Ciliated cyst of the gallbladder: A new case and literature review.

INTRODUCTION: The ciliated cyst is a rare anomaly that develops from the anterior primitive intestine. Its localization is essentially supra diaphragmatic. It's localization in the gallbladder is very uncommon. CASE REPORT: We report the first case in Tunisia of a ciliated cyst of the gallbladder in a 34 years old woman who was operated for a gallbladder stone and in whom the discovery of a cystic mass attached to the neck of the gallbladder was preoperatively. The pathologic study did conclude to a ciliated cyst. DISCUSSION: The ciliated cyst of the gallbladder is a benign congenital lesion that develops from the anterior primitive intestine. The most frequent clinical symptom is abdominal pain and the median age is 45 years old. The most frequent location is the neck. This pathology affects women more than men. Abdominal ultrasound is not very specific exam and describes the ciliated cyst as a cystic lesion often anechogenic and sometimes hyperechogenic. The CT-scan as well as the abdominal MRI are very helpful of the diagnosis. The positif diagnosis of ciliated cyst is histological.
CONCLUSION: The recommended current treatment for this rare pathology is surgery and it consists of a celioscopic cholecystectomie. The place of conservatory treatment hasn't been established due to the rarity of described case and the possibility of degeneration.

Introduction

The ciliated cysts is a rare congenital lesion developed from the primitive anterior intestine who is the origin of the oropharynx, the bronchio-pulmonairytractus, the esophagus, the stomach, the duodenum, the liver, bile ducts and the pancreas. The ciliated cyst is essentially supra-diaphragmatic and located in the bronchial tree, the esophagus, the mediastinum and sublingual. When the cyst is infra diaphragmatic, it’s usually located in the liver especially in medial segments (IV,V,VIII). In can be located in the pancreas as well. It’s location in the gallbladder is exceptional. Therefore, few cases were published. To our knowledge, only 15 cases were described in literature. We report the first case diagnosed in Tunisia and the 16th in the world with a review of literature. This work has been reported in line with the SCARE criteria [1].

Observation

A 34 years old patient with no medical history was admitted for an upper right quadrant pain. The clinical examination found right upper quadrant tenderness. Blood test showed no anomalies and the abdominal ultrasound showed a gallbladder stone with thin intra and extra hepatic bile and hilar adenopathy. The abdominal computed tomography reveled unilocular cystic lesion attached to the neck of the gallbladder (Fig. 1). The patient was operated via a right subcostal incision. The paraopeartively exploration found a gallbladder with thin walls and a 2 cm round cystic mass attached to the neck of the gallbladder. She had a cholecystectomy and the follow ups were simple. The anatomopathologic study found a cystic lesion with thin walls covered with ciliated epithelium which lays on a connective tissue that contains smooth muscle fibers (Fig. 2, Fig. 3). The content of the cyst was a mucoid liquid and there was no communication between the cyst and the gallbladder lumen. There was no sign of degeneration.

Fig. 1

A unilocular cystic lesion attached to the neck of the gallbladder.

Fig. 2

the cyst was lined by pseudostratified ciliated columnar epithelium.

Fig. 3

A low power view of the complex columnar cellined gallbladder mucosa at the top of the field, and at the lower edge a single layer of ciliated epithelium.

Discussion

Cystic lesions of the gallbladder are rare and they can originally congenital, acquired or neoplastic [2]. The ciliated cyst of the gallbladder is a benign congenital lesion that develops from the anterior primitive intestine. Cysts are essentially supradiaphragmatic in the bronchial tree, the esophagus, the mediastinum and sublingual. Under the diaphragm, the ciliated cysts are in the liver and develop from intrahepatic bile ducts. Its location in the gallbladder is exceptional.

There is many hypotheses that describes the pathogenesis of ciliated cysts [3]. Kakitsubata described in 1995 the first case of gallbladder cyst who was covered with cylindrical ciliated epithelium and he named it “Epithelial cyst of the gallbladder” [4]. In 2000, another case was reported by Nam under the title of “Ciliated foregut cyst of the gallbladder” [5]. Afterward, only twelve cases were published.

Table 1, Table 2 present a resume of literature review that permits to deduce the epidemiological and pathologic characteristics related to this entity.

Table 1

Reported case of cyst in gallbladder.

Author	Sex	Age	Clinic	Imaging	Location	Size (cm)
Kakisubata [4]	M	71	nil	Anechogenic cystic lesion of the gallbladder	body	unspecified
Benlolo [9]	F	75	unspecified	unspecified	fundus	3
Nam [5]	F	36	nil	Anechogenic cystic lesion and hypodense in CT-scan	fundus	1.5
Chatelain [10]	F	75	unspecified	Echogenic cystic lesion	unspecified	3.3
Hirono [11]	F	43	nil	Cystic mass	neck	2.5
Muraoka [12]	F	36	nil	Cystic lesion with hyperechogenic focal points, tumoral aspect in CT-scan	body	2.4
Bulut [13]	F	41	Right upper quadrant pain	Benign cystic lesion	neck	3.5
Kim [14]	M	63	nil	Cystic mass	unspecified	2
Tuncyurek [6]	F	42	Right upper quadrant pain	Anechogenic cystic lesions with hyperechogenic focal points	body	0.7
Angot 2013	M	45	Right upper quadrant pain with fever	Cholecystitis with gallbladder sludge	body	3
Giacoustidis [15]	F	29	Epigastric pain with diarrhea and vomiting	Cystic mass adjacent to the gallbladder et al a cholangio MRIa pouch connected to the cystic duct containing debris	neck	3
Lee [16]	M	61	Right upper quadrant pain	Gallbalder stone	fundus	2.7
Agarwal 2016 [17]	M	9	Right upper quadrant pain with vomiting	Diverticula or duplication of the gallbladder	neck	3
Hwang [18]	F	39	Right upper quadrant pain	Cystic lesion	neck	3.5
Eun Han [19]	F	20	Right upper quadrant pain	Cystic lesion attached to the neck of the gallbladder	neck	1.6
Our case	F	34	Right upper quadrant pain	Gallbladder stone	neck	3

Table 2

Pathologic characteristics of ciliated cysts.

Author	Locularity	Cyst content	Cyst wall	Malignity
Kakisubata [4]	Unspecified	Unspecified	Unspecified	Unspecified
Benlolo [9]	Unilocular	Dark brown	Unspecified	Unspecified
Nam [5]	Unilocular	Mucoid	Thin	No
Chatelain [10]	Unspecified	Mucoid	Unspecified	Unspecified
Hirono [11]	Unilocular	Dark brown	Unspecified	Unspecified
Muraoka [12]	Unilocular	Mucoid	Thin	Unspecified
Bulut [13]	Unilocular	Mucoid	Unspecified	Unspecified
Kim [14]	unspecified	Milky	Unspecified	Unspecified
Tuncyurek [6]	unilocular	Dense Mucoid	Unspecified	No
Angot 2013	unilocular	Mucoid	Thin	No
Giacoustidis [15]	unilocular	Gelatinous grey	Unspecified	Unspecified
Lee [16]	unilocular	gelatinous	Thin	No
Agarwal [17]	unilocular	Mucoid	Thin	No
Hwang [18]	unilocular	Mucoid	Thin	No
Eun Han [19]	unilocularunilocular	Mucoid	Unspecified	Unspecified
Our case	unilocular	mucoid	Thin	No

This pathology affects women more than men (10 women to 5 men) with a sex ratio of 2. The median age is 45 years old with extreme ages of 9–75 years old. Only one case of an infant was noted. The median size of the cyst is 2.5 cm with extremes of 0.7–3.5 cm. The most frequent location is the neck, it was observed in 6 cases followed by the body with 4 observed cases. In three patients the cyst was located in the fundus. The location wasn’t mentioned in 2 cases. The most frequent clinical symptom is abdominal pain which was noted in 6 patients. In 5 cases, the discovery of the gallbladder cyst was incidental. On patient had cholecystitis and another one had epigastric pain and vomiting. In two observations, clinical symptoms weren’t mentioned. All patients underwent radiologic exams preoperatively. In two thirds of cases (11 cases), ultrasound revealed a cystic lesion. In one case, ultarsound revealed gallbladder stones and in another an acute cholecystitis. The aspect of diverticula or duplication of the gallbladder was observed in one case. In our patient, the gallbladder lesion was mistakenly interpreted as an adenopathy while CT-scan confirmed the presence of an aspect of a cyst. In 100% of cases, the cyst was unilocular. In the majority of cases, the cyst content was mucoid (9 patients) while gelatinous in 2 patients, dark brown in 2 others and milky in one patient.

The positif diagnosis of ciliated cyst is histological. On an anatomopathogic level, the cystic lesion is caraterized by a thin wall covered with a cylindrical ciliated mucus-secreting epithelium which lays on connective tissue containing smooth muscular fibers. There is now communication between the cyst and the gallbladder lumen.

Concerning imaging techniques, abdominal ultrasound is the fist most sought exam in exploring hepatic and biliairy diseases. Although it is not very specific, it describes the ciliated cyst as a cystic lesion often anechogenic and sometimes hyperechogenic. In our patient, the abdominal ultrasound didn’t establish the diagnosis. When the lesion is hyperechogenic the diagnosis of a neoplastic origine can be evoqued [6] especially when the cysts size is exceeds 1 cm. In this case the CT-scan as well as the abdominal MRI are very helpful of the diagnosis. The ciliated cyst is often observed in hypersignal T1 and T2 and certain cysts can appear in hyposignal T1. In fact, the contrast depends in parts on the viscosity of the cyst content and in other part on the presence or absence of calcium and cholesterol crystals.

Treatment is based on the cholecystectomy [7]. Although, Tuncyurek proposed a close surveillance when the cyst is silent [6]. The systematic cholecystectomy seems the most wise attitude since the distinction between benign and malignant lesion is difficult on imaging techniques and all the more that cases of malpigien metaplasia and epidermoid carcinoma that occurred on hepatic ciliated cysts were observed [8]. Even though no case of malignat transformation of the ciliated gallbladder cyst was described, it’s convenient to look thoroughly into the resection specimen.

Conclusion

The ciliated cyst of the gallbladder is a congenital rare cyst. The preoperative diagnosis is still difficult to establish despite the progress of imaging techniques. The positive diagnosis is established on anatomopathogic data. The recommended current treatment is surgery and it consists of a celioscopiccholecystectomie. The place of conservatory treatment hasn’t been established due to the rarity of described case and the possibility of degeneration.

Conflicts of interest

There aren’t any conflicts of interest for all authors.

Funding

We have no sources of funding.

Ethical approval

Ethical approval has been exempted by our institution.

Consent

Our study is a case report. The consentement of the patient has been obtained.

Author contribution

Wissem Triki: development of the idea and writing of the article.

Oussema Baraket and Ahmed Itami: bibliographic search Ines Marzouk: provision and interpretation of radiological images.

Abdelmajid Baccar: provision and interpretation of anatomopathologic images.

Sami Bouchoucha: final revision.

Registration of research studies

None.

Guarantor

Triki Wissem.