E. Sturm),
juergen.schaefer@med.uni-tuebingen.de (J.F. Schäfer), gallbladder and providing the advantages of minimal invasive
joerg.fuchs@med.uni-tuebingen.de (J. Fuchs), surgery, which to the best of our kniowledge has not
philipp.szavay@med.uni-tuebingen.de (P.O. Szavay). previously been described in the literature.

0022-3468/$ – see front matter © 2012 Elsevier Inc. All rights reserved.
http://dx.doi.org/10.1016/j.jpedsurg.2012.09.043
E48 J. Lieber et al.

Complex heart defect and had already undergone recon-
struction of the aortic arch. Indication for biliary surgical
intervention was a constantly increasing bilirubin value of
7.7 mg/dl and failure to respond to oral ursodeoxycholic acid.
Magnetic-resonance-cholangio-pancreaticography (MRCP)
revealed sludge- and concretion-dependent dilatation of
otherwise normal extrahepatic bile ducts and gallbladder due
to inspissated bile. Serum levels of aspartate aminotransfer-
ase (AST; 105 U/l), alanine aminotransferase (ALT; 53 U/l)
and y-GT (717 U/l) were elevated. Metabolic or infectious
causes were excluded.
Under general anaesthesia and in a supine position,
pneumoperitoneum was achieved using CO2 under 10 cm
H2O pressure and 4 l/min flow. Three trocars were inserted,
one at the umbilicus for a 5 mm camera, and two 3 mm
working ports were positioned in the right and left middle
abdomen (Fig. 1). The gallbladder (Fig. 2A) was fixed to the
ventral abdominal wall using a 4.0 vicryl intracorporal
suture. A purse string suture was placed at the fundus before
punctiform choleystostomy was performed using a mono-
polar instrument. A 6 Charrière balloon catheter was inserted
Fig. 1 Setting of laparoscopy. transabdominally, positioned in the gallbladder and blocked
(Fig. 2B). Tight fixation of the gallbladder was accomplished
using 3 more intracorporal sutures to the ventral abdominal
wall in order to prevent leakage (Fig. 2C). Intraoperative
1. Case report cholangiography showed the concretion-filled choledochal
duct filled with bile sludge and concrements, a considerable
A 99 day old girl presented with ongoing jaundice due to hepatic influx and marginal drainage of contrast agent into
suspected inspissated bile syndrome. She had a Shone– the duodenum (Fig. 3). Finally, a liver biopsy was taken from

Fig. 2 Intraoperative view on the gallbladder (A), after insertion of the flushing catheter (B), after fixation of the gallbladder to the
abdominal wall (C), and after release and closure of the gallbladder (D).
A 2-stage pure laparoscopic approach for IBS E49

2. Discussion
Treatment of inspissated bile syndrome in infancy due to
partial or complete obstruction of the distal extrahepatic biliary
system by impaction of thickened bile or sludge still remains a
major challenge when unresponsive to oral treatment with
ursodeoxycholic acid. Interventional procedures such as
endoscopic retrograde (ERCP) or percutaneous transhepatic
cholangiography (PTTC) with contemporary therapeutic
lavage of the biliary tree are technically difficult because of
the small proportions of duodenum and papilla and small size
of the bile ducts in neonates and infants and share additional
difficulty in obtaining sufficient antegrade flushing pressure to
clear the biliary tree [4]. Laparotomy and choledochotomy are
indicated in those cases, but may be associated with the risk of
Fig. 3 Intraoperatively cholangiography shows partial obstruc-
injury and late stricture in the biliary system [5]. Open
tion of the bile ducts (CBD=common bile duct; DC=choledochal
duct; GB=gallbladder) and hepatic influx (HI).
conventional surgery has generally been associated with
significantly higher complication rates, longer postoperative
pain and hospital stay as well as worse cosmetic results [6]. To
the best of our knowledge, this is the first purely laparoscopic
the macroscopically appearing greenish and fibrotic organ. approach providing ongoing access for repeated irrigation of
Histology revealed advanced liver fibrosis and an initial state the biliary tree in the treatment of IBS avoiding invasive
of cirrhosis. Furthermore, pronounced inflammatory in- surgery. The procedure was effective, technically easy and safe
filtrates and lowgrade centrolobular necrosis were noted, for the patient while preserving the gallbladder. Feasibility of
which both are atypical for impaired biliary drainage. the procedure was supported by an adequately sized and
Postoperatively, bile duct lavage using a 5 ml solution anatomically located gallbladder for placement of the
containing 10 mg ursodeoxycholic acid was performed 3 indwelling catheter and tight fixation to the abdominal wall
times a day for 13 consequtive days until follow up control in order to prevent leakage. A hypoplastic gallbladder or cystic
cholangiography demonstrated free drainage into the duo- duct might limit this approach. Recently, laparoscopic assisted
denum without intrahepatic influx (Fig. 4) and reduction of procedures have been described, where the gallbladder was
the serum bilirubin value to 1.9 mg/dl. pulled out through the abdominal wall at the trocar site to
A second laparoscopy was perfomed after IBS had perform cholecystostomy [7,8]. However, no long-term results
resolved using the same trocar setting for release of the exist regarding spontaneous cholecystostomy closure rates,
cholecystotomy tube and closure of the gallbladder using gallbladder function and cosmetic results. It also remains
two 4.0 vicryl Z-sutures preserving the gall bladder unclear, whether the function of the gallbladder is maintained
(Fig. 2D). Lastest follow up was 7 months after operation with this technique, or whether the patient is at risk for
and showed normal serum bilirubin and transaminase levels. postcholecystectomy symptoms [9]. Finally, adhesions might
Ultrasound imaging showed a normal shape of the be a consequence without active release of the gallbladder.
gallbladder, but also mild hepatosplenomegaly. In our case flushing ursodeoxycholic acid through the
cholecystostomy catheter for local treatment of inspissated
bile was effective. However, other mucolytic agents such as
N-acetylcysteine, pancreatin or pure saline have also been
successful for this purpose but no data about duration of
flushing and hydrostatic forces exist and their relative
advantages cannot be delineated [10]. Ursodeoxycholic
acid up to 30 mg/kg per day is effective in reducing
cholestasis and constitutes an approved medication for
dissolving gallstones and bile sludge [8]. We demonstrated
this dosage to be effective, even though concentrations twice
as high have also been used [7].
IBS has been associated with conditions such as blood
transfusion, parenteral nutrition in preterm infants, diuretic
medication, bowel dysfunction, and previously AB0 incompat-
ibility [3]. In our case the patient had undergone cardio-
Fig. 4 Follow up control cholangiography shows free drainage vascular reconstructive surgery and had received parenteral
into the gut without intrahepatic influx. nutrition for a limited amount of time. Advanced fibrosis and
E50 J. Lieber et al.

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