Ijmas 200
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This case report describes an unusual case of a Phrygian cap (septate gallbladder) that mimicked a choledochal cyst. A 52-year-old male presented with right upper quadrant pain and was found to have an irregular hyperechoic mass in the gallbladder on ultrasound. CT and MRI suggested a choledochal cyst but MRCP revealed a septate gallbladder (Phrygian cap). At surgery, a septate gallbladder with stones was found but the common bile duct was normal. Phrygian cap is a common gallbladder anomaly that is usually asymptomatic but can be mistaken for other pathologies on imaging. Thorough preoperative imaging like MRCP is important to identify anatomical variations
Ijmas 200
- 1. INTERNATIONAL JOURNAL OF MEDICAL AND APPLIED SCIENCES ISSN:2320‐3137 334 www.earthjournals.org Volume 2 Issue 3 2013 CASE REPORT PHRYGIAN CAP MIMICKING A CHOLEDOCHAL CYST: AN UNUSUAL CASE REPORT AND REVIEW OF LITERATURE Anilkumar VR, Narayana Subramaniam, Ganesh Hegde 1. Dr. Anilkumar VR, Associate Professor, General Surgery, M.S. Ramaiah Medical College and Hospital, Bangalore, India 2. Dr. Narayana Subramaniam, Resident General Surgery, M.S. Ramaiah Medical College and Hospital, Bangalore, India 3. Dr. Ganesh Hegde, Assistant Professor, General Surgery, M.S. Ramaiah Medical College and Hospital, Bangalore, India ABSTRACT Major gallbladder and associated biliary malformations are an uncommon entity – incidence varies, but the largest study conducted showed an incidence of around 0.15%. They are of great consequence to surgeons as they may be associated with complex malformations in the extra-hepatic biliary tree and its blood supply, making surgery difficult and dangerous. With intra-operative diagnosis being the only viable option, a thorough knowledge of anatomical variations, the differential diagnosis on imaging and a high index of suspicion is required to avoid intra-operative complications and their significant associated morbidity. This article discusses a case of septate gall bladder (known as ‘Phrygian cap’) mimicking a choledochal cyst. It also discusses the difficulty in pre and peri-operative diagnosis and management of biliary diseases. KEY WORDS: Choledochal Cyst, gallbladder, Phrygian cap, biliary diseases. INTRODUCTION Gallbladder and associated biliary malformations are an uncommon entity – incidence varies, but the largest study conducted showed an incidence of around 0.15%1 . Although asymptomatic, they may also be associated with other systemic malformations. Biliary malformations are of great consequence to surgeons as they may be associated with complex malformations in the extra-hepatic biliary tree and its blood supply, making surgery difficult and dangerous2 . Pre- cholecystectomy imaging with modalities like MRCP has been employed only in a setting of suspected common bile duct stones in order to plan for a concurrent common bile duct exploration3 , however this has not been shown to be justified in all patient of suspected to have common bile duct stones, only those with specific risk factors4 . With intra-operative diagnosis being the only viable option, a thorough knowledge of anatomical variations, the differential diagnosis on imaging and a high index of suspicion is required to avoid intra- operative complications and their significant associated morbidity. This article discusses a case of septate gall bladder (known as ‘Phrygian cap’) mimicking a choledochal cyst. It also discusses the difficulty in pre and peri- operative diagnosis and management of biliary diseases. Case report
- 2. INTERNATIONAL JOURNAL OF MEDICAL AND APPLIED SCIENCES ISSN:2320‐3137 335 www.earthjournals.org Volume 2 Issue 3 2013 A 52 year old male presented to our hospital with a history of intermittent pain in the right upper abdomen since 6 months, continuous in nature, moderate in severity with no radiation in site. He also had frequent complaints of dyspepsia and pain was aggravated around 1 hour after food intake. He had no history of jaundice or fever. He had no similar complaints prior, no co-morbidities and was on no medications. He reported that he had a sedentary lifestyle, no history of smoking or alcohol intake. On examination, he was overweight, with a body mass index of 27. He was not jaundiced. Barring mild tenderness in the right hypochondrium, his abdominal examination was unremarkable. Bowel sounds were normal. Routine blood counts and liver function tests were normal and viral serology was negative. Ultrasonography of the abdomen showed an irregular, hyperechoic mass that appeared to be continuous with the lumen of the gallbladder. However there was no intrahepatic biliary radical dilatation. Contrast enhanced computerized tomography was performed for further delineation of the mass, which showed a cystic mass measuring 47x35mm occurring along the course of the cystic duct, suspected to be a choledochal cyst. No contrast enhancement of the mass was appreciated. For further clarity a magnetic resonance cholangiopancreaticography was performed, which showed cholelithiasis with chronic fibrosis of the gall bladder. No abnormality of the common bile duct was noted. Patient was planned for an open cholecystectomy with a common bile duct exploration if required. Intra-operatively the common bile duct was found to be normal and the gall bladder was found to be septate. Features were consistent with chronic cholecystitis and multiple gall stones of varying sizes were identified. No features of cholangitis were noted. Post-operatively, patient was stable and comfortable. Oral liquids were started on day 2. Abdominal drain was removed on day 4. He was discharged on day 6. He had no subsequent complications and has been asymptomatic on follow-up, 3 months later (Figure 1-7). Figure1: Ultrasound image showing mass in continuity with the gall bladder
- 3. INTERNATIONAL JOURNAL OF MEDICAL AND APPLIED SCIENCES ISSN:2320‐3137 336 www.earthjournals.org Volume 2 Issue 3 2013 Figure 2: CT showing dilated gallbladder Figure 3: MRI showing dilated mass arising from gallbladder fossa Figure 4: MRI showing dilated mass, possibly distended gallbladder or choledochal cyst Figure 5: MRCP suggestive of a large choledochal cyst in the gallbladder fossa
- 4. INTERNATIONAL JOURNAL OF MEDICAL AND APPLIED SCIENCES ISSN:2320‐3137 337 www.earthjournals.org Volume 2 Issue 3 2013 DISCUSSION Phrygian cap, or pseudo-duplication of the gall bladder, has an incidence of around 4% of the population5 . It is the most common anomaly of the congenital anomaly of the gall bladder and is a physiological variant of anatomy, most likely occurring as a result of an incompletely formed congenital septum6 . It is most commonly asymptomatic but may rarely be associated with right upper quadrant pain. Originally identified by Boyden in the year 1935, this anomaly so named because of its resemblance to the headdress worn by people from the ancient empire of Phrygia, located in what is now Turkey7 . He identified it as a normal variation which is detectable on imaging that may be misconstrued as a pathological finding on cholecystography and had to be distinguished from them. Imaging of Phrygian cap and other gall bladder variations can be by ultrasonography, computerized tomography, oral cholecystography, cholescintigraphy and magnetic resonance cholangiopancreaticography8 . Ultrasonography and computerised tomography often has poor resolution, MRCP and cholescintigraphy are the investigations of choice – MRCP has good resolution and cholescintigraphy can show delayed filling of the Phrygian cap area9 10 . Differential diagnosis includes diverticula, embryological folds, choledochal cysts, pericholecystic fluid collection, focal adenomyomatosis of the gall bladder and fibrous bands9 . Anatomical variations of the extra-hepatic biliary system may be detected pre- operatively, in which case they must be thoroughly evaluated, usually with an MRCP or a multi-slice contrast enhanced CT if MRCP is contra-indicated. Those variations picked up intra-operatively mandate common bile duct exploration to detect associated malformations that when missed cause inadvertent injury to the biliary tree11 . CONCLUSION Phrygian cap is the most common embryological gall bladder malformation that may present incidentally on imaging Figure 6: Intra-operative laparoscopic image of gallbladder with Phrygian cap Figure 7: Ex-vivo image of the gallbladder showing Phrygian cap
- 5. INTERNATIONAL JOURNAL OF MEDICAL AND APPLIED SCIENCES ISSN:2320‐3137 338 www.earthjournals.org Volume 2 Issue 3 2013 or during evaluation of biliary colic. When incidental, there is no indication for a cholecystectomy. Thorough pre-operative evaluation with MRCP is indicated to confirm the diagnosis and detect associated anomalies of the biliary tree. Intra-operatively, a common bile duct exploration is indicated if anatomy is not abundantly clear, especially during laparoscopic procedures. REFERENCES 1. Bronshtein M, Weiner Z, Abramovici H, Erlik Y, Blumenfeld Z. Prenatal diagnosis of gallbladder anomalies. Report of 17 cases. Prenat Diagn. 1993;13(9):851–86l. 2. M. Lamah, N. D. Karanjia, and G. H. Dickson, “Anatomical variations of the extrahepatic biliary tree: review of the world literature,” Clinical Anatomy, vol. 14, no. 3, pp. 167–172, 2001. 3. Bahram M, Gaballa G. (2010) 'The value of pre‐ operative magnetic resonance cholangiopancreatography (MRCP) in management of patients with gall stones', Int J Surg, 8(5), pp. 342‐5. 4. Nebiker CA, Baierlein SA et al (2009) 'Is routine MR cholangiopancreatography (MRCP) justified prior to cholecystectomy?', Langenbecks Arch Surg, 394(6), pp. 1005‐10. 5. van Kamp M. et al (2013) 'A Phrygian Cap', Case Rep Gastroenterology, 7(1), pp. 347‐351. 6 Alexander A. Deutsch, Dov Englestein, Maya Cohen (1986). 'Septum of the gallbladder, clinical implications and treatment', Postgraduate Medical Journal, 62(1), pp. 453‐456. 7. Boyden EA: The Phrygian cap in cholecystography: a congenital anomaly of the gallbladder. Am J Radiol 1935;33:589. Meilstrup JW, Hopper KD, Thime GA: Imaging of gallbladder variants. AJR Am J Roentgenol 1991;157:1205‐1208. 8. Koenraad J. Mortelé, Tatiana C. Rocha, (2006) 'Multimodality Imaging of Pancreatic and Biliary Congenital Anomalies gallbladder, clinical implications and treatment', Radiographics, 26(1), pp. 715‐731. 9. Smergel EM, Maurer AH: Phrygian cap simulating mass lesion in hepatobiliary scintigraphy. Clin Nucl Med 1984;9:131‐133. 10. J.‐F. Gigot, B. Van Beers, L. Goncette, et al., “Laparoscopic treatment of gallbladder duplication: a plea for removal of both gallbladders,” Surgical Endoscopy, vol. 11, no. 5, pp. 479–482, 1997. 11. Horattas MC. Gallbladder duplication and laparoscopic management. J Laparoendosc Adv Surg Tech A. 1998;8:231–5