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Case Report J Med Cases. 2014;5(4):208-211
PressElmer
Articles © The authors | Journal compilation © J Med Cases and Elmer Press Inc™ | www.journalmc.org
This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction
in any medium, provided the original work is properly cited
Fatal Pneumothorax Secondary to Duodenal Perforation
After Endoscopic Retrograde Cholangiopancreatography
Muhammad Asim Ranaa
, Murid Hussainb
, Sohail Iqbalc, d
, Omer E Ramadana
, Sameh Ashmawia
,
Mohammed A T Al-Dabbaghc
, Mohammed A Cheemac
, Ahmed F Madya
Abstract
Endoscopic retrograde cholangiopancreatography (ERCP) is a
widely accepted diagnostic and therapeutic modality for extra-
hepatic biliary and pancreatic pathologies. Although minimally
invasive and day case procedure, it is not free from complications
including bleeding, perforation, cholangitis and pancreatitis. Some
of these complications, if left undiagnosed or poorly managed can
become life threatening such as pneumothorax secondary to duode-
nal perforation. Hence, recognition and understanding of potential
complications of ERCP is vital. We report a case of rapidly fatal
duodenal perforation with pneumoperitoneum, pneumoretroperito-
neum, bilateral pneumothoraces, pneumomediastinum, and subcu-
taneous emphysema causing hemodynamic compromise.
Keywords: ERCP; Pneumothorax; Pneumoperitoneum; Pneumor-
etroperitoneum; Pneumomediastinum; Surgical emphysema
Introduction
Towards the end of last century, widespread use of endo-
scopic retrograde cholangiopancreatography (ERCP) has
been instrumental in the management of patients with extra
hepatic biliary tract and pancreatic diseases. Bleeding, perfo-
ration, cholangitis, and pancreatitis are the major complica-
tions of this invasive procedure with an overall mortality rate
of about 1% [1]. Post-procedure pneumoperitoneum, pneu-
momediastinum and subcutaneous emphysema indicate an
iatrogenic duodenal perforation. It is rare but serious compli-
cation having up to 23% mortality rate [2]. Changes in vital
signs, dyspnea and decreasing oxygen saturation, especially
following (pre-cut) sphincterotomy, should be considered a
sensitive indicator of perforation or pneumothorax.
Case Report
A 90 years old lady underwent ERCP for biliary pancreati-
tis as an elective procedure. During the procedure, she was
found to have duodenal diverticulum. Papillotomy with the
drainage of the sludge was performed. Post procedure pa-
tient was shifted to emergency room due to radiographic
evidence of surgical emphysema in the face, head and neck
and chest region (Fig. 1a). She was intubated and transferred
to intensive care unit (ICU) because of rapid hemodynamic
compromise. After initial management contrast enhanced
computerized tomography (CECT) was performed which
revealed extensive surgical emphysema encompassing the
whole body (Fig. 1b) along with air in all body cavities
i.e., bilateral pneumothorax, pneumomediastinum (Fig. 2a),
pneumoperitoneum and pneumoretroperitoneum (Fig. 2b).
Minimal contrast leakage was noticed around the duodenal
area. A second look endoscopy failed to show any duodenal
diverticulum or evidence of perforation at any other level.
Patient succumbed to the overwhelming shock and multi-
organ failure in-spite of the enhanced multidisciplinary sup-
port.
Discussion
Since its introduction in 1968, ERCP has become a com-
monly performed endoscopic procedure [3]. The diagnostic
and therapeutic role of ERCP has been well demonstrated for
a variety of disorders, such as the management of choledo-
cholithiasis, the diagnosis and management of biliary and
pancreatic neoplasms, and the postoperative management of
biliary peri-operative complications [4]. ERCP has proved
superior to its diagnostic counterpart magnetic resonance
Manuscript accepted for publication February 18, 2014
a
Department of Intensive Care Medicine, King Saud Medical City,
Riyadh, Kingdom of Saudi Arabia
b
Department of Internal Medicine, Tameside General Hospital, Ashton
under Lyne, OL6 9RW, England, UK
c
Department of Radiology, Colchester General Hospital, Colchester,
CO4 5JL, England, UK
d
Corresponding author: Sohail Iqbal, Radiology Department,
Colchester General Hospital, Colchester, CO4 5JL, England, UK,
Email: drsohailiqbal@yahoo.com
doi: http://dx.doi.org/10.14740/jmc1700w
208
J Med Cases. 2014;5(4):208-211Rana et al
Articles © The authors | Journal compilation © J Med Cases and Elmer Press Inc™ | www.journalmc.org
cholangiopancreatography (MRCP) in its therapeutic uses
and to its therapeutic competitor laparoscopic cholecystec-
tomy (with or without intraoperative cholangiography) in
diagnostic sphere.
Bleeding, perforation, cholangitis, and pancreatitis are
some common complications of this invasive procedure [5].
Portal venous or systemic air embolism is a very rare com-
plication [6]. Post ERCP pneumothorax is suggested to be
an exceptional complication that can occur at any age and, is
usually right sided or bilateral. It is typically associated with
the presence of retroperitoneal, mediastinal, subcutaneous
and frequently intra-peritoneal air [7].
The main risk factors, for ERCP related pneumothorax
include sphincterotomy (precut), presence of juxta-papillary
diverticula, sphincter of oddi dysfunction, dilated bile duct,
papillary stenosis, age of the patient and extended proce-
dure duration [8]. Perforation can be classified according
to mechanism of injury and anatomical location as follows
in descending order of severity: Lateral or medial duodenal
wall perforation (Type I), Peri-Vaterian (sphincter of Oddi)
injuries (Type II), Wire/basket related distal bile duct inju-
ries (Type III) and Retroperitoneal air alone (Type IV) which
is probably related to the use of compressed air to maintain
patency of the duodenal lumen resulting in intramural and
extra-luminal air diffusion [5].
During ERCP, air can reach pleural cavity through duo-
denal rupture porous diaphragm syndrome [9] or alveolar
rupture. In most cases, pneumothorax coexists with pneu-
moretroperitoneum. This finding indicates that air enters the
retroperitoneal space after interruption of the duodenal bar-
rier, through a site of perforation or a site of low resistance.
Subsequently, air transfers from the retroperitoneal space to
the peritoneum, subcutaneous tissue, mediastinum, and fi-
nally pleural space. Passage of air from the mediastinum to
the pleural space demands a rupture of the parietal pleura.
The retroperitoneal perforation is mainly caused by sphinc-
terotomy and can be differentiated from intra-peritoneal rup-
ture by the absence of peritoneal signs.
Congenital or acquired porous diaphragm may allow
passage of air and fluids between abdominal and thoracic
cavities. However, absence of concomitant intraperitoneal
air in some patients with ERCP associated pneumothorax
does not support this hypothesis as a central mechanism.
Alveolar rupture may occur due to increased intra-tho-
racic pressure in patients who poorly tolerate endoscopic
procedures, in anxious patients with continuous movements
209
Figure 1. a) Chest X-ray showing surgical emphysema in the neck region; b) CECT revealing surgical emphysema
encompassing whole body.
J Med Cases. 2014;5(4):208-211Fatal Pneumothorax
Articles © The authors | Journal compilation © J Med Cases and Elmer Press Inc™ | www.journalmc.org
or retching and can lead to the development of tension pneu-
mothorax. In these cases one may not find free air in retro-
peritoneal space, intraperitoneal cavity, or mediastinum. The
clinical presentation depends upon the amount of escaped air
and its extension into the adjacent tissues and cavities. Most
common signs and symptoms are abdominal distention, ab-
dominal/chest pain, tachycardia, dyspnea, and subcutaneous
emphysema and may become manifest either during or after
ERCP [10].
Roentgenogram can show presence and accumulation of
air and is sufficient in majority of cases whereas oral contrast
studies/CECT abdomen may show retroperitoneal contrast
leakage in complicated cases. Retroperitoneal perforation is
usually not visible with endoscope.
Because of the rarity of this complication, large case se-
ries or controlled studies are not available with respect to the
optimal treatment; however, there are increasing number of
case reports that indicates that a non-surgical approach can
be followed. Conservative management consists of adminis-
tration of intravenous antibiotics, parenteral nutrition with-
holding of oral feeding, and pleural drainage. Patient should
be carefully observed and any concern about the clinical de-
terioration should lead to prompt surgical consultation.
Duodenal perforation secondary to sphincterotomy has
been treated with endoscopic clips or fibrin glue [11]. Signs
of acute peritoneal irritation with or without sepsis, large
contrast extravasation, and presence of intra- or retroperito-
neal fluid collections and failure of initial conservative man-
agement are the indications for surgical closure of the leak
[12].
Iatrogenic post-ERCP perforation triggering a cascade
of retroperitoneal, intraperitoneal, mediastinal and subcuta-
neous air and culminating into hemo-dynamically unstable
pneumothorax is a rare but potentially fatal complication.
The most crucial part of the management is the early detec-
tion of this dreadful complication.
Acknowledgement
We are greatly indebted to Dr. Shagufta Jabeen, (Clinical Fel-
low, Royal Manchester Children Hospital, Manchester) and
Mr. Andy Richardson (Library & E-learning Lead CHUFT,
Colchester) for their help and support provided during prepa-
ration of this manuscript.
References
1.	 Christensen M, Matzen P, Schulze S, Rosenberg J. Com-
plications of ERCP: a prospective study. Gastrointest
Endosc. 2004;60(5):721-731.
2.	 Kwon CI, Song SH, Hahm KB, Ko KH. Unusual com-
plications related to endoscopic retrograde cholangio-
pancreatography and its endoscopic treatment. Clin En-
dosc. 2013;46(3):251-259.
3.	 Kwon W, Jang JY, Ryu JK, Kim YT, Yoon YB, Kang
MJ, Kim SW. Proposal of an endoscopic retrograde
cholangiopancreatography-related perforation manage-
ment guideline based on perforation type. J Korean Surg
Soc. 2012;83(4):218-226.
4.	 Maple JT, Ben-Menachem T, Anderson MA, Appalane-
ni V, Banerjee S, Cash BD, Fisher L, et al. The role of
endoscopy in the evaluation of suspected choledocholi-
thiasis. Gastrointest Endosc. 2010;71(1):1-9.
5.	 Stapfer M, Selby RR, Stain SC, Katkhouda N, Parekh
D, Jabbour N, Garry D. Management of duodenal perfo-
ration after endoscopic retrograde cholangiopancreatog-
raphy and sphincterotomy. Ann Surg. 2000;232(2):191-
198.
6.	 Siddiqui J, Jaffe PE, Aziz K, Forouhar F, Sheppard R,
Covault J, Bonkovsky HL. Fatal air and bile embolism
after percutaneous liver biopsy and ERCP. Gastrointest
Endosc. 2005;61(1):153-157.
7.	 Andriulli A, Loperfido S, Napolitano G, Niro G, Valva-
no MR, Spirito F, PilottoA, et al. Incidence rates of post-
ERCP complications: a systematic survey of prospective
studies. Am J Gastroenterol. 2007;102(8):1781-1788.
8.	 Schepers NJ, van Buuren HR. Pneumothorax following
ERCP: report of four cases and review of the literature.
Dig Dis Sci. 2012;57(8):1990-1995.
9.	 Sampaziotis F, Wiles A, Shaukat S, Dickinson RJ. Bi-
210
Figure 2. a) CECT with bilateral pneumothoraces and pneumomediastinum; b) CECT showing pneumoperitoneum
and pneumoretroperitoneum.
J Med Cases. 2014;5(4):208-211Rana et al
Articles © The authors | Journal compilation © J Med Cases and Elmer Press Inc™ | www.journalmc.org
lateral Pneumothorax and Subcutaneous Emphysema
following Endoscopic Retrograde Cholangiopancrea-
tography: A Rare Complication. Diagn Ther Endosc.
2010;2010.
10.	 Al-Ashaal YI, Hefny AF, Safi F, Abu-Zidan FM. Ten-
sion pneumothorax complicating endoscopic retrograde
cholangiopancreatography: case report and systematic
literature review. Asian J Surg. 2011;34(1):46-49.
11.	 Machado NO. Management of duodenal perforation
post-endoscopic retrograde cholangiopancreatography.
When and whom to operate and what factors determine
the outcome? A review article. JOP. 2012;13(1):18-25.
12.	 Williams EJ, Taylor S, Fairclough P, Hamlyn A, Logan
RF, Martin D, Riley SA, et al. Risk factors for complica-
tion following ERCP; results of a large-scale, prospec-
tive multicenter study. Endoscopy. 2007;39(9):793-801.
211

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Case report fatal pneumothorax after ERCP

  • 1. Case Report J Med Cases. 2014;5(4):208-211 PressElmer Articles © The authors | Journal compilation © J Med Cases and Elmer Press Inc™ | www.journalmc.org This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited Fatal Pneumothorax Secondary to Duodenal Perforation After Endoscopic Retrograde Cholangiopancreatography Muhammad Asim Ranaa , Murid Hussainb , Sohail Iqbalc, d , Omer E Ramadana , Sameh Ashmawia , Mohammed A T Al-Dabbaghc , Mohammed A Cheemac , Ahmed F Madya Abstract Endoscopic retrograde cholangiopancreatography (ERCP) is a widely accepted diagnostic and therapeutic modality for extra- hepatic biliary and pancreatic pathologies. Although minimally invasive and day case procedure, it is not free from complications including bleeding, perforation, cholangitis and pancreatitis. Some of these complications, if left undiagnosed or poorly managed can become life threatening such as pneumothorax secondary to duode- nal perforation. Hence, recognition and understanding of potential complications of ERCP is vital. We report a case of rapidly fatal duodenal perforation with pneumoperitoneum, pneumoretroperito- neum, bilateral pneumothoraces, pneumomediastinum, and subcu- taneous emphysema causing hemodynamic compromise. Keywords: ERCP; Pneumothorax; Pneumoperitoneum; Pneumor- etroperitoneum; Pneumomediastinum; Surgical emphysema Introduction Towards the end of last century, widespread use of endo- scopic retrograde cholangiopancreatography (ERCP) has been instrumental in the management of patients with extra hepatic biliary tract and pancreatic diseases. Bleeding, perfo- ration, cholangitis, and pancreatitis are the major complica- tions of this invasive procedure with an overall mortality rate of about 1% [1]. Post-procedure pneumoperitoneum, pneu- momediastinum and subcutaneous emphysema indicate an iatrogenic duodenal perforation. It is rare but serious compli- cation having up to 23% mortality rate [2]. Changes in vital signs, dyspnea and decreasing oxygen saturation, especially following (pre-cut) sphincterotomy, should be considered a sensitive indicator of perforation or pneumothorax. Case Report A 90 years old lady underwent ERCP for biliary pancreati- tis as an elective procedure. During the procedure, she was found to have duodenal diverticulum. Papillotomy with the drainage of the sludge was performed. Post procedure pa- tient was shifted to emergency room due to radiographic evidence of surgical emphysema in the face, head and neck and chest region (Fig. 1a). She was intubated and transferred to intensive care unit (ICU) because of rapid hemodynamic compromise. After initial management contrast enhanced computerized tomography (CECT) was performed which revealed extensive surgical emphysema encompassing the whole body (Fig. 1b) along with air in all body cavities i.e., bilateral pneumothorax, pneumomediastinum (Fig. 2a), pneumoperitoneum and pneumoretroperitoneum (Fig. 2b). Minimal contrast leakage was noticed around the duodenal area. A second look endoscopy failed to show any duodenal diverticulum or evidence of perforation at any other level. Patient succumbed to the overwhelming shock and multi- organ failure in-spite of the enhanced multidisciplinary sup- port. Discussion Since its introduction in 1968, ERCP has become a com- monly performed endoscopic procedure [3]. The diagnostic and therapeutic role of ERCP has been well demonstrated for a variety of disorders, such as the management of choledo- cholithiasis, the diagnosis and management of biliary and pancreatic neoplasms, and the postoperative management of biliary peri-operative complications [4]. ERCP has proved superior to its diagnostic counterpart magnetic resonance Manuscript accepted for publication February 18, 2014 a Department of Intensive Care Medicine, King Saud Medical City, Riyadh, Kingdom of Saudi Arabia b Department of Internal Medicine, Tameside General Hospital, Ashton under Lyne, OL6 9RW, England, UK c Department of Radiology, Colchester General Hospital, Colchester, CO4 5JL, England, UK d Corresponding author: Sohail Iqbal, Radiology Department, Colchester General Hospital, Colchester, CO4 5JL, England, UK, Email: drsohailiqbal@yahoo.com doi: http://dx.doi.org/10.14740/jmc1700w 208
  • 2. J Med Cases. 2014;5(4):208-211Rana et al Articles © The authors | Journal compilation © J Med Cases and Elmer Press Inc™ | www.journalmc.org cholangiopancreatography (MRCP) in its therapeutic uses and to its therapeutic competitor laparoscopic cholecystec- tomy (with or without intraoperative cholangiography) in diagnostic sphere. Bleeding, perforation, cholangitis, and pancreatitis are some common complications of this invasive procedure [5]. Portal venous or systemic air embolism is a very rare com- plication [6]. Post ERCP pneumothorax is suggested to be an exceptional complication that can occur at any age and, is usually right sided or bilateral. It is typically associated with the presence of retroperitoneal, mediastinal, subcutaneous and frequently intra-peritoneal air [7]. The main risk factors, for ERCP related pneumothorax include sphincterotomy (precut), presence of juxta-papillary diverticula, sphincter of oddi dysfunction, dilated bile duct, papillary stenosis, age of the patient and extended proce- dure duration [8]. Perforation can be classified according to mechanism of injury and anatomical location as follows in descending order of severity: Lateral or medial duodenal wall perforation (Type I), Peri-Vaterian (sphincter of Oddi) injuries (Type II), Wire/basket related distal bile duct inju- ries (Type III) and Retroperitoneal air alone (Type IV) which is probably related to the use of compressed air to maintain patency of the duodenal lumen resulting in intramural and extra-luminal air diffusion [5]. During ERCP, air can reach pleural cavity through duo- denal rupture porous diaphragm syndrome [9] or alveolar rupture. In most cases, pneumothorax coexists with pneu- moretroperitoneum. This finding indicates that air enters the retroperitoneal space after interruption of the duodenal bar- rier, through a site of perforation or a site of low resistance. Subsequently, air transfers from the retroperitoneal space to the peritoneum, subcutaneous tissue, mediastinum, and fi- nally pleural space. Passage of air from the mediastinum to the pleural space demands a rupture of the parietal pleura. The retroperitoneal perforation is mainly caused by sphinc- terotomy and can be differentiated from intra-peritoneal rup- ture by the absence of peritoneal signs. Congenital or acquired porous diaphragm may allow passage of air and fluids between abdominal and thoracic cavities. However, absence of concomitant intraperitoneal air in some patients with ERCP associated pneumothorax does not support this hypothesis as a central mechanism. Alveolar rupture may occur due to increased intra-tho- racic pressure in patients who poorly tolerate endoscopic procedures, in anxious patients with continuous movements 209 Figure 1. a) Chest X-ray showing surgical emphysema in the neck region; b) CECT revealing surgical emphysema encompassing whole body.
  • 3. J Med Cases. 2014;5(4):208-211Fatal Pneumothorax Articles © The authors | Journal compilation © J Med Cases and Elmer Press Inc™ | www.journalmc.org or retching and can lead to the development of tension pneu- mothorax. In these cases one may not find free air in retro- peritoneal space, intraperitoneal cavity, or mediastinum. The clinical presentation depends upon the amount of escaped air and its extension into the adjacent tissues and cavities. Most common signs and symptoms are abdominal distention, ab- dominal/chest pain, tachycardia, dyspnea, and subcutaneous emphysema and may become manifest either during or after ERCP [10]. Roentgenogram can show presence and accumulation of air and is sufficient in majority of cases whereas oral contrast studies/CECT abdomen may show retroperitoneal contrast leakage in complicated cases. Retroperitoneal perforation is usually not visible with endoscope. Because of the rarity of this complication, large case se- ries or controlled studies are not available with respect to the optimal treatment; however, there are increasing number of case reports that indicates that a non-surgical approach can be followed. Conservative management consists of adminis- tration of intravenous antibiotics, parenteral nutrition with- holding of oral feeding, and pleural drainage. Patient should be carefully observed and any concern about the clinical de- terioration should lead to prompt surgical consultation. Duodenal perforation secondary to sphincterotomy has been treated with endoscopic clips or fibrin glue [11]. Signs of acute peritoneal irritation with or without sepsis, large contrast extravasation, and presence of intra- or retroperito- neal fluid collections and failure of initial conservative man- agement are the indications for surgical closure of the leak [12]. Iatrogenic post-ERCP perforation triggering a cascade of retroperitoneal, intraperitoneal, mediastinal and subcuta- neous air and culminating into hemo-dynamically unstable pneumothorax is a rare but potentially fatal complication. The most crucial part of the management is the early detec- tion of this dreadful complication. Acknowledgement We are greatly indebted to Dr. Shagufta Jabeen, (Clinical Fel- low, Royal Manchester Children Hospital, Manchester) and Mr. Andy Richardson (Library & E-learning Lead CHUFT, Colchester) for their help and support provided during prepa- ration of this manuscript. References 1. Christensen M, Matzen P, Schulze S, Rosenberg J. Com- plications of ERCP: a prospective study. Gastrointest Endosc. 2004;60(5):721-731. 2. Kwon CI, Song SH, Hahm KB, Ko KH. Unusual com- plications related to endoscopic retrograde cholangio- pancreatography and its endoscopic treatment. Clin En- dosc. 2013;46(3):251-259. 3. Kwon W, Jang JY, Ryu JK, Kim YT, Yoon YB, Kang MJ, Kim SW. Proposal of an endoscopic retrograde cholangiopancreatography-related perforation manage- ment guideline based on perforation type. J Korean Surg Soc. 2012;83(4):218-226. 4. Maple JT, Ben-Menachem T, Anderson MA, Appalane- ni V, Banerjee S, Cash BD, Fisher L, et al. The role of endoscopy in the evaluation of suspected choledocholi- thiasis. Gastrointest Endosc. 2010;71(1):1-9. 5. Stapfer M, Selby RR, Stain SC, Katkhouda N, Parekh D, Jabbour N, Garry D. Management of duodenal perfo- ration after endoscopic retrograde cholangiopancreatog- raphy and sphincterotomy. Ann Surg. 2000;232(2):191- 198. 6. Siddiqui J, Jaffe PE, Aziz K, Forouhar F, Sheppard R, Covault J, Bonkovsky HL. Fatal air and bile embolism after percutaneous liver biopsy and ERCP. Gastrointest Endosc. 2005;61(1):153-157. 7. Andriulli A, Loperfido S, Napolitano G, Niro G, Valva- no MR, Spirito F, PilottoA, et al. Incidence rates of post- ERCP complications: a systematic survey of prospective studies. Am J Gastroenterol. 2007;102(8):1781-1788. 8. Schepers NJ, van Buuren HR. Pneumothorax following ERCP: report of four cases and review of the literature. Dig Dis Sci. 2012;57(8):1990-1995. 9. Sampaziotis F, Wiles A, Shaukat S, Dickinson RJ. Bi- 210 Figure 2. a) CECT with bilateral pneumothoraces and pneumomediastinum; b) CECT showing pneumoperitoneum and pneumoretroperitoneum.
  • 4. J Med Cases. 2014;5(4):208-211Rana et al Articles © The authors | Journal compilation © J Med Cases and Elmer Press Inc™ | www.journalmc.org lateral Pneumothorax and Subcutaneous Emphysema following Endoscopic Retrograde Cholangiopancrea- tography: A Rare Complication. Diagn Ther Endosc. 2010;2010. 10. Al-Ashaal YI, Hefny AF, Safi F, Abu-Zidan FM. Ten- sion pneumothorax complicating endoscopic retrograde cholangiopancreatography: case report and systematic literature review. Asian J Surg. 2011;34(1):46-49. 11. Machado NO. Management of duodenal perforation post-endoscopic retrograde cholangiopancreatography. When and whom to operate and what factors determine the outcome? A review article. JOP. 2012;13(1):18-25. 12. Williams EJ, Taylor S, Fairclough P, Hamlyn A, Logan RF, Martin D, Riley SA, et al. Risk factors for complica- tion following ERCP; results of a large-scale, prospec- tive multicenter study. Endoscopy. 2007;39(9):793-801. 211