CASE REPORT

Unsuspected Phrygian Cap in a 71-Year- Old Man

Vitorino Modesto dos Santos, Lister Arruda Modesto dos Santos, Adriano Corona Branco e Juliano Fernandes da Costa DOI - 10.14242/2236-5117.2016v51n34a292p287

ABSTRACT Vitorino Modesto dos Santos. Physician, PhD, internal medicine, Hospital Phrygian cap anomaly occurs in 4% of the gene- das Forças Armadas (HFA) and Universidade Católica de Brasília (UCB), Brasília, Distrito Federal, Brazil ral population due to a fold in the gallbladder wall. Cholecystitis with gallstones is a poten- Lister Arruda Modesto dos Santos. Physician,general surgery, State tial complication, which can be associated with Workers Hospital (HSE), São Paulo-SP, Brazil cholestasis. An asymptomatic case of this con- Adriano Corona Branco. Physician,general surgery, State Workers Hospital dition is herein described in a 71-year-old man. (HSE), São Paulo-SP, Brazil

Diagnosis was incidental, based on the images Juliano Fernandes da Costa. Physician, general surgery, State Workers of an ultrasound scan performed during a rou- Hospital (HSE), São Paulo-SP, Brazil tine checkup. Because polyps were suspected, he underwent a prophylactic laparoscopic cho- Correspondence: Prof. Dr. Vitorino Modesto dos Santos. Armed Forces Hospital. Estrada do Contorno do Bosque s/n, Cruzeiro lecystectomy. A microscopic study of the gall- Novo, ZIP70658-900, Brasília-DF, Brazil. bladder detected cholecystitis with cholelithiasis and ruled out the presence of polyps. The role of Phone: #55-61 39662103. Fax: #55-61 32331599. polyps and of routine gallbladder histopathology Internet: [email protected] in cancer is discussed.

Key words: Gallbladder morphology, gallbladder polyp, The authors have no conflict of interests to disclaim. hepatobiliary imaging, Phrygian cap

RESUMO e foi descartada a presença de pólipos. Comenta-se o papel de pólipos e de exames histopatológicos da vesí- Barrete frígio assintomático da vesícula biliar em cula no contexto de câncer. um homem de 71 anos de idade Palavras-chave. Vesícula morfologia; vesícula pó- Anomalia em barrete frígio ocorre em 4% da população lipo; imagem hepatobiliar; barrete frígio em virtude de dobra na parede da vesícula biliar. A co- lecistite com cálculos tem sido uma complicação que INTRODUCTION pode se associar com colestase. Um caso assintomático dessa condição é aqui descrito em um homem com 71 Phrygian capgallbladder is anuncommon con- anos etários. O diagnóstico foi incidental, com base em genital anomaly that may pose difficulties for imagens de ultrassonografia realizada durante exame diagnosis by abdominal imaging study.1-4 This de rotina. Considerando-se a suspeita de pólipos, foi abnormality was first described by Boyden in submetido a colecistectomia profilática. Um estudo ve- 1935,2-5 and its estimated incidence is 4%.2-4 sicular microscópico detectou colecistite com colelitíase This developmental defect appears as folded

287 Brasília Med 2014;51(3.4):287-290 Prof. Dr. Vitorino Modesto dos Santos • Unsuspected Phrygian cap gallbladder in a 71-year-old man

gallbladder and results from kinking between normal. An abdominal ultrasonography showed the body and the fundus or the infundibulum. a Phrygian cap-shaped gallbladder with images These deformities predispose to cholelithiasis of lithiasis (up to 9.4 mm), and two fixed images due to biliary stasis in the corpus and fundus (1.8 mm and 2.1 mm) similar to polyps. Moreover, of the gallbladder.1 Phrygian cap anomaly by it- there was a thickened fold neatly divided, mimi- self does not suggest invasive intervention,2-4 cking the aspect of bilobed gallbladder (figure 1). but the suspicion of associated gallbladder Although the risk of cancer development was con- polypsmay indicate the needfor surgical mana- sidered low, a laparoscopic cholecystectomy was gement.7-9 The malignant potential of 10 mm performed (figure 2). He was discharged from the polyps is high, but the smallerones must also be hospital in the first postoperative day. The excised monitored.6,8 Male patients older than 45 years gallbladder measured 7.4 cm vs 6.3 cm, anditswall presenting with gallbladder polyps10 mm or was 4 mm thick.The mucosal surface was finely greater in diameter and gallbladder wall thick- granular and microlithiasis was seen, but polyps ness greater than 5 mm should be considered at were not found. Histopathology showed an inflam- high risk of local cancer.7-10 Early-stage gallbla- matory process characterized by moderate muco- dder cancer may be mistaken for benign polyps sal lymphoplasmacytic infiltrate, areas of fibrosis in imaging evaluations,7 and this malignancy between muscle bundles, and congested capillaries. hasa poor prognosis because it may be asymp- The inner surface of the mucosa was largely flat- tomatic until advanced stages.6,9,10 tened and presented with some squamous areas. Taken together, these features were consistent with the diagnosis of chronic calculous cholecysti- tis. The patient resumed his habitual activities in a short span of time and persists asymptomatic.

Figure 1. A and B: ultrasonographic scans of a typical Phry- Figure 2. Intraoperative laparoscopic view of the Phrygian gian cap gallbladder, showing a the typical folding in the fun- cap gallbladder, emphasizing its long typical fold (arrows). dus of the organ and images of gall stones (arrows); C and D: images mimicking bilobed gallbladder; E and F: evidence of te tic eband fed imaes suestive of polps arros DISCUSSION

CASE REPORT The gallbladder and the originate from the cranial bud of the hepatic diverticulum, A previously healthy 71-year-old man was sub- which develops on the ventral wall of the pri- mitted to abdominal ultrasonography, which was mitive midgut on the fourth week of gestation.3 included in his routine yearly medical evaluation. However, the gallbladder gradually acquires the There were neither symptoms nor past diseases aspect of a pyriform sac after the twelfth week.3 and, except for cholelithiasis, there was no fami- Disorders of intra-uterine development can cause lial history of diseases. Physical examination wa- gallbladder anomalies.3 Folding or bending of the sunremarkable, and routine laboratory data were fundus over the body gives origin to a deformity

288 Brasília Med 2014;51(3.4):287-290 CASE REPORT

called Phrygian cap gallbladder, similar to a head- type ofcancer.9 Cancer risk factors also include gear used by liberated Greek slaves of an ancient chronic cholecystitis, cholelithiasis, and porce- Turkish region.2-5 Phrygian cap gallbladder is a ra- lain gallbladder.10 Gallbladder adenocarcinoma re congenital anomaly whose incidence is proba- is the most frequent cancer of the bly underestimated. This condition is more often worldwide and is incidentally found during or af- incidentally diagnosed during cholecystectomies ter laparoscopic cholecystectomy in up to 3.3% or autopsies.3,4 However, if associated with cho- of cases.10 Sujata et al. reviewed thepathological lelithiasis or cholecystitis, the chances of clinical features of 622 surgically removed diagnosis increase.2 The diagnosis of Phrygian in a period of six-and-a-half years to cure benign cap gallbladder herein described was incidental cholecystopathy and they revealed six inciden- in a routine check up. The anomaly was charac- tal carcinomas.10 All adenocarcinomas affected terized by observing the classical images of the females between 38 and 85 years of ageand wi- ultrasound scan, and the defect was further con- tha mean age of 53 (±17.6) years.10Terada also firmed on evaluation of the specimen removed reviewed the histopathological findings of 540 by laparoscopic cholecystectomy. Surgery is not gallbladders surgically removed during 10 years recommended for incidentally found Phrygian in patients aged between 18 and 93 years, with cap anomaly without gallbladder disease, but the amean age of64.75 (±14.4) years. The female:male patient had asymptomatic chronic cholecystitis ratio was1.53:1, and 12 invasive adenocarcino- with cholelithiasis and suspected polyps. An ini- mas were detected- seven of them were inciden- tial concern was about bilobed gallbladder with tally diagnosed.11 a Phrygian cap,which is exceedingly rare.2 Other hypotheses were pouch or diverticulum, duplica- The cohort histopathological study of gallblad- tion, septate and hour glass gallbladder.2-4 der specimens conducted by Siddiqui et al. inclu- ded 220 patients aged between 19 and 80 years, Images of Phrygian cap gallbladder may also 88% females, who were submitted to cholecys- mimic the presence of a tumor in the .2-4 tectomy for symptomatic gallstones.12Six (2.8%) Nevertheless, these differential diagnoses were gallbladder adenocarcinomas and one benign all ruled out by features of gross pathologic study. polyp were incidentally diagnosed in association High-resolution ultrasonography has been com- with cholelithiasis and cholecystitis.12 monly used to detect the anomaly, in addition to computed tomography, magnetic resonance, Findings like these strongly highlight the role of retrograde cholangiography, orcholescintigra- microscopic gallbladder evaluation, and this topic phy.2-4 Ultrasonography usually shows a fixed deserves more attention in discussions about the hyperechoic mass, and massessmaller than 10 mm detection of early adenocarcinomas.12 Otherwise, may be undetected.8 It is worth mentioning that minor premalignant conditions and stage 1 adeno- some of the septa of Phrygian cap gallbladders carcinoma will be underdiagnosed.12 may be mistaken for polyps,3 but agreater num- ber of asymptomatic gallbladder polyps has been In conclusion, Phrygian cap gallbladder is usually found by high-resolution imaging.8 asymptomatic and unsuspected, but manifestations of associated cholecystitis and cholelithiasis often Polyps can be neoplastic or non-neoplastic, and lead to incidental diagnosis. There is no consensus cholesterol represents the most common varie- about prophylactic cholecystectomy in patients ty.8 Zhu et al. reviewed data from 4014 patients withsuspected images of small polyps, which are older than 44 years who underwent cholecys- not considered risk factors for gallbladder adeno- tectomy for benign diseasesand found 29 (1.4%) carcinomas. However, a major concern is about the presenting with incidental gallbladder cancer.9 choiceof surgical management for asymptomatic Moreover, they concluded that polyps greater patients, particularly males over 50 years of age and than 12 mm were a predictive factor for this presenting associated gallbladder polyps. Polyps

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greater than 10 mm in diameter and wall thickness 6. Hundal R, Shaffer EA. Gallbladder cancer: epidemiology greater than 5 mm may predict gallbladder carci- and outcome. Clin Epidemiol. 2014;6:99-109. nomas. Considering that gallbladder cancer is more often advanced and unresectable at time of diagno- 7. Sarkut P, Kilicturgay S, Ozer A, Ozturk E, Yilmazlar T. sis, a possible additional concerncould bewhether Gallbladder polyps: factors affecting surgical decision. to perform cholecystectomy for polyps of smaller World J Gastroenterol. 2013;19:4526-30. sizes. The authors also agree with the protocol of routine histopathology of all gallbladder specimens. 8. Yadav SP, Jategaonkar, Bijlani M. Gallbladder polyps: an ambiguous cause of biliary colic. Ann Med Health Sci REFERENCES Res. 2014;4:S332-3.

1. de Csepel J, Carroccio A, Pomp A. Soft-tissue images. 9. Zhu JQ, Han DD, Li XL, Kou JT, Fan H, He Q. predictors of “Phrygian cap” gallbladder. J Can Chir. 2003;46(1):50-1. incidental gallbladder cancer in elderly patients. Hepato- biliary Pancreat Dis Int. 2015;14:96-100. 2. Kannan NS, Kannan U, Babu CPG. Congenital bilobed gallbladder with Phrygian cap presenting as calculus 10. Sujata J, SR, Sabina K, MJ H, Jairajpuri ZS. Incidental gall cholecystitis. J Clin Diag Res. 2014;8(8):ND05-6. bladder carcinoma in laparoscopic cholecystectomy: a report of 6 cases and review of the literature. J Clin Diag 3. Rafailidis V, Varelas S, Kotsidis N, Rafailidis D. Two con- Res. 2013;7:85-8. genital anomalies in one: an ectopic gallbladder with Phygian cap deformity. Case Rep Radiol. vol. 2014, Article 11. Terada T. Histopathologic features and frequency of gall ID 246476, 4 pages, 2014. bladder lesions in consecutive 540 cholecystectomies. Int J Clin Exp Pathol. 2013;6:91-6. 4. van Kamp MJS, Bouman DE, Steenvoorde P, Klaase JM. A Phrygian cap. Case Rep Gastroenterol. 2013;7:347-51. 12. Soddiqui FG, Memon AA, Abro AH, Sasoli NA, Ahmad L. Routine histopathology of gallbladder after elective 5. Boyden EA. The Phrygian cap in cholecystography: a congen- cholecystectomy for gallstones: waste of resources or ital anomaly of the gallbladder. Am J Radiol. 1935;33:589-90. ausifed ac B Surg. 20112.

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