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Missing the main actor: Intra-operative diagnosis of agenesis of the gallbladder - When late is not 'too' late
Giuseppe Portale, Antonio Mazzeo, Chiara Cipollari, Roberto Isoardi, Y Spolverato, Matteo Zuin, Valentino Fiscon
Department of General Surgery, Azienda Euganea ULSS 6, Cittadella, Italy
|Date of Submission||11-Nov-2021|
|Date of Acceptance||10-Jan-2022|
|Date of Web Publication||06-Jul-2022|
Department of General Surgery, Azienda Euganea ULSS 6, Via Casa di Ricovero, 40, 35013 Cittadella, Padova
Source of Support: None, Conflict of Interest: None
Agenesis of the gallbladder (AGB) without extrahepatic biliary atresia is a rare congenital disease. Ultrasound (US) examination can be misleading and reveal a contracted shrunken gallbladder when there is not any and the patient in most cases is taken to the OR for a standard cholecystectomy. We describe the case of a 54-year-old female with colicky right upper abdominal pain with nausea. US revealed a contracted scleroatrophic gallbladder and the patient was listed for laparoscopic cholecystectomy. At laparoscopy, despite careful search, the gallbladder was never visualised, and the suspicion of AGB was raised. An intra-operative cholangiography confirmed the hypothesis. The post-operative recovery was uneventful, and abdominal computed tomography scan failed to show the presence of gallbladder, therefore confirming the diagnosis of AGB. Lack of awareness of this condition among radiologists and surgeons is the main reason for unnecessary operations and potentially damages to the biliary tract.
Keywords: Agenesis of the gallbladder, gallbladder, intra-operative cholangiography, laparoscopic cholecystectomy
|How to cite this URL:|
Portale G, Mazzeo A, Cipollari C, Isoardi R, Spolverato Y, Zuin M, Fiscon V. Missing the main actor: Intra-operative diagnosis of agenesis of the gallbladder - When late is not 'too' late. J Min Access Surg [Epub ahead of print] [cited 2022 Aug 14]. Available from: https://www.journalofmas.com/preprintarticle.asp?id=350046
| ¤ Introduction|| |
Agenesis of the gallbladder (AGB) without extrahepatic biliary atresia is an exceedingly rare congenital disease, with a prevalence of 0.01–0.075% and 0.035% and 0.3% in clinical and autoptic scenarios, respectively. Even if the gallbladder is missing, at least 50% of patients with AGB report symptoms which may mistakenly be considered as suggestive of biliary colicky pain. Further, ultrasound (US) examination can be misleading and reveal a contracted shrunken gallbladder when there is not any and the patient in most cases is taken to the OR for a standard cholecystectomy. A pre-operative diagnosis of AGB is made in <2%–3% of the cases. Therefore, nearly all cases of AGB are diagnosed intraoperatively or postoperatively, when AGB is suspected in the OR and further radiological examinations are planned after explorative laparoscopy.
| ¤ Case Report|| |
We describe the case of a 54-year-old female with colicky right upper abdominal pain with nausea. US revealed a contracted scleroatrophic gallbladder and the patient was listed for laparoscopic cholecystectomy. At laparoscopy, despite careful search, the gallbladder was never visualised, and the suspicion of AGB was raised. An intra-operative cholangiography confirmed the hypothesis [Figure 1]a and [Figure 1]b. The operation was completed. The postoperative recovery was uneventful, and the abdominal computed tomography (CT) scan failed to show the presence of the gallbladder, therefore confirming the diagnosis of AGB [Figure 2].
|Figure 1: Intra-operative cholangiography confirming normal hepatic duct/common bile duct, absence of gallbladder and cystic duct, confirming the hypothesis of agenesis of the gallbladder. A clamp was placed in the common bile duct (a) and then released (b)|
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|Figure 2: Post-operative contrast-enhanced abdominal computed tomography scan (portal venous phase) confirming the absence of the gallbladder in its fossa|
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| ¤ Discussion|| |
AGB is a rare congenital anomaly resulting from incomplete development of the gallbladder and cystic duct from the hepatic bud during the 3rd and 4th week of gestation. In particular, if inappropriate migration of the developing gallbladder explains ectopic gallbladder, failure in completing the vacuolisation process by the 7th week of gestation with consequent absent growth of a lumen leads to AGB. The etiology of AGB is unknown but it is usually considered to have a sporadic occurrence.
It was first reported by Lemrey over three centuries ago, in 1701. Females are usually affected more than males, with a ratio of 3:1, even if cases found during autopsies have a male-to-female gender ratio of 1:1. Since then Lemrey's report, <600 cases have been described, mostly as single case reports.
Generally, AGB may present in three different settings: (1) symptomatic patients (50%); (2) asymptomatic patients (35%); (3) in children in combination with other malformations (15%). Patients in the first group usually present with symptoms (including nausea, dyspepsia, colicky right upper quadrant pain) which make a differential diagnosis between AGB and several biliary tract diseases challenging. It is not easy to distinguish if a colicky pain of a patient is due to AGB or a chronic inflammation of the gallbladder. Unfortunately, AGB has no pathognomonic symptoms to rely on, which makes diagnosis a challenge for clinicians. The explanation for colicky pain in patients who do not have a gallbladder-and as a consequence therefore cannot have an inflamed gallbladder-is probably biliary dyskinesia. This explains why patients with AGB after the diagnosis is made and the colicky pain persists may benefit from muscle relaxants, as in patients with biliary dyskinesia. It has been estimated that up to 50% of these patients will suffer from lithiasis of the biliary tract, due to primary duct stones. Asymptomatic AGB patients, representing one-third of the cases, are discovered incidentally at autopsy. The third group, 1 out of 6 cases of AGB, presents associated multiple foetal anomalies (including tetralogy of Fallot or agenesis of the lungs) and usually these patients die in the perinatal period.
Pre-operative diagnosis is challenging and made in a minimal percentage of cases with AGB. The gallbladder is usually reported as contracted or atrophic and the presence of a peritoneal fold or of perihepatic tissue may lead the radiologist to a wrong diagnosis of cholelithiasis. The lack of awareness of radiologists (as well as surgeons that later on take AGB patients to the OR) for this disorder, the rarity of the condition itself, the fact that US examination is highly operator-dependent and that many AGB patients are symptomatic lead to an erroneous interpretation of chronic cholecystitis or scleroatrophic gallbladder even when this is not visualised. This, in turn, explains why nearly all the correct diagnoses of AGB are delayed to the intra-operative scenario. In 2010 Malde suggested an algorithm for all cases of inconclusive US studies of the gallbladder (gallbladder not identified) or in case of possible chronic cholecystitis (shrunken gallbladder). He recommended further radiological investigation with cholangiography, CT scan, endoscopic retrograde cholangiopancreatography (ERCP) or endoscopic US to better ascertain the presence or absence of the gallbladder. In fact, the shrunken, contracted gallbladder is the US report most commonly seen in patients later found to be affected by AGB.
As already said above, in virtually all cases of AGB the diagnosis is the OR, therefore, it is mandatory to properly manage an intra-operative suspicion of this rare congenital disorder. In particular, once the suspicion of AGB is raised, it is important to avoid excessive dissection of the biliary tract, in search of the gallbladder or of the cystic duct. This brings a high risk of intra-operative common bile-duct iatrogenic injury. Furthermore, lack of awareness of AGB leads some surgeons to quickly convert to an open procedure to better search for the gallbladder, with the wrong idea the impossibility to visualise the gallbladder is an issue related to the laparoscopic technique, rather than to a congenital disorder. In effect fact laparoscopy itself allows a thorough and high-quality visualisation of the hepatic area, especially nowadays that high-definition screens are widely available and 3D and 4K video systems are used by many surgeons. It is not a matter of unclear visualisation due to minimally-invasive technology. Conversion to open surgery clearly exposes the patient to the morbidity associated with a laparotomic incision, with no advantages for the diagnosis. After the search in the most common sites for ectopic gallbladder, including retropancreatic, retroduodenal, retroperitoneal, or within the leaves of the lesser omentum or within the falciform ligament, an intra-operative cholangiography, as in the case of our patient, can easily confirm the suspicion of AGB. A normal biliary tree is depicted with no gallbladder and cystic duct, and this avoids hazardous and unnecessary further exploration. If the patient has already undergone an un-necessary operation, at least surgeons should avoid further, uncalled, biliary tract lesions. Intra-operative diagnosis of AGB is a 'late' diagnosis but it is not 'too late' if at least damage of the main bile duct is avoided. Abdominal CT scan and ERCP have been suggested as useful post-operative modalities to ascertain AGB suspected at laparoscopy, avoiding laparotomy and/or extensive dissection which should always be discouraged. If symptoms continue in the post-operative period, they can be treated as in cases of biliary dyskinesia, but if the patient remain asymptomatic, no further examination is required: the patient is healthy.
| ¤ Conclusions|| |
Lack of awareness of this condition among radiologists and surgeons is the main reason for unnecessary operation and potentially damages to the biliary tract. Instead, clinicians should be aware of the existence of AGB and request further examinations in patients with scleroatrophic and/or contracted gallbladder. In case the gallbladder is not found intraoperatively, even after search for ectopic sites, AGB should be suspected. Thorough dissection is not warranted because this may cause injury to the biliary tree. An intra-operative cholangiogram may easily confirm AGB. CT scan or ERCP may also be helpful to confirm the diagnosis of AGB in the post-operative setting.
An informed consent was obtained from the patient, as requested by the Ethical Committee.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that name and initial will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Research involving human participants and/or animals
This study did not involve animals.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| ¤ References|| |
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[Figure 1], [Figure 2]